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Hyponatraemia in AIDS.

Hyponatraemia is very common in AIDS patients. It is observed in about 40-50% of hospitalized patients. It may contribute to overall mortality in advanced disease. Vasopressin measurements in these patients basically present two distinct syndromes: hyponatraemia and 'normal' vasopressin levels (i.e. measurable vasopressin) and hyponatraemia with suppressed vasopressin. Hyponatraemia with suppressed vasopressin is very rare and has only been observed in AIDS patients with dementia and primary polydipsia. Hyponatraemia and measurable vasopressin can be also divided into two syndromes. In some patients vasopressin is 'appropriately' elevated, i.e. in those with body fluid losses (diarrhoea) or chronic hypovolaemia (adrenal failure); these patients also present with hyperuricaemia and other signs of low blood volume. In other patients vasopressin is 'inappropriately' elevated in those with no clinical evidence of hypovolaemia (typically characterized by low serum uric acid levels) such as in Pneumocystis carinii pneumonia and other opportunistic infections leading to SIADH. CSWS is a relatively frequent complication in some patients with cerebral infection or tumour. High-dose trimethoprim (for Pneumocystis carinii prevention) acts as an amiloride-like drug and induces a clinical state characterized by hyponatraemia and hyperkalaemia which is indistinguishable from hyporeninaemic hypoaldosteronism. The mechanism of the hyponatraemia caused by other drugs (miconazole, pentamidine, amphotericin, vidarabine) is not as yet known.

Acquired Immunodeficiency Syndrome↗

Acute fatty liver of pregnancy: an experience in diagnosis and management of eight cases.

Objective: To investigate the early recognition and management of acute fatty liver of pregnancy (AFLP) to improve the maternal and fetal survival.Study Design: Eight cases presenting with AFLP managed in our hospital during the past 212 years were studied retrospectively with emphasis on presenting symptoms, laboratory findings, and the time for liver biopsy. Also, to report the maternal and fetus outcomes in such cases.Result: The mean gestational age at onset was 34 +/- 2 weeks (range 30-37 weeks). All cases were primigravida. In the early stages, all presented with malaise, nausea, vomiting, and epigastric distress followed by jaundice in the third trimester of pregnancy. Three of eight presented with polyuria and polydipsia. Laboratory findings: all had raised transaminases and serum bilirubin (2.9-29.9 mg/dL), hypoalbuminemia (22.4-30 g/L), hypofibriogenemia (< 180 mg/dL), prolonged prothrombin time, and prolonged partial thromboplastin time. Maternal complication was frequent, including hepatic encephalopathy (6), ascites (6), hypoglycemia (5), hematemesis (2), postpartum hemorrhage (5), and preeclampsia (4). Cesarean was performed in 3 cases. One mother died of fulminant hepatic failure, the other cases were survival. There were no fetal deaths. Liver biopsy was done in 8 cases. It is suggested that percutaneous liver biopsy should not be done until the coagulation tests become normal, the amounts of ascites decrease and platelet counts increase after delivery.Conclusion: With increasing awareness, especially in the early recognition of AFLP cases and prompt progressive management, including early termination of pregnancy, and using large-dose infusion of fresh frozen plasma or albumine alternatively, the prognosis of AFLP is obviously improved.

Journal Article↗

The perioperative challenge of nephrogenic diabetes insipidus: a multidisciplinary approach.

Nephrogenic Diabetes Insipidus (NDI) is characterised by the inability of the kidneys to concentrate urine in response to arginine vasopressin (AVP). Such patients typically experience polyuria and polydipsia because of this inability to autoregulate their water balance. This provides a perioperative challenge that could lead to a life-threatening situation. This article documents a patient with NDI who underwent an elective bowel re-anastomosis. Two peak serum sodium values were attained. The first when the patient was retaining sodium due to an inappropriate fluid regimen and the second due to hypovolaemia. The literature is reviewed and principles for NDI perioperative management are proposed.

Anastomosis, Surgical↗

[Familial hypomagnesemia with hypercalciuria and nephrocalcinosis. Association with ocular abnormalities].

BACKGROUND: Familial hypomagnesemia with hypercalciuria and nephrocalcinosis is an unusual disease that usually leads to end-stage renal failure. There is no specific treatment and, to a variable degree, patients with this disease present ocular abnormalities. The illness is due to a defect in the reabsorption of magnesium and calcium at the thick ascending limb of Henle because of a mutation of the PCLN-1 gene, which encodes a protein, paracellin-1, which intervenes in the reabsorption of both cations. OBJECTIVE: To review outcome and the incidence of ocular abnormalities in our patients and in cases described in Spain and to compare the incidence found with that in groups from other countries. METHOD: Retrospective study of a group of patients with familial hypomagnesemia with hypercalciuria and nephrocalcinosis diagnosed at a hospital. RESULTS: There were six girls and three boys with clinical symptoms of polyuria, polydipsia, and less frequently, urinary tract infections and lithiasis. All had hypomagnesemia, hypercalciuria and nephrocalcinosis. Five of the patients had renal failure at diagnosis and four underwent transplantation without recurrence. Eight patients had diverse ocular abnormalities. Eighty-one percent of Spanish patients had ocular abnormalities compared with 24 % of those from other countries. There was no evidence of successful medical treatment. CONCLUSIONS: Almost half of the patients presented chronic renal failure at diagnosis and most of the patients reached end-stage renal failure in the second or third decade of life. Normal glomerular filtration rate was found only in patients diagnosed at an early age. The most frequent extra-renal association in Spanish patients (81 %) corresponded to ocular abnormalities. Effective treatment consists of kidney transplantation that completely corrects the tubular disorder.

Adolescent↗

Hypercalcemia in Langerhans' cell granulomatosis with elevated 1,25 dihydroxyvitamin D (calcitriol) level.

A 48-year-old woman presented with a history of premature menopause, polyuria, polydipsia, fever, and diffuse bony tenderness. Her evaluation revealed central diabetes insipidus, hypothalamic amenorrhea, an elevated free calcium on multiple occasions with an elevated 1,25 dihydroxyvitamin D level, and osteoporosis by densitometry. Skeletal series revealed multiple lytic lesions involving the long bones. The diagnosis of Langerhans' cell granulomatosis was made. She was treated with hormone replacement therapy, radiotherapy, and vinblastine, with a dramatic improvement in her pain and a near normalization of her free calcium. Whereas hypercalcemia has been described in several granulomatous disorders and is secondary to unregulated extrarenal production of 1,25 dihydroxyvitamin D, it is, however, extremely rare in Langerhans' cell granulomatosis. This is the first case report of Langerhans' cell granulomatosis with hypercalcemia and documented elevated increased 1,25 dihydroxyvitamin D level that responded to the treatment of her primary disease.

Antineoplastic Agents, Phytogenic↗

Actions of the traditional anti-diabetic plant, Agrimony eupatoria (agrimony): effects on hyperglycaemia, cellular glucose metabolism and insulin secretion.

Agrimony eupatoria (agrimony) has been documented as a traditional treatment of diabetes. Here, the effects of dietary administration of agrimony on streptozotocin (STZ)-diabetic mice and on in vitro glucose uptake and glucose metabolism, and on insulin secretion by BRIN-BD11 cells were investigated. Agrimony incorporated into the diet (62.5 g/kg) and drinking water (2.5 g/l) countered the weight loss, polydipsia, hyperphagia and hyperglycaemia of STZ-diabetic mice. Aqueous extract of agrimony (1 mg/ml) stimulated 2-deoxy-glucose transport (1.4-fold), glucose oxidation (1.4-fold) and incorporation of glucose into glycogen (2.0-fold) in mouse abdominal muscle comparable with 0.1 microM-insulin. In acute 20 min tests, 0.25-1 mg/ml aqueous extract of agrimony evoked a stepwise 1.9-3.8-fold stimulation of insulin secretion from the BRIN-BD11 pancreatic B-cell line. This effect was abolished by 0.5 mM-diazoxide and previous exposure to extract did not adversely affect subsequent stimulation of insulin secretion by 10 mM-L-alanine, thereby indicating that there was no detrimental effect of the extract on cell viability. The effect of extract was glucose-independent and was not evident in BRIN-BD11 cells exposed to a depolarizing concentration of KCl. The ability of agrimony extract to enhance insulin secretion was dependent on use of heat during extract preparation. These results demonstrate the presence of antihyperglycaemic, insulin-releasing and insulin-like activity in Agrimony eupatoria.

Analysis of Variance↗

Hypophyseal non-Hodgkin's lymphoma presenting with diabetes insipidus: a case report.

We report the case of a 64 year old male patient with a history of ischemic heart disease who underwent surgery for an abdominal mass. The histological diagnosis was highly malignant non-Hodgkin's lymphoma. After surgery the patient was admitted to our Department and received 6 courses of chemotherapy according to the COP schedule, followed by radiotherapy to the left upper abdominal region and ipsilateral lung base. The patient achieved partial remission. One month later he began to complain of left axillary lymphadenomegaly, polydipsia and polyuria. A NMR brain scan showed a hypophyseal mass. The patient was treated with DDAVP and chemotherapy with the PRO-MACE protocol; the polyuria and lymphadenomegaly disappeared and the size of the hypophyseal mass reduced markedly. The clinical picture was, therefore, attributed to a hypophyseal localization of the non-Hodgkin's lymphoma, which is a very rare manifestation of lymphomatous spread to the central nervous system. Our case is also interesting because it shows that a favorable outcome can be obtained with chemotherapy, provided that the latter is sufficiently aggressive. This is not necessarily the case with radiotherapy which may also be followed by late and severe neurologic sequelae.

Antineoplastic Combined Chemotherapy Protocols↗

Cytogenetic profile of primary pituitary germinoma.

We present a case of a germinoma in the sellar region of a 10-year-old female patient who presented with a history of polydipsia, polyuria and visual disturbances. The tumor was resected and histologically analyzed. Interphase cytogenetics was performed using chromosome specific (peri)-centromeric DNA probes for all the somatic and X chromosomes on fresh frozen tissues. Fluorescent in situ cell hybridization demonstrated accumulated cytogenetic abnormalities involving significant alterations of chromosome 1, 4, 5/19 and 15. The child was treated postoperatively by radiation and now appears well with only minor neurological deficits. At 3-year follow-up no recurrent tumor mass could be demonstrated.

Child↗

A case of lymphocytic infundibuloneurohypophysitis: histophathological studies.

A 57-year-old woman presented with 2-year history of polyuria and polydipsia. Hormonal studies revealed almost normal anterior pituitary function and central diabetes insipidus. Magnetic resonance imaging showed thickening of the pituitary stalk and enlargement of the neurohypophysis without high intensity of the posterior lobe on T1-weighted images, which were compatible with lymphocytic infundibuloneurohypophysitis. Transsphenoidal biopsy was done and histological examination disclosed moderate fibrosis and lymphocytic infiltration not only in the posterior pituitary, but also in the adjacent anterior pituitary part of the gland. The lymphocytes both in the anterior and posterior pituitary were mainly T cells that were positive for UCHL 1, CD 3, and CD 8. Immunofluorescence of frozen tissue detected immunecomplex deposition in small vessels and the interstitium. These findings suggested that allergic reactions may play an important role in the pathogenesis of lymphocytic infundibuloneurohypophysitis.

CD3 Complex↗

Intra- and suprasellar colloid cysts.

During the past 11 years 69 patients underwent transsphenoidal surgery for symptomatic intra- and suprasellar non-neoplastic cysts in our department. Eighteen of them harbored intra- and suprasellar colloid cysts. The most frequent presenting symptoms were oligomenorrhea, galactorrhea, and headaches. One patient presented with polydipsia. One male patient complained about mild hypogonadism and oligospermia. Two male patients presented with symptoms of panhypopituitarism. Endocrine assessment revealed hyperprolactinaemia in 72% of the female patients. Hypogonadism was found in 72%. Panhypopituitarism was found in two cases. During transsphenoidal surgery, a circumscribed collection of colloid material was removed in each case. Additional tumorous tissue was encountered in three cases that harbored a concomitant pituitary adenoma. Biopsies confirmed the surrounding tissue to be normal pituitary tissue. Postoperatively, regular menstrual cycles were found in 82% of the female patients with oligomenorrhea and headaches improved in 80%. Serum prolactin levels were restored in 92%, galactorrhea ceased in 89%. Only in one case deterioration of pituitary function occurred (diabetes insipidus). Symptomatic SIADH occurred in another one. There were no other post-operative complications. We conclude, that transsphenoidal surgery is a safe therapy for treating symptomatic intra- and suprasellar colloid cysts. Surgery is mainly indicated for female patients in childbearing age to restore fertility and to prevent further deterioration of pituitary function. The differential diagnosis is often unclear preoperatively, but a non-enhancing mass on MRI between anterior and posterior lobe may suggest the presence of an intra- and suprasellar colloid cyst.

Adult↗

Risperidone and hyponatremia: a case report.

A 48 year-old white male not suffering from endocrine disease or polydipsia, not taking diuretics, and suffering from no renal disease was started on risperidone and discharged on no other drug from Western Missiouri Mental Health Center (WMMHC) after an 8-day hospitalization. Seven days later he was admitted to a university medical center with generalized seizures, hyponatremia, respiratory failure, and rhabdomyalysis. He eventually recovered, was transferred back to WMMHC, and stabilized on appropriate medication. A search of the literature indicates no case reports linking risperidone to hyponatremia. It is assumed that the mechanism of hyponatremia is similar to other psychotropic medication in that it is secondary to the syndrome of inappropriate antidiuretic hormone (SIADH).

Antipsychotic Agents↗

[Pseudohypoadrenocorticism in two Siberian huskies with gastrointestinal parasitoses].

Two Siberian Huskies were presented because of anorexia, chronic diarrhoea, polydipsia and polyuria. The most significant clinical finding was a severe dehydration. Laboratory results showed severe electrolyte disturbances with sodium: potassium ratios of 13.2 and 15.4, respectively. Hypoadrenocorticism could be excluded in both dogs with an ACTH stimulation test. The plasma aldosterone concentrations before and after stimulation were very high, which might be due to a stimulation by the electrolyte disturbances. The final diagnosis was an infection with the whipworm Trichuris vulpis and secondary pseudohypoadrenocorticism. A life-threatening dehydration syndrome with hyponatremia, hyperkalemia and metabolic acidosis due to intestinal parasitism has been reported in earlier studies. Up till now sodium: potassium ratios less than 14, however, have only been seen in animals with Morbus Addison. The severe hyperkalemia in these dogs might be due to a particular sensitivity in this breed.

Adrenal Insufficiency↗

[Leiomyoma of a kidney in a dog: a rare diagnosis].

A five-year-old, neutered male, Bernese Mountain Dog was referred because of a two-week history of hematuria and a one-week history of polydipsia and polyuria. Palpation of the dorsomedial mesogastrium revealed a mass on the left side, which was seen via radiography and ultrasonography to be attached to the left kidney. The left kidney and the attached mass were excised surgically, and the dog had an uneventful recovery. Histological examination of the mass revealed a dense proliferation of non-polymorphic spindle cells, which stained positive for smooth muscle actin via immunohistochemistry. Leiomyoma of the kidney was diagnosed. Postoperatively, there was mild azotaemia, which did not affect the general condition of the dog and was still present 15 months after surgery. Leiomyoma of the kidney is a benign tumor, and hematuria may be the only clinical sign.

Animals↗

[Polyuria].

Beyond polyuria following psychogenic polydipsia, in a more narrow sense, this condition may be classified into impaired water re-absorption (i) due to tubular injury or (ii) relative or absolute loss of function of antidiuretic hormone (ADH). Tubular injury may be caused by different toxins affecting the ascending Henle loop as hypercalciuria, drugs and antibiotics as tubular necrosis. ADH deficiency, either absolute or relative, occurs with central or peripheral diabetes insipidus, which is based on synthesis failure or loss of peripheral efficacy of ADH due to receptor malfunction. Diagnosis of polyuria rests upon a thirst challenge in conjunction with laboratory studies of osmolality in serum and urine, which discloses the non-function of the hypothalamic-renal axis. Administration of ADH may differentiate between central and peripheral diabetes insipidus.

Humans↗

Varying clinical presentations at onset of type 1 diabetes mellitus in children--epidemiological evidence for different subtypes of the disease?

OBJECTIVE: On the basis of 2121 case observations between 1987 and 1997, we describe the clinical and laboratory characteristics of diabetes mellitus type 1 at its onset. Our objective is to analyze whether clinical presentation follows a uniform pattern or whether there is evidence for different subtypes. RESEARCH DESIGN AND METHODS: Thirty-one pediatric hospitals and one diabetes center in Baden-Wuerttemberg (BW), Germany, participated in this study. The hospital records of 2121 children below 15 yr of age were examined retrospectively. Statistical analysis was done after logarithmic transformation into a normal distribution. RESULTS: The average duration of symptoms was found to be 15.2 d (95% CI (Confidence Intervals) = 14.3-16.1) ranging between 2.0 and 180 d (95% central range). The most frequent symptoms were polyuria and polydipsia; 7.2% presented with altered level of consciousness. The mean blood glucose value was 407.9 mg/dL (95% CI = 400.0-416.0), corresponding to 23.3 mmol/L (95% CI = 22.8-23.8). The median pH value was 7.35 (95% CI = 7.34-7.36), and the median base excess was -5 mmol/L (95% CI =-5 to -4). The younger patients had a shorter duration of symptoms and suffered most frequently from ketoacidosis. CONCLUSIONS: Although the symptoms of diabetes at its onset follow a uniform pattern, the clinical presentation and duration of symptoms indicate that there may be various forms of type 1 diabetes.

Journal Article↗

Myelodysplastic syndrome in transformation to acute myeloid leukemia presenting with diabetes insipidus: due to pituitary infiltration association with abnormalities of chromosomes 3 and 7.

A 31-yr-old woman with myelodysplastic syndrome (MDS) in transformation to acute myeloid leukemia (AML) presented with initial symptoms of polyuria and polydipsia. Cytogenetics revealed monosomy 7 and translocation (3;3)(q21;q26). The initial symptoms, in conjunction with a low serum level of anti-diuretic hormone (ADH) and magnetic resonance imaging (MRI) findings demonstrating loss of the "bright spot" of the neurohypophysis, indicated diabetes insipidus (DI), e.g. caused by leukemic infiltration of the neurohypophysis. After induction chemotherapy the patient's bone marrow revealed blast persistence, and following a second course of chemotherapy and normalisation of MRI, an allogeneic peripheral blood stem cell transplantation (PBSCT) from the patient's HLA-identical brother was performed, resulting in ongoing complete remission. Recently, Lavabre-Bertrand et al. reported an association of AML with DI, elevated platelet counts, and monosomy 7 and chromosome 3 abnormalities in three patients (Eur. J. Haematol. 2001: 66: 66-69). Our report of an MDS with trilineage dysplasia and these karyotypic changes associated with DI indicates that this new entity may also include preleukemic cases.

Acute Disease↗

The propensity for nonregulatory ingestive behavior is related to differences in dopamine systems: behavioral and biochemical evidence.

Previous research has shown that animals predisposed to eat and drink in response to electrical stimulation of the lateral hypothalamus (ESLH) are similarly predisposed to drink excessively when tested for schedule-induced polydipsia. The eating and drinking elicited by both experimental paradigms appears to be unrelated to homeostatic need and has been called nonregulatory ingestive behavior. In this study, the relation between properties of dopaminergic neural systems and the predisposition to exhibit nonregulatory ingestive behavior was investigated. It was found that rats that eat and drink during ESLH show greater behavioral sensitization to a series of amphetamine injections that those that do not exhibit ingestive behavior during ESLH. In addition, footshock stress produced a greater increase in forebrain dopamine utilization in rats that engaged in nonregulatory ingestive behavior. This evidence is consistent with the hypothesis that there are individual differences in the responsiveness of forebrain dopamine systems that are related to the behavioral predisposition to exhibit nonregulatory ingestive behavior.

Animals↗

Delayed hyperphagia and increased body length after hypothalamic knife cuts in weanling rats.

Four-week-old female rats received parasagittal hypothalamic knife cuts. Polydipsia began right surgery, but hyperphagia, obesity, and excessive nose-anal length did not begin until after the rats were 7-8 wk. old. The delayed onset appears to await some maturational event that is required for the expression of hypothalamic obesity. It appears likely that puberty is the critical maturational event.

Animals↗