| Congenital erythropoietic porphyria associated with nephrotic syndrome and renal siderosis. | |
| | |
MedLine Citation:
|
PMID: 8580639 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
A 9-year-old boy with typical features of congenital erythropoietic porphyria who had received more than 50 blood transfusions developed the steroid-resistant nephrotic syndrome in the presence of normal glomerular function and glucosuria. Renal biopsy showed focal segmental glomerulosclerosis and widespread iron deposits. Magnetic resonance scanning revealed advanced siderosis of liver and kidneys. During a 4 year treatment by desferrioxamine the serum ferritin level was reduced, proteinuria dropped and serum proteins increased whilst glomerular filtration decreased slowly. It is suggested that the nephrotic syndrome may be a consequence of renal siderosis amenable to iron-chelating therapy. |
| | |
Authors:
|
B Lange; K Hofweber; R Waldherr; K Schärer |
Related Documents
:
|
8507929 - Nephrotic syndrome with massive accumulation of type i and type iii collagen in the glo... 8124919 - Colchicine induced remission in amyloid nephrotic syndrome. 15549409 - Necrotizing fasciitis in a child: a rare complication of idiopathic nephrotic syndrome. 2465229 - Plasminogen activator in nephrotic syndrome. 6731499 - Use of noninvasive laboratory testing in the prediction of thrombosis in the nephrotic ... 19066979 - Familial forms of nephrotic syndrome. 7749669 - Iris melanoma in a ten-year-old boy with familial atypical mole-melanoma (fam-m) syndrome. 20054529 - Infantile form of scimitar syndrome with contralateral pulmonary vein stenosis. 22108219 - Craniosynostosis and 3-dimensional computed tomography. |
Publication Detail:
|
Type: Case Reports; Journal Article |
Journal Detail:
|
Title: Acta paediatrica (Oslo, Norway : 1992) Volume: 84 ISSN: 0803-5253 ISO Abbreviation: Acta Paediatr. Publication Date: 1995 Nov |
Date Detail:
|
Created Date: 1996-03-18 Completed Date: 1996-03-18 Revised Date: 2004-11-17 |
Medline Journal Info:
|
Nlm Unique ID: 9205968 Medline TA: Acta Paediatr Country: NORWAY |
Other Details:
|
Languages: eng Pagination: 1325-8 Citation Subset: IM |
Affiliation:
|
Department of Paediatrics, University of Heidelberg, Germany. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Anti-Inflammatory Agents
/
administration & dosage,
therapeutic use Child Deferoxamine / administration & dosage, therapeutic use Glomerular Filtration Rate Humans Iron Chelating Agents / administration & dosage, therapeutic use Kidney / physiopathology* Liver / physiopathology Male Nephrotic Syndrome / etiology* Porphyria, Erythropoietic / complications*, drug therapy Prednisone / administration & dosage, therapeutic use Siderosis / drug therapy, etiology*, physiopathology* |
| Chemical | |
Reg. No./Substance:
|
0/Anti-Inflammatory Agents; 0/Iron Chelating Agents; 53-03-2/Prednisone; 70-51-9/Deferoxamine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Imperforate anus, bilateral hydronephrosis, bilateral undescended testes and pituitary hypoplasia: a...
Next Document: Clinical and immunohistochemical findings in a case of neonatal Marfan syndrome.