Different glomerular pathologies in sickle cell anemia

Balal, Mustafa; Paydas, S; Seyrek, Neslihan; Karayaylalı, İbrahim

doi:10.5414/cnp62400

Cited by 4 publications

(3 citation statements)

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“…Membranoproliferative glomerulonephritis and focal segmental glomerulosclerosis accounted for most cases of nephrotic syndrome in SCA patients. In a review of the literature, there are only three adult cases reported with SCA and amyloidosis [3][4][5]. One had M694V mutation, the most common genetic marker in FMF, and for the other two, it was suggested that frequent sickle cell crises are likely to provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses (serum amyloid A) and development of a predisposition to generation and deposition of amyloid protein [3][4][5].…”

Section: Discussionmentioning

confidence: 99%

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Renal amyloidosis in a child with sickle cell anemia

et al. 2006

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The kidney is frequently affected in patients with sickle cell syndrome, i.e., homozygous and heterozygous patients, with a consequently large spectrum of renal abnormalities that may range from minimal functional changes to chronic renal failure. Here, we present a 13-year-old boy with sickle cell anemia (SCA) (HbSS) who was referred to our unit with nephrotic syndrome. Renal biopsy revealed AA type amyloidosis on the basis of light microscopic findings, indicating Congo red staining and immunohistochemistry. He had neither a family history of familial Mediterranean fever (FMF) nor any complaint of recurrent abdominal pain, arthritis, and fever, but frequent painful vaso-occlusive crises. The patient was found to have no MEFV gene (Mediterranean feVer) mutations either. Painful episodic attacks might provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses and cause secondary amyloidosis. To our knowledge, this boy is the first case of SCA complicated by renal amyloidosis observed in childhood.

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Section: Discussionmentioning

confidence: 99%

“…To date, coexistence between SCA and renal amyloidosis in children has never been reported yet and only three adult cases [3][4][5]. Herein, we present a 13-year-old boy with an interesting coexistence between SCA (HbSS) and renal AA type amyloidosis who has been suffering from nephrotic syndrome.…”

Section: Introductionmentioning

confidence: 94%

Renal amyloidosis in a child with sickle cell anemia

et al. 2006

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“…In fact, only four cases of renal amyloidosis in patients with SCA have been documented in the literature. [4][5][6] The E148Q gene mutation is commonly associated with Mediterranean Fever (MEFV) in Turkey, [2] and in more than half of the cases, it is homozygous. The most prominent clinical aspects of patients with the E148Q gene mutation are abdominal pain and fever.…”

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confidence: 99%

Secondary Amiloidosis and Familial Mediterranean Fever Mimicking Recurrent Abdominal Sickling Crisis in a Patient with Sickle Cell Anemia

et al. 2013

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Sickle cell disease complicated by post‐streptococcal glomerulonephritis, cerebral hemorrhage and reversible posterior leucoencephalopathy syndrome

Pashankar

Ment

Pearson

2007

Pediatric Blood & Cancer

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A patient with homozygous hemoglobin SS disease presented with an intracerebral hemorrhage complicating reversible posterior leucoencephalopathy syndrome (RPLS), secondary to hypertension associated with acute post-streptococcal glomerulonephritis (APSGN). Distinguishing potentially reversible causes of central nervous system events from primary cerebral infarction or hemorrhage in patients with sickle cell disease is important because the management and prognosis of these complications is very different. Similarly, because of the difference in prognosis between APSGN and other forms of sickle cell nephropathy, it is also important to differentiate these conditions.

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Different glomerular pathologies in sickle cell anemia

Cited by 4 publications

References 0 publications

Renal amyloidosis in a child with sickle cell anemia

Renal amyloidosis in a child with sickle cell anemia

Secondary Amiloidosis and Familial Mediterranean Fever Mimicking Recurrent Abdominal Sickling Crisis in a Patient with Sickle Cell Anemia

Sickle cell disease complicated by post‐streptococcal glomerulonephritis, cerebral hemorrhage and reversible posterior leucoencephalopathy syndrome

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