دورية أكاديمية
Hypertension, Chronic Kidney Disease, and Renal Pathology in a Child with Hermansky-Pudlak Syndrome
| العنوان: | Hypertension, Chronic Kidney Disease, and Renal Pathology in a Child with Hermansky-Pudlak Syndrome |
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| المؤلفون: | Roberto Gordillo, Marcela Del Rio, David B. Thomas, Joseph T. Flynn, Robert P. Woroniecki |
| المصدر: | International Journal of Nephrology, Vol 2011 (2011) |
| بيانات النشر: | Wiley, 2011. |
| سنة النشر: | 2011 |
| المجموعة: | LCC:Diseases of the genitourinary system. Urology |
| مصطلحات موضوعية: | Diseases of the genitourinary system. Urology, RC870-923 |
| الوصف: | 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. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2090-214X 2090-2158 |
| Relation: | https://doaj.org/toc/2090-214X; https://doaj.org/toc/2090-2158 |
| DOI: | 10.4061/2011/324916 |
| URL الوصول: | https://doaj.org/article/a5c2beda99fc47348400fc1520d4ad3e |
| رقم الأكسشن: | edsdoj.5c2beda99fc47348400fc1520d4ad3e |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 2090214X 20902158 |
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| DOI: | 10.4061/2011/324916 |