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Late termination of pregnancy in North Holland.

OBJECTIVES: To estimate the extent to which late termination of pregnancy (gestational period > 24 weeks) is practised, to find out what fetal diseases and abnormalities lead to termination and to obtain insight into the clinical judgement and decision-making process by gynaecologists with regard to termination. DESIGN: A retrospective study. PLACE: Province of North Holland, The Netherlands. METHODS: An anonymous printed questionnaire, was sent to all associations or departments of gynaecology in the 21 hospitals in North Holland. The period of study covered the years 1990 to 1994. RESULTS: Completed questionnaires were received from 19 associations or departments in the 21 hospitals (90% response). In the five-year period under study in 14 of these hospitals had terminated 103 pregnancies beyond the 24th week of gestation. Antenatal sonography had been performed on all these pregnancies. Anencephaly was diagnosed before termination in 21% and severe chromosomal defects in a further 21%. Other severe abnormalities were neural groove defects, such as hydrocephaly and/or spina bifida (16%), no renal function (12%) and skeletal abnormalities (11%). In 87% the antenatal diagnosis was verified postnatally; in 78% this was done by autopsy. The diagnosis was confirmed in all these cases ('almost confirmed' in two cases). In every case the pregnant woman herself had asked for her pregnancy to be terminated. In 78% the prognosis was discussed with other medical specialists and the professionals concerned with management; there was a consultation about the termination in 91% of the cases. A report was always compiled for the medical records. CONCLUSION: Late termination of pregnancy is practised on a substantial scale in North Holland. The reasons for termination were severe structural abnormalities which, in most cases, were not compatible with survival. The gynaecologists in North Holland dealt prudently and responsibly with late termination of pregnancy, although they did not always adhere to the guidelines set out in the 1994 report of the Netherlands Association for Obstetrics and Gynaecology.

Abortion, Induced↗

Lethal cytomegalovirus infection in preterm infants: clinical, radiological, and neuropathological findings.

Fifteen premature infants with lethal congenital cytomegalovirus infection were studied to determine the clinical, neuroradiological, and neuropathological characteristics of the disease in this population. Nine infants were liveborn but died at a postnatal age of 18 +/- 21 days; 6 infants were stillborn. Clinical findings in liveborn infants included microcephaly (77%), seizures (55%), hypotonia (33%), and multiple contractures (18%). Ophthalmological findings included chorioretinitis, optic atrophy, and corneal opacities. Neuroradiological findings included the postnatal evolution of periventricular calcification in 1 infant, and cerebellar hypoplasia diagnosed by magnetic resonance imaging in 1 infant. Neuropathological findings included periventricular necrosis and calcification (12), associated diffuse calcification frequently involving the convexity of the gyri (6), cerebellar hypoplasia (5), periventricular leukomalacia (2), intraventricular hemorrhage (2), hydrocephalus (2), and porencephalic cyst (1). Intranuclear inclusion bodies within the brain were observed in 4 infants, whereas systemic inclusion bodies were present in all infants. These data indicate several atypical findings in preterm infants rarely reported in term infants, including hypotonia, multiple contractures, periventricular leukomalacia, and optic atrophy.

Atrophy↗

GM2 ganglioside in fetal Tay-Sachs disease brain cultures: a model system for the disease.

Cultured cells derived from Tay-Sachs disease (TSD) fetal cerebellum were shown to accumulate GM2 ganglioside when compared with control cultures. In contrast, fibroblasts derived from TSD fetal lung do not contain GM2. GM2 was identified by thin-layer chromatography (TLC) and confirmed by gas-liquid chromatography (GLC). Unlike fetal TSD brain, the cell cultures established from fetal TSD brain have high concentrations of globoside and GD3 and little or no asialo GM2(GA2). The GM2 and related glycosphingolipids in these cultured cells contain a high percentage of C24:0 and C24:1 fatty acids. In spite of these differences, this TSD cell strain is unique in that it accumulates GM2, and can therefore serve as a useful in vitro model for the study of TSD.

Cells, Cultured↗

Development of a rapid autopsy program for studies of brain immunity.

Human glia are essential cellular models used for studies of neurodegenerative diseases. Fetal neuroglia are commonly used, as they can be recovered in large quantities and sustained for long periods in culture. However, fetal neuroglia may have limitations in reflecting adult diseases and additionally can pose ethical issues in translating products of abortion for research use. To address these concerns, we developed a rapid autopsy program to procure age- and disease-specific neuroglia from adult brain tissues within hours of death. The challenges in developing this initiative, reflecting experiences from 69 autopsies over 4 years, are presented.

Academic Medical Centers↗

Effects of chronic hypoxia and protein malnutrition on growth in the developing chick.

OBJECTIVE: The purpose of this study was to determine how chronic hypoxia and/or protein malnutrition in ovo affect growth in developing chicks. STUDY DESIGN: Chicken eggs were incubated under normoxic (21% oxygen; n = 30 eggs) or hypoxic (14% oxygen; n = 80 eggs) conditions. Hypoxia was imposed from day 0 (n = 38 eggs), day 10 (n = 22 eggs), or from day 0 to 10 (n = 20 eggs). Protein malnutrition alone (n = 20 eggs) or in combination with hypoxia (n = 24 eggs) was induced by removal of 10% of the estimated total albumin content of the egg. Embryos/chicks were killed and weighed at day 10, 15, or immediately after hatch; organs were removed and weighed. RESULTS: Embryos to which hypoxia was imposed from day 0 weighed less than control embryos at day 10, which stayed the same until hatch (64.67% +/- 3.56% egg mass vs 69.36% +/- 3.90% [mean +/- SD]; P <.05). Malnourished chicks at day 15 and at hatch (63.42% +/- 4.28%; P <.05) weighed less than control chicks, as did malnourished plus hypoxia chicks (59.74% +/- 3.41%; P <.001). Malnourished plus hypoxia chicks weighed less than malnourished chicks alone (P <.05). Embryos that were hypoxic from day 0 to 10 weighed less than control embryos at day 15 (P <.05), but not at hatch. At hatch, neither hypoxia nor malnutrition decreased crown-rump length. Brain and heart weights were increased in both malnourished groups, but not chicks that were hypoxic from day 0. CONCLUSION: Chick embryos exposed to malnutrition show asymmetric growth restriction with relative sparing of the brain and heart. Early growth restriction that was induced by hypoxia from the beginning of incubation is reversed by the restoration of normoxia at mid incubation.

Animals↗

Inactivation of the lysyl oxidase gene Lox leads to aortic aneurysms, cardiovascular dysfunction, and perinatal death in mice.

BACKGROUND: The lysyl oxidases are extracellular copper enzymes that initiate the crosslinking of collagens and elastin, 5 human isoenzymes having been characterized so far. The crosslinks formed provide the tensile strength and elastic properties for various extracellular matrices, including vascular walls. We studied the role of the first described isoenzyme Lox by inactivating its gene in mice. METHODS AND RESULTS: Murine Lox gene was disrupted by routine methods. Lox(-/-) mice died at the end of gestation or as neonates, necropsy of the live-born pups revealing large aortic aneurysms. In light microscopy, hazy and unruffled elastic lamellae in the Lox(-/-) aortas were observed, and electron microscopy of the aortic walls of the Lox(-/-) fetuses showed highly fragmented elastic fibers and discontinuity in the smooth muscle cell layers in Lox(-/-) fetuses. The wall of the aorta in the Lox(-/-) fetuses was significantly thicker, and the diameter of the aortic lumen was significantly smaller than that in the Lox(+/+) aortas. In Lox(-/-) fetuses, Doppler ultrasonography revealed increased impedance in the umbilical artery, descending aorta, and intracranial artery blood velocity waveforms, decreased mean velocities across cardiac inflow and outflow regions, and increased pulsatility in ductus venosus blood velocity waveforms. CONCLUSIONS: Lox has an essential role in the development and function of the cardiovascular system. Inactivation of the Lox gene causes structural alterations in the arterial walls, leading to abnormalities in the cardiovascular functions. Alterations in LOX activity may also play a critical role in certain human cardiovascular diseases.

Animals↗