1/291. adrenal insufficiency in smith-lemli-opitz syndrome.We describe three unrelated patients with adrenal insufficiency and RSH or smith-lemli-opitz syndrome (SLOS), a disorder due to deficient synthesis of cholesterol. These patients presented with hyponatremia, hyperkalemia, and decreased aldosterone-to-renin ratio, which is a sensitive measure of the renin-aldosterone axis. All patients had profound serum total cholesterol deficiency (14-31 mg/dl) and marked elevation of 7-dehydrocholesterol (10-45 mg/ dl). Two patients were newborn infants with 46, XY karyotypes and complete failure to masculinize; one of these patients also had cortisol deficiency. Both patients died within 10 days of birth of cardiopulmonary complications while on adrenal replacement therapy. The third patient diagnosed with SLOS at birth presented at age 7months with fever and diarrhea and was noted to have profound hyponatremia. This patient is maintaining normal serum electrolytes on mineralocorticoid replacement. We conclude that adrenal insufficiency may be a previously undetected and treatable manifestation in SLOS. We hypothesize that deficiency of cholesterol, an adrenal hormone precursor, may lead to insufficient synthesis of adrenal steroid hormones.- - - - - - - - - - ranking = 1keywords = hormone (Clic here for more details about this article) |
2/291. Dual facets of hyponatraemia and arginine vasopressin in patients with ACTH deficiency.OBJECTIVE: Hyponatraemia is often observed in patients with ACTH deficiency who are thought not to suffer from volume depletion. Their high plasma AVP levels relative to plasma osmolality are presumed to be maintained by non-osmotic mechanisms. We attempted to assess volume status from changes in selected clinical measurements related to body fluid balance in the course of i.v. fluid supplementation and following glucocorticoid (GC) replacement in ACTH-deficient patients, and to interpret plasma AVP levels in the context of the estimated volume status. patients AND DESIGN: This report consists of three parts. First, an ACTH-deficient patient with hyponatraemia and volume depletion who was followed through volume replacement to recovery after GC replacement is described (case report). Secondly, medical records of five ACTH-deficient patients with hypovolaemia and hyponatraemia were surveyed retrospectively to observe changes in serum levels of sodium, uric acid (UA) and haematocrit (Hct) following i.v. fluid supplementation of low sodium content (retrospective study). Thirdly, five ACTH-deficient patients with or without overt dehydration were studied with regard to body weight, blood pressure, serum sodium, total proteins, Hct and blood urea nitrogen before and after GC replacement (prospective study). plasma AVP levels were measured after i.v. fluid supplementation without GC replacement in the patients of the retrospective study, and before and after GC replacement in the patients of the prospective study. RESULTS: The first patient became more hyponatraemic after i.v. fluid supplementation and recovered ultimately from hyponatraemia after GC replacement. In five patients studied retrospectively, the serum sodium levels fell progressively following i.v. fluid supplementation, concurrent with reduction in UA levels and Hct, which indicated the dilutional nature of the hyponatraemia. In the patients observed prospectively, the accumulation of fluid and sodium was indicated by a rise in body weight, blood pressure and serum sodium levels and a decline in Hct and total proteins after GC replacement. plasma AVP levels rose similarly in patients with dilutional hyponatraemia and in patients with borderline hyponatraemia before GC replacement. CONCLUSION: patients with untreated ACTH deficiency may have either of two kinds of hyponatraemia--i.e. borderline hyponatraemia associated with subclinical hypovolaemia, or dilutional hyponatraemia. Similarity of plasma AVP levels in two hyponatraemic states suggests their AVP secretion is regulated by non-osmotic, non-volume mechanisms, possibly released from GC suppression at low plasma osmolality.- - - - - - - - - - ranking = 25.70868950968keywords = secretion, vasopressin (Clic here for more details about this article) |
3/291. Syndrome of inappropriate secretion of antidiuretic hormone associated with idiopathic normal pressure hydrocephalus.A 79-year-old woman suffering from urinary incontinence and unsteady gait was diagnosed as having idiopathic normal pressure hydrocephalus (NPH) with hyponatremia due to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). The concentration of antidiuretic hormone was high while the plasma osmolality was low in the presence of concentrated urine during the episodes of hyponatremia. magnetic resonance imaging (MRI) of the head showed enlargement of the third and lateral ventricles. After ventriculoperitoneal shunt surgery, the symptoms of NPH and hyponatremia improved. It may be possibly explained that mechanical pressure on the hypothalamus from the third ventricle is responsible for hyponatremia.- - - - - - - - - - ranking = 8599.3471970323keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
4/291. Severe hyponatremia caused by hypothalamic adrenal insufficiency.A 60-year-old woman was admitted with severe hyponatremia. Basal values of adrenocorticotropic hormone (ACTH), thyroid hormone and cortisol were normal on admission. Impairment of water diuresis was observed by water loading test. Initially, we diagnosed her condition as the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). By provocation test, we finally confirmed that the hyponatremia was caused by hypothalamic adrenal insufficiency. The basal values of ACTH and cortisol might not be sufficient to exclude the possibility of adrenal insufficiency. Therefore, it is necessary to evaluate adrenal function by provocation test or to re-evaluate it after recovery from hyponatremia.- - - - - - - - - - ranking = 1614.6136288723keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
5/291. Premenstrual attacks of acute intermittent porphyria: hormonal and metabolic aspects - a case report.We report the case of a 38-year-old woman with acute intermittent porphyria (AIP). Following the observation of an acute AIP attack in the patient's father, the diagnosis was established after genetic and biochemical examinations. At the age of 29, eight months after delivery of her first and only child, the patient was hospitalized due to a first proven attack of AIP. In the following years she suffered several premenstrual AIP attacks, with clinical symptoms ranging from abdominal pain to paralysis. One attack was accompanied by an increased urinary catecholamine output, strongly indicating adrenergic hyperactivity. The precipitation of acute episodes by secretion of gonadotrophins and a severe hyponatraemia due to a syndrome of inappropriate anti-diuretic hormone secretion indicated hypothalamic involvement in the pathogenesis of AIP. This patient has experienced an evolution of treatment regimens. At first, acute attacks were treated by i.v. hypertonic glucose. Afterwards propranolol was instituted as a maintenance therapy. Later on, i.v. injections of haem arginate were very successful in resolving acute AIP episodes. However, until therapy with an LHRH analogue was started, the patient continued to suffer premenstrual AIP attacks. These LHRH analogues cause hypothalamic inhibition of gonadotrophin secretion, with stabilization of endogenous ovarian steroid production at a low level, and therefore may be effective in preventing acute exacerbations of this disease. Since this patient went on a fixed regimen of an LHRH analogue combined with the lowest dose oestrogen patch her quality of life has improved substantially and she has not required hospitalization, now for over 3 years.- - - - - - - - - - ranking = 17.903557278687keywords = secretion, hormone (Clic here for more details about this article) |
6/291. Asymptomatic hyponaturemia in a patient with mild head injury due to syndrome of inappropriate diuretic hormone--a case report.hyponatremia is commonly seen in patients with severe and moderate head injury, but it is rarely reported in those with mild head injury. The authors report a patient with mild head injury who presented with data typical of inappropriate secretion of antidiuretic hormone (SIADH), but showed no clinical deterioration. Though the clinical significance of this condition is unclear, the true incidence of this pathology might well be found to be higher than expected, should it receive more clinical and/or serological attention. Continuing clinical assessment will be needed to determine the significance of this condition in relation to that in patients with SIADH following the various causes reported previously.- - - - - - - - - - ranking = 1615.6136288723keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
7/291. Increased sensitivity to thyroid hormone replacement therapy followed by hyponatremia and eosinophilia in a patient with long-standing young-onset primary hypothyroidism.We describe a 51-year-old woman with long-standing young-onset primary hypothyroidism. serum cortisol, adrenocorticotropin, and arginine vasopressin levels were normal, but urinary excretion of 17-hydroxycorticosteroid was decreased. Administration of a very small initial dose of thyroid hormone induced severe acute complications including fever, palpitation, and sweating associated with a rapid decrease in serum thyrotropin level, a dramatic increase in serum alkaline-phosphatase level, and a decrease in serum total cholesterol level. A week later, the late complications of nausea, severe hyponatremia, and eosinophilia occurred. serum cortisol level decreased slightly but remained within normal limits during this hyponatremic period. This rare case suggests that increased sensitivity to thyroid hormone can occur in long-standing primary hypothyroidism with biphasic clinical course of acute thyrotoxic complications followed by severe hyponatremia resembling hypoadrenocorticism.- - - - - - - - - - ranking = 7.9768759375294keywords = vasopressin, hormone (Clic here for more details about this article) |
8/291. hyponatremia-induced metabolic encephalopathy caused by Rathke's cleft cyst: a case report.Rathke's cleft cysts are sometimes associated with aseptic meningitis or metabolic encephalopathy due to hyponatremia. We treated such a case manifest by lethargy, fever and electroencephalographic abnormalities. A 68-year-old man was admitted to our ward after experiencing general malaise, nausea and vomiting and then high fever and lethargy. On admission, he was drowsy and had nuchal rigidity and Kernig's sign. Physically, he was pale with dry, thickened skin. He had lost 5.0 kg of body weight in the last month. His serum sodium was 115 mEq/l. He had a low serum osmotic pressure (235 mOsmol/l) and a high urine osmotic pressure (520 mOsmol/l). His urine volume was 1200-1900 ml/24 h with a specific gravity of 1008-1015. The urine sodium was 210 mEq/l. He did not have an elevated level of antidiuretic hormone. Electroencephalograms showed periodic delta waves over a background of theta waves. With sodium replacement, the patient become alert and symptom free, and his electroencephalographic findings normalized. However, the serum sodium level did not stabilize, sometimes falling with a recurrence of symptoms. magnetic resonance imaging clearly delineated a dumbbell-shaped intrasellar and suprasellar cyst. The suprasellar component subsequently shrunk spontaneously and finally disappeared. An endocrinologic evaluation showed panhypopituitarism. The patient was given glucocorticoid and thyroxine replacement therapy, which stabilized his serum sodium level and permanently relieved his symptoms. A transsphenoidal approach was performed. A greenish cyst was punctured, and a yellow fluid was aspirated. The cyst proved to be simple or cubic stratified epithelium, and a diagnosis of Rathke's cleft cyst was made. The patient was discharged in good condition with a continuation of hormonal therapy. Rathke's cleft cyst can cause aseptic meningitis if the cyst ruptures and its contents spill into the subarachnoid space. Metabolic encephalopathy induced by hyponatremia due to salt wasting also can occur if the lesion injures the hypothalamus and pituitary gland.- - - - - - - - - - ranking = 531.27905267083keywords = antidiuretic hormone, antidiuretic, hormone (Clic here for more details about this article) |
9/291. hyponatremia during therapy with amiodarone.We describe an elderly woman who presented with significant hyponatremia caused by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) during therapy with amiodarone. The hyponatremia resolved after discontinuation of the drug and did not recur during a follow-up period of 6 months. Such an association has previously been reported only once in the medical literature. Since severe hyponatremia can produce significant neurologic damage and is associated with a high mortality rate, clinicians should be aware that this complication may occur during amiodarone therapy.- - - - - - - - - - ranking = 1613.6136288723keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
10/291. Severe life-threatening hyponatremia during paroxetine therapy.hyponatremia secondary to the syndrome of inappropriate secretion of antiduretic hormone (SIADH) is an uncommon complication of treatment with the antidepressants the selective serotonin reuptake inhibitors (SSRIs). These effective anti-depressant agents are becoming widely used because of their favorable side effect profile and their safety in overdose. Although most reports have implicated fluoxetine in causing hyponatremia, there have also been a few reports of hyponatremia associated with paroxetine. We describe an elderly patient with severe life-threatening hyponatremia in association with paroxetine therapy. The present case and the others previously reported emphasize the need for greater awareness of the development of this serious and potentially fatal complication, and suggest that serum sodium concentration should be measured periodically in elderly patients soon after they start taking any agent of the SSRIs, especially during the first 2 to 4 weeks of treatment.- - - - - - - - - - ranking = 1082.8345762015keywords = inappropriate secretion, secretion, hormone (Clic here for more details about this article) |
| | Next -> |