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%.

[5 years of "concerted action dose reduction in CT" -- what has been achieved and what remains to be done?]. / 5 Jahre Konzertierte Aktion Dosisreduktion CT--was wurde erreicht, was ist noch zu tun?

In May 1998, the German "Concerted Action Dose Reduction in CT" was founded by all parties involved in CT. Its intention was to achieve a significant reduction of the radiation exposure caused by CT, a matter that has increasingly been considered a major challenge since the early nineties. As a result of a number of joint efforts, the essential preconditions have been established by now. The fifth anniversary of the Concerted Action gave rise for both retrospection and outlook on the tasks that have already been accomplished and those that still need to be done. For this purpose, a one-day symposium took place in Berlin on November 4, 2003. The contents of a total of 18 contributions will be outlined here in brief.

QRS axis in isolated perimembranous ventricular septal defect and influences of morphological factors on QRS axis.

To detect the frequency of left axis deviation (LAD) in isolated perimembranous ventricular septal defects (VSD) we retrospectively analyzed electrocardiograms of 59 patients, aged 8 months to 15 years. Patients were grouped into those having ventricular septal aneurysm (VSA) formation (n:20) and those who did not have VSA (n:39). Patients with VSA were then stratified into 2 groups according to the presence of left ventricular-to-right atrial (LV-RA) shunt. Four hundred healthy children served as the control group. We found that 12 (20.3%) of 59 patients with isolated perimembranous VSD had a LAD. Five of 6 patients with perimembranous outlet VSD and 6 with perimembranous inlet VSD had abnormal LAD with a qR pattern in I and aVL and rS in aVF. Abnormal LAD was more prevalent in patients with VSA (40%) than without VSA (7.7%) (P <.01). We also found that mild right ventricular hypertrophy (RVH) with the rsR' or rSR' pattern in V1 was more frequent in patients with VSA, especially those who had LV-RA shunt. However, we could not find significant difference between patients with or without LV-RA shunt for the incidence of abnormal LAD and mild RVH. Localization of perimembranous VSD was not found to have an effect on frequency of abnormal LAD and mild RVH in this patient group. In patients with clinical findings of VSD, the existence of abnormal LAD especially if it is associated with mild RVH, should raise the possibility of perimembranous VSD with VSA formation.

Poststreptococcal reactive arthritis: clinical course and outcome in 15 patients.

Patients with Group A beta-hemolytic streptococcal infection and articular disease, who do not fulfill the modified Jones criteria for diagnosis of acute rheumatic fever (ARF), have been classified as having poststreptococcal reactive arthritis (PSRA). We reviewed the clinical characteristics, laboratory findings and outcome of 15 patients with PSRA. None of these patients had clinical evidence of carditis. The pattern of joint involvement was variable and included arthritis in five patients and arthralgia in the remaining ten patients. Nine patients were treated with salicylates for one to 16 weeks; the others recovered spontaneously. Usually, the patients with arthralgia responded promptly to salicylates, while the response was poor in patients with arthritis. One patient with monoarthritis developed carditis nine months after his first arthritis attack. Another patient presenting with monoarthritis later had two additional episodes of poststreptococcal reactive arthralgia. It seems there is a wide spectrum of poststreptococcal rheumatic diseases, and patients with PSRA are also at risk for cardiac disease; therefore, prophylactic antibiotic therapy should be considered in these patients.

Echocardiographic, morphologic, and geometric variations of the left ventricular outflow tract: possible role in the pathogenesis of discrete subaortic stenosis.

Although the clinical features and natural course of discrete subaortic stenosis (DSS) are well defined, the etiology remains speculative. The purpose of this study was to identify the echocardiographic, morphologic, and geometric variations of the left ventricular outflow tract associated with DSS in children and to determine whether these variations have a role in the pathogenesis of DSS. The aortoseptal angle (ASA), mitral-aortic valve separation (MAS), and the size of the aortic annulus were determined in two groups of children. Group 1 comprised 11 patients with isolated DSS, who were compared with an age- and body surface area- (BSA) matched healthy children (Group 1A, n: 20). Group 2 comprised 10 patients with DSS and ventricular septal defect (VSD). Group 2 was compared with an age- and BSA-matched patients with isolated perimembranous VSD (Group 2A, n: 22). Measurements were carried out from previously recorded echocardiographic studies. The ASA was steeper (119.3 +/- 6.1 degrees vs 137.5 +/- 5.6 degrees , p < 0.001), and the MAS was wider (6.1 +/- 1.6 vs 3.2 +/- 0.7 mm, p < 0.001) in patients with isolated DSS than in healthy control subjects. Similar differences were found between patients in Group 2 and Group 2A; the ASA was steeper (122.2 +/- 6.5 degrees vs 141.3 +/- 5.0 degrees, p < 0.001), and the MAS was wider (5.8 +/- 1.5 vs 3.8 +/- 1.1 mm, p < 0.001). The size of the aortic annulus was not different among the four study groups. Although the MAS was significantly wider in patients with DSS, there was significant overlap in MAS between patients and controls. However, if an ASA < or = 130 degrees was chosen as a predictive variable, it was found to be a highly sensitive, specific, and positive predictive marker for the development of DSS. This study demonstrates that DSS is associated with a steeper ASA, and a wider MAS, in patients with or without associated VSD. These morphologic abnormalities, especially a steeper ASA, may be risk factors for the development of DSS.

Enzymatic hydrolysis of anchovy oil: production of glycerides enriched in polyunsaturated fatty acids.

In an attempt to produce the polyunsaturated fatty acid (PUFA)-enriched glycerides, commercially available Turkish anchovy oil (PUFA content of 27%), was hydrolyzed with 1,3-specific Rhizomucor miehei lipase. After the hydrolysis, the triglyceride (TG), diglyceride (DG), monoglyceride (MG), and free fatty acid (FFA) composition of the reaction mixture was determined, and fatty acid components of these fractions were analyzed. R. miehei lipase released PUFA extremely slowly, resulting in their accumulation in the TG and DG fractions, especially in TG. The PUFA content in the glyceride mixture (including TG, DG, and MG) increased as hydrolysis progressed. The effects of operational parameters (pH, temperature, time, and enzyme concentration) on the extent of hydrolysis were investigated. Based on these results, optimal reaction conditions were established. At optimal conditions (pH 4.0, 35 degrees C, 3 h, and enzyme concentration of 500 U/g oil), the level of PUFA in the glyceride mixture was raised to 40%. The individual TG and DG fractions contained 45 and 30% PUFA, respectively. Less than 2% of the total PUFA was lost in the FFA fraction.

Immune globulin treatment in intractable epilepsy of childhood.

Six children suffering from epilepsy refractory to conventional anti-convulsive therapy were treated with high-dose intravenous immune globulin (IVIG) (200 mg/kg three times per week, repeated after three weeks). In four children clinical and EEG findings markedly improved, while a partial response was noted in the other cases. These results suggest that high-dose intravenous immune globulin may have a beneficial effect in the treatment of intractable epilepsy.

[In vitro evaluation of antibiotic diffusion from antibiotic impregnated xenografts]. / Evaluation in vitro de la diffusion des antibiotiques à partir d'une xénogreffe imprégnée d'antibiotiques.

PURPOSE OF THE STUDY: The purpose of the study was to evaluate xenografts as a slow release antibiotic delivery system, as xenografts also have excellent biocompatibility and osteogenesis-stimulating effect. MATERIAL: Xenograft (Unilab Surgibone) blocks of 5 x 5 x 5 mm were used in the study. Antibiotics used for impregnation were gentamicin sulphate, ciprofloxacin and penicillin G. METHODS: In the pilot study, xenograft blocks impregnated with gentamicin sulphate solution and the contact time was between 1/2 to 10 hours. Then the grafts were incubated and the antibiotic concentration was measured in the elution fluid changed every day. The minimum contact time to release the antibiotic 10 days over 0.5 microgram/ml was 8 hours. The 8 hours contact time was used in the main study to evaluate gentamicin sulphate, ciprofloxacin and penicillin G. The method was the same as in the pilot study. RESULTS: All antibiotics showed similar release patterns reaching their peaks on the 5th day and then gradually decreasing below the effective level (0.5 microgram/ml) approximately on day 10. DISCUSSION: Although various materials have been used to deliver antibiotics, they have some certain disadvantages especially reduced biocompatibility. The superiority of xenografts over other materials, is not only biocompatibility but also to facilitate osteogenesis. The only objection may be the relatively short duration of release (10 days) but the length of antibiotherapy is controversial and standard 6 weeks of administration has no documented superiority over other time intervals. CONCLUSION: Our delivery system is superior to others as its function is not only to deliver antibiotics but has its place in orthopedic practice to fill defects and stimulate osteogenesis. Also the preparation of this system is not time and skill consuming.

High-dose intravenous immune globulin in the management of severe Guillain-Barre syndrome.

Guillain-Barre Syndrome (GBS) is the most common cause of acute generalized paralysis. Although the cause and pathogenesis of GBS remain unknown, there is increasing evidence to suggest that this syndrome has an immunological basis. Two children suffering from GBS were treated with high-dose intravenous immune globulin (IVIG) (1 g/kg/day over two consecutive days). Both children showed marked clinical improvement within 48 hours of the onset of treatment. It is suggested, on the basis of recent case reports, that immunoglobulins may have an important role in the treatment of Guillain-Barre Syndrome.

The influence of maternal iron deficiency anaemia on the haemoglobin concentration of the infant.

Serum ferritin, iron, and haemoglobin (Hb) values of 27 pregnant women who did not receive oral iron therapy during pregnancy, and Hb of their normal full-term babies were determined. Maternal blood samples were obtained at 16 and 24 weeks of pregnancy and infants' blood samples were obtained at the first day and 3 months of life. Mothers were divided into two groups according to their serum ferritin values. By analysing the results we were not able to detect any correlation between maternal ferritin, Hb, and newborn gestational age, and Hb and birth weight. The same was found when the groups were compared by a maternal serum ferritin above and below 12 ng/ml.

Effects of protein energy malnutrition on circulating thyroid hormones.

The effect of protein energy malnutrition (PEM) in the children on serum levels of total thyroxine (TT4), total triodothyronine (TT3) and thyrotropin (TSH) were evaluated. There were 107 children aged 2 to 60 months in the malnutrition group and 54 healthy age and sex matched controls. Serum TT4 and TT3 were all reduced in the malnutrition group. This decrease in TT3 was more significant (p < 0.01) in severe malnutrition than in mild PEM. Serum TSH levels in the malnutrition and control groups were similar. These results suggest that the children remained euthyroid and represent an adaptive response to protein energy malnutrition.

Intravenous immunoglobulin in the treatment of Salmonella typhimurium infections in preterm neonates.

The purpose of this study was to determine the role of intravenous immunoglobulin (IVIG) administration in preterm neonates with S. typhimurium infection. A randomized trial of 47 preterm neonates with intestinal or extraintestinal S. typhimurium infection was performed. Neonates were randomly divided into two groups: 22 neonates were only given cefoperazone (group 1); 25 neonates were given cefoperazone plus IVIG (group 2). IVIG was given at a dose of 500 mg/kg on days 1, 2, 3, and 8 after entry into the study. Following treatment, bacteremia, complications, mortality rate, recovery time, and duration of antimicrobial therapy were evaluated in two groups. Bacteremia was found in 31.4% in group 1 and 8% in group 2 (P < .05); complications developed in 81.8% in group 1 and 16% in group 2 (P < 0.01); mortality was 40.9% in group 1 and 12% in group 2 (P < .05). Recovery took 15 days in group 1 and 8 days in group 2 (P < .01). The duration of antimicrobial therapy was 20 days in group 1 and 14 days in group 2 (P < .01). We conclude that IVIG treatment in combination with antibiotics in preterm neonates with S. typhimurium infection reduces the complications, mortality rate, and duration of therapy.

May-Hegglin anomaly (a presentation of a family)

The May-Hegglin anomaly is a rare autosomal dominant disorder characterized by the presence of large, bizarre, cigar-shaped platelets and Döhle bodies in the leukocytes. This rare anomaly was detected in seven members of a family. It is emphasized that in making a differential diagnosis this disorder must be distinguished from the other mild bleeding disorders.