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

The authors report the case of a young man with dissociated anterior pituitary insufficiency affecting somatotropic and corticotropic functions, presenting as hypoglycaemia as a result of the deficiencies mentioned, primary hypothyroidism and, in addition, pulmonary fibrosis of uncertain aetiology, pulmonary biopsy not having been carried out. The aetiology of this double pulmonary and endocrine involvement is presumably the same. Sarcoidosis seems unlikely, whilst histiocytosis X with pulmonary, anterior pituitary and/or hypothalamic and thyroid involvement is much more probable.
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PMID:[Hypoglycemia caused by anterior pituitary insufficiency associated with pulmonary fibrosis. Apropos of a case]. 121 62

Histiocytosis X is a disease of histiocyte proliferation in response to some unknown etiology. Thyroid involvement is extremely rare in the literature. In this paper, we present an 18-year-old female with histiocytosis X with thyroid involvement. This patient had had a goiter with normal thyroid function since 12 years of age. A thyroidectomy was done under the suspicion of thyroid cancer. Pathology revealed histiocytosis X. Hypoparathyroidism and hypothyroidism were noted after the operation and were treated with thyroid hormone, vitamin D and calcium carbonate. This patient also had lesions on the left side of mandible, in the suprasellar region and possibly in the right mastoid. A curettage biopsy of her mandibular lesion was also compatible with histiocytosis X. She was proven to have hypothalamic and pituitary dysfunction including hypogonadism and hypoadrenalism. Her thyroid lesion did not recur after the thyroidectomy. The toothache that she had also experienced subsided after the curettage biopsy of the mandibular lesion. Hypothalamic and pituitary dysfunction were controlled by hormone replacement. Because her disease had been running a benign clinical course, no chemotherapy was given.
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PMID:Histiocytosis X with thyroid involvement: report of a case. 136 23

A case of histiocytosis X involving the thyroid and hypothalamus is reported. A 16 year old female presented with amenorrhoea and diabetes insipidus. She subsequently developed a painful goitre with biochemical hypothyroidism, and stridor. The stridor and goitre responded to cyclophosphamide. Previous publications on the use of cytotoxics in histiocytosis X involving the thyroid are reviewed. We describe for the first time both the ultrasound appearances of the thyroid in this condition and the use of serial volumetric measurements to monitor therapy.
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PMID:Histiocytosis X involving the thyroid and hypothalamus. 192 59

An 18-year-old woman presented with visual disturbance and endocrine dysfunction (diabetes insipidus, delayed puberty, hypothyroidism, hypoadrenalism, and hyperprolactinemia). Computed tomography and enhanced cisternography showed a single hypothalamic mass, which proved at biopsy to be histiocytosis X. Further studies showed the disease to be restricted to the hypothalamus. The patient was treated with hormonal replacement therapy, but her visual acuity continued to worsen. Visual acuity improved after low-dose irradiation of the pituitary fossa and hypothalamus, which also led to disappearance of the mass. This case shows that localized hypothalamic histiocytosis X can be diagnosed and treated successfully before other systems become involved.
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PMID:Histiocytosis X of the hypothalamus. 278 48

In our recent experience, the posttraumatic diabetes insipidus (PT.DI) and idiopathic (I.DI) are the most common forms of central diabetes insipidus (C.DI) in adult patients. The hypothalamo-pituitary function in these patients may be quite heterogeneous. We evaluated this aspect in 32 patients with different forms of C.DI (19 males and 13 females; aged 16-55 yrs): 12 with previous severe cranial or general trauma; 8 with CNS lesion due to Tuberculosis, Sarcoidosis, Histiocytosis X or to other pathogenic noxa (Secondary DI); 12 with idiopathic form. In all we measured ACTH, TSH, FSH, LH, PRL and target hormones (pl. cortisol, T3 T4, Testosterone) in baseline conditions with and without substitutive DDAVP therapy. In all cases the hormonal pattern was within the normal range. In several patients stimulation test with specific releasing factors (TRH, LHRH, oCRH) were carried out. Although basal anterior pituitary function is usually normal in patients with central DI (post-traumatic, idiopathic or secondary), an isolated subclinical secondary or tertiary hypothyroidism can be observed in some cases. Thus, a more accurate, periodical, and complete hormonal evaluation is indicated in some patients. The maintained response of ACTH to CRH (even increased after acute withdrawal therapy) indicated that AVP is not necessary to ensure normal function to the CRH-ACTH axis.
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PMID:[Anterio-hypophyseal function in central diabetes insipidus]. 281 47

A 27 year old woman with histiocytosis X had an unusual initial presentation with features of primary hypothyroidism and a goitre. Diagnosis was made by lung and thyroid biopsies. Endocrine tests showed the presence of hypopituitarism and a discrete suprasellar mass, consistent with hypothalamic histiocytosis X, was demonstrated by computerised tomography. Radiotherapy and chemotherapy arrested the clinical progression of the disease.
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PMID:Primary hypothyroidism and essential hypernatremia in a patient with histiocytosis X. 387 33

A retrospective study was undertaken of 1005 normal contrast-enhanced head computed tomographic (CT) scans in children to determine normal standards for pituitary stalk diameter, basilar artery diameter, and their ratio for age and gender. The pituitary stalk enlarges with age, and girls have larger stalks than do boys after age 8. The pituitary stalk-to-basilar artery ratio is easily estimated visually. Ratios greater than or equal to 1 are unusual in normal children. A ratio greater than or equal to 1 should prompt direct measurement of the stalk and comparison with age-matched normal values. If the stalk measurement is greater than 2 SD above the age-matched mean, it is presumably abnormal and deserves further evaluation for numerous possible pathologic conditions including histiocytosis X, pituitary adenoma, hypothalamic lesions, Rathke cleft cysts, metastases, sarcoidosis, hypothyroidism, and infection.
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PMID:Normal pituitary stalk size in children: CT study. 387 41

A thyroid involvement is reported in a 10 year-old boy with histiocytosis X. It was revealed by goiter and hypothyroidism following diabetes insipidus and growth hormone deficiency. Hypothyroidism was exclusively due to the invasion of thyroid by the tumor, while the other endocrine changes were in favor of the histiocytic infiltration of hypothalamus. The exceptional feature of thyroid involvement in disseminated histiocytosis X is emphasized.
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PMID:[Thyroid involvement in histiocytosis X]. 698 32

A 46-year-old man with diabetes insipidus, hypothyroidism and hypocortisolism was found to have a tumor of the pituitary stalk. After transsphenoidal resection of the tumor substitution of cortisol and thyroxin was necessary. Pathological examination of the lesion revealed an extremely rare granulomatous infiltration classified as Gagel's granuloma (Langerhans' cell histiocytosis). Pre- and postoperative magnetic resonance imaging (MRI) studies, differential diagnosis and treatment of such lesions are presented.
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PMID:Gagel's granuloma (localized Langerhans cell histiocytosis) in the pituitary stalk. 765 92

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


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