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Biomedical subjects

M R Dische

Publications and source records attributed to M R Dische.

At least 19 recordsLinked to original sources
PubMed2002

Spectrum and progression of conduction abnormalities in infants born to mothers with anti-SSA/Ro-SSB/La antibodies.

The classic cardiac manifestation of neonatal lupus is congenital heart block, attributed to antibody-mediated inflammation and subsequent fibrosis of the atrioventricular (AV) node. In considering the pathologic process of injury it may be that tissue damage results in a range of conduction abnormalities. Identification of less-advanced degrees of block or of fibrosis around the AV node without any conduction abnormality on EKG would support this pathologic model, and serve as a potential marker for treatment if the conduction defect could be shown to progress. To ascertain the spectrum of arrhythmias associated with maternal anti-SSA/Ro-SSB/La antibodies, records of all children enrolled in the Research Registry for Neonatal Lupus were reviewed. Of 187 children with congenital heart block whose mothers have anti-SSA/Ro-SSB/La antibodies, nine had a prolonged PR interval on EKG at birth, four of whom progressed to more advanced AV block. A child whose younger sibling had third degree block was diagnosed with first degree block at age 10 years at the time of surgery for a broken wrist. Two children diagnosed in utero with second degree block were treated with dexamethasone and reverted to normal sinus rhythm by birth, but ultimately progressed to third degree block. Four children had second degree block at birth: of these, two progressed to third degree block. Sinus bradycardia (< 100 bpm) was present in three (3.8%) of 78 fetuses for whom atrial rates were recorded by echocardiogram. Of 40 neonates for whom EKGs were available, the mean atrial rate was 137+/-20 bpm (range 75-200). These data have important research and clinical implications. In contrast to the AV node, permanent sinoatrial nodal involvement is not clinically apparent. Perhaps many fetuses sustain mild inflammation, but resolution is variable, as suggested by the presence of incomplete AV block. Since subsequent progression of less-advanced degrees of block can occur, an EKG should be performed on all infants born to mothers with anti-SSA/Ro-SSB/La antibodies.

Antibodies, Antinuclear↗
PubMed1994

Osseous metaplasia in benign colorectal polyps.

We report two unique cases of metaplastic ossification occurring within a tubulovillous adenoma and a juvenile polyp. In both lesions, well-formed bony spicules were present that were adjacent to living epithelial cells. The metaplastic bone revealed vimentin positivity and cytokeratin negativity. The pathogenesis of osseous metaplasia in colorectal tumors remains unclear, but the process seems to have no clinical significance.

Adenoma, Villous↗
PubMed1991

Fetal fibronectin in cervical and vaginal secretions as a predictor of preterm delivery.

BACKGROUND: Preterm delivery is the leading cause of neonatal mortality in the United States, but efforts to address the problem are hampered by the inability to predict accurately which pregnancies are at risk. We postulated that damage to the fetal membranes may release fetal fibronectin into the cervix and vagina, giving rise to a biochemical marker for preterm delivery. METHODS: We measured fetal-fibronectin concentrations in cervical and vaginal secretions, amniotic fluid, and maternal plasma with a sensitive immunoassay using the monoclonal antibody FDC-6. Immunohistochemical studies were used to determine the distribution of fetal fibronectin in the placenta and amniochorionic membranes and to ascertain its cell of origin. RESULTS: Women with uncomplicated pregnancies (n = 163) who delivered at term rarely had cervicovaginal fetal-fibronectin concentrations above 0.05 micrograms per milliliter between 21 and 37 weeks of gestation (11 of 267 cervical samples [4 percent] and 9 of 267 vaginal samples [3 percent]. High levels of fetal fibronectin were detected in amniotic fluid and in the cervical or vaginal secretions of 93.8 percent of the women with preterm rupture of membranes (n = 65). Cervical or vaginal fetal fibronectin was also present in 50.4 percent of the women with preterm uterine contractions and intact membranes (n = 117), and its presence identified the women who delivered before term (n = 60) with a sensitivity of 81.7 percent and a specificity of 82.5 percent. In the placenta and membranes, fetal fibronectin was found at points of contact with the uterine wall. CONCLUSIONS: The presence of cervicovaginal fetal fibronectin in the second and third trimesters of pregnancy identifies a subgroup of women who are at high risk for preterm delivery. This phenomenon may reflect the separation of the chorion from the decidual layer of the uterus, with the release of intact or degraded chorionic components of the extracellular matrix into the cervical and vaginal secretions.

Amniotic Fluid↗
PubMed1986

Interstitial deletion of the short arm of chromosome 3 (3p14).

Deletions of 3p usually involve the terminal portion (3p25). An interstitial deletion of a proximal 3p segment (3p14) was detected at amniocentesis. The clinical and cytogenetic characteristics of this case and of three previously published cases are reviewed. Cardiovascular and gastrointestinal malformations have not been reported before in association with this particular chromosome abnormality.

Adult↗
PubMed1986

Prenatal shunting of fetal ascites in nonimmune hydrops fetalis.

A case of severe nonimmune hydrops fetalis presenting at 24 weeks gestation is presented. Ultrasound, echocardiographic, and fetal ascitic fluid studies were nondiagnostic. Because of massive fetal ascites an indwelling peritoneal amniotic cavity shunt was placed. Despite functioning of the shunt, a fetal death in utero occurred.

Adult↗
PubMed1986

Identical twins concordant for Crohn's disease.

Identical twin adolescent girls developed Crohn's disease within 15 months of each other. Clinical symptoms, growth retardation, barium studies, disease course, and pathologic findings at the time of resection were remarkably similar. Seventeen pairs of twins concordant for Crohn's disease have now been reported, but only four discordant pairs. Such observations lend support to a considerable genetic influence on the development and course of Crohn's disease.

Adolescent↗
PubMed1981

Right aortic arch, isolated left common carotid and left subclavian arteries, and subclavian steal syndrome: a variant of polysplenia syndrome.

Congenital subclavian steal may result from various anomalies of the aortic arch. The most common predisposing type is the right aortic arch with an isolated left subclavian artery arising from a left ductus arteriosus. A rare variant includes isolation of the left common carotid as well as the left subclavian arteries. The case of an infant with polysplenia--F-anisosplenia syndrome and this rare variant of congenital left subclavian steal is presented. This anomalous constellation has been heretofore unreported and illustrates a new situs abnormality that may complicate the polysplenia syndrome.

Aorta, Thoracic↗
PubMed1980

Dystrophic degeneration of papillary muscle and ventricular myocardium. A basis for mitral valve prolapse in Duchenne's muscular dystrophy.

The hearts of three children who died with Duchenne's progressive muscular dystrophy and features of mitral valve prolapse were examined to find if the valve disorder arose from cardiomyopathy due to the primary disease or from dystrophic changes in th mitral valve itself. Gross, histologic and ultrastructural features of mitral valve annulus, anterior and posterior leaflets, chordae tendineae, right and left ventricles, and anterior and posterior papillary muscles were compared with those of similar tissues from normal children of matched age and sex. Fibrosis and myofibrillar lysis--most extensive in posterior papillary muscle and in the posterobasal segment of the left ventricle--were the main histopathologic findings. Myofibrillar lysis was characterized by a total loss of actin and myosin myofilaments. By contrast, the mitral valve annulus, its leaflets and the origin, distribution pattern, length and thickness of chordae tendineae were entirely normal. These observations establish that mitral valve prolapse syndrome in Duchenne's dystrophy is an expression of cardiomyopathy involving papillary muscle and ventricular myocardium rather than a result of dystrophic changes in the mitral valve leaflets, annulus or chordae tendineae.

Adolescent↗
PubMed1980

Results of repair of coarctation of the aorta during infancy.

One hundred ninety-one infants under 1 year of age underwent repair of coarctation of the aortz during a recent 14 year period. All operations were performed on an urgent, nonelective basis. One third of the infants had isolated coarctation (Group I), one third had associated ventricular septal defect (VSD) (Group II), and one third had other complex anomalies (Group III). The operative mortality rate has decreased to 4% in infants with isolated coarctation of the aorta but remains at 25% for repair of coarctation in association with significant intracardiac disease (Groups II and III). The late mortality rate also was affected by intracardiac defects. No late deaths occurred in Group I patients, whereas the 5 year mortality rate was 25% in Groups II and III. Surgical repair was by resection and end-to-end anastomosis in most (92%) cases. Recurrent coarctation, as defined by arm/leg blood pressure gradient at rest, developed in 54% of the survivors within 7 years following operation. Recoarctation did not affect late mortality rate. Twelve children (11.1% of survivors) underwent repair of recoarctation during this interval. Hypertension developed in 27% of the children followed more than 5 years after repair and was always associated with residual arm/leg blood pressure gradient. There are no cases of idiopathic hypertension in this series.

Aorta, Thoracic↗
PubMed1979

Teratoma in horseshoe kidneys.

Renal tumors are uncommon, but they are significantly increased in horseshoe kidneys. Although nephroblastomas frequently occur in horseshoe kidneys of children, no teratoma has been observed in the past. A teratoma in the horseshoe kidney of a child with unusual congenital anomalies is described.

Abnormalities, Multiple↗
PubMed1979

Genital mycoplasma infection. Intrauterine infection: pathologic study of the fetus and placenta.

Mycoplasma infection was present in the fetuses from three spontaneous abortions and in one second-trimester newborn. Gross examination revealed in most cases a severely infected placenta and membranes, with a fetus of normal appearance. The fetal infection presumably followed placental involvement and appeared to have been acquired shortly prior to delivery. Genital mycoplasmas, Ureaplasma urealyticum or Mycoplasma hominis, were isolated from the placentas and the fetal tissues, and from the genital tracts of the mothers. Isolation of mycoplasmas from the liver indicated that bloodstream dissemination of these organisms occurred in the fetus. In the fetus, the pathologic changes were variable. Lesions were identified in the lung by scanning electron microscopy of the bronchial tree in two cases and were accompanied by interstitial pneumonia. An abnormally dilated left ventricle suggestive of cardiomyopathy was observed in one case.

Adult↗
PubMed1978

The tricuspid valve in pulmonary atresia and intact ventricular septum: a morphological study of 60 cases.

A morphologic study of the tricuspid valve was performed in 70 patients with pulmonary atresia and intact ventricular septum who had died and subsequently undergone autopsy. The right ventricular cavity was enlarged in 8 patients, normal in 4 patients, mildly underdeveloped in 18 patients, and markedly underdeveloped in 24 patients. In the patients with dilated and enlarged right ventricle, the tricuspid valve exhibited features of dysplasia or Ebstein's malformation (downward displacement), or both, with a dilated anulus. The majority of patients had restriction of the tricuspid valve apparatus and an underdeveloped anulus, usually of a severe degree. The right ventricle appeared to have normal dimensions in four patients, but the tricuspid valve and tensor apparatus, even in these patients, exhibited departures from the normal. This study suggests that in many of these patients adequate right ventricular growth will not be achieved because of inadequate right ventricular filling. This seems directly related to moderate or severe underdevelopment of the tricuspid valve.

Child, Preschool↗