PubMed HealthSearch

Biomedical subjects

C C Sun

Publications and source records attributed to C C Sun.

At least 19 recordsLinked to original sources

Pulmonary vein stenosis.

Pulmonary vein stenosis (PVS) is a rare disorder. Accurate diagnosis often requires anatomical examination. We report four children with pulmonary vein stenosis. Autopsy showed bilateral lesions in two patients who were thought clinically to have unilateral disease. A diagnosis of PVS was made at autopsy in the third case. Intimal and medial fibromuscular proliferation was noted in extrapulmonary and intrapulmonary veins. Some of the fibromuscular proliferation were eccentric, resembling organized thrombi. In one case a focal organizing thrombus was found in a clinically unobstructed but anatomically narrowed veno-atrial junction. In another case injection of contrast medium into the stenotic pulmonary vein (PV) showed anastomosis between PV and bronchial vessels as well as small pulmonary arteries. Bilateral hypertensive arteriopathy was observed in unilateral and bilateral PVS. Our histological finding of intrapulmonary venous lesions in the lobes in which PVS was not detected clinically suggests that during surgical correction of unilateral PVS multiple biopsies of the opposite lung may help to evaluate possible bilateral disease. Our study also suggests that thrombosis in a stenotic pulmonary vein may further compromise the lumen and contribute to the progression of pulmonary vein obstruction. The possible pathogenesis of bilateral pulmonary hypertensive arteriopathy in unilateral PVS also is discussed.

Biopsy

Photodamage and skin cancer among paraquat workers.

BACKGROUND: Some workers in paraquat manufacturing, exposed to bipyridines, have developed pigmentation and keratosis on sun-exposed skin. This condition has been described as skin-malignancy or premalignancy. This study was designed to clarify the pathologic features of these lesions and to explore the etiologic role played by bipyridine. METHODS: Twenty-three biopsy specimens, obtained from the affected skin of 10 workers, were scrutinized by a dermatopathologist. A total of 242 exposed workers from 28 paraquat factories were examined and interviewed during the period from 1983 to 1991. The severity of the characteristic skin lesions was graded from the lowest to the highest response to analyze the data by Mantel extension for a trend that focused on the heavy exposure to bipyridines as risk factor. RESULTS: All pathology specimens showed various degrees of solar damage: early actinic change, solar lentigo, actinic keratosis (AK), AK coexisting with squamous cell carcinoma (scc), and scc. Six specimens from four workers were scc or scc in situ. Three of six scc showed the coexistence of AK. Of the workers, 133 had skin lesions ranging in severity from grade 1 to grade 3 on sun-exposed areas. The severity of skin changes is strongly associated with heavy exposure to bipyridines (P < 0.0001). CONCLUSION: This pathologic study proves that all the lesions showed either photodamage or skin cancer. The strong trend in the correlation between severity of photodamage and exposure to bipyridine leads to the speculation about the synergistic role of bipyridine exposure and the solar effect in causing these malignant and premalignant skin lesions.

Adult

Sulfur spring dermatitis.

44 cases of an unusual condition, designated hot spring dermatitis, have been studied. Patients usually presented during the winter months with a history of having taken green sulfur spring baths within the previous 2-20 days. Skin lesions developed about 24 h after bathing and were distributed generally over the trunk and limbs, especially in the skin folds. No micro-organisms were found in either hot spring water specimens or skin lesions. Patch tests showed no positive reactions. Investigations were undertaken to determine the physicochemical characteristics of the hot spring. In its extreme acidity and high content of soluble sulfur and chloride, it differed from other nearby hot springs.

Adult

'Microgastria--limb reduction' complex with congenital heart disease and twinning.

We report a newborn, the second of male twins, with multiple abnormalities, including microphthalmia, a complex cardiovascular malformation, asplenia, anomalous lobation of the lungs, oesophageal atresia, microgastria, intestinal malrotation, anal atresia, multicystic dysplastic kidneys, and reduction defects of the upper extremities. These defects fit into the so-called 'microgastria-limb reduction' complex. Two of twelve previously reported patients with this complex were from discordant twin pairs. The occurrence of twinning in three out of 13 cases suggests that the origin of the 'microgastria-limb reduction' complex may be related to the process of twinning itself.

Abnormalities, Multiple

An unusual mosaic karyotype detected through prenatal diagnosis with duplication of 1q and 19p and associated teratoma development.

A 40-year-old white woman underwent amniocentesis for advanced maternal age at 15.4 weeks gestation. Fetal chromosome analysis demonstrated two distinct cell lines: [46,XX,t(1;19)(p11;p11)]--10%; and [47,XX,t(1;19)(p11;p11) + der(1)t(1;19)(p11;q11)]--90%. The latter karyotype was trisomic for both 1q and 19p. The mother carried the balanced translocation; the father had a normal karyotype. Amniotic fluid alpha-fetoprotein level was elevated and an acetylcholinesterase band was detected. Level II ultrasonography at 17 and 24 weeks revealed several abnormalities, including a large facial cleft and a probable facial teratoma and intracranial tumor. Autopsy following pregnancy termination confirmed the presence of both. Chromosome evaluation of 172 metaphases of both the epignathus and the intracranial teratoma demonstrated a predominance of the cell line with 47 chromosomes (166/172 = 96.5%), while from nonteratoma tissue (lung, liver, skin, and brain) only the balanced karyotype was detected. These observations suggest that the chromosomal imbalance is instrumental in the etiology of the teratoma.

Abnormalities, Multiple

Cytomegalovirus infection, fetal liver disease, and neonatal hemochromatosis.

Neonatal hemochromatosis is an uncommon disorder, clinicopathologically defined by severe and generally fatal liver disease of intrauterine onset associated with extrahepatic siderosis that spares reticuloendothelial elements (hemochromatotic siderosis). The agent or agents of liver disease in neonatal hemochromatosis are not known. It also is not known if intrauterine liver disease of defined infective etiology can lead to hemochromatotic siderosis. We present two patients with fetal liver disease and hemochromatotic siderosis whose cases help address these points. In the first patient rare hepatobiliary and numerous renal tubular cytomegalovirus (CMV) inclusions were found; CMV infection was confirmed by the polymerase chain reaction. Studies of the mother of the second patient 1, 5, and 9 weeks post-partum showed recent seroconversion against CMV; seroconversion against other infectious agents (toxoplasma, rubella, herpes, parvovirus B19, hepatitis A/B/C) was not present. Histologic, immunohistochemical, in situ hybridization, or polymerase chain reaction evidence of CMV infection was not present in infant tissues, even though peripartum maternal seroconversion against CMV was observed. We conclude that hemochromatotic siderosis may accompany chronic fetal liver disease of defined infective etiology (patient no. 1) and that recent maternal seroconversion against CMV in the presence of severe fetal liver disease does not necessarily mean that transplacentally acquired CMV infection caused the fetal liver disease (patient no. 2). Polymerase chain reaction documentation of infective-agent genomic sequences in fetal or infant tissues permits more accurate interpretation of maternal serologic data.

Adolescent

Disordered pathways of fibrin turnover in lung lavage of premature infants with respiratory distress syndrome.

Premature infants who have self-limited respiratory distress syndrome (RDS) rapidly improve, whereas infants with a complicated respiratory course are more likely to develop bronchopulmonary dysplasia (BPD), a chronic lung disorder that is the result of prolonged lung injury and impaired healing. The balance of competing activities of coagulation and fibrinolysis may contribute to the premature lung's response to acute injury and determine, in part, whether there is early resolution or protracted alveolar inflammation. To determine the relative activities of the coagulation and fibrinolytic pathways in neonatal lung injury, procoagulant (PC) and plasminogen activator (PA) activities were measured in undiluted cell-free lung lavage samples obtained serially over the first 28 days of life from 11 infants with self-limited RDS, 11 infants with evolving BPD, and 5 mechanically ventilated control infants without lung disease. Lung lavage from all three groups contained readily detectable procoagulant activity due mainly to the tissue factor-Factor VII complex. Plasminogen activator activity was relatively high in control lavage samples but depressed on the first day of life in the two groups of infants with lung disease: median, 0.3814 IU/ml (control); 0.0541 IU/ml (RDS); and 0.0454 IU/ml (BPD), p < 0.05 in each case compared with control. Two infants with severe lung disease had no detectable plasminogen activator activity in lung lavage on the first day of life. Depressed fibrinolytic activity correlated with severity of lung disease assessed radiographically and by pulmonary function measurements. Plasminogen activator activity was due to both tissue plasminogen activator and urokinase.(ABSTRACT TRUNCATED AT 250 WORDS)

Apgar Score

Alimentary duplication presenting as an hepatic cyst in a neonate.

A large hepatic cyst was excised from an infant who presented on the first day of life with an abdominal mass. Intraoperative, gross, and microscopic observations indicated that this was a cystic duplication of the ileum that extended into the liver. The cyst had a gastric mucosal lining, and there was evidence of ulceration. The occurrence of an alimentary duplication within the liver has been described in only one previous report.

Cysts

[Comparison of central effects produced by intracerebral injection of glutamic acid, quisqualic acid, and kainic acid].

Icv glutamic acid (Glu), quisqualic acid (QA) and kainic acid (KA) significantly increased spontaneous activity of mice in photecell box, and induced dose-dependent rise of blood pressure in anesthetized rats. Their intensities were arranged in the order of KA greater than Glu greater than QA. In mice step-through test Glu 0.1 micrograms icv improved learning and memory; KA 1 ng had no evident effect; and QA 0.1 microgram impaired learning and memory, which were also confirmed by step-down test in normal mice. Therefore, the non-NMDA (N-methyl-D-aspartate) receptor subtype might be different from the NMDA receptor subtype in the action of learning and memory.

Animals

The diagnostic value of IgM to natural trisaccharide phenylpropionyl bovine serum albumin in leprosy patients: a preliminary report from Taiwan.

An evaluation was made of the serum anti-phenolic-glycolipid-I (PGL-I) IgM levels of leprosy patients in the Taiwan area by enzyme-linked immunosorbent assay (ELISA) with a specific synthetic PGL-I antigen and natural trisaccharide phenylpropionyl bovine serum albumin (NT-P-BSA). Fifty-five blood samples were collected from 24 tuberculoid and 31 lepromatous leprosy patients and 21 healthy age- and sex-matched subjects. Among these groups, lepromatous patients had the highest levels of IgM and anti-NT-P-BSA IgM with a good correlation between these two levels (p less than 0.01). Tuberculoid patients also had higher levels than normal subjects. Wide variation in the standard deviation and decreased levels within the cutoff value of some lepromatous patients may be due to various periods of anti-leprosy treatment. Serial anti-NT-P-BSA IgM assessments in response to anti-leprosy treatment may provide more information and serve as a guideline for therapy.

Adult

Inflammatory pseudotumor of the retroperitoneum.

A child presenting with the findings of inflammatory disease was found to have a pseudotumor of the retroperitoneum. Following surgical removal, all signs of the systemic inflammatory process resolved. These rare, benign tumors of unknown etiology must not only be differentiated from locally invasive malignant lesions, but may present with findings suggesting a chronic inflammatory disorder.

Child, Preschool

The effect of immersion formaldehyde fixation on human placental weight.

The prevalence of the human immunodeficiency and hepatitis viruses has led to considerable concern by health care workers about safer means of examining surgical pathological specimens. The human placenta needs to be examined when there are complications during pregnancy, labor, and delivery; when the fetus is born with apparent problems; and when the delivered placenta is abnormal. Placentas are routinely immersion fixed with neutral buffered 3.7% to 4.0% formaldehyde solution before examination and without obtaining a fresh weight. This study was undertaken to determine if there was a significant change in weight between a fresh and fixed placenta. The results show a 7.67% increase in placental weight after formaldehyde fixation for 24 hours. Thus, the practice of formaldehyde fixation prior to weighing and examination can be continued and still allow for accurate estimation of fresh placental weight.

Female

The association of single umbilical artery with cytogenetically abnormal pregnancies.

The clinical significance of the absence of one of the two umbilical arteries (single umbilical artery) lies in its association with congenital malformations. Whether this association includes cytogenetic abnormalities is less clear. A retrospective review of all detected chromosomally abnormal pregnancies at the University of Maryland was carried out. Of 109 cytogenetically abnormal pregnancies, the number of umbilical cord vessels could be documented in 53 cases. Six (11.3%) had a single umbilical artery. A single umbilical artery was noted in two of nine fetuses (22.2%) with trisomy 18 and in two of six fetuses (33.3%) with trisomy 13. Two other unusual chromosomal constitutions were noted in cases of a single umbilical artery. None of the 11 fetuses with sex chromosome abnormalities (including eight with monosomy X) had a single umbilical artery. Of 18 fetuses with trisomy 21, none had a single umbilical artery. This study suggests that a single umbilical artery is preferentially associated with certain karyotypic abnormalities and that trisomy 21 does not appear to be associated with a single umbilical artery.

Amniocentesis

The nephroblastomatosis complex and its relationship to Wilms' tumor.

Nephroblastomatosis (NB), a persistence of abnormal embryonal renal tissue beyond 36 weeks' gestation, is often associated with Wilms' tumor. The exact relationship of NB to the development of Wilms' tumor is unclear. Four cases are presented that elucidate the entire morphological spectrum of this disease. Analyses of these cases suggest these conclusions: (1) the NB complex is a spectrum of lesions from benign multifocal nodular renal blastema, resembling residual nephrogenic zones of immature fetal kidney, to Wilms' tumor; (2) infantile NB is a premalignant variant of Wilms' tumor with a favorable outcome usually, when treated early; (3) neonatal nephromegaly requires a complete evaluation and follow-up imaging; persistence mandates biopsy; (4) "second-look" laparotomy is unnecessary with state-of-the-art imaging; and (5) standardization of terminology is essential.

Female

Intraabdominal desmoplastic small-cell tumors with divergent differentiation. Observations on three cases of childhood.

We studied three intraabdominal tumors that manifested in childhood and were attached to peritoneum, and in which the histologic pattern suggested metastatic tumor of epithelial nature but gave no evidence of a primary neoplasm in the major abdominal organs. Follow-up observation lasted from 1 to 6 years but never disclosed a primary site. Histologic, immunohistochemical, and electronmicroscopic observations indicated a primitive malignant neoplasm of uncertain histogenesis capable of simultaneously expressing epithelial, mesenchymal, and, less consistently, neural phenotypes. In childhood, the possibility of embryonic neoplasm, such as nephroblastoma occurring in atypical sites, is difficult to exclude. Despite the prevailing uncertainty about histogenesis, combined therapy achieved an apparent cure in one of our cases.

Abdominal Neoplasms

Thyroid carcinoma metastatic to the medial rectus muscle.

Isolated extraocular muscle metastasis is rare and is unreported for thyroid carcinoma. The authors describe a 72-year old man who presented with pain, redness, and proptosis of the right eye. Orbital computed tomography showed a large fusiform soft tissue mass along the medial aspect of the right orbit, involving the medial rectus. Orbital exploration disclosed a mass within the medial rectus muscle sheath involving the muscle belly. Histopathologic examination including electron microscopy, revealed metastatic thyroid carcinoma. Systemic treatment with radioactive iodine was recommended and refused by the patient. One year later, he died of complications from his metastatic disease.

Adenocarcinoma