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"The most characteristic in clinical finding of thalassemia are anemia, facies Cooley, and enlargement of the spleen and liver. Osseous alterations occur in response to marrow overstimulation due to ineffective erythropoiesis these alterations result in changes of the skeletal architecture which is most typically reflected in the maxillofacial appearance of the patients. Uncontrolled maxillary overgrowth and procumebency of the anterior teeth make facial disfigurement (Asbell,1969).The therapy of thalassemia is focussed on treating the anemia by giving blood transfusions. It is believed that blood transfusion could prevent bone deformities (Karagiorga-Lagana 1988). Even though dentoskeletal deformity was found in many thalassemia children, not much attention has been given yet to the maxillofacial aspect. The effect but also in the alteration of mastication function. The condition of thalassemia children usually undernourish and anorexia caused by anemia. The dysfunction of mastication made the condition became worse. Therefore, it is necessary to prevent the deformity of dentoskeletal. The purpose of this study was to evaluate growth in thalassemia children.The result of this study revealed that dentoskeletal of thalassemia children in general had smaller size than normal, but not well proportion vertically caused by lower face. The skeletal profile was more convex than normal and indicated class II skeletal due to retruded mandible. The factor of age influenced all linear skeletal and dentoskeletal component, and only one angular skeletal components (SNB). the mean level of hemoglobin pretransfusion influenced the posterior cranial base, skelet of palatal, maxilla and posterior face of thalassemia children."

"ABSTRAKTujuan penelitian adalah mengidentifikasi anak talasemia tentang (1) karakteristik dental dan dentoskeletal, (2) pola deformitas dentoskeletal, (3) indikator sefalometri deformitas dentoskeletal, (4) faktor determinan yang berperan dalam deformitas dentoskeletal.Subjek penelitian meliputi 143 anak yang terdiri dari 74 anak talasemia b mayor dan 69 anak talasemia HbE, di Klinik Thalassemia Bagian IKA RSCM, Jakarta; usia 6-18 tahun, tidak dirawat orthodonsi, dari elnik Deutero Melayu,Tempat Penelitian adalah Klinik Kedokteran Gigi Anak dan Bagian Dental Radiologi FKG UI, serla Klirnik Thalassemia Bagian IKA R5CM, Jakarta, data dikumpulkan dari Februari-Agustus 1994.Pengukuran dilakukan terhadap (1) lengkung geligi pada model gips retaken gigi secara manual dengan kaliper digital dan dengan program komputer yang dilakukan di Department Pediatric Dentistry, Kyushu University, Fukuoka pada Agustus-September 1994, (2) sefalogram lateral dan PA dengan program komputer dilakukan pada bulan September-Oklober 1995 di Kyushu University Fukuoka.Hasil utama(1) Pertumbuhan gigi subjek talasemia lebih lambat daripada anak normal. Gambaran yang khas, yaitu susunan gigi renggang di rahang, diduga karena adanya pembesaran lengkung rahang atau karena red uksi ukuran gigi. Usia dental dan karakteristik dental antara kedua ripe talasemia tidak berbeda bermakna tetapi ukuran lengkung geligi subjek talasemia b mayor pada umumnya lebih besar daripada talasemia HbE. Retardasi panjang mandibula (Ar-Gn) pada talasemia b mayor lebih nyata daripada talasemia HbE dan posisi mandibula lebih retrognati (CS-N-B). Km-apemen garis dentoskeletal subjek perempuan pada umumnya lebih pendek daripada subjek laki-laki.(2) Pada subjek talasemia dijumpai kombinasi hubungan dental Kelas I dengan skeletal Kelas II. Posisi maksila (< S-N-A,