The influence of cardiovascular malformations on the prognosis of esophageal atresia and distal tracheoesophageal fistula

Two hundred and seventy-six cases of esophageal atresia (EA) and tracheoesophageal fistula (TEF) (173 males and 103 females) were studied. A cardiovascular malformation (CVM) was the most common associated anomaly (79 cases, 32%). The most common extracardiac malformations (ECM) included skeletal, gastrointestinal, and urinary tract anomalies. There was a significantly lower mortality in patients without associated anomalies (5%) compared to those with a major CVM (41%) (P<.01) but not compared to those with a minor CVM (10%). No significant difference in survival could be attributed to the specific types of CVM, although the overall survival in those with a non-complex CVM (64%) was higher than in those with a complex CVM (12%). It was not possible to associate a particular ECM with a specific form of CVM, but the greater the number of ECMs present, the greater the likelihood of associated CVM. Offprint requests to: S. H. Ein     

Analysis of Breathing Pattern in Children with Asthma

The breathing pattern in children with asthma was studied, using respiratory inductive plethysmography. The subjects were 31 children with mild to moderate asthma (15 males, 16 females; aged 6∼15 years, average 11 years). The respiratory rate was 25.5±10.7/min. (mean ± S.D.) during asthma attacks and 18.4 ± 5.0/min. between attacks with a significant increase during attacks (P < 0.001). The expiratory time was 1.774 ± 0.833 second during attacks, and 2.125 ± 0.602 seconds between attacks. The expiratory time to total respiratory time ratio (T_E/T_TOT) was 0.631 ± 0.056 during attacks and 0.608 ± 0.035 between attacks. Thus there was a slight prolongation at the time of attack, but the difference was not significant. The tidal volume was increased slightly during mild and moderate attacks. V_T/T_I (mean inspiratory flow) was increased during attacks. The respiratory pattern was thoracic during attacks and changed to the abdominal pattern with improvement.     

Enema reduction of intussusception with a small dose of iopamidol may have advantages over barium

Barium enema (B-enema) has been the standard method for hydrostatic reduction of intussusception, although recently air enema has been used due to the lower risk when perforation occurs. Recently, we have administered a small dose of iopamidol during enema reduction (I-enema) in children with intussusception. From November 1989 to December 1993, we treated 50 children with intussusception at Kiyama Hospital. Barium was used in the first half of the period, and iopamidol in the second half. Reduction was successful in 22 of 24 patients with barium (92%) and 23 of 26 with iopamidol (88%); 25 children had the ileocolic type and 25 the ileoileocolic (-cecal) type of intussusception. Operations were carried out in 3 patients from each group. I-enema avoids some of the drawbacks of barium and air enemas. It is a new method of enema reduction, as a contrast medium is injected first. It is possible to obtain a good image of the advanced portion with a small dose of contrast medium, which is important for treatment. For institutions performing B-enemas, I-enemas can be performed easily with the same equipment and technique. It causes less contamination upon leakage than a B-enema, and also has less influence on the intestinal membrane with very few risks if perforation occurs. Better images are obtained than with air. A large dose of contrast medium is not needed, thereby reducing medical expenses to a minimum. Iopamidol can be used safely for enema reduction of intussusception with an expected high success rate.     

Case report of dysgerminoma in a patient with 46,XX pure gonadal dysgenesis

A clinicopathological study of a 42-year-old female with pure gonadal dysgenesis and dysgerminoma was made. At the age of 29, the patient with primary amenorrhea had been evaluated clinically and cytogenetically. (1) The results of cytogenetic studies were X-chromatin positive and revealed a karyotype in peripheral blood leukocytes of 46,XX. (2) Laboratory studies indicated hypergonadotropic hypogonadism and no response of the gonads to the human menopausal gonadotropin stumulation test. (3) At laparotomy, the gonads were streak-like. Pathological examinations of biopsy specimens from both gonads revealed dense, fibrous connective tissue resembling ovarian stroma and no primary follicles. Eleven years after the laparotomy, the patient complained of lower abdominal distention and severe pain, and laparotomy then revealed a 15 × 17-cm right solid adnexal mass occupying the pelvic cavity. The histological diagnosis of tissues from the partially removed tumor was pure dysgerminoma. Second-look operation after Linac X-ray irradiation showed complete remission of the residual tumor. Insofar as we are aware, the present patient represents the first case of dysgerminoma which occurred in the dysgenetic gonads of a phenotypic female with normal 46,XX sex-chromosomal constitutions in peripheral blood leukocytes and the skin fibroblasts although a possibility exists that mosaicism was possibly present but undetected, particularly since the streak gonads were not analyzed chromosomally.     

Molecular pathogenesis of pulmonary carcinosarcoma as determined by microdissection-based allelotyping

Pulmonary carcinosarcoma is a rare, biphasic tumor composed of malignant epithelial and mesenchymal elements. Its histogenesis is controversial in light of the presence of divergent cell lineages and the clonal nature of malignancy. To address these issues, we performed an extensive comparative genotypic analysis using microdissection to secure representative mesenchymal and epithelial components from each of six cases of pulmonary carcinosarcoma. Loss of heterozygosity was analyzed with a panel of 12 polymorphic microsatellite markers designed to indicate allelic loss and situated in proximity to known tumor suppressor genes located on 1p, 3p, 5q, 9p, 10q, and 17p. In accordance with the relatively greater biologic aggressiveness of this tumor type, both the epithelial and mesenchymal components showed extensive allelic loss, most notably for 3p, 5q, and 17p. More importantly, we found overall equivalent patterns of acquired allelic loss between the two components on an individual case basis, strongly supporting the monoclonal origin of these neoplasms. Minor differences in the allelic fingerprint between the two cell lineages could be explained by progressive accumulation of allelic loss alterations that appear to occur more frequently in the mesenchymal component of the tumor. The data support the efficacy of microdissection-based allelic fingerprinting to delineate the relationship between different morphologic components of a single neoplasm.     

PPARgamma activation and adipocyte differentiation induced by AS-6, a prenyl-phenol antidiabetic antibiotic

The prenyl-phenol antibiotics ascochlorin-related compounds, are known to reduce serum cholesterol and triglyceride, suppress hypertension, and ameliorate types-I and II diabetes. However, little is known about the molecular mechanism for these physiological effects. Here we report that the ascochlorin derivative, 4-O-carboxymethyl ascochlorin (AS-6) acts as a potent activator of the nuclear hormone receptor, PPARgamma, although it does not activate the related receptors, PPARalpha, PPARdelta or RARalpha. AS-6 interacts directly with the PPARgamma molecule in vitro, and induces differentiation of the mouse preadipocyte cell line 3T3-L1. Our results suggest that AS-6 is a partial agonist for PPARgamma with a novel chemical structure.     

Propeptin,a new inhibitor of prolyl endopeptidase produced by microbispora II. Determination of chemical structure

The structure of propeptin, a new inhibitor of prolyl endopeptidase isolated from Microbispora sp. SNA-115, was determined. FAB/MS, Edman degradation and amino acid analysis revealed propeptin to be a cyclic polypeptide consisting of 19 common L-amino acids. By FAB/MS and protein chemical methods, the primary sequence of propeptin was determined to be Gly1-Tyr-Pro-Trp-Trp-Asp-Tyr-Arg-Asp9-Leu-Phe-Gly-Gly-His-Thr-Phe-Ile-Ser-Pro19, which cyclizes between the beta-carboxyl group of Asp9 and the a-amino group of Gly1.     

Prediction of short-term progression or regression of atherosclerotic coronary artery disease by lipoprotein (a): a quantitative coronary angiographic study

This study assessed whether progression of coronary artery atherosclerotic lesions could be predicted in the short term using various lipid profiles. In 37 patients (61.9 +/- 9.5 years) undergoing coronary angioplasty and with 6-month follow-up angiography, quantitative coronary angiography of a new or changed lesion was performed in the follow-up examination, except for intervention vessels. The progression-regression score of the assessed lesion was calculated as the baseline minus the follow-up minimal lumen diameter. The serum lipoprotein (a) level was higher in the progression group (progression-regression score > 0.15 mm), than in the regression group (< or = -0.15 mm; p < 0.01) and the no change group (within +/- 0.15 mm; p < 0.05). Remnant-like lipoprotein particle-cholesterol and apolipoprotein-B levels were also higher in the progression group. However, multiple regression analysis of the progression showed that the progression-regression score was independently correlated with lipoprotein (a) alone (R = 0.50, p < 0.05). This shows that lipoprotein (a) is an independent predictor of coronary atherosclerotic lesion progression over the short term.     

Modification of the radial procedure in a patient with partial atrioventricular septal defect

We successfully cured atrial fibrillation while preserving internodal conduction in a patient with a partial atrioventricular septal defect. Because the anterior and middle internodal tracts are interrupted by the defect, the lower right atrial incision of either the maze or the radial procedure may interrupt the remaining posterior tract, resulting in internodal conduction block. We deleted the posterior septal incision from the radial procedure and replaced it with a right-side left atriotomy. The patient resumed normal sinus rhythm with significant contraction of the right and left atria. The preserved internodal pathway through the posterior interatrial septum was confirmed by electrophysiologic study.