Progressive stridor: extraintestinal airway manifestations in a pediatric patient with inflammatory bowel disease.

Airway manifestations of inflammatory bowel disease are rare in pediatrics. This case describes a nine-year-old female with ulcerative colitis (UC) with progressive stridor and dyspnea for two months. Severe upper airway obstruction was noted on spirometry. CatScan (CT) of the neck and chest revealed tracheal narrowing with circumferential, heterogeneous soft tissue thickening, and posterior wall nodularity. Bronchoscopy visualized the granulation tissue of the large airways and an ulcerative lesion to the right mainstem. Consultation and evaluation by gastroenterology, oncology, and rheumatology determined a diagnosis of extraintestinal manifestations of UC. Systemic steroids led to symptom resolution and improvement in lung function

Biotin analysis of commercial vitamin and other nutritional supplements.

Because previous observations suggest that biotin may be present in vitamin preparations not labeled as containing biotin, we determined the biotin content of several over-the-counter vitamin and nutritional supplements said to contain biotin (group 1) and several in which biotin content was not specified (group 2). Biotin concentration was measured using the 125I-avidin assay which detects total avidin-binding substances. Water extracts were assayed for free biotin and acid hydrolysates were assayed for total biotin. The results of the 125I-avidin assay agreed with the stated biotin content for most vitamin and nutritional supplements in group 1. Biotin was the only avidin-binding substance in the preparations from group 1, based on reversed-phase chromatography. However, some vitamin and nutritional supplements in group 2 contained nutritionally significant amounts of biotin, particularly if the supplement contained liver or yeast extract. Total biotin was significantly higher than free biotin in one supplement; the difference was attributable to release of biotin rather than a biotin analog. We conclude that biotin may be present in some vitamin and nutritional supplements not labeled as containing biotin; biotin intake might be under-estimated if the subject is receiving a nutritional supplement containing extracts of liver or yeast

Gastroesophageal reflux disease in children older than two years of age.

Since there are few studies examining gastroesophageal reflux (GER) in healthy children beyond infancy, we report our experiences treating children older than two with this condition. GER was diagnosed by either an abnormal extended intraesophageal pH monitoring (pH study) or presence of histological esophagitis. Thirty-seven patients met the criteria, ages 3 to 19 years (mean 11) and 68% were males. Common symptoms were vomiting, abdominal or chest pain, heartburn and regurgitation. Mean duration of symptoms was 28.7 months, and six patients had severe esophagitis, and one had Barrett\u27s esophagus. Patients with severe esophagitis were older and had strongly positive pH study parameters compared to the rest of patients (p \u3c 0.05). All patients were treated with prokinetic and acid reducing agents for 8 to 12 weeks. Sixty-two percent responded to initial course and remained asymptomatic during the follow-up period. Nissen fundoplication was recommended to five patients (13.5% of study population) because of refractory GER. Four of these patients who required surgery had severe esophagitis. In summary, GER in normal older children is a chronic disease with potentially severe complications. All patients should be evaluated by pH study and endoscopic esophageal biopsies, and have careful follow up

The diagnostic accuracy of serologic markers in children with IBD: the West Virginia experience.

GOAL: To assess the sensitivity/specificity of the serologic markers: perinuclear antineutrophil cytoplasmic antibody (pANCA) and anti-saccharomyces cerevisiae antibody (ASCA) in children diagnosed with inflammatory bowel disease (IBD), living in West Virginia. BACKGROUND: In recent years, serologic markers have been used to differentiate between CD and UC diseases in children. The clinical usefulness of these markers in children was restricted by their low sensitivity and specificity. Racial and ethnic diversity may alter the accuracy of these markers in children. The demographic homogeneity of the West Virginia population may offer a better clinical setup to reassess the utility of those markers in children with IBD. STUDY: A retrospective analysis of all the charts of children diagnosed with IBD was performed at the gastroenterology clinics of Marshall University, Huntington, WV; and West Virginia University, Charleston Division, Charleston, WV. The diagnosis of IBD was established according to clinical, radiologic, and endoscopic data. Laboratory data and serum markers were recorded, and their accuracy to diagnose UC or CD was assessed. RESULTS: A total of 101 charts were reviewed, of which only 90 (89%) included serologic markers and were considered for further analysis. Disease distribution included: UC-41, CD-44, and indeterminate colitis (IC)-7 (2 patients changed diagnosis after colectomy). Serum antibody pANCA had a sensitivity of 73% and specificity of 84% for UC, but only 16% and 35% for CD, respectively. Serum antibody ASCA had a sensitivity of 58% and specificity of 92% for CD, but only 7% and 49% for UC, respectively. The outer membrane porin to Escherichia coli antibody (anti-OmpC) was available in 54 (59%) children and demonstrated a very poor sensitivity for both diseases (sensitivity\u3c11%). CONCLUSION: Despite our homogeneous patient population, pANCA and ASCA antibodies had an inadequate sensitivity/specificity for children with UC or CD. Those antibodies were not useful for our small number of patients with IC

Benign transient hyperphosphatasemia of infancy and childhood.

Six children, aged 11 to 25 months, were found to have elevated serum alkaline phosphatase (ALP) levels (1,077 to 9,271 U/L) in the absence of disease. Benign transient hyperphosphatasemia (BTH) was diagnosed when the serum ALP levels returned to normal or decreased significantly within 3 months. Several theories have been suggested regarding the pathophysiology of BTH, but the exact cause is still unclear. We report six cases of BTH and review the literature. It is important to recognize this condition and to avoid extensive and unnecessary evaluation

Alpha 1-antitrypsin deficiency (Pi SZ) and biliary atresia.

We report an infant who presented at 2 days of age with conjugated hyperbilirubinemia. Serological, radiographic, and surgical investigations revealed the concurrence of alpha-1-antitrypsin deficiency, protease inhibitor type SZ, and extrahepatic biliary atresia