pituitrin and Histiocytosis--Langerhans-Cell

Diabetes insipidus is a heterogeneous condition characterized by polyuria and polydipsia caused by a lack of secretion of vasopressin, its physiological suppression following excessive water intake, or kidney resistance to its action. In many patients, it is caused by the destruction or degeneration of the neurons that originate in the supraoptic and paraventricular nuclei of the hypothalamus. Known causes of these lesions include: germinoma or craniopharyngioma; Langerhans cell histiocytosis and sarcoidosis of the central nervous system; local inflammatory, autoimmune or vascular diseases; trauma following surgery or accident; and, rarely, genetic defects in vasopressin biosynthesis inherited as autosomal dominant or X-linked recessive traits. Thirty to fifty percent of cases are considered idiopathic. Magnetic resonance imaging (MRI) allows identification of the posterior pituitary hyperintensity and of hypothalamic-pituitary abnormalities. Thickening of the pituitary stalk is the second most common finding on MRI scans in several local inflammatory pathologies and autoimmune diseases or germinoma, but it is not specific to any single subtype. A progressive increase in the size of the anterior pituitary gland should alert physicians to the possibility that a germinoma is present, whereas a decrease can suggest the presence of an inflammatory or autoimmune process. Most children with acquired central diabetes insipidus and a thickened pituitary stalk have anterior pituitary hormone deficiencies during follow-up. Biopsy of enlarged pituitary stalk should be reserved for patients with a hypothalamic-pituitary mass and progressive thickening of the pituitary stalk, since spontaneous recovery may occur.

Topics: Body Water; Diabetes Insipidus; Histiocytosis, Langerhans-Cell; Homeostasis; Humans; Magnetic Resonance Imaging; Pituitary Gland, Posterior; Vasopressins

Topics: Alcohols; Body Water; Central Nervous System Diseases; Child; Diabetes Insipidus; Diabetes Insipidus, Neurogenic; Diuresis; Edema; Ethanol; Histiocytosis, Langerhans-Cell; Homeostasis; Humans; Hyponatremia; Infant; Infant, Newborn; Kidney Diseases; Nicotine; Physiology; Pituitary Gland; Pituitary Gland, Posterior; Potassium Deficiency; Prednisone; Pyloric Stenosis; Vasopressins; Water

Topics: Adult; Antidiuretic Agents; Biopsy; Diabetes Insipidus; Diagnosis, Differential; Female; Histiocytosis, Langerhans-Cell; Humans; Inpatients; Prognosis; Radiography; Risk Factors; Vasopressins

To determine what percentage of diabetes insipidus (DI) in childhood is idiopathic and to assess the natural history of idiopathic DI.. We conducted a retrospective chart review of 105 patients with DI who were born or had DI diagnosed between 1980-1989 at 3 medical centers. A second cohort of 30 patients from 6 medical centers in whom idiopathic DI was diagnosed after 1990 was evaluated retrospectively for subsequent etiologic diagnoses and additional hypothalamic/pituitary deficiencies and prospectively for quality of life.. In the first cohort, 11% of patients had idiopathic DI. In the second cohort, additional hypothalamic/pituitary hormone deficiencies developed in 33%, and 37% received an etiologic diagnosis for DI. Health-related quality of life for all the patients with idiopathic DI was comparable with the healthy reference population.. Only a small percentage of patients with DI will remain idiopathic after first examination. Other hormone deficiencies will develop later in one-third of those patients, and slightly more than one-third of those patients will have an etiology for the DI diagnosed. Long-term surveillance is important because tumors have been diagnosed as long as 21 years after the onset of DI. Quality of life for these patients is as good as the reference population.

Topics: Adolescent; Central Nervous System Neoplasms; Child; Child, Preschool; Cohort Studies; Diabetes Insipidus; Female; Histiocytosis, Langerhans-Cell; Humans; Infant; Male; Peptide Hormones; Prospective Studies; Quality of Life; Retrospective Studies; Vasopressins

Topics: Central Nervous System Diseases; Child; Histiocytosis, Langerhans-Cell; Humans; Nervous System Diseases; Prognosis; Vasopressins

Topics: Adult; Arginine Vasopressin; Cortisone; Diagnosis, Differential; Histiocytosis, Langerhans-Cell; Humans; Hypothyroidism; Male; Mouth Diseases; Periodontal Diseases; Vasopressins

Topics: Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Female; Histiocytosis, Langerhans-Cell; Humans; Intertrigo; Middle Aged; Prednisolone; Skin; Vasopressins

Topics: Administration, Intranasal; Adult; Diabetes Insipidus; Female; Histiocytosis, Langerhans-Cell; Humans; Pregnancy; Pregnancy in Diabetics; Radiotherapy; Vasopressins

We describe a patient with histiocytosis X and compulsive water drinking. The association of diabetes insipidus with histiocytosis X is well recognized, and this patient was initially considered to have diabetes insipidus. It was only after further testing that the proper diagnosis was made.

Topics: Adult; Compulsive Behavior; Diabetes Insipidus; Diagnostic Errors; Drinking; Female; Histiocytosis, Langerhans-Cell; Humans; Kidney Concentrating Ability; Kidney Function Tests; Radiography; Vasopressins; Water

Topics: Arginine; Child; Diabetes Insipidus; Histiocytosis, Langerhans-Cell; Humans; Male; Vasopressins

Topics: Allergens; Anaphylaxis; Animals; Antigen-Antibody Reactions; Child, Preschool; Diabetes Insipidus; Drug Hypersensitivity; Erythrocytes; Hemagglutination Tests; Histiocytosis, Langerhans-Cell; Humans; Immune Sera; Immunization, Passive; Immunochemistry; Immunodiffusion; Immunoglobulin E; Lysine; Male; Oxytocin; Phenylalanine; Sheep; Skin Tests; Vasopressins

Topics: Diabetes Insipidus; Histiocytosis, Langerhans-Cell; Humans; Hypothalamo-Hypophyseal System; Male; Middle Aged; Pituitary Gland; Polyuria; Thirst; Vasopressins

Topics: Child, Preschool; Female; Histiocytosis, Langerhans-Cell; Humans; Periodontal Diseases; Prednisone; Radiography; Tooth Diseases; Tooth Extraction; Vasopressins; Vinblastine

Topics: Alcoholic Intoxication; Calcium Metabolism Disorders; Child; Diabetes Insipidus; Diagnosis; Diuresis; Genetics, Medical; Histiocytosis, Langerhans-Cell; Humans; Kidney Diseases; Measles; Polyuria; Prednisone; Psychosomatic Medicine; Sarcoidosis; Syndrome; Thirst; Toxicology; Urine; Vasopressins; Whooping Cough

Topics: Arginine Vasopressin; Bacitracin; Dental Pulp; Gingiva; Histiocytosis, Langerhans-Cell; Humans; Hydroxides; Neomycin; Pathology; Periodontal Ligament; Periodontium; Polymyxins; Prednisolone; Pulpitis; Ulcer; Vasopressins