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Thyroid adenoma following treatment of acute lymphocytic leukemia.

Sequelae of the treatment of children with acute lymphocytic leukemia (ALL) include multiple effects on the endocrine system, especially as it relates to growth and puberty. Thyroid dysfunction, and in particular, the occurrence of thyroid neoplasia, has been only rarely described. We report the development of benign thyroid neoplasms in two patients 9 years following the diagnosis and treatment of ALL. Both patients were clinically and biochemically euthyroid with noncystic "cold" nodules found on thyroid scan. In light of these observations, and along with previous reports of malignant thyroid neoplasia in children with ALL, long-term careful observation of children successfully treated for ALL is indicated.

Adenoma↗

Late effects of treatment for germ cell tumors during childhood and adolescence.

PURPOSE: To evaluate the long-term sequelae of treatment for malignant germ cell tumors (GCT) during childhood and adolescence. PATIENTS AND METHODS: Of 128 patients treated for GCT at St. Jude Children's Research Hospital between 1962 and 1988, 73 are long-term survivors (continuously disease-free for > or =5 years after diagnosis), with a median follow-up of 11.3 years). Survivors' ages at diagnosis ranged from birth to 18.3 years (median, 9.2 years); 64% (47 patients) were female. Initial surgical resection was followed by observation for stage I germinomas (n = 2), testicular tumors (n = 13), and selected cases of ovarian or sacrococcygeal tumors (n = 2), and by radiation therapy (RT) for patients with stage II to III germinoma (n = 8). The remaining 48 patients received postoperative chemotherapy (vincristine, dactinomycin, and cyclophosphamide [VAC] +/- doxorubicin, 1962 to 1978; VAC and/or cisplatin, vinblastine, and bleomycin [PVB], 1979 to 1988). RT was added to the chemotherapy for 21 patients. Late complications involving various organ systems and their relationship to treatment were evaluated. RESULTS: More than two-thirds of long-term survivors (n = 50) had at least 1 complication, and half (n = 38) had > 1 organ system affected. The systems most often involved included the musculoskeletal (41% of survivors), endocrine (42%), cardiovascular (16% excluding those who had only abnormal chest radiograph), gastrointestinal (25%), genitourinary tract (23%), pulmonary (19%), and neurologic (16%) systems. High-frequency hearing loss occurred in 58% (11 of 19) of patients treated with cisplatin. Musculoskeletal, gastrointestinal, and urinary tract abnormalities were most frequent in patients whose treatment included RT. CONCLUSIONS: A high frequency of late effects after treatment for pediatric GCT, particularly in patients who received RT, was demonstrated. Treatment sequelae could be anticipated from the intensity and type of therapeutic modalities. Treatment-directed screening evaluations may improve quality of life in long-term survivors of pediatric GCT through timely identification of sequelae that can be prevented or ameliorated.

Adolescent↗

Cyclophosphamide-based, seven-drug hybrid and low-dose involved field radiation for the treatment of childhood and adolescent Hodgkin disease.

PURPOSE: The outlook for children and adolescents with Hodgkin disease (HD) is excellent with combined modality therapy. However, the long-term toxicities of multiagent therapy and radiation therapy remain of concern for these patients with curable disease. In an attempt to reduce long-term toxicities while preserving excellent cure rates, we developed a combined-modality protocol using a modified seven-drug hybrid and low-dose (2,000 cGy) involved field radiation therapy (RT). The hybrid used cumulative doses of alkylating agents and anthracyclines that were lower than those used in previous four-drug regimens and substituted a less leukemogenic agent, cyclophosphamide, for nitrogen mustard. PATIENTS AND METHODS: From 1991 through 1994 a cyclophosphamide, vincristine, procarbazine, and prednisone/adriamycin, bleomycin, and vinblastine hybrid was used to treat 29 patients with HD. Median age was 12 years (range 6-16 yrs). Patients who were postpubertal with early stage disease as determined by surgical staging were excluded. Treatment consisted of four cycles of therapy for stages I and IIA, six cycles for stages IIB and III, and eight cycles for stage IV. Twenty-two patients also received low-dose RT to areas of bulky disease. RESULTS: Twenty-eight patients (97%) had a complete response to chemotherapy. Five patients experienced relapse; two died from disease 27 and 29 months after initial diagnosis; three received additional therapy and are alive with no evidence of disease. Follow-up for all other patients is a median of 56 months (range 24-78 mos) from cessation of therapy and all have remained disease-free. At 5 years follow-up, actuarial disease-free survival is 82%, and the overall survival is 93%. There have been no clinically significant cardiac or pulmonary toxicities and no secondary malignancies. CONCLUSIONS: This therapy has resulted in 5-year overall survival and disease-free survival rates similar to regimens using higher doses of alkylating agents, anthracyclines, and radiation. Longer follow-up will be necessary to fully evaluate disease-free survival, organ damage, and quality of life.

Adolescent↗

Alteration in the nocturnal pulsatile release of GH during the induction of puberty using low dose pulsatile LHRH: a case report.

A 16.4 year old girl with idiopathic hypogonadotrophic hypogonadism had successful induction of puberty by administration of low dose LHRH by pulsatile subcutaneous infusion at night. Peak serum GH was increased with therapy but more importantly, pulsatile secretion of GH which was minimal before treatment, was restored after one week of LHRH treatment.

Adolescent↗

Multicentre study on prevalence of endocrine complications in thalassaemia major. Italian Working Group on Endocrine Complications in Non-endocrine Diseases.

OBJECTIVE: Thalassaemia major is a common and serious medical problem, worldwide. However, there are few data concerning the various endocrine disorders which occur in this condition. We have surveyed 25 Italian centres in order to establish the prevalences and times of onset of endocrine disorders in patients with beta-thalassaemia major. PROJECT AND PATIENTS: A questionnaire specifically designed to investigate the prevalences of endocrinopathies was sent to 25 paediatric and haematology departments. The following data were recorded in the questionnaire: sex, age, height and weight, pubertal status according to Tanner's classification, history of secondary amenorrhoea, type of endocrinopathy and hormone levels at diagnosis, associated complications, serum ferritin level, liver enzymes (ALT) and compliance with treatment. RESULTS: An analysis of data from 1861 patients showed that failure of puberty was the major clinical endocrine problem and was present in 51% of boys and 47% of girls, all over the age of 15 years. Secondary amenorrhoea was recorded in 23% of patients (mean age 18.3 years), primary hypothyroidism in 6.2% (mean age 15.8 years), insulin dependent diabetes mellitus in 4.9% (mean age 18.1 years) and hypoparathyroidism in 3.6% of the patients (mean age 18.7 years). At present, the majority are in the second or third decades of life. The prevalences of hypothyroidism, insulin dependent diabetes mellitus and hypoparathyroidism differed in the various centres, whereas the frequency of pubertal disorders was very similar. CONCLUSIONS: Our study has demonstrated several points. Endocrine evaluation in thalassaemic patients must be carried out regularly, especially in those patients over the age of 10 years with iron overload and poor compliance with chelation therapy. The prevalences of some complications, such as insulin dependent diabetes and hypothyroidism, were lower than previously recorded. Hence, it is to be hoped endocrine complications will be less common in the future, for patients who have started chelation therapy during the first years of life. Because of the improved survival of thalassaemic patients with insulin dependent diabetes, and the high incidence of multiple endocrine complications, it is important to carry out careful follow-up studies for the early detection of any other associated complications to facilitate correct treatment.

Adolescent↗

Kallmann syndrome in two sisters with other developmental anomalies also affecting their father.

The classical features of Kallmann syndrome with anosmia and hypogonadotropic hypogonadism were observed in two sisters aged 13 and 19. They had additional malformations including anosmia, bilateral vesico-ureteral reflux and unilateral hearing loss. One of the girls had unilateral coloboma of the optic nerve. The father had unilateral hearing loss and duplication of the left ureter; he died of an unrecognized coarctation of the aorta. He had no clinical signs of hypogonadism or anosmia. It is suggested that the malformations observed in these patients may be due to a dominant inherited defect of embryonic cell migration, resulting in different phenotypic expressions within the same family, including the Kallmann syndrome.

Adolescent↗

Diabetes in women.

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Diabetes Complications↗

Growth and puberty arrest due to prolactinoma.

A 13-year-old male with prolactin secreting pituitary tumor is described. The unusual features were arrested puberty and growth. Previously reported pediatric patients with prolactinoma are reviewed. The importance of serum prolactin measurement and bromocriptine therapy is emphasized.

Adenoma, Chromophobe↗

Puberty in children with idiopathic growth hormone deficiency on growth hormone treatment: preliminary analysis of the data from the Kabi Pharmacia International Growth Study.

In total, 524 children (335 boys, 189 girls) with idiopathic growth hormone deficiency (GHD) who had entered puberty after at least 1 year of growth hormone (GH) treatment were studied. Spontaneous onset of puberty occurred in 319 boys and 174 girls, and puberty was induced in 16 boys and 15 girls. Median chronological and bone ages at onset of spontaneous puberty were 14.8 and 13.4 years in boys, and 13.3 and 11.8 years in girls, respectively. Age at onset of spontaneous puberty was correlated to age at start of GH treatment. Puberty was induced at a median chronological age of 16.0 years in boys and 14.4 years in girls. Height velocity increased in the year before spontaneous puberty in boys and girls, and rose further to a maximum (median, 8.3 cm/year) in the first year of puberty in boys. In girls, the maximum height velocity occurred in the year before puberty (median, 6.5 cm/year). Height velocity increased in the year before spontaneous puberty in boys and girls, and rose further to a maximum (median, 8.3 cm/year) in the first year of puberty in boys. In girls, the maximum height velocity occurred in the year before puberty (median, 6.5 cm/year). Height velocity during the first year of puberty was inversely correlated to age at puberty onset (r = -0.56, p = 0.0001). After medical induction of puberty, height velocities in the first year of puberty were 5.4 and 3.4 cm/year for boys and girls, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Reference values for height, height velocity and weight in Turner's syndrome. Swedish Study Group for GH treatment.

As Northern Europeans are currently the tallest people in the world, specific growth charts for girls with Turner's Syndrome from this area are needed. Based on height and weight measurements from 598 girls with Turner's Syndrome (372 from the Netherlands, 108 from Denmark, 118 from Sweden) not treated with growth-promoting substances and without signs of spontaneous puberty, we constructed growth charts for height-for-age, height-velocity-for-age, weight-for-age, weight-for-height and Body Mass Index for age. Reference tables and regression equations for mean and standard deviation are provided allowing calculation of Standard Deviation Scores. The height and height velocity curves show a low birth length, gradual deviation from the normal percentile curves without pubertal growth spurt, and a prolonged growth until the early 20s. Mean adult height was 146.9 +/- 7.8 cm. Mean weight-for-age was lower than in normal reference children but height-adjusted weight was higher, except in infancy and early childhood. Further studies are required on the factors influencing the weight-height relationship in Turner's Syndrome.

Adolescent↗