1/55. acromegaly: report of two patients with an unusual presentation.The presenting features of functionally active pituitary tumours depend on the specific hormone which is overproduced. growth hormone (GH) producing tumours usually present with the clinical manifestations of acromegaly due to excessive GH secretion or symptoms resulting from mass effects of the enlarging tumour. The changes in physical features and the increase in tumour size are usually insidiously slow and therefore, recognition of the disease is delayed. In this report two patients with acromegaly are described with an atypical presentation due to acute onset of symptoms. The first patient presented with central diabetes insipidus. The diagnosis acromegaly was made on physical examination. The second patient presented with a generalized seizure during sleep. On CT-scanning a large tumour protruding into the left temporal lobe connected to the pituitary gland was seen. immunohistochemistry of the tumour after partial transcranial resection confirmed the clinical diagnosis of acromegaly. At a later stage transsphenoidal resection of the pituitary tumour was performed with full recovery and without loss of pituitary function.- - - - - - - - - - ranking = 1keywords = secretion, hormone (Clic here for more details about this article) |
2/55. Acute adrenal insufficiency during pregnancy and puerperium: case report and literature review.Acute adrenal dysfunction during pregnancy is rare. Nevertheless, adrenal insufficiency can present as an adrenal crisis, and may be life threatening. There is a wide range of clinical symptoms and signs, and the differential diagnosis is challenging. A full adrenal and pituitary evaluation, both structural and hormonal, must be performed to reach the correct diagnosis, and appropriate treatment must not be delayed. A case is presented of acute adrenal insufficiency that occurred 24 hours after a cesarean delivery. The initial symptoms included hypoglycemic seizures and coma. The workup, both hormonal and structural, revealed isolated adrenocorticotrophic hormone deficiency. This considers this case and reviews the differential diagnosis, diagnostic workup, and the treatment of adrenal dysfunction in pregnancy and the puerperium, as well as the obstetric outcome in women suffering from this disorder. TARGET AUDIENCE: Obstetricians & Gynecologists, family physicians. learning OBJECTIVES: After completion of this article, the reader will be able to understand the various presentations of hypopituitarism, the various etiologies of this condition, and the appropriate work up and management of a patient with hypopituitarism.- - - - - - - - - - ranking = 0.13467571201252keywords = hormone (Clic here for more details about this article) |
3/55. Hyponatraemia associated with lamotrigine in cranial diabetes insipidus.We report the cases of two children with cranial diabetes insipidus who were treated with lamotrigine for seizures and who had accompanying changes in desmopressin requirements. Lamotrigine is a new anticonvulsant chemically unrelated to other existing antiepileptic drugs. Studies suggest it acts at voltage-sensitive sodium channels and also decreases calcium conductance. Both of these mechanisms of action are shared by carbamazepine, which can cause hyponatraemia secondary to inappropriate secretion of antidiuretic hormone. It is possible that the effect of lamotrigine on fluid balance in the cases described is also centrally mediated.- - - - - - - - - - ranking = 104.1808856949keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
4/55. Gonadotropin-dependent sexual precocity in a boy affected by pseudohypoparathyroidism.pseudohypoparathyroidism (PHP) is characterized by hypocalcemia and hyperphosphatemia due to PTH resistance. PHP type Ia is due to diminished G(s)alpha activity in several tissues, causing resistance to hormones whose action is mediated by cAMP. Only two cases of males with PTH type Ia who paradoxically showed sexual precocity have been described in the literature. We describe an 11.5 year-old boy affected by PHP without AHO but with associated true precocious puberty, who came to the I.C.U. for tetanic seizures and drowsiness due to severe hypocalcemia. hyperphosphatemia, increased PTH levels and normal 25-OH-vitamin d values were present. Skeletal X-ray showed mild osteopenia. brain MRI revealed symmetric calcifications in basal ganglia and in frontal areas. Thyroid and thyreotropinic function were normal. testosterone levels were in the adult range, as well as basal and stimulated gonadotropin levels. Tanner stage P4, G4; testicular volume 12-15 mi. Molecular cytogenetics studies are now underway to further elucidate the etiology of this form of PHP.- - - - - - - - - - ranking = 0.13467571201252keywords = hormone (Clic here for more details about this article) |
5/55. autoimmunity to munc-18 in Rasmussen's encephalitis.Rasmussen's encephalitis (RE) is a rare disease of the central nervous system characterized by severe epileptic seizures, progressive degeneration of a single cerebral hemisphere, and autoimmunity directed against glutamate receptor subunit, GluR3. We report here the identification of high-titer autoantibodies directed against munc-18 in the serum of a single patient with RE previously shown to have anti-GluR3 antibodies. Munc-18 is an intracellular protein residing in presynaptic terminals, which is required for secretion of neurotransmitters. These findings are consistent with the possibility of intermolecular epitope spreading between GluR3, a postsynaptic cell surface protein, and munc-18, a presynaptic intracellular protein. Immune attack on these two proteins, which participate at distinct steps of synaptic transmission, could act in an additive or synergistic manner to impair synaptic function and lead to seizures and neuronal death.- - - - - - - - - - ranking = 0.73064857597496keywords = secretion (Clic here for more details about this article) |
6/55. exercise induced hypoglycaemic hyperinsulinism.BACKGROUND: hyperinsulinism in childhood is often caused by genetic defects involving the regulation of insulin secretion leading to recurrent episodes of hypoglycaemia. We report two patients with exercise induced hypoglycaemia. methods: Standardised short exercise tests with frequent blood glucose and plasma insulin measurements were performed in the patients and young healthy controls. RESULTS: Short term exercise resulted in insulin induced hypoglycaemia 15 to 50 minutes after the end of exercise. A massive burst of insulin secretion was observed within a few minutes of the start of exercise in both patients. By contrast glucose and insulin concentrations remained unchanged in healthy controls. CONCLUSIONS: Hyperinsulinaemic hypoglycaemia after moderate physical exercise represents a rarely described phenotype of hyperinsulinism with an as yet unknown defect in the regulation of insulin secretion. It should be suspected in individuals with recurrent exercise related syncope or disturbance of consciousness.- - - - - - - - - - ranking = 2.1919457279249keywords = secretion (Clic here for more details about this article) |
7/55. Transient hyponatriemia complicated by seizures after endoscopic third ventriculostomy.We present an infant who underwent endoscopic third ventriculostomy due to symptomatic hydrocephalus secondary to aqueductal stenosis. This is the first reported case of inappropriate secretion of antidiuretic hormone complicated by hyponatriemia and seizures following endoscopic surgery. The possibility of such a neuroendoscopic complication should alert neurosurgeons and close observation of serum electrolytes is highly recommended in the acute postoperative period, particularly in infants.- - - - - - - - - - ranking = 104.1808856949keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
8/55. Transient pseudohypoparathyroidism and neonatal seizure.The case of a neonate is presented who had late onset seizure associated with hypocalcemia, hyperphosphatemia, and raised parathyroid hormone. The infant did not have any stigmata of pseudohypoparathyroidism. The hypocalcemia was initially resistant to calcium therapy, but responded to vitamin d analog therapy. The diagnosis of 'transient neonatal pseudohypoparathyroidism' was entertained, as the infant remained stable and seizure-free with normal serum biochemistry during 8 months of follow-up.- - - - - - - - - - ranking = 0.13467571201252keywords = hormone (Clic here for more details about this article) |
9/55. coma and seizures due to severe hyponatremia and water intoxication in an adult with intranasal desmopressin therapy for nocturnal enuresis.Desmopressin, a synthetic analogue of the antidiuretic hormone, is an effective medication for primary nocturnal enuresis for both children and adults. Its safety is well established. Although it has a favorable side effect profile, because of its pharmacological effect, intranasal desmopressin can rarely induce water intoxication with profound hyponatremia if given without adequate restriction of water intake. The authors describe an adult patient with water intoxication and severe hyponatremia accompanied by loss of consciousness and seizures after 2-day intranasal administration of desmopressin. The present and the previously reported cases emphasize the need for greater awareness of the development of this serious and potentiallyfatal complication. In addition, to adjust the drug to the lowest required dosage, adequate restriction of water intake is recommended, and serum levels of sodium should be measured periodically to allow for early detection of water intoxication and hyponatremia.- - - - - - - - - - ranking = 27.657682348726keywords = antidiuretic hormone, antidiuretic, hormone (Clic here for more details about this article) |
10/55. Efficacy of phenytoin against hyponatremic seizures due to SIADH after administration of anticancer drugs in a neonate.A neonate with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) developed refractory hyponatremic seizures following administration of anticancer drugs. The seizures did not respond to diazepam, phenobarbital, or lidocaine, but resolved immediately with administration of phenytoin. The low water-excretion capacity in neonates should be taken into consideration when fluid loading is attempted, to avoid renal damage upon administration of drugs such as cisplatin that have a potential damaging effect on the kidney. phenytoin could be the therapy of choice for SIADH and resulting seizures in the neonatal period.- - - - - - - - - - ranking = 104.1808856949keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
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