RESUMO
Exponemos nuestra experiencia en cincuenta y cinco casos de sindrome de Schoenlein-Henoch con afeccion renal. Desde el punto de vista clinico se clasificaron en cinco grupos: 1) Hematuria y proteinuria, 2) Hematuria y sindrome nefrotico, 3) Hematuria 4) Hematuria, sindrome nefrotico e insuficiencia renal y 5) proteinuria. Desde el punto de vista anatomico se clasificaron en seis tipos segun los criterios del ISKDC. Se halla correlacion en cuanto a peor pronostico en la forma clinica de inicio con sidrome nefrotico e insuficiencia renal y en la lesion anatomica con semilunas en porcentaje superior al 75% de los glomerulos
Assuntos
Pré-Escolar , Criança , Adolescente , Humanos , Masculino , Feminino , Nefropatias , Vasculite por IgARESUMO
Twenty-six preadolescent children ( 16 boys and 10 girls) with minimal lesion nephrotic syndrome, who were either unresponsive to or dependent on corticosteroid therapy, were treated exclusively with chlorambucil. Evaluation of chlorambucil toxicity on the reproductive system was based on penile and testicular size, serum testosterone values, and sperm count. Nine boys had azoospermia and one had oligospermia. The other six, who received a mean cumulative dose of 10.2 mg/kg of chlorambucil, had a normal sperm count. However, we consider that the total safe dose of chlorambucil should not exceed 8.2 mg/kg, equivalent to six weeks treatment at a daily dose of 0.2 mg/kg. In the ten girls, pubertal development was normal; axillary and pubic hair, breast development, age at onset of menarche, and length of the menstrual cycle were all normal; however, the possibility of abnormalities in ovarian functions cannot be excluded.
Assuntos
Clorambucila/efeitos adversos , Síndrome Nefrótica/tratamento farmacológico , Maturidade Sexual/efeitos dos fármacos , Espermatogênese/efeitos dos fármacos , Adolescente , Adulto , Clorambucila/administração & dosagem , Feminino , Humanos , Masculino , Caracteres Sexuais , Contagem de EspermatozoidesRESUMO
Cystinuria is a disease characterized by the excessive elimination of cystine and of dibasic amino acids (lysine, arginine, and ornithine) through urine of homozygotes. This study included 6 children complaining of abdominal pain with or without hematuria. The existence of renal radio-opaque lithiasis was confirmed in 5 of them and in the sixth, it was vesical. The clinical and analytic data were practically normal with the exception of the qualitative test of the amino acid urinary excretion that showed increase in urinary excretion of cystine. Likewise, percentages of tubular reabsorption were pathological in all the patients showing values between 35.4% and 74%. The diagnosis of systini-lysinuric lithiasis was established through amino acid excretion study in the six patients which was below normal; it fluctuated between 36% and 74%. Lysine, together with cystine, was the most frequently affected.
Assuntos
Cistinúria/diagnóstico , Cálculos Renais/diagnóstico , Lisina/urina , Cálculos da Bexiga Urinária/diagnóstico , Adolescente , Criança , Pré-Escolar , Cisteína/urina , Cistinúria/genética , Cistinúria/urina , Feminino , Humanos , Cálculos Renais/genética , Cálculos Renais/urina , Masculino , Linhagem , Cálculos da Bexiga Urinária/genética , Cálculos da Bexiga Urinária/urinaRESUMO
Batter's syndrome is characterized by retardation in growth pitressin-resistant hypostenuria, hypokalemic alkalosis, high activity of the jux angiotensin-renine-aldosterone system with normal blood pressure; vascular insensibility to angiotensin and hypertrophy and hyperplasia of the juxtaglomerular apparatus. In both patients we studied, we found negative balance of sodium and potassium conditioned to a renal loss of them.
Assuntos
Síndrome de Bartter/fisiopatologia , Hiperaldosteronismo/fisiopatologia , Sistema Justaglomerular/fisiopatologia , Síndrome de Bartter/diagnóstico , Pré-Escolar , Feminino , Taxa de Filtração Glomerular , Humanos , Masculino , Potássio/urina , Sódio/urina , Equilíbrio HidroeletrolíticoRESUMO
In our experience, hypervitaminosis D, which at present should only be a historic remembrance in pediatrics, is still a frequent cause of hipercalcemia in childhood. It is easy understand that its appearance should turn out to be a complication in the treatment of hypoparathyroidisms or in vitamin D resistant rickets, but its persistance as a purely iatrogenic diseases is at present inexplicable. The frequency with which we have seen it has led us to review 15 cases where erroneous administration of high doses of vitamin D has resulted in serious clinical, biological and anatomical problems.