Clinical Manifestations of Pediatric Food Allergy: a Contemporary Review.

Food allergies (FAs) are an emerging health care issue, and a "second wave of the allergy epidemic" was named. There are extensive data that documented the prevalence rate as high as approximately 10%. FAs are immunological adverse reactions, including IgE-mediated mechanisms, cell-mediated mechanisms, or mixed IgE- and cell-mediated mechanisms. A diagnosis of FA is made by specific symptoms encounter with food, detailed past history, sensitization tests, and oral food challenges (OFCs) if necessary. The component-resolved diagnostics (CRD) test can distinguish true or cross-reaction. "Minimal elimination" from the results of CRD and OFC could avoid unnecessary food restriction. Strict food limitation is harsh and stressful on patients and their families. Children with FAs experience a higher rate of post-traumatic stress symptoms (PTSS) and bullying than others. In the last 20 years, oral immunotherapy (OIT), sublingual immunotherapy (SLIT), and epicutaneous immunotherapy (EPIT) are treatment strategies. OIT and EPIT are the most two encouraging treatments for FA. This review aims to introduce FAs in diverse clinical disorders, new perspectives, and their practical implications in diagnosing and treating FA.

Is Langerhan cell histiocytosis complicated with hydrops fetalis exclusively lethal in premature neonates?

Langerhans cell histiocytosis is a rare disease and is lethal in premature neonates. A male premature neonate born at gestational 33 weeks presented with generalized vesicles, hydrops fetalis with pleural effusion, bilateral cataracts, and severe respiratory distress syndrome complicated with persistent pulmonary hypertension. Skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis.

Correlation of troponin I with perinatal and neonatal outcomes in neonates with respiratory distress.

BACKGROUND: The specific aims of the present study were to evaluate the associations between cardiac troponin I (Tn I) and perinatal events and whether Tn I serves as a predictor to evaluate neonatal outcomes. METHODS: Tn I level was assessed in sick neonates with respiratory distress within 12 h after birth. Apgar scores, acidosis, ventilator or oxygen requirement, hospital days and placenta clues were recorded. A total of 80 sick neonates were enrolled (54 preterm and 26 full-term neonates) delivered at Shin-Kong Wu Ho-Su Memorial Hospital between July 2003 and December 2004. RESULTS: There was a significant negative correlation between Apgar scores at 1 min and 5 min (r=-0.383, P= 0.001; r=-0.500, P < 0.001), acidosis (r=-0.309, P= 0.006), base excess (r=-0.332, P= 0.003) and Tn I. The subjects were divided into two groups using the median level of 0.028 ng/mL as a cut-off. There were significantly fewer neonates with high Apgar score (>7 at 5 min; 27/40, 69.2% vs 38/40, 97.4%; P= 0.001) in the higher Tn I group (> or =0.028 ng/mL). Lower pH (7.4 +/- 0.10 vs 7.3 +/- 0.1, P= 0.011), lower base excess (-1.0 +/- 4.3 vs -4.4 +/- 5.1, P= 0.003) and less placental weight (548.8 +/- 195.36 g vs 396.56 +/- 154.30 g, P= 0.019) were also seen in the higher Tn I group. CONCLUSION: Tn I may play a role in the assessment of perinatal outcomes but is not a precise predictor of neonatal outcomes. Tn I level of 0.028 ng/mL is also suggested as a predictor of severity of perinatal outcomes in neonates with respiratory distress.

Impact on neonatal outcome and anthropometric growth in very low birth weight infants with histological chorioamnionitis.

BACKGROUND/PURPOSE: Chorioamnionitis (CAM) is one of the main causes of preterm labor. The specific aim of our study was to evaluate neonatal outcome and anthropometric growth at the corrected age of 2 years after exposure to an adverse intrauterine event of CAM in very low birth weight (VLBW, less than 1500 g) infants. METHODS: One hundred and nineteen VLBW infants had adequate placental histological data available for the study. Maternal and perinatal characteristics and neonatal morbidity were determined. The infants were followed up prospectively and their anthropometric growth was recorded in the neonatal follow-up clinic for 2 years. RESULTS: Histological CAM was evident in 64 cases (53.8%, CAM group). Patients with histological CAM delivered earlier (27.8 +/- 2.9 vs. 29.6 +/- 3.6 weeks, p = 0.003), and they had higher incidence of preterm premature rupture of membranes (PPROM, p less than 0.001) and longer ventilation days (p = 0.001). After adjusting for gestational age, sepsis (aOR, 3.355), bronchopulmonary dysplasia (aOR, 3.018) and mechanical ventilation (aOR, 4.094) had a higher incidence in the CAM group. At the corrected ages of 6, 12, 18 and 24 months, anthropometric measurements, including body weight, body height and head circumference, were similar for the study and control infants. CONCLUSION: Histological CAM was associated with a higher incidence of PPROM, sepsis, bronchopulmonary dysplasia, more mechanical ventilation and longer ventilation days. However, at the age of 2 years, CAM had no impact on anthropometric growth.

Genetic variations of HLA-DRB1 and susceptibility to Kawasaki disease in Taiwanese children.

Although some previous studies have reported that genetic and immunologic factors play important roles in the pathogenesis of Kawasaki disease (KD), the etiologic factors of this enigmatic pediatric disease are still poorly understood. The purpose of this study was to investigate whether polymorphisms of the human leukocyte antigen DRB1 (HLA-DRB1) gene are associated with KD and the development of coronary artery lesions (CAL) in Taiwanese children. Genomic DNA was extracted from whole blood samples from 145 children with KD and 331 healthy controls. The HLA-DRB1 gene was genotyped by polymerase chain reaction (PCR) and sequence-based typing assays. We found that the distribution of HLA-DRB1 allele families and alleles in children with KD did not differ from that in healthy controls. Stratified analysis did not demonstrate any association between particular HLA-DRB1 allele families or alleles and the development of CAL in children with KD. These findings suggest that susceptibility to KD and CAL is not associated with the HLA-DRB1 gene in a Taiwanese population. If immunogenetic determinants are involved in this disease and its complications in Taiwanese children, they must involve genes other than HLA-DRB1.

Generalized lymphangiomatosis presenting as cardiomegaly.

Lymphangioma refers to the local proliferation of well-differentiated lymphatic tissue. Generalized lymphangiomatosis is rare. We report a previously healthy 8-month-old infant who suffered from tachypnea with mild fever for 2 weeks. Imaging studies revealed a well-defined, large mass occupying the mediastinum, which presented as cardiomegaly. The disseminated mass extended to the thymus, lung, and spleen. Lymphangiomatosis was diagnosed by biopsy. Drainage of the pericardial fluid and total parenteral nutrition did not result in improvement of chylopericardium. Secondary hypogammaglobulinemia and septic shock developed sequentially. Surgical removal of the mediastinal mass and spleen were performed. Daily subcutaneous injection of interferon (IFN) alpha-2b was then given for 3 months. No recurrence was noted during 2 years of follow-up. IFN alpha-2b may be considered as an alternative for the treatment of generalized lymphangiomatosis.

Neurodevelopmental outcome of very-low-birth-weight infants with chorioamnionitis.

BACKGROUND: Chorioamnionitis (CAM) is one of the main causes of preterm labor and has been associated with an adverse perinatal outcome in preterm infants. OBJECTIVE: The specific aim of our study was to evaluate whether there is significant difference in the Bayley developmental index scores at 6, 12, 18 and 24 months of corrected age for very-low-birthweight (birth body weight <1500 gm, VLBW) infants with or without placental CAM. METHODS: Ninety-five cases (54 in CAM and 41 in non-CAM groups) available for the study were all VLBW infants with adequate histologic placental material for analysis. Neonatal characteristics and morbidities were recorded. The infants were followed up prospectively with Bayley Scales of Infant Development in the Neonatal Follow-up Clinic for 2 years. RESULTS: We found that 56.8% of placentas presented a picture of CAM. In comparison of the neonatal characteristics, VLBW infants with CAM had shorter gestational age (27.9 +/- 2.8 vs. 30.0 +/- 3.7 weeks, p = 0.003), lower Cesarean delivery rate (48.1% vs. 73.2%, p = 0.011), more maternal steroid use (44.4% vs. 12.2%, p = 0.004) and higher incidence of preterm premature rupture of membrane (PPROM, 37.0% vs. 12.2%, p = 0.009). In comparison of neonatal outcomes, the CAM group had higher incidence of bronchopulmonary dysplasia (BPD, 40.7% vs. 19.5%, p = 0.044), more mechanical ventilation (87.0% vs. 27/41, p = 0.023) and intubation (68.5% vs. 46.3%, p = 0.049), and more median days of ventilation (23.1 +/- 29.1 vs. 7.8. +/- 13.7 days, p = 0.001). As for the follow-up, at any test age, either the mean (Mental Development Index (MDI) / (Psychomotor Development Index (PDI) scores of Bayley test or the incidence of score below 85, there was no significant difference in both groups. CONCLUSIONS: The VLBW infants with histologic chorioamnionitis were not associated with an increased risk of lower MDI or PDI scores at the corrected ages of 6, 12, 18 and 24 months compared with the non-CAM control group.

A novel SCN5A mutation manifests as a malignant form of long QT syndrome with perinatal onset of tachycardia/bradycardia.

OBJECTIVE: Congenital long QT syndrome (LQTS) with in utero onset of the rhythm disturbances is associated with a poor prognosis. In this study we investigated a newborn patient with fetal bradycardia, 2:1 atrioventricular block and ventricular tachycardia soon after birth. METHODS: Mutational analysis and DNA sequencing were conducted in a newborn. The 2:1 atrioventricular block improved to 1:1 conduction only after intravenous lidocaine infusion or a high dose of mexiletine, which also controlled the ventricular tachycardia. RESULTS: A novel, spontaneous LQTS-3 mutation was identified in the transmembrane segment 6 of domain IV of the Na(v)1.5 cardiac sodium channel, with a G-->A substitution at codon 1763, which changed a valine (GTG) to a methionine (ATG). The proband was heterozygous but the mutation was absent in the parents and the sister. Expression of this mutant channel in tsA201 mammalian cells by site-directed mutagenesis revealed a persistent tetrodotoxin-sensitive but lidocaine-resistant current that was associated with a positive shift of the steady-state inactivation curve, steeper activation curve and faster recovery from inactivation. We also found a similar electrophysiological profile for the neighboring V1764M mutant. But, the other neighboring I1762A mutant had no persistent current and was still associated with a positive shift of inactivation. CONCLUSIONS: These findings suggest that the Na(v)1.5/V1763M channel dysfunction and possible neighboring mutants contribute to a persistent inward current due to altered inactivation kinetics and clinically congenital LQTS with perinatal onset of arrhythmias that responded to lidocaine and mexiletine.

Management of hemobilia with transarterial angiographic embolization: report of one case.

A nine-year-old girl who developed life threatening hemobilia after blunt abdominal trauma was successfully managed by embolization of the hepatic artery aneurysm. However, biliary fistula persisted and subcapsular liver abscess occurred after the endoscopic sphincterotomy and the placement of a nasobiliary drain for bile leakage. Debridement of the abscess and insertion of a drain tube eventually cured the event. The relevant literature is reviewed and the management of the hemobilia is discussed.

Fatal community-acquired pneumonia: 18 years in a medical center.

BACKGROUND: Community-acquired pneumonia (CAP) remains a significant cause of childhood morbidity worldwide. We analyzed the etiologies and the clinical characteristics of children who died from CAP. This study aimed at early identification of the poor prognostic factors in order to improve the efficiency of pneumonia management and prevent deaths. METHODS: A retrospective chart review was performed for children younger than 18 years admitted to Shin Kong Wu Ho-Su Memorial Hospital between September 1992 and August 2010 with a diagnosis of pneumonia on admission. Twenty-one patients who died with the diagnosis of pneumonia and its complications were included in the study, along with 63 age- and year-matched survival controls. RESULTS: Twelve patients (57.1%) were younger than 2 years. Gram-negative bacteria (7 patients) were the most frequently identified pathogen, followed by Mycoplasma pneumoniae (6 patients). Four of these six M. pneumoniae infected patients were co-infected with other pathogens. Among the clinical characteristics, fatal CAP was associated mainly with initial presentations of anemia, lymphopenia, thrombocytopenia, bandemia, hyponatremia, sepsis, meningitis, metabolic acidosis, disseminated intravenous coagulopathy, and underlying congenital diseases. In multivariate logistic regression analysis, metabolic acidosis (odds ratio = 8.50; 95% confidence interval = 2.82-25.60; p < 0.001) was a prognostic risk factor for fatality. CONCLUSION: For patients with CAP, blood gas should be included in the routine blood test on admission. Once the initial blood test associated with the aforementioned poor prognostic factors has been identified, an immediate treatment including Gram-negative bacilli antibiotics should be started aggressively in order to prevent deaths.

Association of plasma leptin levels with maternal body weight and body mass index in premature and term newborns.

BACKGROUND: Leptin plays an important role in the regulation of body weight and energy metabolism in adults; its role in neonates also needs to be explored. The current study aims to determine the correlation between serum leptin concentrations and anthropometric variables in newborns and their mothers, and to examine the effects of sex, gestational age and antenatal steroid use on neonatal leptin levels. METHODS: This was a retrospective study. Blood samples were collected from 55 newborns within 24 hours of birth. Plasma leptin levels were measured by immunometric assay. The relationship between neonatal leptin levels and anthropometric parameters was determined using Pearson's correlation and further evaluated by linear regression analysis. RESULTS: Neonatal leptin was significantly correlated with maternal body weight (p < 0.002) and maternal body mass index (BMI) (p < 0.001). However, it was not correlated with gestational age (p = 0.130), birth weight (p = 0.097), or birth BMI (p = 0.336). The leptin levels in premature newborns (gestational age < 37 weeks; 0.69 +/- 1.82ng/mL) were significantly less than those in term newborns (gestational age > or = 37 weeks; 2.09 +/- 2.30 ng/mL, p = 0.031). There were no significant differences between sexes (p = 0.277) or in relation to antenatal steroid use (p = 0.611). CONCLUSION: Neonatal serum leptin concentrations within 24 hours of birth correlated with maternal body weight and BMI, especially in premature newborns. Premature newborns had significantly lower leptin levels than full-term newborns.

The perinatal outcomes of asymptomatic isolated single umbilical artery in full-term neonates.

BACKGROUND: Neonates with a single umbilical artery (SUA) are considered at increased risk for chromosomal and structural abnormalities, and an increased adverse perinatal outcome. OBJECTIVE: The specific aims of our study were to evaluate (1) the association of asymptomatic infants with isolated SUA and perinatal outcomes and (2) whether asymptomatic neonates with isolated SUA at birth need full investigation. METHODS: The inclusion criteria for the study were full-term neonates with isolated SUA delivered from January 1996 to December 2006. For a control group, we used the next consecutive two newborns delivered after the SUA case in the same maternity ward with matched gestational age and without phenotypic features suspicious for aneuploidy delivered after each SUA group subject. All prenatal, peripartum and delivery records were reviewed for maternal demographics, associated anomalies, karyotypic analysis, pregnancy complications and perinatal outcomes. All SUA cases had undergone sonogram for renal anomalies. RESULTS: We enrolled 14 and 28 cases into the SUA and control groups respectively. There was all normal karyotyping for the 14 cases. The placental weight in SUA was significantly Lighter compared to that in the control group (597.1+/-175.4 vs. 709.3+/-95.2 g, p=0.010). All renal sonographic screens and karyotyping in the SUA group were normal. The incidence of small for gestational age (SGA) in SUA group was higher compared to control group (SGA, 5/14, 35.7% vs. 1/28, 3.6%, p=0.011) and less body length (48.7+/-5.0 vs. 50.8+/-1.8 cm, p=0.028). CONCLUSION: SUA is a relatively rare finding. When a SUA is identified, the routine check of karyotyping and kidney sonography for possible chromosome and associated renal anomalies may be unnecessary. According to lighter placental weight probably causing the higher incidence of small for gestational age (SGA), pregnancies with isolated SUA should be carefully monitored for evidence of fetal growth restriction.