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.     

Speech-language clinician personality variables and clinical effectiveness

The relationship between judged clinical effectiveness and personality variables as determined by the Minnesota Multiphasic Personality Inventory (MMPI) was studied in a population of 130 female graduate students in speech-language pathology from nine universities in six different states. An MMPI profile analysis for the total group indicated that the group was similar to other female graduate student populations and that the typical student, while being manifestly normal, might be described as being rather passive, compliant, stereotypically feminine, sensitive, anxious, highly imaginative, creative, and energetic. Although no single MMPI scale was found to differentiate between subject clinical effectiveness groups, the subjects' MMPI profiles were found to predict accurately the clinical effectiveness group to which the subjects were assigned.     

Chyluria

A twenty-four-year-old black female presented through the outpatient department complaining of “white urine.” Pertinent history, physical examination, and diagnostic studies are reviewed with special emphasis on lymphangiography. The literature is reviewed particularly with regard to lateralization of the fistulas, thoracic duct obstruction, and methods of therapy.     

What do cancer survivors believe causes cancer? (United States)

Objective: To describe cancer survivors beliefs about the causes of prostate, colorectal or breast cancers.Methods: A survey of beliefs about cancer causation was completed by 670 cancer survivors (416 with breast cancer, 165 with prostate cancer and 89 with colorectal cancer) enrolled in a population-based study in Colorado. Categorical analysis was used to describe associations between participants beliefs about the cause of their cancer type, both in themselves and in others, and personal characteristics, including gender, age, and familial cancer risk.Results: Cancer survivors most frequently reported genetic factors, smoking, environmental factors (e.g., pollutants or occupation), and psychosocial factors (e.g., stress) as causing their type of cancer. Respondents underestimated the importance of behavioral factors that are known to be associated with increased cancer risk, such as obesity and physical inactivity, while overestimating the importance of stress and environmental pollution.Conclusions: Cancer survivors beliefs about what causes cancer are substantially different than those of experts. Because those affected by cancer should be well informed about the causes of cancer, educational efforts are needed, especially regarding the importance of factors that can be modified to reduce cancer risk.     

Lenalidomide: an immunomodulatory drug

Lenalidomide (CC-5013; Revlimid) represents one compound in a category of new medications known as immunomodulatory drugs. These compounds are thalidomide derivatives. Through relatively minor structural modifications, the potency of the medication is improved compared with the parent compound, and the side-effect profile has changed considerably. The neurologic toxicity and pro-thrombotic effects of thalidomide are reduced in the structural analog, although concerns regarding pro-thrombotic effects are still present when lenalidomide is combined with dexamethasone. Data supporting lenalidomide's use in myelodysplastic syndrome and multiple myeloma has been published over the past several years and presented at the May 2005 meeting of the American Society of Clinical Oncology. Further trials are ongoing for many other malignancies. This report will review the preclinical and clinical results of the investigations with this exciting new therapeutic, its toxicities and future prospects.     

Relapsing polychondritis studied by computed tomography

Computed tomographic findings in a patient with relapsing polychondritis are described. Collapse of the cartilage of the nose and calcification in cartilages of the ears were clearly demonstrated. CT scanning was also helpful in evaluating the tracheobronchial tree for airway compromise, which could prove fatal in this condition.