Neu-Laxova syndrome in an appropriate for gestational age newborn.

Neu-Laxova syndrome is a rare lethal congenital disorder involving multiple systems. Intrauterine growth retardation, ichthyosis, microcephaly, abnormal facial findings, and limb contractures are its key features. We present a case of Neu-Laxova syndrome in a male appropriate for gestational age (AGA) newborn with characteristic features including ichthyosis, microcephaly, severe ectropion, rudimentary ears, eclabion, limb contractures, and hypoplastic genitalia. The patient was born at 38 weeks of gestation to consanguinous Turkish parents. The mother was a 20-year-old primi gravida with lack of prenatal follow-up. Therefore, the case was diagnosed postnatally, and he died 5 days later. Because of the autosomal recessive inheritance of Neu-Laxova syndrome, in countries with high rates of consanguineous marriage, such as Turkey, physicians have to know this syndrome, and serial prenatal ultrasound examinations with genetic counseling should be performed on pregnant women at high risk. To the best of our knowledge, this is the first case described in an AGA newborn.

[Abortion in Japan]. / Schwangerschaftsabbruch in Japan.

In Japan, the artificial abortion is a penal offence; only in the presence of certain conditions it is authorized under the provision of the Eugenic Protection Law which was promulgated in 1948. According to the law, the artificial abortion is restricted to the period, in which the fetus is not viable outside of the uterus. This period is prescribed by notification from the Ministry of Public Welfare; up to now it has been shortened twice (1976, 1991). Due to the introduction of economic reasons in the list of conditions and the simplification of the procedure the artificial abortion in Japan was virtually liberalized. Prosecution for illegal abortion is very rare in recent years. The number of reported artificial abortions decreases; in the about last 30 years it reduced by half. However, the increase in the number of abortions in women younger than 20 years of age is a problem. The abortion in teenagers is late compared with that in other age groups. Although the number of neonaticides does not seem to increase, the increase in the number of abortions in teenagers remains a serious problem in Japan.

PIP: In Japan abortion is essentially a crime except for certain indications. These indications have to do with mental illness, hereditary disease, leprosy, threat to the health of the mother, and pregnancy resulting from rape or threat. These indications entered into force under the eugenic protection law of 1948. On January 1, 1991, a new regulation became effective that shortened the duration of pregnancy termination from 23 weeks of gestation to 21 weeks in view of the advancement of medicine that made it possible for prematurely born children to survive outside the uterus. In one case in 1988, when the fetus of a 16-year old girl was aborted in the 25th week of pregnancy and left unattended although alive, the doctor was indicted and punished, although the probability of survival of the child was estimated at about 50%. Under law the doctor who has performed a legal abortion is obligated to report it to the authorities who compile annual statistics. Since 1955, when the abortion rate was the highest (about 1,150,000 abortions), the number has been decreasing steadily. In 1991 the abortion rate was 13.9/1000 women of reproductive age (15-50 years); however, great differences existed between prefectures (6.4-26.0/1000). The decline of the incidence of abortion can be attributed to the increase of contraceptive usage. It is alarming that the rate of abortions has increased among women under 20 years of age and at later phases of gestation. In 1990, out of a total of 33,286 abortions, 1015 were for women under 20 years old at 20 to 23 weeks of pregnancy, and this rate was higher than the rate in older age groups. Unquestionably, the abolition of the requirement in 1952 mandating that the abortion seeker undergo an examination by two doctors has liberalized the abortion law. However, many young pregnant women who need help do not get adequate support and counseling and may end up in prostitution.

Avaliaçäo antropométrica e perfil plasmático de ácidos graxos de recém-nascidos pré-termo submetidos a duas dietas isocalóricas e com diferentes composiçoes lipídicas / Anthropometric evaluation and plasm fatty acids profile of preterm newborn infants subjected 2 isocaloric diets of different lipid composition

Este trabalho analisou o perfil de ácidos graxos plamáticos eo crescimento somático de 20 recém-nascidos pré-termos, com idade gestacional entre 30 a 35 semanas, admitidos no Berçário do Hospital das Clínicas da Faculdade de Medicina de Ribeiräo Preto da Universidade de Säo Paulo, submetidos, por um período de aproximadamente 20 dias, a duas dietas isocalóricas e com diferentes composiçöes liídicas. Um grupo de 10 crianças GRUPO L.D.S. usou uma dieta contendo 0,5% das calorias totais (54,5 mg/100 cal) como ácido linoléico (C18:2) e relaçäo PS=0,053. Os outros 10 recém-nascidos GRUPO L.D.O. foram colocados numa dieta contendo 10,5% das calorias totais (1171mg/100 kcal) como ácido linoléico (C18:2) e a relaçäo P/S = 0,508. Os grupos no período imediato antes da instituiçäo das dietas em estudo apresentaram valores plasmáticos dos ácidos linoléico (C18:2) e aracdônico (20:4) semelhantes entre si e superiores aos limites mínimos normais estabelecidos por WIESE etalii (1958) e HANSEN ET ALII (1963), refletindo reservas corporais e dietas semelhantes previamente ao estudo. Após o uso da dieta no GRUPO L.D.S. houve uma queda significante (p < 0,05) dos níveis plasmáticos ácidos C16:0 (palmítico), C16:1 (palmitoléico) e C18:1 (oléico) se elevaram significantemente (p < 0,05). As alteraçöes encontradas foram semelhantes as relatadas em diversos trabalhos da literatura e poderiam ser considerados como evidências bioquímicas de deficiência de ácidos graxos essenciais. No GRUPO L.D.O. após o uso da dieta verificamos uma queda nos níveis plasmáticos do ácido aracdônico (C20:4 a níveis inferiores aos mínimos normais estabelecidos na literatura em 90% dos recém-nascidos pré-termos, mas a queda foi menor do que a verificada no GRUPO L.D.S. (p < 0,,5). O ácido linoléico (C18:2) apresentou elevaçäo no seu nível plasmático em 50% dos pré-termos estudados e nos outros 50% os valores foram semelhantes ou ligeiramente inferiores ao período prédieta, mas todos apresentaram níveis superiores aos limites mínimos normais e aos do GRUPO L.D.S. (p , 0,05). Os niveis plasmticos elevados de C18:2 no GRUPO L.D.O. após o uso da dieta com elevado conteúdo de ácido linoléico concordam com a melhor absorçäo de gorduras verificada neste grupo em relaçäo ao GRUPO L.D.S. (p < 0,05)...