Search PubMed⌕ Search

Biomedical subjects

J G Hall

Publications and source records attributed to J G Hall.

At least 91 records · Page 5Linked to original sources
PubMed1991

IgA antibodies in the bile of rats. V. Primacy of the GALT as a source of IgA.

In each of a series of rats the common bile duct and the thoracic duct (cisterna chyli) were cannulated so that both bile and thoracic duct lymph could be collected quantitatively for several hours. The concentrations of IgA in samples of lymph and bile were measured by radioimmunoassay so that the output of IgA per unit time could be calculated. Although the output of IgA in the lymph did not decline significantly, the output in the bile fell so that by 2 hr it had been reduced to less than 20% of the peak value. Similar experiments in rats which had been immunized actively by injecting antigens into the GALT showed a corresponding rapid decline in titres of specific biliary antibodies after fistulation of the thoracic duct. The low levels of IgA in the bile of rats that had been drained of thoracic duct lymph were restored quickly to normal values by the intravenous infusion of a volume of thoracic duct lymph equal to that which had been lost; this restoration was transient, and the concentration of IgA in the bile soon declined again after the infusion ceased.

Animals↗
PubMed1990

A quick method for obtaining lymph-borne dendritic macrophages from rats.

After conventional cannulation of the thoracic duct (cisterna chyli) of rats, the mesenteric nodes were frozen solid by the application of solid carbon dioxide and a metal probe that had been cooled to the temperature of liquid nitrogen. This procedure destroyed the functional integrity of the node so that peripheral intestinal lymph, rich in dendritic macrophages flowed, unaltered, into the thoracic duct. In this way peripheral intestinal lymph could be collected immediately and the time-consuming and expensive two-stage procedure of conventional surgical lymphadenectomy was avoided.

Animals↗
PubMed1990

Three-generation dominant transmission of the Silver-Russell syndrome.

We report on 7 patients with the Silver-Russell syndrome (SRS) in two 3-generation families. Three patients in each of the families had an undergrowth of the left side of the body when compared with the normal right side. The clinical courses were mild as compared to the severity sometimes described in sporadic cases. These patients and a review of 190 SRS cases from the literature showed that there were 23 families in which 38 patients had completely expressed SRS. In 17 of the families, multiple maternal relatives had complete or partial expressions of the SRS. Most SRS patients have been reported to occur sporadically; however, of the 197 propositi analyzed, 19% had more than one affected individual in a family and several different modes of inheritance could have been responsible. Two families (8.7%) had spontaneous dominant mutations (twins) and possible autosomal recessive transmission was present in 4 families (17.4%). Because no male-to-male transmission has yet been documented in the 21 families in the literature and the two families reported here, X-linked dominant inheritance is a possibility in 17 families (74%). Thus, although sporadic occurrences and genetic heterogeneity appear to be involved in the SRS, dominant inheritance may be a major causal factor.

Adult↗
PubMed1990

Familial renal hypophosphatemia, minor facial anomalies, intracerebral calcifications, and non-rachitic bone changes: apparently new syndrome?

We report on two brothers with renal hypophosphatemia, intracerebral calcifications, minor facial anomalies, and short distal phalanges. The children presented with recurrent dental abscesses; one had premature closure of the anterior fontanelle. Biochemical findings included hypophosphatemia and elevated serum alkaline phosphatase with normocalcemia. Blood levels of parathyroid hormone, 1,25(OH)2 and 25(OH) vitamin D levels were normal; TRP (the fractional tubular reabsorption of PO4) and TmP/GFR (the tubular maximum rate of PO4 reabsorption in relation to GFR) were low. Both parents had a normal serum phosphate and brain CT scan without evidence of calcifications. This apparently new syndrome of renal hypophosphatemia associated with intracerebral calcifications appears to be inherited as either an autosomal recessive or an X-linked trait.

Brain↗
PubMed1990

Apparent postnatal onset of some manifestations of the Wiedemann-Beckwith syndrome.

We report on 2 patients who were apparently normal at birth but later developed characteristics of Wiedemann-Beckwith syndrome (WBS). Both had hypoglycemia neonatally and gradually developed coarse facial changes, umbilical hernia, and macroglossia. Renal sonography done after the macroglossia developed showed large kidneys in both. The placentas were carefully examined in both cases but findings described as typical of WBS were only found in one. The clinical evolution of these infants suggests that some WBS manifestations may have their onset postnatally in some cases. We postulate that the cellular hyperplasia and hypertrophy characteristic of WBS may be caused by persistent rests of embryonal cells that secrete paracrine and/or endocrine growth factors.

Beckwith-Wiedemann Syndrome↗
PubMed1990

A sibship with Roberts/SC phocomelia syndrome.

We report on a family in which 3 sibs have Roberts/SC phocomelia syndrome. One child has survived to age 9 years. The clinical manifestations and the natural history of this syndrome are reviewed.

Abnormalities, Multiple↗
PubMed1990

Syndrome of mental retardation, facial anomalies, hypopituitarism, and distal arthrogryposis in sibs.

A brother and a sister presented with a malformation syndrome consisting of facial anomalies, distal arthrogryposis with camptodactyly of fingers and "hammer toes," severe mental retardation, and hypopituitarism. The girl is now 6 1/2 years old and exhibits severe mental retardation. She has abnormal secretion of growth hormone and responded to growth hormone therapy. Her brother was born with the same facial manifestations, distal contractures, and hypopituitarism. He died unexpectedly at age 3 months of no apparent cause. The occurrence of the syndrome in 2 sibs of different sex suggests autosomal recessive inheritance.

Abnormalities, Multiple↗
PubMed1990

Heavy drinking and its correlates in young men.

This study examined the drinking behavior of a sample of 98 college men and the relationship to drinking of a variety of subject variables. The subjects reported drinking an average of nearly eight days a month, about five drinks each time, and were intoxicated more than three times monthly. Nearly half reported having experienced two or more drinking-related adverse consequences within the past year and over a third were intoxicated four or more times monthly. Forty percent of the subjects could be described as problem drinkers. Illicit drug use and the disinhibition factor of the sensation seeking scale were the most consistent correlates of drinking behavior and its adverse consequences, although belonging to a fraternity, consuming alcohol/drugs before age 15, the Macandrew Alcoholism Scale score, and a family member having received alcoholism treatment were also found to be consistently associated with drinking in the subjects. Sociodemographic characteristics, physical health, mental health treatment, childhood behavior problems, adolescent antisocial behavior, and familial alcoholism were by and large not found to be related to drinking behavior. A stepwise multiple regression analysis revealed that five variables accounted for 51% of the total variance in drinking behavior. The significant predictors included a heavy drug use factor, a smoking factor, fraternity membership, drug/alcohol use before age 15, and having a family member who had received alcoholism treatment. Thus, four of the five significant predictor variables were reflective of drug use in the subject or his family. The findings underline the need for further prospective longitudinal research to understand the origins of problem drinking and its relationship to alcoholism.

Adolescent↗
PubMed1990

Congenital rubella syndrome associated with calcific epiphyseal stippling and peroxisomal dysfunction.

An infant girl had the clinical and immunologic findings of congenital rubella syndrome but also had arthrogryposis multiplex and calcific epiphyseal stippling. Spastic quadriparesis developed, and both physical and behavioral development were slow. Increased spasticity of the legs at 5 1/2 years was related not to progressive rubella encephalomyelopathy but to spinal cord compression by abnormal cartilaginous tissue. The presence of a peroxisomal disorder was demonstrated by a greatly increased level of phytanic acid and slightly increased levels of hexacosanoate in serum and by reduced activity of peroxisomal dihydroxyacetone phosphate acyltransferase and a slightly increased ratio of cytosolic to peroxisomal catalase activity in cultured fibroblasts. A reduction in the number and size of peroxisomes was demonstrated in cultured fibroblasts, and a needle biopsy specimen of the liver also showed the peroxisomes to have a smaller diameter than usual. We recommend that any child with epiphyseal stippling be assessed for peroxisomal disease and that the potential for spinal cord compression by dysplastic bone or cartilage be recognized. The association of peroxisomal dysfunction with congenital rubella has not been described previously. The interaction between rubella virus infection and peroxisomal function may need further investigation.

Calcinosis↗
PubMed1990

Turner syndrome and its variants.

Turner syndrome is suspected in females with short stature, gonadal dysgenesis, and lymphedema; however, there are no pathognomonic features of Turner syndrome, and the disorder should be considered in any girl with short stature or delayed puberty. This article discusses the natural history of Turner syndrome and complications that occur in various organ systems; it reviews the physical features and complications seen with various karyotypic changes in Turner syndrome. Age-specific screening and therapies are covered.

Adolescent↗
PubMed1990

Conformational analysis of cyproheptadine hydrochloride.

A nuclear magnetic resonance and theoretical study on the conformations and molecular flexibility of cyproheptadine hydrochloride (1) is reported. In the 1H NMR spectrum of 1 in CDCl3, two conformational forms are observed to occur in an approximate ratio of 1:4. In both forms, NOE and coupling constant measurements suggested that the terminal N-methyl group is equatorial. NOE experiments identified the more populated conformer (labeled D) as similar to the form seen in the X-ray crystal structure of cyproheptadine. The other form observed (A) may in principle be converted to D via either inversion of the central ring (T(inv)) or concerted nitrogen (N(inv)) and piperidine ring inversion (P(inv)). Chemical-exchange peaks in the 400-MHz 2D NOESY/chemical-exchange spectrum suggested that the latter mechanism is responsible for interconversion between the two forms. A theoretical study of the various interconversion processes using both molecular mechanics (MM2) and molecular orbital (AM1) approaches is also reported.

Chemical Phenomena↗
PubMed1990

2D 1H NMR studies of monomeric insulin.

In order to establish the conditions required for the observation of monomeric insulin in solution, a series of proton nuclear magnetic resonance studies of insulin in a variety of solvents was undertaken. Optimal spectra were recorded in trifluoroethanol- water mixtures in a 1:2 ratio. Using the sequential assignment approach the proton nuclear magnetic resonance spectrum of insulin was then assigned. Aspects of the structure of monomeric insulin in solution have been determined using the observed NOE cross peaks and slow exchange protons.

Animals↗