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PubMed2005

Diencephalic syndrome: a cause of failure to thrive and a model of partial growth hormone resistance.

Diencephalic syndrome is a rare but potentially lethal cause of failure to thrive in infants and young children. The diencephalic syndrome includes clinical characteristics of severe emaciation, normal linear growth, and normal or precocious intellectual development in association with central nervous system tumors. Our group initially described a series of 9 patients with diencephalic syndrome and found a reduced prevalence of emesis, hyperalertness, or hyperactivity compared with previous reports. Also, the tumors were found to be larger, occur at a younger age, and behave more aggressively than similarly located tumors without diencephalic syndrome. We have been able to extend our follow-up of the original patients, as well as describe 2 additional cases. Because the mechanism of the growth and endocrinologic findings in diencephalic syndrome has not been explained, we report on these patients in light of current research on hypothalamic factors that affect growth and weight. This study emphasizes diencephalic syndrome as a model for additional study of growth hormone resistance and metabolic regulation of adiposity.

Astrocytoma↗
PubMed1987

[Russel's syndrome. Report of a case with development of precocious puberty].

The Russel's syndrome is characterized by emaciation and hyperactivity in children and is caused by hypothalamic expansive process. This report presents the typical signs of the syndrome associated with growth hormone secretion disorders and precocious puberty pattern appearing at the age of four. The pathogenesis of the disorder is discussed and related to the hypothalamus-pituitary axis dysfunction caused by the tumor growth.

Astrocytoma↗
PubMed1990

[Russel's syndrome: diencephalic tumor in a child].

The case of a male child with Russel's syndrome due to a pilocytic astrocytoma located in the diencephalic region is presented. The diagnosis was made in the 16th month of age, but symptoms began in the 4th months of life, when he started losing weight. By the time he was admitted weight was 6150g and he was 74cm tall, with an emaciated aspect, no panniculus adiposus, irritated, and with symptoms of intracranial hypertension. There was convergent strabismus, vertical nystagmus of the left eye and bilateral papilledema. Tendinous reflexes were exacerbated and he had spastic tetraparesis. The endocrine evaluation showed a basal raise of GH (23ng/ml), TSH (6.2mUI/1) and prolactin (26ng/ml). The first two hormones did not respond to the acute test with TRH, while prolactin had a poor response. He was submitted to radiotherapy with linear acceleration (total dose of 4000 rads) and surgery, during which the tumor could not be completely removed due to its large size. After 9 months, the child is doing well, with a considerable weight gain (2500g).

Astrocytoma↗
PubMed1994

Catecholamine-induced cAMP response in streptozotocin-induced diabetic rat liver.

UNLABELLED: The effect of prolonged diabetic state on catecholamine-induced adenosine 3',5'-monophosphate (cAMP) response in the rat liver was examined using isolated liver perfusion. Epinephrine- or isoproterenol-induced cAMP production was enhanced (10-fold of the control) in the liver from extremely emaciated (intraperitoneal adipose tissue was absent completely) diabetic rats 4 weeks after streptozotocin-injection kept without insulin, but not from adipose tissue-present diabetic rats. Glucagon-induced cAMP production was decreased in the diabetic rat liver 4 weeks after streptozotocin regardless of the presence or absence of adipose tissue. Secretin-induced cAMP production was also decreased in the adipose tissue-absent diabetic rat liver. Plasma levels of glucose or insulin were not different between adipose tissue-present and -absent diabetic rats. Liver dysfunction (elevated AST and ALT levels) was observed 1 week after streptozotocin-injection, and worsened at 4 weeks. Forskolin-induced production of cAMP, and oxymetazoline (an alpha 2-adrenergic agonist)-induced suppression of it were not different among the control, newly diabetic (1 week after streptozotocin-injection), and the adipose tissue-absent diabetic rat liver. IN CONCLUSION: 1) enhanced beta-adrenergic, and decreased glucagon- or secretin-induced cAMP production seems to be caused by different mechanisms; 2) the prolonged severe diabetic state losing adipose tissue may cause a considerable change in metabolism and the characteristics of hepatocyte, and lead to enhanced beta-adrenergic cAMP production.

Adipose Tissue↗
PubMed1981

Long-term parenteral nutrition. I. Clinical experience in 70 patients from 1967 to 1980.

Seventy patients, 37 females and 33 males, median age 46 years, have been treated with long-term parenteral nutrition for 816 patient-months, or 68 patient-years. Short-bowel syndrome was the commonest indication for parenteral nutrition (582 patient-months). Twenty-four patients were receiving home parenteral nutrition. Most had severe short-bowel syndrome following intestinal resection for Crohn's disease or mesenteric infarction. Metabolic complications included zinc deficiency syndrome in four patients before routine zinc administration and progressive halisteresis in five patients. The mortality for 26 patients with short-bowel syndrome was 23%, for 15 patients with intestinocutaneous fistulas 40%, and for 15 patients with severe emaciation for various causes 27%. Parenteral nutrition was withdrawn in 6 (23%) of the 26 patients with short-bowel syndrome, who subsequently were able to maintain body weight with oral feeding. Fifteen patients are still (February 1980) receiving home parenteral nutrition.

Adolescent↗
PubMed1994

von Recklinghausen's disease with diencephalic syndrome in an adult. Case report.

The case of a 39-year-old man with von Recklinghausen's disease, presenting with emaciation and a marked increase in serum growth hormone concentration, is presented. Neuroradiological and histological examination confirmed anaplastic astrocytoma in the optic chiasm-hypothalamic region. This is a rare case of diencephalic syndrome and von Recklinghausen's occurring together in an adult.

Adult↗
PubMed1992

Pathological findings in the Hawaiian monk seal.

Postmortem examinations were performed on 45 Hawaiian monk seals (Monachus schauinslandi) collected during field research on the beaches of the Northwestern Hawaiian Islands (USA) from 1981 to 1985. Both males and females of all age groups, perinatal through adult, were examined. Frequent findings included parasites, trauma, cardiovascular disease (congenital and acquired), and respiratory infections. Emaciation was a common condition. All animals except neonates were infected with parasites; infection was severe in several cases. Splenic hematopoiesis was a universal histopathologic finding. Some cases exhibited lesions consistent with renal, gastrointestinal, and toxic disorders; ectopic tissue calcification; gallstones; and ophthalmologic and dental problems.

Animals↗
PubMed1993

Perinatal mortality in caribou from the Porcupine herd, Alaska.

During the 1989 caribou (Rangifer tarandus) calving season on the Arctic National Wildlife Refuge, Alaska (USA), 61 calf carcasses were examined for cause of death and associated pathology. Dead calves were located by low-level aerial searches with two fixed-wing aircraft and a helicopter over high density calving areas between the Hulahula and Aichilik rivers. Primary diagnoses included emaciation (39%), malnutrition (8%), stillbirth (21%), trauma (16%), other primary causes (7%), and undetermined causes (8%). Twenty calves had contributory renal tubular degeneration. The findings indicate that factors contributing to nutritional deprivation in calves were the major cause of neonatal mortality; however, factors affecting stillbirth, abortion, or the urogenital system may have major effects on neonatal caribou and warrant further investigation.

Alaska↗
PubMed1993

Hiatal hernia in a harbor porpoise (Phocoena phocoena).

We observed the displacement of the first compartment of the stomach through the diaphragmatic hiatus into the thoracic cavity in an immature harbor porpoise (Phocoena phocoena). This hiatal hernia, coupled with a severe pneumonia, contributed to the emaciation and death of the animal.

Animals↗
PubMed1995

Notoedric mange in an ocelot (Felis pardalis) from southern Texas.

Notoedric mange was diagnosed in a free-ranging adult male ocelot (Felis pardalis) found dead in April 1994 in southern Texas (USA). The emaciated carcass had no body fat. The heart was enlarged and flaccid. There was a nonpurulent serosanguineous pericardial and peritoneal exudate. Severe encrusted skin lesions and alopecia extended from the head posteriorly to the shoulders. The forelegs and feet were less severely affected. We observed a massive infection of Notoedres cati in skin scrapings. Epidermal lesions included hyperkeratosis and parakeratosis with necrotic debris and foci of acute inflammatory cells surrounding mites and their eggs in the stratum corneum. There was mild acanthosis and spongiosis of the stratum germativum, but no chronic inflammation in the dermis. This is the first confirmed case in the ocelot, but notoedric manage has been reported from the bobcat (Felis rufus) in southern Texas. Thus, notoedric mange could pose an additional threat to the already endangered remnant population of the ocelot.

Animals↗
PubMed1999

Mass stranding of wedge-tailed shearwater chicks in Hawaii.

Unusual numbers of wedge-tailed shearwater (Puffinus pacificus) chicks stranded on Oahu (Hawaii, USA) in 1994. Compared to healthy wedge-tailed shearwater (WTSW) chicks, stranded chicks were underweight, dehydrated, leukopenic, lymphopenic, eosinopenic, and heterophilic; some birds were toxemic and septic. Stranded chicks also were hypoglycemic and had elevated aspartate amino transferase levels. Most chicks apparently died from emaciation, dehydration, or bacteremia. Because many birds with bacteremia also had severe necrosis of the gastrointestinal (GI) mucosa associated with bacteria, we suspect the GI tract to be the source of disseminated bacterial infection. The identity of the bacteria was not confirmed. The daily number of chicks stranded was significantly related to average wind speeds, and the mortality coincided with the fledging period for WTSW. Strong southeasterly winds were a distinguishing meteorologic factor in 1994 and contributed to the distribution of stranded chicks on Oahu. More objective data on WTSW demographics would enhance future efforts to determine predisposing causes of WTSW wrecks and their effects on seabird colonies.

Animals↗
PubMed1999

Effect of emanciation on liver histology of alpine chamois during winter.

With the aim of describing the effect of severe feed restriction on the liver histology, morphometrical analysis of liver sections of 10 alpine chamois (Rupicapra rupicapra) was performed. Five animals were found dead during the winter season 1995-96 and five were collected during the hunting season 1996. Hepatocyte nuclear size was measured in squared micrometers using Image-Pro Plus software. A significant decrease in the mean size of the nuclei of hepatocytes in emaciated chamois, as compared to harvested animals was observed. The reduction in cell nuclear size may be linked to the mobilization of body protein to prevent ketosis during severe food restriction, as hypothesized for other wild ungulates. The change in hepatocyte size may be the consequence of a strategy to minimize energy expenditure and may be proposed as an index of metabolic stress during winter undernutrition.

Analysis of Variance↗
PubMed2002

Effects of diet and body condition on fecal progestagen excretion in elk.

Recent research demonstrated the utility of fecal progestagens (P4) for detecting pregnancy in elk (Cervus elaphus) during mid- to late gestation. Several factors, however, may influence fecal P4 excretion and limit its use in free-ranging animals. We investigated the effects of nutrition and body condition (percent ingesta-free body fat) on fecal P4 concentrations and incidence of abortion. During mid-gestation (late December 1997 through early March 1998), 40 gravid cow elk varying in body condition were placed on three diets (high, medium, and low) in which the amount of food offered varied. Feces were collected periodically and analyzed for P4 via radioimmunoassay. We found no significant effect of dietary treatment on P4 concentrations, but as body condition declined, P4 concentrations declined significantly. This decline did not impede the ability to detect pregnancy based on previously reported criteria, even for elk in such poor condition that they aborted. However, fecal P4 concentrations in 10% (4/39) of samples collected from 13 non-pregnant animals maintained on a high plane of nutrition were false-positive for pregnancy. We suggest alternate criteria for determining pregnancy in elk using fecal P4 values: > 1.25 micrograms/g feces as pregnant, < 1.0 microgram/g feces as non-pregnant, and 1.0-1.25 micrograms/g feces as inconclusive. Finally, two cows that aborted did not abort until weeks after being classified as emaciated and near death, suggesting that nutrition-associated abortion in elk may not occur during mid-gestation except under extremely harsh conditions.

Abortion, Veterinary↗
PubMed2005

Pathological features in marine birds affected by the prestige's oil spill in the north of Spain.

A total of 2,465 seabirds, mainly common murres (Uria aalge), razorbills (Alca torda), and puffins (Fratercula arctica) that beached in the northwestern part of Spain after the "Prestige" oil spill on 19 November 2002 were examined by pathological methods. Birds were divided into three groups: dead birds with the body covered (group 1) or uncovered (group 2) by oil and birds recovered alive but which died after being treated at a rescue center (group 3). The main gross lesions were severe dehydration and emaciation. Microscopically, hemosiderin deposits, related to cachexia and/or hemolytic anemia, were observed in those birds harboring oil in the intestine. Severe aspergillosis and ulcers in the ventriculus were found only in group 3 birds, probably because of stress associated with attempted rehabilitation at the rescue center. The mild character of the pathological changes suggests that petroleum oil toxicosis causes multiple sublethal changes that have an effect on the ability of the birds to survive at sea, especially weak and young, inexperienced animals. Dehydration and exhaustion seem to be the most likely cause of death.

Animals↗
PubMed2003

Hyena disease (premature physeal closure) in calves due to overdose of vitamins A, D3, E.

Holstein suckling calves on a farm manifested severe emaciation, generalized alopecia, dome-like cranial deformation, and high mortality (Case 1). Metaphyseal growth plates of the femur were achondroplastic; segmented, partially resorped, and replaced with immature bony trabeculae containing degenerated chondrocytes. The skull was thin and partially replaced with connective tissue. Diffuse and severe fatty degeneration was observed in the hepatic stellate (Ito') cells. After 6 mo, surviving calves manifested unthrifty with short and irregular hindquarters (Case 2). The metaphyseal growth plates were poorly formed, irregular, partially disappeared centrally, and often sealed with thin bony trabeculae. The cartilage matrix was not homogeneous but was finely fibrous, and chondrocytes were flat and degenerated. The bone lesion was diagnosed as chondrodysplasia due to premature physeal closure. These calves had been administered excessive amounts of vitamins A, D3 and E, and blood chemistry of acute case showed hypervitaminosis A and E. Case I demonstrated acute disease, while Case 2 demonstrated chronic sequelae. Hypervitaminosis A was the suspected cause.

Animals↗
PubMed1992

Occurrence of cataracts in triploid Atlantic salmon (Salmo salar) on four farms in Scotland.

In September 1991 cataracts occurred in two year-classes of triploid Atlantic salmon. The fish showed varying degrees of blindness, were lethargic and became emaciated owing to their inability to feed. The lesions in the lens were mainly in the anterior and posterior cortex and perinuclear areas, the capsule and embryonic nucleus remaining unaffected. Diploid fish of the same year-class did not have cataracts. The origin of the triploid fish, the method of triploidisation, their diet and disease status were investigated, but no predisposing factors could be found to account for the high incidence of cataracts.

Animals↗
PubMed1990

Trichospirura leptostoma: a possible cause of wasting disease in the marmoset.

An outbreak of wasting disease-like syndrome in a marmoset colony was found to have been caused by a nematode. After passing through several larval stages in an intermediate host, the cockroach, the parasite inhabited the pancreas of the marmoset. The cardinal symptoms were emaciation, anaemia, and elevated hepatic enzyme activities; the pancreas reacted with a fibrosing inflammation.

Animals↗
PubMed1989

[Hypothalamic glioma with diencephalic syndrome and following precocious puberty--a case report].

A 1-year 11-month-old girl was admitted for losing her weight and gait disturbance. At 4 months of age, she began to become emaciated inspite of normal food intake. Physical and neurological examinations were normal except for a marked lack of subcutaneous fat, irritability and nystagmus. CT scans demonstrated a large tumor occupied in the third ventricle and marked dilatation of the lateral ventricles. Endocrinological studies revealed high levels of plasma growth hormone (GH) in contrast with normal levels of somatomedin-C. The basal value of GH returned to normal with a subnormal response to insulin subsequently after VP-shunt. Then, a pilocytic astrocytoma was partially resected with transcallosal approach. Postoperative course was uneventful and her growth rate returned to normal range. CT scans after radiation therapy of 49 Gy showed marked decrease in size of the tumor. At 3 years and 6 months of age, enlargement of her breast was pointed out although MRI indicated no enlargement of the tumor. Basal value of LH, FSH, E 1 and E 2 elevated and LHRH test showed over-response of LH and FSH. Other hypothalamic-pituitary functions were partially preserved. Case of precocious puberty following diencephalic syndrome associated to the hypothalamic and/or optochiasmatic glioma is quite rare in the previous literature. Mechanisms of diencephalic syndrome and following puberty are unclear. However, endocrinological and radiological findings observed in the present case suggest that hormonal disfunction might be due to the failure of inhibition on GH and LHRH secretion mechanism in the anterior hypothalamus.

Astrocytoma↗