Mediastinal mass causing spinal cord compression.

A 4-year-old Cambodian male presented to the emergency room with 2 weeks of gradually increasing leg weakness until he could no longer stand. He was also reported to have a deformity on his back, intermittent fevers and cough. His physical exam was notable for a 2 cm × 1 cm bony protrusion at his T4 vertebrae, and 2/5 strength and positive Babinski reflexes in his lower extremities. A chest x-ray showed a 3.2 cm × 2.9 cm mass in the middle mediastinum extending to the posterior mediastinum. A purified protein derivative test was positive. A computed tomography scan showed findings consistent with pulmonary tuberculosis and a paravertebral mass with amorphous calcifications, which involved destruction of the T4-T5 vertebrae and evidence of cord compression. These findings were all consistent with tuberculous spondylitis (Pott's disease).

The prevalence and molecular basis of hemoglobinopathies in Cambodia.

Blood counts, hemoglobin (Hb) high performance liquid chromatography (HPLC), and DNA analyses were performed on 260 children, aged 5 months to 16 years, at Siem Reap to assess the prevalence of thalassemia and other hemoglobinopathies in regional Cambodia. Hemoglobinopathies were present in 134 children (51.5%) with 20 abnormal genotypes identified. alpha-Thalassemia (thal) (35.4%) was the most prevalent disorder and the -alpha3.7 gene deletion was the most common alpha-globin gene abnormality. The - -SEA deletion and nondeletional forms of alpha-thal, Hb Constant Spring [Hb CS, alpha142, Term-->Gln, TAA-->CAA (alpha2)], Hb Paksé [alpha142, Term-->Tyr, TAA-->TAT (alpha2)] and triplicated alpha genes, were also present but at low frequencies. Hb E [beta26(B8)Glu-->Lys, GAG-->AAG] (28.8%) was the most common beta-globin gene abnormality, whilst beta-thal was only detected in two children (0.8% of cases). Although hemoglobinopathies were common, the majority of abnormalities detected (heterozygous -alpha3.7 and Hb E) were not clinically significant. On the basis of these findings, and with the majority of abnormalities being mild, it seems improbable that thalassemia represents a major health burden in this region of Cambodia.