1/216. familial mediterranean fever. No role of mycobacterium tuberculosis in ten patients.BACKGROUND: Tuberculosis (TB) and familial mediterranean fever (FMF) are two common diseases in our region, turkey. Both share some properties in common: Both cause AA type amyloidosis and have association with some immunological abnormalities. Upon incidentally observing mycobacterium tuberculosis in bone marrow biopsies of three patients with FMF in a previous study, we intended to elucidate this association prospectively. MATERIAL AND methods: In this study, we examined prospectively 10 FMF patients, 5 male and 5 female, with a median duration of 31 years disease activity. All were under colchicine therapy. They had no sign of renal involvement. The bone marrow biopsies of these patients were examined for the presence of M. tuberculosis by polymerase chain reaction (PCR), BACTEC culture and pathological stains. Pathological examination was performed for the existence of granuloma and amyloid deposition by hematoxylin-eosin, Crystal Violet and congo red stains. RESULTS: The examination of all bone marrow specimens by the mentioned methods suggest that mycobacterium tuberculosis has no role in the ethiopathogenesis of FMF. Although the patients had a positive family history of 60% for tuberculosis and in 80% of them with positive tuberculin skin test. CONCLUSIONS: We concluded that although there seemed to be a kind of association between both diseases, this relationship is not via the direct existence of bacteria itself. Considering high family history and skin test positivity, one should look for the presence of autoimmune mechanisms under this suspicious relationship between tuberculosis and FMF. Also, this is the first study examined the state of amyloidosis in the bone marrow at an earlier stage of FMF without overt renal findings.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
2/216. reflex sympathetic dystrophy arising in a patient with familial mediterranean fever.A 14-year-old girl with familial mediterranean fever (FMF) had had acute attacks of fever, abdominal pain, and arthritis for 4 years. Her last arthritis attack was protracted, leading to reflex sympathetic dystrophy (RSD) in her right lower extremity. Physical therapy along with sympathetic ganglion block and corticosteroid therapy was used for the treatment. To our knowledge, this is the first reported case of RSD arising in a patient with FMF. Early recognition of RSD in FMF patients is important, and physical therapy should be applied along with medical treatment.- - - - - - - - - - ranking = 1.2keywords = fever (Clic here for more details about this article) |
3/216. Protracted familial mediterranean fever arthritis.familial mediterranean fever (FMF) is an inherited disorder characterized by recurrent attacks of fever and abdominal, chest, and articular pain. The articular attack of FMF is typically an acute, self-limited, large joint monoarthritis most often affecting the knee or hip. Rarely, a more protracted arthritis may occur. We describe two unusual cases of long-standing FMF arthritis with excellent response to synovectomy.- - - - - - - - - - ranking = 1.2keywords = fever (Clic here for more details about this article) |
4/216. A homozygous M694V mutation of the MEFV gene in a patient with periodic fever and thoracic pain.A Turkish patient with episodic fever and thoracic pain is described in whom a homozygous M694V mutation of the MEFV gene confirmed the clinical diagnosis of familial mediterranean fever. The role of dna analysis is discussed with respect to understanding the pathogenesis of the fever and assessing the risk of amyloidosis in specific mutations of the MEFV gene.- - - - - - - - - - ranking = 1.4keywords = fever (Clic here for more details about this article) |
5/216. Cyclic neutropenia complicated by renal AA amyloidosis.Cyclic neutropenia is a rare disease characterized by regular cyclic fluctuations in the numbers of neutrophils. patients with the disease suffer from recurrent infections at regular intervals of nearly three weeks. Recently, recombinant human granulocyte colony-stimulating factor (rhG-CSF) was reported to be an effective treatment for this disease. here we describe 17-year-old cyclic neutropenic female patient with a very rare association of renal amyloidosis of AA type who was under intermittent rhG-CSF treatment for the previous one and a half years. We conclude that although the disorder is usually benign, reactive amyloidosis may rarely develop in cases who remain untreated for a long period of time. However familial mediterranean fever (FMF) type II should also be born in mind, particularly in predisposed populations.- - - - - - - - - - ranking = 0.2keywords = fever (Clic here for more details about this article) |
6/216. sacroiliitis in familial mediterranean fever: an unusual presentation in childhood.familial mediterranean fever (FMF) is an autosomal recessively transmitted disease characterized by attacks of fever and serositis. The course of arthritis, which is a common manifestation of FMF, is generally benign. sacroiliitis due to FMF has been reported by several authors, but all the patients described so far had roentgenographic abnormalities, and most of them were adult cases. Here we report the youngest FMF patient with sacroiliitis without any abnormality on sacroiliac x-ray. She is also the first FMF patient in whom sacroiliac involvement was diagnosed by computed tomography (CT) in childhood. It is concluded that CT is a useful technique for the early diagnosis of destructive arthritis in FMF patients even in early childhood.- - - - - - - - - - ranking = 1.2keywords = fever (Clic here for more details about this article) |
7/216. polyarteritis nodosa involving the hepatobiliary system in an eight-year-old girl with a previous diagnosis of familial mediterranean fever.polyarteritis nodosa (PAN) is a vasculitis of small- and medium-sized muscular arteries with deposition of immune complex in the vessel wall. Although gastrointestinal involvement is common, the symptomatic involvement of the hepatobiliary system is rare. An eight-year old female patient with a previous diagnosis of familial mediterranean fever (FMF) was hospitalized for right upper quadrant pain and fever. The thickened gall bladder wall by ultrasonography, called for exploration. Histopathological evaluations of the liver biopsy and gall bladder revealed PAN. Corticosteroid therapy was initiated and the patient recovered fully. This case represents one of the rarest forms of PAN in childhood.- - - - - - - - - - ranking = 1.2keywords = fever (Clic here for more details about this article) |
8/216. familial mediterranean fever diagnosed by PCR.The diagnosis of familial mediterranean fever (FMF) was, until recently, based on exclusion of diseases with related clinical signs. Now an exact diagnosis of FMF is possible by polymerase chain reaction (PCR). We report here a case with 2 different mutations in the gene responsible for FMF, thereby being a compound heterozygote (M694V/V726A).- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
9/216. familial mediterranean fever.familial mediterranean fever (FMF) is an inherited multisystem disease manifested by painful, febrile attacks affecting the chest, abdomen, joints, and skin. No simple studies confirm the presence of FMF, contributing to the difficulty in diagnosis. A 10-year-old boy initially presented with a diffuse rash and complaints of bilateral joint pain of the hips, knees, and ankles and pain of the right shoulder. The child responded to daily naproxen. One year later, he continued to complain of hip, knee, ankle, and bilateral wrist pain. He also reported mild to moderate recurrent abdominal discomfort. omeprazole provided intermittent relief. The patient continued to experience episodes of joint and abdominal pain. Two and a half years after he first presented, FMF was considered. In the second case, a 51-year-old man presented to the emergency department with complaints of fever, cough, and abdominal and joint pain. Fever, joint pain, and swelling decreased during the next few days. The patient was maintained on colchicine, with complete resolution of joint pain complaints during the next few days. colchicine, 1 to 2 mg per day taken continuously during flare and quiescent periods, is the treatment of choice for FMF. colchicine reduced the severity and frequency of attacks and may also delay or prevent secondary amyloidosis.- - - - - - - - - - ranking = 1.2keywords = fever (Clic here for more details about this article) |
10/216. Severe hyperkalemia in two renal transplant recipients treated with standard dose of trimethoprim-sulfamethoxazole.hyperkalemia is a serious electrolyte disorder and is a frequent finding in renal transplant recipients. trimethoprim-induced hyperkalemia has been increasingly reported in recent years. We describe two renal transplant recipients who developed end-stage renal disease secondary to familial mediterranean fever and presented with severe hyperkalemia secondary to the use of standard dose of trimethoprim. One of the patients had potential underlying adrenal insufficiency, which might be a contributing factor for the development of hyperkalemia. We concluded that renal transplant patients receiving even the standard dose of trimethoprim should be monitored closely for the development of hyperkalemia. They should be recognized as a group with increased risk in regard to their concurrent renal insufficiency, concomitant use of cyclosporine, and associated tubulointerstitial disease. patients with secondary amyloidosis are at even greater risk, and subclinical adrenal insufficiency may be an underlying risk factor for the development of severe, life-threatening hyperkalemia among this group of patients.- - - - - - - - - - ranking = 0.2keywords = fever (Clic here for more details about this article) |
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