Biomedical subjects

A Perez-Atayde

Publications and source records attributed to A Perez-Atayde.

49 records · Page 3Linked to original sources

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↗

PubMedDate not supplied

Cardiac mitochondrial dysfunction in Leigh syndrome.

An infant with Leigh syndrome and associated cardiomyopathy is described. Abnormal activities of mitochondrial respiratory complexes III and V and a change in mtDNA at nt 8993 were detected in heart and skeletal muscle but not in liver.

Cardiomyopathies↗

PubMedDate not supplied

Is age a contributory factor of mitochondrial bioenergetic decline and DNA defects in idiopathic dilated cardiomyopathy?

While mitochondrial abnormalities are increasingly recognized in cardiac diseases including hypertrophic cardiomyopathy, their presence in idiopathic dilated cardiomyopathy and the role that age plays in their incidence and severity have yet not been assessed. Levels of cardiac respiratory enzyme activities and mitochondrial DNA (mtDNA) were examined in 55 subjects with idiopathic dilated cardiomyopathy divided into 3 age groups. Respiratory enzyme activity levels were significantly lower in 37 patients (67%) compared to age-matched controls and increased activity levels were noted in 9 (16%). Decreased activities were found in complex I (n = 11), III (n = 16), IV (n = 12) and V (n = 13), but not in II, the only respiratory complex entirely nuclear-encoded. No age-specific differences were found in the overall frequency of enzymatic abnormalities. However, older patients had significantly increased multiple enzyme activity defects as well as increases in abundance and frequency of the 7.4 kb deletion. In addition, 3 patients were noted with marked reduction in mtDNA levels. None of the pathogenic mtDNA mutations previously associated with hypertrophic cardiomyopathy were found, nor was there any relationship that could be established between levels of specific mtDNA deletions and enzyme activities. In summary, specific mitochondrial abnormalities are heterogenous and frequent in both adults and children with idiopathic dilated cardiomyopathy. Older patients are more likely to have mtDNA deletions and multiple enzyme activity defects. The molecular basis for these abnormalities remains undefined.

Adolescent↗

PubMedDate not supplied

Solitary nonparasitic cysts of the liver: the Boston Children's Hospital experience.

A 3 3/12-year-old female child with abdominal distention, new onset pain, and tenderness is described. The child underwent surgical resection of a multilocular cyst of the right lobe of the liver. We reviewed the autopsy and surgical files of Children's Hospital for the past 63 years to identify other congenital cysts of the liver. We found 30 additional cases including 4 multilocular cysts. The origin of these cysts, a review of the literature, and a discussion of diagnostic parameters for the identification of such cysts in context with other hepatobiliary cystic lesions are presented.

Child↗

PubMedDate not supplied

Late events in pediatric patients with Ewing sarcoma/primitive neuroectodermal tumor of bone: the Dana-Farber Cancer Institute/Children's Hospital experience.

The outcome for 82 pediatric patients with Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) of bone is reported; the patients were treated at the Dana-Farber Cancer Institute (DFCI) and Children's Hospital (CH) in Boston, MA (USA) from 1971-1988. The charts of all patients with ES/PNET of bone treated during this period were reviewed for disease status, therapy, sites of relapse, information on second malignancies, and survival status. Eighty-two patients with ES/PNET of bone treated at DFCI/CH were identified. The 10-year event-free survival (EFS) rates were 12% (95% confidence interval [CI] 0, 27%) and 38% (95% CI 26, 51%) for patients with and without metastases, respectively (P = 0.002); the overall survival (OS) rates were 17% (95% CI 1, 33%) and 48% (95% CI 35, 61%) for patients with and without metastases (P = 0.001). Median follow-up for surviving patients is 10.2 years. Primary site in the pelvis also was associated with a poor outcome for patients with no metastatic disease (P = 0.006 OS, P = 0.03 EFS). Thirty-one patients survived in first remission at least 5 years from diagnosis, and of these, five experienced relapse of original disease, and five experienced secondary malignancies. Pediatric patients treated for ES/PNET of bone remain at risk for life-threatening events into the second decade of follow-up. After 5 years, the risk of second malignant neoplasm is at least as high as the risk of late relapse. Prolonged follow-up of patients with ES and PNET of bone is indicated.

Adolescent↗