Síndrome nefrótica primária grave em crianças: descrição clínica e dos padrões histológicos renais de seis casos
Carregando...
Data
2006-10-01
Autores
Riyuzo, Márcia Camegaçava [UNESP]
Viero, Rosa Marlene [UNESP]
Macedo, Célia Sperandeo [UNESP]
Bastos, Herculano Dias [UNESP]
Título da Revista
ISSN da Revista
Título de Volume
Editor
Sociedade Brasileira de Patologia ClínicaSociedade Brasileira de PatologiaSociedade Brasileira de Citopatologia
Resumo
Os autores relatam os casos de seis crianças com síndrome nefrótica primária grave de padrão histológico renal incomum na rotina cotidiana dos nefrologistas e patologistas. O diagnóstico da doença foi realizado nas faixas etárias de 3 a 9 meses de idade (n = 4), aos 2 anos e 4 meses (n = 1) e aos 11 anos (n = 1). Um paciente foi prematuro, duas pacientes eram irmãs e seus pais eram primos de primeiro grau. Todos apresentavam edema generalizado; dois pacientes apresentavam desnutrição e hipotireoidismo e dois apresentavam hipertensão arterial e insuficiência renal. A histologia renal mostrou esclerose mesangial difusa (n = 3), proliferação mesangial (n = 2) e síndrome nefrótica do tipo finlandês (n = 1). Quatro pacientes faleceram, as causas de óbito foram infecção (n = 2), insuficiência renal (n = 1) e acidose metabólica (n = 1). Entre os sobreviventes, um paciente foi tratado com vitaminas, tiroxina, captopril e indometacina, apresentando aumento da albumina sérica e melhora do crescimento. O outro paciente apresentava insuficiência renal terminal, sendo tratado com diálise e transplante renal.
The authors report six children with severe primary nephrotic syndrome with unusual renal histological patterns in the daily routine of nephrologists and pathologists. The diagnosis of the disease was made at the age between 3 to 9 months (n = 4), at 2 years and 4 months (n = 1) and at 11 years (n = 1). One patient was born prematurely; two patients were sisters and their parents were first-degree cousins. All patients presented generalized edema, two patients presented malnutrition and hypothyroidism; two patients presented hypertension and renal failure. The renal histology showed diffuse mesangial sclerosis (n = 3); diffuse mesangial hypercellularity (n = 2) and nephrotic syndrome of the Finnish type (n = 1). Four patients died, causes of death were infection (n = 2), renal failure (n = 1) and metabolic acidosis (n = 1). Among the survivors, one patient was treated with vitamins, thyroxine, captopril and indomethacin and presented increase in serum albumin and of growth. The other patient, who presented end-stage renal disease, was dialyzed and transplanted.
The authors report six children with severe primary nephrotic syndrome with unusual renal histological patterns in the daily routine of nephrologists and pathologists. The diagnosis of the disease was made at the age between 3 to 9 months (n = 4), at 2 years and 4 months (n = 1) and at 11 years (n = 1). One patient was born prematurely; two patients were sisters and their parents were first-degree cousins. All patients presented generalized edema, two patients presented malnutrition and hypothyroidism; two patients presented hypertension and renal failure. The renal histology showed diffuse mesangial sclerosis (n = 3); diffuse mesangial hypercellularity (n = 2) and nephrotic syndrome of the Finnish type (n = 1). Four patients died, causes of death were infection (n = 2), renal failure (n = 1) and metabolic acidosis (n = 1). Among the survivors, one patient was treated with vitamins, thyroxine, captopril and indomethacin and presented increase in serum albumin and of growth. The other patient, who presented end-stage renal disease, was dialyzed and transplanted.
Descrição
Palavras-chave
Síndrome nefrótica, Crianças, Nephrotic syndrome, Children
Como citar
Jornal Brasileiro de Patologia e Medicina Laboratorial. Sociedade Brasileira de Patologia ClínicaSociedade Brasileira de PatologiaSociedade Brasileira de Citopatologia, v. 42, n. 5, p. 393-400, 2006.