Document Detail

Thrombotic microangiopathic nephropathy, pulmonary hypertension and nephromegaly: case report of a patient treated with endothelin receptor antagonist.
MedLine Citation:
PMID:  22257549     Owner:  NLM     Status:  MEDLINE    
The coexistence of thrombotic microangiopathic nephropathy and pulmonary hypertension has only been described in association with malignancy and its treatment. Here we describe a 14-year-old boy with no prior medical history who presented with hypertension, proteinuria and nephromegaly, and then developed progressive pulmonary hypertension. Renal histology showed lesions consistent with glomerulopathy due to thrombotic microangiopathy (TMA). Pulmonary hypertension was controlled by the use of an oral endothelin receptor antagonist (bosentan). Although renal function deteriorated at the onset of pulmonary hypertension, an improvement was observed after the bosentan treatment. Nephromegaly persisted, but current creatinine clearance values are within the normal range. While this case exemplifies how thrombotic microangiopathic nephropathy may be associated with pulmonary hypertension, a therapeutic role of endothelin antagonists is suggested, not only for pulmonary hypertension but also for microangiopathic nephropathy.
Attilio Losito; Loretta Pittavini; Carla Covarelli
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Clinical nephrology     Volume:  77     ISSN:  0301-0430     ISO Abbreviation:  Clin. Nephrol.     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-01-19     Completed Date:  2012-04-10     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  0364441     Medline TA:  Clin Nephrol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  164-70     Citation Subset:  IM    
Struttura Complessa Nefrologia e Dialisi, Ospedale Santa Maria della Misericordia, Perugia, Italy.
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MeSH Terms
Antihypertensive Agents / therapeutic use*
Hypertension, Pulmonary / drug therapy*
Kidney / pathology*
Receptors, Endothelin / antagonists & inhibitors*
Sulfonamides / therapeutic use*
Thrombotic Microangiopathies / drug therapy*
Reg. No./Substance:
0/Antihypertensive Agents; 0/Receptors, Endothelin; 0/Sulfonamides; Q326023R30/bosentan

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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