Rare Diseases Symptoms Automatic Extraction

Assessment of renal involvement in patients with familial Mediterranean fever: a clinical study from Ardabil, Iran.

[familial mediterranean fever]

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent episodes of painful inflammation in the abdomen, chest or joints. The association between FMF and non-amyloid glomerulopathies are unusual. In this study, we describe our experiences and observations about renal involvement in patients with FMF.A total of 108 patients with FMF was enrolled in the study. Twelve patients with FMF were referred to the Nephrology Service, for evaluation and assessment of the degree of renal involvement. All the 12 patients underwent percutaneous ultrasound-guided renal biopsies and genetic analysis.On microscopic examination of the kidney specimens, six patients were found to have amyloidosis, five focal segmental glomerulosclerosis and one patient membranoproliferative glomerulonephritis. It seems that in patients with FMF and renal amyloidosis, the response to treatment with colchicine is excellent, but in patients with FMF and focal segmental glomerulosclerosis, the response to treatment with colchicine is poor. We present an evidence-based algorithm, constructed based on literature review, to aid decision making in management of renal involvement in patients with FMF.The results of our study suggest that in patients with FMF and renal involvement, non-amyloid renal lesions should be considered in the differential diagnosis in addition to amyloidosis.

Diseases presenting "fever" symptom

  • 22q11.2 deletion syndrome
  • acute rheumatic fever
  • alexander disease
  • allergic bronchopulmonary aspergillosis
  • canavan disease
  • carcinoma of the gallbladder
  • child syndrome
  • congenital toxoplasmosis
  • cushing syndrome
  • cystinuria
  • dracunculiasis
  • erdheim-chester disease
  • esophageal adenocarcinoma
  • esophageal carcinoma
  • familial mediterranean fever
  • focal myositis
  • hodgkin lymphoma, classical
  • lamellar ichthyosis
  • legionellosis
  • locked-in syndrome
  • malignant atrophic papulosis
  • neonatal adrenoleukodystrophy
  • neuralgic amyotrophy
  • oculocutaneous albinism
  • papillon-lefèvre syndrome
  • pyomyositis
  • pyruvate dehydrogenase deficiency
  • scrub typhus
  • severe combined immunodeficiency
  • sneddon syndrome
  • systemic capillary leak syndrome
  • triple a syndrome
  • typhoid
  • waldenström macroglobulinemia
  • wolf-hirschhorn syndrome

This symptom has already been validated