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Biomedical subjects

C Sklar

Publications and source records attributed to C Sklar.

At least 19 recordsLinked to original sources
PubMed1996

Pulmonary function in children treated for rhabdomyosarcoma.

Chemotherapy, radiation therapy, and surgical intervention have markedly improved the survival of patients treated for rhabdomyosarcoma. Unfortunately, the therapy may have deleterious effects on the lung. Pulmonary functions tests were obtained from 17 patients treated for rhabdomyosarcoma because of our concern regarding potential pulmonary dysfunction in this group of patients who had received bleomycin, which is known to be associated with lung injury. Mean age at the time of the diagnosis of rhabdomyosarcoma was 10.1 (+/- 7.2) years (range 0.01-23.5 years). The mean age at the time of pulmonary function testing was 17.0 (+/- 7.5) years (range 5.8-34.0 years). Study patients reportedly had no pulmonary symptoms. Approximately 87% of study patients had a restrictive ventilatory impairment on pulmonary function testing as measured by total lung capacity (TLC) values less than the lower limit of normal. Approximately 70% of study patients had carbon monoxide diffusing capacity (DLCO) values less than the lower limit of normal. There were no significant differences in pulmonary function parameters when male study patients were compared to female study patients. There was a statistically significant lower forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC) ratio (P=0.03) and percent predicted forced expiratory flow at 25-75% of the FVC (FEF25-75; P=0.03) in the group of patients diagnosed with rhabdomyosarcoma over 8 years of age as compared to those individuals diagnosed under 8 years of age. In addition, there were no statistically significant differences in pulmonary function when the variables of sex and age at diagnosis (as outlined above) were studied in combination. In summary, we identified a high incidence of restrictive ventilatory abnormalities in a group of individuals (predominantly children) treated for rhabdomyosarcoma as well as a significantly lower FEV1/FVC ratio and percent predicted FEF25-75 in the group of patients diagnosed with the neoplasm over 8 years of age. Individuals caring for such patients are encouraged to obtain pre- and sequential posttreatment pulmonary function tests.

Adolescent↗
PubMed1996

Growth and final height after treatment for childhood Hodgkin disease.

PURPOSE: To evaluate in children the impact of contemporary treatment for Hodgkin disease on the pattern of growth and final height. PATIENTS AND METHODS: We studied 80 patients (54 males) aged <14 years at diagnosis, with newly diagnosed, pathologically confirmed Hodgkin disease, treated at a single institution. Forty six patients received chemotherapy (CT) + radiotherapy (RT), 23 patients received RT alone, and 11 patients received CT alone. Heights were obtained at diagnosis, at the end of treatment, 1, 2, and 3 years after the end of treatment and at attainment of final height. Heights were converted to age- and sex-adjusted SD scores (SDS). RESULTS: There was a significant change in height SDS at the end of treatment compared to height SDS at diagnosis for all three groups (CT + RT: -0.33, p<0.001; RT: +0.09, p=0.027; CT: -0.24, p=0.012). Over the 3 years following treatment, the rate of change of height SDS was not statistically different between the three treatment groups. Final height SDS was decreased for patients receiving RT + CT (-0.41 SDS, p=0.02; n=31). The change in final height SDS for patients receiving RT or CT only was -0.36 (n=14) and +0.42 (n=4), respectively. Loss of final height SDS correlated with younger age at diagnosis (p=0.005). Patients receiving higher RT doses tended to fare worse (p=0.08). CONCLUSIONS: Pediatric patients treated for Hodgkin disease with the combination of RT and CT suffer a small but significant decrease in their final height SDS. Younger patients and those treated with higher RT doses appear to experience the greatest loss of height potential.

Adolescent↗
PubMed1995

Growth in children after bone marrow transplantation for acute leukemia.

We evaluated the growth of children with acute leukemia who received a bone marrow transplant (BMT) after preparation with hyperfractionated total body irradiation (TBI). Seventy-two patients (27 female and 45 male patients) with acute lymphoblastic leukemia (ALL; n = 39) or acute myelogenous leukemia (AML; n = 33) who were less than 14 years of age at BMT were studied. Before BMT all had received multiagent chemotherapy and 31 had received cranial irradiation (RT). Preparation for BMT included total body irradiation (1,375 cGy [n = 37] or 1,500 cGy [n = 35]). Heights, expressed as standard deviation scores (SDS), were studied up to 4 years post-BMT. The estimated height SDS for the entire group at the time of BMT was -0.28 +/- 0.05 and decreased to -1.11 +/- 0.22 at 4 years post-BMT (P < .0001). Using a growth curve model to compare covariate groups over the period of study, we found that the loss in height SDS was most significant in those patients who received cranial RT before BMT (P = .005). The estimated height SDS for patients treated with cranial RT went from -0.52 +/- 0.20 at transplantation to -1.83 +/- 0.23 4 years later. In contrast, patients who did not receive cranial RT before BMT showed a smaller decrease in height SDS over the 4-year observation period, ie, -0.11 +/- 0.20 decreasing to -0.73 +/- 0.21. Similarly, patients with a diagnosis of ALL had a greater loss of height SDS than those with AML (P = .033). Fifteen of 18 patients tested were found to be growth hormone (GH) deficient; 9 patients were treated with GH and all showed an improvement in growth velocity (P < .0001). We conclude that (1) children with acute leukemia who have received cranial RT and subsequently undergo BMT, primarily those with ALL, are at high risk for growth failure and GH deficiency, and (2) that fractionation of TBI may have a relative sparing effect on growth.

Acute Disease↗
PubMed1995

Thyroid dysfunction following bone marrow transplantation using hyperfractionated radiation.

Thyroid dysfunction has been reported following single dose and fractionated radiation in the context of bone marrow transplantation (BMT). Limited data are available regarding this complication following hyperfractionated radiation. We undertook a retrospective analysis of thyroid function in 150 patients who received BMT at our institution, and who were alive and disease-free for at least 1 year after transplant. There were 100 pediatric patients and 50 adult patients, with a median follow-up of 6.2 years for the whole group. These patients had acute (n = 91) or chronic leukemias (n = 36), severe aplastic anemia (n = 18) or immunodeficiency disorders (n = 5). The majority of the patients received radiation-based cytoreductive regimens including 129 patients who received hyperfractionated total body irradiation (TBI) to a total dose of 1375 cGy or 1500 cGy and 10 patients who received total lymphoid irradiation (TLI) to a total dose of 600 cGy. Twenty two patients of the cohort of 150 patients (14.7%) and 21 of the 139 patients (15.1%) who received hyperfractionated radiation were found to have developed hypothyroidism, 11-88 months after transplant (median 49 months). Eight patients had received 1375 cGy and 12 patients 1500 cGy TBI, while one patient was treated with 600 cGy TLI and one patient was treated with chemotherapy only (busulfan and cyclophosphamide). Three patients had primary thyroid failure with an elevated TSH and a low T4 index, while 19 patients had compensated hypothyroidism with an elevated TSH but a normal T4 index. Six of eight patients with untreated compensated hypothyroidism recovered spontaneously.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Prolactin-secreting macroadenomas in adolescents. Response to bromocriptine therapy.

OBJECTIVE: To report five cases of prolactin (PRL)-secreting macroadenomas in adolescents, including their presentations and responses to bromocriptine mesylate treatment. PATIENTS: Five adolescents (three females and two males) aged between 12.5 and 17 years were diagnosed as having PRL-secreting macroadenomas at the pediatric endocrine service at New York University Medical Center between 1987 and 1989. Presenting complaints included visual field deficits, gynecomastia, and amenorrhea, both primary and secondary. All patients demonstrated some feature of hypogonadism or pubertal arrest. Diagnostic criteria included an elevated serum PRL level (mean, 1670 micrograms/L; range, 610 to 3700 micrograms/L) and visualization of a pituitary tumor that measured greater than 1 cm by either a computed tomographic scan or magnetic resonance imaging (mean size, 2.7 cm; range, 1.4 to 4 cm). INTERVENTIONS: Each patient was treated with bromocriptine mesylate at an oral dose of 7.5 mg/d. The patients continued with that treatment for the duration of the study period. MEASUREMENTS AND RESULTS: Anterior pituitary function was evaluated in four of five patients before treatment. All four were growth hormone deficient. Three patients were also gonadotropin deficient. Thyrotropin (thyroid-stimulating hormone) and corticotropin (adrenocorticotropic hormone) deficiencies were demonstrated in three patients who had multiple pituitary deficits. Follow-up testing included serial PRL measurements and radiographic imaging of tumor size. All patients demonstrated a marked decrease in PRL levels, as well as in tumor size (mean shrinkage, 70%). The three patients who initially had visual field deficits showed significant improvement of vision with bromocriptine therapy. Follow-up study of anterior pituitary function showed significant improvement with bromocriptine treatment in three patients. CONCLUSIONS: Bromocriptine was quite effective in the shrinkage of PRL-secreting macroadenomas in all our patients. It is a noninvasive treatment that can preserve and restore vision, as well as pituitary function, which is integral to continued growth and sexual maturation of the adolescent. Bromocriptine is preferable to surgery or radiation in the treatment of PRL-secreting macroadenomas in the adolescent.

Adolescent↗
PubMed1993

Final height after treatment for childhood acute lymphoblastic leukemia: comparison of no cranial irradiation with 1800 and 2400 centigrays of cranial irradiation.

We analyzed growth and final heights in 127 patients (68 female patients) treated for childhood acute lymphoblastic leukemia. Central nervous system prophylaxis included either no cranial radiation therapy (CRT) (n = 38), irradiation with 1800 centigrays (cGy) (n = 36), or irradiation with 2400 cGy (n = 53). None of the patients received spinal irradiation. Mean (+/- SEM) age at diagnosis was 6.4 +/- 0.25 years, mean height standard deviation score (SDS) at diagnosis was 0.28 +/- 0.12, and mean age at final height was 18.26 +/- 0.19 years. The change in height SDS between diagnosis and achievement of final height was significant for all treatment groups: -0.49 +/- 0.14, no CRT; -0.65 +/- 0.15, 1800 cGy; and -1.38 +/- 0.16, 2400 cGy. Irradiated patients had a greater loss in height SDS compared with the nonirradiated patients (p < 0.01), and those treated with 2400 cGy CRT had a greater decrease in final height SDS than the patients treated with 1800 cGy (p < 0.01). Both younger age and female sex were significantly associated with a greater decrease in height SDS in the patients treated with CRT; girls < or = 4 years of age at diagnosis had a mean loss in height SDS that was more than twice that observed for others treated with the same dose of CRT. Thus, although modern regimens for acute lymphoblastic leukemia (no CRT or 1800 cGy CRT) appear overall to have only a modest impact on final height, patients, especially girls, treated with 1800 cGy CRT at a young age remain at risk for clinically significant growth failure.

Age Factors↗
PubMed1993

Efficacy of insulin-like growth factor binding protein 3 in predicting the growth hormone response to provocative testing in children treated with cranial irradiation.

Recent data suggest that the plasma concentration of insulin-like growth factor binding protein 3 (IGFBP-3) is useful as a screening test for growth hormone (GH) deficiency. In this study, we measured by radioimmunoassay the levels of IGFBP-3 in a group of 20 subjects (12 males) of 5 years and 7 months to 16 years of age undergoing standard GH testing following cranial irradiation. The patients had received 1800 to > 6000 cGY of radiation to the hypothalamic-pituitary region, a median of 2.7 years (range 2-7 years) prior to testing. The IGFBP-3 concentrations were discordant with the results of GH testing 60% (12/20) of the time. Although IGFBP-3 levels were below the mean for age in 14 of 15 GH-deficient (peak GH < 10 micrograms/l) patients, only three of 15 GH-deficient patients had IGFBP-3 concentrations that fell below age-adjusted norms. In contrast, the IGFBP-3 levels were within the normal range in all five patients with normal GH responses. The low sensitivity (20%) of IGFBP-3 in predicting the subjects with abnormal responses was not improved by adjusting the values for bone age or stage of puberty. We concluded that a single plasma determination of IGFBP-3 is not a useful screening test for GH deficiency among patients previously treated with cranial irradiation.

Adolescent↗
PubMed1992

Juvenile autoimmune thyroiditis and mitral valve prolapse.

An increased incidence of mitral valve prolapse (MVP) has been reported in adult patients with autoimmune thyroid disease. The aim of this study was to assess the incidence of MVP in children and adolescents with juvenile autoimmune thyroiditis (JAT). Cardiac echo studies using M-mode, 2D, and Doppler examinations were performed on 23 patients (21 females, 2 males). The patients were studied at a median age of 12 years (range 5-20 years). Only one patient was found to have evidence suggestive of MVP, an incidence (4.3%) similar to that seen in the normal pediatric population. We, therefore, conclude that the incidence of MVP in children and adolescence with JAT is not increased.

Adolescent↗
PubMed1988

Presence of immunoreactive corticotropin-releasing factor in rat testis.

We attempted to demonstrate the presence of immunoreactive (ir) CRF in rat testis by RIA, and by an immunocytochemical technique. The RIA was performed on 27,000 x g supernatants of phosphate buffer extracts of adult rat testes, and revealed a high concentration of irCRF (51 - 74 ng/g testis), with a clear parallelism to the standard curve prepared with synthetic rat CRF. In contrast, the peripheral blood level of irCRF was extremely low (less than 0.05 ng/ml). Immunocytochemical studies of irCRF revealed strong specific staining in the Leydig cells and germ cells in normal adult rat testis. Epididymal spermatozoa also stained positive. Testicular irCRF fluctuated significantly with age. The levels (mean +/- SD) assayed in 10, 20, 60 and 90-day-old rats were 41.6 +/- 4.7, 8.7 +/- 0.2, 55.5 +/- 3.3 and 71.3 +/- 3.4 ng/g testis, respectively (P less than 0.01). The level was drastically reduced after abdominal translocation of a testis (9.6 +/- 1.3 ng/g testis), and after hypophysectomy (16.7 +/- 1.6 ng/g testis) in adult rats. However, neither hemicastration nor unilateral cryptorchidism influenced the irCRF levels in the contralateral testis. These data suggest a local production of irCRF which may play a role in regulation of Leydig cell function and sperm maturation in testis and epididymis.

Animals↗
PubMed1985

Chronic syndrome of inappropriate secretion of antidiuretic hormone in childhood.

Chronic forms of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) occur infrequently in childhood. We describe three pediatric patients with chronic SIADH. In two patients the persistent SIADH was associated with neoplasms of the central nervous system while in the third patient it was due to congenital anomalies of the central nervous system. We describe the clinical course and management of each patient, including the long-term use of furosemide taken orally once a day.

Child, Preschool↗
PubMed1985

Intraocular lenses and anticoagulation and antiplatelet therapy.

Questionnaires were sent to 200 members of the American Intra-Ocular Implant Society to assess the preoperative, intraoperative, and postoperative management trends with intraocular lens implant patients on warfarin sodium and aspirin therapy. Responses were received from 135 members. A sizable majority of practitioners (75%) withhold warfarin sodium both prior to and following surgery. In many cases, the medicine is withheld for longer than is necessary, increasing the risk of medical complications. Respondents slightly favored (53%) discontinuing aspirin, which is perceived to be less harmful than warfarin sodium, preoperatively. Although management decisions must be individualized, according to both the surgeon's technique and the particular patient, certain general guidelines are applicable.

Anticoagulants↗