RESUMEN
Ulcerative colitis is a chronic relapsing inflammatory disease of the colonic and rectal mucosa, which affects children as well as adults. Inflammation caused by ulcerative colitis is limited to the colon and rectum, to the mucosa and submucosa, and is involved continuously, with varying degrees of ulceration, hemorrhage, edema, and regenerating epithelium. Ulcerative colitis most commonly presents with the onset of diarrhea, later associated with hematochezia, but usually without systemic signs of fever, weight loss, or hypoalbuminemia. Approximately 30% of cases have moderate signs of systemic illness. Although less commonly seen than in Crohn's disease, the first sign of ulcerative colitis may be growth failure characterized by decreased linear growth velocity. We experienced a case of ulcerative colitis with growth disturbance and treated with sulfasalazine. During follow up for a year, the symptoms subsided, and the height and weight increased.
Asunto(s)
Adulto , Niño , Humanos , Colitis Ulcerosa , Colon , Enfermedad de Crohn , Diarrea , Edema , Epitelio , Fiebre , Estudios de Seguimiento , Hemorragia Gastrointestinal , Hemorragia , Hipoalbuminemia , Inflamación , Membrana Mucosa , Recto , Sulfasalazina , Úlcera , Pérdida de PesoRESUMEN
Nezelof's syndrome(combined immunodeficiency with immunoglobulin) is a hereditary primary immunodeficiency characterized by recurrent chronic pulmonary infections, oral and cutaneous candidiasis, failure to thrive, chronic diarrhea, skin infection, urinary tract infection, gram-negative sepsis, severe progressive varicella infection, lymphopenia, diminished lymphoid tissue, abnormal structure of the thymus, and presence of normal or increased levels of one or more of the major immunoglobulin classes, but with impaired antibody synthesis. We experienced a case of Nezelof's syndrome in 5-month-old boy who complained cough, poor feeding, vomiting and diarrhea in first admission day and discharged with recovered general condition in 38th admission day and then died of recurrent bacterial and fungal infections and malnutrition in 15-month-old age. He had no thymic shadow in chest X-ray and immunologic abnormalities including decreased lymphocyte count, decreased T cell count and function, normal number of B cell count and immunoglobulins with funtional impairment in antibody synthesis. We report a case of Nezelof's syndrome with brief review of related literatures.