Prader-Willi Syndrome

(Australia—Australian Family Physician, May 1997, p. 560.) Prader-Willi syndrome is an uncommon genetic disorder of paternal chromosome 15 deletion that results in abnormalities of growth and development, including an insatiable appetite that leads to morbid obesity. The condition occurs sporadically, with an incidence of one per 10,000 to 20,000 births. It affects an equal proportion of males and females. Although Prader-Willi syndrome pregnancies are frequently complicated by mild polyhydramnios, reduced fetal movement and breech delivery, the condition is not usually suspected until delays in development become obvious at about one year of age. Infants with the syndrome have severe hypotonia, hypogonadism, delayed motor development and an unusual weak, squeaky cry. The characteristic appearance of persons with Prader-Willi syndrome includes strabismus, almond-shaped eyes, narrow forehead, triangular mouth, and lighter hair and skin coloring than other family members. Voracious appetite and excessive consumption of food lead to established obesity before six years of age. In addition to the complications of massive obesity, patients with this disease have high pain thresholds, learning difficulties, emotional lability, sleep disorders, osteoporosis, scoliosis and severe hypogonadism. Management of Prader-Willi syndrome is usually extremely difficult even when professionals from several disciplines (e.g., dietitian, behavioral psychologist, developmental pediatrician and family physician) work with the patient and the family.

Benign Prostatic Hyperplasia

(Canada—Canadian Family Physician, August 1997, p. 1395.) Although transurethral prostatic resection (TURP) has been the mainstay of therapy for patients with benign prostatic hyperplasia, new medical and surgical techniques now enable individualization of treatment. In several studies, patients reported an improvement in symptoms while receiving placebo therapy. This and other observations have led to increased use of “watchful waiting” in patients with benign prostatic hyperplasia (periodic review of symptoms and examination for signs of obstructive neuropathy or bladder decompensation). Patients with severe symptoms and those with clinical indications for treatment may be treated with alpha-adrenergic antagonist drugs. Initial studies show that treatment with finasteride can significantly reduce symptom scores and prostate size without causing significant side effects, but the role of this drug is still under investigation. Since both of the classic surgical approaches (TURP and open prostatectomy) have significant associated rates of morbidity and mortality, newer techniques such as transurethral prostatic incision (TUIP), laser prostatectomy, microwave thermotherapy and intraprostatic stenting have been developed. Several forms of laser prostatectomy have been introduced and all appear to provide comparable results to TURP but with approximately one third the rate of serious complications. Microwave techniques also appear to be effective but have been less thoroughly studied. Intraprostatic stents have been mainly used in poor surgical candidates but have the potential for wider clinical application.

Preventing Burnout in Family Physicians

(Australia—Australian Family Physician, July 1997, p. 787.) Concerns have been raised in several countries about declining morale and job satisfaction among primary care physicians. Physicians are reputed to avoid recognition of stress in themselves and to delay seeking or complying with professional help. Some of this behavior is attributed to tendencies to be compulsive, perfectionistic and overly conscientious, leading to difficulty in delegating and in setting limits, particularly to medical practice. Physicians as a group show high rates of suicide, depression, drug and alcohol misuse, and divorce. Early warning signs of burnout include a cynical attitude, a decline in sense of humor, difficulty in making decisions, perceptions of being taken advantage of and hostility. Physicians under stress tend to increase rates of prescribing and ordering tests, identify fewer patient problems and initiate more return visits. They are less likely to practice preventive techniques. Strategies to prevent physician burnout include taking time to reflect on the practice and integrate it with life philosophy, enforcing practical schedules that allow time for a balance of varied activities, adjusting the work environment in terms of place, time and people issues to provide a positive daily routine, joining a peer support group or ensuring open communication about potential stressors, and ensuring good physical and mental health by following a healthy lifestyle, exercising and using techniques such as meditation.—anne d. walling, m.d