Upper tract surveillance in primary bladder cancer follow-up

OBJECTIVE: To determine if there is national variation in regimens of upper urinary tract surveillance in patients with primary bladder cancer. METHODS: A questionnaire was sent to 470 consultant urologists from a British Association of Urological Surgeons list in the UK; 301 anonymous replies were received. Two replies were incomplete and therefore the results of 299 questionnaires (64%) were analysed. RESULTS: Of the 299 surgeons, 19 (6%) use no form of upper urinary tract surveillance; 162 (54%) use surveillance in selected patients, i.e. those with carcinoma in situ (47%), multiple bladder tumours at first presentation (39%) and after cystectomy (70%), and 118 (39%) use upper tract surveillance on all patients with a history of bladder cancer. The median (range) screening interval was 24 (12-60) months and surveillance continued for a median of 10 (2 to indefinite) years, continuing for an indefinite period in 33%. CONCLUSIONS: Most urologists use upper tract surveillance in patients with bladder cancer but there is wide variation in the duration and interval for which it continues, and in the type of patient selected for surveillance. Some patients at high risk of upper tract tumour are not being screened. Asymptomatic upper tract tumours may not be diagnosed because the intervals between surveillance are too long, and the duration for which it continues inadequate. There is a need for multidisciplinary national guidelines to reduce variation in practice.     

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.