RESUMEN
Hodgkin's disease as a second malignancy in childhood acute lymphoblastic leukemia is of rare occurrence, with the first such case having only recently been described. The therapy for acute lymphoblastic leukemia may alter the pattern of presentation of Hodgkin's disease and its histology, making diagnosis difficult. In this report we discuss the clinical, histologic, and ultrastructural findings of a child with acute lymphoblastic leukemia in remission who had pulmonary involvement as an unusual initial presentation of Hodgkin's disease.
Asunto(s)
Enfermedad de Hodgkin/patología , Leucemia Linfoide/patología , Neoplasias Pulmonares/patología , Neoplasias Primarias Múltiples , Enfermedad Aguda , Médula Ósea/patología , Niño , Histiocitos/patología , Humanos , Pulmón/patología , Masculino , Microscopía Electrónica , Monocitos/patologíaRESUMEN
Two new cases plus 13 previously reported cases of Menetrier's disease in childhood are reviewed. The most common features of this illness are severe upper gastrointestinal symptoms (abdominal pain, nausea, vomiting, hematemesis) followed by the onset of generalized edema and ascites. Significant laboratory findings include hypoalbuminemia, eosinophilia, and anemia. Gastrointestinal protein loss and decreased gastric acid secretion can be documented. Upper gastrointestinal radiographs demonstrate the characteristic hypertrophic gastric rugae. Histologic features include hypertrophic tortuous gastric glands, basilar cysts, and interstitial round cell inflammation. The natural course of this disease in childhood is usually benign and self-limited. Uncommonly, the course may be severe and require gastric resection. This is in contrast to the adult form, where chronicity and severity is the rule. The etiology of this problem remains unknown. Endoscopy and biopsy are the diagnostic procedures of choice, although laparotomy may be necessary in equivocal cases. Therapy should be supportive except for those few patients who require surgery to control hemorrhage.
Asunto(s)
Gastritis Hipertrófica/diagnóstico , Gastritis/diagnóstico , Niño , Preescolar , Sistema Digestivo/diagnóstico por imagen , Femenino , Gastritis Hipertrófica/diagnóstico por imagen , Gastritis Hipertrófica/patología , Humanos , Masculino , Radiografía , Estómago/patologíaRESUMEN
Children with nephrotic syndrome frequently have decreased plasma levels of Hageman factor (factor XII). Studies were designed to determine whether this finding results primarily from a loss of Hageman factor in the urine or is related to an alteration in the Hageman factor protein itself. A monospecific anti-Hageman factor goat antibody was prepared and used for quantitative measurements of Hageman factor in urine and plasma by a radial immunodiffusion method. Samples of 24 hr urine collections from 14 children with nephrotic syndrome were examined by this immunoassay technique; no Hageman factor was detected in the urine in 13/14 patients. Comparative studies using plasma from the nephrotic syndrome patients and nine normal controls demonstrated: (1) a significantly reduced level of Hageman ACT in the nephrotic syndrome patients (p < 0.001); (2) a reduction to a lesser degree of the levels of CRM in the same group (p < 0.001); and (3) a significantly increased CRM/ACT ratio in the plasma of the patients with nephrotic syndrome (p < 0.01) indicating partial inactivation of the Hageman factor enzymatic function. However, no inhibitory activity to added Hageman factor could be detected in the plasma or urine of the patients. These findings indicate that the decreased plasma Hageman factor activity that accompanies the nephrotic syndrome cannot be adequately explained by simple urinary loss of Hageman factor as has previously been suggested.
Asunto(s)
Factor XII/orina , Síndrome Nefrótico/complicaciones , Adolescente , Animales , Especificidad de Anticuerpos , Trastornos de la Coagulación Sanguínea/complicaciones , Pruebas de Coagulación Sanguínea , Niño , Preescolar , Factor VIII , Deficiencia del Factor XI/complicaciones , Factor XII/inmunología , Deficiencia del Factor XII/complicaciones , Fibrinógeno , Cabras , Humanos , Inmunoelectroforesis , Lactante , Síndrome Nefrótico/sangre , Síndrome Nefrótico/orina , PrecalicreínaAsunto(s)
Neoplasias Renales/complicaciones , Tumor de Wilms/complicaciones , Enfermedades de von Willebrand/etiología , Pruebas de Coagulación Sanguínea , Factor VIII/análisis , Humanos , Lactante , Neoplasias Renales/cirugía , Masculino , Tumor de Wilms/cirugía , Enfermedades de von Willebrand/sangreRESUMEN
Nine episodes of a unique short-incubation form of hepatitis were observed during five years in six hemophilic children after infusion with commercial factor VIII concentrate prepared by two different manufacturers. Five patients with a single episode had no previous infusion for 14 months to 14 years. One patient with several episodes had no previous infusion for at least seven months preceding each episode. The illness was mild and self-limited. No seroconversions to cytomegalovirus, Epstein-Barr virus, toxoplasmosis, or hepatitis A virus occurred. Acute hepatitis B virus infection was also excluded. The findings suggest the presence of one or more non-A, non-B hepatitis agents associated with factor VIII concentrates.