Contributions
Abstract: PB2414
Type: Publication Only
Background
Nephropathy is a common complication of sickle cell anemia (SCA) and an important poor prognostic factor among these patients. It can rarely present as a nephrotic syndrome.
Aims
We aimed to describe the impact of hydroxyureia on nephrotic syndrome secondary to drepanocytosis in an adolescent.
Methods
Clinical case presentation.
Results
A 12-year-old African girl from São Tomé and Príncipe was diagnosed with sickle cell disease 6 years earlier. She remained asymptomatic and without any organ involvement up until the age of 11 when she moved to Portugal and presented with an inaugural nephrotic syndrome. Her initial urinary protein/creatinine ratio was 18,3mg/dL (normal <0,15) and she was started on prednisolone 60mg/m2/day. A renal biopsy was done which revealed a membranoproliferative glomerulonephritis with hemosiderin deposits in the tubular epithelium. However, she maintained a nephrotic proteinuria with episodic but exuberant malleolar swelling which brought her great discomfort and motivated frequent school absences. A minimum urine protein/creatinine ratio of 8mg/dL was reached while on prednisolone and enalapril. After the discontinuation of corticosteroid, she maintained a clinical and laboratory deterioration. Nine months after the inaugural episode, she was started on hydroxyurea to delay the progression of the renal disease. Since then, she showed regression of the edema, her serum albumin normalized and her urinary protein/creatinine ratio decreased (16,5mg/dL at the beginning of the treatment with hydroxyurea and 6,7mg/dL after 6 months). There was an increase in the fetal hemoglobin from 9% to 18%. She remains on both enalapril and hydroxyurea with no further complications. Ophthalmic and cardiac evaluations were normal and she has a normal middle cerebral artery flow velocity on the transcranial doppler.
Conclusion
The best approach to prevent the progression of nephropathy in patients with sickle cell disease remains to be determined. In this case, the use of corticosteroids and a renin-angiotensin-aldosterone inhibitor reduced the urinary protein/creatinine ratio, but the patient maintained a nephrotic proteinuria with episodes of severe edema. The initiation of hydroxyurea was effective in controlling the progression of the secondary nephropathy. We believe this was a result of limiting intraparenchymal sickling and thus decreasing the glomerular dysfunction. Starting treatment with hydroxyurea early on should therefore be considered in the treatment of renal disease in children with SCA.
Session topic: 27. Sickle cell disease
Keyword(s): Hydroxyurea, Renal
Abstract: PB2414
Type: Publication Only
Background
Nephropathy is a common complication of sickle cell anemia (SCA) and an important poor prognostic factor among these patients. It can rarely present as a nephrotic syndrome.
Aims
We aimed to describe the impact of hydroxyureia on nephrotic syndrome secondary to drepanocytosis in an adolescent.
Methods
Clinical case presentation.
Results
A 12-year-old African girl from São Tomé and Príncipe was diagnosed with sickle cell disease 6 years earlier. She remained asymptomatic and without any organ involvement up until the age of 11 when she moved to Portugal and presented with an inaugural nephrotic syndrome. Her initial urinary protein/creatinine ratio was 18,3mg/dL (normal <0,15) and she was started on prednisolone 60mg/m2/day. A renal biopsy was done which revealed a membranoproliferative glomerulonephritis with hemosiderin deposits in the tubular epithelium. However, she maintained a nephrotic proteinuria with episodic but exuberant malleolar swelling which brought her great discomfort and motivated frequent school absences. A minimum urine protein/creatinine ratio of 8mg/dL was reached while on prednisolone and enalapril. After the discontinuation of corticosteroid, she maintained a clinical and laboratory deterioration. Nine months after the inaugural episode, she was started on hydroxyurea to delay the progression of the renal disease. Since then, she showed regression of the edema, her serum albumin normalized and her urinary protein/creatinine ratio decreased (16,5mg/dL at the beginning of the treatment with hydroxyurea and 6,7mg/dL after 6 months). There was an increase in the fetal hemoglobin from 9% to 18%. She remains on both enalapril and hydroxyurea with no further complications. Ophthalmic and cardiac evaluations were normal and she has a normal middle cerebral artery flow velocity on the transcranial doppler.
Conclusion
The best approach to prevent the progression of nephropathy in patients with sickle cell disease remains to be determined. In this case, the use of corticosteroids and a renin-angiotensin-aldosterone inhibitor reduced the urinary protein/creatinine ratio, but the patient maintained a nephrotic proteinuria with episodes of severe edema. The initiation of hydroxyurea was effective in controlling the progression of the secondary nephropathy. We believe this was a result of limiting intraparenchymal sickling and thus decreasing the glomerular dysfunction. Starting treatment with hydroxyurea early on should therefore be considered in the treatment of renal disease in children with SCA.
Session topic: 27. Sickle cell disease
Keyword(s): Hydroxyurea, Renal