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1/65. patients with morbid obesity don't get life-saving bone marrow transplants.

    When the patient, a registered nurse, was surgically treated for morbid obesity she initially lost 54.5 kg. Approximately 2 years after gastric bypass, she received a diagnosis of chronic myelogenous leukemia and subsequently underwent a successful allogenic bone marrow transplant (BMT). When her surgical history was taken at the transplant facility, the significant weight loss and gastric bypass were discussed. She was informed that at 140 kg, she would not have been eligible nor considered a candidate for transplant. A search of the literature and a survey of other facilities confirmed this view as typical. The reasons cited were that the chemotherapy dosage required for the morbidly obese weight level would cause fatal organ damage as opposed to organ-sparing dosages, which would not eradicate all leukemic cancer cells. An additional general view was that the morbidly obese could not survive the rigors of the transplant preoperative regimen. This patient had an uneventful recovery and remains disease-free today, 3 years after BMT and 5 years after gastric bypass, with a sustained total weight loss of 73 kg.
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ranking = 1
keywords = obesity
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2/65. Adverse psychologic reactions to ileal bypass surgery.

    Of 33 patients who underwent ileal bypass surgery for morbid obesity and were followed up with psychiatric interviews and consultation postsurgery, five appear to have had adverse psychologic sequelae related to the procedure. The emotional problems of these five patients were in part related to or precipitated by their drastic weight loss after ileal bypass. In most cases, the patients generally had depressive symptoms and, in dynamic terms, were dependent individuals with lifelong problems in object relations. The coping styles demonstrated, while not rigorously classified as psychiatric illness, appeared to predispose them for certain difficulties even when weight had been lost. Ileal bypass surgery apparently is not psychologically innocuous as previously thought, and psychiatric follow-up of patients is indicated.
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ranking = 0.2
keywords = obesity
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3/65. A previously unrecognised phenotype characterised by obesity, muscular hypotonia, and ability to speak in patients with angelman syndrome caused by an imprinting defect.

    The clinical features of angelman syndrome (AS) comprise severe mental retardation, postnatal microcephaly, macrostomia and prognathia, absence of speech, ataxia, and a happy disposition. We report on seven patients who lack most of these features, but presented with obesity, muscular hypotonia and mild mental retardation. Based on the latter findings, the patients were initially suspected of having prader-willi syndrome. dna methylation analysis of SNRPN and D15S63, however, revealed an AS pattern, ie the maternal band was faint or absent. Cytogenetic studies and microsatellite analysis demonstrated apparently normal chromosomes 15 of biparental inheritance. We conclude that these patients have an imprinting defect and a previously unrecognised form of AS. The mild phenotype may be explained by an incomplete imprinting defect or by cellular mosaicism.
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keywords = obesity
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4/65. Endocrine effects of lithium carbonate in healthy premenopausal women: relationship with body weight regulation.

    The mechanisms involved in Li-induced weight gain remain unclear. The higher frequency of obesity in women than in men under Li treatment, suggests a role for reproductive hormones. The serum levels of the following hormones were evaluated in healthy young women at diverse stages of a control menstrual cycle, and during Li carbonate (900 mg/day) or placebo administration: prolactin, luteinizing hormone, follicle-stimulating hormone, 17-1 estradiol, progesterone, thyroxine, thyrotropin, cortisol, dehidroepiandrosterone sulfate, free testosterone, leptin and an oral glucose tolerance test, in order to measure the areas under the glucose and insulin curve. The body weight was assessed the day before and the last day of treatment. The Li serum levels 15 hours after the last dose were 0.31 /- 0.1 mEq/L. No significant changes in body weight and in the normal fluctuations of the reproductive hormones along the menstrual cycle were observed during Li administration. An increase in the serum levels of thyrotropic hormone ( p = 0.0001) was the only significant effect of Li, which may predispose to excessive weight gain after prolonged administration of the cation. The remarkable lack of effects of Li on these hormones, question the pertinence of studies conducted in healthy volunteers for the comprehension of the obesity observed in psychiatric patients who may be particularly prone to gain weight under prolonged treatment with high dose of Li.
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ranking = 0.4
keywords = obesity
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5/65. Three children with a syndrome of obesity and overgrowth, atypical psychosis, and seizures: a problem in neuropsychopharmacology.

    Three children presented with a complex syndrome of atypical psychotic and extremely immature behavior, obesity and overgrowth, borderline retardation, and seizures (prominent in two). Weight overgrowth exceeded height overgrowth and was stratospheric (up to 8 SD above mean). obesity seemed related to lack of satiety. The cases fit no known condition: hypothalamic damage, Sotos' syndrome, and prader-willi syndrome were excluded. Empirical treatment with anticonvulsants (carbamazepine and acetazolamide) together with psychotropic agents (selective serotonin reuptake inhibitors and risperidone) controlled seizures, improved behavior, and stopped weight gain in each patient. We have not found this syndrome previously described. The etiology is unknown: perinatal encephalopathy could be a factor in the two patients with prominent seizures; in the third, familial major affective disorder is implicated. Medication responses suggest a low-serotonin state underlying the lack of satiety, an imbalance of serotonin and noradrenergic modulation in the hypothalamus, and epileptogenic disorders (or affective disorder responsive to anticonvulsants in one case) involving these same systems.
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ranking = 1
keywords = obesity
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6/65. proteinuria and focal segmental glomerulosclerosis in severely obese adolescents.

    OBJECTIVE: To describe the clinical and laboratory features of obesity associated proteinuria and focal segmental glomerulosclerosis. STUDY DESIGN: The patients were seen over a 12-year period at two large children's hospitals. Renal biopsies, performed for the diagnosis of unexplained heavy proteinuria and prepared for light, immunofluorescent, and electron microscopy, were read independently by two pediatric pathologists. blood pressure, body mass index, serum levels of creatinine, albumin, and cholesterol, and 24-hour urinary protein were measured. RESULTS: Seven African American adolescents were identified with obesity-associated proteinuria, which was characterized by severe obesity (120 /- 30 kg), markedly elevated body mass index (46 /- 11), mild hypertension (134/74 /- 10/18 mm Hg), slightly low to normal serum albumin levels (3.6 /- 0.2 g/dL), moderately elevated serum cholesterol levels (196 /- 60 mg/dL), and elevated 24-hour protein excretion (3.1 /- 1.3 g/dL). Calculated creatinine clearance was normal in 6 patients and decreased in one. Typical renal histologic features included glomerular hypertrophy, focal segmental glomerulosclerosis, increased mesangial matrix and cellularity, relative preservation of foot process morphology, and absence of evidence of inflammatory or immune-mediated pathogenesis. One patient showed a dramatic reduction in proteinuria in response to weight reduction. Three patients who were given angiotensin-converting enzyme inhibitors had reduced urinary protein losses from 2.9 g to 0.7 g per day. One patient developed end-stage renal disease. CONCLUSION: Obese adolescents should be monitored for proteinuria, which has distinct clinical and pathologic features and may be associated with significant renal sequelae. Such proteinuria may respond to weight reduction and/or treatment with angiotensin-converting enzyme inhibitors.
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ranking = 0.6
keywords = obesity
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7/65. Intraluminal duodenal obstruction by a gastric band following erosion.

    BACKGROUND: duodenal obstruction occurred 4 years following gastric banding for morbid obesity, which had had a good result. METHOD: A 56-year-old female with a history of gastric banding presented with duodenal obstruction. RESULT: Physical and radiological examination was able to give the diagnosis. At surgery, the gastric band in the distal duodenum was removed. She was discharged on postoperative day 4, with no complication. At 6 months following discharge, her nausea and vomiting have not recurred. CONCLUSION: Following gastric banding, band erosion through the gastric wall and internalization into the lumen can cause small bowel obstruction.
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ranking = 0.2
keywords = obesity
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8/65. Reduction of abdominal obesity in lipodystrophy associated with human immunodeficiency virus infection by means of diet and exercise: case report and proof of principle.

    lipodystrophy associated with human immunodeficiency virus infection causes abdominal fat gain, peripheral subcutaneous fat atrophy, insulin resistance, low levels of high-density lipoprotein cholesterol, and hypertriglyceridemia. An exercise program combined with a moderate-fat, low-glycemic-index, high-fiber diet can reverse several aspects of lipodystrophy, and, until specific treatment is available, should be considered for treatment of lipodystrophy.
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ranking = 0.8
keywords = obesity
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9/65. Type 2 diabetes presenting as diabetic ketoacidosis in adolescence.

    We report two black adolescent subjects who presented with diabetic ketoacidosis, but who lacked autoimmune markers and demonstrated clinical and biochemical characteristics more typical of Type 2 diabetes, including obesity, acanthosis nigricans, positive family history for Type 2 diabetes, and Type 2 diabetic dyslipidaemia. Subsequent to acute presentation, insulin was discontinued in both subjects and excellent glycaemic control was achieved with metformin therapy alone. Four months following acute presentation, both had adequate c-peptide responses to intravenous glucagon. Type 2 diabetes can present as diabetic ketoacidosis in obese adolescent subjects.
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ranking = 0.2
keywords = obesity
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10/65. obesity due to proopiomelanocortin deficiency: three new cases and treatment trials with thyroid hormone and ACTH4-10.

    The symptoms of severe early-onset obesity, adrenal insufficiency, and red hair define the proopiomelanocortin (POMC) deficiency syndrome as described so far in two children with complete loss-of-function mutations of the human POMC gene. In POMC deficiency, obesity reflects the lack of POMC-derived peptides as ligands at the melanocortin (MC) MC4 and MC3 receptors, which are expressed in the hypothalamic leptin-melanocortin pathway of body weight regulation. Hypocortisolism and alteration of pigmentation are caused by the lack of POMC-derived peptides at the adrenal MC2 receptor and the skin MC1 receptor, respectively. Here we describe three new cases of complete loss-of-function mutations of the POMC gene. patients were diagnosed based on the clinical trials of red hair, adrenal insufficiency, and early-onset severe obesity. One previously described translation initiation mutation (C3804A) as well as one new nonsense (A6851T) and two new frame-shift mutations (6996del and 7100 2G) were found in homozygosity or compound heterozygosity. The heterozygous parents were found to have high normal or mildly elevated body weight, suggesting a dosage effect of the POMC gene product on weight regulation. To compensate for the lack of hypothalamic melanocortin function, we initiated a trial in the two previously published patients with intranasal ACTH4-10, a melanocortin fragment for which an anorexic effect has been described recently. During 3 months with increasing doses of ACTH4-10, no change of body weight or metabolic rate was observed, suggesting that at least in these two POMC-deficient patients ACTH4-10 is without any compensatory effect. In the same two patients, further investigation revealed a mildly elevated TSH. However, a 1-yr treatment with thyroid hormone did not result in a significant reduction of body weight.
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ranking = 0.6
keywords = obesity
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