Hypertension, Chronic Kidney Disease, and Renal Pathology in a Child with Hermansky-Pudlak Syndrome
Joint Authors
Thomas, David B.
Gordillo, Roberto
Flynn, Joseph T.
Woroniecki, Robert P.
Del Rio, Marcela
Source
International Journal of Nephrology
Issue
Vol. 2011, Issue 2011 (31 Dec. 2011), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2011-07-05
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
We report a child with Hermansky-Pudlak Syndrome (HPS) and chronic kidney disease (stage II) with histological diagnosis of focal segmental glomerulosclerosis (FSGS).
A 15-year-old male of Puerto Rico ancestry with history of HPS, hypertension (HTN), asthma, obesity, and chronic kidney disease (CKD) stage II presented with new-onset proteinuria without edema.
His blood pressure had been controlled, serum creatinine had been 0.9–1.4 mg/dL, and first morning urine protein/creatinine ratio (UPC) ranged from 0.2 to 0.38.
Due to persistent nonorthostatic proteinuria with CKD, renal biopsy was performed and FSGS (not otherwise specified) with chronic diffuse tubulopathy (tubular cytoplasmic droplets) and acute tubular injury was reported.
Ceroid-like material is known to infiltrate tissues (i.e., lungs, colon, and kidney) in HPS, but the reason for the renal insufficiency is unknown.
Nonspecific kidney disease and in one adult case IgA nephropathy with ANCA-positive glomerulonephritis have previously been reported in patients with Hermansky-Pudlak syndrome.
To our knowledge, we report the first pediatric renal pathology case of HPS associated with CKD.
This paper discusses presentation and management of renal disease in HPS.
American Psychological Association (APA)
Gordillo, Roberto& Del Rio, Marcela& Thomas, David B.& Flynn, Joseph T.& Woroniecki, Robert P.. 2011. Hypertension, Chronic Kidney Disease, and Renal Pathology in a Child with Hermansky-Pudlak Syndrome. International Journal of Nephrology،Vol. 2011, no. 2011, pp.1-5.
https://search.emarefa.net/detail/BIM-463593
Modern Language Association (MLA)
Gordillo, Roberto…[et al.]. Hypertension, Chronic Kidney Disease, and Renal Pathology in a Child with Hermansky-Pudlak Syndrome. International Journal of Nephrology No. 2011 (2011), pp.1-5.
https://search.emarefa.net/detail/BIM-463593
American Medical Association (AMA)
Gordillo, Roberto& Del Rio, Marcela& Thomas, David B.& Flynn, Joseph T.& Woroniecki, Robert P.. Hypertension, Chronic Kidney Disease, and Renal Pathology in a Child with Hermansky-Pudlak Syndrome. International Journal of Nephrology. 2011. Vol. 2011, no. 2011, pp.1-5.
https://search.emarefa.net/detail/BIM-463593
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-463593