Valvular surgery in Behcet's disease

BACKGROUND: Behcet's disease is a chronic inflammatory disease with a relapsing course. Behcet's disease affects many systems and causes hypercoagulability, and detection of an intracardiac mass in a Behcet patient should raise the question of an intracardiac thrombus. We analyzed our patients with Behcet's disease operated for valvular disease. METHODS: We operated three patients (one male and two females) who had been diagnosed as having Behcet's disease previously. Using mechanical bileaflet valves, aortic valve replacement in two and mitral valve replacement in the other patient were performed. Mechanical valve replacement was performed using pledgetted-interrupted sutures in the mitral procedure. Patients' steroid therapies were not interrupted and in the postoperative course, steroid was continued. No reoperations were needed. Anticoagulation with warfarin was instituted after the operation with the target of an international normalized ratio (INR) between 3 and 3.5. RESULTS: There was no mortality either early or late follow-up. Intraoperative and postoperative courses were uneventful. Two had ventricular arrhythmias. Total follow-up was 23.3 patient/years with a mean of 93.3 +/- 64.7 months. In the late follow-up, patient with the mechanical mitral valve experienced a cerebrovascular accident 40 months after the operation. Her echocardiographic examination gave a functional valve without any pathology. CONCLUSIONS: Surgeons should remember the hypercoagulable state in Behcet patients and strict anticoagulation protocols should be utilized. In the operations, bileaflet prostheses should be used.     

Assessment of time management attitudes among health managers

These days, working people are finding it difficult to manage their time, get more done at work, and find some balance in their work and personal lives. Successful time management is often suggested to be a product of organizing skills, however, what works for one person may not work for others. Context current competence assessment formats for physicians, health professionals, and managers during their training years reliably test core knowledge and basic skills. However, they may underemphasize some important domains of professional medical practice. Thus, in addition to assessments of basic skills, new formats that assess clinical reasoning, expert judgment, management of ambiguity, professionalism, time management, learning strategies, and teamwork to promise a multidimensional assessment while maintaining adequate reliability and validity in classic health education and health care institutional settings are needed to be worked on. It should be kept in mind that institutional support, reflection, and mentoring must accompany the development of assessment programs. This study was designed to describe the main factors that consume time, effective hours of work, time management opportunities, and attitudes and behaviors of health professionals and managers on time management concept through assessment by the assessment tool Time Management Inquiry Form (TMIQ-F). The study was conducted at the State Hospital, Social Security Hospital, and University Hospital at Kirikkale, Turkey between October 1999 and January 2000, including 143 subjects defined as medical managers and medical specialists. According to the results, a manager should give priority to the concept of planning, which may be counted among the efficient time management techniques, and educate him/herself on time management.     

Mannose Binding Lectin and Macrophage Migration Inhibitory Factor Gene Polymorphisms in Turkish Children with Cardiomyopathy: No Association with MBL2 Codon 54 A/B Genotype, but an Association between MIF -173 CC Genotype

Myocardial inflammation is one of the commonest mechanisms in cardiomyopathy (CMP). Mannose binding lectin (MBL) is a key molecule in innate immunity, while macrophage migration inhibitory factor (MIF) is a constitutive element of the host defenses. We investigated the possible association between polymorphisms of MBL2 and MIF genes and CMP in Turkish children. Twenty-children with CMP and 30 healthy controls were analyzed for codon 54 A/B polymorphism in MBL, and -173 G/C polymorphism in MIF genes by using PCR-RFLP methods. No significant difference was found between genotypes and alleles of MBL2 gene codon 54 A/B polymorphism in patients and controls (p>0.05). However, serum uric acid levels was found higher in dilated CMP patients with AA genotype. Frequency of MIF -173 CC genotype was significantly higher in patients (p<0.05), and sodium levels were higher in patients with MIF -173 CC genotype. This study is the first to investigate the MBL and MIF gene polymorphisms in Turkish children with CMP. We conclude that CC genotype of MIF (-173) polymorphism may be a risk factor for CMP patients. However, further studies with larger samples are needed to address the exact role of this polymorphism in CMP.     

APOL1 variants increase risk for FSGS and HIVAN but not IgA nephropathy

A chromosome 22q13 locus strongly associates with increased risk for idiopathic focal segmental glomerulosclerosis (FSGS), HIV-1-associated nephropathy (HIVAN), and hypertensive ESRD among individuals of African descent. Although initial studies implicated MYH9, more recent analyses localized the strongest association within the neighboring APOL1 gene. In this replication study, we examined the six top-most associated variants in APOL1 and MYH9 in an independent cohort of African Americans with various nephropathies (44 with FSGS, 21 with HIVAN, 32 with IgA nephropathy, and 74 healthy controls). All six variants associated with FSGS and HIVAN (additive ORs, 1.8 to 3.0; P values 3 × 10(-2) to 5 × 10(-5)) but not with IgA nephropathy. In conditional and haplotype analyses, two APOL1 haplotypes accounted for virtually all of the association with FSGS and HIVAN on chromosome 22q13 (haplotype P value = 5.6 × 10(-8)). To assess the role of MYH9 deficiency in nephropathy, we crossbred Myh9-haploinsufficient mice (Myh9(+/-)) with HIV-1 transgenic mice. Myh9(+/-) mice were healthy and did not demonstrate overt proteinuria or nephropathy, irrespective of the presence of the HIV-1 transgene. These data further support the strong association of genetic variants in APOL1 with susceptibility to FSGS and HIVAN among African Americans.     

Elevated plasma advanced oxidation protein products in children with Henoch�CSchonlein purpura

Henoch–Schonlein purpura (HSP) is a systemic vasculitis characterized by involvement of skin, joints, gastrointestinal tract (GIT), and kidney; its pathogenesis is still controversial. The aim of our study was to investigate the role of oxidative stress in the pathogenesis of HSP. Plasma advanced oxidation protein products (AOPP) level was measured in 29 children with HSP at the onset of the disease and during remission in comparison with 30 healthy subjects. Patients at the active stage had significantly higher AOPP levels than those at the remission stage of HSP and the controls (42.9 ± 25.7, 30.6 ± 11.8, 27.9 ± 11.2 mmol/l; P = 0.027 and P = 0.023 respectively). The mean AOPP levels of the patients with arthritis and/or arthralgia were significantly higher those than without joint involvement (48.3 ± 26.0 and 22.3 ± 9.3, P = 0.036 respectively). However, AOPP levels were similar in patients with and without gastrointestinal involvement. Plasma AOPP levels were positively correlated with leukocyte and thrombocyte count at disease onset, whereas they were found to be negatively correlated with serum glucose and sodium levels. The mean thrombocyte count was the only independent predictor of increased level of AOPP in regression analysis (β = 0.407; P = 0.029). In conclusion, this study showed that increased oxidative stress may play an important role in the pathogenesis of HSP. Also, we suggest that higher platelet count might be an indirect indicator of oxidative stress in these patients. Further research is required to identify the potential association between oxidative stress and increased thrombocyte count in children with HSP.     

Familial primary carpal tunnel syndrome with possible skipped generation

Carpal tunnel syndrome, an entrapment neuropathy of the median nerve, is rarely seen in childhood. Familial carpal tunnel syndrome, an even more exceptional entity, is frequently associated with inherited systemic disorders. Rarely it can be presented as a primary familial form with Mendelian autosomal dominant inheritance. We report the occurrence of carpal tunnel syndrome in two generations of a family in which the index case was a 6-year-old boy with bilateral hand pain and paresthesias. Our report demonstrates an interesting inheritance pattern of carpal tunnel syndrome in a family transmitted by an autosomal dominant gene with variable expressivity and reduced penetrance. To our knowledge, it is the first report of familial bilateral carpal tunnel syndrome in a family with possible skipped generation.     

Evaluation of aortic stiffness in children with chronic renal failure

The measurement of aortic stiffness (As) [aortic strain (S), pressure strain elastic modulus (Ep) and pressure strain normalized by diastolic pressure (Ep*)] is suggested as an excellent marker of subclinical arterial sclerosis. We aimed to investigate the presence of As and to determine the relationship between As and some risk factors in children with chronic renal failure (CRF). Twenty-six pre-dialysis (PreD) [female/male (F/M) 7/19] patients and 23 chronic peritoneal dialysis (CPD) (F/M 13/10) patients were assessed. Twenty-nine healthy children were selected as a control group (F/M 14/15). We determined anemia, abnormal calcium/phosphate metabolism, hypertension, diastolic dysfunction, increased left ventricular mass (LVM), hypertriglyceridemia, increased stiffness (Ep, Ep*), and decreased strain (S) in the CRF (PreD and CPD) group compared with the controls (P < 0.05). Presence of renal disease, LVM and usage of angiotensin-converting enzyme inhibitor (ACE-I) in all groups; female gender, duration of disease and the usage of anti-hypertensive drug therapy in CRF patients; and LVM and LVM index in healthy children were found to be independent predictors for aortic stiffness and/or strain. In conclusion, CRF is associated with significant arterial functional abnormalities in uremic children and not controlled by dialysis treatment. These results suggest that, even in young children, uremia has a profound impact on arterial function.