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Biomedical subjects

W H Hoffman

Publications and source records attributed to W H Hoffman.

16 recordsLinked to original sources
PubMed1992

The natural history of autonomous gonadal function, adrenarche, and central puberty in gonadotropin-independent precocious puberty.

Gonadotropin-independent precocity (GIP) is a syndrome marked by precocious pubertal development in the absence of pubertal levels of gonadotropins. To investigate the discrete patterns of central nervous system, gonadal, adrenal, and skeletal maturation in this syndrome, we conducted longitudinal studies spanning up to 10 yr in two such affected individuals. A cross-sectional analysis of adrenal androgen secretion was performed in nine additional patients to assess further the time course of adrenarche in GIP. Serial evaluations revealed progression of secondary sexual characteristics, statural growth, and skeletal maturation, all consistent with ongoing exposure to pubertal gonadal steroid levels. On the other hand, adrenarche (n = 11) and spontaneous and GnRH-stimulated gonadotropin secretion (n = 2) progressed in chronological age-appropriate manners despite long term pubertal levels of gonadal sex steroid secretion. After the development of central puberty, as documented by the appearance of pulsatile gonadotropin secretion, we sought to determine whether the potential for gonadal autonomy persisted. Despite complete pituitary desensitization induced by administration of a GnRH agonist, both patients studied demonstrated an ongoing capacity to secrete pubertal levels of gonadal steroids. Our study suggests that the timing of adrenarche and central puberty in these subjects with GIP was apparently unaltered by prolonged exposure to gonadal steroids. Subsequent to the development of central puberty, pulsatile gonadotropin secretion may override and, thus, mask the underlying defect(s) in adolescents and adults with histories of GIP.

Adrenal Glands↗
PubMed1992

Correlates of brain edema in uncontrolled IDDM.

Blood glucose, plasma sodium, bicarbonate (HCO3-), vasopressin, and hematocrit were monitored before and during treatment in patients with uncontrolled insulin-dependent diabetes mellitus (IDDM). These parameters were correlated with simultaneous serial cranial computed tomography readings of brain edema. Six of seven patients had positive computed tomography readings for brain edema on admission. Initial brain edema correlated directly with blood glucose (r = 0.79, P = 0.033) and inversely with HCO3- (r = -0.76, P = 0.047). At 6 h, brain edema still correlated with acidosis (HCO3-; r = -0.79, P = 0.033) but no longer with blood glucose. At that time, however, brain edema correlated with the rate of change in blood glucose (r = 0.915, P = 0.005). Results of interactive stepwise regression analysis suggest that the change in the calculated effective plasma osmolality plays a predominant role in the progression of brain edema during therapy (r = 0.995, P less than 0.001). Thus, although hyperglycemia and acidosis probably predispose to diabetic brain edema, osmotic factors may be major predictors of its evolution. No relationships were detected between brain edema and initiation of insulin therapy, plasma vasopressin, or changes in hematocrit. The factors responsible for initial brain edema and its progression, statistically identified in this study, require reassessment of common theories that attribute brain edema exclusively to therapy.

Adolescent↗
PubMed1991

Bursting-induced epileptiform EPSPs in slices of piriform cortex are generated by deep cells.

Previous study revealed that bursting activity generated by a variety of means in slices of piriform cortex induces persistent epileptiform EPSPs in superficial pyramidal cells by an NMDA-dependent process. The present study was undertaken to test the hypothesis that the observed epileptiform EPSPs in superficial pyramidal cells are driven by deep cells. This hypothesis was suggested by recent findings from in vitro studies of the properties of deep cells and in vivo studies indicating that the deep part of the piriform cortex or neighboring deep structures are involved in the generation of seizure activity in animal models of epilepsy. Results from simultaneous cell-pair recordings, examination of subdivided slices, and local application of excitatory and inhibitory agents provided strong evidence in support of this hypothesis. It was concluded that the endopiriform nucleus, a collection of cells immediately deep to the piriform cortex, plays a central role in generation, but that cells in the deep part of layer III and the claustrum may also contribute. Furthermore, it was found that generation of prolonged ictal-like activity only occurs in slices of piriform cortex in which the endopiriform nucleus is present. Implications of these findings for epileptogenesis are discussed.

Animals↗
PubMed1990

Autoantigenic determinants on human thyroglobulin. II. Determinants recognized by autoantibodies from patients with chronic autoimmune thyroiditis compared to autoantibodies from healthy subjects.

Using a panel of 20 well-characterized mouse monoclonal antibodies (mAbs) to native human thyroglobulin (Tg), the fine specificities of autoantibody responses in human autoimmune thyroiditis patients were compared to naturally occurring autoantibodies from healthy individuals. The antigenic determinants recognized by the human autoantibodies were assessed using competitive binding assays in which murine mAbs were inhibited by human autoantibodies. All determinants of human Tg recognized by the mouse were also responded to by the human subjects. Autoantibodies from adult and juvenile patients with chronic lymphocytic thyroiditis recognized principally 10 of 19 determinants defined by the panel of murine mAbs. The mAbs which define these 10 determinants do not react with Tg from seven other mammalian species; therefore, autoantibody responses in individuals with thyroiditis are directed primarily to species specific determinants of human Tg. These same determinants on human Tg were the principal ones recognized by the mouse following immunization. Particular patterns of fine specificities were not inherited from parents by probands or their siblings, even if identical twins. Naturally occurring Tg autoantibodies from healthy subjects recognized mainly thyroxine-containing cross-reactive determinants.

Adolescent↗
PubMed1990

Thyroid autoantibodies in black and in white children and adolescents with type 1 diabetes mellitus and their first degree relatives.

Genetic susceptibility is an important issue in understanding the mechanism of the autoimmune endocrinopathies and in assessing the risk of these conditions in pediatric patients. To this end, we evaluated autoantibodies to thyroid antigens, thyroglobulin (TgA) and microsomal antigen (TMA), in white and in American black juvenile patients with Type I diabetes mellitus (DM) to determine the predictive value of thyroid autoantibodies for the development of autoimmune thyroid disease. Sera from 159 patients (77 black and 82 white) with Type I DM were evaluated. A greater number of whites (41/82 or 50%) than blacks (12/72 or 16%) had thyroid autoantibodies (p less than 0.01). Fourteen patients (4 black and 10 white) exhibited hypothyroidism, and all had both TgA and TMA. Three patients (all black) had Graves' disease, one of whom had both TgA and TMA. Families of each racial group that had a diabetic child (proband) with thyroid autoantibodies (seropositive) or without thyroid autoantibodies (seronegative) were assessed for TgA and TMA as well as autoimmune thyroid disease. The prevalence of thyroid autoantibodies among siblings of seropositive probands was significantly greater than among the siblings of seronegative probands (p less than 0.01). The white sibling population showed a closer association of thyroid autoantibody prevalence with increasing age (p less than 0.05) than the blacks. Significantly more parents of probands than control parents exhibited thyroid autoantibodies (p less than 0.01). The general pattern of inheritance of either racial group showed that if one or both parents had thyroid autoantibodies, their progeny developed a significantly higher prevalence of thyroid autoantibodies than those of the seronegative parents. While there was no increase in overt thyroid disease among siblings of seropositive probands, a risk of developing autoimmune thyroid disease is probably imparted to these siblings by virtue of the thyroid autoantibodies.

Adolescent↗
PubMed1978

Solitary maxillary central incisor and normal stature.

Two children, one boy and one girl, each with solitary maxillary deciduous and permanent central incisors, normal height, and normal plasma growth hormone levels, are reported. Review of the literature reveals fourteen reports of patients with a similar dental anomaly. Of these, three are normal in height. The height and growth rate of patients with a single maxillary central incisor should be evaluated prior to growth hormone evaluation; such investigation should be undertaken only if the height is two standard deviations below the mean or if there is evidence of plateauing of linear growth.

Abnormalities, Multiple↗
PubMed1977

Alterations in serum prolactin heterogeneity by provocative tests in a patient with a pituitary tumour.

The proportion of serum prolactin heterogeneity were investigated in a patient with a pituitary adenoma producing both prolactin and growth hormone. Glucose tolerance (GTT) and insulin tolerance (ITT) tests were performed alone and in combination with chlorpromazine both before and after hypophysectomy. Serum prolactin multiple forms were separated by Sephadex G-100 column chromatography at 3 degrees C. None of the provocative tests altered the already elevated serum prolactin either before (300 ng/ml) or after (100 ng/ml)hypophysectomy. Chlorpromazine or ITT did not alter the proportion of prolactin heterogeneity; GTT, whether in conjunction with chlorpromazine or alone and both before and after hypophysectomy, increased the proportions of the larger molecular species. In five tests in which the GTT was not performed the prolactin heterogeneity was as follows: 'void volume', 4.9%; 'big', 13.1%; 'little', 82.1%; in those experiments in which the GTT was performed the proportions of prolactin heterogeneity were: 'void volume', 13.5%, 'big', 19.2%; 'little', 67.3%.

Adenoma, Acidophil↗
PubMed1977

Lack of a circannual cycle of daytime serum prolactin in man and monkey.

Serum samples were obtained from 7 subjects (6 men and 1 woman) in the morning (09.00 h) and in the afternoon (15.00 h) of each week for a period of 13 months and assayed for prolactin by RIA. In addition, 4 female monkeys were sampled once weekly in the afternoon for approximately two years. The data were analyzed for cyclicity by power spectrum analysis. In both species a serum prolactin circannual cycle was not obvious. In most subjects statistically significant (P less than 0.05) cycles were observed; however, there was considerable variability among individuals in the duration of the cycle, which ranged between 4 and 50 weeks. A similar result was also observed for the monkey data; here the cycles ranged between 7 and 51 weeks. The physiologic significance of these cycles is unknown at present. It is suggested that although there appears to be no diurnal serum prolactin circannual cycle, this does not rule out the possibility that there may be a nocturnal circannual cycle.

Adult↗
PubMed1975

Glucose, insulin, pancreatic glucagon and glucagon-like immunoreactive materials in the plasma of normal and diabetic children. Effect of the initial insulin treatment.

Pancreatic glucagon (PG) and other glucagon-like immunoreactive materials (GLI) were measured in the plasma of normal and of newly diagnosed untreated diabetic children, using an antiglucagon serum (AGS) highly specific for pancreatic glucagon (AGS 18) and an AGS which crossreacts with extracts of intestinal mucosa (AGS 10). Gut GLI was considered to be the difference between "total" GLI (AGS 10) and PG (AGS 18). Glucose and immunoreactive insulin (IRI) were also measured. PG, total GLI and gut GLI were significantly elevated in children with severe insulin insufficiency and were reduced to normal by insulin treatment, even though a significant fasting hyperglycemia was still present. In three diabetic children who had high initial plasma IRI levels the three glucagon fractions were normal. We conclude that insulin insufficiency is characterized not only by high plasma levels of PG as previously reported, but also of gut GLI. These abnormalities can be corrected by the administration of insulin.

Adolescent↗
PubMedDate not supplied

Macroorchidism and testicular fibrosis associated with autoimmune thyroiditis.

A 16-year-old male with long-standing atrophic chronic lymphocytic thyroiditis was evaluated for macroorchidism. A testicular biopsy prior to treatment revealed peritubular and interstitial fibrosis, reduced spermatogenesis and sparse Leydig cells with nonprominent smooth endoplasmic reticulum. Biological/immunological LH and FSH ratios were reduced, I-LH and FSH response to GnRH was blunted, and levels of testosterone and androstenedione were low. Twenty-two months after thyroid treatment, the testicular size was unchanged, and the degree of fibrosis showed minimal regression. Spermatogenesis with normal morphology was present, Leydig cells with Reinke crystals were present, and surface area and diameter of the seminiferous tubules had increased only slightly. There was a normal I-LH and FSH response to GnRH, and normal levels of testosterone and androstenedione. This study, along with previous reports, suggests that the etiology of the hypothyroid state may influence the development of testicular fibrosis.

Adolescent↗
PubMedDate not supplied

Service and education for the insulin-dependent child.

A pilot program of service and education was designed to actively involve the inner-city, insulin-dependent child in his own diabetes management. A telephone service for questions and advice, managed by a pediatric nurse specialist, was responsible for a significant reduction in hospital admissions. The project was enthausiastically received and utilized by inner-city residents and resulted in an increased referral rate from the entire metropolitan area.

Ambulatory Care↗