Annals of Diagnostic Paediatric Pathology

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Patients with Rotor syndrome ultrastructurally presented fairly similar features to Gilbert and to Dubin-Johnson simultaneously: some injury of vascular and canicular pole concomitant with an increase of smooth endoplasmic reticulum and pleomorphic mitochondria in hepatocytes. These similarities between some ultrastructural features of Gilbert and Dubin- Johnson syndrome were signaled in literature before [8]. The presence of microvilli along the lateral surface of hepatocytes was a particular feature, which distinguished patients with Rotor syndrome from other patients. Patients with Rotor syndrome exhibited lower level of direct bilirubin than unconjugated, and this observation does not agree with main idea of liver defect [5]. However, similar data on a direct bilirubin in serum have been mentioned in literature [3]. Electron microscopic methods permit on recognition of non-specific changes in the ultrastructure of the liver tissue among samples obtained from patients with non-hemolytic hyperbilirubinemias and can be still usefull in diagnosis of jaundince. References 1. Bethelot P, Dhumeaux D (1978) New signts in the classification and mechanism of hereditiary, chronic, nonhaemolytic hyperbilirubinemias. Gut 19:474-480 2. Chowdhury JR, Wolkoff AW, Wolkoff N, Chodhury R, Arias JM (1995) Hereditiary jaundice and disorders of bilirubin metabolism. In: Scriver ChR, Beaudet AL, Sly WS, Walle D (eds) The metabolic basis of inherited diseases, New York, Vol 1, pp 2161-2208 3. Czarnecki J, Cichosz H, Slomke M, Siezieniewska G, Czechowska G (1988) Clinical, biochemical and morphologic analysis of congenital non-hemolytic hyperbilirubinemia. Wiad Lek 11(17):1151-1156 (in Polish) 4. Dawson J, Carr-Loske DL, Talbot JC, Rossenthal FD (1978) Hepatic ultrastructure in Gilbert syndrome: evidence two populations. Gut 19A:995-999 5. Iyanagi T, Emi Y, Ikushiro S (1998) Biochemical and molecular aspects of genetic disorders of bilirubin metabolism. Biochem Biophys 1407:173-184 6. McGee JOD, Allan JG, Russel RJ, Patrick RS (19750 Liver ultrastructure in Gilbert syndrom. Gut 16: 220-224 7. Nishida T, Gatmaitan Z, Roy Chodhry R, Arias IM (1992) Two distinct mechanisms for bilirubin glucuronide transport by rat ble membrane vesicles. Demonstration of defective ATP-dependent transport in rats (TR-) with inherited conjugated hyperbilirubinemia. J Clin Invest 90:21-2135 8. Okolicsanyi L, Torrado A, Nussle D, Gardid D, Gautier A (1969) Etude clinique et ultrastructurale d’un cas de syndrome de Rotor. Revue Int Hepat 19:393-398
Annals of Diagnostic Paediatric Pathology 2004, 8(3-4): © Copyright by Polish Paediatric Pathology Society Congenital Heart Diseases Database: analysis of occurrence, diagnostics and coexistence with anomalies of other systems. Single institution experience Annals of Diagnostic Paediatric Pathology Anna Rybak, Aneta Wójcik, Bogdan WoŸniewicz Department of Pathology The Children’s Memorial Health Institute Warsaw, Poland Abstract The Congenital Heart Diseases Database was created in the Children’s Memorial Health Institute during the period of 2001 – 2003. It contains over 1000 preparations of congenital heart defects of children aged from 0 till 14 years. According to our data, the most common defect was ventricular septal defect. The compatibility of the clinic and pathological diagnoses was high (about 80% of the diagnoses were confirmed), however significantly higher after 1990. Although in our Database plenty of coexisting anomalies and diseases of the other systems or organs occurred, we found no significant relations between them and congenital heart defects. Key words: congenital heart disease, database, heart defects Introduction Since 1980 until now, the Children’s Memorial Health Institute of Warsaw has assembled over 1200 congenital heart defects preparations from patients aged 0-14 years. Between September 2001 and March 2003 we have made the repeated sections of those preparations and created “The Congenital Heart Diseases Database” (CHDDb) that includes 1037 records with full descriptions of the hearts’ and defects’ anatomy, clinical and sectional diagnoses, anomalies of other systems and performed operations. This is to present the direct conclusions that have arisen from this Database, including the frequency of occurrence of the particular heart defects, compatibility of the clinical and autopsy diagnoses and finally, regularity in coexistence of the congenital heart defects with the anomalies of the other systems. Material and methods The Congenital Heart Diseases Database is composed of 1037 preparations of the hearts with the congenital defects. They are fully described in four files which contain personal patients’ data, clinical diagnoses with the descriptions of defects Address for correspondence Bogdan Wozniewicz Department of Pathology The Children's Memorial Health Institute Aleja Dzieci Polskich 20, 04-736 Warsaw, Poland of the other systems and types of performed operations, pathologic diagnoses of the heart defect and coexisting diseases. The last file shows complete measurement of all the hearts including external dimensions as well as internal measurements of the valves, arteries, veins or myocardium. The patients’ age was between 1 day and 14 years. There were 437 female and 600 male patients described and they were all admitted to Children’s Memorial Health Institute during the period 1980 – 2003 and almost half of them were operated because of the heart defect. A nomenclature for the congenital heart disease was based on the International Nomenclature for Congenital Heart Surgery that was officially adopted at the Annual Meeting of the EACTS in Glasgow on September 6, 1999 [3]. Results and discussion Occurrence of the congenital heart defects The most common anomaly that appeared in our material was ventricular septal defect (VSD) with the frequency just under 30% (Fig.1). In details, there were 31 cases of VSD in muscular part of the septum (VSD single–29; VSD multiple-2 cases) and 259 cases of this defect in other parts of the interventricular septum (VSD; NOS). Phone: +4822-815-1960 E-mail: b.wozniewicz@czd.waw.pl
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Annals of Diagnostic Paediatric Pathology

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