Children's Memorial Health Institute Research Articles

Heterogeneity of humoral immune abnormalities in children with Nijmegen breakage syndrome: an 8-year follow-up study in a single centre.

During an 8-year period of observation, defects of immune responses were characterized and monitored in 40 of 50 Polish children with Nijmegen breakage syndrome referred to the Children's Memorial Health Institute in Warsaw. The following parameters were determined at diagnosis: (1) concentrations of serum IgM, IgG, IgA; (2) concentrations of IgG subclasses; and (3) lymphocyte subpopulations. In addition, naturally acquired specific antibodies against Streptococcus pneumoniae were determined in 20 patients with a history of recurrent respiratory infections. During follow-up, total serum immunoglobulins and IgG subclasses were monitored systematically in 17 patients who did not receive immunomodulatory therapy. Moreover, anti-HBs antibody response was measured after vaccination of 20 children against HBV. We found that the immune deficiency in NBS is profound, highly variable, with a tendency to progress over time. Systematic monitoring of the humoral response, despite good clinical condition, is essential for early medical intervention.

The European Congenital Heart Defects Surgery Database experience: Pediatric European Cardiothoracic Surgical Registry of the European Association for Cardio-Thoracic Surgery

The initial purpose of collecting data on the outcome of congenital heart surgery procedures across Europe was to make possible comparison of results and definition of mortality and morbidity risk factors as well as targeting research activities. The European Congenital Heart Surgeons Foundation, established in 1992, created the European Congenital Heart Defects Database, precursor to today's Pediatric European Cardiothoracic Surgical Registry. In 1999, initiatives of the Society of Thoracic Surgeons and the European Association for Cardio-Thoracic Surgery resulted in a series of conferences aimed at arriving at a standardized nomenclature and reporting strategies as a foundation for an international database. In April 2000 the International Congenital Heart Surgery Nomenclature and Database Project published a minimum dataset of 21 items and lists of 150 diagnoses, 200 procedures, and 32 complications, as well as 28 extracardiac anomalies and 17 preoperative risk factors. Since January 2000 the Pediatric European Cardiothoracic Surgical Registry has officially operated from the Department of Cardiothoracic Surgery at the Children's Memorial Health Institute in Warsaw, Poland, under the auspices of the European Association for Cardio-Thoracic Surgery and the responsibility of Bohdan Maruszewski. As of March 2001, 84 cardiothoracic units from 33 countries had registered in the database and data on almost 4,000 procedures have been collected. Participation in the database is free of charge through the internet for all participants. Development of data validation protocols is a work in progress. Copyright © 2002 by W.B. Saunders Company

Reluctance to accept FG syndrome diagnosis

American Journal of Medical GeneticsVolume 83, Issue 5 p. 430-430 Letter to the Editor Reluctance to accept FG syndrome diagnosis K. Chrzanowska, Corresponding Author K. Chrzanowska chrzanowska@czd.waw.pl Department of Medical Genetics, The Children's Memorial Health Institute, Warsaw, PolandDepartment of Medical Genetics, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-736 Warsaw, PolandSearch for more papers by this authorK. Kozlowski, K. Kozlowski Department of Radiology, Royal Alexandra Hospital for Children, Sydney, AustraliaSearch for more papers by this author K. Chrzanowska, Corresponding Author K. Chrzanowska chrzanowska@czd.waw.pl Department of Medical Genetics, The Children's Memorial Health Institute, Warsaw, PolandDepartment of Medical Genetics, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-736 Warsaw, PolandSearch for more papers by this authorK. Kozlowski, K. Kozlowski Department of Radiology, Royal Alexandra Hospital for Children, Sydney, AustraliaSearch for more papers by this author First published: 05 April 1999 https://doi.org/10.1002/(SICI)1096-8628(19990423)83:5<430::AID-AJMG19>3.0.CO;2-YAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume83, Issue523 April 1999Pages 430-430 RelatedInformation

Risk factors of recurrence in 157 MB/PNET patients treated in one institution.

To evaluate the risk factors for recurrence of MB/PNET we analyzed the medical records of 157 patients treated at the Children's Memorial Health Institute between February 1981 and February 1997. The following factors were evaluated: age at diagnosis, gender, tumor size, tumor cells in the CSF, postoperative status, extent of resection and methods of treatment. We evaluated chemotherapy (CHT) doses, interval between courses, interval between surgery (S) and first course of CHT, interval between S and radiotherapy (RTX), and breaks during RTX. We divided patients into six groups: S alone, S+CHT, S+RTX, S+CHT+ RTX, S+RTX+CHT, S+CHT+RTX+ CHT. Age at diagnosis, gender, tumor size, extent of resection, postoperative status, intervals between courses of CHT, between S and the first course of CHT, and between S and RTX, and breaks during RTX had no statistical influence on relapse occurrence. Tumor cells in CSF were routinely checked for from January 1992 onward. In this group of 75 patients, 40 had tumor cells positive at surgery (28 relapsed), while in the group of 35 patients with negative tumor cells 14 relapsed (P=0.004). Out of 26 patients treated with S+RTX alone, 13 relapsed. Among 14 patients treated with S+RTX and prolonged CHT 6 relapsed. Out of 14 patients treated with S+CHT 13 relapsed; among 49 who received S+CHT+RTX 35 relapsed; and out of 51 patients treated with S+CHT+RTX+CHT 30 relapsed. In the multivariate analysis of treatment methods chemotherapy implemented after radiotherapy had a positive, though not statistically significant, influence on outcome (P=0.06). Among those receiving CHT the mean percentage of the ideal dose administered had a statistically significant influence on relapse: in the group of relapsed patients the mean dose was 76.1%, while in the group in continuous remission it was 83.7% (P=0.0013). On the basis of our data, we conclude that the presence of tumor cells in the CSF had a significant influence on the occurrence of relapse. Administration of appropriate doses of chemotherapy is extremely important for the occurrence of relapse and the final outcome of treatment. Prolonged adjuvant chemotherapy after radiotherapy seems to lower the risk of recurrence.

Poor mental development in patients with tuberous sclerosis complex: clinical risk factors.

To identify clinical risk factors for poor mental development among patients with tuberous sclerosis complex (TSC). Case-control analysis of a clinic population. Specialty clinic in a hospital. One hundred six patients with TSC consecutively seen between January 1984 and December 1995 at the Child Neurology Clinic of the Children's Memorial Health Institute in Warsaw, Poland. Seizure type, age at seizure onset, sex, and history of diphtheria, tetanus, and pertussis immunization. Moderate to profound developmental delays. Seizure type (ie, infantile spasms) was the only analyzed risk factor that showed a consistent and independent association with poor mental development (adjusted odds ratio, 3.0; 95% confidence interval, 1.1-8.4; P =.03). Age at seizure onset, which initially showed a significant association with poor mental development, was no longer significantly associated after adjustment for seizure type (adjusted odds ratio, 1.6; P = .43). Neither sex (odds ratio, 1.1; P = .96) nor history of diphtheria, tetanus, and pertussis immunization (odds ratio, 1.0; P = .80) showed evidence of being a risk factor for poor mental development among patients with TSC. Infantile spasms, as the type of seizure on initial examination, is a significant risk factor for poor mental development in patients with TSC. Age at time of first seizure is not an independent risk factor but reflects the early ages at which these patients are seen with infantile spasms. Neither sex nor history of diphtheria, tetanus, and pertussis immunization is a risk factor for the subsequent development of poor mental development among patients with TSC.

Contamination of parenteral nutrition additives, aminoacids solutions and lipid emulsions with aluminum : Popińska K., Socha J., Lyszkowska M., Ksiazyk J., Kierkuś J., Pietraszek E., Kmiotek W. Children's Memorial Health Institute, Warsaw, Poland

Contamination of parenteral nutrition additives, aminoacids solutions and lipid emulsions with aluminum : Popińska K., Socha J., Lyszkowska M., Ksiazyk J., Kierkuś J., Pietraszek E., Kmiotek W. Children's Memorial Health Institute, Warsaw, Poland

Infectious and metabolic complications of parenteral nutrition in children : Lyszkowska M., Pawińska A., Ksiazyk J., Popinska K., Kierkuś J., Porczyk A. Children's Memorial Health Institute, Warsaw, Poland

Infectious and metabolic complications of parenteral nutrition in children : Lyszkowska M., Pawińska A., Ksiazyk J., Popinska K., Kierkuś J., Porczyk A. Children's Memorial Health Institute, Warsaw, Poland

Is the use of H 2 receptor antagonists justified in short bowel syndrome? : Kierkuś J., Ksiazyk J., Socha P., Lyszkowska M. Children's Memorial Health Institute, Warsaw, Poland

Is the use of H 2 receptor antagonists justified in short bowel syndrome? : Kierkuś J., Ksiazyk J., Socha P., Lyszkowska M. Children's Memorial Health Institute, Warsaw, Poland

Energy balance in short bowel syndrome : Ksiazyk J., Lyszkowska M., Kierkuś J. Children's Memorial Health Institute, Warsaw, Poland

Energy balance in short bowel syndrome : Ksiazyk J., Lyszkowska M., Kierkuś J. Children's Memorial Health Institute, Warsaw, Poland

Technical complications of parenteral nutrition in children : Bogucki K., Porczyk A., Kierkuś J. Children's Memorial Health Institute, Warsaw, Poland

Technical complications of parenteral nutrition in children : Bogucki K., Porczyk A., Kierkuś J. Children's Memorial Health Institute, Warsaw, Poland