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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 44-year-old woman with diabetes insipidus of 3 years duration was found to have histiocytosis X. This was based on clinical, radiological and pathological findings consistent with the diagnosis. Furthermore, she developed spontaneous galactorrhea during the course. Endocrine studies of hypothalamic-pituitary function revealed completely impaired secretion of gonadotropin, growth hormone and anti-diuretic hormone, and possible partial impairment of adrenocorticotropic hormone secretion, while thyroid stimulating hormone secretion remained intact. Persistently elevated plasma levels of human prolactin were also demonstrated, which were unaffected by administration of either thyrotropin releasing hormone, l-DOPA or water loading, but suppressed significantly by CB-154, an ergot alkaloid. These results suggest that abnormalities of the patient's endocrine function may be mainly accounted for by a single hypothalamic lesion.
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PMID:Diabetes insipidus and galactorrhea caused by histiocytosis X. 17 21

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.
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PMID:[Growth retardation in histiocytosis X. Evaluation of anterior pituitary function (author's transl)]. 21 2

A 43 year old man with diabetes insipidus who showed panhypopituitarism and marked hypergammaglobulinemia due to histiocytosis X is reported. His low basal plasma adrenocorticotropin (ACTH) and growth hormone (GH) failed to respond to insulin-induced hypoglycemia. His basal serum thyroid hormone level was below normal and normal basal plasma thyrotropin (TSH) showed a delayed response with normal peak value to TSH-releasing hormone (TRH). Normal basal plasma pituitary gonadotropin also showed a delayed response with normal peak value to luteinizing hormone-releasing hormone (LH-RH). Suppression of plasma prolactin (PRL) by levodopa (l-dopa) was impaired and elevation of basal plasma PRL was noted at the second admission. These results, combined with diabetes insipidus, suggested that the panhypopituitarism in these patients was hypothalamic in origin. The polyclonal hypergammaglobulinemia was characterized by elevated serum IgG and IgE levels which returned to normal after corticosteroid treatment with concomitant clinical improvement. Elevated serum IgE levels, tissue and peripheral eosinophilia, and the effectiveness of corticosteroid therapy support the hypothesis that some allergic mechanism may be involved in the pathogenesis of this disease.
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PMID:A case of histiocytosis X associated with panhypopituitarism and hyperimmunoglobulinemia G and E. 22 67

Pituitary dwarfism is a genetically heterogeneous group of disorders associated with a variety of pathogenetic mechanisms which may involve any level of the hypothalamic-pituitary-somatomedin-peripheral tissue axis. Developmental defects of the pituitary, such as congenital absence of the pituitary, and developmental defects of the hypothalamus, such as anencephaly and holoprosencephaly, have been described. Degeneration of the hypothalamus and pituitary, as in histiocytosis X, may also result in pituitary dwarfism. In "idiopathic" forms of pituitary dwarfism, defects limited to either the hypothalamus or pituitary have been suggested by releasing hormone stimulation studies and it is quite likely that specific defects of the hypothalamus, and pituitary, as well as defects in releasing hormonal synthesis and secretion and growth hormone synthesis and secretion all exist. In Laron dwarfism the basic defect appears to lie in the ability to generate somatomedin, in the Pygmy the defect appears to involve peripheral unresponsiveness to somatomedin. Further pathologic studies of the hypothalamus and pituitary in pituitary dwarfism; analysis of growth hormone and somatomedin cellular receptors; and elucidation of the structure of growth hormone releasing factor and techniques for its synthesis and assay should allow for the delineation of the specific pathogenetic mechanism in each of these syndromes.
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PMID:Hereditary forms of growth hormone deficiency and resistance. 82 85

At the time children with suspected hypopituitarism are seen with a subnormal growth rate, it is expected that testing will demonstrate growth hormone (hGH) deficiency. Seven patients with organic CNS lesions--three with histiocytosis X, one with septo-optic dysplasia, one with neonatal meningitis, one with an anterior encephalocele and meningitis, and one with neurofibromatosis who had normal growth hormone concentrations (greater than 7 ng/mL) despite a subnormal growth rate--were studied. Subsequent retesting 0.5 to 4.6 years later demonstrated the development of growth hormone deficiency. Four of the patients had evidence of other pituitary hormone deficiencies at the time of initial testing whereas two subsequently developed other deficiencies. The initial subnormal growth rate in these children may be secondary to their organic CNS lesion or an evolving hypopituitarism, perhaps with deficient somatomedin generation. These studies point out the need for continued observation and retesting in such patients.
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PMID:Evolving hypopituitarism in children with central nervous system lesions. 640 95

In order to document anterior pituitary dysfunction in patients with biopsy-proven Langerhans cell histiocytosis (LCH) and diabetes insipidus and to correlate this with structural changes on imaging, we performed an insulin tolerance test, enhanced computed tomography (CT), and unenhanced magnetic resonance imaging (MRI) in nine patients. Six of the nine patients had growth hormone deficiency, which in two patients was part of panhypopituitarism and in one was associated with poor cortisol response to insulin hypoglycemia. One patient had an exaggerated growth hormone response and one who had had neck irradiation as an infant, had a high resting thyroid stimulating hormone (TSH) suggesting compensated primary hypothyroidism. All enhanced CTs were abnormal, bony defects being the only abnormality in two patients and opaque mastoids in one. The remaining six patients all had structural changes in the hypothalamic/pituitary region. Unenhanced MRI confirmed the CT findings except in one child who had been treated with radiotherapy in the intervening period, but, in addition, confirmed diabetes insipidus by showing absence of the posterior pituitary bright signal and picked up white matter changes in a child with clinical neurological dysfunction. Our findings indicate that the development of diabetes insipidus in LCH is commonly associated with anterior pituitary dysfunction and is usually associated with structural changes in the hypothalamic/pituitary axis.
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PMID:Anterior pituitary function and computed tomography/magnetic resonance imaging in patients with Langerhans cell histiocytosis and diabetes insipidus. 841 98

Diseases in childhood have an impact on growth. The influence of Langerhans cell histiocytosis (LCH) on growth has never been studied well. Recently a patient with LCH was treated with human growth hormone (GH) because of severe GH deficiency due to LCH involvement of both the hypothalamus and pituitary. This led us to review our charts from 1971 onward for evaluation of the growth patterns in patients with LCH. Here the long-term growth of 22 patients with LCH is reported, the median follow up being 7 years and 1 month. The height data were converted into standard deviation scores (SDS). At diagnosis the mean SDS of patients with isolated LCH at diagnosis was 0.04 and -0.37 in patients with disseminated LCH. Of the total group, 12 patients did not show any influence from the LCH or therapy on their growth. The remaining 10 patients reached, after a minimum of 3 years, a percentile clearly higher than that at diagnosis. However all the ten above mentioned patients, either isolated or disseminated LCH, had a lesion in the facial side of the skull. CONCLUSION. GH deficiency is not a common manifestation of LCH in childhood and GH provocation tests are only indicated when there is a poor or decelerating growth rate. In our patients the number of organs involved and/or the treatment modality did not influence the growth in all but one.
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PMID:Growth of children with Langerhans cell histiocytosis. 852 81

Langerhans cell histiocytosis (LCH) is a rare disorder and may be complicated with hypopituitarism and diabetes insipidus (DI) due to invasion of the hypothalamic-pituitary area. In this study, 10 patients with complete (4) and partial (6) type central DI were found among 125 LCH patients in our hospital records. The water deprivation test, followed by the pitressin test, was performed to confirm DI. Hypothalamic-pituitary endocrine function tests were carried out on these 10 patients at the initial diagnosis and during follow-up. All patients revealed growth hormone insufficiency in the insulin hypoglycemic tolerance test. Four patients had impairment of cortisol secretion, demonstrated by insulin hypoglycemic stimulating test results. Two patients had poor response in the thyrotropin releasing hormone stimulating test. Two patients had only partial responses in the luteinizing hormone releasing hormone test. Four patients had hyperprolactinemia. All patients underwent surgical treatment followed by chemotherapy and/or radiotherapy. One patient completely recovered from the endocrine disorder, 3 patients required smaller doses of desmopressin, and one patient had normal adrenal, thyroid, and gonadal function. Hypothalamic-pituitary disorders in LCH should not be neglected. Treatment of LCH can partially or completely reverse associated endocrine disorders. Therefore, endocrine studies and hormone replacement should be mandatory for patients with LCH.
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PMID:Endocrinological aspects of Langerhans cell histiocytosis complicated with diabetes insipidus. 976 56

Erdheim-Chester disease (ECD) and Langerhans' cell histiocytosis (LCH) are rare granulomatosis-like diseases of unknown etiology which are characterized by lipoidgranulomatous infiltrates in various organs. Contrary to LCH, endocrine and cerebral lesions were infrequently observed in ECD. We report on a patient with the clinical diagnosis of ECD displaying endocrine and cerebral manifestations and skeletal, pulmonary and soft tissue involvement. Disturbance of the endocrine system was revealed by enlargement of the pituitary, partial deficiency of growth hormone (GH), hyperprolactinemia and testosterone deficiency. Cerebral involvement included sinus vein thrombosis, pathologic acoustic evoked potentials, persistence of gadolinium enhancement after magnetic resonance imaging and hypomania. These findings emphasize the importance to assess endocrine and cerebral function in patients with rare granulomatous diseases like ECD and multiorgan involvement.
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PMID:Psychoneuroendocrine disturbances in a patient with a rare granulomatous disease. 1214 90

There have been concerns that growth hormone (GH) therapy may be associated with an increased risk of cancer. Although data are limited and conflicting, one recent report on cancer risk in individuals with no cancer history or risk factors for cancer who were treated with pituitary GH demonstrated a small increased risk of colon cancer and deaths from colon cancer and Hodgkin disease. The data from cancer survivors have consistently shown no increased risk of recurrence of the primary tumor in survivors of all tumor types who are treated with GH. One recent study did show a small increased risk of second solid tumors in survivors previously treated with GH. Limited data suggest that GH therapy is not associated with excess cancer risk in individuals with Langerhans cell histiocytosis and neurofibromatosis type 1. Overall, the clinical data are reassuring, but continued surveillance is mandatory.
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PMID:Growth hormone treatment: cancer risk. 1553 96


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