Lotte Strauss

Twin transfusion syndrome: A review of 19 cases studied at one institution

In 130 monochorial twin pregnancies studied at one institution, twin transfusion syndrome was diagnosed 19 times. In 10 instances both twins were lost in the perinatal period; in 5, only one twin survived; in 4, both twins survived. Most of the deaths occured in utero early in pregnancy. Hydramnios was frequent. The twin transfusion syndrome is shown to account for a significant number of the fetal and neonatal deaths in twin pregnancies and is responsible in large measure for the greater fetal and neonatal risk in monochorial than in dichorial twin gestation.

Neonatal Hepatitis and Biliary Atresia Associated with Trisomy 17–18 Syndrome

Abstract Among 19 autopsied cases of 17–18 trisomy syndrome, 10 confirmed by cytogenetic studies, nine diagnosed on the basis of phenotypic characteristics, seven cases of neonatal hepatitis were found, including two cases of biliary atresia. Liveborn infants had early onset of icterus, with elevated total and conjugated serum bilirubin concentrations. All had intrahepatic cholestasis and variable combinations of hepatocellular and portal-tract involvement. Giant-cell transformation was present in two infants. Focal obliteration of bile ducts, secondary to cholangitis, was frequent, and may have been the cause of extrahepatic biliary atresia in two cases. The nature of the association between this chromosomal aberration and hepatobiliary disease is unclear. The possible role of virus has not been sufficiently explored.

Immunocytochemical Observations in Periarteritis Nodosa

Excerpt The pathogenesis of periarteritis is controversial. Apparently it is not a nosologic entity, but a collective designation for various forms of arteritis with different pathogeneses. Clinica...

Aneurysm of the left ventricle due to congenital muscle defect in an infant: Report of a case with discussion of pathogenesis of associated endocardial fibroelastosis

Abstract The desire for specificity has evolved special interest in antihypertensive drugs which act principally at the sympathetic arteriolar neuroeffector site and/or on norepinephrine, the neurohumoral transmitting agent. The present report deals with pharmacologie and clinical studies on several such drugs: reserpine, guanethidine and bretylium. As is well known and confirmed by us, reserpine and guanethidine deplete catecholamines from neuroeffector junctions as well as from other storage sites. Bretylium, on the other hand, does not deplete catecholamines and can prevent the catecholamine depleting actions of reserpine and guanethidine. Differences also exist in their influences on tritium-labeled nor-epinephrine uptake by cardiac tissue. It is known that the hemodynamic responses to administered norepinephrine are exaggerated and responses to tyramine and certain other pressor amines are reduced when intrinsic catecholamine stores are depleted. Applying these tools to the present problem, animals have shown the expected exaggerated responses to norepinephrine and reduced tyramine when reserpine and guanethidine were given and the unaltered responses when bretylium was given. In humans, most of the responses coincided with those of animals. The responses to the challenges of presser amines as well as hypotensive effects could be altered by administering these medications in various sequences. These studies indicate that, although many agents exert their major effects at the sympathetic neuroeffector site, the precise actions are not necessarily identical. Investigations into the mechanisms of action of these drugs have altered long established physiologic concepts of the efferent sympathetic nervous system.

Pneumonitis in acute lymphatic leukemia during methotrexate therapy

Summary Four cases of an acute, self-limited respiratory illness in children who were receiving intermittent methotrexate therapy for the maintenance of remission of acute lymphatic leukemia are described; the description includes clinical and radiographic features, viral and serologic studies, and pulmonary biopsies in two of the patients. The clinical, radiographic, and histologic features were nonspecific. Changing complement fixation titers for respiratory syncytial virus in two patients and pleuropneumonia-like organisms in a third suggested that this syndrome may be caused by an infectious process, modified by the underlying illness or by the antimetabolite therapy.

Congenital absence of the right pulmonary artery: Report of a case in a five month old infant, with suggestive evidence of unilateral pulmonary hypertension∗

Abstract The clinical history and anatomic findings in a case of isolated congenital absence of the right pulmonary artery are described and the literature reviewed. Medial hypertrophy and narrowing of the lumen of small arteries only in the lung supplied by the pulmonary artery, together with hypertrophy of the right ventricle, are interpreted as evidence of unilateral pulmonary hypertension. It is suggested that hypertension in the left lung may be the result of vasoconstriction in response to increased blood flow.

Chromosomal sex detection in the human newborn and fetus from examination of the umbilical cord, placental tissue, and fetal membranes.

Five years ago Barr and his associates (Moore et al., 1953) announced a new and relatively simple histological method utilizing a skin biopsy for the diagnosis of sex. This is based upon the fact that intermitotic nuclei of females contain a characteristic mass of chromatin, the sex chromatin which is rarely, if ever, present in males. Since these particular chromocenters presumably represent fused heterochromatic portions of the XX sex-chromosomal complex of females, nuclei possessing the sex-chromatin body are found only in specimens from chromosomal females (Barr, 1957; Reitalu, 1957). Rapidly confirmed by numerous investigators (Hunter et al., 1954; Marberger and Kelson, 1954; Emery and McMillan, 19.54; Sohval et al., 1955), these observations have been applied to a variety of anatomical preparations and clinical conditons. As a result there have been two salient developments: first, cytological methods for the detect-ion of chromosomal sex have been described employing buccal scrapings (Moore and Barr, 1955; Marberger el al., 1955), smears from the vagina and urogenital sinus (Carpentier et al., 1956), leukocytes from the peripheral blood (Davidson and Smith, 1954), and cells of the amniotic fluid obtained prenatally (Scrr et al., 1955; James, 1956). In the last instance, the cells are of €eta1 origin, derived principally from the mucosae of the alimentary and genitourinary tracts rather than from the skin or amnion (Rosa and Fanard, 1951; Lennox, 1956; Makowski el al., 1956). I n addition, a nuclear sex difference has been observed histologically by nilarberger and Nelson (1955) in mesenchymal cells of thc amnion of human fetal membranes, by Roitman (1955) in the nuclei of smooth muscle cells of the umbilical vessels, by Glcnister (1956) and by I’ark (1957) in early human trophoblastic nuclei, and by Bohle and Hienz (1056) in the connective tissue and endothelial cells of human term placental villi. Second, t h e determination of chromosomal sex in certain patients with congenital errors of sex development has contributed significantly to our understanding of the pathogenesis and to the management of these conditions. The purpose of this communication is to report the results of a systematic investigation of the nuclear sex chromatin in the umbilical cord, placental tissue, and fetal membranes of the human newborn and of fetuses of various ages. The demonstration of concordant nuclear sex differences i n these structures represents an additional technique for chromosomal sex detection. Some advantage accrues from the fact that availability or manipulation of the newborn or fetus itself is not required.

Tumor infiltration of the bone marrow: Comparative study using computed tomography

During the past two years five patients with malignant primary bone tumors undergoing amputation or resection of the involved bone had preoperative computed tomography (CT) examination of the medullary cavity. The contralateral extremity was used as a control in all cases. The positive attenuation coefficient of the marrow indicated the extent of tumor infiltration. The correlation between the preoperative CT and pathologic measurement ranged between 1–8 mm, an accuracy greater than 94%.

Trisomy 8 mosaicism syndrome. Report of monozygotic twins.

Monozygotic twins were born with the phenotypical appearance of the trisomy 8 syndrome. The first twin, a stillborn, had autopsy findings suggestive of trisomy 8 syndrome. Cultured lymphocytes and skin fibroblasts of the second, liveborn twin, showed trisomy 8. While the lymphocyte culture showed 46/47,+8 mosaicism, with normal cells predominating, skin fibroblasts yielded only cells with trisomy 8. Bone marrow preparation showed only normal cells, 46,XY. Repeat lymphocyte culture at age 14 months, showed a reduced number of trisomy 8 cells. We would like to emphasize the importance of cytogenetic studies in early infancy, using both peripheral lymphocyte and skin fibroblast cultures, to increase the chance of detecting chromosomal abnormalities.

Demonstration of Gamma Globulin in Vascular Lesions of Experimental Necrotizing Arteritis in the Rat.

Summary Experimental necrotizing arteritis was produced in female Sprague-Dawley rats by administration of large doses of DOCA and NaCl after unilateral nephrectomy. Presence of RGG in damaged vessel walls was demonstrated by fluorescence antibody technic of Coons. Gamma globulin is deposited in smooth muscle of damaged small arteries, especially in early phases of vascular damage, suggesting that antibody may not be required for this process.