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.     

Controversies in transfusion medicine. Perioperative hemodilution: pro

Hemodilution can obviate the need for allogeneic transfusion in some patients. However, it is only one component of a comprehensive blood conservation program and should be combined with other techniques whenever possible. While most often employed in teenagers and healthy young adults, ANH has been used in small children, the elderly, and patients of all ages who are undergoing cardiac surgery. Extreme caution should be exercised if ANH is used in patients with coronary artery disease or aortic stenosis who are undergoing noncardiac surgery, because their normal compensatory mechanisms are impaired. What usually is a safe and relatively simple procedure can become a disaster if employed in inappropriately selected patients. Careful hemodynamic monitoring and maintenance of normovolemia are critical.     

Insulin Infusion Normalizes Cardiovascular Responses and Plasma Noradrenaline after Oral Glucose in Type 1 (Insulin-dependent) Diabetes

ABSTRACT. We examined whether the abnormal regulation of the cardiovascular system and plasma noradrenaline observed after oral glucose in insulin-dependent diabetic patients could be normalized by intravenous infusion of insulin. Eight patients with type 1 (insulin-dependent) diabetes were examined after an oral glucose load with and without simultaneous infusion of insulin. Insulin infusion increased plasma insulin from 0.07 to 0.31 nmol/1. In the control experiment (glucose only), mean heart rate and mean arterial systolic blood pressure remained unchanged and plasma noradrenaline (NA) decreased (p < 0.05). After oral glucose plus intravenous insulin, mean heart rate increased by 11 % and mean systolic blood pressure by 5 % (p < 0.05, p < 0.01), whereas plasma NA did not change significantly. The present study indicates that physiologic increments in plasma insulin concentration are of importance in the regulation of the cardiovascular system and plasma NA following an oral glucose load.     

Development of an aftercare program in a nonmetropolitan area

This article describes the development of a nontraditional aftercare program set in a predominantly rural area. The program was initiated by a citizen's board concerned with the lack of follow-up services provided to patients discharged from a distant state hospital. The planning format as conceived by representatives of this board is outlined and the steps involved in operationalizing specific services and a research program are described. Lastly, a number of limitations of the program and future directions are enumerated.     

Possible sequelae of trauma and somatic disorder in early life.

All children experience trauma. The age, state of development and constitutional factors will determine whether some children will have a traumatic effect. Trauma occurring before the age of three, at a time when the ego has not developed its synthetic and integrative functions, may be reproduced in later life as an isolated symptom, by selected sensations involved in a sensory imprint or screen sensation of the trauma as a simple recording. After the age of three, under the influence of a more mature ego, excessive traumatic stimuli will be integrated and elaborated in symptom formations as phobias or other conditions and extended as part of the total personality. Recurrence in later life is triggered by events related not only to the original experience, but also to the content of its elaboration. The earlier in life the trauma occurs, the more likely that somatic imprints of primitive physiological symptoms would result as an archaic, biological defense or screen sensations. Recurrent sensory imprints or screens may appear as organic illness or functional somatic symptoms. Diagnostically, a detailed early life history is necessary to uncover the presence of a sensory screen memory of a trauma and so avoid diagnostic medical search for organic causation. Case material illustrating the two groups are presented. Indications for psychoanalysis and for supportive psychotherapy are discussed from our theoretical framework as well as from the literature.     

L’apprentissage de la relation et de l’examen cliniques en cancérologie

Résumé: Malgré la multiplication des examens complémentaires, lexamen et la relation cliniques restent au cur de la pratique médicale. Ils justifient donc un enseignement structuré et attentif. Cet enseignement fait désormais lobjet dune disposition législative fondée sur le respect des patients. Il tient compte dune approche globale, biopsychosociale. Ses objectifs méritent dêtre précisés en vue dune relation de qualité avec des malades en situation difficile. En pratique on insistera sur la dédramatisation dun diagnostic de cancer, sur la façon dapporter de mauvaises nouvelles (diagnostic initial de cancer ou évolution terminale) et sur la pratique des touchers pelviens. Toute cette formation en cancérologie doit sinscrire en cohérence avec une formation clinique générale.