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Literature DB >> 15278422

Paroxysmal nocturnal hemoglobinuria in a girl with hemolysis and "hematuria".

Zdenek Dolezel¹, Dana Dostalkova, Jan Blatny, Jiri Starha, Hana Gerykova.

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder of unknown frequency. In its classic form, PNH is characterized by hemolysis accompanied by nocturnal hemoglobinuria. The clinical course is unpredictable and may vary from severe hemolysis and recurrent venous thrombosis to latent periods with milder symptoms. We report a 15-year-old girl with hemolytic episodes, abdominal pain, and passage of dark urine. Hemoglobinuria was demonstrated by a "blood"-positive dipstick test in the absence of red blood cells in the urinary sediment. The diagnosis of PNH was confirmed by flow cytometry.

Entities: Disease Species

Mesh：

Year: 2004 PMID： 15278422 DOI： 10.1007/s00467-004-1563-4

Source DB: PubMed Journal: Pediatr Nephrol ISSN： 0931-041X Impact factor: 3.714

18 in total

1. Paroxysmal nocturnal haemoglobinuria. MRI of renal cortical haemosiderosis in two patients, including one renal transplant.

Authors: G Verswijvel; D Vanbeckevoort; B Maes; R Oyen
Journal: Nephrol Dial Transplant Date: 1999-06 Impact factor: 5.992

2. Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. French Society of Haematology.

Authors: G Socié; J Y Mary; A de Gramont; B Rio; M Leporrier; C Rose; P Heudier; H Rochant; J Y Cahn; E Gluckman
Journal: Lancet Date: 1996-08-31 Impact factor: 79.321

Review 3. Clinical manifestations of paroxysmal nocturnal hemoglobinuria: present state and future problems.

Authors: Wendell F Rosse; Junichi Nishimura
Journal: Int J Hematol Date: 2003-02 Impact factor: 2.490

Review 4. Management issues in paroxysmal nocturnal hemoglobinuria.

Authors: Gabrielle Meyers; Charles J Parker
Journal: Int J Hematol Date: 2003-02 Impact factor: 2.490

5. The kidneys in paroxysmal nocturnal hemoglobinuria.

Authors: D A Clark; S A Butler; V Braren; R C Hartmann; D E Jenkins
Journal: Blood Date: 1981-01 Impact factor: 22.113

6. Recurrent PIG-A mutation (IVS5+1G-->A) in a paediatric case of paroxysmal nocturnal haemoglobinuria: detection by the protein truncation test.

Authors: C Maugard; G Margueritte; S Tuffery; H Rabesandratana; J Demaille; M Claustres
Journal: Br J Haematol Date: 1997-07 Impact factor: 6.998

Paroxysmal nocturnal hemoglobinuria in a girl with hemolysis and "hematuria".

1. Paroxysmal nocturnal haemoglobinuria. MRI of renal cortical haemosiderosis in two patients, including one renal transplant.

2. Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. French Society of Haematology.

Review 3. Clinical manifestations of paroxysmal nocturnal hemoglobinuria: present state and future problems.

Review 4. Management issues in paroxysmal nocturnal hemoglobinuria.

5. The kidneys in paroxysmal nocturnal hemoglobinuria.

6. Recurrent PIG-A mutation (IVS5+1G-->A) in a paediatric case of paroxysmal nocturnal haemoglobinuria: detection by the protein truncation test.

7. Paroxysmal nocturnal hemoglobinuria with onset in childhood and adolescence.

Review 8. Recent insights into the pathophysiology of paroxysmal nocturnal hemoglobinuria.

Review 9. Laboratory diagnosis of paroxysmal nocturnal hemoglobinuria.

10. Natural history of paroxysmal nocturnal hemoglobinuria.

1. Acute renal failure in a patient with severe hemolysis.

2. New Insights in Abdominal Pain in Paroxysmal Nocturnal Hemoglobinuria (PNH): A MRI Study.