Vulvar syringoma aggravated by pregnancy

Syringoma is a benign tumors of eccrine sweat gland. They appear as multiple, tiny, firm, skin-colored papules. Vulvar involvement of syringoma is rare. Only 24 cases with vulvar syringoma have been previously reported in the literature. The majority of patients with vulvar syringomas are asymptomatic. A case of syringoma of the vulva exacerbated during pregnancy is presented. The case appears remarkable for the experienced aggravated pruritic symptoms of the patient during her pregnancy.     

Combined retrosigmoid retrolabyrinthine vestibular nerve section: results of our experience over 10 years.

OBJECTIVE: We aimed to evaluate the results of our experience in vestibular nerve sectioning (VN), which was performed using combined retrosigmoid-retrolabyrinthine approach. STUDY DESIGN: Medical records of 280 patients who were consecutively operated on for incapacitating peripheral vertigo were retrospectively evaluated, and 210 patients who completed 2 years follow-up and had adequate follow-up data were found to be suitable for inclusion in the study. METHODS: Hearing results, vertigo control rates, and complications of the retrosigmoid-retrolabyrinthine VN were evaluated. RESULTS: The patients were suffering from vertigo for a mean period of 32.2 months. Bilateral Meniere's disease occurred in 5.7% of the patients in the follow-up period. A complete or substantial vertigo control could be achieved in 94.4% of the patients (191 [90.1%] in Class A and 9 [4.3%] in Class B). Preoperative speech reception threshold, pure-tone average, and speech discrimination score of the patients were 56.5 dB, 47.4 dB, and 73.6%, respectively. Postoperative corresponding values were 62.2 dB, 43.4 dB, and 68.5%, respectively (p > 0.05). The complication rate was low (2.5%). Most common complication was abdominal hematoma, which was seen in 4.5%. CONCLUSION: VN performed using retrosigmoid-retrolabyrinthine approach has low complication and high vertigo control and hearing preservation rates. It can be applied as an initial surgery or reserved as the last step when the other surgical treatments have failed to control vertigo.     

Role of cholesteryl ester transfer protein activity and genetic polymorphism in ischemic heart disease in type 2 diabetic patients

Cholesteryl Ester Transfer Protein (CETP) facilates the exchange of triglycerides (TG) and cholesteryl ester between lipoproteins particles. Diabetic subjects have been reported to have higher TG levels and lower high density lipoprotein-cholesterol (HDL-C) levels which contribute to the increased cardiovascular risk observed in some of these patients. The CETP activity was shown to be more important in a group of 93 non insulino-dependant diabetics with coronary artery disease than in a group of 92 healthy subjects (p = 0.033). Several polymorphisms have been reported in the CETP gene. The common Taq IB polymorphism is associated with decreased CETP activity and increased HDL-C. We have observed a frequency of 0.31 for B2 allele in deference to those reported in subjects from Caucasian population. An association between the presence of the B2B2 genotype, decreased CETP activity and increased of plasma HDL-C was observed in healthy subjects but not in diabetics with coronary artery disease.     

Hartnup Disease Masked by Kwashiorkor

This report describes an 11-month old girl with Hartnup disease presenting with kwashiorkor and acrodermatitis enteropathica-like skin lesions but free of other clinical findings. This case with kwashiorkor had acrodermatitis enteropathica-like desquamative skin eruption. Since zinc level was in the normal range, investigation for a metabolic disorder was considered, and Hartnup disease was diagnosed.Key words: Acrodermatitis, Case studies, Hartnup disease, Kwashiorkor, Turkey     

Postprandial cholesteryl ester transfer and high density lipoprotein composition in normotriglyceridemic non-insulin-dependent diabetic patients

Altered postprandial HDL metabolism is a possible cause of defective reverse cholesterol transport and increased cardiovascular risk in diabetic patients with a normal fasting lipoprotein profile. Ten normolipidemic, normoponderal non-insulin dependent diabetes mellitus (NIDDM) patients and seven controls received a 980 kcal meal containing 78 g lipids with 100000 IU vitamin A. Chylomicron clearance was not different, but area under the curve (AUC) for retinyl palmitate in chylimicron-free serum (remnant clearance) was greater in patients (P < 0.02). LCAT activity increased postprandially to the same extent in both groups. In control subjects, cholesteryl ester transfer protein (CETP) activity (CETA) also increased by 20% (P < 0.01 at 6 h) in parallel with a 20% decrease in HDL₂-CE (r= −0.55, P = 0.009). In NIDDM patients, on the contrary, CETA which was 35% higher in the fasting state (P < 0.005), decreased postprandially yet HDL₂-CE remained unchanged. Postprandial HDL₃ of controls were enriched with phospholipid (PL) (30.3 ± 2.6% at 6 h) with respect to fasting (25.6 ± 2.5%, P < 0.01) and to NIDDM-HDL₃ (25.8 ± 1.7% at 6 h, P < 0.01). These results show that variation in plasma CETA has little impact on HDL₂-CE in NIDDH subjects. They support the concept that, in controls, the combined enrichment of HDL₃ with PL, increased LCAT and CETA create the conditions for stimulation of cell cholesterol efflux and CE transfer to apo B lipoproteins. In NIDDM, because of the lesser HDL₃ enrichment with PL and of the inverse trend of CETA, these conditions fail to occur, depriving the patients of a potentially efficient mechanism of unesterified cholesterol (UC) clearance, despite their strictly normal preprandial profile.     

Reverse modulation of the HDL Anionic Peptide Factor and phospholipid transfer protein activity in coronary artery disease and type 2 diabetes mellitus

ObjectivesThe high density lipoprotein Anionic Peptide Factor (HDL₃-APF) was previously described as an apolipoprotein that promotes the reverse cholesterol transport. Since phospholipid transfer protein (PLTP) is involved in such mechanism we attempted to focus on the two APF and PLTP proteins.Design and methodsWe recruited 56 type 2 diabetic patients with (n = 36) or without (n = 20) coronary artery disease (CAD) and 19 CAD patients. The three groups were compared to 39 healthy control subjects. In all groups, lipid profile was determined and plasma APF concentrations and PLTP activity were measured.ResultsIn all patients, the PLTP activity was significantly increased in comparison with controls (p < 0.01), in concomitance with a plasma APF level decrease in groups with CAD (with and without type 2 diabetes) (p < 0.01). Multiple linear regression analysis demonstrated that, when apoA-I, HDL-C, HDL-phospholipids and PLTP activity were taken into account as independent variables (after univariate regression analysis), HDL-PL was positively and independently related to APF (p < 0.0001 in whole population; p = 0.0090 in controls) and PLTP activity was negatively and independently related to APF in whole population and all patients' groups (all p < 0.05), but positively and independently associated to APF in controls (p = 0.0005).ConclusionsAPF could be considered as a specific marker against CAD and type 2 diabetes mellitus and our results confirm the atherogenic behavior of PLTP in CAD. Thus, these two proteins are likely to be regulated in a reverse manner.