Prevalence of RIB anomalies in normal Caucasian children and childhood cancer patients

Johannes H. M. Merks, Anne M. Smets, Rick R. van Rijn, Jasmijn Kobes, Huib N. Caron, Mario Maas, Raoul C. M. Hennekam

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Abstract

Purpose. - To evaluate the prevalence of abnormalities of rib development in normal Caucasian children and patients with childhood cancer. Materials And Methods. - Chest radiographs of 881 Caucasian pediatric controls and 906 childhood cancer patients were reviewed, and independently scored by four blinded observers, using strict definitions. Prevalences of 6 major rib anomaly categories in controls were compared to their prevalence in the total group of childhood cancer patients, and the 12 individual larger tumor groups using Chi-square tests. Results. - Values in the control population were generated for the occurrence of six major rib anomaly categories; cervical rib anomalies were present in 6.1% of controls, aplasia of 12(th) ribs in 6.6%, lumbar ribs in 0.9%, bifurcations in 0.7%, synostosis-bridging in 0.3%, and segmentations were not found. The overall prevalence of total rib anomalies in cases and controls was equal (14.9% and 14.2%, respectively). Cervical rib anomalies were found significantly more often in cases (8.6%) compared to controls (p-value=0.047), three groups accounting for this higher prevalence: 12.1% of acute lymphoblastic leukemia patients (p=0.011), 18.2% of astrocytoma patients (p=0.023), and 14.7% of germ cell tumor patients (p=0.046) had a cervical rib anomaly. Conclusion. - Prevalence figures for the presence and type of rib anomalies in a large group of normal Caucasian children were generated. In childhood cancer patients a significantly higher prevalence of cervical rib anomalies was demonstrated in patients with acute lymphoblastic leukemia, astrocytoma, and germ cell tumors. (c) 2004 Elsevier SAS. All rights reserved
Original languageEnglish
Pages (from-to)113-129
JournalEuropean journal of medical genetics
Volume48
Issue number2
DOIs
Publication statusPublished - 2005

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