Atypical meningioma and extensive calvarium defects in neurofibromatosis type 1

A 9-year-old girl with neurofibromatosis type 1 (NF1) presented with a massive atypical meningioma and calvarial defect. Skull radiographs and cranial CT showed an extensive lytic bone lesion at the vertex. MRI demonstrated a large mass invading the calvarium and sagittal sinus. The histopathological and immunohistochemical diagnosis of the resected mass was atypical meningioma. To our knowledge, this is the first case of NF1 associated with atypical meningioma and massive calvarial defect in a child.     

Inflammatory myofibroblastic tumor (inflammatory pseudotumor) of the maxillary sinus mimicking malignancy: a case report of an unusual location (is that a true neoplasm?)

The inflammatory myofibroblastic tumor (IMT) is a space-occupying lesion of unknown etiology and a distinctive but controversial lesion. This type of tumor is recently considered neoplastic rather than inflammatory. It is usually occurring during childhood, composed of fascicles of bland myofibroblastic cells admixed with a prominent inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. IMT of the maxilla is very rare. The diagnosis of IMT can be made on the basis of histopathology and immunohistoche-mistry. Herein, we presented a patient who had IMT of the maxillary sinus that was initially misinterpreted as a malignant neoplasm upon clinical and radiographic examinations. We discussed the diagnostic and therapeutic procedures and may consider it a true neoplasm.     

Intravenous immunoglobulin and thymoglobulin facilitate kidney transplantation in complement-dependent cytotoxicity B-cell and flow cytometry T- or B-cell crossmatch-positive patients

BACKGROUND: The aim of this study was to investigate the effect of Thymoglobulin and intravenous immunoglobulin (i.v.IG) therapy on the clinical outcome of a putatively high-risk group of kidney transplant recipients who have positive B-cell complement-dependent cytotoxicity (CDC) along with positive T- or B-cell flow cytometry (FC) crossmatch results. METHODS: We prospectively studied the effects of i.v.IG and Thymoglobulin induction treatment in B-cell CDC, and T- or B-cell FC crossmatch-positive kidney transplant recipients (seven women and one man; mean age, 43+/-12 years). RESULTS: Mean peak panel-reactive antibody (PRA) was 47+/-32. Three patients had donor-specific antibody by flow PRA (two anti-DR4 and one anti-A2). Each recipient received induction treatment with i.v.IG 100 mg/kg for 3 days and Thymoglobulin 1.5 mg/kg for 5 days after transplantation. No acute cellular rejections occurred during a median follow-up of 15 months (range, 12-17 months). Only one acute humoral rejection occurred 8 days after transplantation, which responded to plasmapheresis, i.v.IG, and rituximab. One allograft was lost because of polyoma nephritis. Patient survival was 100% and allograft survival was 88%. CONCLUSION: Our results indicate that i.v.IG and Thymoglobulin induction treatment may facilitate kidney transplantation in B-cell CDC and T- or B-cell FC crossmatch-positive patients.     

Gastrointestinal complications after transperitoneal abdominal aortic surgery

A major gastrointestinal complication after transperitoneal aortic surgery, though unusual, may be disastrous. We determined retrospectively the risk factors, associated events, and outcomes of gastrointestinal complications that developed after transperitoneal aortic revascularization to treat aneurysmal or occlusive disease over a 10-year period. Among 750 patients reviewed, gastrointestinal complications developed postoperatively in 65 of them (8.6%), including paralytic ileus in 35 patients, gastrointestinal bleeding and mechanical ileus in 6 patients each, colonic necrosis in 2, ischemic colitis and diarrhea in 10, acute cholecystitis in 2, ascites in 1, as well as aortoduodenal fistula, which developed about 2 months postoperatively in 3 patients. Five of the patients died of multiorgan failure. Mean stay in the intensive care unit was 3 days, and hospital stay ranged from 15 to 60 days. No risk factors were identified for the occurrence of gastrointestinal complications. These results show that gastrointestinal complications after transperitoneal aortic surgery prolong hospital stay and may have serious consequences.     

GJB2 gene mutations causing familial hereditary deafness in Turkey

Mutations in Connexin 26 (Cx26) play an important role in autosomal non-syndromic hereditary hearing loss. In this study, our objective was to find out the significance of Cx26 mutations in Turkish families who had hereditary deafness. Fourteen families who had at least two prelingually deaf children per family were included in the study. One affected child from each of the 14 families was selected for single-stranded conformational polymorphism SSCP analysis. Three PCR reactions were used for each subject to amplify the entire Cx26 coding region with overlap. PCR products were sequenced on an Applied Biosystems (ABI) model 3700 automated sequencer. Six of the 14 representative family members (42.9%) demonstrated shifts on SSCP and were subsequently sequenced for Exons 1 and 2 of GJB2 and were tested for the 432 kb upstream deletion. No mutations were found in Exon 1 and no 432 kb deletions were noted. Three different GJB2 mutations were found in Exon 2 of the probands, which were 35delG, 299-300delAT, and 487G > A (M163V). GJB2 mutations were detected in 21.4% of the families. Two patients were homozygous for 35delG and 299-300delAT mutations, and were given a diagnosis of DFNB1 deafness (14.3%). Two different polymorphisms, 457G > A (V153I) and 380G > AG (R127H) were also found. In conclusion, although GJB2 mutations were detected in 21.4% of the families tested, only 14.3% of subject representatives were homozygous and therefore deafness caused by Cx26 mutation segregated with DFNB1. Thus, contribution of GJB2 mutations appears less significant in familial deafness. This necessitates further assessment for the other known gene regions as well as a search for new genetic factors in familial type of genetic deafness.     

Recurrent syncope in a case of metastatic malignant melanoma--an unusual presentation of an uncommon disease.

Cardiac tumours may be rare causes of syncope. Here we report a malignant melanoma that metastasized to the right atrium as an intracavitary mass that intermittently obstructed the tricuspid valve producing syncope episodes.     

Comparison of Early and Late Changes in Immunoglobulins and Acute Phase Reactants after Laparoscopic Adjustable Gastric Banding in Patients with Morbid Obesity

Background

There are differences in the levels of inflammation mediators, lipids, and formed elements of the blood in morbidly obese patients compared with individuals of normal weight. In the current study, the change in these parameters was determined in patients who achieved weight loss by undergoing laparoscopic adjustable gastric banding (LAGB) by comparing preoperative, and early (3 months) and late (12 months) postoperative values.

Methods

The body mass index (BMI), weight, blood pressure, and waist circumference of 72 patients treated by LAGB procedures between September 2006 and February 2009 were measured and recorded. Pre- and postoperative 3- and 12-month C-reactive protein (CRP), immunoglobulin (Ig) G, IgA, IgM, fibrinogen (Fbg), complement components C3 and C4, total cholesterol, triglycerides, low-density lipoprotein-C and high-density lipoprotein-C levels, and leukocyte, neutrophil, lymphocyte, and platelet counts were also measured. Results were presented as mean?±?SD. The preoperative values were compared with the 3- and 12-month values. A p value?<?0.05 was considered statistically significant.

Results

BMI, weight, blood pressure, and waist circumference measurements were reduced at 3 and 12 months postoperatively compared with preoperative values (p?<?0.05). Among the inflammatory mediators, IgG, IgM, and Fbg were reduced to near-normal values, beginning in the early postoperative period (p?<?0.05). There was no significant reduction parallel to weight loss with respect to CRP, C3, C4, and IgA values at 3 months postoperatively (p?>?0.05). However, the 12-month values of these parameters were significantly reduced (p?<?0.05).

Conclusions

Morbid obesity leads to significant changes in the levels of inflammation mediators. While there is a significant reduction in some of these mediators accompanying slight weight loss in the early period following an LAGB procedure, significant changes occur in other mediators in the late period when there is a larger amount of weight loss.