PubMed Health⌕ Search

Biomedical subjects

H Thom

Publications and source records attributed to H Thom.

At least 19 recordsLinked to original sources

3 alpha, 7 alpha, 12 alpha-trihydroxy-24-nor-5 beta-cholan-23-sulfonate: synthesis and suitability for the study of cholate transport.

In order to facilitate the study of transport processes of unconjugated C-24 bile salts, simple syntheses of 3 alpha, 7 alpha, 12 alpha-trihydroxy-24-nor-5 beta-cholan-23-sulfonate (norcholansulfonate) and 3 alpha, 7 alpha, 12 alpha-trihydroxy-24-nor-5 beta-[7 beta 5H] cholan-23-sulfonate were devised. The hydrophilic-hydrophobic properties of norcholansulfonate, as determined by its chromatographic behavior as well as by its partition between l-octanol and water, are more similar to those of cholyltaurine than to those of cholate. Self-association of norcholansulfonate in phosphate buffer, pH 7.4, with an ionic strength of 150 mM begins at a concentration of about 1 mM, comparable to that of cholyltaurine and cholate, as determined by spectral changes in fluorescence emissions of {N-[7-(4-nitrobenzo-2-oxa-1, 3-diazol)]-7b-amino-3a, 12a-dihydroxy-5b-cholan-24 - oyl}-2'-aminoethanesulfonate (7 beta-NBD-NCT). The apparent CMC value obtained from solubilization of the dye Orange OT, 8.5 mM, is comparable to that of cholytaurine. 7.5 mM, and lower than that of cholate, 9.5 mM. Norcholansulfonate is readily taken up by rat liver and completely excreted unmetabolized into bile with about the same secretion maximum (Tm) as cholyltaurine. Biliary excretion of norcholansulfonate is inhibited by cholyltaurine, and, vice versa, norcholansulfonate inhibits cholyltaurine secretion. Concerning metabolism and excretion, norcholansulfonate with the sulfonate group in the position where cholate has the carboxylate group should behave as an appropriate cholate analogue in mediated transport processes.

Animals↗

Comparison of IgG subclasses in foetal serum, maternal serum at delivery and milk in IgA-deficient and control women.

Immunoglobulin G subclass concentrations were measured in paired foetal (cord) and maternal serum specimens at delivery from 27 IgA-deficient (serum IgA < 0.01 g/l) and 15 control women. IgA-deficient women had significantly higher serum IgG1 and IgG3 concentrations than control women but 2 of the group had concomitant IgG2/IgG4 deficiency and a further 12 had low IgG4 concentrations (serum IgG4 < 0.025 g/l). Foetal serum also had significantly higher IgG1 concentrations than control foetal serum but lower IgG2 and IgG4 levels. Concentrations of IgG subclasses and IgM were measured in breast milk collected on the fifth day postpartum from 19 of these IgA-deficient and 18 control women. Between-group differences in IgG subclass levels resembled those in serum. Compared with serum, proportionally less IgG3 was present in milk in both groups although the contribution of IgG3 to total IgG was not less than that of IgG4. Slightly higher IgM was found in milk from the IgA-deficient mothers.

Case-Control Studies↗

IgG subclass concentrations and Gm allotypes in IgA-deficient and normal pregnant women.

IgG subclass concentrations were measured in the serum of 40 IgA-deficient (IgA concentration < 0.01 milligram) and 38 normal women in the second trimester of pregnancy. In the IgA-deficient group, 2 women were also deficient in IgG2 and IgG4, while a further 10 had very low concentrations of serum IgG4 only. These low IgG4 values were unrelated to the presence of anti-IgA antibodies. The incidence of low IgG4 values was significantly greater (p < 0.01) than in the control group where only 1 woman had low IgG4 and none had low IgG2. Mean values for total IgG, IgG1 and IgG3 were very significantly higher (p < 0.001) in the IgA-deficient group than in the control group. These differences were unrelated to Gm phenotype.

Female↗

Ammonia and carnitine concentrations in children treated with sodium valproate compared with other anticonvulsant drugs.

Plasma ammonia was measured in 59 children requiring anticonvulsant drugs: 37 children (group 1) on sodium valproate alone or in combination with other drugs and 22 children (group 2) on drugs other than sodium valproate. Plasma ammonia was higher in group 1 children. Total and free carnitine was measured in plasma and erythrocytes of all children and in the urine of 16 children from group 1 and eight from group 2. Plasma and erythrocyte free carnitine was significantly lower in the children on sodium valproate, along with a significant increase in the ratio of acyl (bound) carnitine to free carnitine. No significant correlation was found between plasma ammonia and carnitine concentrations for either group of children. Plasma and erythrocyte concentrations were not related. Urinary free carnitine was reduced in children treated with valproate, with a significant increase in the ratio of bound to free carnitine. Carnitine supplementation is discussed.

Adolescent↗

[Prevention of hip dislocation in children with spastic paralysis by using a specific therapeutic wheelchair].

The hip joints of children with spastic cerebral palsy, notably in those with more severe forms of paresis as well as those with bilateral or tetraparetic involvement, are in any case exposed to extraordinarily great risks. Totally inconspicuous at birth, constant dominance of the spastically contracting hip adductor and flexor muscles leads to gradually advancing malpositioning of the femoral heads, along with flattening of the hip socket and its eventual total destruction. Complete dislocation of the hip joints results, in very severely affected cases already at age 2-3, but in the majority of children only several years later. In this equally tragic as, on the other hand, almost invariably preventable maldevelopment, the wheelchair takes on a doubly crucial role. This, for one, has to do with the many hours the child has to spend in it every day. On account of the fact that the regular wheelchair, above all when "sportively styled", is forcing both legs, i.e. the thighs and lower legs as well as the feet, to adopt a strictly parallel position, it enhances, and accelerates, the highly undesirable development of spastic hip dislocation. If, however, the wheelchair is redesigned in an appropriate manner, i.e. provided with an abduction seating orthosis, along with laterally repositioned foot rests, it will in the majority of cases be possible to prevent the development of hip dislocation. Detailed guidelines to achieve this end are included, and instructions are given for adapting commercially available wheelchairs.

Adolescent↗

The influence of maternal glucose metabolism on fetal growth, development and morbidity in 917 singleton pregnancies in nondiabetic women.

To study the effects on the fetus of variations in maternal glucose tolerance, a 25 g rapid intravenous glucose tolerance test was performed at or about 32 weeks gestation in 917 randomly selected nondiabetic women with singleton pregnancies. The results were withheld from the patients and their obstetricians and paediatricians, and no treatment or advice was offered. Fasting plasma glucose and indices of glucose disposal (including a new index which we have termed "summed glucose") were distributed unimodally, with no evidence of a separate pathological group towards the diabetic end of the distributions. Significant associations were found between maternal glucose metabolism and various measures of neonatal nutrition and morbidity, including the incidence of congenital malformations and morbidity related to asphyxia, suggesting that variations within the normal range in maternal glucose metabolism can influence growth and development in the fetus. These relationships were continuous throughout the range of maternal glucose tolerance and were not of predictive value in individual cases.

Birth Weight↗

Abnormal endothelial factor VIII associated with pulmonary hypertension and congenital heart defects.

In patients with pulmonary hypertension associated with congenital heart defects, ultrastructural abnormalities are observed in endothelial cells, which suggest heightened metabolic function. If endothelial production of the von Willebrand factor (vWF) is increased, this may be associated with abnormal interactions with platelets leading to worsening of the pulmonary hypertension. We therefore evaluated vWF in 30 patients with pulmonary hypertension (25 with congenital heart defects) and in 30 individuals with normal pulmonary arterial pressure (12 with congenital heart defects). We measured the antigenic (vWF: Ag) and biologic (VWF: rist) activity of vWF in plasma and assessed endothelial vWF: Ag directly by an immunoperoxidase stain applied to lung biopsy tissue. Because of considerable variance and small size, the group of five patients with pulmonary hypertension and without congenital heart defects were excluded from statistical analyses. Patients with pulmonary hypertension and congenital heart defects had significant higher vWF: Ag levels than individuals with normal pulmonary arterial pressure without congenital heart defects (p less than .05), whereas values in those with normal pressure and congenital heart defects were intermediate. In lung biopsy tissue available from 29 patients in this study and from 11 others we previously reported, immunostain of pulmonary arterial endothelium for vWF was intense (suggesting increased production) in 29 of 32 with pulmonary hypertension and congenital heart defects and in only one of eight with normal pulmonary arterial pressure and congenital heart defects (p less than .01). Only three patients with congenital heart defects and pulmonary hypertension and increased vWF: Ag, however, had increased vWF: rist. Compatible with this discrepancy was a loss of vWF high-molecular weight forms as determined by both crossed immunoelectrophoresis and multimeric analysis. Our results suggest that increased vWF in most patients with congenital heart defects and pulmonary hypertension is associated with increased production of a biologically deficient molecule lacking high-molecular weight forms.

Adolescent↗

Maternal immunoglobulin allotype (Gm and Km) and neonatal group B streptococcal infection.

Gm and Km(1) allotypes in 37 mothers of neonates with severe Group B streptococcal (GBS) infection were compared with 115 mothers of non-infected infants, 36 of whom were known to be colonized with GBS. Deficits in G1m(1) and Km(1), and an increased incidence of G2m(23), were found in mothers of infected infants. Km(1) was associated mainly with the phenotype Gm(1, (2), 3, 17; 23; 5, 10, 11, 21) in mothers of infected infants while being uniformly distributed in mothers of non-infected infants. This study would seem, therefore, to support reports of Gm and Km(1) allotype involvement in maternal response to GBS infection and immunity in the new-born.

Female↗

Selective vitamin B12 malabsorption without anaemia but with profound failure to thrive.

A 7-month-old boy presented with vomiting and failure to thrive associated with proteinuria, methylmalonic aciduria and macrocytosis, but without anaemia. Plasma vitamin B12 levels were normal by a radio-dilution method. He was treated as an inborn error of metabolism with intramuscular cyanocobalamin and a low protein diet. However when treatment was withdrawn he remained well for 11 months before relapsing with vomiting and weight loss. Re-investigation again showed methylmalonic aciduria, but the haemoglobin was low and plasma vitamin B12 levels by a specific method showed them to be reduced. Studies of vitamin B12 absorption showed the picture of selective malabsorption. He was started on regular cyanocobalamin injections.

Child, Preschool↗

The effect of a glucose polymer mixture (caloreen) on stool composition in normal neonates.

Stool composition was studied in 10 newborn infants following (a) a modified milk formula (Cow & Gate Premium) and (b) a high energy formula [104 kcal (435 kJ) per 100 ml] in which the additional energy was provided in the form of 10 per cent Caloreen. The only significant difference in stool composition in the Caloreen-fed babies was a lower sodium concentration. The results suggest that the high energy diet did not produce diarrhoea or other changes in the stool composition which might have an adverse effect on the infant.

Carbohydrates↗

Maternal serum alpha-fetoprotein levels in low birth weight singleton pregnancies.

Maternal serum alpha fetoprotein (MSAFP) measurement between 16 and 21 weeks gestation is used to define a group of women with an increased risk of fetal abnormality, particularly open neural tube defect. The test is strongly gestation dependent and women with high MSAFP levels require sonar scan examination to define gestation, exclude twins and examine the fetus for obvious malformation or death. It has been reported that women with no primary cause for raised MSAFP have an increased incidence of low birth weight babies. Conflicting reports have separately ascribed these to premature delivery and to intra-uterine growth retardation. We have studied the relationship between MSAFP and low birth weight infants with respect to both prematurity and retarded fetal growth. MSAFP values were expressed as multiples of the appropriate weekly median (MOM) values relating to normal pregnancies with normal outcomes at term. For our normal population an MSAFP value of 2 MOM is the 95% centile, i.e. 5% of normal outcome pregnancies of sure gestation will have MSAFP values in the second trimester which are at or above 2 MOM. Information was available on 389 women whose infants were liveborn singletons weighing 2.5 kg or less. 33 (8.5%) of these women had MSAFP greater than 2 MOM (p less than 0.005) and of the 145 women whose babies weighed less than 2 kg, 17 (11.7%) had MSAFP at this level (p less than 0.001) Tab. I).(ABSTRACT TRUNCATED AT 250 WORDS)

Abortion, Spontaneous↗

Categorization of pediatric neoplasms by immunostaining with antiprekeratin and antivimentin antisera.

Forty-six tumors in children were examined using light microscopy and subsequently frozen sections were stained with antiprekeratin and antivimentin antisera, so that the tumors could be classified by tissue of origin. Except for two adrenal cortical carcinomas and four liver tumors, most epithelial neoplasms continued to produce prekeratin filaments, a characteristic of normal epithelial cells. Tumors and cells of epithelial origin did not produce vimentin filaments, whereas normal and neoplastic mesenchymal cells did. Tumors with both epithelial and mesenchymal components produced vimentin filaments in mesenchymal areas and prekeratin in epithelial areas. Tumors of lymphoid origin showed variable production of vimentin filaments, depending on the amount of cell cytoplasm, but did not contain prekeratin filaments. Of the neuroectodermal tumors, only the ganglioneuroma contained vimentin filaments and none contained prekeratin filaments. Thus, antibodies to both prekeratin and vimentin filaments are useful in diagnosing childhood neoplasms and studying their histogenesis.

Child↗

Role of antibody to S100 protein in diagnostic pathology.

Normal tissues and various tumors were examined for S100 protein, using anti-S100 protein antiserum, in an immunoperoxidase reaction. Among normal tissues, in addition to the previously reported presence of S100 protein in some neurons, glial, and Schwann cells of the nervous system, melanocytes and Langerhans cells of the skin, interdigitating reticulum cells of lymph nodes, and chondrocytes, we demonstrated it in myoepithelial cells and ducts of sweat glands, salivary glands, and the breast, serous glands of the lung, fetal neuroblasts, and sustentacular cells of the adrenal medulla. Among neoplasms, S100 protein previously has been reported in neurogenic tumors, melanomas, and neuroblastomas; we have demonstrated it in mixed sweat gland tumors, histiocytosis X, pleomorphic adenomas of the salivary gland, medullary carcinomas of the breast, bronchioloalveolar carcinomas of the lung, sustentacular cells of pheochromocytomas, teratomas of the ovary, and tumors of cartilage (enchondromas, osteochondromas, and chondrosarcomas). With S100 protein producing tumors, a normal progenitor cell was identified, indicating that demonstration of S100 protein in tumors confirmed their origin.

Carcinoma↗

[Physical therapy of post-traumatic ankyloses].

Within the scope of physiotherapy as well as ergotherapy, we dispose of an extraordinary multitude of partly very different therapy methods for prophylaxis and physical treatment of ankyloses and soft tissue contractures. Due to local or anatomic particularities of the individual joints, it is often necessary to change our therapeutic measures in an adequate manner. All therapy methods have to be exactly indicated. They can be applied alone or in combination. In order to avoid therapy failures and to achieve the most favorable result, the treatment has to be adapted at the right moment, with a correct dose, and in a strictly individual manner.

Ankylosis↗

Unexplained diarrhoea and failure to thrive in 2 siblings with unusual facies and abnormal scalp hair shafts: a new syndrome.

A family is described in which 2 siblings born to healthy parents presented with abnormal facies, persistent diarrhoea, and early death. Exhaustive pathological and biochemical investigations failed to find a cause. The scalp hair of both babies had an abnormal amino-acid composition, and presented an appearance that was unique on scanning electron microscopical examination; this fact and the clinical picture probably represents a new syndrome.

Amino Acids↗