Roberts-SC syndrome, a rare syndrome and cleft palate repair.

Roberts SC syndrome is a rare syndrome with only 17 previously recognized patients reported in medical literature. The syndrome is characterized by multiple malformations, particularly, symmetrical limb reduction, craniofacial anomalies such as bilateral cleft lip and palate, micrognathia, and severe growth and mental retardation. Our patient, a young child of five years having Roberts-SC, was successfully operated for cleft palate under general anesthesia.

Lessons to be learned--Langerhans' cell histiocytosis.

Langerhans' cell histiocytosis (LCH) is a neoplastic disease due to uncontrolled proliferation of Langerhans' cells (LCs); damage to organs involved appears to be due to the various cytokines secreted. The true cause of LCH remains a mystery. LCH can present at any age; there is female predominance and the skin is often the first site to be involved.

Pre-existing histopathological changes in the cephalic vein of renal failure patients before arterio-venous fistula (AVF) construction.

Background: Native cephalic vein remains the superior dialysis conduit, even 30 years after it was first described. However, up to 37% of hemodialysis patients develop progressive stenosis in the venous circuit of arterio-venous fistula (AVF), which may later cause thrombosis and occlusion.

Material And Methods: To study the pre-existing morphological changes in the wall of the cephalic vein before AVF construction, we collected 23 cephalic vein specimens from 3 normal, young trauma patients and 20 renal failure patients.

Lessons to be learned: a case study approach. Malignant melanoma of soft tissue.

Malignant melanoma of soft tissue, also called clear cell sarcoma (CCS) of tendons and aponeuroses, presents as a soft tissue mass, usually in the lower extremity of young females. We report two cases of CCS of the upper extremity--in a Korean and a Saudi Arabian girl. Because of the presence of melanin, melanosomes, S-100 protein and the tendency for regional nodal metastases, it has been suggested that this entity be considered a melanoma rather than a soft tissue sarcoma.

Intimal changes in the cephalic vein of renal failure patients before arterio-venous fistula (AVF) construction.

Native cephalic vein remains the superior dialysis conduit, even thirty years after it was first described. However, up to 37% of hemodialysis patients develop progressive stenosis in the venous circuit of arterio-venous fistula (AVF), which may later cause thrombosis and occlusion. To study the pre-existing morphological changes in the wall of the cephalic vein before AVF construction, we collected 23 cephalic vein specimens from 3 normal, young trauma and twenty renal failure patients.