Hypoventilation Syndrome Secondary to Club-Shaped Chest Wall Deformity.

Hypoventilation syndrome is defined as a decrease in alveolar ventilation leading to hypercapnia (PaCO2 > 35-45 mmHg) and hypoxemia. There are multiple causes of hypoventilation syndrome described in the literature, of which central and obesity-related causes are more prevalent. Other causes such as neuromuscular disorders and chest wall deformities are relatively less common. Multiple defects in the normal functioning of the respiratory function are implicated in the pathophysiological mechanism of hypoventilation syndrome, such as a hypoactive central ventilatory drive, decreased airway function, ventilation-perfusion mismatch, defective pulmonary mechanics, and respiratory muscle fatigue. Patients often present with dyspnea, headache, lethargy, repeated pulmonary infections, hypoxia that usually improves with low flow oxygen, and hypercapnia that may alter mental function. Nocturnal or diurnal assisted mechanical ventilation is proven to be an effective therapy for patients suffering hypoventilation syndromes. We describe a case of a 47-year-old woman with hypoventilation syndrome resulting from a rare chest wall deformity with inward protrusion of the costochondral junction of the ribs with ossification of the costal cartilage on CT who presented with dyspnea and hypercapnia.

Autologous hematopoietic stem cell transplantation in 48 patients with end-stage chronic liver diseases.

The only presently viable treatment for end-stage liver disease is whole organ transplantation. However, there are insufficient livers available. The aim of the present study is to provide autologous bone marrow-derived stem cells as a potential therapeutic for patients with end-stage cirrhosis. This is a retrospective chart review of autologous stem cell treatment in 48 patients, 36 with chronic end-stage hepatitis C-induced liver disease and 12 with end-stage autoimmune liver disease. For all patients, granulocyte colony-stimulating factor was administered to mobilize their hematopoietic stem cells. Following leukapheresis, CD34(+) stem cells were isolated, amplified, and partially differentiated in culture, then reinjected into each subject via their hepatic artery or portal vein. Treatment was generally well tolerated with the expected moderate but transient bone pain from G-CSF in less than half of the patients. Three patients had serious treatment-related complications, and only 20.8% of these end-stage liver disease patients died during 12 months of follow up. For all patients there was a statistically significant decrease in ascites. There was clinical and biochemical improvement in a large percentage of patients who received the transplantation. In the viral group, there were marked changes in albumin (p = 0.0003), bilirubin (p = 0.04), INR (p = 0.0003), and ALT levels (p = 0.02). In the autoimmune group, values also improved significantly for albumin (p = 0.001), bilirubin (p = 0.002), INR (p = .0005), and ALT levels (p = 0.003). These results suggest that autologous CD34(+) stem cell transplantation may be safely administered and appears to offer some therapeutic benefit to patients with both viral and autoimmune-induced end-stage liver disease.

Detection of simian virus 40 DNA sequences in Egyptian patients with different hematological malignancies.

SV40 DNA sequences have been detected in non-Hodgkin's lymphoma patients. A link between SV40 and NHL is biologically plausible since SV40 causes hematological malignancies in laboratory rodents. We investigated 266 Egyptian cases of hematological malignancies (158 NHL, 54 HD, 26 ALL, 13 AML, 8 CLL, 7 CML) and 34 subjects as a control for detection of SV40 DNA using nested PCR. SV40 DNA sequences were found in (53.8%) of NHL, (29.6%) of HD and in (40.7%) of different types of leukemia cases. Frequency of SV40 DNA sequences was higher in NHL patients compared with those with the other tumors and control group (p < 0.05). The highest frequency was in Burkitt's lymphoma followed by diffuse large B-cell lymphoma. The present study suggests that SV40 is significantly associated with non-Hodgkin's lymphoma and most probably acts as a cofactor in the pathogenesis of these tumors. This could lead to new diagnostic, therapeutic, and preventive approaches.