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Moyamoya proteinuria

Elen A Romão, Charles M Lourenço, Wilson Marques Júnior, Arndt Rolfs, Veronica Muñoz, Osvaldo M Vieira Neto, Marcio Dantas, Gyl E B Silva, Roberto S Costa
Fabry disease is an X-linked inborn error of metabolism, which is caused by the deficiency of α-galactosidase A, leading to progressive accumulation of neutral glycosphingolipids and a-galactosyl breakdown products in most body fluids and several tissues, resulting in the clinical manifestations. The onset of Fabry disease symptoms in females is not observed as early as in males. We report a novel presentation of Fabry disease in a female patient with medical history of relapsing strokes and brain magnetic resonance angiography showing signs of microangiopathy and multiple lacunar strokes that were first diagnosed as Moyamoya disease (a chronic progressive cerebrovascular disease)...
April 2013: Clinical Nephrology
Jun Kakogawa, Miyuki Sadatsuki, Norio Masuya, Hideto Gomibuchi, Hiroyasu Ohno, Tetsuo Hara, Hiroko Oda, Akio Kimura, Shigeki Minoura
PURPOSE: Moyamoya (meaning a "hazy puff of smoke" in Japanese) disease is a rare cerebrovascular occlusive disease. Moyamoya disease may become symptomatic for the first time during pregnancy. We report a case of antepartum intracranial hemorrhage due to unrecognized unilateral moyamoya disease, which was subsequently diagnosed as HELLP syndrome during the postpartum period. STUDY DESIGN: A case report of a 29-year-old Japanese primigravida who was transported to our hospital at 39 weeks of gestation because of sudden loss of consciousness and left hemiplegia...
March 2011: Archives of Gynecology and Obstetrics
F Kuwayama, Y Hamasaki, T Shinagawa, C Kubota, I Ichikawa, Y Kato, S Oi, O Shinohara
A 7-year-old boy with moyamoya disease developed sustained hypertension, nephrotic syndrome, hyperreninemia, and occlusion of the right renal artery. After right nephrectomy, hyperreninemia and hypertension improved. Proteinuria was resolved after nephrectomy, in parallel with the decrease in plasma renin activity. Moyamoya disease can cause nephrotic-range proteinuria, which is caused hemodynamically by hyperreninemia.
March 2001: Journal of Pediatrics
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