PubMed:10064668 JSONTXT

{"project":"DisGeNET5_gene_disease","denotations":[{"id":"10064668-1#80#105#gene6023","span":{"begin":165,"end":190},"obj":"gene6023"},{"id":"10064668-1#107#110#diseaseC0220748","span":{"begin":192,"end":195},"obj":"diseaseC0220748"}],"relations":[{"id":"80#105#gene6023107#110#diseaseC0220748","pred":"associated_with","subj":"10064668-1#80#105#gene6023","obj":"10064668-1#107#110#diseaseC0220748"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"NCBIDiseaseCorpus","denotations":[{"id":"T1","span":{"begin":23,"end":29},"obj":"DiseaseClass:D009369"},{"id":"T2","span":{"begin":47,"end":72},"obj":"SpecificDisease:C535916"},{"id":"T3","span":{"begin":138,"end":144},"obj":"DiseaseClass:D009369"},{"id":"T4","span":{"begin":165,"end":190},"obj":"SpecificDisease:C535916"},{"id":"T5","span":{"begin":192,"end":195},"obj":"SpecificDisease:C535916"},{"id":"T6","span":{"begin":275,"end":278},"obj":"SpecificDisease:C535916"},{"id":"T7","span":{"begin":364,"end":367},"obj":"SpecificDisease:C535916"},{"id":"T8","span":{"begin":580,"end":586},"obj":"Modifier:D009369"},{"id":"T9","span":{"begin":705,"end":711},"obj":"DiseaseClass:D009369"},{"id":"T10","span":{"begin":745,"end":748},"obj":"SpecificDisease:C535916"},{"id":"T11","span":{"begin":853,"end":875},"obj":"SpecificDisease:D008228"},{"id":"T12","span":{"begin":988,"end":1008},"obj":"SpecificDisease:D002280"},{"id":"T13","span":{"begin":1093,"end":1099},"obj":"Modifier:D009369"},{"id":"T14","span":{"begin":1176,"end":1182},"obj":"Modifier:D009369"},{"id":"T15","span":{"begin":1274,"end":1280},"obj":"DiseaseClass:D009369"},{"id":"T16","span":{"begin":1293,"end":1315},"obj":"SpecificDisease:D008228"},{"id":"T17","span":{"begin":1382,"end":1385},"obj":"SpecificDisease:C535916"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"PubmedHPO","denotations":[{"id":"T1","span":{"begin":138,"end":144},"obj":"HP_0002664"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"DisGeNET","denotations":[{"id":"T0","span":{"begin":47,"end":72},"obj":"gene:6023"},{"id":"T1","span":{"begin":23,"end":29},"obj":"disease:C0006826"},{"id":"T2","span":{"begin":47,"end":72},"obj":"gene:6023"},{"id":"T3","span":{"begin":23,"end":29},"obj":"disease:C1306459"},{"id":"T4","span":{"begin":165,"end":190},"obj":"gene:6023"},{"id":"T5","span":{"begin":138,"end":144},"obj":"disease:C0006826"},{"id":"T6","span":{"begin":165,"end":190},"obj":"gene:6023"},{"id":"T7","span":{"begin":138,"end":144},"obj":"disease:C1306459"},{"id":"T8","span":{"begin":165,"end":190},"obj":"gene:6023"},{"id":"T9","span":{"begin":192,"end":195},"obj":"disease:C0220748"}],"relations":[{"id":"R1","pred":"associated_with","subj":"T0","obj":"T1"},{"id":"R2","pred":"associated_with","subj":"T2","obj":"T3"},{"id":"R3","pred":"associated_with","subj":"T4","obj":"T5"},{"id":"R4","pred":"associated_with","subj":"T6","obj":"T7"},{"id":"R5","pred":"associated_with","subj":"T8","obj":"T9"}],"namespaces":[{"prefix":"gene","uri":"http://www.ncbi.nlm.nih.gov/gene/"},{"prefix":"disease","uri":"http://purl.bioontology.org/ontology/MEDLINEPLUS/"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"NCBI-Disease-Train","denotations":[{"id":"T3204","span":{"begin":23,"end":29},"obj":"DiseaseClass"},{"id":"T3205","span":{"begin":47,"end":72},"obj":"SpecificDisease"},{"id":"T3206","span":{"begin":138,"end":144},"obj":"DiseaseClass"},{"id":"T3207","span":{"begin":165,"end":190},"obj":"SpecificDisease"},{"id":"T3208","span":{"begin":192,"end":195},"obj":"SpecificDisease"},{"id":"T3209","span":{"begin":275,"end":278},"obj":"SpecificDisease"},{"id":"T3210","span":{"begin":364,"end":367},"obj":"SpecificDisease"},{"id":"T3211","span":{"begin":580,"end":586},"obj":"Modifier"},{"id":"T3212","span":{"begin":705,"end":711},"obj":"DiseaseClass"},{"id":"T3213","span":{"begin":745,"end":748},"obj":"SpecificDisease"},{"id":"T3214","span":{"begin":853,"end":875},"obj":"SpecificDisease"},{"id":"T3215","span":{"begin":988,"end":1008},"obj":"SpecificDisease"},{"id":"T3216","span":{"begin":1093,"end":1099},"obj":"Modifier"},{"id":"T3217","span":{"begin":1176,"end":1182},"obj":"Modifier"},{"id":"T3218","span":{"begin":1274,"end":1280},"obj":"DiseaseClass"},{"id":"T3219","span":{"begin":1293,"end":1315},"obj":"SpecificDisease"},{"id":"T3220","span":{"begin":1382,"end":1385},"obj":"SpecificDisease"}],"attributes":[{"id":"A3204","pred":"database_id","subj":"T3204","obj":"D009369"},{"id":"A3205","pred":"database_id","subj":"T3205","obj":"C535916"},{"id":"A3206","pred":"database_id","subj":"T3206","obj":"D009369"},{"id":"A3207","pred":"database_id","subj":"T3207","obj":"C535916"},{"id":"A3208","pred":"database_id","subj":"T3208","obj":"C535916"},{"id":"A3209","pred":"database_id","subj":"T3209","obj":"C535916"},{"id":"A3210","pred":"database_id","subj":"T3210","obj":"C535916"},{"id":"A3211","pred":"database_id","subj":"T3211","obj":"D009369"},{"id":"A3212","pred":"database_id","subj":"T3212","obj":"D009369"},{"id":"A3213","pred":"database_id","subj":"T3213","obj":"C535916"},{"id":"A3214","pred":"database_id","subj":"T3214","obj":"D008228"},{"id":"A3215","pred":"database_id","subj":"T3215","obj":"D002280"},{"id":"A3216","pred":"database_id","subj":"T3216","obj":"D009369"},{"id":"A3217","pred":"database_id","subj":"T3217","obj":"D009369"},{"id":"A3218","pred":"database_id","subj":"T3218","obj":"D009369"},{"id":"A3219","pred":"database_id","subj":"T3219","obj":"D008228"},{"id":"A3220","pred":"database_id","subj":"T3220","obj":"C535916"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"NCBI-Disease-Corpus-All","denotations":[{"id":"T3204","span":{"begin":23,"end":29},"obj":"DiseaseClass"},{"id":"T3205","span":{"begin":47,"end":72},"obj":"SpecificDisease"},{"id":"T3206","span":{"begin":138,"end":144},"obj":"DiseaseClass"},{"id":"T3207","span":{"begin":165,"end":190},"obj":"SpecificDisease"},{"id":"T3208","span":{"begin":192,"end":195},"obj":"SpecificDisease"},{"id":"T3209","span":{"begin":275,"end":278},"obj":"SpecificDisease"},{"id":"T3210","span":{"begin":364,"end":367},"obj":"SpecificDisease"},{"id":"T3211","span":{"begin":580,"end":586},"obj":"Modifier"},{"id":"T3212","span":{"begin":705,"end":711},"obj":"DiseaseClass"},{"id":"T3213","span":{"begin":745,"end":748},"obj":"SpecificDisease"},{"id":"T3214","span":{"begin":853,"end":875},"obj":"SpecificDisease"},{"id":"T3215","span":{"begin":988,"end":1008},"obj":"SpecificDisease"},{"id":"T3216","span":{"begin":1093,"end":1099},"obj":"Modifier"},{"id":"T3217","span":{"begin":1176,"end":1182},"obj":"Modifier"},{"id":"T3218","span":{"begin":1274,"end":1280},"obj":"DiseaseClass"},{"id":"T3219","span":{"begin":1293,"end":1315},"obj":"SpecificDisease"},{"id":"T3220","span":{"begin":1382,"end":1385},"obj":"SpecificDisease"}],"attributes":[{"id":"A3204","pred":"database_id","subj":"T3204","obj":"D009369"},{"id":"A3205","pred":"database_id","subj":"T3205","obj":"C535916"},{"id":"A3206","pred":"database_id","subj":"T3206","obj":"D009369"},{"id":"A3207","pred":"database_id","subj":"T3207","obj":"C535916"},{"id":"A3208","pred":"database_id","subj":"T3208","obj":"C535916"},{"id":"A3209","pred":"database_id","subj":"T3209","obj":"C535916"},{"id":"A3210","pred":"database_id","subj":"T3210","obj":"C535916"},{"id":"A3211","pred":"database_id","subj":"T3211","obj":"D009369"},{"id":"A3212","pred":"database_id","subj":"T3212","obj":"D009369"},{"id":"A3213","pred":"database_id","subj":"T3213","obj":"C535916"},{"id":"A3214","pred":"database_id","subj":"T3214","obj":"D008228"},{"id":"A3215","pred":"database_id","subj":"T3215","obj":"D002280"},{"id":"A3216","pred":"database_id","subj":"T3216","obj":"D009369"},{"id":"A3217","pred":"database_id","subj":"T3217","obj":"D009369"},{"id":"A3218","pred":"database_id","subj":"T3218","obj":"D009369"},{"id":"A3219","pred":"database_id","subj":"T3219","obj":"D008228"},{"id":"A3220","pred":"database_id","subj":"T3220","obj":"C535916"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"NCBI-Disease-Corpus-2stage-All","denotations":[{"id":"T1","span":{"begin":23,"end":29},"obj":"DiseaseClass"},{"id":"T2","span":{"begin":47,"end":72},"obj":"SpecificDisease"},{"id":"T3","span":{"begin":138,"end":144},"obj":"DiseaseClass"},{"id":"T4","span":{"begin":165,"end":190},"obj":"SpecificDisease"},{"id":"T5","span":{"begin":192,"end":195},"obj":"SpecificDisease"},{"id":"T6","span":{"begin":275,"end":278},"obj":"SpecificDisease"},{"id":"T7","span":{"begin":364,"end":367},"obj":"SpecificDisease"},{"id":"T8","span":{"begin":580,"end":586},"obj":"DiseaseClass"},{"id":"T9","span":{"begin":705,"end":711},"obj":"DiseaseClass"},{"id":"T10","span":{"begin":745,"end":748},"obj":"SpecificDisease"},{"id":"T11","span":{"begin":853,"end":875},"obj":"SpecificDisease"},{"id":"T12","span":{"begin":988,"end":1008},"obj":"SpecificDisease"},{"id":"T13","span":{"begin":1093,"end":1099},"obj":"DiseaseClass"},{"id":"T14","span":{"begin":1176,"end":1182},"obj":"DiseaseClass"},{"id":"T15","span":{"begin":1274,"end":1280},"obj":"DiseaseClass"},{"id":"T16","span":{"begin":1293,"end":1315},"obj":"SpecificDisease"},{"id":"T17","span":{"begin":1382,"end":1385},"obj":"SpecificDisease"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"NCBI-Disease-Corpus-rezarta-All","denotations":[{"id":"T1","span":{"begin":23,"end":29},"obj":"DiseaseClass"},{"id":"T2","span":{"begin":47,"end":72},"obj":"SpecificDisease"},{"id":"T3","span":{"begin":138,"end":144},"obj":"DiseaseClass"},{"id":"T4","span":{"begin":165,"end":190},"obj":"SpecificDisease"},{"id":"T5","span":{"begin":192,"end":195},"obj":"SpecificDisease"},{"id":"T6","span":{"begin":275,"end":278},"obj":"SpecificDisease"},{"id":"T7","span":{"begin":364,"end":367},"obj":"SpecificDisease"},{"id":"T8","span":{"begin":580,"end":586},"obj":"DiseaseClass"},{"id":"T9","span":{"begin":705,"end":711},"obj":"DiseaseClass"},{"id":"T10","span":{"begin":745,"end":748},"obj":"SpecificDisease"},{"id":"T11","span":{"begin":853,"end":875},"obj":"SpecificDisease"},{"id":"T12","span":{"begin":988,"end":1008},"obj":"SpecificDisease"},{"id":"T13","span":{"begin":1093,"end":1099},"obj":"DiseaseClass"},{"id":"T14","span":{"begin":1176,"end":1182},"obj":"DiseaseClass"},{"id":"T15","span":{"begin":1274,"end":1280},"obj":"DiseaseClass"},{"id":"T16","span":{"begin":1293,"end":1315},"obj":"SpecificDisease"},{"id":"T17","span":{"begin":1382,"end":1385},"obj":"SpecificDisease"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"NCBI-Disease-Corpus-4oGuideline-All","denotations":[{"id":"T1","span":{"begin":23,"end":29},"obj":"SpecificDisease"},{"id":"T2","span":{"begin":47,"end":72},"obj":"SpecificDisease"},{"id":"T3","span":{"begin":138,"end":144},"obj":"SpecificDisease"},{"id":"T4","span":{"begin":165,"end":190},"obj":"SpecificDisease"},{"id":"T5","span":{"begin":192,"end":195},"obj":"SpecificDisease"},{"id":"T6","span":{"begin":275,"end":278},"obj":"SpecificDisease"},{"id":"T7","span":{"begin":364,"end":367},"obj":"SpecificDisease"},{"id":"T8","span":{"begin":580,"end":586},"obj":"SpecificDisease"},{"id":"T9","span":{"begin":705,"end":711},"obj":"SpecificDisease"},{"id":"T10","span":{"begin":745,"end":748},"obj":"SpecificDisease"},{"id":"T11","span":{"begin":853,"end":875},"obj":"SpecificDisease"},{"id":"T12","span":{"begin":988,"end":1008},"obj":"SpecificDisease"},{"id":"T13","span":{"begin":1093,"end":1099},"obj":"SpecificDisease"},{"id":"T14","span":{"begin":1176,"end":1182},"obj":"SpecificDisease"},{"id":"T15","span":{"begin":1274,"end":1280},"obj":"SpecificDisease"},{"id":"T16","span":{"begin":1293,"end":1315},"obj":"SpecificDisease"},{"id":"T17","span":{"begin":1382,"end":1385},"obj":"SpecificDisease"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}

{"project":"NCBI-Disease-Corpus-Simple-All","denotations":[{"id":"T1","span":{"begin":23,"end":29},"obj":"DiseaseClass"},{"id":"T2","span":{"begin":47,"end":72},"obj":"SpecificDisease"},{"id":"T3","span":{"begin":138,"end":144},"obj":"DiseaseClass"},{"id":"T4","span":{"begin":165,"end":196},"obj":"SpecificDisease"},{"id":"T5","span":{"begin":275,"end":278},"obj":"SpecificDisease"},{"id":"T6","span":{"begin":364,"end":367},"obj":"SpecificDisease"},{"id":"T7","span":{"begin":580,"end":586},"obj":"DiseaseClass"},{"id":"T8","span":{"begin":705,"end":711},"obj":"DiseaseClass"},{"id":"T9","span":{"begin":745,"end":748},"obj":"SpecificDisease"},{"id":"T10","span":{"begin":853,"end":875},"obj":"SpecificDisease"},{"id":"T11","span":{"begin":988,"end":1008},"obj":"SpecificDisease"},{"id":"T12","span":{"begin":1093,"end":1099},"obj":"DiseaseClass"},{"id":"T13","span":{"begin":1176,"end":1182},"obj":"DiseaseClass"},{"id":"T14","span":{"begin":1274,"end":1280},"obj":"DiseaseClass"},{"id":"T15","span":{"begin":1293,"end":1315},"obj":"SpecificDisease"},{"id":"T16","span":{"begin":1382,"end":1385},"obj":"SpecificDisease"}],"text":"Increased incidence of cancer in patients with cartilage-hair hypoplasia.\nOBJECTIVE: Previous reports have suggested an increased risk of cancer among patients with cartilage-hair hypoplasia (CHH). This study was carried out to further evaluate this risk among patients with CHH and their first-degree relatives.\nSTUDY DESIGN: One hundred twenty-two patients with CHH were identified through 2 countrywide epidemiologic surveys in 1974 and in 1986. Their parents and nonaffected siblings were identified through the Population Register Center. This cohort underwent follow-up for cancer incidence through the Finnish Cancer Registry to the end of 1995.\nRESULTS: A statistically significant excess risk of cancer was seen among the patients with CHH (standardized incidence ratio 6.9, 95% confidence interval 2.3 to 16), which was mainly attributable to non-Hodgkin's lymphoma (standardized incidence ratio 90, 95% confidence interval 18 to 264). In addition, a significant excess risk of basal cell carcinoma was seen (standardized incidence ratio 35, 95% confidence interval 7.2 to 102). The cancer incidence among the siblings or the parents did not differ from the average cancer incidence in the Finnish population.\nCONCLUSIONS: This study confirms an increased risk of cancer, especially non-Hodgkin's lymphoma, probably attributable to defective immunity, among patients with CHH."}