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1/32. angiotensin ii blockade in hypertensive dialysis patients.

    Five hypertensive haemodialysis patients have been infused with saralisin. The infusion appears to be a simple diagnostic test separating patients into two groups. First, there are those whose blood pressure does not fall with saralasin pre-dialysis, but does fall with weight removal during dialysis; the blood pressure in these patients can be controlled by a reduction in pre-dialysis weight. Second, there are those whose blood pressure does fall with saralasin either pre- or post-dialysis; their arterial pressure does not fall with weight removal, but can be controlled by anti-hypertensive drugs. In two of the patients who responded to saralasin, the mechanism of the high blood pressure appeared to change from volume dependency, partial or complete, with suppressed renin release, to angiotensin dependency, partial or complete, as weight was removed during dialysis. These patients illustrate the importance of the interaction between volume and the level of angiotensin ii in the maintenance of hypertension.
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2/32. empty sella syndrome: does it exist in children?

    OBJECT: The empty sella syndrome (ESS) is well documented in adults, and although the same phenomenon of herniation of the arachnoid space into the enlarged sella turcica has been noted in children, it is not widely known that children suffer from this syndrome. Therefore, the aims of this paper are to increase neurosurgeons' awareness of the existence of this phenomenon in children and to add to the scant body of literature on the subject. methods: The authors treated 12 children, ranging in age between 2 and 8 years, in whom neuroradiological studies demonstrated an enlarged sella turcica filled with cerebrospinal fluid and herniation of suprasellar and arachnoid spaces. The causes of ESS in these children were high intracranial pressure, neglected or improperly treated hydrocephalus, and suprasellar arachnoid cyst. Primary ESS was found as well. Most of the children presented with headache, abnormal body weight (the majority being underweight), and short stature. The results of hormone assays were normal in all children. CONCLUSIONS: If undiagnosed and untreated, ESS in children may lead to serious consequences, including impairment of pituitary and hypothalamic function and damage to the optic chiasm. It is important to raise awareness in the neurosurgical community about the existence of ESS in children so that it can be diagnosed and treated at an early stage. A classification system for the diaphragma sellae is recapitulated.
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3/32. 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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4/32. Initial experience with fenoldopam in children.

    fenoldopam is a direct-acting vasodilator that acts at the postsynaptic dopamine 1 receptors in renal, coronary, cerebral, and splanchnic vasculature resulting in arterial dilation and a lowering of the mean arterial pressure (MAP). Preliminary evidence suggests its efficacy in the treatment of hypertensive urgencies and emergencies in adults. We present four children in whom fenoldopam was used to control MAP in various clinical scenarios, including hypertensive emergencies and urgencies, intraoperative reduction of MAP for controlled hypotension, and control of MAP during extracorporeal membrane oxygenation. The possible applications of fenoldopam and suggested dosing regimens in children are reviewed.
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5/32. hypertension: a cause of growth impairment.

    The effects of high blood pressure on growth are not fully understood and while hypertension may be associated with failure to thrive, hypertension causing failure to thrive in children is poorly documented. We describe four children presenting with failure to thrive due to hypertension consequent to various aetiologies. Control of hypertension with appropriate therapy resulted in improved growth. The exact pathogenesis of failure to thrive in hypertensive children is not known. These cases demonstrate the importance of careful measurement of blood pressure in children with failure to thrive.
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6/32. Relieving pressure.

    Mrs Smith has been resident on a continuing care ward for approximately six years. She was originally admitted after a left cerebral vascular accident that resulted in a right-sided hemiplegia. She is dysphasic but appears to understand much of what is said to her, and she is continent of urine and faeces, using facial expressions and body gestures to communicate her needs. In recent years her weight has increased from 63 kg to 83 kg and she is now chairbound, following a recent leg fracture.
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7/32. nitrogen metabolism and insulin requirements in obese diabetic adults on a protein-sparing modified fast.

    A protein-sparing modified fast (PSMF), which is a total fast modified by the intake of 1.2-1.4 gm. protein per kilogram ideal body weight (IBW), fluids ad libitum, and vitamin and mineral supplementation, allows effective control of carbohydrate metabolism and hunger. It reduces serum glucose and insulin concentrations in obese diabetic patients and increases free fatty acid and ketone body concentrations; ketonuria appears within 24-72 hours. When this fast was applied to seven obese adult-onset diabetics who were receiving 30-100 units of insulin per day, insulin could be discontinued after 0-19 days (mean, 6.5). In the three patients who had extensive nitrogen-balance studies, balance could be maintained chronically by 1.3 gm. protein per kilogram IBW, despite the gross caloric inadequacy of the diet. The PSMF was tolerated well in an outpatient setting after the initial insulin-withdrawal phase had occurred in the hospital. Significant improvements in blood pressure, lipid abnormalities, parameters of carbohydrate metabolism, and cardiorespiratory, symptoms were associated with weight loss and/or the PSMF. For diabetics with some endogenous insulin reserve, the PSMF offers significant advantages for weight reduction, including preservation of lean body mass (as reflected in nitrogen balance) and withdrawal of exogenous insulin.
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8/32. Is weight loss possible in patients treated with thiazolidinediones? Experience with a low-calorie diet.

    BACKGROUND: weight gain is a frequent side-effect of thiazolidinediones, possibly related to fluid retention and stimulation of pre-adipocyte differentiation. methods: We report our experience with a low-calorie diet (800 cal, sodium content 1500 mmol/day) combined with behavior modification on eight patients treated with thiazolidinediones (six pioglitazone and two rosiglitazone). RESULTS: All patients had reported previous weight gain following treatment with thiazolidinediones. All patients lost weight over 12 weeks in the program with their mean /- SD body weight falling from 270 /- 54 lbs (123 /- 25 kg) to 244 /- 61 lbs (111 /- 28 kg) (p < 0.01). The weight loss observed was no different from that observed in 16 age- and gender-matched patients with type 2 diabetes not treated with thiazolidinediones (from 263 /- 54 lbs (120 /- 25 kg) to 239 /- 52 lbs (109 /- 24 kg); p < 0.01). Glycemic control improved while reducing insulin treatment. blood pressure control also improved and antihypertensive medications were decreased. The degree and time course of weight loss is no different from that in patients treated with other diabetic therapies and is associated with improved glycemic and blood pressure control. CONCLUSIONS: We conclude that a program of caloric restriction and behavior modification is effective in leading to weight loss in patients treated with thiazolidinediones. This effect is reassuring, since thiazolidinediones stimulate adipogenesis.
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9/32. weight loss pressure on a 5 year old wrestler.

    The case is reported of a 5 year old boy who was pressured to lose weight in order to wrestle at a lower weight class. Although a minority of athletes engage in unhealthy weight management practices, this is an unusual case because of the age of the athlete and the influential role of a parent.
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10/32. Right-sided diaphragmatic hernia in infants after liver transplantation.

    liver transplantation is just as successful in infants as in older children, but more challenging. This relates to the low weight of the recipients and to their rapidly deteriorating clinical condition (malnutrition and end-stage liver disease) ( J Pediatr 1990;117:205-210; BMJ 1993;307:825-828; Ann Surg 1996;223:658-664; Transplantation 1997;64:242-248; J Pediatr Surg 1998;33:20-23). In addition, higher rates of diaphragmatic complications have been shown to significantly correlate with a younger age ( Transplantation 2002;73:228-232; Transpl Int 1998;11:281-283; Pediatr Transplant 2000;4:39-44), but diaphragmatic hernia has never been reported as a complication of liver transplantation. In this report, 2 patients who developed diaphragmatic hernia after liver transplantation are presented. The possible role of several contributing factors resulting in diaphragmatic hernia is discussed. These factors include (1) diaphragm thinness related to low weight and malnutrition, (2) direct trauma at operation (dissection and diathermy), (3) increased abdominal pressure after transplantation caused by the use of a slightly oversized liver graft, and (4) the medial positioning of the partial liver graft in the abdomen.
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