Cluster of iniencephaly in Miami.

This report details a cluster of 5 cases of iniencephaly with anencephaly and rachischisis occurring over a 4-month period at Jackson Memorial Hospital/University of Miami Medical Center in Miami, Florida. All 5 cases of this rare, lethal, congenital malformation seen in the cluster included diaphragmatic defects with accompanying hernia, omphalocele, small adrenals, renal dysmaturity, gastrointestinal malformations, cleft lip and palate, and hypoplastic lungs. No single causative agent for this cluster was identified. A brief review of the literature regarding categorization of these malformations and as a discussion of the embryological basis for these lesions and possible etiologic factors are included.

Congenital syphilis: the University of Miami/Jackson Memorial Medical Center experience, 1986-1988.

Between January 1, 1986 and July 1, 1988, 56 cases of congenital syphilis were identified at the University of Miami/Jackson Memorial Medical Center. The overall rate was 18.4 cases per 10,000 births, with a threefold increase found from 1986 to 1988. A case-control study using matched pairs was done to identify differences in maternal demographics and pregnancy outcome. Congenital syphilis case mothers were predominantly black American women who lacked prenatal care (67%) and who were substance abusers (71%) significantly more often than their matched controls (P less than .005). Three cases of seroconversion in pregnancy were identified. Failure to screen or inappropriate treatment occurred in four patients. Seven women were treated during pregnancy: Five received benzathine penicillin G for 3 consecutive weeks and two received erythromycin. All treated patients presented for initial care in the late second or third trimester. Thirty-seven infants (66%) were live-born and 19 (34%) were stillborn. Preterm labor and premature rupture of the membranes were significantly more common in infected pregnancies than in controls (P less than .005). Live-born case infants had significantly lower birth weights than controls (P less than .005), with 21% of case infants growth-retarded. Seven neonatal deaths and one infant death occurred. The resultant perinatal mortality rate from congenital syphilis in this series was 464 pe 1000.

Histopathologic features of the liver in pediatric acquired immune deficiency syndrome.

Autopsy and liver biopsy specimens from 30 pediatric patients with acquired immune deficiency syndrome (AIDS) or AIDS-related complex (ARC) were retrospectively reviewed. Of 28 cases with histologic abnormalities, the following findings were noted singly or in combination: giant-cell transformation, cytomegalovirus inclusions, Kaposi's sarcoma, diffuse lymphoplasmocytic infiltrate, granulomatous hepatitis, mild portal inflammation, necrosis around central veins, steatosis, and cholestasis. For the most part, abnormalities in the liver were not predictive of those in other organs, but the two children with the diffuse parenchymal lymphoplasmocytic infiltrate also had lymphoid interstitial pneumonitis (LIP). Liver histopathology in pediatric patients with AIDS shares some features with that in adults, but appreciable differences are noted. In particular, these differences include the higher frequency of giant-cell transformation and the lower frequency of granulomas in children and the observation of diffuse lymphoplasmocytic infiltrate associated with LIP.

Congenital syphilis and necrotizing funisitis.

During the past 5 years we encountered 16 cases of necrotizing funisitis, a deeply seated inflammatory process within the matrix of the umbilical cord, and established that all of them were associated with maternal and congenital syphilis. During that time necrotizing funisitis was not found to be associated with any other infection. The relationship was unexpected on the basis of current knowledge of the condition, but we found the older medical literature documents syphilis as the virtually specific cause of deep funisitis with angiophlebitis. Necrotizing funisitis has a characteristic macroscopic appearance, the "barberpole" cord, that permits the presumptive diagnosis of congenital syphilis at the time of birth.

Morphologic alterations in rat brain following systemic and intraventricular methotrexate injection: light and electron microscopic studies.

To determine the morphological substrate of acute methotrexate (MTX) encephalopathy, light and electron microscopic studies were performed on rat brains after short-term intraperitoneal (IP) and intraventricular (IV) injections of MTX. In both models, Alzheimer type II astrocytosis was the initial and major pathologic alteration seen by light microscopy. The neurons, oligodendrocytes, myelin and endothelial cells were relatively spared. Ultrastructural studies showed pleomorphism and condensation of mitochondria, membrane-bound vacuoles, prominent stacks of sparsely granular, rough endoplasmic reticulum and progressive hydropic swelling of astrocytic perikarya and their processes. The astroglial alterations were reversible after cessation of the drug but persisted for a longer time with repeated IP administration. Gastrointestinal complications and overall mortality were also greater with higher doses and increasing frequency of IP MTX injection. White matter necrosis was noted only after IV injection of high-dose MTX. The neuropathologic changes of MTX leukoencephalopathy can be replicated in an animal model by IV injection of the drug. The reversibility of the changes that were seen following IP administration correlates with the transient neurologic deficits observed in some patients after high-dose systemic MTX therapy. The initially selective astroglial effect suggests that astrocytes might be a target for MTX toxicity, although other central nervous system components may also be adversely affected by the drug.

Listeriosis as a cause of maternal death: an obstetric complication of the acquired immunodeficiency syndrome (AIDS).

A case of maternal death due to Listeria monocytogenes bacteremia, with survival of the prematurely delivered infant, is presented. Lymphopenia and a Haitian origin suggest that the fatal outcome was related to the acquired immunodeficiency syndrome (AIDS). To our knowledge, this is the first recorded instance of a maternal death due to listeriosis.

Microangiopathic hemolytic anemia and thrombocytopenia in a neonate associated with a large placental chorioangioma.

The case of an infant born in association with a large chorioangioma of the placenta is presented. The maternal complications of polyhydramnios, preeclampsia, and premature labor are those described in the "syndrome" of a chorioangioma. The immediate neonatal course was unusually complicated by severe microangiopathic anemia with persistent thrombocytopenia and hemolysis which required three exchange blood transfusions. The relationship between the neonatal complications and the placental tumor is discussed in terms of possible pathophysiologic mechanisms.

The association of Wilms' tumor with second primary malignancies.

Medulloblastoma occurs uncommonly in the neonatal period. Metastatic Wilm's tumor in a newborn is extremely rare. The coincidence of these two neoplasms in one infant, and an equally uncommon combination of Wilms' tumor and a cerebellar medulloephithelioma in another child prompted this report. The authors speculate on the mechanism of oncogenesis in these and other infants with neonatal malignancies.

Metastatic neonatal Wilms' tumor: a case report with review of the literature.

An unusual case of congenital Wilms' tumor is discussed. Although the very existence of neonatal Wilms' has been challenged, this case represents a well documented example, rendered all the more unusual by concurrent metastatic disease and a second primary malignancy in the posterior fossa.