[Intrauterine and perinatal mortality: comparative analysis of 3,904 necropsies, Hospital de Clínicas, Curitiba, from 1960 to 1995] / Mortalidade intra-uterina e perinatal: análise comparativa de 3.904 necropsias do Hospital de Clínicas de Curitiba no período de 1960 a 1995.

OBJECTIVE: To analyze the influence of the perinatal, fetal or maternal pathological processes in the induction of intra or extrauterine death as a guideline to a better perinatal medical assistance.METHODS: The authors studied retrospectively 3,094 consecutive necropsy cases carried out in stillborn fetuses (NM) and newborns (ON) in the period between 1960 and 1995 in the SAP of HC, Curitiba. The data analyzed included sex, time of gestation and causes of death of fetus and newborns. All maternal conditions that might have contributed to intra or extrauterine fetal death were also studied.RESULTS: The prevalence of intrauterine death due to maternal diseases was two times higher than extrauterine. In contrast, the fetal diseases were responsible mainly for extrauterine deaths. Primary placental diseases were responsible for 30% of the deaths with low time of gestation and 40% of them in the end of gestation. The same group of diseases was responsible for only 15-9% of the extrauterine deaths. Diseases of labor contributed to 12-20% of deaths after birth while only 7 to 17% of intrauterine deaths.CONCLUSION: Necropsy studies contribute to the identification of pathologic processes which affect the patients of any medical center. If a pregnancy is associated with a certain disease, the epidemiologic data of this study can help to identify the period of major risk of death of the fetus or newborn.

[Krabbe s disease - case report] / Doença de Krabbe - relato de caso.

OBJECTIVE: Report a case of Krabbés disease with necropsy. METHODS: Review of medical and necropsy records. RESULTS: An 8 months-old male patient developed tremors, swallowing difficulty and excessive salivation for 4 months prior to admission, evolving with vomiting and fever. Physical examination showed microcephaly and diffuse pigmentation of the retinae. Neurological examination showed flexion of upper limbs with spastic hyperthony, symmetrical global hyperreflexia, nystagmus and spontaneous spasms. EEG showed multifocal irritative activity. There was increase in both CSF protein and gamaglobulin. The patient evolved with transitory hyperthermia, vomiting and pneumopathy, dying on the 23rd day after admission. Post mortem studies revealed microcephaly with widening of brain sulci. Histological examination revealed several globoid cells in the deep portion of the white matter, reactive gliosis and demyelination. CONCLUSIONS: These findings were similar to those in the world literature, indicating a poor prognosis due to substantial brain damage.