New species of Amanita subgen. Lepidella from Guyana.

New species of Amanita subgen. Lepidella are described from Guyana. Amanita cyanochlorinosma sp. nov., Amanita fulvoalba sp. nov., and Amanita guyanensis sp. nov. represent the latest additions to the growing body of newly described ectomycorrhizal fungi native to Dicymbe-dominated tropical rainforests. Macro- and micromorphological characters, habitat, and DNA sequence data for the ITS, nrLSU, rpb2, and ef1-α are provided for each taxon, and ß-tubulin for most. Distinctive morphological features warrant the recognition of the three new species and a molecular phylogenetic analysis of taxa across Amanita subgen. Lepidella corroborates their infrageneric placements.

Maternal phenylketonuria syndrome: congenital heart defects, microcephaly, and developmental outcomes.

OBJECTIVE: A cohort of women with phenylketonuria (PKU) were selected to explore the impact of phenylalanine (Phe) levels and other factors on congenital heart defects (CHDs), microcephaly, and development of their offspring. STUDY DESIGN: Three hundred fifty-four women with PKU were followed up weekly with diet records, blood Phe levels, and sonograms obtained at 18 to 20 and 32 weeks' gestation. At birth, 413 offspring were examined and followed up at 6 months and annually by means of Bayley Mental Developmental Index and Psychomotor Developmental Index tests at 1 and 2 years. The women had Wechsler Adult Intelligence Scales and DNA testing. RESULTS: Thirty-one offspring had CHDs; of these, 17 also had microcephaly. Mean Phe levels at 4 to 8 weeks' gestation predicted CHDs (P <.0001). An infant with a CHD had a 3-fold risk of having microcephaly when the mother had higher Phe levels (P =.02). The Bayley Mental Developmental Index and Psychomotor Developmental Index scores correlated with both CHDs (P =.037 and.0015, respectively) and microcephaly (P =.0001 for both). No direct relationship to the PKU mutation was found. CONCLUSION: None of the women whose offspring had CHDs had blood Phe levels in control during the first 8 weeks of gestation. Women with PKU need to be well controlled on a low-phenylalanine diet before conception and throughout pregnancy.

Country watch: Venezuela.

PIP: Since 1990, ACCSI has worked to safeguard the rights of people living with HIV/AIDS in Venezuela. The Pan American Health Organization (PAHO) had recognized ACCSI's program by 1992, enabling the nongovernmental organization (NGO) to establish fixed office hours during which it can receive clients. ACCSI does not distinguish between people living with HIV/AIDS and others, but considers it important to regulate AIDS as a public health issue within the context of human rights and ethics. Almost all of the Legal Office's initial cases were related to discrimination in the workplace, health centers, and educational institutions, but the ACCSI now also addresses family problems related to adoption, insurance, and inheritance, among others. To cope with the increase in service caseload, services have been extended through collaboration with specialized organizations concerned with human rights, women, children, prisons, and indigenous people. ACCSI influences governmental policy-making through its participation in the National AIDS Program. Moreover, ACCSI's networking activities ensure that the topic of HIV/AIDS and human rights is now always included in seminars and conferences in Venezuela, even when they are concerned with sexually transmitted diseases or other medical issues. Almost every time the press report upon AIDS, they consult the NGO. Everyday more people in Venezuela are standing up for their rights and denouncing discrimination against people infected with HIV.^ieng

Phenylalanine hydroxylase expression in liver of a fetus with phenylketonuria.

The expression and activity of phenylalanine hydroxylase was studied in the liver of a fetus aborted after prenatal diagnosis of phenylketonuria. No phenylalanine hydroxylase enzymatic activity or immunoreactive protein was detectable in the PKU liver specimen, though both enzymatic activity and immunoreactive protein were detectable in control specimens of similar gestational age. Phenylalanine hydroxylase messenger RNA of normal size was present in the PKU fetal liver at normal abundance. These results confirm the genetic diagnosis of PKU in this fetus and indicate that the mutations in this fetus affect translation or stability of the phenylalanine hydroxylase protein.

A lente intraocular de camara anterior. Suas vantagens, requisitos e os fundamentos de um novo desenho. / Anterior chamber intraocular lens: advantages, requirements and basis of a new design

O autor propoe uma nova lente de camara anterior, de sua autoria.Relaciona uma serie de qualidades, extraidas de muitas outras ja em uso, as quais estariam reunidas nesta sua lente. Enumera uma serie de vantagens em defesa das lentes de camara anterior

Preliminary report on the effects of diet discontinuation in PKU.

A collaborative study of diet discontinuation in children with PKU was initiated in 1973. Children treated with the phenylalanine-restricted diet since early infancy were randomly assigned to continue or discontinue dietary therapy at age 6 years after parental consent was obtained. The 115 children participating in this study range in age from 8 to 13 years. At 6 years of age, the IQ of continuers and discontinuers was 101 and 97, respectively. At 8 years, WISC Full-Scale IQ scores adjusted for mean differences on the 6-year Stanford-Binet IQ were 101 for continuers and 98 for discontinuers (P = 0.075). School performance measured by the Wide Range Achievement Test showed significant differences on reading (3.9 vs 3.2) and spelling (3.3 vs 2.9) grade placement, although scores were above actual grade placement (2.7 vs 2.6) for both groups. Continuers and discontinuers were not different in arithmetic scores, with performance at grade placement of 2.7 and 2.6 respectively. Although these data are preliminary in nature, they suggest that subtle changes in cerebral function may occur in children with PKU in whom the phenylalanine diet has been discontinued.