Choreic syndrome and coeliac disease: a hitherto unrecognised association.

Coeliac disease has been associated with a variety of neurological conditions, most frequently cerebellar ataxia and peripheral neuropathy. To date, chorea has not been associated with coeliac disease. We present the case histories of 4 individuals with coeliac disease and chorea (4 women, average age of onset of chorea 61 years). Unexpectedly, most of these patients showed a notable improvement in their motor symptoms after the introduction of a gluten-free diet.     

Clinical and radiological diagnosis of Crohn's disease in children

Diagnosis of Crohn's disease in children may be delayed because of obscure presentation and inappropriate investigation. The clinical and radiological features in 35 children with Crohn's disease were reviewed retrospectively. The distal ileum and proximal colon were the most common sites of disease. Intestinal symptoms and signs were often overshadowed by nonspecific complaints. Proximal small bowel disease was characterised by oedema, clubbing, anaemia, and stunting of growth. The delay in diagnosis was less than in other series, although most prolonged in the group with proximal disease. We have identified a group of clinical features that strongly suggests Crohn's disease. In these patients, we believe a small bowel enema is justified. Small bowel enema, although invasive, is more effective than barium meal and follow-through for demonstrating Crohn's disease. Severity of radiological changes did not correlate with clinical assessment. Neither bowel stricture nor fistula was found. Radiology of the colon was insensitive and colonoscopy is better for assessing colonic disease.     

Colorectal anastomotic stenosis results of a survey of the ASCRS membership

Anastomotic stenosis is a poorly understood and underexamined complication of gastrointestinal surgery, reportedly most frequent in the coloproctostomy. In order to better define this problem, a questionnaire was sent to members of the American Society of Colon and Rectal Surgeons regarding patients with gastrointestinal anastomotic stenosis. A total of 123 patients with intestinal anastomotic stenosis were analyzed. Eighty-two anastomoses were stapled and 41 were handsewn. Nearly all stenoses occurred in the distal bowel (70 rectal, 23 sigmoid colon). Preoperative risk factors identified were obesity (28 patients) and abscess (12 patients). Incomplete “doughnuts” were noted in 12 patients. Postoperative anastomotic leaks (15 patients), pelvic infection (13 patients), and postoperative radiation (7 patients) were believed to be contributing factors. Dilatation, using a variety of techniques, was the sole treatment for 65 patients, however, intra-abdominal surgery was necessary in 34 patients. Large intestinal anastomotic stenosis probably occurs most commonly following coloproctostomy (both with handsewn and stapled anastomoses). Dilatation alone resulted in adequate treatment in most patients in the study. Major surgery was required to correct this problem in a significant number of patients (28 percent) in this series. The true incidence of anastomotic stenosis in colorectal surgery is unknown and warrants further study. Poster presentation at the meeting of the American Society of Colon and Rectal Surgeons, Anaheim, California, June 12 to 17, 1988.     

Release of Proinflammatory Cytokines During Pediatric Cardiopulmonary Bypass: Heparin-Bonded Versus Nonbonded Oxygenators

Background. Heparin bonding of the cardiopulmonary bypass (CPB) circuit may be associated with a reduced inflammatory response and improved clinical outcome. The relative contribution of a heparin-bonded oxygenator (ie, >80% of circuit surface area) to these effects was assessed in a group of pediatric patients.

Methods. Twenty-one pediatric patients undergoing CPB operations were assigned randomly to receive either a heparin-bonded oxygenator (group H, n = 11) or a nonbonded oxygenator (group C, n = 10) in otherwise nonbonded circuits. The two groups were similar in pathology, age, weight, CPB time, and cross-clamp time. Plasma levels of the cytokines tumor necrosis factor-, interleukin-6, and interleukin-8, as well as terminal complement complex, neutrophils, and elastase, were analyzed before, during, and after CPB.

Results. Significant levels of tumor necrosis factor- were not detected in either group. Plasma levels of all other markers increased during and after CPB compared with baseline. Plasma levels of interleukin-6 peaked in both groups 2 hours after the administration of protamine but remained significantly higher in group C 24 hours after operation. Plasma concentrations of interleukin-8 peaked at similar levels in both groups 30 minutes after protamine administration and returned to baseline thereafter. Levels of terminal complement complex and elastase peaked in both groups 30 minutes after protamine administration. Plasma levels of terminal complement complex were significantly higher at the end of CPB and after protamine administration in group C. Elastase levels were significantly higher 2 and 24 hours after CPB in group C. The ventilation time of patients in group H was significantly lower than that of patients in group C: 10 (range, 3 to 24) versus 22 (range, 7 to 24) hours, respectively (p < 0.01).

Conclusions. The present study confirms the proinflammatory nature of pediatric operations and demonstrates a lessened systemic inflammatory response with the use of heparin-bonded oxygenators. This is achieved without bonding of the entire circuit, which could have significant cost-benefit implications by negating the need for custom-built heparin-bonded circuitry.     

THE SURGICAL MANAGEMENT OF OSSEOUS CRANIAL BASE TUMOURS IN CHILDREN

Background : Osseous cranial base tumours in children present as a diverse collection of both benign and malignant pathologies. Concerns raised by the difficulty in accurate diagnosis and local recurrence of benign lesions and by the long-term sequelae of radiotherapy for malignant cranial tumours (marked local growth disturbances, pituitary dysfunction, visual disturbances, late new tumour induction) prompted an evaluation of surgical resection of cranial base tumours in children, with specific regard to safety, efficacy and aesthetic result. Methods : A retrospective review was performed of 10 consecutive children presenting with tumours either arising from or eroding into bone of the cranial base who were managed by surgical resection in a 10-year period from 1986 to 1996. The patients demonstrated a great variation in both presentation and pathology. All underwent surgical resection of tumour with reconstruction where indicated. Results : There were no postoperative complications or mortality. All patients remained clinically free of disease at follow-up, which ranged from 17 months to 9 years (mean 6 years and 4 months). Conclusion : The aggressive surgical resection and craniofacial reconstruction of cranial base tumours in the paediatric population offers a safe and efficacious mode of treatment that obviates problems of diagnosis and local recurrence for benign lesions and of the long-term sequelae of radiotherapy for malignant lesions.     

Thoracoscopic Retrieval of Foreign Bodies After Penetrating Chest Trauma

Video-assisted thoracic surgery or thoracoscopy has proved to be valuable in many settings in thoracic surgery. The use of video-assisted thoracic surgery in trauma has been limited, especially with respect to penetrating trauma. We report the use of thoracoscopy to remove intrathoracic fragments of glass and avert the need for a thoracotomy.     

No regression of renal AL amyloid in monoclonal gammopathy after successful autologous blood stem cell transplantation and significant clinical improvement.

BACKGROUND: High-dose chemotherapy followed by autologous blood stem cell transplantation induces remission of plasma cell dyscrasia in patients with AL amyloidosis. The impact of this treatment on the glomerular amyloid mass is still unknown. METHODS: In the present study, the quantity of the renal amyloid mass before and more than 3 years after high-dose melphalan treatment and autologous blood stem cell transplantation was assessed in two patients. At the time of the second renal biopsy, both patients were in complete remission without detectable serum and urinary monoclonal IgA-lambda and a normal percentage of plasma cells in the bone marrow. RESULTS: In both patients with biopsy-proven AL amyloidosis, urinary protein excretion decreased from 7 g/24 h to <2 g/24 h more than 3 years after autologous blood stem cell transplantation. In contrast, glomerular amyloid deposits persisted, as shown in the second biopsy. CONCLUSION: Despite complete remission of the plasma cell dyscrasia and improvement of glomerular permeability, the amount of glomerular amyloid mass did not regress.