Biomedical subjects

A H Perelman

Publications and source records attributed to A H Perelman.

5 recordsLinked to original sources

PubMed1993

Long-term effectiveness of depot gonadotropin-releasing hormone analogue in the treatment of children with central precocious puberty.

OBJECTIVE: To assess the efficacy and safety of a long-acting gonadotropin-releasing hormone analogue (GnRHa), leuprolide acetate for depot suspension (Lupron Depot), in the treatment of central precocious puberty in children, and to determine the reversibility of GnRHa therapy after it has been discontinued. RESEARCH DESIGN: Children with documented central precocious puberty were treated with Lupron Depot for 1.6 to 3.5 years. Their course of pubertal development, growth rate, skeletal maturation, and response to gonadorelin hydrochloride testing were compared before and during treatment. For those girls who finished treatment, an assessment of the reversibility of the GnRHa was performed by documenting a return to pubertal responses to gonadorelin testing, and by documenting menarche at an appropriately mature bone age. SETTING: Community teaching hospital. PATIENTS: Ten girls with central precocious puberty defined as pubertal maturation statistically advanced for age combined with a pubertal gonadotropin response to gonadorelin testing. Children who had been treated for less than 1.5 years were excluded, as were those with congenital adrenal hyperplasia. Patients who finished treatment have been followed up for up to 5 years, and will continue in follow-up throughout their reproductive life. SELECTION SAMPLE: A consecutive group of children with documented central precocious puberty was studied. INTERVENTIONS: Lupron Depot was administered as a single monthly subcutaneous injection to each patient. Treatment was usually discontinued by 10 to 11 years of age, at which time pubertal progression was allowed to resume. MEASUREMENT AND RESULTS: Mean peak serum concentrations of follicle-stimulating and luteinizing hormone responses to gonadorelin testing decreased significantly after the initial dose (from 21.8 +/- 4.5 [+/- SEM] to 2.4 +/- 0.2 IU/L for follicle-stimulating hormone and from 50.1 +/- 11.2 to 5.0 +/- 0.8 IU/L for luteinizing hormone) and remained suppressed for the duration of treatment. The progression of puberty slowed or reversed in all patients. Mean growth rate for chronologic age was significantly increased initially by 3.9 SDs and decreased to 0.9 SDs during treatment. The mean rates of skeletal maturation divided by the change in chronologic and height age changes over time were advanced (1.4 +/- 0.1 and 1.1 +/- 0.15, respectively) at the onset of therapy and decreased significantly to 0.7 +/- 0.1 and 0.8 +/- 0.1, respectively, on treatment. There was an increase in mean predicted height of 3.4 cm for all patients, and this was statistically significant. Thus, treatment with Lupron Depot at least maintained the predicted height at the onset of therapy. Girls who completed their course of treatment had pubertal gonadotropin responses to gonadorelin testing within 2 to 6 months, and menarche within the first year if skeletal maturation reached 13.0 to 13.5 years. No significant side effects of therapy were noted. CONCLUSIONS: Treatment of central precocious puberty in children using Lupron Depot is safe and efficacious. Its effects are readily reversible after treatment is discontinued, and menarche occurs at a normal bone age. Measurement of serum luteinizing hormone concentrations using an assay that is specific for the beta-subunit is necessary to monitor chemical suppression of luteinizing hormone during treatment. Longer-term studies, including reproductive history, will be needed before the potential effects of treatment on fertility can be assessed.

Age Determination by Skeleton↗

PubMed1992

The fetus in maternal hyperthyroidism.

A recent article in the New England Journal of Medicine reported the successful diagnosis and treatment of fetal goitrous hypothyroidism in a mother with Graves' disease. The fetus is being recognized as an important patient in its own right in terms of thyroid disease. The fetal thyroid system develops independently of the normal maternal thyroid axis. Presence of feedback suppression of TSH by T4 has been demonstrated in a 35-week fetus. Information learned from congenital hypothyroidism suggests that lack of fetal thyroid hormones may have a negative impact on the developing fetal brain with lack of normal myelination. It is uncertain at what gestational age the fetus and the developing central nervous system become adversely affected by thyroid hormone deficiency. Since congenital hypothyroidism is sporadic and since there is no current method for easily screening all pregnancies for hypothyroidism, the thrust in fetal diagnosis and therapy has been in those pregnancies suspected of having a hypothyroid fetus when a fetal goiter is detected by ultrasonography or in a hyperthyroid mother who may be on antithyroid therapy. Intraamniotic injections of L-thyroxine have proven successful for fetal therapy. Amniotic fluid TSH may prove useful in the diagnosis and treatment of a hypothyroid fetus. Previous studies have suggested that the period of thyroxine dependency of the fetal central nervous system is limited predominantly to the last 4-8 weeks of gestation. Fetal hyperthyroidism due to transplacental transmission of thyroid-stimulating immunoglobulins may occur in a mother with a history of hyperthyroidism due to Graves' disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗

PubMed1990

Intrauterine diagnosis and treatment of fetal goitrous hypothyroidism.

Newborn screening programs for the detection of congenital hypothyroidism have dramatically shortened the time before treatment is begun. However, concern still exists about central nervous system sequelae which may persist due to a period of untreated intrauterine hypothyroidism. Presence of polyhydramnios led to the ultrasound diagnosis of a fetal goiter. Hypothyroidism was confirmed at 34 weeks gestation by percutaneous fetal blood sampling, which revealed an elevated TSH (186 mU/L) and a low T4 (19.3 nmol/L). Intraamniotic fluid injections of 500 micrograms levothyroxine sodium (T4) every 10-14 days increased fetal serum T4 (59.2 nmol/L), decreased fetal serum TSH (14 mU/L), decreased amniotic fluid TSH, and decreased the size of the fetal goiter. The infant was born at term without perinatal complications. Thyroid function studies on cord blood were normal (T4, 109.4 nmol/L; TSH, 1.3 mU/L), and the infant was discharged on oral T4. Follow-up examination at age 6 weeks revealed that the infant was developmentally normal and clinically and chemically euthyroid. Intrauterine T4 therapy can suppress fetal TSH and treat fetal hypothyroidism despite hypothyroid levels of serum T3. Highly sensitive TSH assays may allow the use of amniotic fluid TSH as a marker for fetal hypothyroidism.

Adult↗

PubMed1989

Fetal goitrous hypothyroidism. A new diagnostic and therapeutic approach.

We present a first case in whom fetal hypothyroidism with goiter was both successfully diagnosed and treated in utero. An obstetrical sonogram at 33 weeks revealed a bilobed fetal neck mass, compatible with enlarged thyroid gland, associated with neck hyperextension, reduced gastric fluid, and polyhydramnios. Umbilical blood sampling after volume reduction amniocentesis confirmed fetal hypothyroidism with a euthyroid mother. Fetal T4 measured 1.3 micrograms/dl, free T4 0.3 ng/dl, and thyroid-stimulating hormone 186 microU. Intraamniotic levothyroxine, 500 micrograms, was given twice with a 14-day interval. The head flexed, gastric fluid increased, and amniotic fluid levels returned to normal. Prenatal (36 weeks) and neonatal blood sampling demonstrated return to euthyroid indices. Ultrasonic estimates of thyroid volume decreased by over 50%. Vaginal delivery at 39 weeks was uncomplicated. The newborn appeared normal and is being maintained on thyroid replacement therapy.

Adult↗

PubMed1985

The occult insulinoma operative localization by quick insulin radioimmunoassay.

Hypoglycemia secondary to organic hyperinsulinism in children can be caused by diffuse or localized pancreatic lesions. Differentiation between these two types of lesions is of utmost importance since the surgical approach will be different. Some tumors escape detection by all preoperative investigations including ultrasound, scintiscan, arteriography, and computerized tomography. We are reporting on a 12-year-old boy with organic hyperinsulinism in whom we were unable to localize a tumor preoperatively. Peroperative determination of insulin levels from a number of sites on the pancreas enabled us to localize an insulin-producing pancreatic adenoma. This technique can be done easily by catheterizing the splenic and portal vein through a branch of the splenic vein and by serial sampling at 2 cm intervals along the portal and splenic veins. Insulin levels at these sites were determined by quick double-antibody radioimmunoassay, which allows the determination of the insulin levels within 50 minutes after sampling. There was a perfect biochemic and anatomic correlation allowing us to perform a precise distal pancreatectomy. The technique can be used to localize pancreatic adenomas and to decide how much pancreas to remove in diffuse lesions avoiding "blind" pancreatectomies.

Adenoma, Islet Cell↗