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Biomedical subjects

M M Silver

Publications and source records attributed to M M Silver.

At least 19 recordsLinked to original sources

Gastric-outlet obstruction induced by prostaglandin therapy in neonates.

BACKGROUND: An infusion of prostaglandin E1 is widely used to maintain patency of the ductus arteriosus in neonates with congenital heart disease. After gastric-outlet obstruction was recognized in several infants who received prostaglandin E1, we studied the association between the drug and this complication. METHODS: We evaluated all neonates who received prostaglandin E1 in our hospital between October 1, 1989, and September 30, 1991, for clinical, radiologic, or pathological evidence of acute gastric-outlet obstruction. RESULTS: Of the 74 neonates evaluated, 65 had no signs of gastric obstruction and were considered normal; 5 had clinical and radiologic or pathological evidence of gastric obstruction consistent with the presence of antral mucosal hyperplasia. The remaining four neonates had clinical signs of gastric obstruction, but no radiologic or pathological examinations were performed. The 5 neonates with antral hyperplasia had received prostaglandin E1 for longer periods (mean [+/- SD] duration, 569 +/- 341 hours) than the 65 normal neonates (54 +/- 58 hours, P less than 0.001) or the 4 neonates with clinical signs of gastric obstruction (119 +/- 60 hours, P less than 0.05). The cumulative dose of prostaglandin E1 was higher in the neonates with antral hyperplasia (2982 +/- 1392 micrograms per kilogram of body weight) than in the normal neonates (279 +/- 270 micrograms per kilogram, P less than 0.001) or the neonates with signs of gastric obstruction (528 +/- 306 micrograms per kilogram, P less than 0.01). In two neonates with antral hyperplasia, the cessation of therapy lessened the gastric-outlet obstruction. CONCLUSIONS: The administration of prostaglandin E1 to neonates can cause gastric-outlet obstruction due to antral hyperplasia. Neonates who receive prostaglandin E1 at recommended doses for more than 120 hours should be closely monitored for evidence of antral hyperplasia.

Alprostadil

The balloon posterior tracheal split: a technique for managing tracheal stenosis in the premature infant.

Congenital tracheal stenosis (CTS) in the premature infant almost invariably leads to death for lack of effective reconstructive techniques. We hypothesized that the complete or near complete cartilaginous rings of CTS have a weak point in the posterior portion. Aggressive balloon dilation would result in a posterior longitudinal disruption of the trachea, and an expanded tracheal wall would be created after an appropriate period of endotracheal stenting. Three premature infants with multiple anomalies and CTS were treated with endoscopic posterior tracheal splitting, dividing the trachea as predicted. Although two died of complications of congenital heart disease 2 weeks after the procedure, the third child is alive and well at 9 months of age without airway symptoms. The posterior tracheal wall also split when we performed the procedure in a fresh cadaveric CTS patient. We believe that balloon dilation may prove to be a valuable technique in the treatment of premature infants with congenital or acquired tracheal stenosis and/or in children with such complex diseases that major surgery is precluded.

Cadaver

Diaphragmatic contraction band necrosis in a perinatal and infantile autopsy population.

A morphologic study of the anterolateral costal diaphragm in 125 newborns and infants who died suddenly showed that contraction band necrosis is a common finding. In cases that showed the most extensive lesions, acute asphyxia was the usual mode of death; within eight diagnostic categories birth asphyxia (11 of 26 cases) and sudden infant death syndrome (19 of 30 cases) had the highest frequency of lesions. It was more frequent than myocardial contraction band necrosis and myocardial coagulation necrosis among the cases studied. The morphologic age and, if present, the stage of healing in each case suggested that the diaphragmatic lesion commenced at or shortly before death or at the time of the cardiac arrest that led to death. Thus, the lesion appeared to represent a very early event after a lethal injury, but it had no specificity for the nature of the injury. Because skeletal muscle of the respiratory diaphragm structurally and functionally resembles myocardium, the pathogenesis of contraction band necrosis may be similar in the two muscle types.

Autopsy

Unusual cardiovascular localization of nonamyloid immunoglobulin deposition disease causing sudden death.

The case of a 38-yr-old man is presented in whom sudden death was attributed to coronary ostial stenosis caused by aortic intimal deposits of an amyloid-like material. Autopsy revealed wide-spread lymphocytic lymphoma with kappa light chain restriction. Histochemistry, immunoperoxidase light microscopy, transmission electron microscopy, and immunoelectron microscopy identified the coronary ostial, and massive other cardiovascular, deposits as para-amyloid of kappa light chain origin.

Adult

T-cell lymphoma and mature nephroblastoma after synchronous bilateral Wilms' tumor.

A 15-year-old boy had lymphoblastic lymphoma of the left tonsil after being treated for bilateral Wilms' tumor (BWT) at 7 months of age. In addition, a fully differentiated Wilms' tumor was diagnosed in the remaining, partially nephrectomized left kidney. Development of second malignancies in patients with a history of BWT, as compared with those with unilateral Wilms' tumor, is discussed. A possible explanation for the concurrently diagnosed, fully differentiated Wilms' tumor in the remaining left kidney is suggested.

Humans

Primary pulmonary hypertension in a patient with systemic-onset juvenile arthritis.

We describe a 16-year-old girl with systemic-onset juvenile arthritis who presented with pulmonary hypertension, without evidence of pleural or parenchymal involvement of the lung, pulmonary vasculitis, or immune deposition in the pulmonary vasculature. Pleuropulmonary involvement occurs occasionally in juvenile arthritis, but primary pulmonary hypertension has not, to our knowledge, been previously reported. Histocompatibility typing showed positivity for HLA-DR3 and DRw52, both of which are associated with idiopathic pulmonary hypertension in children, and with pulmonary hypertension among patients with systemic sclerosis. Treatment with cyclosporine and corticosteroids resulted in a marked improvement in the clinical findings and pulmonary function in our patient.

Adolescent

Diffuse infantile haemangiomatosis: clinicopathological features and management problems in five fatal cases.

The clinicopathological features of five fatal cases of diffuse haemangiomatosis presenting in neonatal life or early infancy are presented. The infants all had multiple skin haemangiomas as well as deep-seated lesions in many different tissues that caused protean clinical manifestations and management problems. Because the outlook may be improved by early diagnosis and application of new modes of treatment, any infant with multiple cutaneous haemangiomas should be closely assessed for possible visceral involvement. Development of hepatomegaly, high-output cardiac failure, unexplained anaemia or thrombocytopenia in these infants should immediately suggest disseminated disease. Early recognition with implementation of steroid and/or antiangiogenic therapy, embolization and/or surgery is essential to improve the chances of survival.

Female

Fibrolamellar carcinoma of the liver in a patient with Fanconi anemia.

A 9-year-old boy with Fanconi anemia treated with oxymethalone, a synthetic androgen, died of intracerebral hemorrhage. At autopsy, the liver contained several adenomas and a large fibrolamellar hepatocellular carcinoma, as well as phlebectatic peliosis hepatis. The 11 previously reported cases of hepatocellular carcinoma in Fanconi anemia were not, apparently, of the fibrolamellar type, which has a better prognosis, occurs in children of both sexes, and generally is not associated with cirrhosis. The malignant potential of primary liver tumors associated with Fanconi anemia as well as the nature of their relationship to Fanconi anemia and to anabolic steroid therapy is discussed.

Carcinoma, Hepatocellular

Fatal pulmonary arterial occlusive vascular disease following chemotherapy in a 9-month-old infant.

Fatal pulmonary hypertension developed in an infant during the 7-month period in which he received, via a central venous catheter, combination chemotherapy for stage IV neuroblastoma as well as intermittent parenteral feeding. In a lung biopsy and at autopsy, small pulmonary arteries showed diffuse medial hypertrophy and peripheral muscularization, very extensive concentric intimal fibrosis, and focal eccentric fibrosis evolving from organizing thrombi. Pulmonary veins were normal. Hypothetically, chemotherapeutic drug therapy (possibly potentiated either by the parenteral nutrition or simply by the vehicular fluids causing volume loading of the pulmonary circulation) could cause occlusive pulmonary arterial disease by several mechanisms, but the association has not been described previously, although use of such drugs has been reported with pulmonary veno-occlusive disease.

Antineoplastic Combined Chemotherapy Protocols

Marshall-Smith syndrome: new radiographic, clinical, and pathologic observations.

Radiographic, clinical, and histologic findings in two infants and a neonate with a syndrome of profoundly accelerated skeletal maturation included features closely resembling those of Marshall-Smith (MS) syndrome, but patients had dysmorphic ears as well as distinctive generalized skeletal abnormalities suggestive of a bone dysplasia. Among these was an instability at the craniocervical junction with severe spinal stenosis. These previously unrecognized abnormalities may represent uncommon manifestations within the spectrum of MS syndrome or indicate the existence of a separate disorder.

Atlanto-Axial Joint

Chronic hypoxic pulmonary hypertension in rats and increased elastolytic activity.

Previously in rats injected with the toxin monocrotaline and administered SC-39026, a serine elastase inhibitor, pulmonary hypertension was decreased in association with reduced muscularization of peripheral pulmonary arteries. To determine whether inhibition of elastolytic activity might prevent this vascular change in other conditions producing pulmonary hypertension, we administered SC-39026 to rats during a 10-day exposure to chronic hypobaric hypoxia. We also measured elastolytic activity in the central pulmonary arteries of rats using [3H]elastin substrate and determined whether there was an increase in activity either as early as 2 days or at completion of the hypoxic exposure, which could be inhibited by SC-39026. to further determine whether the mechanism of muscularization of peripheral arteries is modulated by degradation of elastin or other elastase-susceptible extracellular matrix proteins, we assessed desmosine excretion and ultrastructural alterations in elastin as well as in type IV collagen, fibronectin, and laminin. SC-39026 reduced the number of muscularized arteries and the level of pulmonary arterial pressure during exposure to chronic hypoxia. Elastolytic activity was fourfold higher in central pulmonary arteries 2 days after hypoxia when compared with values in control vessels, and the activity was inhibited by SC-39026. In small peripheral pulmonary arteries there were no significant changes with hypoxia reflected in desmosines or in the immunocytochemistry of elastase-susceptible glycoproteins, with the exception of decreased laminin. This feature was not inhibited by SC-39026. To further assess whether the protective effect of SC-39026 was related to its inhibition of elastase, an extended study was carried out using a different elastase inhibitor, alpha 1-proteinase inhibitor. An even greater reduction in hypoxia-induced pulmonary hypertension and vascular changes was observed with this elastase inhibitor and the latter included medial hypertrophy.

Animals

Comparison of Novak and Pipelle endometrial biopsy instruments.

A randomized study compared the Novak and Pipelle endometrial biopsy instruments with respect to quality of the biopsy obtained and pain related to the procedure. Fifty-five subjects were randomized to one of two groups. Twenty-six women had a biopsy using the Pipelle, followed immediately by one using the Novak instrument; 29 had the procedures performed in the reverse sequence. After the procedures, each woman completed a pain questionnaire. Individual histology slides were reviewed in a blinded fashion. The two biopsies from each subject were paired, and a reviewer indicated the preferred type of biopsy. The scores were analyzed by nonparametric tests. The instruments yielded biopsies of similar quality (z = -0.18, P = .856). Pain scores were lower for the Pipelle (z = -3.40, P = .001). The pathologist showed no preference when choosing Novak or Pipelle slides (chi 2 = 2.08, P = .149). In our patient population, the Pipelle instrument was comparable to the Novak instrument in obtaining adequate tissue and was significantly less painful.

Adult