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Biomedical subjects

Z Spirer

Publications and source records attributed to Z Spirer.

At least 73 records · Page 4Linked to original sources
PubMed1985

Retinol transport proteins and concentrations in human amniotic fluid, placenta, and fetal and maternal sera.

1. The proteins binding retinol, and retinol concentrations, were determined in amniotic fluid, placental cytosol and in the fetal and maternal circulation. 2. In non-pregnant women, plasma retinol was almost exclusively found in a transthyretin-retinol-binding-protein complex whereas, in pregnant women, retinol-binding-protein-bound retinol was observed not complexed to transthyretin. This latter fraction increased in concentration with fetal age. These two fractions were the major retinol-protein complexes in amniotic fluid and their relative amounts changed with progress of gestation. 3. In fetal blood both of these fractions were again found, with higher proportions of retinol-binding-protein-bound retinol in the umbilical artery than in the umbilical vein.

Amniotic Fluid↗
PubMed1985

Association of Gaucher's disease and lymphoid malignancy in 2 children.

2 cases of acute lymphoblastic leukaemia (ALL) of childhood in association with Gaucher's Disease (GD) are presented, which are the first to be described. One of them is a common non-B non-T cell ALL and the other is at T-cell ALL. The association of GD with benign and malignant B-cell proliferation has recently been described. It is suggested that chronic stimulation of the immune system by the accumulated glucocerebroside may predispose to B cell proliferation.

B-Lymphocytes↗
PubMed1985

Pseudohypoaldosteronism in a female infant and her family: diversity of clinical expression and mode of inheritance.

Pseudohypoaldosteronism was diagnosed in an infant that clinically presented severe failure to thrive and vomiting. Evaluation of her extended family revealed many other affected family members with a vast range of clinical expression. The mode of inheritance is most likely autosomal dominant. Salt supplementation during infancy was effective in restoring normal growth, weight gain and serum electrolytes.

Adult↗
PubMed1985

Does 1,25-dihydroxyvitamin D participate in the regulation of hormone release from endocrine glands?

The presence of receptors for 1,25-dihydroxyvitamin D3 in the pituitary, pancreas, testis, and ovary has raised the question of a possible direct role for 1,25-dihydroxyvitamin D (1,25(OH)2D) in the regulation of hormone synthesis and secretion. To evaluate this problem, six children with the syndrome of resistance to 1,25(OH)2D with rickets and alopecia underwent dynamic tests of insulin, TSH, PRL, GH, and testosterone secretion. Oral glucose loading resulted in normal glucose curves, subnormal peak insulin responses of 12-20 microU/ml in three hypocalcemic patients, and normal peak serum insulin values of 30-40 microU/ml in two normocalcemic patients. Basal serum, TSH, PRL, T4, and T3 concentrations were normal in all patients. Peak serum TSH values after TRH were 11-17 and 16-32 microU/ml in the hypo- and normocalcemic patients, respectively. The PRL response to TRH stimulation in either hypocalcemic or normocalcemic patients was normal [mean 26.2 +/- 5.1 (SD) ng/ml]. Peak serum GH levels were greater than 8 ng/ml in all five patients studied after one or more of the various stimuli. Serum testosterone concentrations after hCG stimulation were normal in the three patients studied (4.1-8.0 ng/ml). Thus, in children with resistance to 1,25(OH)2D, we could find no significant abnormalities in hormone secretion from the pituitary, pancreas, and testis apart from those presumably due to the hypocalcemia itself.

Calcitriol↗
PubMed1985

Decreased prolactin secretion by decidual tissue of pre-eclampsia in vitro.

In vitro prolactin (hPrl) secretion by explants of decidual tissue was studied in pre-eclampsia and normal controls. Our results indicate diminished hPrl production by decidua of pre-eclampsia as compared to the normal controls. Incubation of normal decidual tissue in the presence of serum obtained from pre-eclamptic patients did not induce an inhibitory effect on hPrl production.

Culture Media↗
PubMed1985

Interleukin-2 production by cord blood lymphocytes stimulated with mitogen and in the mixed leukocyte culture.

We examined the ability of cord blood lymphocytes to produce interleukin-2 (T cell growth factor) in response to phytohemagglutinin and in the mixed leukocyte culture. Interleukin-2 production was measured by the proliferative response of an interleukin-2-dependent mouse T cell line to the addition of supernatant obtained from cord blood lymphocyte cultures. Using these assays we have shown that cord blood lymphocytes have a normal ability to produce interleukin-2 in both PHA-stimulated cultures and in the mixed leukocyte culture. The normal production of interleukin-2 by cord blood cells indicates, that newborn T lymphocytes are mature, and that a dissociation exists between their normal ability to produce interleukin-2 and their ability to produce other lymphokines, which was reported to be impaired. The reported deficiency of HLA-DR antigen expression on newborn monocytes does not seem to interfere with the production of interleukin-2 in the mixed leukocyte culture.

Fetal Blood↗
PubMed1985

Effect of alpha-methyldopa excreted in human milk on the breast-fed infant.

A nursing infant whose mother took alpha-methyldopa (alpha-MD) was followed for 3 months. Analysis of maternal serum and milk as well as the infant's serum and urine for alpha-MD revealed that the drug was excreted into maternal milk, absorbed by the infant and excreted in her urine, but no adverse clinical effects were noted during the follow-up period. alpha-MD is excreted in human milk in concentrations that probably do not harm the breast-fed infant.

Adult↗
PubMed1985

The immune system in isolated IgA deficiency.

The immune systems were compared in 23 subjects with isolated IgA deficiency and 15 controls with normal levels of IgA. The IgA deficient group had higher levels of serum IgM, and lower stimulation indices for the mitogens Con A, PWM and PHA; only the index for PHA was statistically significant. Their suppressor T-cell activity was decreased and chemotactic activity significantly decreased. They also exhibited a decrease in Leu 3, and a significantly lower ratio of Leu 3 to Leu 2. The mean percent of positive NBT neutrophils was decreased. An imbalance in the immunoregulatory mechanism is suggested as a possible explanation for IgA deficiency.

Adult↗
PubMed1984

Rapid diagnosis of congenital adrenal hyperplasia by high performance liquid chromatography.

This manuscript describes methods for the quantitation of serum concentrations of 17-hydroxyprogesterone and 11-deoxycortisol (Compound S) employing reverse-phase high-performance liquid chromatography (HPLC) and ultraviolet (UV) absorbance which are applicable to the diagnosis of congenital adrenal hyperplasia due to deficiencies of 21- and 11-hydroxylase activities, respectively. These methods are simple, specific, precise and rapid. Data obtained by the HPLC-UV methods are highly correlated (p less than 0.001) with radioimmunoassay measurements.

17-Hydroxycorticosteroids↗
PubMed1984

Lesch-Nyhan syndrome: biochemical characterization of a case with attenuated behavioral manifestation.

The activity and kinetic properties of hypoxanthine-guanine phosphoribosyltransferase (HGPRT) were studied in fibroblasts from a Lesch-Nyhan syndrome (LNS) variant, with complete HGPRT deficiency in hemolysates, but with attenuated behavioral manifestation of the syndrome. The mutant HGPRT exhibited a 100-fold increase in Km for substrate phosphoribosyl-pyrophosphate, manifest in markedly decreased enzyme activity, being 2.5% of normal in cell extracts and about 0.6% of normal in intact cells. This degree of residual activity of the mutant enzyme is within the range found in patients with classical LNS.

Adult↗
PubMed1984

Interleukin-2 production and response to exogenous interleukin-2 in a patient with the acquired immune deficiency syndrome (AIDS).

The ability of lymphocytes from a patient suffering from the acquired immune deficiency syndrome (AIDS) to produce interleukin-2 (IL-2) was found to be comparable to that of his healthy sex partner and to that of a normal control. Addition of exogenous IL-2 to lymphocyte cultures did not improve the poor mitogen response to phytohaemagglutinin in this patient. Our data suggest that the underlying defect in this AIDS patient is due to an IL-2 receptor defect.

Acquired Immunodeficiency Syndrome↗
PubMed1983

Campylobacter enteritis in normal and immunodeficient children.

Campylobacter fetus subspecies jejuni (CBJ) has been recently recognized as a common pathogen in bacterial gastroenteritis in children. During a period of 16 months, 51 cases of C fetus subspecies jejuni gastroenteritis were diagnosed. Five of the children in whom the cases were diagnosed were previously known to be immunodeficient: two had X-linked agammaglobulinemia, one had agammaglobulinemia, one had combined immunodeficiency, and one had transient hypogammaglobulinemia. Average duration of fever and diarrhea was longer in the five immunodeficient children (15 and 23 days, respectively) compared with the normal children (four and five days, respectively). Excretion of C fetus subspecies jejuni in stool persisted for 20 to 27 days in four of the immunodeficient children and for one year in the fifth, whereas normal children excreted C fetus subspecies jejuni for only four to 16 days. Campylobacter fetus subspecies jejuni may be added to the list of bacterial pathogens most likely to infect immunodeficient children, especially those with a defect of the humoral system.

Campylobacter Infections↗
PubMed1983

Mucocutaneous lymph node syndrome (Kawasaki dIsease) in Israel. A review of 13 cases: is pseudomonas infection responsible?

In this report the clinical, laboratory and histopathological findings of 13 children with Mucocutaneous Lymph Node Syndrome (Kawasaki disease; MCLS) are reviewed. This is the first report of a series with a description of the clinical findings as well as pathological findings in patients from Israel. Pseudomonas infection appeared to be the underlying cause of both the clinical symptoms and the pathological changes of some (or all) cases of Kawasaki disease.

Adolescent↗