An unusual presentation in a patient with metachromatic leukodystrophy

A 2-year-old child was admitted with bilateral bronchopneumonia. During convalescence he sustained sudden respiratory arrest for which he required ventilatory support. He had undergone cholecystectomy 1 month prior to the above episode. The pathological examination of the gall bladder mass had revealed the features of metachromatic leukodystrophy. Gastro-oesophageal reflux, which is associated with this neurological disorder, was suspected to be the cause of this sudden respiratory arrest and its presence was confirmed by barium swallow examination.     

Effect of panel reactive antibody on live related donor kidney transplantation: Indian experience

Serum samples from 95 recipients, transplanted with kidneys from live related donors, were tested for the presence of panel reactive antibodies (PRA) in pre- and post-transplant serum samples by the extended microdroplet lymphocytotoxicity test. The immunoglobulin class of antibodies was tested by treatment of serum with dithiothreitol. A significant correlation was found between the high PRA found in the 75 pretransplant sera tested and the subsequent rejection episodes. In addition, the level of pretransplant PRA activity was associated with graft survival in that patients with low PRA had significantly superior graft survival than those with high PRA. Furthermore, the present data show that patients with historical high PRA, but current low PRA, had graft survival similar to that in recipients who had moderate PRA in their current sera. High PRA patients had more often a positive crossmatch than patients with low PRA. The PRA level was also associated with prolonged waiting period. Immunoglobulin class of antibodies was related to graft acceptance in that the presence of IgM antibodies was not detrimental to transplantation. The results in the present study suggest that PRA of < 10% is negligible, while more attention should be paid to patients with PRA > 10%.     

Coloplasty for congenital short colon

The radioprotective properties of 12 compounds of 9a-homo-13-thiaprostanoid series were investigated under gamma irradiation using the molecular model of beta-carotene radio-oxidation in oleic acid in vitro, erythrocyte radiomimetic model in ex vivo-in vitro system as well as in vivo radiation damage in mice. Most of these compounds stimulated the radio-oxidation of beta-carotene, however in this model two prostanoids with natural alpha-chain displayed radioprotective properties. Expressed membrane stabilizing effect of two 9a-homo-13-thiaprostanoid nor-analogues was revealed in radiomimetic model experiments. Two 10, 10-dimethyl-13-thiaprostanoids raised animal radioresistance during in vivo experiments.     

Glyceryl nonivamide: a capsaicin derivative with cardiac calcitonin gene-related peptide releasing, K+ channel opening and vasorelaxant properties

In this study, the aorta vasorelaxant, coronary calcitonin gene-related peptide (CGRP) releasing, and atrial contractility effects of glyceryl nonivamide (GLNVA) were investigated in guinea pigs. In the isolated thoracic aorta, although GLNVA (0.01-50 microM) concentration dependently induced endothelium-independent relaxations and relaxed phenylephrine-(1.0 microM) induced contractions, it failed to relax 80 mM KCI-induced contractions. The GLNVA (1.0 microM) relaxation response in the aorta was significantly inhibited by tetraethylammonium (2.5-10 mM) or ouabain (5.0 microM) and was attenuated by increased extracellular potassium gradient (10-30 mM). Glibenclamide (0.01-10 microM) dose dependently antagonized the GLNVA relaxant effect. In the isolated perfused guinea pig heart, GLNVA (0.1-10 microM) increased CGRP-like immunoreactivity outflow from coronary circulation in a concentration-dependent manner. In the isolated right and left guinea pig atria, GLNVA (0.01-10 microM) produced concentration-dependent positive inotropic and chronotropic effects, but these effects were inhibited by pretreatments with ruthenium red (1.0 microM), capsazepine (10 microM), human calcitonin-gene-related peptide (CGRP(8-37)) (1.0 microM) and sensory neuron denervation, respectively. Based on these findings, we suggest that CGRP may be released by GLNVA from cardiovascular sensory neuron, and it then activates CGRP receptors on the coronary artery and atrium. The GLNVA-induced vasorelaxant effect in the vascular smooth muscle of the aorta is due to CGRP release associated K+ channel opening, and this effect eliminates capsaicin-derived excitability-associated K+ channel blocking activities.     

Primary angioplasty for acute myocardial infarction

The comparative stability of eight different triplexes constituted with 16-mer pyrimidine-modified oligodeoxynucleotides (wild-type ODN, PS-ODN, alpha-ODN, or alpha-PS-ODN) or oligoribonucleotides (wild-type ORN, alpha-ORN, 4'-thio-ORN, or 2'-O-MeORN) and a DNA hairpin, termed H36, was studied in five different buffers by UV melting curve analysis. The composition of buffers varied in pH (5.5 and 6.5), in salt concentration (100 mM and 1 M Na+), and in the presence or absence of divalent cation (0 or 3 mM Mg2+) or spermine (0 or 1 mM). At pH 5.5, the eight triplexes are formed with Tm values ranging from 24.7 degrees C to 50.9 degrees C (delta G298K between -8.1 and -16.8 kcal/mol). At pH 6.5, the triplexes are less stable, and thus 4'-thio-ORN and PS-ODN showed broad transitions that did not allow us to conclude triplex formation. An increase of salt concentration or the presence of spermine stabilizes the triplexes, whereas Mg2+ has a destabilizing effect (excepted for alpha-ORN). In general ORN:H36 and 2'-O-MeORN:H36 triplexes were the most stable. Finally, introduction of alpha-anomeric nucleosides led to an alpha-ORN analog that showed low binding with H36 and to alpha-ODN and alpha-PS-ODN analogs. Triplexes formed with alpha-ODN were slightly less stable than those formed with unmodified ODN. Surprisingly, introduction of phosphorothioate in an alpha analog led only to a low destabilization.     

Influence of beta-adrenergic and vagal activity on the effect of exogenous adenosine on supraventricular tachycardia termination

Adenosine, which binds to cell surface receptors and couples with guanosine triphosphate-binding inhibitory proteins (G(i)), is potent in terminating supraventricular tachycardia (SVT). However, whether the differences in autonomic tone will influence this effect remains unknown. This study was designed to investigate the role of beta-adrenergic and vagal activity on the action of adenosine. Forty patients with clinically documented SVT (22 with atrioventricular node reentrant tachycardia and 18 with atrioventricular reciprocating tachycardia) were divided into 4 groups with 10 patients in each group. In groups 1 and 2, adenosine was intravenously injected during the baseline state and during infusion of isoproterenol (2 and 4 microg/min, respectively). Group 2 patients received atropine (0.04 mg/kg) injection before isoproterenol infusion. In groups 3 and 4, intravenous injection of adenosine was given during the baseline state and after injection of atropine (0.02 and 0.04 mg/kg, respectively). Group 4 patients received propranolol (0.2 mg/kg) before atropine injection. The minimal dose of adenosine to terminate tachycardia during isoproterenol infusion of 2 microg/min was greater than that during the baseline state in both groups 1 and 2. The minimal dose of adenosine during isoproterenol infusion with 4 microg/min was higher than that with 2 microg/min in group 2, but not in group 1 patients. In both groups 3 and 4, the minimal dose of adenosine required to terminate tachycardia during atropine injection with 0.02 mg/kg was greater than that during the baseline state. The minimal effective dose of adenosine during atropine injection with 0.04 mg/kg was higher than that with 0.02 mg/kg in group 4, but not in group 3 patients. In conclusion, either limb of the autonomic nervous system may modulate the adenosine dosage required for termination of SVT. Patients taking drugs such as beta blockers or vagolytic agents may need alterations in the dose of adenosine for therapy.     

Detection of serum transforming growth factor-alpha in patients of primary epithelial ovarian cancers by enzyme immunoassay

OBJECTIVE: To survey the social outcome of patients with schizophrenia attending State mental health facilities in southern Tasmania. METHOD: Using the Statewide Mental Health Register, patients using inpatient and outpatient facilities who received a diagnosis of schizophrenia between 1981 and 1988 were identified (n = 771), and demographic and illness measures, and admissions and length of inpatient stay were compiled. The Life Skills Profile (LSP) was completed by mental health personnel for the 247 who were regular attenders or inpatients in 1991. RESULTS: Social morbidity as indexed by the LSP was highest in psychiatric hospital inpatients and patients in long-term rehabilitation programs, and lower in patients attending community centres. The majority of patients in suburban settings and attending community centres lived with their families, whereas patients in the inner city or in the rehabilitation service were mainly in hostel accommodation or living alone. Patients with schizophrenia attending State services were of a similar age range but had a longer duration of illness and more admissions, and had spent more days in hospital than patients who were not in regular contact with the service. CONCLUSIONS: The distribution of social morbidity in schizophrenia confirms that the public health system is supporting a group with high social morbidity. Patients with the highest morbidity are receiving the highest levels of care and intervention.     

Changes in membrane-bound hydrolases by metronidazole in rat renal brush border

The antiprotozoal drug metronidazole, when administered orally at a dose level of 100 mg/kg body wt. daily for 7 days to rats, brought about significant elevation of renal brush-border-membrane-bound hydrolytic enzymes, such as alkaline phosphatase, maltase, sucrase, and leucine aminopeptidase (LAP). Kinetic analysis of the enzymes (substrate saturation) indicated that the drug produced an increase in the maximum of apparent initial enzyme velocity (Vmax), while the substrate affinity constant (Km) remained unaltered. These changes were not recovered to the normal level even after the drug regimen was stopped and the animals were allowed to recover for a period of 7 days. Lipid analysis of brush border membrane (BBM) revealed a significant elevation in the cholesterol, phospholipid, and ganglioside levels, while no marked change was recorded in triglyceride, free fatty acid and plasmalogen. Study of the temperature-dependent parameters of the enzymes showed that metronidazole induced a shift in the transition temperature (To) in LAP with nearly total reversibility in the recovery group. No such change was seen in the other enzymes. However, there also was a lowering in the energy of activation (Ea) below To, which returned to normal after the treatment was withdrawn.