Operative management of asphyxiating thoracic dystrophy after pectus repair.

Pectus excavatum repair usually results in unchanged or improved pulmonary function. However, a small subset of patients will experience severely impaired pulmonary function after pectus repair caused by restrictive lung disease, and no adequate surgical approach has been described for this condition. A procedure is described that is a variation of an operation for Jeune's thoracic dystrophy, that resulted in marked respiratory improvement in this setting.

Spontaneous resolution of myelodysplastic cytogenetic abnormality developed during the treatment of leukemia.

Purpose: We describe the spontaneous resolution of a myelodysplastic cytogenetic abnormality developing during the treatment of acute lymphocytic leukemia.

Patients And Methods: A 6-year-old girl with acute lymphocytic leukemia had a clinical picture of myelodysplasia 18 months after diagnosis. The clonal cytogenetic abnormality, 46,XX,del(5)(q12q12), resolved spontaneously 4 months after the discontinuation of chemotherapy.

Hepatocyte nuclear factor-4 prevents silencing of hepatocyte nuclear factor-1 expression in hepatoma x fibroblast cell hybrids.

Hepatocyte nuclear factors-1alpha (HNF1alpha) and -4 (HNF4) are components of a liver-enriched transcription activation pathway which is thought to play a critical role in hepatocyte-specific gene expression, including activation of alpha1-antitrypsin gene expression. HNF1alpha, HNF4 and alpha1-antitrypsin (alpha1AT) genes are extinguished in hepatoma/fibroblast somatic cell hybrids, suggesting that fibroblasts contain a repressor-like activity. To determine the molecular basis for silencing of these genes in cell hybrids, ectopic expression of HNF1alpha and HNF4 was used.

Sleep apnea in patients receiving growth hormone.

Among 145 patients treated with recombinant human growth hormone (GH), four developed sleep apnea (two obstructive, two mixed) associated with tonsillar and adenoidal hypertrophy in three. These four patients had no local risk factors predisposing to upper airway obstruction (i.e.

Surgical management of large breast tumors in the adolescent.

The management of adolescent females with giant cystosarcoma phylloides remains controversial. Since in this age group the overwhelming majority of these neoplasms are benign, aesthetic outcome is as important as adequate tumor resection. Traditional, treatment has been wide local excision, which may lead to substantial breast deformity.