Familial hemangioblastoma and von Hippel-Lindau's disease: case report.

Thirty-five members of a family affected with von Hippel-Lindau's disease (VHL) were examined and lesions were found in nine of them. Four of the lesions were located in the central nervous system; two cerebellar, one spinal, one cerebral. Eye lesions were present in eight of the nine patients and complications developed in seven; six unilateral and one bilateral blindness. Both mortality and morbidity were found to be higher than in other series. This is attributed to insufficient screening of the patients with familial VHL, the late diagnosis was established only after the onset of complications. The importance of screening tests in VHL risk patients is emphasized.     

Comparison of local and general anesthesia in tension-free (Lichtenstein) hernioplasty: a prospective randomized trial

To compare pulmonary effects, postoperative pain and fatigue, morbidity, patient satisfaction, and cost of different anesthetic techniques for inguinal hernia repair, 50 patients were randomized to local and general anesthesia groups (LA and GA). All patients received the same premedications and the same postoperative analgesic regimen. The standardized postoperative analgesic, intramuscular pyroxicam 20 mg, was given to all patients in the recovery room and an additional 20 mg on the same day was given as requested by each patient. Pulmonary function studies and arterial blood gas analysis were performed 1 h prior to the operation and at the postoperative 8th and 24th hours. All patients underwent Lichtenstein's tension-free hernioplasty. Postoperative pain and fatigue were registered 8 h and 24 h after the operation. A questionnaire was filled out by the patients, and they were asked to give grades for the general comfort of the anesthesia and the surgical procedure (1=worst, 10=best). Postoperative pulmonary function tests were significantly poorer in the GA group both on 8th- and 24th-hour measurements (P<0.05). Patients who underwent LA had significantly lower PCO₂ and higher PO₂ at the postoperative 8th hour (P<0.05). Mean postoperative pain and fatigue scores revealed a significant difference in favor of local anesthesia at only the 8th hour (P<0.05). There were two complications, one in each group (a hematoma in LA and a urinary retention in GA). Patient satisfaction grades were not different in the two groups. We conclude that LA in inguinal hernia repair does not adversely affect pulmonary functions, patients feel less pain, and patient satisfaction is comparable to that with GA. Electronic Publication     

Results of salvage retroperitoneal lymphadenectomy (RLA) in the treatment of patients with nonseminomatous germ cell tumours remaining marker positive after inductive chemotherapy

The authors describe the evidence and results obtained in 21 out of 100 patients who underwent salvage retroperitoneal lymphadenectomy for advanced testicular cancer (UICC stage II/B bulky and stage III) in the period 1982–1993, and in whom inductive chemotherapy was not followed by marker conversion. It is stated that if AFP positivity is low (titres below 100 ng/ml) salvage retroperitoneal lymphadenectomy (RLA) is of high therapeutic value, whereas in all cases with HCG positivity or AFP titres higher than 500 ng/ml tumorous death ensued without exception. In our cases viable tumour residues occurred in 81%. Salvage resection was feasible in 76%, but every incomplete resection (19%) was followed by death due to tumour. In all but one of the cases marker positivity, an indicator of therapy-resistant viable tumour residues, persisted after having used more than four PVB combinations or changes in the chemotherapeutic regimen.     

Formulation and in Vitro-in Vivo Evaluation of Sustained-Release Lithium Carbonate Tablets

The release of lithium carbonate incorporated into polymethylmethacrylate, poly vinyl chloride, hy-drogenated vegetable oil, and carbomer matrix tablets was studied in vitro. The formulation containing 10% carbomer showed a sustained-release profile comparable to that of a standard, commercially available, sustained-release preparation containing 400 mg lithium carbonate embedded in a composite material. In vivo the newly formulated and standard sustained-release lithium carbonate tablets were compared to an oral solution and conventional lithium carbonate tablets in 12 healthy subjects. These crossover studies showed that the sustained-release tablets produced a flatter serum concentration curve than the oral solution and conventional tablet, without loss of total bioavailability.     

Peripheral seventh nerve palsy due to transorbital intracranial penetrating pontine injury

The case of a child injured by a knitting needle penetrating transorbitally and intracranially, resulting in carotid cavernous fistula and pontine injury, is reported. After receiving medical and endovascular treatment, the only remaining abnormal neurological manifestation was right peripheral facial nerve palsy. The clinical sequences of events and the demonstration of a pontine lesion leading to peripheral facial palsy are presented. Facial nuclear injury with a penetrating trauma is an extremely rare condition. It is important to identify the anatomical regions injured in penetrating traumas. The lesions must be identified by computerized tomography, magnetic resonance imaging, clinical and laboratory investigation.     

Expression of histidine decarboxylase in human colonic cancer cells and adenomatous polyps

Inflammation Research -     

Low-dose erythropoietin is effective and safe in children on continuous ambulatory peritoneal dialysis

Hypertension is one of the most important complications of erythropoietin (rHuEPO) therapy in dialysis patients. In this study, the effect of two different dosage regiments of subcutaneous rHuEPO on blood pressure [BP] was evaluated in 20 anemic children on continuous ambulatory peritoneal dialysis (CAPD). Patients were randomized to receive rHuEPO 50 U/kg, either once a week (group 1, 50 U/kg per week) or three times a week (group 2, 150 U/kg per week). At the beginning of the study, 8 patients in group 1 and 8 patients in group 2 were on antihypertensive therapy. In group 1, the hematocrit increased gradually and significantly from 18.98%±1.79% to 30.1%±1.62% after 6 months, while in group 2 it rapidly increased from 19.53%±1.86% to 32.4%±1.11% after 3 months. A significant increase in the mean arterial BP was observed in group 2. Antihypertensive therapy had to be increased in all of the 8 previously hypertensive patients and had to be initiated in 1 of the 2 originally normotensive patients in the same group. None of the patients in group 1 required a change in antihypertensive medication. We conclude that during treatment with rHuEPO pre-existing hypertension and the dose of rHuEPO are the most important risk factors for the development or worsening of hypertension in children on CAPD, and gradual elevation of hematocrit by low-dose rHuEPO avoids the development of severe hypertension. Received December 11, 1995; received in revised form September 16, 1996; accepted September 19, 1996     

Percutaneous radiofrequency rhizotomy of lumbar spinal facets: The results of 46 cases

The results of percutaneous radiofrequency rhizotomy of lumbar spinal facets in 46 patients followed at least three months (mean 15 months) are reported and compared with those reported previously. Satisfactory pain relief three months after the procedure was achieved in 36.4 percent of patients without operations and in 41.7 percent of patients with operations other than fusion.No patient had previously undergone fusion.Treatment of low-back pain by using radio-frequency thermocoagulation of spinal facets is a simple, safe, and well-tolerated procedure. It can be used to relief of pain in spite of decreasing rates of success within the follow-up period.     

1.25 (OH)2 cholecalciferol pulse therapy and the effects of different dialysis membranes on serum PTH levels of haemodialysis patients

Either oral, intravenous or subcutaneous 1.25 (OH)₂ cholecalciferol is used in the therapy of hyperparathyroidism, which is a serious complication in patients on haemodialysis. We studied a total of 30 patients (10 women and 20 men) and divided them into two groups depending on the different types of dialysis membranes used. In the poly sulfone group, mean age was 43.7±0.97 years and the average dialysis period lasted 29.9±1.23 months. For the 15 cases in which we used cuprophane membrane the mean age was 40.2±1.31 years and the average dialysis period lasted 16.2±0.86 months. The calcium level of the dialysate in both groups was 1.5 mmol/l. According to the study protocol, the determined oral calcitriol dose was 0.07 mg/kg and it was administered intermittently. After one month on high dose calcitriol therapy, treatment was continued with a maintenance dose of 0.03 mg/kg for a further six months. As a phosphate binding agent, daily 3 g calcium carbonate was administered. Before starting this treatment protocol, patients went on a 1 mg/day calcitriol therapy, although the mean PTH level was 424.63 pg/ml and the mean serum alkaline phosphatase level was 290.2 U/l. During the pretreatment period, levels of PTH, alkaline phosphatase, ionized calcium, and total calcium remained significantly within normal limits as a result of the new therapy protocol applied. PTH and phosphorus clearance rates were compared in the patient groups in which different dialysis membranes had been used. PTH and phosphorus clearances were 15.2±3 ml/min and 239.1±19.2 ml/min, respectively, in the polysulfone membrane group, and 1.1±0.3 ml/min and 112.8±9.88 ml/min, respectively, in the cuprophane membrane group (p<0.05).