Peritonitis with Paecilomyces complicating peritoneal dialysis.
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Adolescent↗
Biomedical subjects
M M Silver
Publications and source records attributed to M M Silver.
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↗
The association of lingual thyroglossal duct remnants with sudden death in infancy.
Two cases of sudden infant death are described in which relatively large posterior lingual midline cysts were demonstrated at autopsy. Death in both patients was attributed to upper airway obstruction due to the cysts, both of which represented thyroglossal duct remnants.
Airway Obstruction↗
Acute heart failure in an 8-year-old diabetic girl.
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Aortic Arch Syndromes↗
Immunogold quantitation of immunoglobulin light chains in renal amyloidosis and kappa light chain nephropathy.
By quantitative immunoelectron microscopy using protein A-gold, the authors compared the content and distribution of immunoglobulin light chain (LC) antigens in glomeruli from 11 cases of renal amyloidosis with that in two cases of kappa LC glomerulopathy and two cases of diabetic glomerulosclerosis. In a supplementary study and using a similar immunogold technique, the authors identified amyloid A in deparaffinized renal tissue from three of the 11 cases of renal amyloidosis. Each patient had similar clinical manifestations (chronic renal failure with proteinuria) and similar glomerular morphology (thickened glomerular basement membranes and nodular expansion of the mesangium). In 12 cases (10 amyloid, 2 kappa LC), immunoelectron microscopy localized LC antigens over the glomerular deposits and allowed indirect tissue quantitation of each LC antigen to the various cellular and interstitial compartments. In 6 of the 11 cases of renal amyloidosis, the amyloid labeled only for lambda, and in one, only for kappa. In one patient with Waldenström's macroglobulinemia, who had a biclonal gammopathy, both LC were identified in the amyloid. In two cases, both of whom had a history of chronic suppurative lung disease, both LC antigens as well as amyloid A were localized to the amyloid fibrils. In only one case, in which glomerular amyloid labeled for amyloid A, the amyloid did not label for either LC. Whereas lambda LC-derived fibrils often appeared as spicules in the glomerular subepithelial space, other amyloid deposits usually accumulated in the subendothelial zone and did not form spicules. The epimembranous location of spicules suggested that the amyloid precursor protein transformed into amyloid fibrils after filtration into the urinary space. Presence of epimembranous spicules may explain the more severe proteinuric renal failure and the more rapid progression to glomerulosclerosis described in primary amyloidosis.
Amyloidosis↗
Liver cell dysplasia and early liver transplantation in hereditary tyrosinemia.
Two cases of hereditary tyrosinemia presented with ascites and coagulopathy in infancy. Both patients underwent liver transplantation at the age of 25 and 36 mo, respectively. Both cases had normal liver function 37 and 24 mo later. The native liver in each case showed mixed micro- and macronodular cirrhosis with hepatocellular dysplasia, including both the large and small cell varieties. One of the subjects had also shown dysplasia in a prior liver biopsy. We compared the hepatic morphology with that from two other cases from our autopsy files. One of these (a female, 9 mo old) showed dysplasia, and the other (her male sibling, 4 yr old) had a liver cell carcinoma with lung metastases. These observations confirm prior reports that neoplastic transformation occurs early in the natural history of hereditary tyrosinemia despite meticulous dietary management and other supportive treatment. With the detection of liver cell dysplasia, efforts should be intensified to find an appropriate donor. Liver transplantation cures the hepatic disease and should be performed before malignancy develops.
Amino Acid Metabolism, Inborn Errors↗
Case 6. Perinatal hemochromatosis.
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Female↗
Perinatal pulmonary hyperplasia due to laryngeal atresia.
A premature newborn boy, who died at birth because of cartilaginous laryngeal atresia, showed lung development that was far more advanced than normal for gestational age. The lungs, which were histologically normal, were three times the expected weight and showed a degree of alveolarization appropriate for 3 months' postnatal age. The lungs crowded the chest cavity so that the diaphragm was flattened and immobilized; the massive ascites, documented by ultrasound 5 weeks prior to delivery, appeared to be due to obstructed venous return. Thus, the pulmonary hyperplasia, a new finding in perinatal pathology, caused severe fetal ascites. Subsequently, the hyperplasia led to acute polyhydramnios that, ultrasonographically, was observed to develop 2 weeks prior to delivery. The observation that fetal lungs may be hyperplastic has a bearing on the known relationship between fetal lung growth and retention of lung fluid.
Ascites↗
Hemorrhagic endovasculitis-like lesion induced in placental organ culture.
In organ culture, human chorionic villi develop vascular changes that resemble so-called hemorrhagic endovasculitis. The latter is a morphologic finding more prevalent in placentas of stillborn infants but seen also in those of liveborn infants, in whom the lesion is localized rather than generalized. We compared the histologic vascular changes in short-term organ cultures of 15 placentas (10 term, 5 preterm) with the naturally occurring vascular lesion in 6 placentas (2 liveborn, 4 stillborn). All organ cultures of placentas from liveborn infants developed hemorrhagic endovasculitis-like lesions in the fetal stem arteries; these lesions were present as early as 1 day and persisted for 7 days in culture. A mechanism common to both the in vivo and in vitro systems depending on hypoxia and vascular smooth muscle contraction may explain both the naturally occurring and tissue culture-induced lesions.
Chorionic Villi↗
Calcitonin and chromogranin A localization in medullary carcinoma of the thyroid by immunoelectron microscopy.
We used a post-embedding immunoelectron microscopy method, using protein A-gold, to detect calcitonin and chromogranin A immunoreactivity in three cases of human medullary thyroid carcinoma. Because the epoxy-embedded tissue had been fixed (glutaraldehyde or formaldehyde) and osmicated before embedment, the proteins were identified in optimally preserved tissue. Uranyl and lead staining was used after immunolabeling, so that the tissue was also optimally contrasted. The morphological advantage provided by osmication was tested by labeling rat thyroid gland C-cells for calcitonin. The protein A-gold technique allowed localization of both antigens to the contents of membrane-bound secretory granules in the tumor cells. In one case, labeling density for each antigen was measured over several intercellular compartments and the interstitium. Calcitonin, but not chromogranin A, reactivity was also identified in intracellular amyloid fibrils in two cases, showing that the constant region of calcitonin is preserved in amyloid deposits related to the tumor cells.
Calcitonin↗
Pulmonary hypoplasia in neonatal hypophosphatasia.
Morphological, biophysical, and biochemical parameters of lung growth were studied at autopsy on a male infant with hypophosphatasia who died with asphyxia immediately after birth. The lungs were hypoplastic because of a marked decrease in airspace formation but lung maturation was normal for gestational age by all the parameters used. Diaphragmatic development, assessed by weight and fiber measurement, was in keeping with the decreased chest size. The proposed mechanism for this late onset type of pulmonary hypoplasia, attributed to decreased thoracic volume, is correlated with antenatal ultrasonographic observations of normal fetal breathing movements in the affected infant.
Asphyxia Neonatorum↗
Cyclopia and congenital cytomegalovirus infection.
We describe the first documented association of congenital cytomegalovirus (CMV) infection and cyclopia. A previous report has suggested that any infant with congenital ocular defects should be investigated for CMV infection [Frenkel et al, 1980]. Our case underlines this suggestion and questions a teratogenic role for CMV in cyclopiaholoprosencephaly. More documented cases may help clarify the relationship of transplacental CMV infection to the holoprosencephaly developmental field defect, including cyclopia.
Abnormalities, Multiple↗
The role of insulin resistance in the pathogenesis of myotonic muscular dystrophy.
A study of glucose, insulin, lipids and lipoproteins in myotonic dystrophy (MyD) has shown elevation of fasting plasma insulin, triglycerides and very low density lipoproteins (VLDL) but no significant difference from normal in the fasting plasma glucose, total cholesterol or low and high density lipoproteins. Elevation of the total triglyceride and VLDL levels showed a direct relationship to hyperinsulinaemia. Insulin binding to cultured MyD fibroblasts under optimal conditions was significantly reduced but there was no difference in receptor affinity between MyD and control cells. In contrast to insulin binding, LDL binding to MyD fibroblasts was normal although there was a tendency to reduced LDL binding at 37 degrees C that may reflect mildly reduced lipid metabolism. The alterations in lipids and insulin in MyD are compatible with insulin resistance. Laboratory and clinical findings in MyD were compared with other inherited insulin-resistant diseases. MyD showed marked similarity to a group of disorders that have mild insulin resistance and mildly elevated plasma insulin in contrast to others with severe hyperinsulinaemia and insulin resistance. It is suggested that at least some clinical features of MyD may be due to diminished overall effect of insulin or other trophic factors on cell metabolism.
Adolescent↗
Postembedding immunoelectron microscopy using protein A-gold.
Immunogold techniques offer the advantage of identifying antigens in tissues that show optimal ultrastructural morphology. The technique is applicable to any antigen that can be shown to withstand the denaturizing effects of fixation, osmication, dehydration, and embedding in epoxy resin. The list of antigens that survive these procedures is long and growing.
Antigens↗
Perinatal hemochromatosis. Clinical, morphologic, and quantitative iron studies.
Three sibling and two isolated-case perinates (4 newborn, 1 stillborn) died with siderotic cirrhosis and widespread parenchymal siderosis, the latter similar to that seen in both hereditary and secondary hemochromatosis. Reticuloendothelial siderosis was absent, as occurs in primary hemochromatosis. Studies of iron metabolism were performed antemortem in two of the siblings and ante-, post- and internatally in their mother, who showed hyperferremia antenatally. The only finding in the affected family suggestive of hereditary hemochromatosis was the commonly associated HLA haplotype (A3, B7) in the mother and an infant. Liver morphology, including immunocytochemistry and ultrastructure, was similar in the 5 infants and suggested that liver disease commenced as massive necrosis in midfetal life. Histologic grading and chemical assays for iron and copper on liver and spleen of the 5 index cases were compared with 26 controls; placentas were compared with 12 control placentas. Hepatic iron concentration, but not hepatic copper concentration, was significantly increased in index cases, compared with controls. Hepatic iron to copper ratio was significantly increased in index cases, compared with controls, but this ratio was unaltered in spleen and placenta. Total hepatic iron, but not total hepatic copper, was significantly increased in index cases, compared with a subgroup of 11 controls of low gestational age, similar to the fetal stage when liver disease commenced in utero. The results suggest that, irrespective of the fetal liver disease being genetic or acquired, hepatic iron overload was directly involved in pathogenesis.
Copper↗
Acute acalculous cholecystitis in a premature infant treated with parenteral nutrition.
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Acute Disease↗
Morphologic and morphometric analysis of muscle in X-linked myotubular myopathy.
The X-linked form of myotubular myopathy is highly lethal in neonates. Several autopsy-derived muscles from two probands of a new kindred who survived for 100 days because of intensive supportive care were analyzed by light microscopy, morphometry, enzyme histochemistry, and electron microscopy. The results were compared with a similar analysis of muscle from control fetal and neonatal subjects. The findings, in addition to the characteristic centronucleated hypotrophic myofibers, included widespread myofiber degeneration and focal contraction band necrosis that differed from the types seen in other myopathic and dystrophic muscle diseases. A high frequency of degenerating nuclei that often contained large nucleoli was observed. Because of the paradoxic nuclear morphology, nuclear failure (in migration and myofibrillogenesis) is believed to be of central importance in the pathogenesis of this disease.
Histocytochemistry↗