The Journal of Pediatrics

Blame inappropriate implementation for failure of intrapartum antibiotic prophylaxis for group B Streptococcus

Sarah S. Long — 2010-03-01

In this issue of The Journal, Blaschke et al from Salt Lake City, Utah, provide the case that was bound to happen. With increasing resistance of group B Streptococcus (GBS) to erythromycin and clindamycin, and too-quick, too-easy reflex to substitute clindamycin for penicillin or ampicillin when mother has a history of penicillin “allergy,” the math was going to add up to transmission and disease due to a clindamycin-resistant GBS in a newborn. Blaschke et al provide the case—an “old fashioned” one—of severe early-onset disease in an otherwise healthy term infant, whose mother received IAP with clindamycin.

A new concern—in utero iron status

Alan H. Jobe — 2010-03-01

Clinicians caring for preterm infants routinely evaluate the hematocrit/hemoglobin status at birth, track hematocrit/hemoglobin with postnatal age, transfuse as necessary, and supplement with oral iron. Questions remain about how much transfusion is “necessary” and when and how much iron to supplement. In this issue of The Journal, Amin et al have added a new wrinkle to concerns about iron status. They used cord blood ferritin measurements to identify fetal iron status of infants born preterm. Of 80 infants born between 27 and 33 weeks gestation, 44% had latent iron deficiency, which correlated with abnormal auditory neural maturation – a window to brain maturation. Iron deficiency is well known to be a modulator of brain development, and some prenatal conditions (maternal iron deficiency, maternal diabetes, maternal smoking, and pregnancy-induced hypertension) are linked to fetal iron deficits at term. This paper now identifies a new concern – latent iron deficiency in preterms at birth. Should this deficiency as measured by blood ferritin levels be quickly corrected after birth? Most preterm infants do not end up with hearing deficits as children, but the abnormal auditory maturation may simply be an indicator of more generalized abnormalities in brain maturation.

Pertussis compared with viral respiratory tract illnesses in neonates

Sarah S. Long — 2010-03-01

Using polymerase chain reaction techniques to confirm the diagnosis of pertussis and a variety of tests to confirm the diagnosis of viral respiratory tract infections in neonates, clinical features of the two groups were studied retrospectively. Comparing 33 cases each of pertussis and non-pertussis viral disease, investigators found certain symptoms (paroxysmal cough, apnea, cyanotic spell) and severe course (prolonged hospitalization, need for oxygen, and use of respiratory supportive care upon discharge) more common in pertussis cases. An additional important clue to the microbiologic diagnosis was probable acquisition – from ill parent (82% for pertussis vs. 50% for non-pertussis cases) and from ill sibling(s) (30% for pertussis vs. 75% for non-pertussis cases). Thinking of the diagnosis of pertussis in the absence of a whoop is a critical first step in neonates in order to anticipate a prolonged and complicated course for which supportive care may be lifesaving, including mechanical ventilation when needed and consideration of leukopheresis in those who develop critical pulmonary hypertension.

Missed opportunities?

Thomas R. Welch — 2010-03-01

The Journal has published a number of studies related to diabetic ketoacidosis (DKA) in the past few years; most of these have made reference to prevention as the best strategy for avoiding the significant morbidity (and occasional mortality) consequent to DKA.

Relapsing polychondritis in childood: rare but important

Sarah S. Long — 2010-03-01

French investigators report a case series with detailed clinical information and longterm follow-up of 10 children with relapsing polychondritis (RP). They also report a systematic review of other pediatric cases and compare the clinical characteristics of RP in children with those of adults (in whom disease is more common). Together these data provide a snapshot of childhood presentation and complications of RP, which in most ways looks like adult-onset disease. But the authors point out a few important clinical points. Chondritis is the heralding event in most patients. Children frequently have a family history of autoimmune disorders. There is a long delay in diagnosis in children, likely related to lack of familiarity of pediatricians with the entity. Cardiac complications may be under-recognized in children, and were fatal in one French adolescent. The authors also report that despite multiple episodes of inflammatory chondritis, linear growth appears to be unimpaired in children with RP – a finding that provides food for thought about cartilage versus bone health, local versus systemic inflammation, and their impact on growth.

Clostridial spores in powdered infant formula

Sarah S. Long — 2010-03-01

Infant botulism, the intestinal toxemia form of botulism, most commonly follows unavoidable exposure to spores in the environment, such as through contact with dust or soil. During a transient period in infancy, gut flora may not inhibit intestinal outgrowth of ingested clostridial spores. Clostridia then can replicate and produce toxin that is absorbed and causes neuromuscular blockade. Until the practice of avoiding the feeding of honey to infants under 1 year of age, infant botulism in California was highly associated with infants consuming honey that contained clostridial spores.

Evaluation of the child who presents with coughing

Robert W. Wilmott — 2010-03-01

Coughing is the most common presenting complaint to pediatricians in the United States, and the possible etiologies vary by season and the age of the child. The topic of coughing in children, including diagnosis and treatment, is the subject of a Medical Progress report by Goldsobel and Chipps in this issue of The Journal.

2010-03-01

Editorial Board

2010-03-01

Information for Readers

2010-03-01

e-Professionalism: Challenges in the Age of Information

2010-03-01

Recent advances in technology have revolutionized the medical environment and expanded the reach of communication both within and outside the medical community. Clayton Christensen, a noted professor at Harvard Business School, has discussed the concept of “disruptive technologies” and their role in creating organizational change. “Health care may be the most entrenched, change-averse industry in the United States,” according to Christensen. The age of information clearly has affected the culture of medicine in ways that we are just beginning to understand. Never before have there been so many ways to communicate. In the past, we were limited to face-to-face conversations, postal mail, telegrams, and telephone calls. Physicians communicated about their credentials through listings in the telephone book and in local medical society publications, or by hanging a shingle outside the office.

Role of Intervention Strategies for At-risk Preterm Infants

Arend F. Bos — 2010-03-01

Despite increased survival rates of preterm infants, the prevalence of developmental disabilities in preterm survivors is still high. Although the rates of major handicaps have remained relatively constant in the last decade, the prevalence of milder dysfunctions seems to be increasing. Cognitive, behavioral, and mild motor problems without major motor deficits are now the most dominant neurodevelopmental sequelae in children born preterm. These problems include learning disabilities, borderline to low IQ scores, attention-deficit, and specific neuropsychological deficits affecting visuomotor integration and executive function. These abnormalities occur in >50% of children born preterm with very low birth weight (VLBW; < 1500 g) and often do not occur in isolation. The situation is further confounded by the social, ethnic, and educational background of the parents also possibly influencing the prevalence of these disabilities.

The Pulmonary Collectins and Respiratory Syncytial Virus: Is There a Clinical Link?

Paul S. Kingma, Jeffrey A. Whitsett — 2010-03-01

More than a decade of basic science research suggests that the pulmonary collectins, surfactant protein A (SP-A) and D (SP-D), are critical components of the lung innate immune system. Mouse models of collectin deficiency indicate that SP-A and SP-D are required for normal clearance of a variety of pulmonary pathogens such as respiratory syncytial virus (RSV) and that if infection occurs in the absence of these proteins, the lungs fill with host immune cells that release excessive amounts of inflammatory cytokines, oxygen radicals, and metalloproteinases. Despite the considerable innate immune defects that are associated with SP-A or SP-D deficiency in animal models, we have yet to find susceptibility to pulmonary infection that is clearly caused by mutations in either of these genes.

Treatment Adherence in Patients with Sickle Cell Anemia

Dennis Drotar — 2010-01-08

Advances in the treatment of sickle cell anemia (SCA), including hydroxyurea therapy, hold the promise of improving health outcomes for children and adolescents with this very challenging chronic condition. Close adherence to the prescribed treatment is necessary to maximize the efficacy of hydroxyurea. However, this treatment is part of a multifaceted and very demanding regimen that can include multiple medications and hydration. Earlier evidence suggests that adherence to the range of prescribed treatments for pediatric SCA, including hydroxyurea, is less than ideal. Consequently, there is an important need to gather clinically relevant scientific data on the prevalence and potential health-related consequences of non-adherence.

Cough in the Pediatric Population

Alan B. Goldsobel, Bradley E. Chipps — 2010-03-01

Cough is the most common presenting symptom for medical office visits in the United States. Cough in children is usually related to viral respiratory tract infection and typically resolves spontaneously. Between 35% and 40% of school-age children still cough 10 days after the onset of a common cold, and 10% of preschool children have cough 25 days after respiratory tract infection. In children, cough has been associated with environmental factors, such as outdoor and indoor air pollution, including particulate matter, irritant gases, environmental tobacco smoke exposure, and dampness in the home. The frequent presentation of cough in children is further complicated by studies documenting that parental reporting of cough in children correlates poorly with objective measurement of frequency, duration, or intensity of cough. Cough in children disrupts both the parent's and the child's daily activities and can be associated with impaired quality of life in the child and significant stress in parents that improves with cough resolution. It is extremely common for parents to treat children with over-the-counter (OTC) cough and cold medications (CCMs) before seeing a health care provider. In a recent survey, approximately 10% of US children were found to be receiving an OTC CCM in any given week. Although OTC CCMs receive Food and Drug Administration (FDA) approval for adults, testing for efficacy and safety in young children has not been adequate, and inappropriate use of CCMs in children has been documented. Adverse events associated with use of OTC CCMs do occur and rare infant deaths have been reported. In January 2008, the FDA issued a public health advisory regarding OTC CCM use in children questioning safety and efficacy and whether the clinical benefits justify potential risks; it now recommends avoiding these medications in children under age 2 years. The FDA also has supported the recent recommendation by the Consumer Health Product Association to avoid OTC CCM use in children under age 4 years. An American Academy of Pediatrics (AAP) position statement questions the efficacy and safety of these medications in children under age 6 years.

A Neurobehavioral Intervention and Assessment Program in Very Low Birth Weight Infants: Outcome at 24 Months

2009-11-02

Objective: To determine whether the Infant Behavioral Assessment and Intervention Program (IBAIP) improves development and behavior in very low birth weight (VLBW) infants at 24-month corrected age.Study design: In a multicenter, randomized, controlled trial 86 infants received postdischarge intervention until 6-month corrected age. The intervention consisted of supporting infants' self-regulation and development, and facilitating sensitive parent-infant interactions; 90 control infants received regular care. At 6 months, positive intervention effects were found. At 24 months, development and behavior were evaluated with the Bayley Scales of Infant Development-II (BSID-II) and the Child Behavior Check List (CBCL).Results: Eighty-three intervention and 78 control infants were available for follow-up. After adjustment for differences in perinatal characteristics, an intervention effect of 6.4 points (± standard error, 2.4) on the Psychomotor Developmental Index favored the intervention infants. Groups did not differ on the Mental Developmental Index, the Behavioral Rating Scale of the BSID-II, or on the CBCL. Subgroup analyses revealed improved motor as well as improved mental outcomes in intervention infants with bronchopulmonary dysplasia and with combined biological and social risk factors.Conclusions: The IBAIP shows sustained motor improvement in VLBW infants until 2-year corrected age.

Neurobehavioral Assessment Predicts Motor Outcome in Preterm Infants

2009-11-02

Objective: To determine whether Neonatal Intensive Care Unit Network Neurobehavior Scales (NNNS) at 44 weeks predict motor outcome at 2 years in preterm infants from the Maternal Lifestyles Study (MLS).Study design: Data were collected on all preterm infants (<36 weeks) in the MLS who underwent an NNNS at 44 weeks (n = 395) and neurologic examination at 12 to 36 months or Bayley Psychomotor Development Index (PDI) at 24 months (n = 270). Logistic regression analyzed NNNS summary scores associated with cerebral palsy (CP) or PDI <70, while controlling for birth weight ≤1250 g.Results: Eighteen of 395 infants (5%) had CP; 24 of 270 infants (9%) had PDI <70. CP was associated with low quality of movement (odds ratio [OR], 1.95; 95% CI, 1.24-3.06; P = .004) and high lethargy (OR, 1.67; 95% CI, 1.01-2.76; P = .045). The model contributed 19% of the variance in CP diagnosis at 12 to 36 months (R2 = .19, P < .001). Low PDI was associated with low handling (OR, 1.83; 95% CI, 1.12-2.99; P = .017), low quality of movement (OR, 2.16; 95% CI, 1.38-3.38; P = .001), and hypotonia (OR, 1.63; 95% CI, 1.14-2.32; P = .007). The model contributed 26% of the variance in PDI <70 at 24 months (R2 = 0.26, P < .001).Conclusions: The neurobehavioral profile of under-arousal in 44-week-old preterm infants may predict poor motor outcome.

50 Years Ago in The Journal of Pediatrics: Etiology of Mongolism

Howard M. Saal — 2010-03-01

Warkany J. J Pediatr 1960;56:412-9 Although Down syndrome had been described by John Down in 1866, it took almost 100 years until the cause of this disorder was identified, leading to a “Conference on the Etiology of Mongolism” held on October 19, 1959. Only 3 years before, it was determined that the actual number of human chromosomes was 46. This exciting new discovery led to identifying the cause of numerous syndromes, including Down syndrome (trisomy 21), the most common chromosome disorders. The field of genetics and birth defects has significantly progressed since this publication, and it is likely that the authors did not foresee the sequencing of the entire human genome, including identification of all the genes on chromosome 21.

Near-Infrared Spectroscopy Measurements of Cerebral Oxygenation in Newborns during Immediate Postnatal Adaptation

2009-11-16

Objective: In view of growing concerns regarding the optimal supplementation of oxygen at birth, we measured cerebral oxygenation during the first minutes of life.Study design: Using near-infrared spectroscopy, changes in cerebral oxygenated hemoglobin (O2Hb), dexoxygenated hemoglobin (HHb), and tissue oxygenation index (TOI) were measured during the first 15 minutes of life in 20 healthy newborn infants delivered at term by elective cesarean section.Results: O2Hb and TOI increased rapidly within the first minutes of life (median slope for O2Hb, 3.4 μmol/L/min; range, 1.4 to 20.6 μmol/L/min; median slope for TOI, 4.2 %/min; range, -0.4 to 27.3%/min), and cerebral HHb decreased (median slope, -4.8 μmol/L/min; range, -0.2 to -20.6 μmol/L/min). O2Hb, TOI, and HHb all reached a plateau within 8 minutes.Conclusions: A significant increase in cerebral O2Hb and TOI and a significant decrease in HHb occur during immediate adaptation in healthy term newborns, reaching a steady plateau at around 8 minutes after birth.

In Utero Iron Status and Auditory Neural Maturation in Premature Infants as Evaluated by Auditory Brainstem Response

2009-11-25

Objective: To determine whether cord ferritin (CF) concentration, an index of in utero iron status, is associated with auditory neural maturation in premature infants.Study design: A prospective cohort study was performed to compare auditory neural maturation in infants with latent iron deficiency (CF 11-75 ng/mL) and infants with normal iron status (CF > 75 ng/mL) at birth. Our inclusion criteria were infants of 27-33 weeks gestational age who were admitted to the neonatal intensive care unit between July 2007 and November 2008 within 12 hours after birth and had cord blood collected. Infants with TORCH infections (toxoplasmosis, other infections, rubella, cytomegalovirus infection, and herpes simplex), chromosomal disorders, craniofacial anomalies, culture-proven sepsis, and/or unstable conditions were excluded. CF level was measured using a chemiluminescence immunoassay method. Bilateral monaural auditory brainstem evoked response (ABR) was assessed using 80-dB nHL click stimuli at a repetition rate of 29.9/seconds within 48 hours after birth.Results: Of the 80 infants studied, 35 had latent iron deficiency. After controlling for confounders, the infants with latent iron deficiency had significantly prolonged absolute wave latencies I, III, and V and decreased frequency of mature ABR waveforms compared with the infants with normal iron status.Conclusion: Premature infants with in utero latent iron deficiency have abnormal auditory neural maturation compared with infants with normal in utero iron status.

Caffeine for Apnea of Prematurity Trial: Benefits May Vary in Subgroups

2009-11-19

Objective: To determine whether the benefits of caffeine vary in three subgroups of 2006 participants in the Caffeine for Apnea of Prematurity (CAP) trial.Study design: Post-hoc subgroup analyses were performed on the basis of: (1) indication for commencement of study drug: treat apnea, prevent apnea, or facilitate extubation; (2) positive pressure ventilation (PPV) at randomization: endotracheal tube (ETT), noninvasive ventilation, or none; and (3) timing of commencement of study drug: early or late (≤3 versus >3 days). Outcomes assessed were those showing treatment effects in the original analyses. We investigated the consistency of caffeine effects by using regression models that incorporated treatment/subgroup factor interactions.Results: There was little evidence of a differential treatment effect of caffeine in subgroups defined by the clinical indication for starting study drug. The size and direction of the caffeine effect on death or disability differed depending on PPV at randomization (P = .03). Odds ratios (95% CI) were: no support, 1.32 (0.81-2.14); noninvasive support, 0.73 (0.52-1.03); and ETT, 0.73 (0.57-0.94). Adjustment for baseline factors strengthened this effect (P = .02). Starting caffeine early resulted in larger reductions in days of respiratory support. Postmenstrual age at time of discontinuing PPV was shorter with earlier treatment (P = .01). Mean differences (95% CI) were: early, 1.35 weeks (0.90-1.81); and late 0.55 weeks (–0.11-0.99). Adjustment for baseline factors weakened this effect (P = .03).Conclusions: There is evidence of variable beneficial effects of caffeine. Infants receiving respiratory support appeared to derive more neurodevelopmental benefits from caffeine than infants not receiving support. Earlier initiation of caffeine may be associated with a greater reduction in time on ventilation.

Early Changes in the Biophysical Properties of the Aorta in Pre-Adolescent Children Born Small for Gestational Age

2009-11-25

Objective: To assess the biophysical properties of the aorta in children born small for gestational age (SGA) with an echo-Doppler method and to determine associations with known perinatal risk factors.Study design: In this cross-sectional study, 39 SGA and 41 control subjects aged 8 to 13 years were recruited. Perinatal risk factors were recorded. The aortic diameters and pulse wave transit time around the aortic arch were measured with echo-Doppler and the blood pressure recorded. Pulse wave velocity, aortic input impedance (Zi), characteristic impedance (Zc), arterial pressure-strain elastic modulus (Ep), and arterial wall stiffness index (β-index) were calculated.Results: Pulse wave velocity (374 ± 46 vs 348 ± 47cm/sec, P < .02); Zi (177 ± 39 vs 142 ± 27 dynes · sec/cm5, P < .0001); Zc (185 ± 29 vs 152 ± 37 dynes · sec/cm5, P < .0001); Ep (286 ± 101 vs 216 ± 41 mm Hg, P < .0001); and β-index (2.43 ± 0.32 vs 2.17 ± 0.15, P < .0001) were all higher in SGA. We found negative associations between the following: birth weight and Zi, Zc, Ep, and β-index; as well as body mass index and Zi, Zc.Conclusion: This simple echo-Doppler method demonstrated abnormal biophysical properties of the aorta in a cohort of pre-adolescent patients born SGA who remain small in stature and continue to have normal blood pressure.

Lack of Effects of Oral Probiotics on Growth and Neurodevelopmental Outcomes in Preterm Very Low Birth Weight Infants

2009-11-16

Objective: To evaluate growth and neurodevelopmental outcomes in preterm very low birth weight (PVLBW) infants treated with oral probiotics for the prevention of necrotizing enterocolitis (NEC).Study design: A prospective follow-up study was performed in a cohort of PVLBW infants enrolled in a single center with a masked randomized control trial to evaluate the efficacy of oral probiotics in preventing NEC. Growth measures included weight, length, and head circumference. Neurologic and sensory performance was evaluated with standard techniques. Psychometric parameters were measured used the Bayley Scales of Infant Development II (BSID-II). The studies were performed at 3 years corrected age. The primary outcome was death or neurodevelopmental impairment.Results: Of the 367 subjects enrolled in trial, 301 (89.9%) were evaluated (153 in the probiotics group and 148 in the control group). There were no significant differences in growth or in any of the neurodevelopmental and sensory outcomes between the 2 groups.Conclusions: Oral probiotics given to PVLBW infants at 1 week after birth to reduce the incidence of NEC did not affect growth and neurodevelopmental and sensory outcomes at 3 years corrected age.

Lactobacillus GG Improves Recovery in Infants with Blood in the Stools and Presumptive Allergic Colitis Compared with Extensively Hydrolyzed Formula Alone

2009-11-02

Objectives: To determine the benefits of Lactobacillus rhamnosus GG (LGG) in an extensively hydrolyzed casein formula (EHCF) in improving hematochezia and fecal calprotectin over EHCF alone.Study design: Fecal calprotectin was compared in 30 infants with hematochezia and 4 weeks after milk elimination with that of a healthy group. We also compared fecal calprotectin and hematochezia on 26 formula-fed infants randomly assigned to EHCF with LGG (Nutramigen LGG) (EHCF + LGG) or without (Nutramigen) (EHCF − LGG) and on 4 breastfed infants whose mothers eliminated dairy.Results: Fecal calprotectin in those with hematochezia was significantly higher than in comparisons (mean ± SD 325.89 ± 152.31 vs 131.97 ± 37.98 μg/g stool, t = 6.79, P < .0001). At 4 weeks, fecal calprotectin decreased to 50% of baseline but was still significantly higher than in comparisons (157.5 ± 149.13 vs 93.72 ± 36.65 μg/g, P = .03). Fecal calprotectin mean decrease was significantly larger among EHCF + LGG compared with EHCF − LGG (−214.5 ± 107.93 vs −112.7 ± 105.27 μg/g, t = 2.43, P = .02). At 4 weeks, none of the EHCF + LGG had blood in stools, and 5/14 on EHCF − LGG did (P = .002).Conclusion: Fecal calprotectin is elevated in infants with hematochezia and possible allergic colitis. EHCF + LGG resulted in significant improvement of hematochezia and fecal calprotectin compared with the EHCF alone.

Presence of Soil-Dwelling Clostridia in Commercial Powdered Infant Formulas

Jason R. Barash, Jennifer K. Hsia, Stephen S. Arnon — 2009-12-10

Objective: Because Clostridium botulinum was isolated from powdered infant formula (PIF) fed to an infant in the United Kingdom who subsequently developed infant botulism and from unopened PIF from the same manufacturer, we tested PIF manufactured in the United States for the presence of clostridial spores.Study design: Thirty PIF ingested by 19 California infants with botulism within 4 weeks of onset of illness (48% of all patients fed PIF during study) in 2006-2007 were cultured anaerobically to isolate clostridia. All isolated clostridia were identified to the species level and enumerated with standard microbiologic and molecular methods.Results: Five of 30 (17%) PIF samples ingested by patients contained clostridial spores. Spores were also found in 7 of 9 (78%) market-purchased PIF samples. Clostridium sporogenes was isolated most frequently, followed by Clostridium butyricum and at least 10 other soil-dwelling clostridial species. No neurotoxigenic clostridia were isolated. The most probable number of clostridial spores in PIF ranged between 1.1 to >23 per 100 g.Conclusions: With the notable exception of production of botulinum neurotoxin, C sporogenes is physiologically comparable with proteolytic strains of C botulinum, and both share the same natural reservoir (soils and dust worldwide). The isolation of C sporogenes and potentially pathogenic clostridia from U.S.-manufactured PIF suggests that neurotoxigenic clostridial spores have the potential to be present in these products.

50 Years Ago in The Journal of Pediatrics: Current Immunization Problems

Sarah S. Long — 2010-03-01

Gaisford W, Feldman GV, Perkins FT. J Pediatr 1960;56:319-30 Vaccinologists from Manchester and London provide primary data and much insight surrounding problems of providing protection for infants against major pathogens of 1960. They ask the same questions of each vaccine—questions that we ask today. Is an effective vaccine antigen available? Is protection important? Can the vaccine be given at the age when protection is most needed? Is it safe?

Surfactant Protein A2 Polymorphisms and Disease Severity in a Respiratory Syncytial Virus-Infected Population

2009-11-16

Objective: To examine whether genetic variations within the surfactant protein A2 (SP-A2) gene are associated with respiratory syncytial virus (RSV) disease severity in infected children.Study design: Naturally infected children aged ≤24 months were prospectively enrolled in 3 RSV seasons. SP-A2 genotyping was performed. Independent clinical predictors of disease severity were analyzed. The association of SP-A2 genetic diversity and disease severity was tested by using multivariate logistic regression models and 4 levels of disease gradation as outcome measures.Results: Homozygosity of the 1A0 allele was protective against hospitalization (odds ratio [OR] = 0.15, P = .0010). This remained significant in African American patients (OR = 0.24, P = .042) and Caucasian patients (OR = 0.05, P = .021) after adjustment for other co-variates. Hospitalized children with the 1A2 allele demonstrated significant protection from severe disease with univariate analyses, but only a trend for protection with multivariate analyses. Patients homozygous or heterozygous for an asparagine at amino acid position 9 were twice or more likely to need intensive care unit admission (OR = 2.15, P = .022), require intubation (OR = 3.04, P = .005), and have a hospitalization lasting ≥4 days (OR = 1.89, P = .02) compared with children homozygous for a threonine at this position.Conclusions: SP-A2 polymorphisms are associated with the severity of RSV infection in infants.

50 Years Ago in The Journal of Pediatrics: Evaluation of Prepared Milks for Infant Nutrition: Use of the Latin Square Technique

Jon A. Vanderhoof, Susan Hazels Mitmesser — 2010-03-01

Brown GW, Tuholski JM, Sauer LW, Minsk ID, Rosenstern I. J Pediatr 1960;56:391-8 Fifty years ago in The Journal of Pediatrics, Brown et al proposed the use of the Latin square technique as a means of evaluating and comparing the nutritional adequacy of infant formulas. Although some of the concepts and ideas proposed by Brown still hold true today, modern technology and knowledge have changed the way clinical trials are conducted in a pediatric population.

Adherence to Hydroxyurea Therapy in Children with Sickle Cell Anemia

Courtney D. Thornburg, Agustin Calatroni, Marilyn Telen, Alex R. Kemper — 2009-11-02

Objectives: To assess adherence to hydroxyurea therapy in children with sickle cell anemia (SCA), evaluate the association between adherence and hematologic profile, and identify barriers and facilitators of adherence.Study design: Children with SCA (n=75) receiving hydroxyurea were recruited for a single-institution cross-sectional study. The primary outcome was association between treatment adherence and percent fetal hemoglobin (HbF).Results: Good adherence was estimated at 82% with visual analog scale, 84% with Morisky score, 85% with medical provider report, 77% with clinic visits, and 49% on the basis of pharmacy refills. Increase in HbF was moderately associated with good adherence as measured with the parent/proxy Morisky score (r=−0.39; 95% CI, −0.58–0.17; P < .01) and prescription refills (r=0.39; 95% CI, 0.16–0.57; P < .01). The number of pharmacy refills and the Morisky score explained 23% of the variation in HbF response.Conclusions: Adherence was ≥75% with 4 of 5 measures. Pharmacy refills and the Modified Morisky Scale may be used to identify children at high risk for poor response because of non-adherence and children with good adherence with poor response because of individual pharmacodynamics. Future research should prospectively compare adherence measures and evaluate methods to improve treatment adherence.

Prevalence of Developmental Disabilities and Receipt of Special Education Services among Children with an Inborn Error of Metabolism

2009-11-16

Objective: To examine the presence of developmental disabilities and receipt of special education services in children with an inborn error of metabolism.Study design: The study population was children born from 1988 through 2001 in whom a metabolic disorder was diagnosed after identification by newborn screening (n = 97) or after clinical identification (n = 34). These children were linked to the Metropolitan Atlanta Development Disability Surveillance Program (MADDSP) and Special Education Database of Metropolitan Atlanta (SEDMA) to determine developmental outcomes at 8 years of age and 3 through 10 years of age, respectively. Medical and educational records were examined to consider factors contributing to developmental outcomes.Results: Of 97 children with a metabolic disorder identified with newborn screening, 12 (12.4%) were identified by SEDMA as receiving special education services and 2 (2.7%) were identified by MADDSP as having a developmental disability. Of the 34 children with a clinically identified metabolic disorder, 8 (23.5%) were identified with SEDMA, and 5 (17.2%) were identified with a MADDSP developmental disability.Conclusion: Early identification and treatment have been successful in limiting the impact of severe developmental disabilities. Continued surveillance and research are needed to monitor less severe developmental outcomes.

Newborn Population Screening for Classic Homocystinuria by Determination of Total Homocysteine from Guthrie Cards

2009-11-16

Objective: To allow early recognition of cystathionine β-synthase by newborn screening.Study design: Total homocysteine was determined in dried blood spots with a novel, robust high-performance liquid chromatography method with tandem mass spectrometry. Quantification of homocysteine was linear over a working range up to 50 μmol/L. For mutation analysis, DNA was tested for 2 mutations common in Qatar.Results: Both methods proved to be suitable for high throughput processing. In 2 years, 7 infants with classic homocystinuria were identified of 12 603 native Qatari infants, yielding an incidence of 1:1800. Molecular screening would have missed 1 patient homozygous for a mutation not previously identified in the Qatari population. Over a period of 3 years, a total of 14 cases of classic homocystinuria were detected by screening of homocysteine from all newborn infants born in Qatar (n = 46 406). Homocysteine was always elevated, whereas methionine was elevated in only 7 cases.Conclusions: The study offers a reliable method for newborn screening for cystathionine β-synthase deficiency, reaching a sensitivity of up to 100%, even if samples are taken within the first 3 days of life.

The Behavioral Phenotype of School-Age Children with Shwachman Diamond Syndrome Indicates Neurocognitive Dysfunction with Loss of Shwachman-Bodian-Diamond Syndrome Gene Function

Elizabeth N. Kerr, Lynda Ellis, Annie Dupuis, Johanna M. Rommens, Peter R. Durie — 2009-11-11

Objective: To investigate the cognitive, behavioral and adaptive functioning of children with Shwachman-Diamond syndrome (SDS).Study design: Thirty-two children with SDS (6-17 years) were evaluated by use of standardized neuropsychological tests. Results were compared with normative data, unaffected siblings (n = 13), and age-and sex-matched children with cystic fibrosis (CF; n = 20).Results: Although intragroup variability in performance was evident, children with SDS displayed weaker overall intellectual reasoning, higher-order language skills, perceptual reasoning, visual-motor processing speed, visual motor- integration, visual executive problem-solving, attention, and aspects of academic achievement, as well as lower functional level of independence relative to the general population. Significant issues with behavior were also identified, including prior formal diagnoses and social problems. Lower abilities were found relative to sibling and CF control subjects and were not associated with secondary complications of SDS, age, or sex.Conclusion: Neurocognitive deficits in subjects with SDS are largely independent of family environment and having a chronic illness and are likely the consequences of Shwachman-Bodian-Diamond syndrome gene dysfunction. There is a need for a broad-based approach to the assessment of cognitive function and appropriate remediation of individuals with SDS.

50 Years Ago in The Journal of Pediatrics: Hydrocephalus

Gordon Li, Paul Graham Fisher — 2010-03-01

Ransohoff J, Shulman K, Fishman RA. J Pediatr 1960;56:399-411 Fifty years ago in The Journal, Ransohoff et al reviewed the physiology, etiology, diagnosis, and treatment of hydrocephalus. They defined hydrocephalus as “a pathologic state characterized by an increased volume of cerebral spinal fluid (CSF) under increased pressure, most commonly due to an obstruction in the circulation of this fluid.” Little has changed since then in understanding hydrocephalus pathophysiology; however, we have made significant improvements in diagnosis, management, and prevention.

Health-Related Quality of Life in Adolescents and Young Adults with a History of Kawasaki Disease

Hiromi Muta, Masahiro Ishii, Motofumi Iemura, Toyojiro Matsuishi — 2009-12-07

Objective: To investigate the health-related quality of life (HRQOL) in adolescents and young adults with Kawasaki disease (KD).Study design: We conducted a cross-sectional observational study. Patients were eligible for this study when they were ≥16 years of age and had received a diagnosis of KD ≥5 years before the start of this study. The patients were divided in 3 groups according to their coronary status: normal, aneurysms, and giant aneurysms/ischemia. A self-administered questionnaire (Medical Outcome Study Short Form 36) was mailed to the patients to evaluate the HRQOL.Results: Of 624 total questionnaires mailed, 377 were delivered, and 250 were returned. The response rates of the normal, aneurysm, and giant aneurysms/ischemia groups were 33%, 62%, and 72% of all eligible patients, respectively. All subscale scoress of HRQOL, except vitality and role-emotional (limitations in the usual role activities because of emotional problems) in patients with KD were significantly higher than scores from the national norms.Conclusions: The HRQOL of adolescents and young adults with a history of KD is favorable. However, long-term follow-up is necessary, because the general health perceptions of older patients tended to be lower.

50 Years Ago in The Journal of Pediatrics: Testicular Feminization and Color Blindness

Francesco De Luca — 2010-03-01

RJ Blattner. J Pediatr 1960;56:425-7 A comprehensive description of the clinical manifestations of resistance to androgens was first provided in 1953 by John Morris, who coined the term “testicular feminization.” Seven years later, Blattner used the same term in the title of his comments on this disorder of sex development. In the past few years, most clinicians have abandoned the term “testicular feminization” and adopted the term “complete androgen insensitivity syndrome” (CAIS), which more accurately reflects the pathophysiology of this disorder and is less controversial and disturbing to patients and families. In his article, Blattner correctly describes the physical characteristics of a woman with CAIS (normal breast development, absent or scant pubic and axillary hair, blind-ending vagina, absent uterus and Fallopian tubes, and testes located intra-abdominally, in the inguinal canals, or in the labia majora). In addition, he recognizes that this condition is not due to a chromosomal abnormality and supports the hypothesis previously put forth by Grumbach and Barr that “testicular feminization is associated with either a sex-linked recessive gene or a sex-limited autosomal dominant gene.” Indeed, CAIS is due to deletions or (more commonly) single base substitutions in the androgen receptor gene, which is located on the long arm of the X chromosome. The phenotypes resulting from impaired androgen action depend on the severity of the androgen receptor disruption and encompass mild androgen insensitivity (normal male phenotype associated with infertility or gynecomastia), partial androgen insensitivity (various degrees of genitalia ambiguity), and CAIS. In addition, a particular androgen receptor mutation may be associated with a heterogeneous phenotypic expression, with the same mutation reported in different individuals associated with either complete or partial androgen insensitivity. Finally, another interesting aspect of this article is the description of families in which testicular feminization and color blindness were variably cosegregated in XY individuals, consistent with the notion that both were sex-linked traits. The genes encoding the red and green photopigments are located on the long arm of the X chromosome, and red-green color vision defects are in fact inherited as X-linked recessive traits. Despite the concomitant occurrence of these 2 genetic abnormalities in the families described in the article, the prevalence of CAIS is quite rare (between 1 case in 20 000 and 1 case in 100 000 genetic males), and that of red-green color vision defects is much more common (in 8% of males of European descent).

Relationships between 25-Hydroxyvitamin D Levels and Plasma Glucose and Lipid Levels in Pediatric Outpatients

Michael D. Johnson, Nicole S. Nader, Amy L. Weaver, Ravinder Singh, Seema Kumar — 2009-11-19

Objective: To study the relationships between serum vitamin D levels and plasma glucose or lipid levels in children and adolescents.Study design: We conducted a retrospective record review of pediatric outpatients (age, 2-18 years) with simultaneous measurement of 25-hydroxyvitamin D (25[OH] D) and fasting plasma glucose (n = 302) or 25(OH) D and a lipid panel (n = 177). Pearson correlation coefficient was used to estimate the correlation between 25(OH) D and logarithmic transformed plasma glucose or lipid levels. Plasma glucose and lipid levels were compared in subjects with 25(OH) D concentrations greater or less than 30 ng/mL.Results: 25(OH) D levels were inversely correlated with fasting plasma glucose levels (r = –0.20, P < .001). Lower 25(OH) D levels were also associated with lower serum high-density lipoprotein cholesterol (HDL) concentrations (r = 0.41; P ≤ .001). The relationship between 25(OH) D levels and fasting glucose and HDL levels did not vary significantly with sex, age, body mass index z-score, or season. Children who were vitamin D insufficient (25[OH] D ≤30 ng/mL) had higher fasting plasma glucose (P = .002) and lower HDL levels (P < .001) than children who were vitamin D sufficient (25[OH] D >30 ng/mL).Conclusions: Low 25(OH) D levels in children and adolescents are associated with higher plasma glucose and lower HDL concentrations.

50 Years Ago in The Journal of Pediatrics: A Critical Evaluation of Therapy of Febrile Seizures

Cynthia J. Campen, Paul Graham Fisher — 2010-03-01

Millichap JB, Aledort LM, Madsen JA. J Pediatr 1960;56:364-8 In the 1950s after a febrile seizure, initiation and continuation of phenobarbital was the norm, although a minority of neurologists hypothesized that anticonvulsant therapy was indicated only at the time of febrile illness. In a landmark paper 50 years ago in The Journal, Millichap et al showed that “continued administration of anticonvulsant drugs is unwarranted in the prophylactic treatment of seizures which occur only in association with fever.” Instead they recommended phenobarbital in “relatively large doses” at the onset of fever, in conjunction with antipyretic therapy.

Regional Changes in Bone Area and Bone Mineral Content in Boys with Duchenne Muscular Dystrophy Receiving Corticosteroid Therapy

Nicola J. Crabtree, Helen Roper, Heather McMurchie, Nicholas J. Shaw — 2009-11-02

Objective: To examine the functional and skeletal effects of 30 months of steroid treatment in boys with Duchenne muscular dystrophy.Study design: Lumbar spine (L2L4) and subcranial dual energy X-ray absorptiometry scanning was performed on 25 boys (mean age 7.4 years) at baseline and after 30 months of steroid treatment.Results: At baseline, L2L4 bone mineral content (BMC) was significantly low for projected bone area although appropriate for reduced lean body mass (LBM). Subcranial bone area for height and subcranial BMC for area and LBM were all significantly reduced. After 30 months of steroid therapy there was a significant increase in subcranial bone area for height but a significant reduction of subcranial BMC for area. At the lumbar spine there were no significant changes in bone area but small increases in L2L4 BMC both for bone area and LBM.Conclusion: At baseline reduced mechanical load from diminished muscle function results in narrow light bones more noticeable in the subcranial region than the lumbar spine. Increases observed in subcranial bone area at 30 months suggest a gradual adaptation to increased gravitational load whereas at the spine there were no apparent detrimental effects on bone after 30 months of steroid therapy.

Dietary Quality Index and Associated Factors among Adolescents of the State of Sao Paulo, Brazil

2009-12-14

Objective: To assess dietary quality and associated factors in adolescents.Study design: We conducted a population-based cross-sectional study in a sample of 1584 adolescents living in areas of the state of São Paulo, Brazil. Dietary intake was measured with the 24-hour recall method, and dietary quality was assessed by means of the Health Eating Index (HEI), adapted to fit to the local requirements. Linear regression analyses were performed to assess the association between the HEI and demographic, socioeconomic, and lifestyle variables.Results: A total of 97.1% of the adolescents studied had an inadequate diet or a diet that needed improvement. The mean overall HEI score was 59.7. Lower mean HEI scores were found for fruits, dairy products, and vegetables. Male adolescents who were physically active and lived in a house or apartment had higher HEI scores. The multiple regression analyses showed that the quality of the diet improved as age decreased. Adolescents who lived in houses or apartments had higher HEI scores than adolescents living in shacks or slums, regardless of age and energy intake.Conclusions: Dietary quality is associated with income and age. A better understanding of the factors associated can provide input to the formulation of policies and development of nutritional actions.

Magnetic Resonance Imaging of the Lumbosacral Spine in Children with Chronic Constipation or Non-Retentive Fecal Incontinence: A Prospective Study

2009-11-06

Objective: To determine the prevalence of lumbosacral spine (LSS) abnormalities in children with defecation disorders, intractable constipation, or non-retentive fecal incontinence (NRFI) and evaluate whether LSS abnormalities on magnetic resonance imaging (MRI) are clinically detected by neurologic examination.Study design: MRI of the LSS and complete neurologic examination by a pediatric neurologist blinded to the MRI results were performed in patients with intractable defecation disorders.Results: Patients with intractable constipation (n = 130; 76 males; median age, 11 years; range, 6-18 years), and patients with NRFI (n = 28; 18 males; median age, 10 years; range, 7-15 years) participated. One occult spina bifida (OSB) and 3 terminal filum lipomas were found in patients with a normal neurologic examination. One patient had a terminal filum lipoma and neurologic complaints. Gluteal cleft deviation was found in 3 of 4 patients with LSS abnormalities. Neurosurgical treatment was not required in any patient during the 12-week follow-up.Conclusions: MRI showed LSS abnormalities in 3% of patients with defecation disorders and normal neurologic examination, all of whom reported symptom relief at the 12-week follow-up without neurosurgical intervention. Thus, whether or not LSS abnormalities play a role in defecation disorders remains unclear.

Premature Thelarche: Age at Presentation Affects Clinical Course but Not Clinical Characteristics or Risk to Progress to Precocious Puberty

Liat de Vries, Anat Guz-Mark, Liora Lazar, Adi Reches, Moshe Phillip — 2009-11-16

Objectives: To determine whether age at premature thelarche (PT) onset affects the clinical characteristics, course, and risk of progression to precocious puberty (PP).Study design: Data regarding course of growth and puberty were retrieved from the medical files of 139 girls with PT followed up from 1995 to 2005. Analysis was based on age at PT appearance (birth, 1-24 months, and 2-8 years); course was categorized as regressive, persistent, progressive, or cyclic.Results: At diagnosis, height standard deviation score, bone age-chronological age ratio, and hormonal values were comparable in the 3 age groups. PT regressed in 50.8%, persisted in 36.3%, progressed in 3.2% and had a cyclic course in 9.7%. A progressive or cyclic course was significantly more prevalent among girls presenting after 2 years (52.6%) compared with girls presenting at birth (13.0%) or at 1 to 24 months (3.8%) (P < .001). PP occurred in 13% irrespective of age at PT presentation or clinical course.Conclusions: Clinical and anthropometric characteristics at admission and risk of PP were similar in all girls with PT, regardless of age at onset. There are currently no clinical or laboratory tests that can predict the risk of progression to PP at presentation.

Is Diabetic Ketoacidosis at Disease Onset a Result of Missed Diagnosis?

Helen Bui, Teresa To, Robert Stein, Kinwah Fung, Denis Daneman — 2009-12-04

Objective: To determine the frequency of medical encounters before diagnosis of diabetes in children in Ontario, Canada; to determine risk factors for diabetic ketoacidosis (DKA).Research design: All medical encounters within 4 weeks before date of diagnosis for all new cases of diabetes in children <18 years were identified from April 1994 to March 2000 by use of administrative databases. The main outcome measure was the frequency of medical encounters before diagnosis in children presenting with and without DKA.Results: A total of 3947 new cases of diabetes were identified, 735 (18.6%) with DKA. DKA rates were 39.7% for children ≤3 years and 16.3% for children >3 years (P < .001). During the week before diagnosis, 285 children with DKA (38.8%) and 1104 children with diabetes without DKA (34.4%; P = .026) had at least 1 medical visit. Children with diabetes overall had more medical encounters before diagnosis than control subjects. Children with DKA were less likely to have had relevant laboratory testing before diagnosis than children with diabetes without DKA.Conclusions: Children with diabetes presenting with DKA more frequently had a medical encounter before diagnosis compared with children with diabetes without DKA. These data have important implications for enhancing public and physician awareness of diabetes in children.

Invasive Pneumococcal Infections among Vaccinated Children in the United States

2009-12-04

Objective: Because 7-valent pneumococcal conjugate vaccine (PCV7) is highly efficacious, pneumococcal infections in vaccinated children raise concerns about immunologic disorders. We characterized a case series of US children in whom invasive pneumococcal infections developed despite vaccination.Study design: We reviewed invasive (sterile site) pneumococcal infections in children aged <5 years who had received ≥1 PCV7 dose as identified from October 2001 to February 2004 through national passive surveillance and the Centers for Disease Control and Prevention's Active Bacterial Core surveillance. Vaccine serotype infections were considered breakthrough cases; the subset of breakthrough cases occurring in children who completed an age-appropriate vaccination series were considered PCV7 failures.Results: We identified 753 invasive infections; 155 infections (21%) were breakthrough cases, predominantly caused by serotypes 6B (n = 50, 32%) and 19F (n = 45, 29%). The proportion of breakthrough cases decreased with the increasing number of PCV7 doses received (P < .001, X2 for linear trend). Children with co-morbid conditions accounted for 31% of breakthrough infections. Twenty-seven cases (4%) were classified as vaccine failures. Most failures (71%) occurred in children who were vaccinated according to catch-up schedules; 37% had co-morbid conditions.Conclusion: Invasive pneumococcal infections identified in vaccinated U.S. children were primarily caused by disease resulting from serotypes not covered with PCV7, rather than failure of the vaccine. Incomplete vaccination and co-morbid conditions likely contribute to breakthrough vaccine-type pneumococcal infections.

Pediatric-Onset Relapsing Polychondritis: Case Series and Systematic Review

2009-11-02

Objectives: To study the pediatric presentation and evolution of relapsing polychondritis (RP), a rare inflammatory disease characterized by recurrent inflammation of cartilage.Study design: We retrospectively collected data from 10 patients observed in 3 French hospitals for relapsing polychondritis, with an age at onset <18 years. We also analyzed 37 cases of pediatric-onset RP from a systematic review.Results: The mean age at first symptoms was 8.6 years, and the sex ratio was 6 male patients and 4 female patients. Children came to medical attention with joint pain, ocular inflammation, and chondritis. Outcomes included severe visual impairment, chronic destructive chondritis, and 1 death caused by aortic dilatation. Treatment mainly consisted of non-steroidal-anti-inflammatory drugs, corticosteroids, and immunosuppressants. Growth was normal in 7 examined patients. Systematic literature review also suggested a high number of tracheostomy in pediatric cases, but this was not confirmed in our series.Conclusion: RP in childhood shares the main clinical features of its adult counterpart, including destructive chondritis and systemic symptoms, but unlike adults, children frequently have a family history of autoimmunity and infrequently have other associated autoimmune diseases. RP can be fatal; close screening for complications is mandatory. Growth does not appear to be impaired by cartilage inflammation.

Caffeine Increases Cerebral Cortical Activity in Preterm Infants

Sirma Supcun, Patrizia Kutz, Wolfgang Pielemeier, Claudia Roll — 2010-03-01

Caffeine improves the rate of survival without neurodevelopmental disability in preterm infants. The mechanisms underlying neuroprotection are incompletely understood. In 51 preterm infants studied by amplitude-integrated electroencephalography from 2 hours before to 2 hours after intravenous caffeine administration, we found that caffeine increases amplitudes and periods of continuity.

Very Long-Chain Acyl-CoA Dehydrogenase Deficiency in a Patient with Normal Newborn Screening by Tandem Mass Spectrometry

Can Ficicioglu, Curtis R. Coughlin, Michael J. Bennett, Marc Yudkoff — 2010-01-08

Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) can be detected through newborn screening with tandem mass spectrometry. We report a patient who died as a result of severe brain injury due to hypoglycemia. Newborn screening was normal. Postmortem enzyme analysis and molecular testing confirmed the diagnosis of VLCADD.

50 Years Ago in The Journal of Pediatrics: Epidemic Nephritis in South Trinidad

Sharon P. Andreoli — 2010-03-01

Symonds BER. J Pediatr 1960;56:420-4 This manuscript was a detailed description of a large outbreak of post-streptococcal glomerulonephritis that occurred in Trinidad in late 1958 and early 1959. Two-hundred-sixty-three children were admitted to the San Fernando General Hospital, and the author notes that several children with acute nephritis were admitted to other hospitals during this interval. In addition, several children with milder disease were treated as outpatients. This manuscript and subsequent studies of these children were seminal in defining the acute clinical course and the short- and long-term prognosis of children with post-infectious glomerulonephritis.

Adrenal Insufficiency in Newborns with Congenital Diaphragmatic Hernia

Beena D. Kamath, Lucy Fashaw, John P. Kinsella — 2010-01-08

Newborns with congenital diaphragmatic hernia frequently have catecholamine-unresponsive systemic hypotension and respiratory failure. We found that adrenal insufficiency frequently complicates the clinical course of infants with congenital diaphragmatic hernia and was associated with increased severity of illness.

Clinical Characteristics and Outcomes of Neonatal Pertussis: A Comparative Study

Luis A. Castagnini, Flor M. Munoz — 2010-01-08

We describe the features and outcomes of neonatal pertussis and compare these with neonates with non-pertussis acute respiratory illness from July 2000 through December 2007. Patients with pertussis had a more severe course of disease as evidenced by the clinical presentation, length of hospitalization, and oxygen requirement. Clinicians should have a high index of suspicion so that appropriate supportive care can be initiated promptly.

Clindamycin-Resistant Group B Streptococcus and Failure of Intrapartum Prophylaxis to Prevent Early-Onset Disease

2010-01-08

Guidelines recommend intrapartum antibiotic prophylaxis (IAP) for parturient women who have a screen positive for group B Streptococcus (GBS). Clindamycin should be used for IAP only if the maternal GBS isolate is susceptible. We report a case of clindamycin-resistant GBS disease in a newborn infant whose mother received clindamycin IAP, and we review clindamycin susceptibility testing.

Complication after “Home Treatment” of Polydactyly

Laura Dattner, Monica Marin — 2010-01-08

A 24-day-old African American infant presented to our Pediatric clinic for an initial visit. At birth he was found to have bilateral pedunculated postaxial extra digits on the upper extremities. An outpatient appointment had been scheduled with a pediatric surgeon. The infant was born premature but was otherwise healthy. Two days before presentation, the patient's grandmother tied the base of both extra digits with thread.

An Unusual Dermatosis in a Child

2010-01-08

A 9-year-old boy presented to the emergency room with various macular erythematoses and ecchymotic lesions with geometric shapes and well-defined edges on his left hand and forearm (). Initially, the child denied that the lesions were self-inflicted. But during the anamnesis, his grandmother mentioned that she had noticed that her albuterol inhaler had run out before she had expected. We confirmed that the shape of the distal part of the inhaler corresponded exactly with the borders of the child's skin lesions. Finally, the child confessed that he had created the lesions by heating the inhaler on a vitroceramic hotplate and then applying it to his skin.

An interactive booklet reduces antibiotic use for children with respiratory tract infections

Michael Moore — 2010-03-01

Francis NA, Butler CC, Hood K, Simpson S, Wood F, Nuttall J. Effect of using an interactive booklet about childhood respiratory tract infections in primary care consultations on reconsulting and antibiotic prescribing: a cluster randomised controlled trial. BMJ 2009;339:b2885.

Propranolol may benefit those with severe infantile hemagiomas

Sheila Fallon Friedlander — 2010-03-01

Sans V, de la Roque ED, Berge J, Grenier N, Boralevi F, Mazereeuw-Hautier J, et al. Propranolol for severe infantile hemangiomas: follow-up report. Pediatrics 2009;124:e423-31.

Anti-pyretic agents are ineffective in the prevention of febrile seizures

Patricia K. Duffner — 2010-03-01

Strengell T, Uhari M, Tarkka R, Uusimaa J, Alen R, Lautala P, et al. Antipyretic agents for preventing recurrences of febrile seizures: randomized controlled trial. Arch Pediatr Adolesc Med 2009;163:799-804.

Two developmental screening tests may identify different groups of children

Michael Shevell — 2010-03-01

Sices L, Stancin T, Kirchner HL, Bauchner H. PEDS and ASQ developmental screening tests may not identify the same children. Pediatrics 2009;124:e640-7. Among children at increased risk for developmental delays, do the Parents' Evaluation of Developmental Status (PEDS; parent concern questionnaire) and the Ages & Stages Questionnaires (ASQ; parent report of developmental skills) perform similarly as a screening test?

Nomogram can help estimate risk of serious hyperbilirubinemia in healthy infants

Thomas A. Trikalinos — 2010-03-01

Varvarigou A, Fouzas S, Skylogianni E, Mantagou L, Bougioukou D, Mantagos S. Transcutaneous bilirubin nomogram for prediction of significant neonatal hyperbilirubinemia. Pediatrics 2009;124:1052-9.

Decision rules can identify children at very low risk of clinically important traumatic brain injury

Ran Goldman — 2010-03-01

Kuppermann N, Holmes J, Dayan P, Hoyle JJ, Atabaki S, Holubkov R, et al. Identification of children at very low risk of clinically important brain injuries after head trauma: a prospective cohort study. Lancet 2009;374:1160-70.

Neuraminidase inhibitors beneficial for treatment and prevention of influenza in children

Nicholas J. Matheson — 2010-03-01

Shun-Shin M, Thompson M, Heneghan C, Perera R, Harnden A, Mant D. Neuraminidase inhibitors for treatment and prophylaxis of influenza in children: systematic review and meta-analysis of randomised controlled trials. BMJ 2009;339:b3172.

The optimal use of probiotics in acute diarrhea of childhood

Zvi Weizman — 2010-01-08

To the Editor: I read with great interest the study by Misra et al. Although it is a well-designed randomized controlled trial, I would like to delineate a few limitations of this study. The trial evaluates a highly selective population, hospitalized malnourished infants and children in rural India. Unfortunately, no data are presented about the indication for hospitalization. Such subjects may have a variety of systemic disorders that affect the study results. In addition, the high proportion of lactose intolerance (81.9%) may result from diffuse small intestinal mucosal damage caused by malnutrition, other gastrointestinal abnormalities, or both. There is no information about the results of testing of stool for parasites. Such confounding factors may adversely affect the clinical benefits of probiotic agents.

Reply

Sudipta Misra, Tapas K. Sabui — 2010-01-08

To the Editor: We thank Weizman for his comments. However, we would like to clarify his concerns about our patient population. These were by no means sick, hospitalized, malnourished, and rural children with widespread small intestinal mucosal damage. This study was done at the suburb of the megametropolis Kolkata, with a strong industrial base. Though the enrolled children were moderately malnourished, it was not severe enough to cause mucosal damage and make them vulnerable to intractable diarrhea cycle (the so-called threshold effect of malnutrition). In fact, all of them recovered from the index episode. These children were admitted for diarrhea per hospital policy and World Health Organization guidelines for treatment at diarrhea management centers (Plan B), not for any other illness. Severely ill children were excluded from the study. Eleven of 210 children had parasites in the stool. This is common in this setting, and such a small number is unlikely to affect the outcome of the study.

Hispanic youth: violence and aggression ecological influences

Alesia Brooks — 2010-03-01

To the Editor: The focus on social influences of Hispanic youth is intriguing because they are an underrepresented ethnic group in family therapy research literature. Although Ferguson et al report that their results cannot be generalized across ethnic groups, Szapocznik et al has facilitated 25 years of research specifically with antisocial delinquent Hispanic youth.

Reply

Christopher J. Ferguson, Claudia San Miguel, Richard Hartley — 2010-01-25

To the Editor: Brooks comments on risk factors that we did not explore that also might play some role in youth violence. Naturally, in any single analysis, it is difficult to consider all risk factors, although we explored a number that had been identified as important in past research.

Exhaled nitric oxide and childhood asthma

Daan Caudri, Johan C. de Jongste — 2010-03-01

To the Editor: Sivan et al investigated the use of exhaled nitric oxide (FeNO) in the diagnosis of asthma in school-age children. They found a remarkable high diagnostic yield of FeNO and concluded that the test should be considered in the evaluation of children suspected of having asthma.

Reply

Yakov Sivan, Tali Gadish, Ruth Soferman, Elizabeth Fireman — 2010-01-25

To the Editor: We thank Caudri and de Jongste for their interest in our article and thoughtful comments. Their first comment has been addressed at length in the discussion of our article and also in the meticulous debate presented by Bush and Eber. In brief, there is no question that asthma may be diagnosed without difficulty by the primary physician on the basis of typical history, response to therapy, and, when needed, additional tests. However, even though this statement holds for many or even most children in the community with clinical symptoms suggesting asthma, it may not be true for a relatively small percentage of children with less-specific complaints or who ignore mild to moderate symptoms or do not respond characteristically to treatment. Because of the high incidence of asthma, this group still includes a substantial number of children referred to special clinics. It is this minority, albeit a considerable load, that consumes more healthcare resources and can benefit the most from early diagnosis, with the emphasis on “early.” We agree that the diagnostic yield of asthma also will increase in this minority population by adding IgE levels and other tests, such as skin tests for allergy, adenosine, exercise, and methacholine challenge tests. However, when the patient arrives for the first visit to a special clinic after being referred by his or her physician, the doctor at the clinic has the choice to either ask for a battery of these tests and see the patient again for evaluation or, as our data suggest, perform a cheap, cost-effective FeNO test and get immediate results. This may improve early institution of treatment. Given the test's ease of performance compared with provocation tests and induced sputum, and its better specificity and sensitivity compared with spirometry, FeNO may offer the best single, cost-effective test for diagnosing asthma in children.

Errata

2010-03-01

In the article, “Association and Socioeconomic Status with the Use of Chronic Therapies and Healthcare Utilization in Children with Cystic Fibrosis,” by Schechter et al, J Pediatr 2009;155:434-9, the cities and states should be added for the following authors: Susanna A. McColley, MD, Chicago, IL; Stefanie Silva, MS, San Francisco, CA; Tmirah Haselkorn, PhD, Sunnyvale, CA; and Michael W. Konstan, MD, Cleveland, OH.

Erratum

2010-03-01

In the Reply to the Letter to the Editor, “Cerebral blood flow velocities in extremely low birth weight infants,” by Kaiser and Lightburn, J Pediatr 2009;155:944-5, the order of the authors should be as follows: Marla H. Lighburn, MD, and Jeffrey R. Kaiser, MD, MA.

The Journal of Pediatrics

Blame inappropriate implementation for failure of intrapartum antibiotic prophylaxis for group B Streptococcus

A new concern—in utero iron status

Pertussis compared with viral respiratory tract illnesses in neonates

Missed opportunities?

Relapsing polychondritis in childood: rare but important

Clostridial spores in powdered infant formula

Evaluation of the child who presents with coughing

Table of Contents

Editorial Board

Information for Readers

e-Professionalism: Challenges in the Age of Information

Role of Intervention Strategies for At-risk Preterm Infants

The Pulmonary Collectins and Respiratory Syncytial Virus: Is There a Clinical Link?

Treatment Adherence in Patients with Sickle Cell Anemia

Cough in the Pediatric Population

A Neurobehavioral Intervention and Assessment Program in Very Low Birth Weight Infants: Outcome at 24 Months

Neurobehavioral Assessment Predicts Motor Outcome in Preterm Infants

50 Years Ago in The Journal of Pediatrics: Etiology of Mongolism

Near-Infrared Spectroscopy Measurements of Cerebral Oxygenation in Newborns during Immediate Postnatal Adaptation

In Utero Iron Status and Auditory Neural Maturation in Premature Infants as Evaluated by Auditory Brainstem Response

Caffeine for Apnea of Prematurity Trial: Benefits May Vary in Subgroups

Early Changes in the Biophysical Properties of the Aorta in Pre-Adolescent Children Born Small for Gestational Age

Lack of Effects of Oral Probiotics on Growth and Neurodevelopmental Outcomes in Preterm Very Low Birth Weight Infants

Lactobacillus GG Improves Recovery in Infants with Blood in the Stools and Presumptive Allergic Colitis Compared with Extensively Hydrolyzed Formula Alone

Presence of Soil-Dwelling Clostridia in Commercial Powdered Infant Formulas

50 Years Ago in The Journal of Pediatrics: Current Immunization Problems

Surfactant Protein A2 Polymorphisms and Disease Severity in a Respiratory Syncytial Virus-Infected Population

50 Years Ago in The Journal of Pediatrics: Evaluation of Prepared Milks for Infant Nutrition: Use of the Latin Square Technique

Adherence to Hydroxyurea Therapy in Children with Sickle Cell Anemia

Prevalence of Developmental Disabilities and Receipt of Special Education Services among Children with an Inborn Error of Metabolism

Newborn Population Screening for Classic Homocystinuria by Determination of Total Homocysteine from Guthrie Cards

The Behavioral Phenotype of School-Age Children with Shwachman Diamond Syndrome Indicates Neurocognitive Dysfunction with Loss of Shwachman-Bodian-Diamond Syndrome Gene Function

50 Years Ago in The Journal of Pediatrics: Hydrocephalus

Health-Related Quality of Life in Adolescents and Young Adults with a History of Kawasaki Disease

50 Years Ago in The Journal of Pediatrics: Testicular Feminization and Color Blindness

Relationships between 25-Hydroxyvitamin D Levels and Plasma Glucose and Lipid Levels in Pediatric Outpatients

50 Years Ago in The Journal of Pediatrics: A Critical Evaluation of Therapy of Febrile Seizures

Regional Changes in Bone Area and Bone Mineral Content in Boys with Duchenne Muscular Dystrophy Receiving Corticosteroid Therapy

Dietary Quality Index and Associated Factors among Adolescents of the State of Sao Paulo, Brazil

Magnetic Resonance Imaging of the Lumbosacral Spine in Children with Chronic Constipation or Non-Retentive Fecal Incontinence: A Prospective Study

Premature Thelarche: Age at Presentation Affects Clinical Course but Not Clinical Characteristics or Risk to Progress to Precocious Puberty

Is Diabetic Ketoacidosis at Disease Onset a Result of Missed Diagnosis?

Invasive Pneumococcal Infections among Vaccinated Children in the United States

Pediatric-Onset Relapsing Polychondritis: Case Series and Systematic Review

Caffeine Increases Cerebral Cortical Activity in Preterm Infants

Very Long-Chain Acyl-CoA Dehydrogenase Deficiency in a Patient with Normal Newborn Screening by Tandem Mass Spectrometry

50 Years Ago in The Journal of Pediatrics: Epidemic Nephritis in South Trinidad

Adrenal Insufficiency in Newborns with Congenital Diaphragmatic Hernia

Clinical Characteristics and Outcomes of Neonatal Pertussis: A Comparative Study

Clindamycin-Resistant Group B Streptococcus and Failure of Intrapartum Prophylaxis to Prevent Early-Onset Disease

Complication after “Home Treatment” of Polydactyly

An Unusual Dermatosis in a Child

An interactive booklet reduces antibiotic use for children with respiratory tract infections

Propranolol may benefit those with severe infantile hemagiomas

Anti-pyretic agents are ineffective in the prevention of febrile seizures

Two developmental screening tests may identify different groups of children

Nomogram can help estimate risk of serious hyperbilirubinemia in healthy infants

Decision rules can identify children at very low risk of clinically important traumatic brain injury

Neuraminidase inhibitors beneficial for treatment and prevention of influenza in children

The optimal use of probiotics in acute diarrhea of childhood

Reply

Hispanic youth: violence and aggression ecological influences

Reply

Exhaled nitric oxide and childhood asthma

Reply

Errata

Erratum