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Biomedical subjects

T Moshang

Publications and source records attributed to T Moshang.

At least 19 recordsLinked to original sources

The effects of adjuvant chemotherapy on growth in children with medulloblastoma.

BACKGROUND: Current therapy for children with medulloblastoma includes craniospinal radiation therapy (CSRT) with or without adjuvant chemotherapy. The difference in growth of children after the two different therapeutic modalities is unknown. METHODS: The growth of 38 prepubertal children who survived medulloblastoma was reviewed retrospectively. Fifteen of these patients received CSRT alone; 23 received chemotherapy in addition to the radiation therapy. RESULTS: The average growth velocity of all patients with medulloblastoma during the 4 years of the study was below the mean for age and sex in all patients except one. Most patients grew at velocities more than two standard deviations below the mean. The overall growth of children who received chemotherapy in conjunction with CSRT was significantly worse than the growth of those who received only CSRT. The children who received chemotherapy showed little or no improvement in growth velocity by year 4; those who did not receive chemotherapy had some improvement. CONCLUSIONS: These findings suggest that chemotherapy potentiates the deleterious effects of radiation on growth.

Antineoplastic Combined Chemotherapy Protocols

Growth in children after bone marrow transplantation for advanced neuroblastoma compared with growth after transplantation for leukemia or aplastic anemia.

The linear growth of 26 children with progressive and advanced neuroblastoma treated with high-dose chemotherapy, total body irradiation, and bone marrow transplantation between 1978 and 1988 at the Children's Hospital of Philadelphia was compared with the growth of 33 children who had transplants for leukemia and of 12 who had transplants for aplastic anemia. The mean growth velocity, expressed as a standard deviation score, for the children who underwent bone marrow transplantation for neuroblastoma was -2.83. This was significantly (p less than 0.005) less than the standard deviation scores for children with transplants for acute lymphoblastic leukemia, acute nonlymphocytic leukemia, and aplastic anemia, which were -0.98, -0.07, and -1.05, respectively. A 6-year follow-up study of 32 long-term survivors of cancer revealed that the 11 patients with neuroblastoma continued to grow poorly, whereas a comparison group of 21 survivors of bone marrow transplantation for leukemia had essentially normal growth 2 years after the procedure. Major therapeutic differences between the two groups included the doses of local radiotherapy and the type and number of cytotoxic agents used. In comparison with the relatively mild growth-inhibiting effects of preparative regimens for leukemia and aplastic anemia, the very intensive preparative regimens used in patients with neuroblastoma have significant negative effects on growth.

Anemia, Aplastic

Diagnostic dilemmas. Results of screening tests for congenital hypothyroidism.

The natural history of congenital hypothyroidism has changed drastically since the institution of newborn screening in North America, Europe, and Japan. Before screening was available, diagnosis of this condition was often delayed. The IQ of 65% of all patients with congenital hypothyroidism was below 85, and 19% were profoundly retarded (IQ less than 15). In two large screening programs in which long-term IQ data are available, no child has tested below the IQ of 74. Furthermore, a comparison of affected children treated within the first month of life with matched controls demonstrated no significant difference in IQ scores between the two groups. Widespread screening has lead to the evaluation of over 7 million infants annually. This represents over 1200 fewer children with subnormal intelligence, and approximately 360 children annually who are saved from the effects of profound mental retardation. There is little doubt that newborn screening is one of the greatest advances in diagnosis and treatment of endocrine disease in the newborn period to occur in the past 20 years. However, even experienced screening programs are not perfect, so the practitioner must remain alert to the possibility of undiagnosed hypothyroidism presenting in infancy.

Congenital Hypothyroidism

Diagnostic dilemma. The goiter.

The incidence of goiters in children is about 4% to 5%. The first step in the evaluation is to decide whether the swelling indeed involves the thyroid. After careful examination, one determines if the thyroid is diffusely or focally enlarged. A solitary nodule merits an extensive workup because of the high rate of malignancy. The evaluation of a diffuse goiter proceeds after deciding if the patient is euthyroid, hypothyroid, or hyperthyroid. In most cases, the child is euthyroid and the diagnosis is either CLT or simple colloid goiter. Laboratory tests for thyroid function and antibodies usually make the diagnosis. The hypothyroid patient most likely has CLT, although drugs or goitrogens, dyshormonogenesis, and thyroid resistance are also possible. The hyperthyroid patient usually has Graves' disease. The incidence of malignancy of solitary thyroid nodules is 15% to 40%; therefore, evaluation must be sensitive enough not to miss cancer. Ultrasonography helps to delineate the anatomy and to reveal if the nodule is cystic. Radionuclide scans are useful, as warm or hot lesions are rarely malignant. Cold nodules require further investigation, and in most institutions, this amounts to open biopsy. Fine-needle aspiration may be used if the clinician and pathologist are experienced. With this aggressive approach to thyroid nodules, malignancies are given early treatment. The prognosis is good in most thyroid carcinomas.

Child

True hermaphroditism: sonographic demonstration of ovotestes.

A 15-year-old boy with normal external genitalia presented with severe bilateral gynecomastia, intermittent painful scrotal swelling, and a recent history of scrotal trauma. Ultrasound (US) revealed testicles of normal size with diffusely heterogeneous echotexture due to scattered cysts. Ovotestes were found at surgery and pathologic examination. Bilateral partial gonadectomies were performed. Four months later he had testicular pain and swelling. US revealed bilateral multiseptate cystic masses. After repeat surgery, he is now free of symptoms. The diagnosis of true hermaphroditism and ovotestis should be considered in a phenotypic male adolescent with gynecomastia when testicular parenchyma is heterogeneous at US because of multiple scattered cysts.

Adolescent

Serum adrenal steroid concentrations in premature infants.

To evaluate serum adrenal steroid concentrations in preterm infants, 17-hydroxyprogesterone (17-OHP), 17-hydroxypregnenolone, 11-deoxycortisol, cortisol, dehydroepiandrosterone (DHEA), DHEA sulfate, androstenedione, 18-hydroxycorticosterone, and aldosterone values were determined in 9 sick and 13 healthy premature infants. Serum steroid concentrations were compared to previously reported data from healthy full-term infants. 17-OHP, 11-deoxycortisol, and aldosterone values were higher in sick preterm infants than in healthy preterm infants. Compared to healthy full-term infants, the premature infants-had significantly higher 17-hydroxypregnenolone, 17-OHP, and DHEA sulfate concentrations. Cortisol values were not different between the sick and healthy preterm infants and were similar to full-term values. Aldosterone values were also similar between the premature and the full-term infants. The findings of elevated steroid precursors in preterm infants and low cortisol levels in stressed sick preterm infants may indicate a relative immaturity of adrenal enzyme activity and inadequate adrenal reserve for stress.

17-alpha-Hydroxypregnenolone

The prolactin response to thyrotropin-releasing hormone does not distinguish teenaged males with hypogonadotropic hypogonadism from those with constitutional delay of growth and development.

We attempted to confirm the results of a previous study in which patients with hypogonadotropic hypogonadism (HH) could be readily distinguished from normal adolescents with constitutional delay of growth and development (CDGD) by their lower serum PRL responses to TRH. We compared the PRL responses to TRH of 13 teenaged males with HH to those of 14 teenaged males with CDGD. Although the mean maximum serum PRL concentration after TRH in HH patients (29.5 ng/ml) was significantly less (P less than 0.05) than that in the CDGD subjects (41.1 ng/ml), there was considerable overlap between the 2 groups. Seven of the 13 HH patients had peak serum PRL concentrations in response to TRH that were greater than 25 ng/ml, the lowest value in the CDGD subjects. These results suggest that a normal PRL response to TRH in a male who has delayed puberty does not exclude the diagnosis of HH, but that a subnormal response probably does support that diagnosis.

Adolescent

Pituitary enlargement mimicking pituitary tumor.

Primary hypothyroidism can result in reactive enlargement of the pituitary gland which is indistinguishable from primary pituitary lesions on computerized tomography (CT) scans. The presenting symptoms may be due to pituitary gland enlargement, as in two of the three cases reported here. Therefore, the diagnosis of pituitary hypertrophy or hyperplasia secondary to hypothyroidism must be based on the endocrinological work-up. Following treatment of primary hypothyroidism, the diminution in size of the pituitary gland can be demonstrated with CT.

Adolescent

Propranolol and exercise as a screening test for growth hormone deficiency.

There is a need for a safe, inexpensive, and reliable screening test for growth hormone (GH) reserve. Exercise has been utilized for this purpose but false-negative responses (inadequate GH release in non-GH-deficient patients) has limited the effectiveness of this stimulus as a screening test. Beta-adrenergic blockade (propranolol) was used to enhance the effect of exercise on GH release. Thirty-two non-GH-deficient children and five GH-deficient children were evaluated. All of the non-GH-deficient children responded to propranolol and exercise with serum GH levels exceeding 7 ng/ml. The peak serum GH levels in the five GH-deficient patients did not exceed 4 ng/ml. Propranolol and exercise appears to be an effective screening test for GH function.

Adolescent

Low serum triiodothyronine in patients with anorexia nervosa.

Patients with anorexia nervosa can demonstrate clinical and/or laboratory findings suggestive of reduced thyroid hormone secretion. In this study, the thyroxine (T4) and triiodothyronine (T3) serum concentrations, and thyrotropin (TSH) response to intravenous administration of thyrotropin releasing hormone (TRH) were determined in 6 patients (aged 9 to 15 yr) with anorexia nervosa and the results compared to those found in a group of 15 normal subjects. The mean basal TSH concentration and mean maximum increase in TSH after TRH were comparable to those in the normal subjects. The mean T4 concentration (7.2 mug/100 ml) in the anorexia nerovsa group was slightly but significantly lower than in the normal group (9.5 mug/100 ml). Five of the 6 patients had serum T3 concentrations below the lower limits of normal and the mean T3 concentrations (49.7 ng/100 ml) was significantly lower than in the normal group (106 ng/100 ml). The extremely low serum levels of T3 in these patients with anorexia nervosa suggest that peripheral conversion of T4 to T3 is impaired during chronic starvation.

Adolescent