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Biomedical subjects

B M Lippe

Publications and source records attributed to B M Lippe.

At least 73 records · Page 4Linked to original sources
PubMed1979

Relative usefulness of three growth hormone stimulation screening tests.

One hundred ninety-one children were tested by one of three screening procedures for growth hormone deficiency over a five-year period. Sleep screen yielded a 31.3% false-positive rate; levodopa administration alone yielded a 20.5% false-positive rate and levodopa in combination with propranolol yielded a 5.2% false-positive rate. These results support the view that the combined levodopa-propranolol hydrochloride screen test is superior to either the sleep or the levodopa screen tests in limiting the number of false-positive results and thus the need for further, more extensive testing.

Child↗
PubMed1979

Inherited abnormal thyroid hormone-binding protein causing selective increase of total serum thyroxine.

A 9-yr-old boy is described in whom increased serum T4 concentration, increased T3 uptake, and increased free T4 index were associated with a euthyroid clinical state with normal total serum T3. T4-binding globulin (TBG), measured by RIA, was decreased. Reverse flow paper electrophoresis of serum proteins after reaction with radioactively labeled T4 demonstrated increased binding of T4 to a protein with electrophoretic mobility corresponding to albumin. Displacement of serum protein-bo-nd [125I]T4 activity by increasing concentrations of T4 revealed the presence of a low affinity, high binding capacity system with an association constant similar to that of T4-binding prealbumin. This low affinity binding protein cochromatographed with TBG on a DEAE-Sephadex column which normally separates TBG from T4-binding prealbumin. At free T4 concentrations equivalent to those present in the plasma of normal individuals, the T4 bound to free ratio is higher in the patient than in normals and the total serum T4 level is increased in the presence of normal free T4 concentrations. The relative affinity of this abnormal T4-binding protein for T3 is low compared to that of TBG. The patient's father had the same abnormal binding protein, which was not found in his mother or fraternal twin brother. These data suggest an autosomal dominant mode of inheritance of an aberration leading to synthesis of a new protein instead of normal TBG. The new protein is different from TBG in electrophoretic mobility, T4 and T3 binding, and antigenic properties.

Child↗
PubMed1978

Disproportionate growth of the lower extremities. A major determinant of short stature in Turner's syndrome.

The relationships between the height (H), sitting height (SH), and the lower segment (H-SH) were studied in a group of 21 patients with Turner's syndrome. The SH/H ratio was markedly abnormal (0.55), equivalent to that of 6-year-old normal girls. The abnormality in ratio was a result of markedly shortened lower extremities. Further, there was a significant inverse correlation between SH/H-SH ratio and height such that patients with the most shortening of their legs were the shortest. The degree of abnormality in lower segment, therefore, is a major determinant of stature in Turner's syndrome.

Adolescent↗
PubMed1978

Pelvic ultrasonography in pediatric and adolescent endocrine disorders.

Pelvic ultrasonography was employed in the evaluation of 57 pediatric and adolescent patients with disorders of sexual development or differentiation. It provided a rapid and accurate method for: (1) detection of the presence of a uterus and its stimulation by estrogen; (2) detection of ovaries in children over the age of two years and assessment of their size and symmetry, and (3) identifying ovarian pathology such as cystic areas. Its value as an additional diagnostic modality in the approach to the infant with ambiguous genitalia, the child with precocious puberty, and the adolescent with amenorrhea, hirsuitism, or virilization was demonstrated.

Adolescent↗
PubMed1978

Increased monocyte receptor binding of [125I]insulin in infants of gestational diabetic mothers.

Monocyte insulin receptor binding has been shown to be inversely correlated with basal insulin concentrations in a variety of clinical conditions and is believed to reflect autoregulation of receptor properties of insulin. We examined the insulin receptor in the infant of the gestational diabetic mother (IGDM) where hyperinsulinism has been implicated in the attendant metabolical abnormalities. Insulin concentrations in plasma of cord blood of IGDM were significantly greater than in normals delivered between 36-38 weeks by elective cesarean section (101.3 +/- 9.8 vs. 59.9 +/- 9.4 microU/ml; P less than 0.005). [125I]Insulin binding to receptors of monocytes obtained from cord blood showed that IGDM had more receptor sites per monocyte than normal adults and normal infants. In normal infants of similar gestational age, a significant correlation was found between birthweight and binding which was not observed in IGDM. Monocytes from both normal infants and IGDM showed greater affinity for insulin than those from adults. At plasma insulin concentrations of 1 and 4 ng/ml, monocytes from IGDM had about 10 times and normal infants had about 4 times as many sites occupied as those from normal adults. Monocytes from IGDM seem to develop increased concentrations of insulin receptors with hyperinsulinemia. Thus, despite increased ambient levels of insulin, monocytes of IGDM seem to develop increased concentrations of insulin receptor as well as increased affinity for the hormone.

Birth Weight↗
PubMed1978

Management of congenital adrenal hyperplasia using serum dehydroepiandrosterone sulfate and 17-hydroxyprogesterone concentrations.

Simultaneous serum concentrations of dehydroepiandrosterone sulfate (DHEA-S) and 17-hydroxyprogesterone (17-OHP) were compared with urinary 17-ketosteroid (17-KS) and pregnanetriol (PT) excretion during therapy in 18 prepubertal patients with the 21-hydroxylase deficiency form of congenital adrenal hyperplasia (CAH). Patients were classified into those in good, poor, or questionable control on the basis of clinical examination, skeletal age, and 17-KS and PT excretion. During therapy, use of serum steroid concentrations was found to be nearly as accurate in judging adequacy of control as use of urine steroid concentrations. Of 34 evaluations, a definite assessment of adequacy of control could be arrived at 25 times using urinary values and 22 times using both serum DHEA-S and 17-OHP concentrations. DHEA-S concentration responded sluggishly when treatment was not adequate, being greater than 100 microgram/dl only in patients significantly undertreated. It was never elevated in well-controlled patients. Mid-afternoon 17-OHP concentrations were less than 200 ng/dl in well-controlled patients but readily escaped suppression and could not be used to differentiate poor from borderline control or from temporary noncompliance. Therefore, an increases DHEA-S concentration indicated poor control and a suppressed 17-OHP concentration indicated good control. The combination of normal DHEA-S level with elevated 17-OHP level, however, did not permit exact evaluation of the degree of control. Of significance is that not all patients with CAH present with an elevated DHEA-S concentration, and only in those in whom an elevated level has been documented can DHEA-S level be used as an index of control during therapy.

17-Ketosteroids↗
PubMed1977

Congenital adrenal hypoplasia and hypogonadotropic hypogonadism.

A 19-year-old male patient with congenital adrenal hypoplasia and elevated plasma adrenocorticotropic hormone level was found to have gonadotropin deficiency. Other hypothalamic-pituitary functions were normal. A possible relationship between hypothalamic-pituitary dysfunction and development of the cytomegalic form of congenital hypoplasia of the adrenals is suggested.

Adrenal Insufficiency↗
PubMed1977

Pancreatic alpha and beta cell functions in cystic fibrosis.

Insulin and glucagon secretions were studied during oral glucose tolerance testing and arginine infusion in 13 patients with cystic fibrosis. Two groups of patients were identified; Group I (N=6) whose OGTT was entirely normal and Group II (N=7) who had some abnormality in glucose during OGTT. In each group basal glucagon concentrations were normal and supressed appropriately (p less than 0.05) after glucose; insulin responses were attenuated and the peak responses delayed. During arginine stimulation, insulin secretion was impaired in each group. However, glucagon secretion was diminished only in Group II. Thus, insulinopenia was found in both groups and hyperglucagonemia was not found as a contributory factor to the hyperglycemia in Group II.

Adolescent↗
PubMed1977

Testis tumors associated with congenital adrenal hyperplasia: a continuing diagnostic and therapeutic dilemma.

A 9-year-old boy with documented congenital adrenal hyperplasia owing to a deficiency in C21 hydroxylation is described. Precocious virilization and testicular asymmetry were noted when he was 5 years old. Both of these conditions persisted despite progressively larger doses of replacement steroid therapy. The right testis was 3 times larger than the left testis and serum testosterone was well within the adult male range. A right inguinal orchiectomy was performed. Pathologic diagnosis was interstitial cell tumor. A review of the literature emphasizes the continuing dilemma of separating interstitial cell tumors from hypertrophy of adrenal rest tissue in the presence of congenital adrenal hyperplasia. Possible methods of distinction are discussed.

Adrenal Hyperplasia, Congenital↗
PubMed1977

Gray-scale ultrasonography of the normal female pelvis.

The pelvis was evaluated with gray-scale ultrasound in 45 normal females, important aspects of instrumentation, changes in scanning techniques, and pitfalls in the identification of various normal anatomical structures are described, and normal ranges of uterine size and ovarian volume are reported.

Adolescent↗
PubMed1977

Value of simultaneous T3, T4, and TSH measurements for management of graves' disease in children.

One hundred simultaneous determinations of serum T4 and T3 were performed during the course of treatment of 23 children and adolescents with Graves' disease. Five patients were previously untreated and six were experiencing relapse after treatment was discontinued. During relapse, increased T3 concentration is frequently present when serum T4 concentration is normal and T3 measurement is therefore more reliable for early detection of relapse. During therapy with thionamides, T4 measurement alone is often misleading in assessing adequacy of control achieved by therapy. Commonly, patients who clinically have hyperthyroidism have serum T4 concentrations within the normal range but continue to have elevated T3 concentrations (T3 toxicosis). Similarly, T4 can be suppressed into the hypothyroid range in the clinically euthyroid patient with either a normal or high T3 concentration. In these patients, determination of serum T3 often prevents premature reduction of thionamide dosage. When T4 and T3 concentrations do not clearly demonstrate presence or absence of hypothyroidism, measurement of serum thyroid stimulating hormone can be of value.

Graves Disease↗
PubMed1976

Vasopressin analog DDAVP in the treatment of diabetes insipidus.

A synthetic vasopressin analog, 1-desamino-8D-arginine vasopressin (DDAVP also known as desmopressin), was found to be highly effective in the treatment of seven children and one adult with vasopressin-sensitive diabetes insipidus. The average duration of action of DDAVP was 10 to 11 hours, and with proper adjustment of dose, the subjects were able to control their symptoms satisfactorily with one or two inhalations daily. The youngest child in whom adequate control was achieved was 2 years of age. All subjects found the use of intranasal DDAVP superior to other forms of therapy, and none experienced any known side-effects after six months of treatment. All subjects in this study are currently using 2.5 mug to 10 mug of DDAVP once or twice daily.

Adolescent↗
PubMed1976

The occurrence in a neonate of a pancreatic adenoma with nesidioblastosis in the tumor.

A newborn with persistent hypoglycemia and fixed insulin secretion unresponsive to medical management underwent 80% pancreatectomy. A 1-cm. nonencapsulated islet-cell adenoma near the tail of the pancreas was identified histologically. Islet-cell granules stained with indirect aldehyde fuchsin. Throughout the tumor, areas of nesidioblastosis were identified. The persistence of this embryologic process suggests a basic mechanism for the adenoma formation.

Adenoma, Islet Cell↗
PubMed1975

Pubertal gynecomastia and transient elevation of serum estradiol level.

Serum levels of testosterone, estradiol, progesterone, 17alpha-hydroxyprogesterone, follicle-stimulating hormone, and luteinizing hormone were measured in 16 boys with pubertal gynecomastia. Six patients had elevated serum estradiol concentrations, and four of these six also had elevated progesterone levels. Serum estradiol/testosterone ratios were high for the stage of puberty in 11 of the 16 patients. In five patients who had two or more determinations, the steroid concentrations returned toward or into the normal range. Transient increases in serum estradiol concentration or an abnormally high estradiol/testosterone ratio may be causally related to the development of gynecomastia in adolescent boys.

17-Ketosteroids↗
PubMed1975

Reversible hypothyroidism in growth hormone-deficient children treated with human growth hormone.

Six children with human growth hormone (hGH) deficiency became hypothyroid during the course of their therapy with hGH. This was accompanied by a decreasing growth rate, clinical symptoms of hypothyroidism and decreased serum T4 concentrations. Three of the 6 patients returned to the euthyroid state, both clinically and biochemically, with cessation of hGH therapy, and reinstitution of hGH precipitated hypothyroidism again in 2 of the three. The patients who remained hypothyroid have evidence of multiple pituitary trophic hormone deficiencies while those who reverted to euthyroidism appear to have isolated hGH deficiency. Evaluation of thyroid function while on hGH showed low T4, free T4 and T3 concentrations. The serum thyrotropin (TSH) response to thyrotropin-releasing hormone (TRH) was absent or markedly blunted in 4 of 6 patients while receiving long-term hGH therapy but was normal or exaggerated in all patients when tested before or after only 5 days of hGH therapy. These data indicate that exogenous hGH results in an inhibition of the TSH response to TRH. The mechanism of this inhibition is unclear, but we postulate that it may be mediated by somatostatin secretion in response to pulse doses of hGH.

Age Determination by Skeleton↗