1/7. 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 = physical (Clic here for more details about this article) |
2/7. Suprasellar and intrasellar paragangliomas.neoplasms of the sellar region are entities with a large differential diagnosis. Although paraganglionic cells have not been demonstrated in the pituitary or adjacent structures, the existence of sellar region paragangliomas is well-documented. To elucidate, in this area the nature of these unusual tumors is relatively difficult. Clinical history, physical examination, radiographic investigation as well as intraoperative gross observation are the same as those of sellar meningioma or pituitary adenoma. immunohistochemistry, using neuroendocrine markers and electron microscopy are the two definitive diagnostic methods to differentiate among these entities. The clinical management, the possible pathogenesis of the tumor, the importance of immunohistochemistry in making the diagnosis and the clinical outcome of these patients are discussed.- - - - - - - - - - ranking = 0.5keywords = physical (Clic here for more details about this article) |
3/7. A family case of nephrogenic diabetes insipidus.Two brothers, patient 1 with fever and vomiting, and patient 2 with failure to gain weight were studied. After 4 hr of water deprivation test, the urinary osmolality of the patient 1 was only 105 mOsm/liter and his body weight showed a 4.6% reduction. In response to desamino-8-D arginine vasopressin intranasal administration, no significant elevation of urinary osmolality of patient 1 occurred. After low dose vasopressin tests, the maximal urinary osmolality of their father was in the normal range, but that of their mother was below the normal range. Moreover, the patients showed no significant increase of urinary osmolality after the same tests. The brothers were diagnosed as nephrogenic diabetes insipidus (NDI) and their mother was diagnosed as a carrier. An early diagnosis of NDI is important, since adequate managements such as low-solute diet with restricted protein and salt intake or such as water intake at frequent intervals can prevent the hyperosmolality which would develop the delayed mental and physical developments. The usefulness of the combination of indomethacin with thiazide diuretics is described.- - - - - - - - - - ranking = 0.5keywords = physical (Clic here for more details about this article) |
4/7. Normal homeostasis of fibrinolysis in nephrogenic diabetes insipidus in spite of defective V2- receptor-mediated responses of tissue plasminogen activator release.Three patients with congenital, nephrogenic diabetes insipidus (NDI) from two unrelated families were tested for haemostatic and fibrinolytic responses to DDAVP infusion and venous occlusion. None of the three patients showed a response of factor viii:C, vWF:Ag or t-PA to DDAVP, a V2-agonist. However, the baseline levels of these factors in the patients' plasma were normal and during venous occlusion a rise in t-PA antigen and t-PA activity was observed in all patients. One patient showed a definite response of the t-PA antigen level to exercise. It is concluded that (extrarenal) V2-receptor-mediated responses are absent in these patients, but that baseline homeostasis and the response to venous occlusion and physical exertion are intact. Presumably, these depend on other mechanisms. This observation denies a central role for vasopressin receptors in the on-demand regulation of clotting and clot dissolving properties of the blood.- - - - - - - - - - ranking = 0.5keywords = physical (Clic here for more details about this article) |
5/7. Nephrogenic diabetes insipidus presenting with infantile hypotonia. A report of 2 cases.Nephrogenic diabetes insipidus usually presents with polyuria, polydipsia, fever, vomiting, dehydration and failure to thrive. However, in infancy polyuria may be absent because of dehydration and reduced glomerular filtration rate. In 2 cases the main presenting feature was hypotonia, with marked head lag. family studies confirmed the X-linked mode of inheritance of the disease; in case 1 the disease appeared to have arisen as a new mutation in the mother, and in case 2 the carrier status was traced back to the great-grandmother. Pitfalls in the diagnosis and detection of the carriers are discussed. Treatment with thiazide diuretics and prostaglandin synthesis inhibitors is effective in reducing urine volumes and polydipsia. The early detection of the disease and adequate management may prevent such complications as megacystis, mega-ureter and hydronephrosis, with resulting renal failure. Mental and physical retardation may also be avoided.- - - - - - - - - - ranking = 0.5keywords = physical (Clic here for more details about this article) |
6/7. diabetes insipidus as an early sign of pineal tumor.A 13-year-old boy's acute onset of diabetes insipidus was the first manifestation of a pineal tumor. One year after the physical symptoms of diabetes insipidus occurred, neurological examinations, tumor markers, and computed tomography brain scan were all normal. Three years later, rapid development of neurological changes was observed with the prevailing symptoms of increased intracranial pressure. The changes were related to a pineal germ cell tumor identified by a high level of beta-human chorionic gonadotropin in plasma and neuroradiological investigations, including computed tomography and magnetic resonance imaging. The clinical course of this case is unusual and we advocate the following: (1) the follow-up of children with diabetes insipidus is important for determining whether or not it is an idiopathic type and (2) elevation of plasma beta-human chorionic gonadotropin and development of neurological signs in patients with so-called idiopathic diabetes insipidus should arouse clinical suspicion of an intracranial germ cell tumor. It should be noted that the brain tumor is significant as the underlying pathology of childhood diabetes insipidus.- - - - - - - - - - ranking = 0.5keywords = physical (Clic here for more details about this article) |
7/7. A case of Rathke's cleft cyst presenting with diabetes insipidus.Rathke's cleft cysts (RCCs) are considered to arise from the remnants of Rathke's pouch, an invagination of the stomodeum. They are classically described as benign epithelium lined intrasellar cysts containing mucoid material, and also found in 2-33% of routine autopsy series. The most common presenting symptoms are visual impairment, hypothalamic dysfunction, hypopituitarism and headache. diabetes insipidus has been described in patients with RCC. Very few cases presented with only diabetes insipidus in adults. To our knowledge, our patient is the first case of RCC presenting with only diabetes insipidus in childhood. A 9-year-old girl presented with diabetes insipidus. The physical, neurological and endocrinological examinations were normal, except for diabetes insipidus. magnetic resonance imaging scan revealed a hyperintense lesion with supra sellar extension in the posterior pituitary both on T1 and T2 weighted images. Subtotal excision of RCC was performed via transsphenoidal surgery. However, diabetes insipidus persisted after the surgery.- - - - - - - - - - ranking = 0.5keywords = physical (Clic here for more details about this article) |