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Biomedical subjects

A Perez-Atayde

Publications and source records attributed to A Perez-Atayde.

16 recordsLinked to original sources
PubMed1992

Pneumatocele complicating hyperimmunoglobulin E syndrome (Job's Syndrome).

The case of a boy with hyperimmunoglobulin E syndrome or Job's syndrome is presented to demonstrate the occurrence of pneumatoceles in this syndrome as well as their unusual natural history and failure to spontaneously resolve. Surgical resection was required for two complications in this patient, persistent bronchopleural fistula and a pulmonary abscess that destroyed one lung and required pneumonectomy. Pathologic examination of the specimens demonstrated the wall of the cysts consisted of granulation tissue with chronic active inflammation surrounded by infarcted pulmonary parenchyma with coagulative necrosis. The mechanism responsible for increased immunoglobulin E production in this syndrome is unknown, as is the manner in which elevated immunoglobulin E levels impair normal immune function.

Aspergillosis↗
PubMed1991

Human intestine matures as nude mouse xenograft.

This report describes a novel system for the study of the development and function of human intestine. Human fetal bowel transplanted into a subcutaneous tunnel on the back of athymic nude (nu/nu) mice develops a new microcirculation within 4 weeks. Tissues undergo morphological development, become similar to adult human bowel tissue, and may survive for 6 months after transplantation. Monoclonal antibody immunoperoxidase staining shows that the epithelial and some endothelial elements are of human phenotype, but the circulating blood cells and migrating mucosal lymphocytes are of mouse origin.

Animals↗
PubMed1991

Ovarian granulosa-stromal cell tumors are characterized by trisomy 12.

Eleven ovarian granulosa-stromal cell tumors including 1 thecoma, 2 fibromas, 6 fibrothecomas, and 2 granulosa cell tumors, were karyotyped after direct harvest and/or short-term tissue culture. Bilateral fibrothecomas from one patient appeared to lack cytogenetic aberrations: the remaining nine tumors were characterized by trisomy for chromosome 12. Cytogenetic aberrations in the two granulosa cell tumors were much less complex than those described previously in undifferentiated carcinomas; accordingly cytogenetic analyses might be useful in distinguishing these categories. The consistent occurrence of trisomy 12 in different varieties of granulosa-stromal cell tumors suggests a common mechanism of oncogenesis within this diverse group of neoplasms. That mechanism probably involves promotion of low-grade, orderly cell proliferation.

Chromosomes, Human, Pair 12↗
PubMed1990

Surgery only for the treatment of patients with stage I (Cassady) Wilms' tumor.

A prospective pilot study was undertaken to examine the outcome of patients with Stage I (Cassady) Wilms' tumor treated with nephrectomy only. Eight consecutive patients fulfilling the criteria for Stage I (Cassady) Wilms' tumor (age less than 2 years with unilateral, nonmetastatic, favorable histopathologic type, and tumor weight less than 550 g) underwent nephrectomy with no further therapy. All eight patients were alive and free of disease with a mean follow-up period of 5 years. There was one tumor recurrence that involved a metachronously occurring bilateral tumor. Overall survival (100%) and event-free survival (88%) were comparable with clinical trials in which patients received adjuvant therapy. Patients with Stage I (Cassady) Wilms' tumor can be successfully treated with nephrectomy alone, thereby eliminating the toxicity of adjuvant therapy.

Female↗
PubMed1990

A proposed cause for the hepatic dysfunction associated with parenteral nutrition.

Total parenteral nutrition (TPN) is associated with cholestasis and hepatic steatosis, which can be lethal in infants who cannot be fed orally. It was determined that route of administration was not the critical variable in the development of hepatic steatosis. Two groups of young rats received equivalent amounts of a standard TPN solution either orally or intravenously for an 8- to 10-day period during which they received no other nutrition. Both groups gained equivalent weight and developed marked hepatic steatosis. To test whether the solution was toxic or deficient, three groups of rats were given TPN solution orally and a fraction of their usual daily intake of rat chow. Rats receiving less than 10% of their usual chow intake developed steatosis; rats receiving more than that did not. To determine the solubility of the protective material in chow, two groups of rats were given TPN solution orally and chow that been extracted with either water or the organic solvent chloroform. Rats eating the water-extracted chow developed steatosis, rats eating chloroform-extracted chow did not. Although the protective component in chow was apparently water soluble, addition of a water soluble extract of chow to the TPN solution fed another two groups of rats did not prevent steatosis at 0.1 mg/mL and only partially, if at all, at 10 mg/mL. TPN-related hepatic dysfunction, as measured by the development of hepatic steatosis in this model, may be due to a deficiency in the TPN solution. The missing constituent(s) appears to be present in rat chow and can be extracted with water, but not with an organic solvent.

Animals↗
PubMed1990

Orthotopic heart transplantation for complex congenital heart disease.

Orthotopic heart transplantation has become standard therapy for end-stage cardiomyopathy in children and adults, but there has been much less experience with transplantation for complex congenital heart disease. In this report experience with orthotopic transplantation in seven children with various forms of complex congenital heart disease is reviewed. Diagnoses included hypoplastic left heart syndrome in two (after stage I palliation), left ventricular diverticulum in one, single ventricle in two (dextrocardia, atrial situs inversus, and total anomalous pulmonary venous return in one patient and post-Fontan repair in the second), D-transposition of the great arteries and ventricular septal defect (post-Senning repair and ventricular septal defect closure) in one, and Ebstein's anomaly with biventricular dysplasia in one. Six of the seven were hospital survivors and there has been one late death at 2 1/3 years. Modifications of the standard operative technique to fit the anatomic variations in these defects are reviewed.

Adolescent↗
PubMed1989

Cardiovascular manifestations of human immunodeficiency virus infection in infants and children.

Thirty-one pediatric patients with human immunodeficiency virus infection were prospectively evaluated using 2-dimensional and M-mode echocardiography, Doppler cardiography, electrocardiography and Holter monitoring. Left ventricular shape, wall motion and valve morphology were evaluated with 2-dimensional echocardiography. Valve function was assessed using Doppler cardiography. Left ventricular performance was evaluated with shortening fraction, afterload with end-systolic wall stress and contractility with the end-systolic wall stress and rate-corrected velocity of shortening relation. Although left ventricular performance, afterload and contractility varied widely, 2 patterns of left ventricular function abnormalities were noted. Hyperdynamic left ventricular performance, generally with enhanced contractility and reduced afterload, was the most common echocardiographic finding (63%). Diminished contractility was noted in 8 patients (26%), including 4 patients with symptomatic dilated cardiomyopathy. Serial echocardiographic evaluation revealed changes from the original level (elevated, normal or depressed) of left ventricular function, afterload or contractility in 89%. Pericardial effusion without tamponade was seen in 8 patients (26%). Mononuclear pericarditis, myocarditis and inflammation of the intracardiac conduction tissue as well as peripheral nerve were seen in autopsy specimens, yet histologic or culture evidence of myocardial infection with opportunistic organisms was lacking. High grade atrial (1 patient) and ventricular (3 patients) ectopy, as well as second-degree atrioventricular block, were observed. Cardiac abnormalities, detectable by noninvasive methods but often clinically inapparent, appear to be common in children with human immunodeficiency virus infection and may cause symptoms or even death.(ABSTRACT TRUNCATED AT 250 WORDS)

Acquired Immunodeficiency Syndrome↗
PubMed1989

Sonography of testicular microlithiasis.

The sonographic appearance of testicular microlithiasis detected in a patient presenting with torsion is described. A "speckled" pattern of multiple, tiny bright echoes is produced by calcific concretions in the seminiferous tubules and seems to be characteristic of microlithiasis. Although this condition is not treatable, it should be recognized because it is often associated with extratesticular abnormalities and can obscure superimposed testicular disease.

Adolescent↗
PubMed1989

Apparent association between thymic cysts and prior thoracotomy.

Three male subjects, aged 8 months to 18 years, who had previously undergone thoracic surgery, developed mediastinal thymic cysts. Two cysts, both multilocular, were shown by plain radiography and computed tomography, and one was found incidentally during cardiac surgery. These three cysts constitute 15% of the 20 thymic cysts removed surgically or found at autopsy at the authors' hospital in the past 27 years. These cases and three previously reported cases in male adolescents and adults suggest that some thymic cysts are related to trauma during surgery.

Adolescent↗
PubMed1988

Selective cell transplantation using bioabsorbable artificial polymers as matrices.

To date, selective cell transplantation has involved injecting cell suspensions into tissues or the vascular system. This study describes attaching cell preparations to bioerodable artificial polymers in cell culture and then implanting this polymer-cell scaffold into animals. Using standard techniques of cell harvest, single cells and clusters of fetal and adult rat and mouse hepatocytes, pancreatic islet cells, and small intestinal cells have been seeded onto biodegradable polymers of polyglactin 910, polyanhydrides, and polyorthoester. Sixty-five fetuses and 14 adult animals served as donors. One hundred fifteen polymer scaffolds were implanted into 70 recipient animals: 66 seeded with hepatocytes; 23 with intestinal cells and clusters; and 26 with pancreatic islet preparations. The cells remained viable in culture, and in the case of fetal intestine and fetal hepatocytes, appeared to proliferate while on the polymer. After four days in culture, the cell-polymer scaffolds were implanted into host animals, either in the omentum, the interscapular fat pad, or the mesentery. In three cases of fetal intestinal implantation coupled with partial hepatectomy, successful engraftment occurred in the omentum, one forming a visible 6.0 mm cyst. Three cases of hepatocyte implantation, one using adult cells and two using fetal cells, have also engrafted, showing viability of hepatocytes, mitotic figures, and vascularization of the cell mass. To date, no pancreatic islets have survived implantation. This method of cell transplantation, which we have termed "chimeric neomorphogenesis," is an alternative to current methods and requires further study.

Animals↗
PubMed1988

Disseminated intravascular coagulation in a case of fatal lindane poisoning.

After an accidental or intentional ingestion of lindane, clinical manifestations of poisoning may include rapid onset of nausea and vomiting, coma, seizures, respiratory failure, and death. While rhabdomyolysis, secondary renal failure, and aplastic anemia have also been reported, coagulopathies have not been observed following poisoning with this pesticide. In this case report we describe a 43-year-old female who intentionally ingested 8 oz of a 20% lindane solution. Her serum lindane concentration reached 1.3 mcg/ml and her clinical manifestations included seizures, coma, rhabdomyolysis, secondary renal failure, and disseminated intravascular coagulation. The coagulopathy presented early in her clinical course and resolved when serum lindane levels fell. The patient died 11 days after the ingestion.

Adult↗
PubMed1987

Weekly high-dose methotrexate and doxorubicin for osteosarcoma: the Dana-Farber Cancer Institute/the Children's Hospital--study III.

Weekly high-dose methotrexate with leucovorin rescue and vincristine (HDMTX) and doxorubicin was administered as adjuvant postoperative therapy to 46 patients with a diagnosis of conventional high-grade nonmetastatic osteosarcoma of an extremity between July 1976 and December 1981. The primary lesions were managed by wide or radical amputation (26 patients) or by limb-sparing resection in 20 selected patients. The margins of the resections were retrospectively classified as marginal in three, wide in 16, and radical in one. The 5-year relapse-free survival (RFS) for all patients is 59% (95% confidence interval [CI], 43%, 74%) and overall survival is 78% (95% CI, 65%, 91%). The RFS for patients initially having a limb resection procedure is 55% (95% CI, 32%, 77%) compared with 62% (95% CI, 43%, 81%) for those initially having amputations (P = .52). Using multivariate analysis, the only significant prognostic variables that predicted RFS of greater than or equal to 3 years, were the presence of moderate to marked lymphocytic infiltration of the primary tumor (P less than .002), primary site outside of the proximal humerus (P less than .005), and the absence of a predominance of osteoblastic pattern in the primary tumor (P less than .03).

Actuarial Analysis↗
PubMed1986

Central giant cell lesions of the jaws: a clinicopathologic study.

The biologic behavior of central giant cell lesions of the jaws ranges from quiescent to aggressive with destructive expansion. To date, these variations have not been explained by the findings of routine histologic examination. This retrospective clinicopathologic study of giant cell lesions was performed to search for histologic correlates of biologic behavior. Lesions in 17 patients were classified clinically as nonaggressive (group I) or aggressive (group II). In general, group II lesions affected children at an earlier age, were larger at the time of diagnosis, and recurred more frequently. The following histologic parameters were assessed: fractional surface area occupied by giant cells (FSA), relative size index of giant cells (RSI), stromal characteristics, mitotic index, inflammatory cells, and hemosiderin content. Histologic differences between the two groups were not as clear as the differences in biologic behavior. However, aggressive lesions had a higher RSI, and recurrent giant cells lesions had a higher RSI and FSA; these parameters warrant further study. In addition, electron microscopic differences in a small number of aggressive and nonaggressive lesions were documented.

Adolescent↗
PubMed1986

Pancreatic cystosis in cystic fibrosis.

Two patients with cystic fibrosis and pancreatic cystosis are described who had complete replacement of the pancreas by multiple macroscopic cysts. The possible causes and clinical significance of this condition are discussed.

Adolescent↗