Increased incidence of cancer in patients with cartilage-hair hypoplasia  

Oleh:

Makitie, Outi ; Pukkala, Eero ; Teppo, Lyly ; Kaitila, Ilkka

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The Journal of Pediatrics vol. 134 no. 3 (Mar. 1999), page 315-318.
Topik: CHH ; Cartilage-hair hypoplasia; CI ; Confidence interval; SIR ; Standardized incidence ratio

Ketersediaan

Perpustakaan FK Nomor Panggil: J45.K Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

Objective: 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. Study 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. Results: 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. Conclusions: This study confirms an increased risk of cancer, especially non-Hodgkin’s lymphoma, probably attributable to defective immunity, among patients with CHH.

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