The HGAR1 familial hypercholesterolemia pedigree

Data on 232 members of a single pedigree, descended from two pairs of original parents, were made available to the participants of Genetic Analysis Workshop 8 (GAW8). In addition to information concerning age and sex, measurements for 10 quantitative traits and genotypes at 22 polymorphic marker loci were also provided for a subset of 193 of these family members. © 1993 Wiley-Liss, Inc.     

Sutureless bowel anastomosis using Nd:YAG laser

Small bowel anastomoses were performed without sutures by using the Nd:YAG laser to produce welded enterotomies. Optimal energy levels for contact and noncontact laser were determined. Anastomoses produced using five target energy levels between 100 and 500 J were examined. Short-term anastomotic strength of these enterotomies was measured 1 min after the welding. Bursting pressure of the laser welded enterotomies was compared to the bursting pressure of traditional two-layer, inverting, interrupted sutured bowel anastomoses. The overall mean bursting pressure of non-contact-welded enterotomies was 50.6 mmHg. Optimal laser settings determined in this initial phase were then used to produce anastomoses in rabbits which are recovered postoperatively for 1 or 2 weeks in order to examine long-term viability and integrity of the anastomoses. All chronic rabbit preparations recovered without complication. The mean bursting pressure was 200 mmHg, not significantly different from that of uncut bowel or two-layer sutured anastomosis.     

Peripheral T-cell lymphoma in childhood. A clinicopathological study of six cases

A review of the pathological material from 42 children with non-Hodgkin's lymphoma seen over a 44 month period revealed 10 large cell tumours. Of these, six were classified as peripheral T-cell lymphoma, an entity rarely reported in childhood. Three patients were boys and three girls (median age 9.5 years), and extranodal presentation was a feature of two patients. Five had high-grade tumours; of these, three were classified as large cell anaplastic, Ki-1 positive and two as pleomorphic large cell. The remaining patient had a low-grade tumour of angioimmunoblastic type. T-cell subsets were examined in three cases and showed the following phenotypes: CD4-, CD8-; CD4+, CD8-; CD4-, CD8+. Three of the patients with high-grade tumours died, with a mean survival of 22 weeks. The remaining patients are alive and clinically disease-free for between 10 and 24 months after treatment.     

Comparison of airway pressure-triggered and airflow-triggered ventilation in very immature infants

Failure of patient-triggered ventilation in very immature infants may be due to the use of inappropriate triggering systems. Two types of airflow trigger were therefore compared consecutively to an airway pressure (SLE) triggering system. Each comparison was made in 10 infants, ≤28 weeks of gestation. Comparison was made of the delivered volume, trigger performance and blood gases using each system for 1 h. Both comparisons showed that the airflow triggering systems performed better: one (Draeger Babylog 8000) had a higher sensitivity ( p < 0:01) and the other (Bird VIP airflow trigger), in which inflation was terminated by sensing a reduction in inspiratory flow, had a lower degree of asynchrony ( p < 0:01) and a tendency to deliver higher volumes. These results suggest that triggering systems sensing airflow changes may be superior to those sensing airway pressure changes in very immature infants. The use of a mechanism to synchronize the termination of inflation to the end of the patient's inspiration may offer further advantages.     

THE EFFECT OF HYPERTENSIVE EPISODES AND CARDIAC HYPERTROPHY ON THE CANINE CARDIAC BAROREFLEX

1. Left ventricular (LV) hypertrophy has been implicated in the reduction of baroreflex sensitivity present in hypertension. The aim of the current study was to investigate the mean arterial pressure-heart rate reflex (MAP-HR) in a model which induced left ventricular hypertrophy but no sustained blood pressure elevation. 2. Five mongrel dogs were exposed to transient blood pressure elevation of between 20 and 30 mmHg, through hindlimb compression using a pneumatic pressure suit, for 7 h per day, 6 days per week for 6 weeks. Resting blood pressure was not altered by the 6 week hindlimb compression intervention. 3. Echocardiographically determined LV mass (mean ± s.e.m.) was 116.0 ± 7.4 g prior to hindlimb compression (baseline) and elevated to 125.4 ± 8.1 g (P= 0.003) after 6 weeks of compression. A reduction in the early (E) to late (A) transmitral diastolic flow ratio (E/A) from 1.80 ± 0.06 at baseline to 1.54 ± 0.09 (P = 0.037) after the 6 week intervention suggested that cardiac compliance was reduced. 4. The maximum gain of the MAP-HR reflex, studied using the ‘steady-state’ drug technique, when blood pressure was normal, showed a trend for reduction from 3.85 ± 0.43 beats/min per mmHg at baseline to 3.10 ± 0.45 beats/min per mmHg (P= 0.067) after 6 weeks of compression. This gain reduction became significant after β-adrenoceptor blockade with propranolol (3.13 ± 0.55 vs 2.32 ± 0.25 beats/min per mmHg; P= 0.039). Covariant analysis showed a significant inverse correlation between LV mass and maximum gain (r= 0.96; P<0.001) during the 6 week compression period. 5. The MAP-HR reflex changes in this model mimic those present in hypertension and implicate cardiac hypertrophy as one possible mediator.     

Continuous recording of urethral activity in healthy female volunteers

The pressure variations at the maximal urethral closure pressure (MUCP) were continuously recorded in healthy female volunteers by means of a two-point microtip transducer catheter for one hour. Before the investigation a normal voiding was assured objectively and bladder instability was excluded. All women showed pressure variations both at the MUCP and more distally. The pressure variations, from 3 to 66 cm H₂O, showed rhythmicity and three frequency ranges could be identified. Slow pressure waves with a frequency of one in eight to 19 minutes were observed. Relatively fast-pressure waves were observed (one every one to four minutes) and relatively fast-frequency pressure waves were observed (rate: one to eight per minute). The pressure variations of the urethra seem to be an aspect of normal urethral physiology possibly contributing to continence and urinary tract infection prevention.     

Effect of Central Muscarine Receptor Blockade with DKJ-21 on the Blood Pressure and Heart Rate in Stress-Induced Hypertensive Rats

Da-Nian Zhu, Long-Mei Xue, Peng Li. Effect of central muscarine receptor blockade. with DKJ-21 on the blood pressure and heart rote in stress-induced hypertensive rats.

The experiments were performed on Wistar or Sprague-Dawley rats of both sexes divided at random into stress and control groups. The rats in the stress groups were put into cages and subjected to electric foot-shocks and noises for 9-15 days, which caused an increase in blood pressure (BP) and heart rate (HR). In hypertensive rats DKJ-21 (4mg/lml) was injected intravenously (i.v.), and 0.5-1.0h after administration the BP and HR dropped from the high level to normotensive level. In normotensive rats, however, administration of DKJ-21 had no effect on BP or HR. In separate groups of normotensive rats, pretreatment of DKJ-21 (4 mg/l ml, i.v.) blocked the pressor and tachycardiac effect induced by microinjection of physostigmine (0.4μg/0.1 /μl/site), corticosterone (40μg/0.1μl/site) or aldosterone (40 μg/0.1 μl/site) into the rostral ventrolateral medulla (rVLM). Furthermore, DKJ-21 also attenuated the enhancement of the pressor response to stimulation of the defense area in the midbrain, which was induced by microinjection of drugs (mentioned above) into the rVLM. These results indicate that i.v. DKJ-21 can selectively block the muscarinic receptors in the rVLM in stress-induced hypertensive rats, which suggests that abnormal enhancement of cholinergic mechanism in the rVLM may be related to hypertensive effects of corticoids in this area.     

Hereditary Hypertriglyceridemic Rat: A New Animal Model of Metabolic Alterations in Hypertension

Hereditary hypertriglyceridemic rats (hHTg) were developed as a new genetic model for the study of relationships between blood pressure (BP) and metabolic abnormalities. This strain has been produced by selective inbreeding from Wistar rats according to the rise of plasma triglycerides induced by a high-sucrose diet. Though hHTg rats display hypertriglyceridemia, impaired glucose tolerrance, hyperinsulinemia, insulin resistance and increased BP even without nutritional stimuli, high sucrose feeding further aggravates these symptoms. High plasma triglycerides levels in hHTg rats seem to be a consequence of their hyperproduction. Impaired insulin action is responsible for the defective glucoregulation in this strain. The loss of insulin responsiveness might be due to a reduction in the number of glucose transporters. Highly significant relationships among plasma triglycerides, ouabain-resistant Na⁺ transport and BP were demonstrated in the hHTg rats. Segregating populations (F2 hybrids) should be used for genetic analysis of the primary role of lipid and/or ion transport abnormalities in the pathogenesis of this form of genetic hypertension.     

Remarkable correlation between increased HLA-DQ antigen positive monocytes and prognosis of renal transplantation

Since cyclosporin A (CsA), a widely used immunosuppressive drug, strongly suppresses interleukin-2 (IL-2) secretion, it is frequently difficult to estimate T lymphocyte activation in early acute rejection. We found that, when evaluated based on HLA-DQ antigen expression, monocyte activation in the peripheral blood of renal transplantation patients was a very sharp parameter in diagosing acute rejection. All of 16 episodes of early acute rejection, which were relatively easily suppressed by steroid pulse therapy, showed a sharp increase in the proportion of HLA-DQ antigen-positive monocytes (DQ⁺ mono) and a quick return of DQ⁺ mono to previous values, along with a fall in serum creatinine levels. Since, however, HLA-DR antigen-positive T lymphocytes (DR ⁺T) were markedly increased over a long period in episodes of therapy-resistant and chronic rejection, their prolonged high value was regarded as a parameter indicative of poor prognosis.