Danazol and urogenital sinus formation in pregnancy.
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Adult↗
Biomedical subjects
M A Greco
Publications and source records attributed to M A Greco.
Hermansky-Pudlak syndrome: a clinicopathologic study.
The Hermansky-Pudlak syndrome is a rare disease characterized by multisystemic involvement. Seven families with the disorder were identified in the Puerto Rican population of one municipal hospital, suggesting that the incidence in the Puerto Rican community is sufficient to warrant both dissemination of information about the disease and further investigation. The present study was an attempt to achieve both of these goals.
Adolescent↗
Nosocomial fungal infection during hospital renovation.
Nosocomial fungal pulmonary infections (Zygomycetes, Aspergillus sp.) developed in two premature infants in a special care unit (SCU) adjacent to an area of renovation. Inspection showed that inadequate barriers permitted the passage of airborne particles between the two areas, and cultures confirmed a significantly higher (p less than or equal to 0.05) density of mold spores in the SCU (0.88 cfu per hour per settling plate) compared to a construction-free comparison area (0.22 cfu per hour per settling plate). The major source of mold was the dust above the hospital's false ceiling. In another construction area, imperious barriers were shown to effectively restrict the dispersal of mold. Our experience adds Rhizopus to Aspergillus as a possible cause of construction-related nosocomial infection. Sporadic episodes will continue to occur until the hazards of renovation are appreciated and effective preventive measures are routinely instituted.
Air Microbiology↗
Lymphangioma presenting as a nasal polyp in an infant.
We describe a pedunculated lymphangioma presenting as an isolated nasal polyp in an 11-month-old male infant with light and electron microscopic findings. This lesion occurring in an infant must be distinguished from more common benign and malignant intranasal masses.
Diagnosis, Differential↗
Pathologic findings in adenosine deaminase-deficient severe combined immunodeficiency. I. Kidney, adrenal, and chondro-osseous tissue alterations.
The authors have reviewed the autopsies of 8 patients with adenosine-deaminase-deficient severe combined immunodeficiency disease (ADA-SCID). Several new findings in nonlymphoid organs, including kidney and adrenal gland, and chondro-osseous tissue indicate the multisystem nature of this disorder. Examination of renal tissue in 7 of 8 cases showed mesangial sclerosis. This was confirmed in 3 cases by electron microscopy. One case, treated with multiple erythrocyte partial exchange transfusions for several years, had no mesangial sclerosis. Six of 8 cases showed adrenal-gland cortical sclerosis. Chondro-osseous tissue from vertebrae and costochondral junctions of 4 cases examined showed typical alterations previously reported in ADA-SCID such as short growth plates with few proliferating and some hypertrophic chondrocytes. The authors report the new observations of necrotic chondrocytes, as well as large amounts of cellular debris. These changes were not observed in the 2 other patients examined, who received bone marrow or multiple partial exchange transfusions. The distribution and severity of these lesions, their relationship to ADA replacement therapy, and their homology to mice treated with a potent ADA inhibitor suggests that, in addition to lymphoid dysfunction, disordered nucleoside metabolism due to absent ADA activity in ADA-SCID may be the cause of diverse multi-system pathologic changes in tissues which continue to differentiate or mature after birth.
Adenosine Deaminase↗
Congenital granular cell epulis. Immunohistochemical and ultrastructural observations.
Tumor cells of the congenital granular cell epulis (GCE), unlike those of the granular cell tumor (GCT), did not stain with antiserum to S-100 protein. Based on immunohistochemical findings it is concluded that the GCE and GCT differ in histogenesis; furthermore, ultrastructural studies of the authors are in agreement with others in supporting a mesenchymal origin of the GCE.
Diagnosis, Differential↗
Dyssegmental dwarfism: a histologic study of osseous and nonosseous cartilage.
Dyssegmental dwarfism is probably an autosomal recessive, lethal, generalized chondrodysplasia; it is characterized by anisospondyly , shortening of long bones, and narrow chest. Maturation of chondrocytes at the epiphyseal plates is disturbed. The pathologic features of osseous and nonosseous cartilage in a stillborn female infant with dyssegmental dwarfism are described. The cartilage matrix of tubular bones, vertebrae, ilia , ribs, nose, larynx, and trachea showed acellular areas with accumulation of acid mucopolysaccharides and no increase in collagen fibers. The findings, although not pathognomonic, suggest that this type of dwarfism may be a generalized connective tissue disorder. The abnormal synthesis, structure, or secretion of the components of the cartilaginous matrix may lead to accumulation of acellular material in both skeletal and respiratory tract cartilage.
Adult↗
Fibrous hamartoma of infancy: an ultrastructural study.
The fine structures of three fibrous hamartomas of infancy were studied. All three components of these lesions were examined. The principal cells in the fibrous and myxoid areas were fibroblasts. Cells with stellate cytoplasmic projections were more prominent in myxoid areas. Myofibroblasts were abundant in two cases and not in the other. The adipose tissue component was formed by lipocytes and occasional preadipose fibroblasts. Blood vessels were more numerous in myxoid areas. Larger vessels were accompanied by smaller vessels and by clusters of cells with primitive junctions, suggesting early blood vessel formation. Some small vessels were surrounded by concentric layers of mesenchymal cells, as if attempting to form media. Electron microscopy seems to confirm the hamartomatous nature of fibrous hamartomas of infancy. The lesion appears to recapitulate the formation of blood vessels and fat, as seen in fetal tissues. Cellular myxoid areas showed prominent vasoformative proliferation, which decreased in the adipose tissue and was least prominent in the dense fibrous component. The latter may represent the end stage of the fibrous proliferation.
Adipose Tissue↗
Testicular stromal tumor with myofilaments: ultrastructural comparison with normal gonadal stroma.
The ultrastructural features of two testicular stromal tumors were compared with those of normal gonadal stroma. The two patients with tumor were 28 and 48 years old and had no endocrine abnormalities. No metastases or recurrences occurred after 32 and 12 months of follow-up, respectively. The tumors were composed of bundles of oval to spindle-shaped cells. Ultrastructurally, intracytoplasmic myofilaments were characteristic of the tumor cells, which resembled the contractile peritubular and interfollicular cells of normal testis and ovary. In normal testicular tissue, an intertubular mesenchymal cell may differentiate into a peritubular contractile cell or into an interstitial (Leydig) cell. Therefore, testicular stromal tumors with myofilaments may originate from an intertubular mesenchymal cell that is capable of differentiating into a cell with contractile elements.
Adult↗
Virus-associated hemophagocytic syndrome due to Epstein-Barr virus.
The clinical and autopsy findings in a case of virus-associated hemophagocytic syndrome in a previously healthy 17-year-old girl are presented. Serum titers to Epstein-Barr viral antigens were unusually elevated. In addition to widespread proliferation of hemophagocytic histiocytes and lymphoid depletion, there were areas of non-suppurative necrosis in lymph nodes and spleen, which have not been previously reported.
Adolescent↗
Congenital cystic adenomatoid malformation of the lung in adults.
Congenital cystic adenomatoid malformation of the lung presents mainly in neonates, is rare in children beyond infancy, and has not been reported in adults. Two adult males (aged 24 and 35) had congenital cystic adenomatoid malformation of the right and left lower lobes respectively. A third case, that of a 7-year-old girl, provided the link between the typical neonatal and these adult cases. All three lesions consisted of single or multiple macroscopic cysts, as well as a network of interconnecting spaces (the adenomatoid component). The lining varied from pseudostratified columnar ciliated, to simple mucinous and cuboidal epithelium. Abundant smooth muscle was present in two cases. Cartilage was absent in all three cases. The absence of inflammation is typical of the lesion in neonates. By contrast, all three of our patients had clinical and pathologic evidence of chronic inflammation. We postulate that congenital cystic adenomatoid malformation, when confined to a lobe or segment, may be clinically silent in infancy and may present as pneumonia associated with a cystic lesion on chest x-ray in childhood or later life.
Adult↗
Deletion of the short arm of chromosome 3: a case report with necropsy findings.
A male infant with partial deletion of the short arm of chromosome 3 is described. The features this patient shares with six previously reported cases include microcephaly, dolichocephaly, micrognathia, epicanthic folds, ptosis, low set or malformed ears, postaxial polydactyly, and growth or mental retardation or both. In addition, visceral anomalies not previously reported in association with this chromosomal abnormality are described. These characteristics may constitute a recognisable clinical syndrome.
Abnormalities, Multiple↗
Late presentation of congenital cystic adenomatoid malformation of the lung.
Although most often recognized in neonates and young children, congenital cystic adenomatoid malformation of the lung (CCAM) occasionally appears in later years. Three patients, aged 35, 24, and 7 years, are reported. Chest radiographs in each case suggested a localized patchy density, a cystic mass, or a multicystic mass, but computed tomography (CT) best demonstrated the cystic and solid components while ruling out bronchiectasis or major bronchial obstruction. Bronchography contributed no further diagnostic information compared with CT. Each patient underwent lobectomy. Histologically, the characteristic overgrowth of bronchiolar elements replacing normal parenchymal architecture was accompanied by some superimposed inflammatory change. Each patient had a history of pneumonia, and in such patients, characteristic radiographic features should suggest the possibility of late presentation of CCAM.
Adult↗
Cerebellocele and associated central nervous system anomalies in the Meckel syndrome.
Three cases with the Meckel syndrome were autopsied and found to have: arhinencephaly , polymicrogyria , aqueductal stenosis, heterotopia of glial tissue, hypoplasia or agenesis of the cerebellar vermis, cranium bifidum associated with large occipital ventriculocele and others. The anomalies at the level of posterior fossa in this condition are classified as those belonging to the Chiari type III group of anomalies. This unusual set of anomalies which forms pathogenetic link between the Dandy-Walker and Chiari-Arnold group of anomalies in the posterior fossa seems to be very frequent in the Meckel syndrome. The therapeutic emphasis is on genetic counseling in view of the recessive inheritance of the syndrome.
Abnormalities, Multiple↗
Neural defects in Say-Gerald (VATER) syndrome.
The Say-Gerald (VATER) syndrome consists of vertebral defects, anal atresia, tracheoesophageal fistula, radial dysplasia and renal defects. 2 children with Say-Gerald (VATER) syndrome were autopsied: the first child was found to have hydrocephalus, aqueductal stenosis and probable craniosynostosis, and the second child had hypoplasia of the nerve roots and anterior and posterior horns of the spinal cord, corresponding to the hypoplastic limb. It is suggested that the Say-Gerald syndrome is a multifocal developmental disorder in which central nervous system anomalies may be found. The children with Say-Gerald syndrome should have a complete neurological examination in order to rule out a potentially treatable central nervous system defect.
Abnormalities, Multiple↗
Intracranial lipomas, hydrocephalus and other CNS anomalies in oculoauriculo-vertebral dysplasia (Goldenhar-Gorlin syndrome).
13 cases of Goldenhar-Gorlin syndrome are presented in which numerous central nervous system anomalies have been found. These include occipital encephalocele, hydrocephalus, aqueductal stenosis, agenesis of corpus callosum, multiple congenital lipomas and many others. Pertinent literature has been reviewed. It is concluded that any part of the central nervous system can be involved in this condition and that careful evaluation is indicated in order to rule out a treatable intracranial anomaly.
Adolescent↗
The pleomorphic cells of advanced mycosis fungoides. An ultrastructural study.
Five patients with advanced disseminated mycosis fungoides were studied, using light and electron microscopy. All had lesions with large pleomorphic cells by light microscopy. In three of the patients, nonlymphoid tissue was studied, including pericardium, epididymis, and small intestine. All areas selected for ultrastructural study showed large pleomorphic cells. We conclude that these represent poorly differentiated lymphoid cells derived from Sézary lymphocytes. This is based on nuclear and organelle similarities between the Sézary and pleomorphic cells, as well as the demonstration of intermediate forms.
Cell Nucleus↗
Encephalocele (cerebellocele) in the Goldenhar-Gorlin syndrome.
A patient with unilateral preauricular appendages and occipital mass is described who died on the fifth day of life. An autopsy demonstrated congenital megabladder and megaureters and dysplastic kidneys. The neuropathological examination revealed cranium bifidum in the occipital region, defective posterior arch of the C1 vertebral body, agenesis of vermis and a large cerebellocele. It is suggested that some patients with the Goldenhar-Gorlin syndrome may have prominent central nervous system involvement in spite of only relatively slight facial involvement.
Cerebellar Diseases↗