Bone disease in primary biliary cirrhosis: Lack of association with distal renal tubular acidosis

Background and Aims: Primary biliary cirrhosis (PBC) might be complicated by osteoporosis, whose etiology remains unknown but seems to be multifactorial. Prevalence rates of 30% to 60% for distal renal tubular acidosis (DRTA) have been reported in PBC patients, generally as incomplete DRTA. Although it is undisputed that a reduced bone mineral density (BMD) is the expected outcome among patients who have been suffering from longstanding chronic metabolic acidosis, it is unclear if incomplete DRTA is also associated with metabolic bone disease in PBC patients. The present study was undertaken to compare the BMD of PBC patients with and without DRTA.
Methods: The BMD of 23 PBC patients (11 with DRTA and 12 without), all with normal clearance of creatinine, was assessed by dual energy radiograph absorptiometry. The diagnosis of DRTA was made if the urine pH was above 5.4 in all samples after the oral acid overload, showing tubular inability to acidify urine in the presence of test-induced systemic metabolic acidosis.
Results: Densitometric signs of osteoporosis were found in 82% of DRTA cases and in 83% of patients without DRTA (difference not significant). There were no significant differences in BMD measurement, T and Z scores of patients with and without DRTA.
Conclusions: The present study could not support a correlation between the presence of DRTA and the bone loss observed in PBC patients.     

Regional differences in calcium-release channels from heart.

The heart is a heterogeneous tissue composed of several cell types tailored for specialized functions. We found that intracellular channels also exhibit regional specialization. In cardiac and skeletal muscle these channels are called the calcium-release channel and are identified by activation with either calcium or caffeine and inhibition by the hexavalent cation ruthenium red. The calcium-release channel of the sarcoplasmic reticulum from the interventricular septum has a smaller conductance (31 pS vs. 100 pS) and has longer open and closed times when compared with the channel from left-ventricular free wall. An additional calcium-permeable channel with an even smaller conductance (17 pS) was found in the septum, and this channel is similar to the inositol 1,4,5-trisphosphate-gated channel from smooth muscle and different from the calcium-release channel (ryanodine receptor) from skeletal and cardiac muscle. The inositol 1,4,5-trisphosphate-activated channel may be derived from specialized conducting tissue that is relatively abundant in the septum, whereas the other calcium-release channels may be derived from regionally specialized myocardial cells in the septum and free wall.     

A mixing model for polychlorinated dibenzo-p-dioxins and dibenzofurans in surface sediments from Newark Bay,New Jersey using polytopic vector analysis

The identity and relative contributions of various sources of polychlorinated dibenzo-p-dioxins (PCDDs) and dibenzofurans (PCDFs) to recently deposited sediments collected in Newark Bay and its major tributaries were determined using polytopic vector analysis (PVA), a multivariate statistical technique relatively new in the chemometric literature. The concentrations of 2,3,7,8-substituted PCDD/Fs were assayed in eighty-one surface and near-surface sediment samples collected from the Passaic River, Hackensack River, Arthur Kill, Elizabeth River, Kill Van Kull, Port Elizabeth, and Port Newark navigation channels and Robins Reef, which is located in New York Harbor. PVA modeling revealed five predominant 2,3,7,8-substituted PCDD/F fingerprint patterns in geographically plausible distributions throughout the estuary. This was consistent with the current understanding of hydrodynamic and sedimentation conditions reported in the literature for Newark Bay. Three patterns contained 2,3,7,8-tetrachlorodibenzo-p-dioxin (2,3,7,8-TCDD), which is alleged to originate from a single industrial source on the lower Passaic River. One of the fingerprints containing 2,3,7,8-TCDD was present in moderate proportions (10–20%) in surface sediments near the site, but was generally observed in low abundance (<5%) elsewhere in the estuary. A fingerprint pattern characteristic of PCDD/F profiles in effluents from municipal sewage and waste water treatment plants was widely distributed in the estuary, but reached its highest relative proportions in the Elizabeth River. A third fingerprint pattern was highest in the Arthur Kill and lower Passaic River and closely matched the residue patterns found in several types of combustion sources. A fourth finger-print pattern in Hackensack River and lower Passaic River sediment matched the PCDD/F profile reported in PCB Aroclor^® formulations. A fifth fingerprint pattern matched the profile in recycled pulp and paper mill effluents and was highest in Kill van Kull and upper Passaic River sediment. The majority of PCDD/Fs in sediment from Reaches B, C, and D of Port Newark and Port Elizabeth were attributable to sediments transported via the Arthur Kill and the Kill Van Kull. These results are consistent with those previously reported using principal components analysis, which indicated that 2,3,7,8-substituted PCDD/F patterns in the sediments of Newark Bay are consistent with discharges from multiple sources.     

Efficiency of work of inspiratory muscles in standing and in exercising dogs

The objective of this study was to elucidate the resistive, elastic, and total inspiratory muscle work and mainly the efficiency of the inspiratory muscle work in still standing and in exercising dogs. In 180 experiments with nine mongrel dogs, respiratory and circulatory functions were recorded at rest and during a 1.5-mph walk on a treadmill belt which was inclined by 9 degrees. Each dog had been trained and surgically prepared before the series of experiments started. An electronic pressure transducer together with a fluid-filled catheter was attached to the ribs at the midthoracic level. Airflow was monitored with a pneumotachygraph attached to a face mask. Aortic flow was monitored with an electromagnetic flow transducer. Catheters in the atria, the ventricles, and the pulmonary and systemic vessels were used to determine hemodynamic parameters and blood gas levels. All recorded and all derived functions were evaluated by a dedicated computer. At rest, resistive inspiratory work (0.98 kg cm) was smaller than elastic work (1.36 kg cm). Elastic work did not change with exercise but resistive work increased to 1.46 kg.cm. The increase of total work from 2.33 kg.cm at rest to 2.79 kg.cm during exercise did not reach significance. Inspiratory power increased with exercise. Estimated diaphragmatic oxygen consumption increased from 2.23 ml/min at rest to 3.64 ml/min during exercise. It was 1.6 percent of the total oxygen consumption at rest and 1.7 percent of the total oxygen consumption during exercise. The estimated efficiency of the work of inspiratory muscles was 15.8 percent at rest and 25.8 percent during exercise. These values did not vary substantially between different animals.     

UROLITHIASIS IN AUSTRALIAN ABORIGINAL CHILDREN

Thirty-six Australian Aboriginal children with urolithiasis were reviewed. Males dominated the series. The age distribution ranged from 8 months to 12 years and nearly 70% were 2 years or younger. Thirty-five patients had upper tract stones. Ultrasound was diagnostic in 35 patients and was falsely negative in one. Dietary factors, dehydration and recurrent diarrhoea are incriminated in the aetiology, because ammonium urate and oxalate were the main constituents of the stones. Malformations of the urinary tract were rare and known metabolic disorders were not seen. Chemical dissolution of the stones was found to be a safe and effective adjuvant in the management of urate stones.     

Optimal daytime feeding regimen to prevent postprandial hypoglycemia in type 1 glycogen storage disease.

To determine the optimal daytime dietary regimen for type 1 glycogen storage disease (GSD), we used uncooked cornstarch (UCS) at a basal glucose production rate (GPR) in single and divided doses, with mixed meals at 0700 and 1700 h. This regimen was compared with a 1.5 times larger single dose of UCS at 0700 h, and with dextrose at GPR at 1200 h. Two-hour UCS loads (amount equal to GPR in 2 h) given with a mixed meal at 0700 h and 180 min later maintained mean blood glucose (BG) concentrations at greater than or equal to 4.2 mmol/L for 300 min. BG was significantly greater from 240 to 300 min compared with a single 4-h UCS load, and at 300 min compared with a single 6-h UCS load. Similar effects were noted when the divided UCS regimen was given with a mixed meal at 1700 h, but not when isoenergetic amounts of dextrose were given on the same schedules with a mixed meal at 1200 h. A daytime schedule of six UCS feedings (with the three main meals and 180 min later) at GPR maintains BG at concentrations that should minimize biochemical abnormalities and optimize clinical outcome in patients with GSD.     

Influence of acupuncture on physical performance capacity and haemodynamic parameters.

In a single blind study the question as to whether the needling of specific acupuncture points is able to produce an increase in physical performance capacity and better regulation of heart rate and blood pressure was examined. Thirty-six healthy young men were assigned at random to three groups, receiving either actual acupuncture, placebo acupuncture or no stimulation. Performance was determined by means of a spiro-ergometer test which was carried out at the beginning and at the end of five weeks of treatment consisting of one session per week. The subjects from the group which actually received acupuncture were able to increase maximum performance capacity significantly and also physical performance at the anaerobic threshold. This may be interpreted as a sign of functional improvement in haemodynamic and metabolic mechanisms. There was, on the whole, no noticeable effect produced by the placebo acupuncture. The control group, which received no stimulation, showed unfavourable changes in the values obtained compared with the results of the performance test at the commencement of the study.