Dyspnea, pulmonary function and exercise capacity in adult Saudi patients with sickle cell disease.

OBJECTIVE: To examine pulmonary function, dyspnea and exercise capacity in adult Saudi sickle cell disease (SCD) patients. METHODS: The patients were recruited from the hematology clinic at King Khalid University Hospital in Riyadh from January to December 2005. The study involved 39 patients with stable SCD (20 women and 19 men), with a mean age of 22.7+/-7.1 years, hemoglobin level of 95.5+/-14.6 g/L and hemoglobin F level of 13.7+/-8.6%. Patients underwent pulmonary function tests (PFT) (forced expiratory volume in first second [FEV1], forced vital capacity [FVC], and diffusion capacity of carbon monoxide [DLco] data are presented as a percentage of the normal prediction), a 6-minute walk test (6MWT) and echocardiography. Dyspnea was assessed using the Borg score. The 6MWT data were compared to body mass index-matched healthy controls. RESULTS: Forty-one percent of SCD patients had mild dyspnea at rest, and this increased to 61% at the end of the 6MWT. Pulmonary function tests were abnormal in 51% (36% of patients had a restrictive pattern, 10% had isolated decrease in DLco, and 5% had a mixed restrictive-obstructive pattern). The 6MWD was shorter in SCD patients compared to the controls (368+/-67 versus 407+/-47m, p=0.005). No hematological variables correlated with outcome variables. CONCLUSION: Chronic pulmonary complications in adult Saudi SCD patients are relatively mild but common. Pulmonary function in these patients differs from that published for African-origin SCD patients. This difference may reflect a different natural history of SCD in the 2 populations.

Is serum alanine transaminase level a reliable marker of histological disease in chronic hepatitis C infection?

BACKGROUND: Approximately 20-30% of patients chronically infected with hepatitis C virus (HCV) have persistently normal alanine transaminase (PNALT) levels. These patients are described to have a mild degree of histological liver damage. We aimed to assess the histological liver changes in HCV patients with PNALT. PATIENTS AND METHODS: Sixty-five patients with HCV and PNALT (group A) underwent a liver biopsy. PNALT was defined as three or more determinations identified to be within the normal range over 6 months or longer. The demographical features and histological changes were compared with 66 consecutive patients with chronic HCV infection and elevated ALT (group B). All patients had a detectable HCV RNA. Histological disease was scored according to the METAVIR system. RESULTS: Females were more likely to have normal ALT levels (65%). The mean ALT level in Group A and B was 30 and 105 IU/L respectively. No patient in either group had normal histology. The mean necro-inflammatory scores in groups A and B (2.0+/-0.68 vs 2.09+/-0.67) and the mean fibrosis scores (2.11+/-0.87 vs 2.24+/-1.04) were not significantly different. Bridging fibrosis in groups A and B was seen in 24.6 and 37.9% patients, respectively, while cirrhosis was seen in 6.2 and 7.6% patients respectively. Hepatic steatosis in groups A and B (0.94+/-0.86 vs 1.0+/-1.02 respectively) was also not significantly different and did not show any association with the fibrosis scores across the two groups. In group A, the necro-inflammatory and fibrosis scores of patients with and without steatosis were not statistically significant. Age was the only predictor of normal ALT levels. However, increasing age did not show a significant increase in histological activity in either group beyond a certain age. CONCLUSION: This study demonstrates that ALT is a poor surrogate marker for inflammation and fibrosis in HCV patients. Given the presence of significant necro-inflammation in PNALT patients, the risk/benefit ratio justifies treatment without the need for a liver biopsy.

Six Minute Walk Test to assess functional capacity in chronic liver disease patients.

AIM: To examine the utility of Six Minute Walk Test (6MWT) in patients with chronic liver disease (CLD). METHODS: Two hundred and fifty subjects between the ages of 18 and 80 (mean 47) years performed 6MWT and the Six Minute Walk Distance (6MWD) was measured. RESULTS: The subjects were categorized into four groups. Group A (n = 45) healthy subjects (control); group B (n = 49) chronic hepatitis B patients; group C (n = 54) chronic hepatitis C patients; group D (n = 98) liver cirrhosis patients. The four groups differed in terms of 6MWDs (P < 0.001). The longest distance walked was 421 +/- 47 m by group A, then group B (390 +/- 53 m), group C (357 +/- 72 m) and group D (306 +/- 111 m). The 6MWD correlated with age (r = -0.482, P < 0.01), hemoglobin (r = 0.373, P < 0.001) and albumin (r = 0.311, P < 0.001) levels. The Child-Pugh classification was negatively correlated with the 6MWD in cirrhosis (group D) patients (r = -0.328, P < 0.01). At the end of a 12 mo follow-up period, 15 of the 98 cirrhosis patients had died from disease complications. The 6MWD for the surviving cirrhotic patients was longer than for non-survivors (317 +/- 101 vs 245 +/- 145 m, P = 0.021; 95% CI 11-132). The 6MWD was found to be an independent predictor of survival (P = 0.024). CONCLUSION: 6MWT is a useful tool for assessing physical function in CLD patients. We suggest that 6MWD may serve as a prognostic indicator in patients with liver cirrhosis.