Fenfluramine in Prader-Willi syndrome: a double blind, placebo controlled trial

Article Abstract:

The Prader-Willi syndrome is a birth-related disorder that affects 1 out of 10,000 persons. It is characterized by infantile hypotonia, or reduced muscle tone; hypogonadism, or defective internal secretion of the gonads; and facial dysmorphism, or abnormalities of the face. Hyperphagia (excessive eating), abnormal behavior, and decreased intellectual function are evident only after two years of age. Hyperphagia associated with the Prader-Willi syndrome is severe and chronic, contributing to early death. Behavior problems include aggressive outbursts and picking at small skin lesions, and may persist into adolescence and adulthood. Such behavior problems and hyperphagia have adverse effects on the family of patients with the Prader-Willi syndrome. Management of affected patients involves control of the environment, including the availability of food, and behavior management techniques. These methods are not completely effective. The effectiveness of anorexic agents, drugs that decrease appetite, in treating patients with this syndrome has not been clearly demonstrated. The effects of the anorexic agent fenfluramine on the weight and behavior of 15 patients with Prader-Willi syndrome who were 5.5 to 27 years of age, were assessed over six weeks. Fenfluramine caused a decrease in weight, improved food-related behavior, and decreased aggressive behavior towards others. However, the drug did not improve skin-picking or self-mutilation. Patients receiving fenfluramine did not suffer adverse side effects. These findings suggest that short-term therapy with fenfluramine may be useful in managing patients with Prader-Willi syndrome. Fenfluramine may be used during unavoidable exposure to large amounts of food, management of very aggressive behavior, and treatment of patients with life-threatening complications of obesity. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Eating disorders in children, Childhood eating disorders, Fenfluramine, Prader-Willi syndrome

---

Monitoring sudden infant death syndrome: an update

Article Abstract:

Sudden infant death syndrome (SIDS) is the sudden, unexpected death of a seemingly normal healthy infant. Some studies have shown that there are similarities between features in SID infants and infants who have experienced apparently life-threatening episodes. Although the actual factors precipitating the events are not clear, the events are related to the respiratory, cardiac, or central nervous system. It is believed that SIDS siblings are at risk for similar events. Families can be given a device that monitors the breathing patterns of the infant, but the use of home respiratory monitors is controversial. In 1985, a report on the use of respiratory monitors at home stated that there was no evidence that children have a period of apnea (breathing cessation) before irreversible brain damage occurs. It was also argued that the monitors have not yet been perfected and that the risk of SIDS in a subsequent sibling is small. It was suggested that families receive extensive counseling and support, and that respiratory monitors be used only in selected cases. Since this report, no studies have identified a significant increase in risk among SIDS siblings. Currently available monitors are reviewed. A monitor can either reduce or promote anxiety in families with a previous experience with SIDS. Infants who have had an experience with apnea, blue skin coloring from lack of air (cyanosis), episodes of paleness, and severe limpness (hypotonia), are candidates for monitoring. Adequate parental support is essential. (Consumer Summary produced by Reliance Medical Information, Inc.)

United Kingdom, Usage, Reports, Measurement, Sudden infant death syndrome, Patient monitoring, Physiologic monitoring, Apnea, Great Britain

---

Eight and 12 week courses of cyclophosphamide in nephrotic syndrome

Article Abstract:

Nephrotic syndrome is a type of kidney damage that results from a variety of diseases. Cyclophosphamide is an anticancer drug which has also been used in combination with steroids to treat relapsing nephrotic syndrome. One benefit of cyclophosphamide may be to reduce the steroid dose needed. However, cyclophosphamide, like steroids, can have severe side effects, and it is important to determine the lowest effective dose possible. The responses to 8 or 12 weeks of cyclophosphamide by 73 children with nephrotic syndrome were evaluated. All subjects had relapsed when steroid doses were lowered and had symptoms of steroid toxicity. Cyclophosphamide was given at 2 milligrams/kilogram body weight/day. Steroids were also administered during the study according to the extent of remission. The effects the 8-week and the 12-week regimen of cyclophosphamide treatment were similar. About 75 percent of children treated for either 8 weeks or 12 weeks relapsed within five months. A small subgroup of children in each treatment group did not relapse. The cumulative steroid dosage used during cyclophosphamide treatment was reduced by half or more. The frequency and type of side effects, chiefly a drop in white blood cell levels, was similar in each group. The study suggests cyclophosphamide treatment should be given to children for no more than eight weeks at the dosage used in this study. (Consumer Summary produced by Reliance Medical Information, Inc.)

Dosage and administration, Cyclophosphamide, Nephrotic syndrome in children

Similar abstracts:

• Abstracts: Withdrawal syndrome after the double-blind cessation of caffeine consumption
• Abstracts: Stopping smoking in pregnancy: effect of a self-help manual in controlled trial. A randomized controlled trial of extra-amniotic ethinyloestradiol in ripening the cervix at term
• Abstracts: Proline metabolism in adult male burned patients and healthy control subjects. Menstrual cycle effects on the metabolism of tryptophan loads
• Abstracts: Fetal alcohol syndrome in adolescents and adults. Zonisamide for weight loss in obese adults: a randomized controlled trial
• Abstracts: Phenobarbitone and febrile convulsions. Febrile convulsions: a 'benign' condition? New use of anticonvulsant medications among children enrolled in the Tennessee Medicaid program

This website is not affiliated with document authors or copyright owners. This page is provided for informational purposes only. Unintentional errors are possible.