Clinical Characteristics Study of Pseudohypoparathyroidism / 대한소아내분비학회지
Journal of Korean Society of Pediatric Endocrinology
;
: 105-111, 2002.
Artigo
em Coreano
| WPRIM
| ID: wpr-33528
ABSTRACT
PURPOSE:
Pseudohypoparathyroidism(PHP) is caused by a defect of G protein and receptor despite of normal parathyroid hormone(PTH) secretion. It is a rare disorder characterized by hypocalcemia, hyperphophatemia, elevated PTH levels and albright hereditory osteodystrophy(AHO). We retrospectively reviewed the clinical characteristics of PHP.METHODS:
We reviewed clinical features, laboratory findings, and outcome to treatment of 8 PHP patients, diagnosed at Seoul National University Hospital from 1988 to rool.RESULTS:
Male to Female ratio was 1.71 and mean age at diagnosis was 11.8 years old. The initial average height SDS was 0.13+/-.08 and the average weight SDS was 0.43+/-.31. The most common symptom was seizure. Only one patient had typical AHO, three patients had mental retardation. Brain MRI or CT showed basal ganglia calcification in 3 patients. All patients treated with vitamin D and calcium supplementation could maintained normal serum levels of calcium and phophorus.CONCLUSION:
PHP should be suspected in patient with seizure of unknown origin, aged above 5 year-old. AHO and mental retardation could be adjuvant signs to the diagnosis of PHP. But definite diagnosis could be made by laboratory work up.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Pseudo-Hipoparatireoidismo
/
Convulsões
/
Gânglios da Base
/
Vitamina D
/
Encéfalo
/
Imageamento por Ressonância Magnética
/
Cálcio
/
Estudos Retrospectivos
/
Proteínas de Ligação ao GTP
/
Diagnóstico
Tipo de estudo:
Estudo diagnóstico
/
Estudo observacional
Limite:
Criança, pré-escolar
/
Feminino
/
Humanos
/
Masculino
País/Região como assunto:
Ásia
Idioma:
Coreano
Revista:
Journal of Korean Society of Pediatric Endocrinology
Ano de publicação:
2002
Tipo de documento:
Artigo
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