Renal involvement in the Laurence-Moon-Bardet-Biedl syndrome: report of five cases

.We report five patients with Laurence-Moon-Bardet-Biedl syndrome (LMBBS) who had renal involvement. Intravenous pyelography showed bilateral or unilateral calyceal clubbing and blunting in all patients. In addition, one patient had a parapelvic cyst in the left kidney and another had bilateral lobulated renal outlines of the fetal type. One patient had a urinary concentrating defect and two patients showed increased fractional sodium excretion. Estimated tubular phosphate reabsorption values were in normal limits in all of five patients. No patient had a urine acidification defect, proteinuria, glycosuria, or hyperaminoaciduria. One patient died from end-stage renal failure. The remaining four patients had normal serum creatinine values and estimated creatinine clearances. ^99mTechnetium-diethylenetriamine pentaacetate renal scanning showed prolonged and delayed concentration and delayed excretion in three of the four patients who survived. A focal scar was determined on the left kidney of one of four patients by ^99mtechnetium-dimercaptosuccinic acid renal scanning. All LMBBS cases with or without renal symptoms should be routinely evaluated for renal abnormalities. Renal scanning is a valuable method, especially for determining the renal involvement in the early stage of disease. Received November 14, 1995; received in revised form and accepted May 23, 1996     

An algorithm for deciding alternative grafting materials used in secondary rhinoplasty.

Severe middle vault deformity with disturbed nasal form and function is one of the most challenging procedures to correct in a secondary rhinoplasty. Reconstructing the deformity with autologous septal cartilage would be the primary choice of most surgeons, if it were always available. However in certain cases the lack of a sufficient quantity of autologous cartilage has forced surgeons to explore other viable options. This paper discusses our experience with the combined use of spreader and dorsal onlay grafts from various materials in the reconstruction of severe middle vault deformity in 110 patients. In follow up, (between 6 and 42 months; mean 21 months) all patients were noted to have improved in both aesthetics and function with no major complications noted. In summary, this study proposes that any engrafting material can be used safely when the proper surgical principals and technique are employed.     

The effects of hypothyroidism in rats on serum leptin concentrations and leptin mRNA levels in adipose tissue and relationship with body fat composition

Both thyroid hormones and leptin affect sympathetic nervous system activity, basal metabolic rate, body fat mass, food intake, and thermogenesis, and each one also affects the actions of the other. We examined the alterations in serum leptin concentrations and leptin mRNA expression in hypothyroid rats and investigated the relation between serum leptin and leptin mRNA levels with the total adipose tissue mass and total body weight. Twenty male Wistar rats were divided into 2 groups, euthyroid and hypothyroid. Their body compositions were examined by Dual Energy X-ray Absorptiometry at the beginning and end of the study. Serum leptin concentrations and levels of leptin mRNA in the retroperitoneal white adipose tissue were measured at the end of the study. Serum leptin concentrations did not show any difference between the two groups (1.9 +/- 0.2 ng/ml in the hypo and euthyroid group, P > 0.05), but the fat mass of the hypothyroid rats were lower than the euthyroid rats (21.1 +/- 2.5 g in the euthyroid group and 14.2 +/- 1.9 g in the hypothyroid group, P > 0.05 between groups at the end of the study) although the difference between the groups was statistically not significant. Leptin mRNA level was significantly higher in the hypothyroid group than in the euthyroid group (21.6 +/- 1.6 vs. 15.1 +/- 1.2 ng respectively, P = 0.002) although the dissected retroperitoneal fat weight was significantly lower in the hypothyroid group versus the euthyroid group (1.0 +/- 0.2 vs. 1.8 +/- 0.2 g respectively, P = 0.013). In conclusion, the change of leptin mRNA expression in white adipocytes was thought to be the direct result of hypothyroidism or a compensatory response to metabolic changes caused by hypothyroidism.     

Low level of Nesfatin-1 is associated with gestational diabetes mellitus

Introduction: Gestational diabetes mellitus (GDM) occurs in ～10–25% of pregnancies. Nesfatin-1, plays a role in carbohydrate metabolism by inhibiting glucagon secretion, besides has a glucose-dependent insulinotropic effect. Explanation of the GDM pathogenesis is important due to preventing gestational complications. We aimed to investigate relationship between GDM and Nesfatin-1.

Material and methods: Seventy-nine pregnant subjects were randomly allocated to either GDM group (GDG, n?=?38) or control group (CG, n?=?41). For GDM diagnosis, 50 and 100?g oral glucose tolerance test (OGTT) were used. Nesfatin-1, insulin and other parameters were measured for all subjects. The homeostasis model assessment-insulin resistance (HOMA-IR) was calculated.

Results: Nesfatin-1 was found lower and insulin was found higher in GDG than CG. Negative correlation has been founded between Nesfatin-1 with weight, BMI, fasting glucose, serum glucose level at first hour of the 50?g OGTT and HOMA-IR.

Conclusion: In this study, patients with GDM had lower Nesfatin-1 levels than without GDM. Therefore, when the Nesfatin-1 effects on the GDM pathogenesis is clear, it may be contributed to diagnosis and treatment of the GDM.     

Current role of laser angioplasty of restenotic coronary stents

Treatment of in-stent restenosis (ISR) with conventional percutaneous transluminal coronary angioplasty (PTCA) causes significant recurrent neointimal tissue growth in 30-85%. Therefore, laser ablation of intrastent neointimal hyperplasia before balloon dilation can be an attractive alternative. However, the long-term outcomes of such treatment have not been studied thoroughly enough. This prospective case-control study evaluated angiographic and clinical outcomes of PTCA alone and a combination of excimer laser coronary angioplasty (ELCA) and adjunct PTCA in 125 patients with ISR. ELCA was performed before balloon dilation in 67 patients, PTCA alone was performed in 58 patients. Basic demographic and clinical data were comparable in both groups. Lesions included in ELCA group were longer (17.1+/-9.9 vs 13.6+/-9.1 mm; p = 0.034), more complex (36.5% type C stenoses vs 14.3%; p = 0.006), and more frequently had reduced distal blood flow (TIMI <3: 18.9% vs 4.8%; p = 0.025) compared to lesions in the PTCA group. Immediate angiographic results of PTCA and ELCA + PTCA appeared to be comparable. PTCA alone was successful in 57 patients (98.3%), ELCA + PTCA, in 66 patients (98.5%). The rates of hospital complications were comparable (3.0% in ELCA group vs 8.6% in PTCA group). The 1-year follow-up showed that the rates of major adverse cardiac events (MACE) were comparable in the 2 groups (37.3% in ELCA group vs 46.6% in PTCA group). The rates of target vessel revascularization (TVR) within 1 year after the intervention were also similar in the 2 groups (32.8% vs 34.5%). The data mean that ELCA in patients with complex ISR is efficient and safe. Despite a higher complexity of lesions in the ELCA group, no increase in the rate of complications was registered.     

The effect of pentoxifylline on the healing of intestinal anastomosis in rats with experimental obstructive jaundice

The aims of this study were (1) to investigate the effect of experimental obstructive jaundice on the healing of intestinal anastomosis, and (2) to investigate the effect of pentoxifylline on the healing of intestinal anastomosis in rats with obstructive jaundice. Obstructive jaundice was induced in rats by the ligation and division of the common bile duct. Four days after this operation, either pentoxifylline or isotonic saline solution was administered intraperitoneally to these jaundiced rats and controls, and then intestinal anastomosis was performed. The concentrations of serum tumor necrosis factor α (TNF-α) and serum triglyceride of jaundiced and nonjaundiced rats were measured, and the quality of healing was evaluated by measuring the bursting preasure and hydroxyproline content of the anastomoses on the fifth and tenth days of anastomotic healing. Obstructive jaundice resulted in an impaired wound healing of the intestinal anastomosis in the rats. The administration of pentoxifylline to the jaundiced rats resulted in better anastomotic wound healing. The beneficial effects of pentoxifylline on anastomotic healing in rats with obstructive jaundice was attributed to its inhibitor effect on the endotoxin-induced TNF-α release from macrophages and monocytes, and the stabilizing effect on the neutrophils. Received: March 29, 1999 / Accepted: March 24, 2000