Management of maple syrup urine disease in the peri-operative period.

Maple syrup urine disease (MSUD) has an incidence of 1:125,000 newborns in Ireland. Patients, when fasting, or in a catabolic state build up toxic metabolites leading to progressive neurological dysfunction. We describe the necessary peri-operative management of a patient with MSUD who developed symptomatic gallstones requiring a laparoscopic cholecystectomy.

Randomized clinical trial to determine the effect of nasogastric drainage on tracheal acid aspiration following oesophagectomy.

BACKGROUND: The aim of this study was to investigate tracheal acid aspiration after oesophagectomy and to determine whether it is influenced by nasogastric (NG) drainage. METHODS: Thirty-four patients undergoing oesophagectomy were randomized to one of three methods of NG drainage: a single-lumen tube with free drainage and 4-hourly aspiration, a sump-type tube on continuous suction drainage, or no NG tube. A tracheal pH probe was used to collect information on acid aspiration for 48 h after surgery. A pH < 5.5 was considered abnormal (normal pH 6.8-7.2). Total time with tracheal pH < 5.5, number of reflux episodes and longest reflux time were compared between groups. RESULTS: There was significant and persistent tracheal acid aspiration in all patients. Patients with a sump-type tube had a significantly shorter total time with tracheal pH < 5.5 than those in the other groups (sump-type tube versus single-lumen tube, P = 0.0069; sump-type tube versus no tube, P = 0.0071). Patients randomized to no NG tube experienced more respiratory complications after surgery than those who had either single-lumen or sump-type tubes (seven of 12 versus four of 22 patients; P = 0.023). Insertion of a NG tube was necessary in the first week after surgery in seven of 12 patients in this group. CONCLUSION: Routine NG drainage after oesophagectomy is necessary. A sump-type NG tube is better at preventing tracheal acid aspiration and may reduce the incidence of respiratory complications.

Accelerated hyperfractionated total-lymphoid irradiation, high-dose chemotherapy, and autologous bone marrow transplantation for refractory and relapsing patients with Hodgkin's disease.

PURPOSE: To evaluate the feasibility and therapeutic effect of accelerated hyperfractionated total-lymphoid irradiation (TLI), high-dose chemotherapy, and autologous bone marrow transplantation (AuBMT) in patients with relapsing or chemotherapy-resistant Hodgkin's disease (HD). PATIENTS AND METHODS: Forty-seven patients with HD who either relapsed after chemotherapy (n = 19), or failed to respond (n = 28) to at least two regimens of combination chemotherapy were studied. No patient received prior radiation therapy (RT). Treatment started with reinduction with standard-dose chemotherapy, followed by involved-field irradiation (15 Gy) to areas of relapsed or persistent disease and TLI (20.04 Gy given in 1.67 Gy fractions three times per day for 4 days). Subsequently, patients received etoposide and high-dose cyclophosphamide, followed by infusion of unpurged autologous bone marrow. All surviving patients had a minimum follow-up duration of 1 year. The median follow-up duration for survivors was 40+ months, and the maximum follow-up duration was 80+ months. RESULTS: Of the 47 patients treated, eight (17%) died of toxicity during the peritransplant period. Twenty-nine of the remaining 39 assessable patients (74%) attained a complete response (CR), while 10 remained with residual disease and progressed early after AuBMT. Four of the CR patients (14%) relapsed and 25 patients remained alive and free of disease. The actuarial disease-free survival (DFS) rate for the entire group at 6.5 years was 50%. Patients who received the protocol for relapsing HD had a significantly better DFS rate (79%) compared with patients treated for continuous refractory disease (DFS, 33%; P < .03). CONCLUSION: Previously unirradiated patients with relapsing or chemotherapy-resistant HD who have exhausted conventional chemotherapy may still respond to an aggressive therapeutic approach consisting of accelerated hyperfractionated TLI, high-dose chemotherapy, and AuBMT rescue. This program offers a potential for long-term DFS to approximately one half of patients who would otherwise have a dismal prognosis with standard-dose salvage therapy.

Direct Observation and Analysis of CuO2 Shear Defects in La2-xSrxCuO4.

Direct observations of CuO(2) sheet defect structures in superconducting La(2-x)Sr(x)CuO(4), with x in the range 0.05 crystallographic directions, by a pure shear mechanism along the edge of the octahedral copper-oxygen units. The line defects are partial screw dislocations, with characteristic displacement vectors of the type <(a/2), 0, (c/6)>, bounding the stacking faults. The existence of this type of defect demonstrates that there is an oxygen deficiency within the CuO(2) layers. However, unlike the open ReO(3) type-related structures, the packing density of the K(2)NiF(4) structure necessarily requires that anion defects be accompanied by the loss of cations of the A type (lanthanum, strontium). Under identical synthesis conditions, no defects are observed in the parent compound La(2)CuO(4).