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1. Growth and Endocrine Data in Children After Brain Tumor and Irradiation

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1. Growth and Endocrine Data in Children After Brain Tumor and Irradiation ENDOCRINE REGULATIONS, Vol. 33, 37 – 53, 2000 37 1. GROWTH AND ENDOCRINE DATA IN CHILDREN AFTER BRAIN TUMOR AND IRRADIATION G. HEUSSLER, K. UTERMANN, H. FRISH, 1E. SCHULLER, 1I. SLAUVE Endocrinology and 1Oncology, Pediatric Department, University of Vienna, Austria Due to advances in therapeutic regimes of malig- cases the thyroid function noramlizes. These stud- nant brain tumors in childhood the survival rates have ies retrospectively analyzed the data of patients af- significantly improve over the last decades. Besides ter various treatment protocols with a wide range neurological problems, long-term survivors of child of irradiation doses and time after the end of treat- brain tumors are at high risk of developing irradia- ment. tion induced hypothalamic-pituitary dysfunction, pri- We recently started an endocrine and auxiologi- mary hypothyroidism and severe growth retardation cal analysis of patients treated for brain tumors in due to diminished growth of the spine after cran- our department and so far treated 18 of such patients. iospinal irradiation and additional hormonal defi- The aim of this study was to define the frequency ciency. In several patients the first clinical sign of and time course of growth- and endocrine deficien- endocrine disturbance is the precocious or early pu- cies in this homogenous groups of patients as de- berty. Thus, the growth velocity may be normal even fined by the rather high irradiation dose combined in spite of the presence of growth hormone deficien- with chemotherapy. On the basis of these data we cy which results in late diagnosis and treatment and further aim to define a prospective protocol for aux- further results in severely affected body height in iological and endocrine investigations. It is essential adulthood. that these patients are seen by an pediatric endocri- In numerous previous investigations, the frequen- nologist from the very beginning, since our experi- cy of endocrine defects, the relation to irradiation nece showed that the correct interpretation of the dose and time after the end of treatment have been growth curve requires sufficient frequency of the data documented, indicating that the growth impairment on body proportions and pubertal development as of the spine and hypothalamic deficiencies may still a basis for the timing of endocrine testing and even- develop after 5-10 years or more, while in several tual hormonal replacement. 2. FINAL HEIGHT AND BODY MASS INDEX AFTER TREATMENT FOR CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA N. BRATANIC, N. URSIC-BRATINA, M. ZERJAV-TANSEK, L. ZALETEL-ZADRAVEC, J. JAZBEC, C. KRZISNIK, T. BATTELINO University Childrens Hospital, Department of Pediatric and Adolescent Endocrinology, Diabetes and Metabolic Diseases, Ljubljana, Slovenia Objective. The growth and weight expressed as boys) long-term survivors of childhood lymphoblas- body mass index (BMI) of 48 patients (28 girls, 20 tic leukemia was retrospectively analyzed. 38 (MEWPE 1999) – ABSTRACT Methods. Height standard deviation score (HSDS) ed with higher radiation dose (2000-3000 cGy) were according to Tanner and body mass index standard more severely affected than boys (P<0.01). In both deviation score (BMISDS) before the treatment and groups treated with different radiation doses signif- at follow-up were calculated. At the time of analysis icant increase of BMISDS was found between the all patients remained in the first remission. Twenty height at diagnosis and at the end of treatment nine patients had cranial radiation with 1200-1800 (P<0.001) with no significant difference between the cGy and 15 with 2000-3000 cGy. Four patients had treatment groups. In the treated girls the menarche no radiotherapy. All patients were treated with stan- occurred significantly earlier in comparison with dard chemotherapy including intrathecal Methotrex- normal girls according to Tanner with no significant at. Mean age (±S.D.) at the diagnosis was 6.1 (±3.60), difference between both radiation regimens used. range 1.1-13.8 years and that at the time of evalua- Conclusions. Significant deterioration of HSDS tion was 17.9 (±3.71), range 13.8-31.5 years. Statis- and increament of BMISDS was observed regard- tical comparisons were made by Student’s paired and less to the radiation dose. The relative roles of crani- unpaired t-test. al radiation and chemotherapy in the disturbance of Results. We observed significant decrease in growth, puberty and body composition, as observed HSDS from diagnosis to the final geight in both ra- in the survivors of childhood ALL, is most liely diation groups (P<0.01), but the decrement in final a multifactorial cumulative role of radiation induced height was similar with both radiation dose regimens. growth hormone failure, early puberty, steroids and The decrement in final height SDS was greater in chemotherapy all having the role. the patients treated at young age (P<0.01). Girls treat- 3. ENDOCRINOPATHIES IN PATIENTS AFTER TREATMENT FOR HODGKIN’S DISEASE AND SARCOMAS IN CHILDHOOD C. KRZISNIK, B. JEREB, N. BRATANIC, N. BRATINA-URSIC, M. ZERJAV-TANSEK, J. ANZIC University Childrens Hospital, Department of Pediatric and Adolescent Endocrinology, Diabetes and Metabolic Diseases, Ljubljana, Slovenia Background. In patients treated for Hodgkin’s 14.0), while that of the patients with SA was 9.5 years disease (HD) and soft tissue or bone sarcomas (SA (range 5.0 to 13.2). in childhood by surgery, chemotherapy and radio- At the time of the follow-up examination the mean therapy, late sequelae including endocrine dysfunc- age of HD patients was 22.2 years (range 17.3 to tion are common. 30.0) and that of SA patients was 18.8 years (range Patients and methods. Standard endocrinologi- 16 to 22). cal tests were used to determine endocrine function The evaluation was performed at least 5 years af- in 23 patients, among them 14 (10 males and 4 fe- ter the treatment, the mean interval being 11.7 years males) being treated for HD and 9 (7 males and 2 (range 5-21) for the patients with HD and 9.3 (range females) for SA.In this group, 8 patients were suf- 6-12) for the patients with SA. fering from sarcoma of the soft tissue and one from Results. Endocrine dysfunction was found in over oestrogenic sarcoma. At diagnosis the mean age of 80 % of all treated, and it was clinically evident only the patients with HD was 10.4 years (range 5.0 to in 26 %. We detected a high prevalence of hypotha- (MEWPE 1999) – ABSTRACT 39 lamic-pituitary-gonadal dysfunction due to gonadal Pituitary dwarfism with diminished levels of lesion comprising 11 (78.6%) patients with HD and growth hormone and IGF-I was found in one patient 7 (77.8%) patients with SA. treated for SA. Thyroid dysfunction was found in 7 (30.4%) pa- Conclusions. According to the results of our study, tients examined, primary latent hypothyroidism in for the patients for HD and SA in childhood the func- 3 patients with HD and in 2 with SA, secondary tion of the hypothalamic-pituitary-gonadal axis and hypothyroidism in one patient with HD and in one of the thyroid gland should be assessed at frequent with SA. There were 2 cases of adrenal dysfunc- intervals in order to detect endocrinological abnor- tion, one in a patient with HD and one in a patient malities and to introduce a replacement therapy when with SA. necessary. 4. ENDOCRINE FUNCTIONS AFTER ANTILEUKEMIC TREATMENT Z. MISIKOVA, L. KOSTALOVA, A. FOLTINOVA, Z. PRIBILINCOVA Childrens Hospital, Comenius University, Bratislava, Slovakia Objective. The aim of this study was to reveal AuxologicFemales (N=31) Males (N=28) potential disturbances in endocrine functions in data adults who were treated with antileukemic therapy H 145-170 cm (m 160 cm) 164-191 cm (m 175 cm) during childhood. MPHT +5.5 to -12.0 cm (m -5.5 cm) +9.0 to -6.0 cm (m -3.5 cm) Patients and methods. We evaluated 59 adoles- W 35-84 kg (m 60 kg) 54-197 kg (m 68 kg) BMI 14/32 (m 24) 17-36 (m 22) cents and young adults (28 males and 31 females) aged 15-25 years (mean 18 years) who underwent inten- sive chemotherapy and cranial irradiation (24 Gy or Conclusions. Median height was lower than the 18 Gy) because of acute leukemia, and were disease average height of general population. Apparently free for a minimum of three years (3-12 years, mean short were 2 males (164 and 165 cm) and 1 fe- 7 years) after the completion of treatment. The fol- male (145 cm). The majority of study subjects did lowing parameters were evaluated: basal auxologic not attain their midparental target height. A strong data (H=final height, W=weight, BMI=body mass tendency to overweight was apparent both in the index, MPTH=difference to midparental target height), male and female subjects. Evidenmtly obese were TSH=thyrotropin, T4=thyroxine, USG=thyroid ultra- 5 females and 3 males. No signs of central hypog- sound, sexual development staging, age of menarche. onadism were found, but two male subjects suf- Results. As shown in the Table, there was no sta- fered from primary hypogonadism after the irra- tisticaly important difference in auxologic parame- diation of gonads. In the majority of females me- ters between those with higher (24 Gy) and lower narche started earlier than the average in our gen- (18 Gy) dose of cranial irradiation. Hypothyroidism eral population. One male and 4 females were was diagnosed in 1 female and 3 males, smaller thy- already parents and had a total of 6 healthy chil- roid volume by USG in 3 males and 1 female. Sexu- dren. Regular evaluation of thyroid functions re- al development was appropriate to the age in all ex- vealed hypothyroidism in 4 subjects which were cept of two male subjects with primary hypogo- then treated by hormonal substitution.
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