(benign paroxysmal peritonitis)
- Familial Mediterranean fever with onset in the 70s showing various neutrophilic dermatosis. [Letter]
- JEJ Eur Acad Dermatol Venereol 2016; 30(11):e129-e131
- Case report: recurrent abdominal symptoms in a child with panhypopituitarism - there is always a differential. [Journal Article]
- IJInt J Pediatr Endocrinol 2016; 2016:18
- CONCLUSIONS: The clinical pictures of panhypopituitarism and FMF can be overlapping. It is imperative to take a detailed and accurate history in order to find the right diagnosis, particularly a precise family history. In conditions like FMF an early diagnosis is crucial, as initiation of treatment with colchicine is important to prevent long-term complications due to amyloid fibril deposition.
- Role of galectin-3 in autoimmune and non-autoimmune nephropathies. [Review]
- ARAutoimmun Rev 2016 Sep 23
- Galectins are evolutionary conserved β-galactoside binding proteins with a carbohydrate-recognition domain (CRD) of approximately 130 amino acids. In mammals, 15 members of the galectin family have b...
Galectins are evolutionary conserved β-galactoside binding proteins with a carbohydrate-recognition domain (CRD) of approximately 130 amino acids. In mammals, 15 members of the galectin family have been identified and classified into three subtypes according to CRD organization: prototype, tandem repeat-type and chimera-type galectins. Galectin-3 (gal-3) is the only chimera type galectin in vertebrates containing one CRD linked to an unusual long N-terminal domain which displays non-lectin dependent activities. Although recent studies revealed unique, pleiotropic and context-dependent functions of gal-3 in both extracellular and intracellular space, gal-3 specific pathways and its ligands have not been clearly defined yet. In the kidney gal-3 is involved in later stages of nephrogenesis as well as in renal cell cancer. However, gal-3 has recently been associated with lupus glomerulonephritis, with Familial Mediterranean Fever-induced proteinuria and renal amyloidosis. Gal-3 has been studied in experimental acute kidney damage and in the subsequent regeneration phase as well as in several models of chronic kidney disease, including nephropathies induced by aging, ischemia, hypertension, diabetes, hyperlipidemia, unilateral ureteral obstruction and chronic allograft injury. Because of the pivotal role of gal-3 in the modulation of immune system, wound repair, fibrosis and tumorigenesis, it is not surprising that gal-3 can be an intriguing prognostic biomarker as well as a promising therapeutic target in a great variety of diseases, including chronic kidney disease, chronic heart failure and cardio-renal syndrome. This review summarizes the functions of gal-3 in kidney pathophysiology focusing on the reported role of gal-3 in autoimmune diseases.
- Secondary bladder amyloidosis with familial Mediterranean fever in a living donor kidney transplant recipient: a case report. [Journal Article]
- BRBMC Res Notes 2016 Oct 19; 9(1):473
- CONCLUSIONS: We presented a case of systemic amyloidosis with FMF, involving the bladder region, myocardium, and renal allograft, diagnosed after renal transplantation. Bladder amyloidosis should be considered in patients with macroscopic hematuria, particularly in the kidney transplant recipients with idiopathic chronic renal disease. Diagnosis of secondary bladder amyloidosis may result in the early detection of underlying diseases, which may contribute to patient prognosis.
- Vasculitis in the autoinflammatory diseases. [Journal Article]
- COCurr Opin Rheumatol 2016 Oct 13
- CONCLUSIONS: In AID, neutrophilic dermatoses are more common and prominent than vasculitis. This may reflect a minor role for interleukin-1 in the pathogenesis of vasculitis. The rarity of vasculitis in AID suggests that in most reported cases its occurrence has been probably coincidental rather than being an integral feature of the disease.
- The role of MEFV mutations in the concurrent disorders observed in patients with familial Mediterranean fever. [Journal Article]
- EJEur J Rheumatol 2016; 3(3):118-121
- CONCLUSIONS: The presence of M694V mutation may predispose patients with FMF to developing other inflammatory disorders.
- Beyond Gout: Colchicine Use in the Cardiovascular Patient. [Editorial]
- HPHosp Pharm 2015; 50(10):859-867
- Colchicine is one of the oldest medications still in use today and is commonly used for the treatment of gout and familial Mediterranean fever. Its anti-inflammatory properties have raised the questi...
Colchicine is one of the oldest medications still in use today and is commonly used for the treatment of gout and familial Mediterranean fever. Its anti-inflammatory properties have raised the question of its utility in managing several cardiovascular diseases, including postoperative atrial fibrillation and pericarditis. This article will review the evidence for colchicine in these conditions and provide recommendations for use.
- Familial Mediterranean Fever with Rheumatoid Arthritis Complicated by Pulmonary Paragonimiasis. [Journal Article]
- IMIntern Med 2016; 55(19):2889-2892
- A 42-year-old woman presented with an intermittent fever and chest and back pain, and an abnormal chest shadow was detected. She was diagnosed with paragonimiasis caused by Paragonimus westermani. Pr...
A 42-year-old woman presented with an intermittent fever and chest and back pain, and an abnormal chest shadow was detected. She was diagnosed with paragonimiasis caused by Paragonimus westermani. Praziquantel therapy improved the abnormal chest shadow, but did not relieve her symptoms. She was also diagnosed with familial Mediterranean fever (FMF), and colchicine therapy resolved her symptoms. She subsequently developed arthralgia and morning stiffness in her hands. We also diagnosed the patient with rheumatoid arthritis (RA), and corticosteroid and salazosulfapyridine therapy improved her symptoms. The existence of paragonimiasis complicated the diagnosis of FMF. The coexistence of FMF and RA is very rare, but does exist.
- Relationship between endothelial dysfunction and microalbuminuria in familial Mediterranean fever. [Journal Article]
- EJEur J Rheumatol 2016; 3(2):61-64
- CONCLUSIONS: Endothelial dysfunction and renal damage occurred as a result of low-grade chronic inflammation. Microalbuminuria, which is the indicator of renal damage and endothelial dysfunction, and FMD show that endothelial functions can be used in the following of early detection of renal damage and endothelial functions in FMF patients.
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- Image analysis of neutrophil nuclear morphology: Learning about phenotypic range and its reliable analysis from patients with Pelger-Huët-Anomaly and treated with Colchicine. [Journal Article]
- CBCytometry B Clin Cytom 2016 Sep 29
- The nuclear morphology of neutrophils depends on different endogenous and exogenous factors, which can lead to hypo- or hypersegmentation of the normally 2-4 segmented nucleus. Hyposegmentation can b...
The nuclear morphology of neutrophils depends on different endogenous and exogenous factors, which can lead to hypo- or hypersegmentation of the normally 2-4 segmented nucleus. Hyposegmentation can be due to mutations in the LBR-gene (Pelger-Huët-Anomaly) or can be induced, for example, by colchicine treatment. The range of this phenotypic variation is known as "norm of reaction", which can be of major relevance for clinical diagnosis and therapeutic intervention. In this project, we studied the "norm of reaction" in 26 subjects with 0-3 wild type LBR alleles. In addition, the phenotypic variation was analyzed in 3 patients with Familial Mediterranean Fever (FMF), before and after colchicine treatment. We measured the phenotype nuclear segmentation of neutrophils based on two conventional qualitative methods, the "rule of threads" and the "rule of thirds". In addition, we tested a morphometric quantitative approach, the "circularity index". The circularity index" was superior in cases with hyposegmentation; the "rule of thirds" with respect to hypersegmentation. Approximately 65% of the observed phenotypic variance was explainable by the number of LBR wild type alleles. The gene-dosage effect followed a non-additive, hysteresis-like characteristic with lower and upper plateaus. Colchicine treatment had a clear, although minor phenotypic effect compared to the number of LBR wild type genes or the mutation type. Thus, the nuclear morphology of granulocytes and its "norm of reaction" can be regarded as an excellent model both for detailing the interplay between endogenous and exogenous factors and for clinical diagnostic purposes. This article is protected by copyright. All rights reserved.