ABSTRACT
In the last 20 years, eleven children with adrenocortical functional tumors were treated in the National Children Hospital of Costa Rica. There were nine females and two males and their ages ranged from nine months to 14 years. Eleven patients had features of virilism, five had stigmas of Cushing's syndrome and three hyperaldosteronism. The clinical diagnosis was established given the symptoms, hormonal tests and radiological and imagenological studies. The histologic diagnosis was carcinoma in six by clinical picture in one, and were adenoma in four. Three patients had regional and distant metastases. Four patients with carcinoma were treated by surgery and five received chemotherapy, two of them in presurgical stage, and four received radiotherapy. Two patients with carcinoma are alive and had no evidence of tumor recurrence ten and six years after diagnosis. Five are dead, two of them after partial response to chemotherapy. The four patients with adenoma were cured by complete surgical tumor resection, furthermore one of them received chemotherapy because there was not sure of his histologic benign condition. It is necessary more studies in use of chemotherapy in treatment of this tumors but in our experience CFM, VCR, Epi and Actin is a regimen that appears to be an active combination for the treatment in presurgical stage of adrenal cortical carcinoma.
Subject(s)
Adrenal Cortex Neoplasms , Adolescent , Adrenal Cortex Neoplasms/diagnosis , Adrenal Cortex Neoplasms/physiopathology , Adrenal Cortex Neoplasms/therapy , Child , Child, Preschool , Female , Humans , Infant , MaleSubject(s)
Obesity/complications , Thrombosis/etiology , Adolescent , Blood Coagulation Tests , Child , Female , Humans , Lipids/blood , Male , Obesity/blood , Risk , Thyroid Function TestsSubject(s)
Blood Coagulation , Lipids/blood , Obesity/blood , Blood Coagulation Tests , Child , Disease Susceptibility , Female , Humans , Male , Obesity/complications , Platelet Aggregation , Thrombosis/etiologySubject(s)
Cartilage, Articular/drug effects , Somatomedins/blood , Adolescent , Adult , Animals , Biological Assay , Cartilage, Articular/cytology , Child , Child, Preschool , Culture Media , Humans , Hypopituitarism/blood , Infant , Rabbits , Somatomedins/pharmacology , Starvation/metabolism , Sulfates/metabolism , Thymidine/metabolismABSTRACT
Among six patients with histiocytosis X of long duration and growth retardation of 3 to 4 standard deviations, three had a blunted growth hormone response to stimulation tests, associated to diabetes insipidus. In two of these three children there was a partial catchup of growth without treatment by human growth hormone. The causes and mechanisms of growth retardation in histiocytosis X, the influence of hydroelectrolytic disorders on growth in these patients and their need for treatment with human growth hormone are discussed.
Subject(s)
Growth Disorders/complications , Histiocytosis, Langerhans-Cell/complications , Pituitary Gland, Anterior/physiopathology , Child , Diabetes Insipidus/complications , Female , Growth Disorders/drug therapy , Growth Disorders/physiopathology , Growth Hormone/deficiency , Growth Hormone/therapeutic use , Histiocytosis, Langerhans-Cell/drug therapy , Humans , Male , Water-Electrolyte ImbalanceSubject(s)
Growth Disorders/etiology , Histiocytosis, Langerhans-Cell/complications , Hypopituitarism/complications , Hypopituitarism/diagnosis , Pituitary Function Tests/methods , Age of Onset , Anti-Inflammatory Agents/therapeutic use , Biopsy , Causality , Child , Diabetes Insipidus/complications , Diabetes Insipidus/diagnosis , Diabetes Insipidus/drug therapy , Female , Follicle Stimulating Hormone/blood , Growth Disorders/blood , Growth Disorders/diagnosis , Growth Disorders/drug therapy , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/therapy , Human Growth Hormone/blood , Human Growth Hormone/therapeutic use , Humans , Hypopituitarism/drug therapy , Luteinizing Hormone/blood , Male , Prednisone/therapeutic useABSTRACT
The size of the sella turcica was measured in 66 cases of so-called idiopathic hypopituitary dwarfism, and compared to Silverman's standards. In 55 % of patients the sella was abnormally small for age, and in 45 % abnormally small for height. No significant differences were found from male to female patients nor from isolated somatotropic deficiencies to multiple tropic hormones deficiencies.
Subject(s)
Dwarfism, Pituitary/diagnosis , Sella Turcica/anatomy & histology , Adolescent , Adult , Age Factors , Child , Child, Preschool , Dwarfism, Pituitary/blood , Female , Growth Hormone/blood , Humans , Infant , Male , Sex FactorsABSTRACT
Among 135 consecutive cases of hypopituitary dwarfism, 42 related to detectable intracranial tumour or defect. In 13 cases the tumour had been previously operated and/or irradiated. In 29 others the defect was suggested by neurological abnormalities or headache, skull radiographs, ocular examination or by associated post-hypophyseal deficiency and was demonstrated by pneumoencephalography. When none of these associated abnormalities was found, pneumoencephalography failed to demonstrate any intracranial lesion. Among the 93 so-called idiopathic cases there was a large majority of males (60/93) with a history of birth difficulties (34/60) and especially of breech delivery (23/60). TSH, FSH/LH and ACTH deficiencies were associated to GH deficiency in 81% of patients with detectable intracranial lesions, 57% of male and 39% of female idiopathic cases. The number of patients with idiopathic isolated GH deficiency was similar in boys and girls, suggesting in them the hypothesis of a recessive autosomic genetic defect in spite of the scarcity of familial cases. Peculiar clinical associations may contribute to the diagnosis.
