[Spinal and appendicular osteoporosis in streak gonad syndrome. Photon absorptiometric and roentgen-morphometric studies]. / Axiális és appendikuláris osteoporosis csíkgonád szindrómában foton abszorpciometriás és röntgenmorfometriás vizsgálatok.

Patterns of bone loss in the axial and the appendicular skeleton were studied in 48 women with streak gonad syndrome (age: between 14-38 years, mean: 25.5 years). Bone mineral content was measured in vivo at the lumbar spine and right femoral neck by dual photon absorptiometry and at the midradius and distal radius by single photon absorptiometry. The bone mineral status of 20 patients was evaluated by radiomorphometrical indices of the metacarpals, femorals and lumbar spine, too. Mean bone mineral content and radiomorphometrical indices were significantly lower in patients with streak gonad syndrome than in age-matched normal subjects at all three scanning sites. The patients lost cortical and trabecular bone mineral content at the same rate, suggesting that bone loss in streak gonad syndrome is a generalized phenomenon. In spite of serious bone loss osteoporotic fractures were not observed in the patients, in contrast to patients with pathological post-menopausal osteoporosis having equal degree of bone deficiency. The authors did not find any relationship between the occurrence of osteoporosis and the karyotype of the patients. It is suggested that the osteoporosis in streak gonad syndrome--at least after puberty--is a consequence of gonadal hormone deficiency.

Serum bone Gla protein in streak gonad syndrome.

Osteoporosis is one of the most common complications of streak gonad syndrome (SGS), however its pathogenesis is still unclear. Bone Gla protein (BGP) has been found to be a serum marker of bone turnover in various metabolic disease states. In the present study serum BGP and alkaline phosphatase (AP) were measured in 13 osteoporotic patients with SGS and in 56 healthy women. Mean (+/- SD) serum BGP levels were normal (7.5 +/- 2.0 ng/ml) in seven patients who had been on estrogen-progestin replacement therapy and became significantly elevated (P less than 0.001) 2 and 3 months after discontinuation of the treatment (15.3 +/- 2.3 and 13.2 +/- 1.0 ng/ml, respectively). Mean (+/- SD) serum AP (207 +/- 65 U/l) showed significant increases (P less than 0.05) 2 months after withdrawal of hormonal substitution (287 +/- 74 U/l). Mean (+/- SD) serum BGP (15.4 +/- 3.5) and AP (287 +/- 49) levels were significantly higher (P less than 0.001 and less than 0.05, respectively) in six patients with SGS who had not been on hormonal substitution. These findings are consistent with those obtained in postmenopausal women suffering from "high remodelling osteoporosis" and suggest that bone turnover in osteoporotic patients with SGS is increased and the skeletal loss is a consequence of accelerated bone loss rather than decreased bone formation.

Characteristics of cholinergic neuroeffector transmission of ganglionic and aganglionic colon in Hirschsprung's disease.

Differences in the release and content of acetylcholine and the alpha 2 adrenoceptor mediated interaction between noradrenergic and cholinergic neurons were investigated by neurochemical and pharmacological methods in aganglionic and ganglionic segments of isolated human colon taken from children suffering from Hirschsprung's disease. Both at rest and during transmural stimulation the release of acetylcholine was significantly higher in the spastic (aganglionic) segment than in the proximal dilated bowel. Significant differences were found in the tissue concentration of acetylcholine between ganglionic and aganglionic specimens. The pattern of response to transmural stimulation was also different in the spastic and dilated bowel. Transmural stimulation induced relaxation and contraction in ganglionic specimens but only contractions in aganglionic specimens. The sensitivity of the smooth muscle in the aganglionic portion to exogenous acetylcholine and to field stimulation was found to be higher than in the ganglionic portion. While noradrenaline added to the organ bath reduced the stimulation-evoked release of acetylcholine from spastic segments, via an alpha 2 adrenoceptor mediated process, yohimbine did not enhance the release. It is suggested that in Hirschsprung's disease the increased acetylcholine release, the enhanced sensitivity of smooth muscle cells to acetylcholine, and the lack of alpha 2 adrenoceptor mediated noradrenergic modulation of acetylcholine release from cholinergic interneurons might be responsible for the spasm of aganglionic segments.

Calcitonin secretion in streak gonad syndrome (Turner's syndrome).

Osteoporosis in one of the most common complications of streak gonad syndrome (SGS), however, its pathogenesis is still unclear. To test whether SGS is associated with calcitonin (CT) deficiency, 11 affected individuals and 8 age-matched healthy women were studied. Calcium, 3.6 mg/kg b.w. as a 10% solution of calcium chloride, was given intravenously for 3 minutes. Serum levels of CT and calcium were measured before and at 5, 30, 60, and 120 minutes after the injection. There was a statistically significant rise in serum calcium levels both in the control subjects and patients with SGS, with significantly lower levels prior to and at 30, 60, and 120 minutes following calcium load in the control group. The CT rise following calcium load was also significant at 5, 30, and 60 minutes in the controls and at 5 and 30 minutes in patients with SGS, with a significantly lower baseline and 30, 60, and 120 minutes levels in the latter group. Maximum levels of calcium and CT occurred 5 minutes after the calcium load and were statistically indistinguishable. There were no significant differences in either the calcium or the CT incremental changes between the two groups. These findings are consistent with decreased basal (and 30-120 minute) CT levels in SGS and suggest that CT deficiency may be involved in the development of osteoporosis in patients with SGS. The possible causal relationship of estrogen deficiency to the reduced CT levels in SGS is discussed.

[Gonadotropin-secreting liver cancer in ulcerative colitis]. / Gonadotropin-sezernierendes Leberkarzinom bei Colitis ulcerosa.

The authors present the clinical and pathological data of a 28 year-old female patient, who died after several months of observation. The patient had a 15-year history of mild ulcerative colitis. On admission the diagnosis of primary sclerosing cholangitis was made, but subsequently paraneoplastic gonadotropin secretion became apparent. At autopsy a cholangio- and hepatocellular carcinoma was found and the extrahepatic bile ducts were infiltrated by the cholangiocarcinoma. Using the immunoperoxidase method, the hepatocellular part of the tumour was found to contain human choriogonadotropin.

Sulpiride and thyrotrophin releasing hormone loading test in patients with acromegaly.

The effects of sulpiride, a dopamine receptor blocking agent, and of thyrotrophin-releasing hormone (TRH) were studied on prolactin secretion in 17 patients with acromegaly and 25 control subjects. THe basal serum prolactin level was increased in 9 acromegalic patients. After sulpiride loading, maximum changes in prolactin level were significantly smaller in acromegalic patients, irrespective of the basal prolactin concentration. TRH loading resulted in a decrease of prolactin secretion predominantly in the patients with high basal serum hormone levels. A blunted thyrotrophin response to TSH was found only in those acromegalic patients who had concomitant thyroid disease. Higher than normal basal prolactin levels in some of the acromegalic patients and abnormal prolactin responses following sulpiride and TRH loading in most of the patients with acromegaly are attributed to deranged hypothalamo-hypophyseal regulation.

Histamine receptors in the guinea pig ileum.

Histamine and some related compounds acting selectively on H2-or H1 receptors were tested for their ability to contract the guinea pig ileum, in the usual whole ileum preparation and in the longitudinal muscle preparation. The concentrations elicited by histamine in both kinds of preparations were not potentiated by cimetidine or metiamide and were not inhibited by administration of H2 receptor selective agonists in doses which were subthreshold for contracting the guinea pig ileum; higher doses of the H2 agonists could actually potentiate the effect of histamine. The results obtained suggest that H2 receptors with relaxing effect do not occur in the guinea pig ileum or at least that they are not involved in the contraction of the longitudinal muscle layers. The possibility that a sub-type of H2 receptors with properties different from those of the "classical" H2 receptors so far known, exists in the guinea pig ileum, cannot be excluded.

Effect of prostaglandin E1 and indomethacin on responses of longitudinal muscle of guinea-pig ileum to cholecystokinin.

The effect of prostaglandin E1 (PGE1) and indomethacin (IND) cholecystokinin (CCK)-induced contractions of guinea-pig isolated ileum longitudinal muscle were studied. PGE1 (2.8--28 nM) consistently and dose dependently increased the contractions evoked by CCK (indirect muscle stimulation) or by ACh. IND (2.7 microM) decreased the contractions to both compounds and this was reversed with 2.8--7 nM PGE1. Pretreatment of the preparations with phentolamine (2.6 microM) or pretreatment of the animals with reserpine (2 mg/kg i.p. 24 h before killing) did not affect PGE1 potentiation or IND inhibition of CCK-induced contractions. The results indicated that PGE1 potentiated CCK-induced contractions of the longitudinal muscle of guinea-pig ileum by increasing the response to released ACh. Experiments with IND suggested that endogenous PGs may modulate the effect of CCK or related gastrointestinal hormones.