PubMed Health⌕ Search

Biomedical subjects

T J Pysher

Publications and source records attributed to T J Pysher.

At least 37 records · Page 2Linked to original sources
PubMed1987

Dyskinetic cilia syndrome: clinical, radiographic and scintigraphic findings.

The clinical, electron microscopic and radiographic data of 9 patients with dyskinetic cilia syndrome (DCS) are presented. Scintigraphic evaluation of mucociliary dynamics in six patients showed evidence of dyskinesia. Ventilation and perfusion studies were performed to evaluate obstructive lung disease. Retrospectively, bronchiectasis could be detected in 77% of the patients by analysis of the chest radiograph and lung scintigraphy, and bronchography potentially avoided in the seven patients who underwent this procedure.

Biopsy↗
PubMed1986

Comparison of adrenal cortical tumors in children and adults.

Morphologic features (abnormal mitoses, necrosis, vascular and capsular invasion, broad fibrous bands, cellular pleomorphism, size) previously suggested to be predictors of malignant behavior in adrenal cortical tumors were assessed individually in 23 (17 benign, 6 malignant) pediatric and 42 (29 benign, 13 malignant) adult tumors. Of these features, size was the only predictor of malignancy in pediatric tumors. All pediatric tumors weighing more than 500 g were malignant and all but one weighing less than 500 g were benign. The remaining features were present in both benign and malignant pediatric tumors, and pediatric benign tumors were significantly more likely to have mitoses (P less than 0.01), necrosis (P less than 0.001), broad fibrous bands (P less than 0.005), and moderate to severe pleomorphism (P less than 0.01) than were adult benign tumors. The authors conclude that pediatric tumors are more likely to be benign than previously thought, and that size is the only morphologic predictor of their biologic behavior.

Adenoma↗
PubMed1985

Differential toxicity of RCAII (ricin) on rabbit intestinal epithelium in relation to postnatal maturation.

The purpose of this work was to assess the toxic lectin ricin (RCAII) as a probe for the study of intestinal permeability in the developing small bowel. Jejunal explants from suckling and adult rabbits were exposed to varying dosages of RCAII for 30 min at 25 degrees C and then cultured in toxin-free medium. The RCAII dose required to inhibit protein synthesis during 6 h of culture increased from 0.1 microgram/ml in 4-day-old rabbits to 25 micrograms/ml in weanling rabbits. RCAII cytotoxicity was almost completely blocked by 0.1 M lactulose in all age groups. The kinetics of 125I-RCAII binding to purified microvillus membranes were determined by incubating a fixed concentration of membrane protein (30 micrograms) with increasing concentrations of labeled lectin (2-18 micrograms/ml). Binding attained saturation with adult but not with suckling animal membranes. The latter yielded a curvilinear relationship in Scatchard plots, suggesting either several classes of binding sites or negative cooperativity. RCAII binding was confined to the delipidated fraction of the membranes and decreased by 42% from 6 days old to adult age. The extreme sensitivity of colostral epithelium to RCAII is probably related to the high level of endocytosis exhibited by the immature membrane of suckling rabbits. The development of increasing resistance to the toxin, and associated decrease in binding, might be related to disappearance of saccharide sites in productive surface receptors occurring in the developmental course of intestinal glycosylation.

Animals↗
PubMed1984

Peripheral neuroepithelioma in childhood.

Peripheral neuroepithelioma is a rare and controversial neoplasm that may occur at any age. Fifteen of the 38 previously reported cases have involved children from birth to 17 years of age. The authors observed the course of a 3-month-old girl who presented with an enlarging mass in the left arm and manifested hepatic metastases at the time of diagnosis. The urinary level of vanillylmandelic acid (VMA) was moderately elevated. The primary lesion was excised and metastatic foci showed response to a regimen of vincristine, cyclophosphamide, Adriamycin (doxorubicin), and cisplatin. However, tumor recurred in the brain and liver and the child died 14 months after diagnosis. At autopsy, there was no involvement of adrenal glands or sympathetic ganglia and the liver contained numerous involuted lesions as well as active metastases. It is suggested that this is a unique neoplasm, derived from neural crest but distinct from neuroblastoma, which can be characterized by peripheral origin, a histologic pattern of confluent pseudorosettes, and aggressive clinical behavior.

Adolescent↗
PubMed1984

Concurrent Cushing's and Zollinger-Ellison syndromes in a patient with islet cell carcinoma. Case report and review of the literature.

This report documents the simultaneous occurrence of Cushing's and Zollinger-Ellison syndromes in a patient with islet cell carcinoma. The clinical concurrence of these two syndromes has been recorded in only three previous case studies, while three other case reports record evidence suggesting the presence of both gastrin and ACTH within the tumor but without clinical sequelae. In the present report, evidence based on multiple clinical and biochemical data supports the concept that both syndromes are a result of "ectopic" hormone production by the tumor.

ACTH Syndrome, Ectopic↗
PubMed1984

Phaeohyphomycosis of the maxilloethmoid sinus caused by Drechslera spicifera: a new fungal pathogen.

"Phaeohyphomycosis" refers to soft tissue and systemic infections caused by dematiacious septate fungi. Drechslera spicifera, a dematiacious fungus, rarely pathogenic in humans was found to cause maxilloethmoid sinus disease in two immunocompetent children. The clinical presentation was similar to noninvasive aspergillosis. Intracavitary surgical excision without adjuvant chemotherapy resulted in apparent cure. The microbiologic and clinicopathologic aspects of this mycotic sinus disease are reviewed and discussed in relation to the entire spectrum of human disease reported which has been attributed to this organism.

Adult↗
PubMed1982

Improved results with hepatic portoenterostomy: a reassessment of its value in the treatment of biliary atresia.

As reported in 1974, the initial experience at the Oklahoma Children's Memorial Hospital with the Kasai procedure for biliary atresia was unsatisfactory. A subsequent series of 20 patients, in which 50% of the patients are alive and improved and 25% jaundice-free, is described. Modifications of the initial operative technique have been utilized. Postoperative complications in the ten children with sustained bile flow included cholangitis in five, hyponatremia in four, esophageal variceal hemorrhage in two, stomal bleeding in two, and gallbladder conduit malfunction in two patients. The improved outcome is attributed to earlier diagnosis and correction, attention to operative details, intensive postoperative nutritional support, and prompt recognition and management of complications.

Adolescent↗
PubMed1981

Hepatic abscess in neonates.

Hepatic abscess in the neonatal period is a rare but serious disorder. To our knowledge, only 24 cases have been reported in the literature; this study presents 13 additional cases. The most common associated factors are sepsis, vessel cannulation, and abdominal surgery. The etiologic agent is variable. The abscess is usually multiple in the liver and involves other organs as well. In such instances, only supportive and vigorous antibiotic therapy can be offered. However, solitary liver abscess does occur, and this is theoretically amenable to surgical drainage. Thus, its recognition is of great importance.

Female↗
PubMed1981

Differentiation of cholestatic jaundice in infants. Utility of diethyl-IDA.

Time-activity curves of technetium-labeled diethyl-IDA were used to evaluate 31 patients in an effort to differentiate infants with cholestatic jaundice. Results can be obtained rapidly with minimal radiation exposure and no known secondary effects. The combination of diethyl-IDA time-activity curves and gamma glutamyl transferase activity was found to discriminate biliary atresia from neonatal hepatitis. The group with neonatal hepatitis was characterized by abnormal, yet lower, gamma glutamyl transferase activities and diethyl-IDA time-activity curves which peaked within 1 minute of injection, yet decayed in a manner similar to cardiac activity. The group with biliary atresia was characterized by elevated gamma glutamyl transferase activities, hepatic peaks at 8 minutes and markedly delayed decay. In that early operative intervention is associated with increased survival in biliary atresia, prompt differentiation should improve prognosis.

Bile Ducts↗
PubMed1981

Bilateral juvenile granulosa cell tumors in a 4-month-old dysmorphic infant. A clinical, histologic, and ultrastructural study.

Juvenile granulosa cell tumors were encountered within bilateral cystic ovarian masses in a 4-month-old infant. The child was the product of a consanguinous pregnancy, and manifested poor growth, relative microcephaly, facial asymmetry, and a malformed left ear. There was no history of gestational drug or hormone ingestion, and no evidence of abnormal endocrine activity after birth, however, the serum alpha-fetoprotein level was mildly elevated. The tumors were well defined by sonography and there was no evidence of metastasis. Histologically, a dense proliferation of round to oval tumor cells showed considerable individual cell necrosis and frequent microcyst formation. There was no evidence of luteinization and only mild nuclear pleomorphism. Immunoperoxidase study failed to reveal alpha-fetoprotein. Ultrastructural study supported the granulosa cell nature of the tumor, but a few cells contained bundles of intracytoplasmic filaments. There has been no evidence of recurrent disease during a 16-month follow-up period, and serial alpha-fetoprotein determinations have remained in the reference range. Comparison with two previously reported bilateral juvenile granulosa cell tumors suggests that this tumor occurs in young infants, and is amenable to conservative therapy.

Abnormalities, Multiple↗
PubMed1980

Discordant congenital malformations in monozygous twins: the amniotic band disruption complex.

A stillborn monozygous twin delivered after 26 weeks gestation manifested multiple severe anomalies in association with an aberrant band of amniotic membrane which attached to the fetus' head (exencephaly) and abdomen (gastroschisis), entangled his lower extremities (flexion contractures, atrophy and amputation of digitis) and fixed his body in a position of lateral flexion. He also had bilateral proptosis, lateral facial cleft, absence of the right kidney, and an imperforate anus. The other twin expired after 36 hours but autopsy disclosed no malformations. Discordancy in monozygous twins due to congenital malformations is well known, but has been noted only rarely in association with the amniotic band disruption complex (ABDC). comparison with previous reports shows that in all cases only one member of the twinship was affected. Furthermore, isolated limb lesions (constriction rings and amputations), which are more common manifestations of ABDC than severe eviscerations or craniofacial deformities, have not been reported in twins. These observations support the concept that the severe form of ABDC is an acquired intrauterine lesion and they suggest that isolated limb lesions may not be part of the same malformation complex.

Adult↗
PubMedDate not supplied

Familial Wilms' tumor with neural elements: characterization by histology, immunohistochemistry, and genetic analysis.

Wilms' tumor (WT) is the most common renal malignancy of children. While most occur sporadically, a small percentage are familial or occur as part of a developmental syndrome. Classic WTs exhibit a triphasic histologic pattern composed of blastema, epithelium, and stroma. Occasionally, heterologous elements may also be observed. In this study we investigated a series of four WTs that occurred within a single familial aggregate and contained focal areas of neural differentiation. The tumors were evaluated histologically for the presence of neural elements and immunohistochemically for expression of neural-related markers. Genetic linkage analysis was performed on 3 of the 4 WTs. In addition to the classic triphasic histology, the WTs contained tumor rosettes (4/4), ganglion cells (2/4), foci of ganglioneuromatous differentiation (2/4), and anaplasia (1/4). Staining for chromogranin, S-100, synaptophysin, vimentin, and neuron-specific enolase was positive in all 4 tumors within the areas of neural differentiation whereas staining for CD99 (013) and glial fibrillary acidic protein was negative. Linkage analysis studies suggest that the familial predisposition gene segregating in this family is at 19q13.4. To our knowledge, this is the first reported series of WTs with neural differentiation that occurred within a single family aggregate. Genetic linkage analysis of this family is consistent with linkage to the FWT2 WT predisposition gene at 19q13.4. We propose that these tumors may represent a unique manifestation of tumor susceptibility in this family.

Biomarkers, Tumor↗