Multiple Anterior Pituitary Hormone Deficiencies Research Articles

Histiocytose langerhansienne multiviscérale associée à un syndrome d'interruption de la tige pituitaire

Clinical manifestations of hypothalamic-pituitary Langerhans'cell histiocytosis are commonly, diabetes insipidus and sometimes growth hormone deficiency. Their morphologic characteristics on magnetic resonance imaging are absence of posterior pituitary hyperintensity and thickening of the pituitary stalk. Pituitary stalk transection is characterized on magnetic resonance imaging by the absence of pituitary stalk visibility, hypoplasia of the anterior hypophysis and ectopic posterior pituitary hyperintense signal. This syndrome has been shown to be associated with either isolated growth hormone deficiency or multiple anterior pituitary hormone deficiency, but normal posterior pituitary function. Case report. – We report our experience with a six-year-old boy who had been treated for three years for a multisystem Langerhans'cell histiocytosis with diabetes insipidus and who was admitted because of short stature. Endocrinological examinations demonstrated a profound growth hormone deficiency and a partial central hypocorticism. Magnetic resonance imaging showed pituitary stalk transection and a midline anomaly of the brain (Arnold Chiari type I malformation). Conclusion. – Although, some events of his perinatal history lead to the hypothesis of a malformative origin, the progression of Langerhans'cell histiocytosis affected tissues to fibrosis, suggest that this disease is the cause of the patient's hypothalamohypophyseal lesions.

Growth hormone deficiency with ectopic neurohypophysis: anatomical variations and relationship between the visibility of the pituitary stalk asserted by magnetic resonance imaging and anterior pituitary function.

In GH-deficient children showing ectopic posterior pituitary hyperintense signal (EPP), the anatomical details of the pituitary-hypothalamic region and the relationship between the visibility of the pituitary stalk and anterior pituitary function were studied by magnetic resonance imaging (MRI). The absence or presence of the pituitary stalk was recorded by MRI before and after the injection of gadolinium in 25 children with GH deficiency and EPP at the age of 8.7+/-5.0 yr (16 males and 9 females). Patients were classified into 2 groups according to the presence (group 1; n = 14), or the absence (group 2; n = 11) of pituitary stalk visibility after gadolinium injection. Most patients in group 1 (12 of 14) demonstrated isolated GH deficiency, whereas all but 1 patient in group 2 showed multiple anterior pituitary hormone deficiency. The prevalence of a normally sized adenohypophysis was higher in group 1 than in group 2 (50% vs. 9%; P<0.05). Although the EPP was found at the median eminence in all group 2 patients, it was visualized in group 1 at different levels of the pituitary stalk in 60% of cases (8 of 14; at the proximal end of the pituitary stalk, n = 4; in the middle of the pituitary stalk, n = 2; at the distal end of the pituitary stalk, n = 2). This demonstrates that the ectopic posterior pituitary migration abnormality may be complete or partial. In conclusion, although the pathogenesis of GH deficiency with EPP remains unclear, these results suggest that in cases of GH deficiency associated with ectopic posterior pituitary hyperintense signal, patients with no visible pituitary stalk on MRI after gadolinium injection present a more severe form of the disease in childhood associated with multiple anterior pituitary hormone deficiency, whereas visibility of the pituitary stalk is related to isolated GH deficiency. Nevertheless, careful follow-up of these latter patients is necessary, as the natural history of the disease is not established until adulthood.

Lymphocytic Adenohypophysitis and Lymphocytic Infundibuloneurohypophysitis.

One hundred and twenty-four cases of lymphocytic adenohypophysitis have been reported since 1962. Nearly 60% of the cases in women involved inset of the disease in relation to pregnancy. Headache and visual field defects were the most frequent symptoms. Most patients showed signs of either isolated or multiple anterior pituitary hormone deficiency. ACTH secretion was impaired the most frequently, followed by TSH, gonadotropins, GH and PRL secretion. One third of the cases involved hyperprolactinemia. Tissue from patients with lymphocytic infundibuloneurohypophysitis, also suffering from DI, revealed lymphocytic inflammation limited to the infundibulum, stalk, and neurohypophysis. Twenty of these 124 lymphocytic adenohypophysitis patients developed DI before treatment, and neuroimaging studies revealed thickening of the pituitary stalk in some. At least in a few cases, chronic lymphocytic infiltration occurred in both the infundibuloneurohypophysitis and adenohypophysis. Although both lymphocytic adenohypophysitis and infundibuloneurohypophysitis may be caused by autoimmune disorders, the antigens involved may differ.

EFFECTS OF HUMAN PANCREATIC TUMOUR GROWTH HORMONE RELEASING FACTOR ON GROWTH HORMONE AND SOMATOMEDIN C LEVELS IN PATIENTS WITH IDIOPATHIC GROWTH HORMONE DEFICIENCY

Human pancreatic tumour growth hormone releasing factor (hpGRF-40) 10 μg/kg was administered intravenously to 6 normal young men and 12 adult patients who had presented in childhood with growth hormone (GH) deficiency (7 patients had isolated GH deficiency, 4 had multiple anterior pituitary hormone deficiencies, and 1 had Hand-Schüller-Christian [HSC] disease). hpGRF-40 administration increased serum GH concentrations in all normal subjects and in 3 of 7 patients with isolated GH deficiency and in the 1 with HSC disease; however, the mean serum GH concentration in the patients who responded was less than that of the normal subjects. Somatomedin C concentrations were increased 24 h after a single dose of hpGRF-40 in 8 of 10 patients with GH deficiency. All subjects experienced flushing in response to hpGRF-40. A patient with isolated GH deficiency received O·33 μg/kg hpGRF-40 every 3 h for 5 days. Despite the modest increase in GH in response to a subsequent dose of 10 μg/kg hpGRF-40, serum somatomedin C levels increased within 12 h from 0·06 to 0·1 U/ml and peaked at 0·36 U/ml at 72 h; in addition the patient with HSC disease, treated with hpGRF-40 daily for 5 days, demonstrated an increase in somatomedin C from 0·4 to 0·58 U/ml. The increase after hpGRF-40 in serum GH levels in this patient and the similar or greater responses in 3 of 7 patients suggest that at least some of these patients may have hypothalamic GH-releasinghormone deficiency. hpGRF-40 may be useful in distinguishing pituitary disease from hypothalamic disease. After hpGRF-40 administration serum somatomedin C levels may increase without a change in serum immunoreactive GH concentrations. Further studies are needed to determine whether hpGRF-40 is useful in promoting linear growth in children with GH deficiency.

Hypothalamic-pituitary functions in patients with idiopathic pituitary dwarfism. Further evidence for hypophysiotropic human deficiencies.

Somatotropic, thyrotropic, gonadotropic and corticotropic functions in 10 patients with idiopathic hypopituitary dwarfism (IH) were investigated. The patients were divided into two groups: Group I (5 patients) had normal plasma T4 levels, and Group II (5 patients) had T4 levels of less than 4.6 microgram/dl. In Group I three cases had isolated growth hormone (GH) deficiency and two cases had GH and gonadotropin (Gn) deficiencies; in Group II the 5 cases showed multiple anterior pituitary hormone deficiencies. In Group II, the plasma thyroid stimulating hormone (TSH) was 4.1-9.4 muU/ml and the response to thyrotropin releasing hormone (TRH) was greatly delayed and prolonged, with a maximum after 120 min instead of 15 min. The basal prolactin (PRL) level in Group II was 12-31 ng/ml, which was significantly higher than normal (P less than 0.001). In 4 cases in Group II, the plasma cortisol level increased 120 min after the infusion of lysine-vasopressin, whereas oral administration of metyrapone and hypoglycemia induced by insulin did not increase the plasma cortisol levels. From these findings it is concluded that hypothalamic lesions caused the pituitary hormone deficiencies in 4 Group II cases, and Group I may tentatively be differentiated from Group II by T4 determinations.