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Biomedical subjects

H Baker

Publications and source records attributed to H Baker.

At least 181 records · Page 10Linked to original sources
PubMed1986

Electrophysiologic features of abetalipoproteinemia: functional consequences of vitamin E deficiency.

We performed electrophysiologic testing in 10 patients with abetalipoproteinemia (ABL). Peripheral nerve studies implied an axonal disorder. Visual evoked potentials demonstrated prolonged P100 latency in three patients and abnormal electroretinograms in six. Somatosensory evoked potentials indicated dorsal column dysfunction in eight patients. Brainstem auditory evoked potentials were normal. Findings were consistent with the known neuropathology of ABL and of experimental vitamin E deficiency. Stabilization or improvement in electrophysiologic findings occurred with vitamin E supplementation. Neurophysiologic tests document retinal, central somatosensory and peripheral nerve lesions in vitamin E deficiency and provide an objective indication of response to treatment.

Abetalipoproteinemia↗
PubMed1986

Blood vitamin and choline concentrations in healthy domestic cats, dogs, and horses.

Blood concentrations of thiamin, biotin, nicotinates, pantothenates, folates, riboflavin, vitamins A, B6, B12, C, E, beta-carotene and choline were analyzed in healthy animals (23 horses, 25 dogs, and 29 cats). B-Complex vitamins and choline also were analyzed in the liver of the dogs and cats. Vitamin concentrations in the blood and livers of dogs were similar; however, blood vitamin A and beta-carotene concentrations were lower in the cat than in the dog. Horses had a higher B12 blood concentration than did the dogs and cats. These data can be useful for detecting overt and hidden vitamin deficits in these species due to various conditions.

Animals↗
PubMed1985

Strain differences in pituitary prolactin content: relationship to number of hypothalamic dopamine neurons.

BALB/cJ mice have more tuberoinfundibular dopamine neurons, and thus greater tyrosine hydroxylase activity, than CBA/J mice. Strain differences in the synthesis and release of prolactin would also be predicted since dopamine released from the tuberoinfundibular neurons is the prolactin inhibitory factor which plays a role in the regulation of both prolactin synthesis and release. As expected, CBA/J mice, with fewer dopamine neurons, synthesized and released significantly more prolactin than BALB/cJ mice; that is, both pituitary and serum prolactin concentrations were greater in CBA/J mice. To determine if there were more cells containing prolactin or more prolactin per cell, pituitaries were stained with antibodies to prolactin and densitometric analysis made of both the average staining per unit area and total staining per pituitary. For both indices CBA/J mice had more staining than BALB/cJ mice. Using these criteria the difference in staining was attributed to more prolactin-stained lactotrophs in the CBA/J strain. Although no differences in the number of acidophils demonstrated by Pearse Trichrome method were observed, acidophils from BALB/cJ mice appeared smaller and contained less cytoplasm than those from CBA/J mice. We conclude that strain differences in the number of tuberoinfundibular dopamine neurons are inversely related to the number of immunocytochemically demonstrable prolactin-containing cells in the anterior pituitary.

Animals↗
PubMed1985

Biotin deficiency complicating parenteral alimentation: diagnosis, metabolic repercussions, and treatment.

Biotin deficiency associated with total parenteral nutrition is an emerging clinical problem; criteria for diagnosis and dosage for treatment are unclear. We have diagnosed and successfully treated biotin deficiency in three patients. Each patient had alopecia totalis, hypotonia, and developmental delay. Two developed the characteristic scaly periorificial dermatitis; one had only an intermittent scaly rash on the cheeks and occipital scalp. Zinc and essential fatty acid supplements were adequate; serum zinc levels and triene/tetraene ratios confirmed sufficiency of these nutrients. None of the patients received biotin prior to diagnosis, and each had decreased excretion of urinary biotin and increased urinary excretion of organic acids diagnostic of deficiency of two biotin-dependent enzymes (methylcrotonyl-coenzyme A carboxylase and priopionyl-coenzyme A carboxylase). Only one patient had a plasma biotin concentration below the normal range (Ochromonicas danica assay). The rash, alopecia, and neurologic findings responded dramatically to biotin therapy (100 micrograms/day in all patients; an initial larger dose of 1 mg/day for 1 week plus 10 mg/day for 7 weeks in one patient), and did not recur. However, abnormal organic acid excretion persisted in one patient who did not receive the larger dose. We conclude that plasma biotin concentration does not reflect biotin status in all cases and speculate that the biotin supplement currently recommended for pediatric patients (20 micrograms/day) may not be adequate therapy for biotin deficiency and might not even be adequate to maintain normal biotin status during TPN.

Alopecia↗
PubMed1985

Blind loop syndrome, vitamin E malabsorption, and spinocerebellar degeneration.

A 72-year-old man had severe malabsorption, progressive retinopathy, and spinocerebellar degeneration 32 years after gastric surgery, blind loop formation, and intestinal bacterial overgrowth. Clinical and pathologic features were typical of vitamin E deficiency; vitamin E was nearly undetectable in serum and profoundly low in adipose tissue. Vitamin E blood levels initially improved on treatment with antibiotics; after additional vitamin E supplementation, there was clinical improvement.

Absorption↗
PubMed1985

Pyridoxine deficiency in children treated with isoniazid.

Isoniazid-induced deficiency of pyridoxine (vitamin B6) is reportedly not uncommon in adults but rare in children. In the present study, 38 children had serum levels of pyridoxine tested while receiving therapy with isoniazid. A biologic assay using the protozoan Tetrahymena thermophila determined pyridoxine status after 2 to 18 months of therapy with isoniazid. Five children (13 percent) were deficient. None had definitive clinical symptoms or signs consistent with pyridoxine deficiency. Three had normal nerve conduction velocity. Children receiving isoniazid in dosages greater than 10 mg/kg/day had a higher incidence of deficiency. Present recommendations for withholding pyridoxine prophylaxis from children receiving isoniazid therapy must be reconsidered in light of these findings, particularly in those children who are debilitated or have a poor nutritional history with a known pyridoxine deficit prior to therapy with isoniazid.

Child↗
PubMed1985

Genetic effects and sexual dimorphism in tyrosine hydroxylase activity in two mouse strains and their reciprocal F1 hybrids.

This study further analyzed the environmental and genetic mechanisms underlying the previously reported strain differences in tyrosine hydroxylase (TH) activity in the nigrostriatal and hypothalamic dopamine system of the BALB/cJ (B) and CBA/J (C) inbred mouse strains and related behavioral processes using parental and reciprocal F1 hybrid generations. Significant strain differences were found in both sexes in all the measured characters. Comparing males and females, sexual dimorphisms were found in TH activity of substantia nigra (SN), corpus striatum (CS) and hypothalamus (H), and in exploratory behavior (IE). Presence of sexual dimorphism was genotype dependent, with the exception of TH activity in H. Major components of strain differences, maternal effects and additive gene effects, were separated by biometrical genetic methods. The analysis indicated that significant maternal effects were present in TH activity of TH and CS with a trend towards this phenomenon in the SN. Additive gene effects were significant in all characters and various degrees of dominance were expressed in the hybrids in TH activity of SN and CS, as well as in behavioral traits, IE and spontaneous locomotion (SL). All the biochemical and behavioral parameters were expressed at lower levels in CBA/J than in BALB/cJ mice and reciprocal F1 hybrids took intermediate positions between the two parental strains for all phenotypes examined, with the exception of IE, where complete dominance was found in (CXB)F1 females. These results are consistent with the hypothesis that some of the genes affecting TH activity in brain dopamine systems contribute to the expression of dopamine mediated behaviors. Our analysis also indicates the possibility that the maternal effects on TH activity in CS and SN are the consequences of X-chromosome linked gene effects. We suggest that the influence of the X-chromosome linked gene(s) is dependent upon the action of gonadal steroids during the critical period of ontogenesis, and X-chromosome linked gene(s) play a major role in the genotype dependent expression of sexual dimorphism in TH activity.

Animals↗
PubMed1984

Distribution of catecholamine neurons in the hypothalamus and preoptic region of mouse.

The distribution and morphology of cells containing tyrosine hydroxylase (TH) were mapped by using the immunoperoxidase technique in the hypothalamus and preoptic area in two strains of mouse, CBA/J and BALB/cJ. On the basis of rostral-caudal contiguities between cell aggregates, hypothalamic preoptic neurons were subdivided into three arbitrary groups: (1) dorsal, (2) intermediate, and (3) ventral. New or more prominent collections of TH cells were observed, and in some regions, cells were more complexly organized than originally described. In the dorsal group, a rostral collection of small ovoid cells, previously not described, were located in the anterior preoptic nucleus (APN) of Loo ('31) and extended rostrally and ventrally into the preoptic periventricular gray. The next constituent occupied the paraventricular nucleus (PVN), and was composed of two classes of cells: (1) a small ovoid cell within anterior and medial parvocellular PVN in contiguity rostrally with a similar cell in APN and (2) a larger, angular cell within and adjacent to the lateral PVN in contiguity caudally with cells in the zona incerta (ZI). Further caudally, a larger and more pleomorphic collection of TH neurons was localized in the medial ZI, particularly at midtuberal levels. These cells were not scattered, as previously reported, but were differentiated into two clear-cut densities, a larger medial island and a more elongated lateral island. Cells of ZI, both large and small, extended caudally into the dorsal hypothalamic and subparafascicular nuclei and periventricular gray. In contrast to previous descriptions, no cells were seen in the nucleus reuniens. In the intermediate group, the most rostral constituent occupied the preoptic periventricular gray, extended as far as the lamina terminalis, and merged dorsocaudally with cells in APN. While the variably shaped cells of the hypothalamic periventricular gray (PVG) were still present in the retrochiasmatic region, a striking absence of these cells was noted at midtuberal levels between the dorsomedial and arcuate hypothalamic nuclei. At this level, a new group of small-round TH cells, resembling those of the arcuate nucleus, was identified in the dorsomedial hypothalamic nucleus (DMN). At caudal tuberal levels, similar neurons were found in the posterior hypothalamic nucleus (PH). These neurons overflowed medially into the PVG and caudoventrally into the arcuate nucleus. In the ventral group, the most rostral constituent, composed of both small and ovoid cells in the retrochiasmatic area, appeared to represent the rostral commissural portion of the arcuate nucleus (Arc).(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1984

Vitamin content of some normal human brain segments.

Nicotinates, pantothenates, riboflavin, vitamins B6 and B12' free (acetyl) and total (free and bound) choline, biopterin, thiamin, biotin, methylated and nonmethylated folates in frontal, temporal, precentral, postcentral, and occipital cortex, thalamus, cerebellum, pons, basal ganglia, and substantia nigra were estimated. Nicotinates are significantly more concentrated in basal ganglia and thalamus than pons. Nonmethylated folate content is not significantly varied in brain segments; the pons contains more methylated folate. Riboflavin content is higher in the basal ganglia and temporal cortex than frontal cortex. Biotin is concentrated in pons and basal ganglia. Thiamin concentration is less in the postcentral cortex than the thalamus and substantia nigra. Biopterin is significantly higher in substantia nigra and basal ganglia than the other brain segments. Total choline content is high in substantia nigra, pons, and thalamus; free (acetyl) choline is significantly elevated in basal ganglia. B12 content is less concentrated in the cortex segments. B6 is highly concentrated in the basal ganglia. Pantothenate content is elevated in pons when compared to the various cortex segments and cerebellum.

Adult↗
PubMed1984

Expression of phenylethanolamine N-methyltransferase in sympathetic neurons and extraadrenal chromaffin tissue of chick embryos in vivo and in vitro.

Previous studies suggested the existence of two populations of cells in the mammalian sympathetic nervous system which differ in their ability to express phenylethanolamine N-methyltransferase (PNMT), the enzyme which specifically subserves the biosynthesis of epinephrine: (1) sympathoblasts and their progeny, the noradrenergic sympathetic neurons (PNMT negative); and (2) phaeochromoblasts , the precursors of the adrenergic cells of the adrenal gland and extra-adrenal chromaffin tissue (PNMT-positive). We sought to determine whether similar differences between sympathoblasts and phaeochromoblasts exist in other classes of vertebrate embryos. Using immunohistochemical and biochemical techniques to assay PNMT in sympathetic organs, we have found that chick embryo paravertebral ganglia contain PNMT activity both in vivo and in vitro. In vitro PNMT immunostaining was detected in principal neurons as well as in small process bearing neurons similar to mammalian SIF cells. In vivo, cells containing PNMT were seen not only in the adrenal gland, but also in other sympathetic structures such as the extra-adrenal chromaffin tissue and, unexpectedly, also in some cells of the kidney. Tyrosine hydroxylase, the first enzyme of the catecholamine (CA) biosynthetic pathway, was found in the same cells which could be stained with PNMT antibodies as well as in the sympathetic ganglia. Thus, we conclude that in contrast to the rat, chick sympathoblasts share with phaeochromoblasts the property of expressing all the CA enzymes, including PNMT.

Adrenal Medulla↗