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Biomedical subjects

T Moshang

Publications and source records attributed to T Moshang.

At least 37 records · Page 2Linked to original sources
PubMed1996

Growth in children undergoing bone marrow transplantation after busulfan and cyclophosphamide conditioning.

PURPOSE: The purpose of this study was to evaluate the effect of busulfan and cyclophosphamide (BUCY) as conditioning regimen for bone marrow transplantation (BMT) on growth of children following BMT. PATIENTS AND METHODS: Growth assessment was prospectively done in all 25 children who underwent BMT following BUCY conditioning from 1989 to 1994 at Children's Hospital of Philadelphia. The height and growth rates were expressed as standard deviation scores (SDS). The height SDS prior to BMT were compared with 1, 2, and 3 years post-BMT. The growth rate SDS 1 year and 2 years post-BMT were also compared. Pubertal children were excluded from the analysis of growth rate. Median age of patients was 7 years (range, 1.3-15 years). A total of 22/25 patients were transplanted for AML, and three patients had myelodysplastic syndrome. Equal numbers of patients had autologous and allogeneic transplants. Seven patients received corticosteroids for varying lengths of time. The pre-BMT height SDS (mean +/- SD) for the group was -0.4 +/- 1.3. The mean height SDS for 19 children at 1 year post-BMT was +/- 0.1 +/- 1.2 and for 10 children, 2 years post-BMT was -0.3 +/- 1.6. Seven children who were 3 years post-BMT had the mean height SDS of -0.2 +/- 1.5. There was no statistically significant difference between pre-BMT height SDS and 1 year post-BMT (p = 0.49) and 2 years post-BMT (p = 0.42). The mean growth rate at 1 year post-BMT was -0.1 +/- 2.7 and at 2 years post-BMT was -0.9 +/- 2.3. The difference was not statistically different (p = 0.15). Somatomedin-C (insulin growth factor 1, IGF-1) levels were normal in all 13 children tested at 1 year post-BMT. IGF binding protein (BP)-3 levels were done in 10 children at 1 year and were found to be normal in all. Thyroid function studies were done in all patients pre-BMT and 1 year post-BMT and were normal for all. Bone age assessment was appropriate for age in all 14 patients tested at 1 year post-BMT. CONCLUSIONS: There was no evidence of growth failure following BMT with BUCY conditioning in this group of patients.

Adolescent↗
PubMed1996

Arm span as measurement of response to growth hormone (GH) treatment in a group of children with meningomyelocele and GH deficiency.

Children with meningomyelocele (MMC) frequently have impaired linear growth. A number have associated structural brain defects with resultant GH deficiency (GHD). Reproducible measurements of height or length in MMC patients are often hampered by lower limb contractures, spasticity, and scoliosis. Arm span has been proposed as a more reproducible measure of linear growth. Five MMC children documented to have GHD were treated with recombinant human GH (hGH) for 1-3 yr. Their height, arm span, and growth velocity were compared with 32 children with idiopathic GHD treated similarly with hGH. These measures are compared with normal children by being expressed as standard deviation scores. The results of this study indicate that arm span measurements in GHD MMC patients are almost identical to height measurements in idiopathic GHD patients both before and during hGH therapy. The physical condition of children with MMC makes reproducible longitudinal height measurements difficult. Routine determinations of arm span measurements for children with MMC will assist in recognizing growth failure as well as monitoring treatment results.

Adolescent↗
PubMed1996

Safety of recombinant deoxyribonucleic acid-derived growth hormone: The National Cooperative Growth Study experience.

The National Cooperative Growth Study has monitored the safety of recombinant human GH (rhGH) since 1985. Data have been collected from more than 19,000 children representing over 47,000 patient-years of rhGH treatment. Children receiving GH for renal disease were more likely to develop problems such as intracranial hypertension than those with GH deficiency (P < 0.01). Children with idiopathic short stature were less likely to develop slipped capital femoral epiphysis than those with GH deficiency or Turner's syndrome (P < 0.01). There was no evidence of an increased recurrence of leukemia or central nervous system tumors. There were 3 new cases of leukemia in children without known risk factors for developing leukemia and 5 cases in children with known risk factors. Growth deceleration associated with high affinity, high capacity antibodies to GH was found in only 2 of 5039 subjects tested (0.04%). Major adverse events in association with rhGH treatment have been rare, and preexisting medical conditions such as renal insufficiency may affect their frequency.

Adolescent↗
PubMed1995

True hermaphroditism and normal male genitalia: an unusual presentation.

True hermaphroditism, the rarest form of intersex, is usually diagnosed during the newborn period in the course of evaluating ambiguous genitalia. As an exception, we present an unusual case of a 15-year-old boy with phenotypically normal male genitalia and bilaterally descended ovotestes, who was seen for evaluation of intermittent scrotal swelling and pain. Although rare, true hermaphroditism should be included in the differential diagnosis of apparently normal males presenting with similar complaints.

Adolescent↗
PubMed1995

Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery.

The feasibility of radical surgery for astrocytomas of the optic chiasm/hypothalamus has been reported by several groups. Such surgery carries significant risks, however, including permanent damage to the pituitary gland, optic apparatus, hypothalamic structures, and carotid arteries. The benefits of radical surgery, both in terms of efficacy and toxicity, should, therefore, be evaluated against standard therapy, as is usually done for new chemotherapeutic protocols. To this end, a retrospective review was performed of 33 patients treated at Children's Hospital of Philadelphia between 1976 and 1991 who met criteria that would have made them eligible for radical surgery in many centers today, but were treated with either no surgery or conservative surgery (< 50% resection) or biopsy followed by adjuvant therapy with local radiation therapy (29 patients) and/or chemotherapy with actinomycin-D and vincristine (18 patients). The review encompassed all children with a globular enhancing mass of at least 2 cm in the hypothalamic/chiasmatic region, no evidence of optic nerve involvement or involvement of the optic radiations by computerized tomography or magnetic resonance imaging, and follow up of at least 3 years. All but one patient had tissue confirmation of a low-grade or pilocytic astrocytoma. Thirteen of the patients were 2 years of age or younger at diagnosis. Five individuals died: three of tumor progression, one of acute shunt malfunction, and one of intercurrent infection. The remaining 28 were alive at last follow up, a mean of 10.9 years from diagnosis. Twenty-three surviving patients have functional vision in at least one eye, 12 require no endocrine replacement, and 16 are in or have completed schooling with regular academic requirements. If radical surgery is to become standard care for children with low-grade astrocytomas of the hypothalamic/chiasmatic region, long-term survival and functional outcome will have to equal or surpass those of historical controls who were treated conservatively.

Adolescent↗
PubMed1993

Growth hormone deficiency in two siblings with Alström syndrome.

OBJECTIVE: To investigate if there is an endocrinologic explanation for the short stature in patients with Alström syndrome. DESIGN: Patient reports. SETTING: The Children's Hospital of Philadelphia, Pa. PARTICIPANTS: Two siblings with Alström syndrome who were referred to the Endocrine Division for evaluation of obesity, growth, and glucose metabolism. INTERVENTIONS: None. MEASUREMENTS/MAIN RESULTS: The low growth velocity in both patients prompted growth hormone evaluation. The abnormal results of two provocative tests of growth hormone function, as well as low concentrations in frequent overnight sampling of serum growth hormone, were compatible with growth hormone deficiency. However, the patients had advanced bone ages, early normal growth, and normal insulinlike growth factor 1 concentrations. The results of the glucose tolerance tests revealed marked elevation of insulin (5955 pmol/L and 7677 pmol/L) and glucose intolerance consistent with insulin resistance. Pituitary stimulation studies revealed normal thyroid and gonadotropin axes. CONCLUSIONS: Growth hormone deficiency may account for the short stature in some patients with Alström syndrome. The advanced bone age and normal early growth may be due to hyperinsulinism. A specific defect in the signal transduction of insulin action and possibly hormone spillover on another receptor may account for the existence of insulin resistance in the presence of normal growth and growth hormone deficiency.

Age Determination by Skeleton↗
PubMed1993

Growth hormone function and treatment following bone marrow transplant for neuroblastoma.

Previously, we reported that 26 children with stage III or IV neuroblastoma (NBL) treated with BMT grew poorly post-BMT and significantly worse than a comparison group of hematologic BMT patients. Furthermore, unlike the hematologic patients, there was no apparent catch-up growth. Six of these previously reported long-term (> 2 years) NBL patients surviving BMT were evaluated with growth hormone (GH) provocative testing, frequent (every 20 min) overnight GH sampling and IGF-1 determinations. Three of 6 patients were GH deficient based on subnormal responses to provocative stimuli and subnormal pooled 12 h GH values. Only one child had completely normal GH testing and his growth was normal. Four patients were tested with recombinant GH for a period of 12-21 months. Three patients demonstrated an improvement in their growth velocity on therapy. However, the overall response to GH treatment was significantly less than the growth response in children who are GH-deficient due to causes other than BMT. In summary, GH deficiency may be a frequent complication of BMT treatment of NBL. It also appears that the BMT treatment protocol employing total body irradiation and high-dose melphalan may induce GH resistance.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1992

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↗
PubMed1992

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↗
PubMed1991

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↗
PubMed1991

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↗
PubMed1991

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↗
PubMed1989

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↗