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.

Churg-Strauss syndrome presenting as an abdominal mass in a non-asthmatic child.

A 2-year-old boy presented with an abdominal mass and was diagnosed as Churg-Strauss syndrome (CSS). There was no history of asthma. He developed fatal gastro-intestinal disease, despite treatment with corticosteroids and cyclophosphamide. CSS is extremely rare in young children and gastro-intestinal involvement might carry a worse prognosis than in adults.

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<11%). 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.

Hiperfosfatasemia transitória benigna na infância / Benign transient hyperphosphatasemia in childhood

A hiperfosfatasemia transitória benigna na infância (HTBI) é uma condição pouco conhecida, que se traduz por um estado benigno e de bom prognóstico. A HTBI resulta do aumento súbito e transitório na concentração sérica da fosfatase alcalina (FA) em crianças sadias. A sua etiopatogenia é ainda incerta, porém a diminuição do clearance da enzima na circulação tem sido considerada o mecanismo mais provável. Para divulgar os conhecimentos atuais acerca do diagnóstico da HTBI, foram selecionados, no Medline, os artigos de periódicos médicos internacionais mais relevantes desde a descrição inicial desta condição, em 1954. Os critérios para diagnóstico de HTBI são: acometer crianças com idade inferior a cinco anos, ausência de sintomas ou presença de sintomas associados a doenças não relacionadas, sem evidência clínica ou laboratorial de doença óssea ou hepática. A elevação da fosfatase alcalina varia entre 3 e 50 vezes o limite superior do valor para a idade. A análise das isoenzimas mostra elevação das formas óssea e hepática. Os níveis séricos da fosfatase alcalina retornam ao normal em quatro meses. A elevação sérica da fosfatase alcalina foi relatada em patologias como hiperfosfatasemia familiar persistente assintomßtica e sintomática e hiperfosfatasemia persistente não-familiar. É indispensável que a HTBI seja considerada no diagnóstico diferencial de um aumento considerável dos níveis séricos de fosfatase alcalina em crianças, sem que seja detectada uma causa óbvia, o que evitará procedimentos desnecessários e dispendiosos durante a investigação diagnóstica.