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M Tunca

Publications and source records attributed to M Tunca.

At least 19 recordsLinked to original sources
PubMed2006

Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations.

OBJECTIVE: To prospectively monitor inflammatory activity over a prolonged period in a cohort of Turkish patients with FMF, their healthy relatives and healthy controls and to relate this to their MEFV genotypes. METHODS: 43 patients with FMF and 75 of their asymptomatic relatives underwent fortnightly assessments and venesection for measurement of CRP and SAA over 5 months. 50 unrelated healthy population matched controls were also studied. MEFV genotyping was performed on all participants and comparisons were made between the different groups. RESULTS: Paired MEFV mutations were detected in 84% of FMF patients and single mutations in 12%. Substantial acute phase reactivity was seen among the patients with FMF during attacks (median SAA 693 mg/l, CRP 115 mg/l). Between attacks there was also some inflammatory activity (median SAA 6 mg/l, CRP 4 mg/l). Among healthy controls 16% were heterozygotes for MEFV mutations and 4% had two mutations. As expected there was a substantial carrier rate among healthy relatives with mutations detected in almost 92%. Asymptomatic MEFV heterozygotes had elevated acute phase proteins compared to wild type subjects. CONCLUSION: Substantial sub-clinical inflammation occurs widely and over prolonged periods in patients with FMF, indicating that the relatively infrequent clinically overt attacks represent the 'tip of the iceberg' in this disorder. Both basal and peak acute phase protein concentrations were greater in MEFV heterozygotes than in wild-type controls, regardless of mutation demonstrating a 'pro-inflammatory' phenotype among FMF carriers. Upregulation of the acute phase response among carriers of FMF may augment their innate host response and contribute to better resistance to infection.

Acute-Phase Reaction↗
PubMed2004

A case of recurrent pancreatitis due to hyperlipidemia misdiagnosed as familial Mediterranean fever.

Familial Mediterranean fever (FMF) is prevalent among Arabic, Turkish, Armenian, and Jewish people and it must always be considered in the differential diagnosis of patients from these ethnic groups presenting with recurrent abdominal pain with fever. In cases of fever and recurrent abdominal pain, acute pancreatitis is an important clinical condition, which should be considered in the differential diagnosis. Serum amylase concentration in acute pancreatitis is usually more than three times the upper limit of normal. However, in recurrent pancreatitis secondary to hypertriglyceridemia, serum amylase levels, for reasons that are not well understood, may be normal or mildly elevated. Recurrent pancreatitis secondary to hypertriglyceridemia may thus pose a problem in the differential diagnosis and may lead to an erroneous diagnosis of FMF. Measurement of serum triglyceride along with amylase levels should be required for a suspected diagnosis. Computerized examination of the abdomen may need to be undertaken to exclude acute pancreatitis in the presence of hypertriglyceridemia since serum amylase levels may be normal or slightly elevated.

Adult↗
PubMed2000

The genetic basis of autosomal dominant familial Mediterranean fever.

Familial Mediterranean fever (FMF) is classically an autosomal recessive periodic inflammatory disease occurring in Mediterranean and Middle Eastern populations. It is caused by mutations affecting both alleles of MEFV, a gene that encodes pyrin (marenostrin), an uncharacterized neutrophil protein. Occasional reports of autosomal dominant FMF have often been discounted, on the basis that asymptomatic FMF carriers are common in certain populations, and give rise to pseudo-dominant inheritance. We performed comprehensive MEFV genotyping in five families in whom FMF appeared to be inherited dominantly. Transmission proved to be pseudo-dominant in two cases, but true dominant inheritance of FMF with variable penetrance was supported by the genotyping results in the other three families. The disease in these cases was associated with heterozygosity for either pyrin DeltaM694 alone or the compound pyrin variant E148Q/M694I, the latter occurring in two unrelated families. Complete MEFV sequencing failed to identify any coding region abnormality in the other allele in any of these cases, and, in the largest kindred, single-allele disease transmission was further supported by analysis of silent single nucleotide polymorphisms, which proved that affected individuals had at least three different complementary alleles. Studies of two further unrelated British patients with FMF associated with simple heterozygosity for pyrin DeltaM694 were also consistent with autosomal dominant inheritance. The clinical features of dominantly inherited FMF were absolutely typical, including AA amyloidosis in a patient with pyrin DeltaM694. These findings extend the spectrum of FMF, and suggest that the methionine residue at position 694 makes a crucial contribution to pyrin's function, and that a 50% complement of normal pyrin activity does not prevent susceptibility to FMF.

Amyloidosis↗
PubMed1998

Coexistence of familial Mediterranean fever with sacroiliitis and Behçet's disease: a rare occurrence.

Familial Mediterranean fever (FMF) and Behçet's disease are relatively rare but may still coexist in the same patient. Sacroiliitis is another feature whose significance is controversial in either of the diseases. We report a case of longstanding FMF with sacroiliitis who later developed typical characteristics of Behçet's disease. Although occurrence by chance cannot be ruled out, this unusual patient may enhance the claims that FMF and Behçet's disease have common aetiopathogenetic mechanisms. It would be appropriate to include this coexistence in the list of differential diagnoses of the two diseases.

Adult↗
PubMed1997

The efficacy of interferon alpha on colchicine-resistant familial Mediterranean fever attacks: a pilot study.

About a quarter of familial Mediterranean fever (FMF) patients have recurrent painful attacks of polyserositis despite regular colchicine treatment. There is no known alternative drug for colchicine-resistant cases. We had previously observed a patient with FMF whose painful attacks disappeared during the 6 month period of interferon alpha (IFN) treatment for his chronic hepatitis B. The objective of the present study was to investigate the possible beneficial effect of IFN on these episodes. Twenty-one consecutive attacks in seven adult patients with FMF were treated at early onset with IFN, the dosage being 3-10 million I U s.c. Eighteen of the 21 attacks could be halted in a mean time of 3.05 h, while the intensity of abdominal pain remained very low. Observed side-effects were generally mild and acceptable. IFN may be a useful adjunct for the treatment of colchicine-resistant attacks in FMF patients.

Adult↗
PubMed1997

Phase I study with the DNA sequence-specific agent adozelesin.

Adozelesin, a synthetic analog of the antitumor antibiotic CC-1065, is a novel cytotoxic agent which inhibits DNA synthesis by binding to the minor groove of the DNA helix. Preclinical studies have shown a broad spectrum of activity against a variety of murine and human tumor xenograft models. We conducted a phase I study of adozelesin to (i) determine a recommended dose for phase II testing using a 10 min i.v. infusion, (ii) characterize the toxic effects of the drug using this schedule and (iii) document any antitumor activity observed. Adozelesin was administered as an i.v. infusion every 6 weeks. CBC and biological parameters were performed weekly. The starting dose of 10 microg/m2, corresponding to 1/30 the mouse equivalent lethal dose, was escalated, according to a modified Fibonacci scheme, until dose-limiting toxicity was encountered. Forty-seven adult patients with solid malignancies were entered in the study. Successive dose levels used were 10, 20, 33, 50, 70, 105, 120, 150 and 180 microg/m2. The main toxic effect was myelosuppression, which was dose limiting. The maximally tolerated dose was defined as 180 microg/m2. A minor response with a 4 month duration was reported in one previously treated patient with melanoma. We conclude that the recommended phase II dose of adozelesin given as a 10 min infusion is 150 microg/m2, repeated every 4 weeks.

Adult↗
PubMed1993

Elderly hypertensive patients: are they adequately treated?

To better understand the scope and importance of hypertension in elderly persons, we analysed 551 outpatients, > or = 65 years of age. Arterial BP values > 160 mmHg systolic or 90 mmHg diastolic were documented in 233 cases (42.29% of total). Among this hypertensive group of patients, 174 of them (74.68%) were chronic cases and 59 patients (25.32%) were newly diagnosed. Only 39 of the 174 chronic patients were receiving adequate treatment (22.41%), 87 patients were not receiving any treatment (50%) and 40 others lacked information about previous treatments (23%). Apart from the 35 patients (15.02%) who were treated with salt-restricted diet only, we prescribed antihypertensive drugs to all the remaining 198 patients (84.98%): diuretics (41.92%), calcium antagonists (33.33%), beta-blockers (17.86%), ACE inhibitors (15.66%) and various other drugs (8.1%), with good patient compliance and minimal side-effects. Of the 233 hypertensive patients, 64.81%, had accompanying health problems such as cerebrovascular disease (17.95%), diabetes (16.67%), coronary artery disease (16.24%) and cancer (7.27%). It is concluded that hypertension among the elderly is an important and underestimated part of an already complicated health picture of this expanding population of modern society.

Aged↗
PubMedDate not supplied

Increased frequency of familial Mediterranean fever in Central Anatolia, Turkey.

OBJECTIVE: It has been observed that familial Mediterranean fever is more prevalent among people coming from central Anatolia in Turkey. To test this observation the frequency of FMF was investigated by afield survey in Sivas, a city located in central of Turkey. METHODS: The survey was conducted in a cohort of 4809 persons selected by systematic sampling from 2 districts of Sivas, with a total population of 83,274. Face to face interviewing was done with registered households using a standard questionnaire developed to screen FMF A second interview was conducted by a rheumatologist and an internist of those individuals who were regarded to have possible FMF. RESULTS: The suspicion of FMF emerged in the cases of 46 individuals during the survey and 36 were interviewed for a second time. FMF was diagnosed in 10 cases. Only one had a previous diagnosis of FMF. The overall frequency of FMF among a cohort of 3,948 inhabitants of Sivas was 1/395 (0.25%). CONCLUSION: This study indicates that the prevalence of FMF in Sivas may be higher than that in general Turkish population, which has been reported to be 0.1%.

Adolescent↗