Alagille syndrome.

Syndromic paucity of bile ducts or "Alagille syndrome" is characterized by peculiar facies, chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects and peripheral pulmonary artery hypoplasia or stenosis. We present a two-year-old female child with the 'partial' or 'incomplete' Alagille syndrome. The child had three of the five major features of the syndrome.

Peripheral blood leucocytes ornithine decarboxylase activity in chronic myeloid leukemia patients: prognostic and therapeutic implications.

Leukocytes ornithine decarboxylase (ODC) activity was measured in normal individuals and in patients with chronic myeloid leukemia (CML) in chronic phase (CML-CP) as well as in accelerated phase (CML-AP), with an aim to examine the role of ODC activity in prognostic evaluation of CML patients. Our results showed that ODC activity was significantly higher in CML-CP (41.02+/-25.

Estrogen receptors in rat bone: their interaction with estrogen receptor modulators.

Estrogen receptors (ER) were studied in rat bone cytosol using immunoprecipitation, and Western blot technique. Ligand specificity of bone ER was studied using various known modulators of ER. Competitive experiments were performed under exchange conditions in bone tissue obtained from one day old rats.

Cranial irradiation--an unusual cause for diabetes insipidus.

Central diabetes insipidus frequently occurs due to tumours in the region of pituitary or hypothalamus or following surgical trauma to these regions. Rarely it has been reported following cranial irradiation. We report the case of a middle aged woman who underwent surgical removal of a frontal capillary hemangioblastoma and received cranial irradiation.

Effect of light intensity on in vitro multiple shoot induction and regeneration of cotton (Gossypium hirsutum L. cv Khandawa-2).

Cotyledonary nodes taken alongwith shoot apex from seedlings of cotton (G. hirsutum) proliferated into shoots on nutrient agar medium supplemented with cytokinins. In the presence of optimal plant growth regulators, low light intensity enhanced the number of shoots initiated per explant in cotton.

Validation and utility of novel volume reduction technique for determination of parallel conductance.

The parallel conductance volume, created by the conductivity of structures surrounding the ventricular blood pool, can be estimated by using a saline dilution technique. This paper examines the use of a novel volume reduction method, during a standard vena caval preload reduction maneuver, as an alternative to the routinely used saline dilution method to calibrate conductance catheter measurements in the left (LV) and right ventricle (RV) of animals and humans. The serial reproducibility of both methods was examined by measurement of percent difference, and by assessing the coefficient of repeatability 1) between two measurements within the same subject, 2) between the two techniques, and 3) interobserver variability.

The delta-endotoxin proteins accumulate in Escherichia coli as a protein-DNA complex that can be dissociated by hydrophobic interaction chromatography.

The insecticidal protein CryIAc accumulated to form inclusion bodies in Escherichia coli upon overexpression of the cloned gene. The solubilized inclusion bodies contained the delta-endotoxin in association with DNA fragments of about 25 kb. The protein-DNA complex could be dissociated and the delta-endotoxin purified by hydrophobic interaction chromatography on phenyl-Sepharose.

Degradation and de novo synthesis of D1 protein and psbA transcript levels in Chlamydomonas reinhardtii during UV-B inactivation of photosynthesis and its reactivation.

UV-B induces intensity and time dependent inhibition of photosynthetic O2 evolution and PS II electron transport activity in Chlamydomonas reinhardtii. The D1 and D2 proteins of chloroplast membranes are rapidly and specifically degraded in the course of irradiation of cells to UV-B. Continuous synthesis of the two proteins was essential for the repair of damaged PS II as chloramphenicol accelerated UV-B inactivation of photosynthesis and prevented photoreactivation.

Impact of a Simple Inexpensive Quality Assurance Effort on Physician's Choice of Thrombolytic Agents and Door-to-Needle Time: Implication for Costs of Management.

The objective of this study were to assess the impact of a quality assurance effort on the door-to-needle time and the choice of thrombolytic agent for the management of acute myocardial infarction in the emergency department. The study design involved a prospective collection of data on a series of consecutive patients who received a thrombolytic agent for a presumed acute myocardial infarction. The study was carried out in the emergency department of a major university urban tertiary care center.

Iron overload in paediatrics undergoing cardiopulmonary bypass.

Pathological changes in iron status are known to occur during bypass and will be superimposed upon physiological abnormalities in iron distribution, characteristic of the neonatal period. We have sought to define the severity of iron overload in these patients. Plasma samples from 65 paediatric patients undergoing cardiopulmonary bypass (CPB) were analysed for non-haem iron, total iron binding capacity, transferrin and bleomycin-detectable iron.

Inhibition of nitric oxide synthesis improves left ventricular contractility in neonatal pigs late after cardiopulmonary bypass.

Background: Following neonatal open heart surgery a nadir occurs in left ventricular function six to 12 hours after cardiopulmonary bypass. Although initiated by intraoperative events, little is known about the mechanisms involved.

Objective: To evaluate the involvement of nitric oxide in this late phase dysfunction in piglets.

Acute right ventricular restrictive physiology after repair of tetralogy of Fallot: association with myocardial injury and oxidative stress.

Background: Acute right ventricular (RV) restrictive physiology after tetralogy of Fallot repair results in low cardiac output and a prolonged stay in the intensive care unit (ICU). However, its mechanism remains uncertain.

Methods And Results: In the first 24 hours after tetralogy of Fallot repair (n=11 patients), serial prospective measurements were performed of cardiac troponin T, indexes of NO production (NO(2)(-) and NO(3)(-) combined as NOx), and iron metabolism and antioxidants.

Antioxidant protection against iron toxicity: plasma changes during cardiopulmonary bypass in neonates, infants, and children.

Cardiopulmonary bypass surgery is associated with the release of low molecular mass iron, which increases the saturation of plasma transferrin to over 50% in all adult patients treated. In a significant minority, however plasma transferrin becomes 100% iron saturated and non-transferrin bound iron can be detected in the plasma. An iron-saturated transferrin is also a common physiological finding in normal term and pre-term infants at a time when their plasma antioxidants, which protect against iron toxicity and radical scavenging, are profoundly different from those seen in adults.

Modified ultrafiltration improves global left ventricular systolic function after open-heart surgery in infants and children.

Objectives: Modified ultrafiltration increases blood pressure and cardiac index following open-heart surgery in children, but it is unclear if this is secondary to an improvement in global left ventricular function. A previous report has suggested that left ventricular systolic function as assessed in a single chord is improved by ultrafiltration (Davies MJ, Nguyen K, Gaynor JW, Elliott MJ. Modified ultrafiltration improves left ventricular systolic function in infants after cardiopulmonary bypass.

Urgent homograft aortic root replacement for aortic root abscess in infants and children.

Objective: To assess the results of early homograft aortic root replacement in infants and children with an aortic root abscess.

Design: Descriptive study of all patients with an aortic root abscess during 1987-97, identified by retrospective review of the echocardiographic and surgical registries.

Setting: A tertiary referral centre.

Purification of alpha feto protein from human cord blood.

Alpha Fetoprotein (AFP) is a major serum protein in the developing fetus and is of clinical significance as it is an oncofetal protein being synthesized by fetal organs and malignant tumors. AFP is here used as a diagnostic marker for hepatic carcinomas. In view of structural homology and similarities in physico-chemical properties with serum albumin, the separation and purification of AFP has always been a problem.

The effect of changing excitation frequency on parallel conductance in different sized hearts.

Objective: An important component of the ventricular volume measured using the conductance catheter technique is due to parallel conductance (Vc), which results from the extension of the electric field beyond the ventricular blood pool. Parallel conductance volume is normally estimated using the saline dilution method (Vc(saline dilution)), in which the conductivity of blood in the ventricle is transiently increased by injection of hypertonic saline. A simpler alternative has been reported by Gawne et al.

Left ventricular dysfunction after open repair of simple congenital heart defects in infants and children: quantitation with the use of a conductance catheter immediately after bypass.

Objective: Quantification of myocardial injury after the simplest pediatric operations by load-independent indices of left ventricular function, using conductance and Mikro-Tip pressure catheters (Millar Instruments, Inc., Houston, Tex.) inserted through the left ventricular apex.