Hypergonadotroper Hypogonadismus bei Galaktosämie

Classical galactosaemia due to galactose-1-phosphate-uridyltransferase deficiency is an inherited metabolic disorder with an estimated incidence of 1:40,000 in the Caucasian population. In neonates the disease presents as hepatopathy with cerebral involvement. Without treatment classic galactosaemia leads to cataract, hepatic insufficiency and failure to thrive, and may finally be fatal. Treatment consists of a lactose-free diet. Despite early initiation of dietary treatment and long-term compliance, more than 80% of female patients develop hypergonadotropic hypogonadism. This paper aims to give practical recommendations for diagnosis and treatment of hypergonadotropic hypogonadism in patients with classical galactosaemia.     

Recurrence of posterior uveal melanoma after 60Co episcleral plaque therapy

The authors analyzed the clinical and follow-up data on 277 selected patients with primary choroidal or ciliochoroidal melanoma who were treated with 60Co plaque radiotherapy between 1976 and 1982. Local recurrence of the irradiated melanoma developed in 39 (14%) patients during the follow-up interval. The 5-year tumor recurrence rate (Kaplan-Meier) was estimated to be 12%. Multivariate prognostic factor analysis (Cox proportional hazards modeling) identified the largest linear tumor dimension and proximity of the posterior margin of the tumor to the optic nerve head as predictors of recurrence. The 5-year survival rate of patients whose tumors recurred (58%) was significantly (log-rank test P = 0.0023) worse than that of patients whose tumor remained clinically controlled (82%).     

Sacral acupuncture for pain relief in labour: initial clinical experience in Nigerian women

Sacral acupuncture was used for pain relief during labour in 30 pregnant Nigerian women. It produced clinically adequate analgesia in 19 women (63.3%). 6 women in this group (31.6%) reported that they had experienced no pain whatsoever throughout the period of labour and delivery (average duration - 8 hours). 11 women (36.7%) had no pain relief and required pethidine injection when sacral acupuncture proved ineffective. 24 women (80%), including 5 who did not obtain relief, indicated their wish to have sacral acupuncture during their next confinement. 2 women (6.7%) objected to needling, 3 considered acupuncture useless while another 2 did not believe in it. The patients' cardio-respiratory functions and uterine contractions were not adversely affected. There were no untoward effects on the mothers or their neonates. The procedure was technically simple, the equipment light and cheap. The needles did not interfere with nursing or obstetric manouvres. The procedure was however time consuming. The results were inconsistent and unpredictable. Despite these limitations, the simplicity, cheapness and absence of physiological complications associated with the procedure, make it a worthwhile medical armament for pain relief in the Nigerian environment, with limited resources and specialized manpower.     

Effect of minimizing preoperative fasting on perioperative blood glucose homeostasis in children

Two hundred healthy, unpremedicated children, ages 1–10 years, scheduled for elective outpatient surgery were studied in order to examine the effect of minimizing preoperative fasting on perioperative blood glucose concentrations in paediatric patients. None of the patients ingested solids after midnight. On the day of surgery, the children were assigned to one of two groups. Group A children (n= 113) were not allowed any liquids for at least 6 h prior to surgery (NPO). Children in Group B (n= 87) ingested 10 ml·kg^?1 of apple juice 2–4 h prior to the induction of anaesthesia. All patients received lactated Ringer's solution intraoperatively, unless BG at induction was < 50 mg·dl^?1 (2.8 m·mol·l^?1) in which case dextrose 2.5% in lactated Ringer's solution was administered. None of the patients who received apple juice was hypoglycaemic during induction of anaesthesia. However, two children in the NPO group had blood glucose values ± 50 mg·dl^?1 (2.8 m·mol·l^?1) at the time of induction of anaesthesia. Thirteen (11%) patients in Group A and 6 (7%) patients in Group B showed either no change or a further decrease in their postoperative BG concentration as compared with their induction values. Two of 43 patients in Group A and 2 of 41 patients in Group B had gastric fluid volumes > 0.4 ml/kg. All patients in both groups had gastric pH < 2.5. This study shows that gastric fluid volume and pH following a 2–4 h fast are not different from the values measured in children who were subjected to a traditional fasting period of 6 h or longer. Moreover, apple juice consumed 2–4 h prior to surgery neither buffers gastric pH nor does it modify intraoperative glucose homeostasis in children.     

Temperature-controlled radiofrequency catheter ablation of AV conduction: first clinical experience

A new technique for catheter ablation of atrioventricular (AV)conduction, using temperature-controlled radiofrequency energyand a bipolar asymmetrical electrode configuration, was appliedto 12 patients (mean age, 48 ± 15 years; range, 18–69years) with medically refractory atrioventricular nodal reentranttachycardia (AVNRT) or rapid atrial rhythms. The energy sourcewas a 500 kHz generator with automatic power regulation to apreselected temperature of 80 °C. A specially designed 7F bipolar asymmetric thermo-catheter was used for ablation inall cases. The endpoints of the procedure were: first-degreeAV block in patients with AVN R T and third-degree block inpatients with atrial fibrillation or flutter. Energy was appliedover a range of 1–14 times per patient. After a mean follow-upof 8±4 months, third- or first degree AV block persistedin eight patients. In comparison to constant-power radiofrequencyablation, where impedance rises are commonly observed, no impedancerise or coating of the electrode occurred during any of the97 energy applications in this study. Variable wall contactof the electrode was identified in 20 of 97 applications bya slow temperature rise or a drop in temperature and frequentpower adjustments. Thus, monitoring temperature and automaticpower regulation may help to reduce the total delivered energy.Temperature control during radiofrequency energy avoids coagulumformation and consequently the associated potential hazardsof constant-power application.     

Cross-calibration of liquid and solid QCT calibration standards: Corrections to the UCSF normative data

Quantitative computed tomography (QCT) has been shown to be a precise and sensitive method for evaluating spinal bone mineral density (BMD) and skeletal response to aging and therapy. Precise and accurate determination of BMD using QCT requires a calibration standard to compensate for and reduce the effects of beam-hardening artifacts and scanner drift. The first standards were based on dipotassium hydrogen phosphate (K₂HPO₄) solutions. Recently, several manufacturers have developed stable solid calibration standards based on calcium hydroxyapatite (CHA) in water-equivalent plastic. Due to differences in attenuating properties of the liquid and solid standards, the calibrated BMD values obtained with each system do not agree. In order to compare and interpret the results obtained on both systems, cross-calibration measurements were performed in phantoms and patients using the University of California San Francisco (UCSF) liquid standard and the Image Analysis (IA) solid standard on the UCSF GE 9800 CT scanner. From the phantom measurements, a highly linear relationship was found between the liquid- and solid-calibrated BMD values. No influence on the cross-calibration due to simulated variations in body size or vertebral fat content was seen, though a significant difference in the cross-calibration was observed between scans acquired at 80 and 140 kVp. From the patient measurements, a linear relationship between the liquid (UCSF) and solid (IA) calibrated values was derived for GE 9800 CT scanners at 80 kVp (IA=[1.15×UCSF]-7.32). The UCSF normative database for women and men obtained with the liquid standard was corrected for use with the solid standard. Proper procedures for cross-calibrating QCT measurements and the appropriate uses of normative data are discussed.     

Kinetics of soluble TNF-receptors and soluble adhesion molecules ICAM-1, E-selectin and VCAM-1 under systemic rhTNFα therapy

Cytostatic as well as cytotoxic effects of tumour necrosis factor alpha (TNF-α) therapy have been shown in vitro and in experimental in vivo models. Nevertheless, the mechanism of anti-tumour activity in humans in vivo remains unclear. To determine the role of the vascular lining endothelial cells as important mediators of several immunological interactions, we investigated changes in the levels of the soluble endothelial cell adhesion molecules intercellular adhesion molecule 1, E-selectin and vascular cell adhesion molecule 1 as well as of soluble TNF receptors I and II during systemic therapy with recombinant human rhTNF-α (rhTNF-α). All tests were performed by enzyme-linked immunosorbent assays (ELISAs). The clinical efficacy of the intravenous rhTNF-α therapy was poor. Only one patient with isolated intra-arterial limb perfusion had a delayed, marked, but only temporary necrosis of tumour cells. In contrast, we found a marked, significant and (during therapy) undulating augmented increase in the levels of soluble adhesion molecules as well as of the soluble TNF receptors. Taken together, these data support the hypothesis that a sufficient tumour-specific cellular immunity is required to achieve a clinically apparent efficacy of systemic rhTNF-α therapy in addition to cytokine-dependent inducible activation mechanisms. In this context, the vascular lining endothelial cells might play an important role as mediators of the complex immunological antitumoral activity.     

Safety and immunogenicity of Salmonella typhi Ty21a vaccine in young Thai children.

Salmonella typhi Ty21a vaccine in a liquid formulation was evaluated in 634 Thai children 2 to 6 years of age. The seroconversion rate was 69% for those who received vaccine versus 14% for those who received placebo (P < 0.005). The immune responses among vaccine recipients ranged from 60% in 3-year-olds to 81% for 6-year-olds.     

A comparison of three diuretic regimens in heart failure

Abstract. Eight patients with mild heart failure were treated in random order for 1 week with 2 mg bumethanide at 0800 and 1200 (treatment I) h, 1 mg bumethanide at 0800, 1200, 1800, 2200 (treatment 2) and 5 mg bendroflumethiazide at 0800 and 1800 (treatment 3) h. The 'quality of life' did not differ significantly between the three treatment periods. At the presumed trough of the diuretic effect the circulating blood volume was largest during treatment 1; it was 6.3% smaller during treatment 2 ( P< 0.02) and 6.7%) lower during treatment 3 (P<0.05). In comparison with treatment 1, the maximal increase in rate-pressure product during physical exercise was 24.6% higher in treatment 3. Compared with treatment 1 the area under the curve (AUC) for plasma lactate during physical exercise was 14% lower during treatment 2 (P<0.05) and 18% lower during treatment 3 (P<0.01). These findings suggest that the type of program for diuretic therapy influences the magnitude of inevitable diurnal fluctuations in body fluids, the ability of the heart to work and the ability of the body to adjust to the oxygen demand.