Volume 5, Issue 16 And 17 (4-1996)
JGUMS 1996, 5(16 And 17): 47-51 |
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Kohsari M, Borhani M. DRASH SYNDROME AND ITS FIRST CASE REPORT IN IRAN. JGUMS 1996; 5 (16 and 17) :47-51
URL: http://journal.gums.ac.ir/article-1-1881-en.html
URL: http://journal.gums.ac.ir/article-1-1881-en.html
Abstract: (1686 Views)
ABSTRACT:
DRASH Syndrome is a rare congenital disorder,and the triad of drash syndrome are progressive renal insufficiency ,male pseudohermaphroditism & Wilms tumor .All of the reported cases were sporadic and autosomal dominant inheritance was suspected in some references.
We diagnosed a 32- month child with this syndrome in IRAN in 1993. He was presented with wilms tumor and also had male pseudohermaphroditism and renal insufficiency. The patient died due to fluid & electrolyte disturbance .His parents had consanguinity marriage and thus there is a possibility of autosomal recessive inheritance. In this patient , the age for onset of nephropathy was higher than that in similar cases.
DRASH Syndrome is a rare congenital disorder,and the triad of drash syndrome are progressive renal insufficiency ,male pseudohermaphroditism & Wilms tumor .All of the reported cases were sporadic and autosomal dominant inheritance was suspected in some references.
We diagnosed a 32- month child with this syndrome in IRAN in 1993. He was presented with wilms tumor and also had male pseudohermaphroditism and renal insufficiency. The patient died due to fluid & electrolyte disturbance .His parents had consanguinity marriage and thus there is a possibility of autosomal recessive inheritance. In this patient , the age for onset of nephropathy was higher than that in similar cases.
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Received: 2019/03/10 | Accepted: 2019/03/10 | Published: 2019/03/10
Received: 2019/03/10 | Accepted: 2019/03/10 | Published: 2019/03/10
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