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Biomedical subjects

S H LaFranchi

Publications and source records attributed to S H LaFranchi.

9 recordsLinked to original sources
PubMed1989

Puberty in the syndrome of septo-optic dysplasia.

To determine the patterns of puberty associated with the syndrome of septo-optic dysplasia, 13 older children with optic nerve hypoplasia and hypopituitarism were studied. Three patterns of puberty were observed: early, rapidly progressive puberty (group 1); appropriately timed puberty (group 2); and delayed puberty associated with gonadotropin deficiency (group 3). In the six patients in group 1, puberty began at an early bone age. Pubertal changes progressed rapidly and the bone age advanced faster than chronologic time so that, despite a normal to increased growth rate, growth potential was lost. Group 2 comprised three patients with multiple pituitary hormone deficiencies but without gonadotropin deficiency who had the timing and progression of puberty expected in hypopituitarism. The four patients in group 3, all with multiple pituitary hormone deficiencies, had gonadotropin deficiency requiring sex steroid replacement.

Adolescent↗
PubMed1989

Massive levothyroxine ingestion. Conservative management.

The clinical course of a 29-month-old girl who was referred for evaluation after ingesting ninety 0.2-mg tablets of levothyroxine is reported. Despite an initial thyroxine (T4) level of 282 micrograms/dl and a triiodothyronine (T3) level of 1,837 ng/dl at 48 hours postingestion, her symptoms were mild and included irritability, vomiting, tremor, and tachycardia. Treatment was limited to activated charcoal and propranolol. Thyroid hormone levels fell to normal by 13 days postingestion. The child's clinical course was benign. Even after massive acute ingestions of levothyroxine, children's symptoms are usually mild and may be controlled with propranolol. This conservative approach should be considered before expensive and potentially dangerous therapies are undertaken.

Charcoal↗
PubMed1979

Sexual precocity with hypothalamic hypopituitarism.

Two girls, one with septo-optic dysplasia and the other with posttraumatic brain damage, had the unusual combination of human growth hormone, thyrotropin, adrenocorticotrophic hormone, and vasopressin deficiencies that were associated with sexual precocity in one patient and early sexual maturation in the second patient, and of adult follicle-stimulating hormone and luteinizing hormone concentrations. At autopsy, the first patient had optic nerve aplasia, a normal pituitary gland, and some disorganization of myelinated fibers in the hypothalamus. The second patient had a normal thyrotropin and prolactin response to thyrotropin-releasing hormone, plus hyperphagia, deranged thirst mechanism, and temperature instability. These findings suggest that the lesion may be a defective hypothalamic regulation of pituitary hormone secretion. Congenital or traumatic hypothalamic-pituitary lesions may not affect all releasing factors or trophic hormones in a similar fashion.

Adolescent↗
PubMed1979

Hypothyroidism.

Explore the source record for details and available documents.

Age Factors↗
PubMed1979

Neonatal hypothyroidism detected by the Northwest Regional Screening Program.

The Northwest Regional Screening Program to detect congenital hypothyroidism in infants born in Oregon, Montana, Alaska, and Idaho (combined birthrate of 69,000/ yr) was added to our ongoing screening program in 1975. The program utilizes dried blood filter paper specimens collected routinely in the first few days of life in all four states and again at about 6 weeks of age in Oregon only. The screening test consist of an initial thyroxine (T4) measurement; a thyroid-stimulating hormore (TSH) determination is performed on those specimens with T4 concentrations in the lowest 3% group. Serum samples obtained by venipuncture are requested for confirmation of the diagnosis. In the first two years of the program, 25 infants with primary hypothyroidism were detected amont 110,667 infants screened, a frequency of 1:4,430. Fourteen cases of thyroxine-binding globulin deficiency were also detected, a frequency of 1:7,900. Using the T4 followed by TSH testing approach, the frequency of request for repeat specimens was 0.4% in Oregon and 0.05% in the other states. The cost per specimen was $1.96. The majority of infants lacked clinical signs or symptoms of hypothyroidism; only one infant was clinically suspected of having hypothyroidism prior to detection. The most common neonatal symptoms were constipation, lethargy, and prolonged jaundice, while the most common physical signs were hypotonia, umbilical hernia, and large fontanels. Thyroid scans showed the most common etiology to be thyroid aplasia, followed by an ectopic gland, hypoplasia, and goiter. Serum T4 concentrations were lowest in those infants with aplasia, intermediate in infants with an ectopic gland or hypoplasia, and normal in the infant with the goiter. Neonatal hypothyroidism varies in degree and has several different causes; the capacity to secrete thyroid hormone, the duration before hypothyroidism becomes clinically manifest, and possibly the eventual prognosis for intellectual function depend on the nature of the underlying cause. While the mean age at treatment was 59 days, the goal of diagnosing congenital hypothyroidism and treating affected infants by 1 month of age seems realistic.

Congenital Hypothyroidism↗
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↗