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[Severe diarrhea associated with X-linked lissencephaly with absent corpus callosum and abnormal genitalia: a case report of successful treatment with the somatostatin analogue octreotide].

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We report the clinical course of a case of X-linked lissencephaly with absent corpus callosum and abnormal genitalia (XLAG) exhibiting severe diarrhea. The patient demonstrated lactose intolerance and his intractable seizures were relieved with lactose-free, extensively hydrolyzed whey protein

Hyperinsulinism Hyperammonemia Syndrome, a Rare Clinical Constellation.

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We present the unique case of adult hyperinsulinism hyperammonemia syndrome (HI/HA). This condition is rarely seen in children and even more infrequently in adults. A 27-year-old female with HI/HA, generalized tonic-clonic seizures, staring spells, and gastroesophageal reflux disease presented with

The effects of metformin on body mass index and glucose tolerance in obese adolescents with fasting hyperinsulinemia and a family history of type 2 diabetes.

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OBJECTIVE The prevalence of type 2 diabetes in American adolescents has increased markedly during the past generation. Although the factors that contribute to the development of type 2 diabetes are complex and not wholly elucidated, the triad of severe obesity, hyperinsulinemia, and a family history

Efficacy of Dose-Titrated Glucagon Infusions in the Management of Congenital Hyperinsulinism: A Case Series

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Background: Congenital hyperinsulinism (CHI), a rare disease of excessive and dysregulated insulin secretion, can lead to prolonged and severe hypoglycemia. Dextrose infusions are a mainstay of therapy to restore normal glycemia, but can be associated with volume overload, especially in

Late presentation of necrotizing enterocolitis associated with rotavirus infection in a term infant with hyperinsulinism on octreotide therapy: A case report.

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BACKGROUND Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infancy that can cause permanent brain damage. Consequently, optimal management is extremely important. Current pharmacologic and surgical treatment were available that included diazoxide and

Effect of Intravenous Small-Volume Hypertonic Sodium Bicarbonate, Sodium Chloride, and Glucose Solutions in Decreasing Plasma Potassium Concentration in Hyperkalemic Neonatal Calves with Diarrhea.

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BACKGROUND Hyperkalemia is a frequently observed electrolyte imbalance in dehydrated neonatal diarrheic calves that can result in skeletal muscle weakness and life-threatening cardiac conduction abnormalities and arrhythmias. OBJECTIVE Intravenous administration of a small-volume hypertonic NaHCO3

Metformin in the treatment of HIV lipodystrophy syndrome: A randomized controlled trial.

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BACKGROUND A syndrome of lipodystrophy, characterized by fat redistribution and insulin resistance, has been estimated to affect the majority of human immunodeficiency virus (HIV)-infected individuals who are treated with combination antiretroviral therapy. There are no proven therapies for the

Dietary methionine intake and neural tube defects in Mexican-American women.

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BACKGROUND Nutrients other than maternal folic acid are also thought to play a role in preventing neural tube defects (NTDs). Evidence suggests that methionine interacts with folic acid and vitamin B(12) in the methylation of contractile proteins involved in closing the neural folds. The role of

Neural tube defects on the Texas-Mexico border: what we've learned in the 20 years since the Brownsville cluster.

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We reviewed the published findings from the Texas Neural Tube Defect Project, a 6-year case-control study (1995-2000) of neural tube defects (NTDs) on the Texas-Mexico border. In this review, we highlight what was learned about environmental, genetic, and nutritional factors (i.e., those related to

[Modifications of the glucose curves and gastric voiding of children with Dumping syndrome after laparoscopic Nissen surgery].

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BACKGROUND There has been an important evolution of surgical treatment in recent years due to the progressive generalization of laparoscopic surgery. All of the available techniques can be done laparoscopically with similar results to those of open surgery. In 1978, Villet et al. described the

Early enteral feeding of patients with major burns: prevention of catabolism.

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The immediate onset of hypermetabolism after a major burn dictates that nitrogen and calories be supplied as early as possible to such patients to prevent the well-documented catabolic state. However, intravenous hyperalimentation is not always possible, and enteral feeding is not usually attempted

Cola-induced hypokalemia-a case report and review of the literature.

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OBJECTIVE We aim to describe an unusual and underappreciated cause for hypokalemia. METHODS We describe a patient diagnosed with cola-induced hypokalemia and review the literature. RESULTS Ingestion of massive amounts of cola can lead to severe hypokalemia through several pathophysiologic

Therapeutic effect of guar gum in patients with non-insulin-dependent diabetes mellitus.

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Diets with a high-fiber content have been shown to produce some beneficial effects on metabolic factors in subjects with NIDDM. However, some controversies still exist. In this report, the long-term effect of guar gum (Guarina) on both glycemic and blood lipid profiles was assessed in a randomized,

The efficacy and safety of miglitol therapy compared with glibenclamide in patients with NIDDM inadequately controlled by diet alone.

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OBJECTIVE To compare the therapeutic effects of the alpha-glucosidase inhibitor miglitol (BAY m 1099), the sulfonylurea glibenclamide, and placebo on parameters of metabolic control and safety in patients with NIDDM that is inadequately controlled by diet alone. METHODS After a 4-week placebo run-in

[alpha-Glucosidase inhibitors in the therapy of diabetes mellitus].

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The onset and progression of long-term complications in diabetes mellitus appear to be related to the degree of hyperglycemia and the overall metabolic control. Therefore, an important goal in the therapy of subjects with diabetes is to avoid wide fluctuations in blood glucose concentrations and

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