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.

Isolated left ventricular ischemia after the Norwood procedure.

Aortic atresia is the most severe variant of hypoplastic left heart syndrome (HLHS), and has been associated with significant mortality after stage I palliation. Coronary artery abnormalities are more prominent in this group of patients, especially in the presence of a patent mitral valve. Herein, we describe a case of isolated left ventricular ischemia after the Norwood procedure in a neonate with hypoplastic left heart syndrome, left ventricular hypertrophy, mitral stenosis, aortic atresia, and anomalous left coronary artery.

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.

Juvenile granulosa cell tumor of the testis: a comparative immunohistochemical study with normal infantile gonads.

This study concerns the nature of two different cell populations in a juvenile granulosa cell tumor (GCT) of the infantile testis. Immunohistochemical features of the tumor were compared with those of normal infantile testes (six cases) and ovaries (six cases). The testicular neoplasm showed follicles, cysts and solid nodules composed of an internal layer of polyhedral cells that expressed cytokeratin and vimentin.

Heart transplantation for end-stage cardiomyopathy caused by an occult pheochromocytoma.

Dilated cardiomyopathy has been found in patients with pheochromocytoma. The cardiomyopathy and resultant congestive heart failure can be progressive and fatal, or reversible, if the source of the excess catecholamines is removed. Histologic and autopsy studies revealed a characteristic focal myocarditis and contraction band necrosis, indicating that the circulating catecholamine excess secreted by the pheochromocytoma is the cause of the cardiomyopathy.

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.

Giant cell vasculitis with extravascular granulomas in an adolescent.

We describe an 18-year-old white male who developed lower extremity ischemia requiring amputation. He presented at 14 with pulmonary infiltrates, hepatosplenomegaly, fever, rash, adenopathy, uveitis, and arthralgias; clinical and laboratory findings were consistent with Mycoplasma pneumoniae infection. Despite adequate treatment with antibiotics, he developed chronic arthralgias and fevers, with rash and pericardial effusion.

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.

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.

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.

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.

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.

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).

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.

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.

Mixed teratoid tumors of the liver and neck in trisomy 13.

A fetus with trisomy 13 syndrome and teratomas of liver and neck is described. The relationship of aneuploidy and chromosomal instability to neoplasia is discussed. Teratomas of the liver are reviewed.

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.

Conal anatomy in aortic atresia, ventricular septal defect, and normally developed left ventricle.

Although aortic valve atresia is usually associated with severe underdevelopment of the mitral apparatus and left ventricle in rare cases of aortic atresia, the left ventricle may be of normal size, or even enlarged. This occurrence seems related to the presence of a significant ventricular septal defect. We have presented the morphologic findings in seven patients with aortic atresia and normally developed left ventricle, (six necropsied patients, and one studied angiocardiographically).

Aneurysms of the coronary arteries in infants and children. A review, and report of six cases.

In recent years large numbers of the so-called "mucocutaneous lymph node syndrome" or "Kawasaki's disease" have been described by Japanese workers, but instances of this disorder are only now being reported as isolated cases by European or North American physicians. The disease has, therefore, been considered to be a new entity. One of its most striking features is the development of aneurysms of the coronary arteries in infants or children, which may lead to sudden death.