A new surgical technique for adenoid cystic carcinoma involving tracheal carina

Reported is the successful treatment of a 24-year-old male with adenocystic carcinoma involving the tracheal carina, in which the tumor extended along the right main bronchus across the orifice of the right upper lobe. The patient underwent a carinal resection plus right upper lobectomy and reconstruction of the carina, resulting in neither anastomotic complication nor recurrence of disease during 28 months of follow-up.     

The MEFV mutations and their clinical correlations in children with familial Mediterranean fever in southeast Turkey

The aim of this study was to determine the Mediterranean fever (MEFV) gene mutations and their clinical correlations in children with familial Mediterranean fever (FMF) in southeast Turkey. Clinical and laboratory characteristics of 147 (65 males, 82 females) consecutive children with FMF having a positive MEFV gene mutation were prospectively investigated. Patients with negative MEFV gene mutations or atypical FMF presentations and those from other regions of the country were excluded. Clinical manifestations and disease severity scores were recorded. The six most frequent MEFV mutations including M694V, V726A, R726H, P369S, E148Q and P369S were investigated by a reverse hybridization test method. The median age of study group was 9.0 years, median age at diagnosis was 7.8 years, median age at disease onset was 5.0 years, and median follow-up duration was 4.0 years. A positive family history of FMF and parent-to-offspring transmission was found in 58.5 and 42.2 % of families, respectively. The frequencies of independent alleles, with decreasing order, were E148Q (30.7 %), M694V (26.0 %), R761H (13.5 %), V726A (13.0 %), P369S (10.5 %) and M680I (6.3 %) in FMF patients. The M694V subgroup had higher mean disease severity score and longer attack duration compared with E148Q and other mutations subgroups (p < 0.05). Two patients with amyloidosis had the M694V homozygote genotype. In conclusion contrast to other regions and many other ethnicities of the world, the most frequent MEFV gene mutation was E148Q in southeast Turkey. The M694V mutation frequency was lower, and disease severity was relatively mild in FMF children of this region.     

Effects of Nasal CPAP Treatment on Insulin Resistance, Lipid Profile, and Plasma Leptin in Sleep Apnea

Background  Obstructive sleep apnea has been linked with metabolic syndrome characterized by dyslipidemia, dyscoagulation, hypertension, and diabetes mellitus type 2 and their cardiovascular consequences. This study was designed to determine the effects of 8 weeks of therapy with continuous positive airway pressure (CPAP) on insulin resistance, glucose, and lipid profile, and the relationship between leptin and insulin-resistance parameters in patients with moderate-to-severe obstructive sleep apnea. Methods  In 44 patients, serum cholesterol, triglycerides, high-density lipoprotein, low-density lipoprotein, very low-density lipoprotein, leptin, and insulin parameters were measured at baseline and after 8 weeks of CPAP. Insulin resistance index was based on the homeostasis model assessment (HOMA-IR) method. Insulin sensitivity (HOMA-S) and insulin secretion capacity (HOMA-β) also were calculated. Thirteen patients were excluded from statistical analyses due to noncompliant CPAP usage (<4 h night⁻¹). Results  In 31 patients who used CPAP for ≥4 h night⁻¹, CPAP therapy reduced total cholesterol (P < 0.05), low-density lipoprotein (P < 0.05), and leptin (P < 0.05). Circulating leptin levels showed significant correlation with both HOMA-S and HOMA-IR at baseline and follow-up (P = 0.03 for all). In addition, there was no correlation between HOMA-IR and the severity of sleep apnea, which was shown by apnea-hypopnea index. Conclusions  In patients with moderate-to-severe obstructive sleep apnea, compliant CPAP usage may improve insulin secretion capacity, reduce leptin, total cholesterol, and low-density lipoprotein levels. Leptin showed significant relationship with insulin resistance, and this relationship remained after 8 weeks of CPAP therapy.     

Polyarteritis nodosa and Henoch–Schönlein purpura nephritis in a child with familial Mediterranean fever: a case report

Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Approximately 5% of individuals with familial Mediterranean fever have been reported to have Henoch–Schonlein purpura and about 1% to have polyarteritis nodosa. A 7-year-old girl presenting with complaints of purpuric rash, abdominal pain, arthritis, hematuria, and proteinuria and having IgA depositions on renal biopsy was diagnosed as Henoch–Schönlein nephritis. She had a history of recurrent fever, abdominal and joint pain and M694 V compound homozygote mutation. Colchicine treatment was started for the diagnosis of FMF. When constitutional symptoms such as myalgia, weight loss, fatigue, fever, and hypertension were added to the clinical picture, the diagnosis of polyarteritis nodosa HSP was thought and confirmed by the demonstration of microaneurisms on renal arteries. There was no response to corticosteroid and cyclophosphamide treatments; however, the symptoms were rapidly and dramatically reduced after the administration of intravenous immunoglobulin. In conclusion, polyarteritis nodosa and Henoch–Schonlein purpura can be seen together with familial Mediterranean fever. It is also suggested that IVIG might be an important adjunct therapy in selected patients with polyarteritis nodosa, especially in the lack of response to steroids and immunsuppressive drugs.     

Bone mineral density in familial Mediterranean fever

The aim of this study was to evaluate the bone mineral density (BMD) in familial Mediterranean fever (FMF) and to search the effects of genetic factors, family history of FMF and types of clinical attacks on BMD. Forty-four attack-free patients with FMF and 36 healthy voluntary subjects were included in the study. BMD measurements of lumbar spine and left proximal femur were performed by dual energy X-ray absorptiometry (DEXA). There was no statistically significant difference between patient and control groups regarding median values of lumbar BMD (P?=?0.06), lumbar T (P?=?0.08) and Z (P?=?0.12) scores, femoral neck BMD (P?=?0.13), femoral T (P?=?0.22) and Z (P?=?0.16) scores and total femur BMD (P?=?0.14), T (P?=?0.19) and Z (P?=?0.27) scores. Patients with negative FMF family history had significantly lower femoral neck BMD (P?=?0.018), femoral neck T (P?=?0.009) and Z (P?=?0.01) scores and total femur BMD (P?=?0.033) than patients with positive FMF family history. There was no significant difference among the groups regarding mutation characteristic and types of attacks in lumbar BMD, T and Z scores, femoral neck BMD, T and Z scores and total femur BMD, T and Z scores (P?>?0.05). We found that the bone loss of patients with FMF is not different from that of the controls. The increased bone loss in the patients with negative family history for FMF should be further investigated with larger patient groups taking into consideration of the risk factors related to family history for osteoporosis.     

Prospective study of combined modality treatment or radiotherapy alone in the management of early-stage adult Hodgkin's disease

PURPOSE: To determine the efficacy and toxicity of combined modality treatment (CMT) or radiotherapy (RT) alone in the management of clinical Stage I-IIA adult Hodgkin's disease patients. METHODS AND MATERIALS: Forty-seven patients with supradiaphragmatic clinical Stage I-IIA Hodgkin's disease without bulky mediastinal lymphadenopathy were enrolled into this prospective study between September 1997 and February 2002. Patients with very favorable criteria presenting with one or two nonbulky nodal areas involved, an erythrocyte sedimentation rate of <50 mm/h, age <40 years, and either lymphocyte predominant or nodular sclerosing histologic findings were treated by RT alone. Patients missing any of these favorable criteria were classified as the other favorable group and were treated with three courses of doxorubicin, bleomycin, vinblastine, and dacarbazine chemotherapy followed by involved-field RT. The median age was 36 years (range, 19-53 years). Of the 47 patients, 15 were women and 32 were men. Only 3 patients were classified as the most favorable group and treated with mantle RT alone; the remaining 44 were treated with CMT. RESULTS: The median follow-up was 51 months (range, 20-74 months). Only 2 patients developed recurrence, both out of the irradiated field, one in the contralateral neck and the other in the abdomen. The 5-year relapse-free and overall survival rate was 95.4% and 97.8%, respectively. Although none of the prognostic factors were statistically significant for relapse-free survival, a trend was noted for the response to chemotherapy (p = 0.06). Only 2 patients developed treatment-related complications. One patient treated with mantle RT alone developed severe ischemic heart disease and one in the CMT arm developed subclinical hypothyroidism. CONCLUSION: Despite the short follow-up, CMT or RT alone tailored according to the clinical prognostic factors were successful in terms of disease control in clinical Stage I-IIA Hodgkin's disease. Longer follow-up is required to make definitive conclusions.     

Toxic effects of anatoxin-a on testes and sperm counts of male mice

Anatoxin-a, a potent neurotoxin, is one of a number of toxins produced by cyanobacteria especially some strains of Anabaena. Toxic cyanobacteria are found worldwide in inland and coastal water environments. The present study was performed to evaluate the toxicity of anatoxin-a on testes and sperm counts of male mice. The animals of the treatment groups were administered with 50, 100 and 150microg/kg/day anatoxin-a for seven consecutive days by intraperitoneal (i.p.) route. Although there were no significant changes in body weight gain, and absolute and relative testes weights, absolute and relative weights of cauda epididymis reduced significantly in the 100 and 150microg/kg groups when compared with control group. The number of sperm count in cauda epididymis was reduced dose dependently in all treatment groups compared with control animals. Anatoxin-a caused dose-dependent histopathological changes in the testes of mice such as degenerations in seminiferous tubules, intercellular disassociation of spermatogenetic cell lines, sloughing of germ cells into tubular lumen, vacuolisation in Sertoli cells and loss of germ cells. The epithelial thickness of seminiferous tubules decreased significantly in all treatment groups in a dose-dependent manner.     

Reattachment of subgingivally fractured central incisor with an open apex

Abstract –  A case report of a 6-year-old girl with a fractured maxillary left central incisor with an open apex is presented. The procedure used to repair the fracture included flap surgery with an intrasulcular incision and endodontic treatment. The patient was called for 3 months regular follow-up to check the root formation. At the end of 32 months just before the root was obturated by guttaperka, she fractured the same tooth. Flap surgery was repeated and the tooth was restored. The root canal was obturated with a root filling paste and guttaperka as the apex was closed. Examination 10 months after treatment revealed good periodontal health, aesthetics and normal function.     

Abdominal insufflation-deflation injury in small intestine in rabbits.

OBJECTIVE: To investigate the effect of increased intra-abdominal pressure followed by abdominal deflation on small intestine with or without previous intestinal ischaemia. DESIGN: Randomised experimental study. SETTING: University hospital, Turkey. ANIMALS: 78 male New Zealand white rabbits. INTERVENTIONS: Experiment 1: 30 rabbits (10 in each group) were subjected to intra-abdominal pressure of 0 mmHg (controls), 15 mmHg or 25 mmHg for 60 minutes. Experiment 2: 48 rabbits were divided into four groups (n = 12 in each). The first comprised sham-operated controls. In the other 3, the superior mesenteric artery was occluded for 60 minutes. Reperfusion was started and maintained under intra-abdominal pressure of 0 mmHg, 15 mmHg, or 25 mmHg for one hour. Intestinal specimens were obtained five and 60 minutes after abdominal deflation in the pressure groups and at identical times in the other groups in both experiments. MAIN OUTCOME MEASURES: Intestinal malondialdehyde concentration, wet:dry intestinal weight ratio, and mucosal injury score under light microscopy. RESULTS: In experiment I the malondialdehyde concentration, wet:dry weight ratio, and mucosal injury scores were higher in the 25 mmHg group than in the other groups in both specimens. There was a significant increase in malondialdehyde concentration in the 15 mmHg group in only the 5-minute specimen. In experiment 2, except for the wet:dry weight ratio in the 5-minute specimen in the 15 mmHg group, there were significant increases in malondialdehyde concentration, wet:dry weight ratio, and mucosal injury score in all groups except controls in both specimens. Malondialdehyde concentration and wet:dry weight ratio were higher in the 25 mmHg group than in the 0 mmHg group in the 60-minute specimen, and higher than the 15 mmHg group in both specimens. Wet:dry weight ratio was less in the 15 mmHg group than the 0 mmHg group in both specimens. Mucosal injury score was higher in the 25 mmHg group than the other groups in both specimens. CONCLUSION: Increased intra-abdominal pressure for 60 minutes followed by abdominal deflation led to an ischaemia-reperfusion-like injury in normal small intestine in rabbits, and added to reperfusion injury in the ischaemic small intestine at an intra-abdominal pressure of 25 mmHg.