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Biomedical subjects

D J de Sa

Publications and source records attributed to D J de Sa.

12 recordsLinked to original sources

Fetal liver disease may precede extrahepatic siderosis in neonatal hemochromatosis.

Three children of a mother with biopsy-confirmed posttransfusional hepatitis of undetermined etiology (non-A, non-B hepatitis) died in utero or in infancy. All had liver disease of intrauterine onset. The two liveborn children died of the consequences of severe hepatic insufficiency manifest at birth and met clinicopathologic criteria for neonatal hemochromatosis. Although hepatic architecture in the stillborn fetus was markedly disordered, with hepatocyte giant cell transformation, extrahepatic siderosis was not present and hepatic siderosis was minimal. These findings indicate that in some cases of neonatal hemochromatosis, extrahepatic siderosis may be caused by hepatic injury rather than primarily due to excessive transport of iron from mother to fetus and support speculation that in some instances an infective agent may be responsible.

Adolescent

Fetal inflammatory response in second trimester candidal chorioamnionitis.

Candidal chorioamnionitis is an uncommon and apparently rather indolent intrauterine infection in which the fetus is able to marshal some of the immunological forces at its disposal against an easily visualized antigen impinging on lung mucosal surfaces. In a retrospective histological study of one of the largest reported series of these cases, we have encountered one each of 13, 16 and 22 weeks gestation, respectively. Of these, the youngest at 13 weeks showed no inflammatory response or positive cells on immunoperoxidase staining for immunoglobulins and proliferating Candida colonies were evident in the lungs. The 16 and 22 week cases revealed a unique giant cell response in the terminal airways and increasing numbers of cells staining positively for immunoglobulins, predominantly IgM, but with an increased proportion of IgA positive cells in the older case. Preliminary studies with a pan-T-cell antiserum on paraffin-embedded lung tissue from these cases have been encouraging with few positive cells seen in sections from the 13-week or control cases but abundant cells in the lungs of the two older infected cases. Some aspects of the relationship of these uncommonly encountered cases to the ontogeny of human immunity are discussed.

Candidiasis

Fatal malformations of the larynx and upper trachea.

Five infants with a spectrum of rare malformations of the larynx and trachea are presented. All infants died because of problems with intubation and ventilation. The series reflects the wide variety and complexity of malformations that may co-exist, and successful management may require open tracheotomy.

Abnormalities, Multiple

Ultrastructure of lung in pulmonary veno-occlusive disease.

A 17-year-old boy died of severe pulmonary hypertension due to pulmonary veno-occlusive disease. The condition was diagnosed in a lung biopsy specimen and confirmed at necropsy. The lung specimen was studied by electron microscopy and immunofluorescence microscopy. The occluded pulmonary veins were lined by intact endothelial cells, beneath which was a haphazard proliferation of collagen fibrils and smooth muscle cells. The alveolar capillaries showed thickening of the endothelial cell basement membrane with an increase in the number of cytoplasmic processes of pericytes. Electron-dense deposits were located within the thickened basement membrane. These deposits were considered to represent disintegrating extravasated erythrocytes rather than immune complexes because immunofluorescence microscopy showed no immunoglobulin or complement deposition within the lung.

Adolescent

Coronary arterial lesions and myocardial necrosis in stillbirths and infants.

In a detailed study of the coronary arterial tree and myocardium in 256 stillbirths and infants, abnormalities of the coronary arterial tree were noticed in 79 infants, and necrotic lesions of the myocardium in 111 infants. Of the 79 infants with arterial lesions, 70 had associated myocardial necrosis or scarring, or both; the group with coronary arterial lesions, therefore, accounted for the majority of cases with myocardial damage. The myocardial lesions varied from small zones of subendocardial damage, to larger 'geographical' zones of necrosis scattered haphazardly through the myocardium, and a small group where massive necrotic lesions of the papillary muscles were present. While the coronary arterial lesions were associated with all three patterns, they were particularly found in association with the 'geographical' and papillary muscle changes. The coronary arterial lesions varied from zones of acute focal, medial necrosis to severe proliferative intimal lesions and medial defects, with a distinct progression of changes from the acute to the more established lesions. The coronary arterial lesions were seen most commonly in association with conditions that could produce severe hypoxia, and it is argued that they result from hypoxia. It is suggested further that the myocardial damage, so commonly associated with the coronary arterial lesions, could compromise the ability of the hypoxic infant to respond to such an insult. The coronary arterial lesions seen in this group of young infants could offer one explanation for the later development of a variety of other pathological conditions seen in adolescents and young adults.

Coronary Disease

Chorioamnionitis and funisitis due to Corynebacterium kutscheri.

When isolated from the female genital tract, diphtheroids are usually regarded as commensal organisms. Corynebacterium kutscheri however is a pathogen in laboratory rodents. We report a case in which C. kutscheri was isolated as a pure culture from the umbilical cord and from other surface sites in an infant. Histological examination of the cord and membranes demonstrated the organisms within these fetal tissues. The organisms evoked a fetal cellular response. The importance of recognising commensals as potential pathogens in states of altered host resistance in stressed.

Corynebacterium

Stress response and its relationship to cystic (pseudofollicular) change in the definitive cortex of the adrenal gland in stillborn infants.

The adrenal glands of 41 fresh stillbirths were studied and a 'stress response' pattern could be seen in 28. In these glands the stress response was characterised by compact cell change, lipid depletion, excess pyroninophilia, and dilatation of the very prominent granular endoplasmic reticulum. Scattered areas of cytolysis of cells, especially of the definitive cortex, gave rise to the commonly seen cystic (pseudofollicular) change and it was obvious that cells undergoing lysis were severely 'stressed'. In 2 infants there was a 'clear cell reversal' pattern. Histological and ultrastructural changes of the stress response were not identified in 11. Infants of low birthweight score were somewhat more commonly represented in the group that did not show a stress response. Cytolytic changes accompanying a stress response were commoner in immature infants. It is argued that cystic (pseudofollicular) change in the adrenal cortex of the newborn signifies a previous stress reaction.

Adrenal Cortex