The international collaborative study of maternal phenylketonuria: status report 1994

Neonatal screening for phenylketonuria (PKU) has created a problem as females with PKU are reaching child-bearing age. Surveys have revealed that maternal phenylalanine blood concentrations greater than 1200 μmol/l are associated with fetal microcephaly, congenital heart defects and intrauterine growth retardation. It is estimated that as many as 3000 hyperphenylalaninemic females may be at risk of producing these fetal abnormalities. To examine this problem, the international maternal PKU collaborative study was developed to evaluate the efficacy of a phenylalanine-restricted diet in reducing fetal morbidity. Preliminary findings have indicated that phenylalanine restriction should begin before conception for females with PKU planning a pregnancy. Dietary control should maintain maternal blood phenylalanine levels between 120 and 360 μmol/l and should provide adequate energy, protein, vitamin and mineral intake. Pregnant hyperphenylalaninemic females who achieved metabolic control after conception or by the 10th week of pregnancy had a better offspring outcome than anticipated. The results of 402 pregnancies are reviewed.     

The Abortion and Sterilization Act in 1975--experience of the Johannesburg Hospital Pregnancy Advisory Clinic

The experience of a Pregnancy Advisory Service operating in terms of the Abortion and Sterilization Act of 1975 is reviewed. A total of 454 applications for abortion were considered, of which 198 (44%) were accepted and the abortions performed. The urgent need for sex education at schools, colleges and universities is stressed, in view of the large number of scholars and students under the age of 17 who seek an abortion. There are also patients who discontinue contraception for a variety of reasons, on medical advice or otherwise, without an alternative being suggested or sought. Motivation of patient, nurse and doctor needs urgent improvement.     

Patient-reported outcome and survivorship after Kinemax total knee arthroplasty

BACKGROUND: We examined the long-term survivorship and patient-reported outcomes at a minimum of ten years following primary total knee arthroplasty. We hypothesized (1) that the survival rate would be at least 90% at ten years; (2) that age, gender, body-mass index, and primary diagnosis would not affect the survival rate; and (3) that the functional status of patients would be comparable with that of an age and gender-matched normal population. METHODS: A total of 407 patients (523 knees) who had had primary total knee arthroplasty between January 1988 and April 1991 were identified. The mean age of the patients at the time of surgery was sixty-nine years, and 68% of the patients were women. At ten years, 165 patients (211 knees) had died; seven of these 211 knees had been revised before the time of death. Of the remaining 242 patients, 208 (86%) completed a questionnaire, which included the Western Ontario and McMaster University Osteoarthritis Index (WOMAC), the Short Form-36 (SF-36), and questions regarding patient satisfaction and revision surgery. In the group of patients who participated in the study, ten patients (eleven knees) had had a revision before the review. RESULTS: A total of eighteen knees were revised. Twelve knees were revised because of aseptic failure and, of these, nine were revised because of polyethylene wear. The probability of survival at ten years was 96.1% with revision for any reason as the end point (and 97.2% when only aseptic failures were considered). Because of the small number of failures, we were unable to draw conclusions about associations between failure and age, gender, diagnosis, and body-mass index. The mean WOMAC scores (and standard deviation) at the time of the evaluation were 88 +/- 17 for pain and 79 +/- 20 for function. The SF-36 scores were similar to those for an age and gender-matched normal population, with only the physical functioning score being significantly lower (p < 0.001) and with the general health score being significantly higher (p < 0.001). Patients generally were very satisfied with all aspects of the outcome. CONCLUSIONS: At ten years, the survival of the prosthesis was excellent and the majority of patients were functionally independent, had very little knee pain, and were very satisfied with the result. The health benefits of this total knee arthroplasty were maintained after a minimum duration of follow-up of ten years.     

Developing a new model for cross-cultural research: synthesizing the Health Belief Model and the Theory of Reasoned Action

This article discusses the development of a new model representing the synthesis of two models that are often used to study health behaviors: the Health Belief Model and the Theory of Reasoned Action. The new model was developed as the theoretic framework for an investigation of the factors affecting participation by Mexican migrant workers in tuberculosis screening. Development of the synthesized model evolved from the concern that models used to investigate health-seeking behaviors of mainstream Anglo groups in the United States might not be appropriate for studying migrant workers or persons from other cultural backgrounds.     

Association of achalasia and pulmonary Mycobacterium fortuitum infection

A case of achalasia complicated by Mycobacterium fortuitum pulmonary infection and empyema is reported. This association has been documented in the medical literature. Possible mechanisms explaining this association are discussed.