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.     

Liposomal amphotericin B in hepatic candidosis

A 4 year old girl treated with a standard chemotherapy protocol for acute lymphoblastic leukaemia developed hepatic candidosis during the consolidation phase. This relapsed after a prolonged course of amphotericin B and flucytosine. An eight week course of liposomal amphotericin produced a marked clinical improvement which was sustained for one year. A subsequent relapse was associated with transformation to myelodysplastic leukaemia.     

Prevention of 5-Fluorouracil-Caused Growth Inhibition in Sordaria fimicola

Growth (dry weight accumulation) of Sordaria fimicola in standing liquid culture (sucrose-nitrate-salts-vitamins) is inhibited by the presence of 5 μM 5-fluorouracil in the medium. This inhibition is completely prevented by uracil, deoxyuridine, and 5-bromouracil, partly prevented (40 to 90% of growth observed without 5-fluorouracil) by uridine, thymidine, and 5-bromodeoxyuridine, and slightly prevented by trifluorothymine, cytosine, cytidine, deoxycytidine, and 5-methylcytosine (all at 0.5 to 1 mM). Thymidine and thymine riboside were without any apparent effect. Growth is also inhibited by 0.2 mM 6-azauracil, and this inhibition was completely prevented by uracil and uridine, partly prevented by deoxyuridine, 5-bromouracil, cytidine, and 5-methylcytosine, and slightly prevented by thymine, thymidine, 5-bromodeoxyuridine, cytosine, and deoxycytidine. The data suggest that the observed inhibition of growth by 5-fluorouracil is due to inhibition of both ribonucleic acid and deoxyribonucleic acid synthesis. The data also allow inferences concerning pyrimidine interconversions in S. fimicola; i.e., thymine can be anabolized to thymidylic acid without first being demethylated, although demethylation appears to occur also.     

Reconstructive preprosthetic surgery 2. Pre-implantologic surgery

Reconstructive preprosthetic surgery is, amongst others, aimed at the creation of an environment which is favourable to the construction of an implant supported prosthesis. Not in all cases the pre-existent volume of bone is sufficient to place an implant in the planned position. In this paper various techniques to augment local bone defects for reliable implant placement are described.     

Dentoalveolar traumatology

The dentist will be confronted unexpectedly with a dentoalveolar trauma patient. This patient has to be seen immediately and has to be treated adequately. The risk of overlooking trauma-related signs when examining these patients, can be minimized by following a strict protocol. This article describes a protocol for examination and treatment of a patient with a dentoalveolar trauma. The prognosis after treatment of the trauma is discussed. Also some recommendations regarding aftercare and prevention are presented.     

Safety and biocompatibility of the Myosplint system--a passive implantable device that alters ventricular geometry for the treatment of heart failure

A passive implantable device developed for the treatment of heart failure, the Myosplint System, has demonstrated therapeutic efficacy in a canine model of pacing induced heart failure. The current study sought to demonstrate chronic device safety and biocompatibility, in vivo, in a normal porcine model. Two devices were implanted into each normal, beating heart of 6 juvenile and 15 adult pigs without cardiopulmonary bypass. Animals survived 90 (juvenile and adult) or 180 days (adult only). Serial hematologic and biochemical profiles were evaluated in each pig during the study period. A comprehensive necropsy study was performed in each pig to evaluate device stability, healing response, thromboembolism, hemorrhage, and intravascular hemolysis related to the Myosplint system. Six adult animals died from infectious disease (four) or perioperative (two) complications unrelated to device design or function and were excluded from the final analysis. No clinical, biochemical or pathologic evidence of significant, device related adverse events was observed in surviving animals. The chronic myocardial healing response appeared normal at term, and all devices maintained their structural integrity throughout the study. The Myosplint system was easily implanted in beating hearts and was rapidly incorporated into host tissues without clinically significant morbidity in this porcine model.