Parenchymal calcification is associated with the neurological prognosis in patients with congenital rubella syndrome

Congenital rubella syndrome (CRS) results from maternal rubella virus infection in early pregnancy. Abnormal neuroimaging findings have been analyzed in a small number of CRS patients in the past; however, their clinical significance has been poorly addressed. Therefore, we have investigated the neuroimaging findings of 31 patients with CRS from previous studies. The most common finding was parenchymal calcification, which was observed in 18 of 31 patients (58.1%). A multivariable logistic regression model showed that it was associated with psychomotor or mental retardation (p = 0.018), suggesting that parenchymal calcification in CRS could be a prognostic factor.     

Health-related quality of life and pressure ulceration assessment in patients treated in the community

Little is known of the impact of pressure ulceration on adult patients' health-related quality of life. The purpose of this study was to determine the impact pressure ulceration has on pressure ulcer patients cared for in the community. A case control study design was used by drawing a random sample from patients receiving community nursing care, stratified by the presence of pressure ulceration. In all, 75 patients with pressure ulcers were compared with 100 controls without ulcers using the four-point ulcer grading scale described by United Kingdom consensus guidelines. Patients were interviewed using the Short Form-36 (SF-36) questionnaire and activities of daily living assessed using the modified Barthel scale. Patients with pressure ulcers had significantly poorer physical function (mean difference (d) = 37.6, 95% CI 28.6-46.6, p < 0.001) and social functioning (d = 33.9, 95 % CI 24.0-43.9, p < 0.001) than published age- and sex-matched normative data from the United Kingdom. The difference between cases and controls was much smaller in these domains, with neither approaching statistical significance. After adjustment for age and gender, scores for bodily pain were poorer in patients with no ulceration (d = -10.5, 95% CI - 20.6 to - 0.4, p = 0.042) indicating greater pain in these patients compared with the cases with ulceration. Activities of daily living determined by the modified Barthel scale showed reduced self-care (d = -7.6, 95% CI -12.5 to - 2.7, p = 0.010) and mobility (d = -9.2, 95% CI -14.6 to - 3.8, p = 0.001) in patients with pressure ulceration. The overall ability to perform these activities was also significantly poorer in this group (d = -16.3, 95% CI -27.3 to -5.3, p = 0.004). While patients with pressure ulceration experience some deficits in their health-related quality of life compared with a normal population, these differences are similar to those experienced by other patients receiving community nursing care.     

Serum cytokine profiles associated with clinical presentation in Vietnamese infected with hepatitis B virus.

BACKGROUND/OBJECTIVES: An ineffective cytokine response is thought to be one of the reasons for the failure to suppress hepatitis B virus (HBV) replication and to eliminate the virus. We investigated the serum levels of interleukin (IL)-6, IL-10, IL-12, and interferon (IFN)-gamma in HBV-infected Vietnamese patients to determine whether they were related to the outcome of HBV infection. STUDY DESIGN: Samples from a total of 154 HBV-infected patients with well-characterised clinical profiles and 56 healthy controls were assessed. RESULTS: Serum IL-6 levels, which were inversely correlated with transaminase levels, were highest in patients with liver cirrhosis (LC) and hepatocellular carcinoma (HCC) and lowest in those with either asymptomatic (ASYM), acute or chronic HBV, and thus, represented the best marker of HBV-related clinical progression. Compared with the healthy control group, serum IL-12 was uniformly elevated in all HBV-infected patients apart from those with ASYM infections, implying no impairment of production of this cytokine in HBV-infected individuals. Serum IL-10 and IFN-gamma levels, however, were uniformly low and showed no association with clinical presentation. Cytokine profiles were not influenced by the presence of hepatitis B e antigen (HbeAg). CONCLUSIONS: Serum IL-6 and IL-12 but not IL-10 and IFN-gamma are associated with the clinical presentation in HBV-infected Vietnamese patients.     

Laryngeal and velopharyngeal sensory impairment in obstructive sleep apnea

STUDY OBJECTIVE: To determine whether mucosal sensory dysfunction is present at multiple upper-airway sites in patients with obstructive sleep apnea (OSA). DESIGN: Physiologic testing of consecutive patients with OSA and nonsnoring controls. SETTING: University hospital sleep center. PARTICIPANTS: Thirty-nine subjects with OSA and 17 controls. INTERVENTIONS: Endoscopic testing was used to determine sensory detection thresholds for air-pressure pulses delivered to the oropharynx, velopharynx, hypopharynx, and larynx (aryepiglottic eminence). The air-pulse stimulus intensity required to elicit the protective laryngeal adductor reflex was also determined. MEASUREMENTS AND RESULTS: There was a significant impairment in sensory detection threshold for OSA versus control subjects in the oropharynx, as previously described by ourselves using other techniques, as well as at the velopharynx (median 11 mm Hg [confidence interval 9-11] for subjects with OSA vs 8 mm Hg [confidence interval 4-11] for controls, P = .03) and, at the larynx, 4 mm Hg [confidence interval 2-9] for subjects with OSA vs 2 mm Hg [confidence interval 2-3] for controls, P < .001). The threshold stimulus intensity for the laryngeal adductor reflex was also significantly higher for OSA subjects. For OSA patients with abnormal laryngeal sensation (61% of OSA subjects), there were significant correlations between laryngeal sensory values and measures of apnea severity, including apnea-hypopnea index (r = 0.82, P < .001) and nadir SaO2 (r = -0.48, P < .05). CONCLUSION: Mucosal sensory function is impaired at multiple upper-airway sites in OSA.     

Three novel PROC gene lesions causing protein C deficiency

Hallam PJ, Mannucci P, Tripodi A, Bevan D, Laursen B, Tengborn L, Wacey A, Cooper DN. Three novel PROC gene lesions causing protein C deficiency. Clin Genet 1998: 54: 231–233. 0 Munksgaard, 1998
Missense mutations. three of them novel (Am210→Val, Asn248→ Ile, Ah355→Val), were found in the protein c ( PROC ) genes of 7 patients with inherited protein C deficiency associated with venous thrombosis. Comparison with the phenotypic effects of mutations in the analogous residues of factor IX causing hdernophilia B and the use of molecular modelling has provided explanations as to how these lesions might alter either the structure, function or secretion of the protein C molecules encoded.