Good's syndrome and pure white cell aplasia complicated by cryptococcus infection: A case report and review of the literature.

Thymomas can present with a variety of paraneoplastic manifestations, mostly of autoimmune origin, including Good's syndrome when there is associated hypogammaglobulinemia. Although pure red cell aplasia is a recognised complication of thymoma, selective white cell aplasia is very rare, particularly in Good's syndrome. Lethal opportunistic infections are a feature of Good's syndrome, usually occurring in those patients with associated severe T lymphocyte defects. Although the cryptococcus is a recognised fungal pathogen in patients with other causes of CD4+ T cell lymphopenia, surprisingly this complication has not been reported in patients with Good's syndrome. We now describe a 70 year old man with Good's syndrome and pure white cell aplasia who presented with disseminated cryptococcosis, and provide an up-to-date review of the relevant literature. Despite meningeal involvement our patient recovered after combined treatment with intravenous globulin, granulocyte stimulating growth, corticosteroids and antifungal therapy.

Endocarditis due to Salmonella enterica subsp. arizonae in a patient with sickle cell disease: a case report and review of the literature.

Human cases due to Salmonella enterica subsp. arizonae are especially rare, but it may affect immunocompromised patients and infants. We present a case of endocarditis in a patient with sickle cell disease and a review of earlier cases caused by this rare human pathogen. The patient was successfully treated with ceftriaxone and ciprofloxacin. There are only few cases of salmonella endocarditis reported in the last six decades and it is the first case of Salmonella enterica subsp. arizonae endocarditis in the literature to the best of our knowledge.

Serum adiponectin in chronic hepatitis C and B.

Adiponectin possesses anti-inflammatory, insulin-sensitizing and anti-atherosclerotic properties. The aim of this study was to assess the levels of serum adiponectin in patients with chronic viral hepatitis C and B and correlate them with parameters exploring insulin resistance and indices of chronic liver disease. Seventy-two patients with chronic hepatitis C virus (HCV) infection and 73 patients with chronic hepatitis B virus (HBV) infection, matched for age and sex, were studied. All individuals were examined for serum concentrations of adiponectin, insulin, C-peptide and homeostasis model assessment for insulin resistance (HOMA-IR). Viral parameters and liver histology were also evaluated. Serum adiponectin levels were significantly higher in HCV compared with HBV-infected patients. Correlation analysis in the whole group demonstrated that serum adiponectin was positively correlated with aspartate aminotransferase, alkaline phosphatase, globulins, high-density lipoprotein cholesterol and staging score, while it was negatively correlated with body mass index, insulin, C-peptide and HOMA-IR. Logistic regression analysis identified type of infection (HCV vs HBV), alcohol consumption more than 25 g daily, serum total globulin and low C-peptide as significant predictive variables associated with high adiponectin levels. Higher levels of serum adiponectin in HCV compared with HBV patients could have a role in the slower disease progression of chronic HCV infection. In addition, alcohol intake more than 25 g daily seems to be a significant predictor for hyperadiponectinaemia in patients with chronic viral hepatitis C or B. Finally, in this study, a clear positive association between adiponectin and hepatic necroinflammation or staging score was not found.

Serum lipid pattern in chronic hepatitis C: histological and virological correlations.

Lipoproteins are closely connected to the process of hepatitis C virus (HCV) infection. The aim of this study was to evaluate the lipaemic profile in patients with chronic HCV infection, and to identify any association between serum lipid levels and viral load, HCV genotype or liver histology. Total cholesterol (TC), high-density lipoprotein-cholesterol (HDL-C), low-density lipoprotein-cholesterol (LDL-C) and triglycerides (TG) were measured in the sera of 155 patients with chronic HCV infection and 138 normal subjects, matched for age and sex. Viral parameters and liver histology were evaluated in HCV-infected patients. Serum TC (P < 0.0005), HDL-C (P < 0.0005) and LDL-C (P < 0.0005) were lower in chronic hepatitis C patients compared with controls. Grading score was positively correlated with TC and LDL-C. Patients with HCV genotype 3a had significantly lower levels of TC, HDL-C, LDL-C, higher viral load and higher frequency of hepatic steatosis than those with other genotypes. Logistic regression analysis identified genotype 3a (OR, 6.96; 95% CI, 2.17-22.32, P = 0.0011) as the only significant predictive variable associated with low serum cholesterol concentration. HCV infection is associated with clinically significant lower cholesterol levels (TC, LDL and HDL) when compared with those of normal subjects. This finding is more pronounced in patients infected with HCV genotype 3a. Further studies are necessary to define the pathophysiology of the relationship between lipid metabolism and HCV infection.

Virological, immunological and histological aspects in adult beta-thalassemic patients with chronic hepatitis C virus infection.

OBJECTIVES: Multitransfused adult beta-thalassemic patients constitute a population with high prevalence of hepatitis C virus (HCV) infection, because of transmission of HCV from infected blood donors prior to the introduction of anti-HCV screening. The aim of this study was to compare them with otherwise normal patients with HCV infection. METHODS: Forty-two adult multitransfused beta-thalassemics and 49 otherwise normal patients of the same age, with chronic HCV infection were studied. Viral parameters, autoimmunity indices and liver histology were evaluated. RESULTS: Serum HCV RNA levels were found significantly lower in thalassemic (median: 65,150 international units per milliliter (IU/ml); range: 3 059 380 IU/ml) than in non-thalassemic (NT) patients (median: 580,000 IU/ml; range: 10,956,000 IU/ml; P=0.001). The most prevalent genotype in thalassemic group was genotype 4 (32.4%) while in NT group was genotype 3a (59.2%). Cryoglobulins were detected in 8/42 (19%) thalassemic patients and in 12/49 (24.5%) NTs. Thalassemic patients had significantly lower levels of C3 and C4 components of complement and higher incidence of anti-nuclear antibodies than those without thalassemia. In patients with thalassemia a lower grading score was noted in liver biopsy compared with those without thalassemia (4.41+/-1.98 vs 5.38 +/- 2.09, P=0.038). On the contrary, thalassemic patients were found to have a higher staging score (3.08 +/- 1.51 vs 2.33 +/- 1.34, P=0.024). CONCLUSIONS: Adult beta-thalassemic patients, compared with other patients with HCV infection, present lower necroinflammatory activity and lower viral load but higher staging score. Autoimmune features are marginally different. Age of acquiring the infection, iron overload and modulation of immune system by transfusions are the proposed causes of these differences.

Keratoconjunctivitis sicca and chronic HCV infection.

BACKGROUND: The aim of this study was to determine the prevalence of keratoconjunctivitis sicca (KCS) in Greek patients with chronic hepatitis C virus (HCV) infection and its association with HCV genotypes and liver histology. PATIENTS AND METHODS: 93 HCVAb (+) patients underwent lacrimal function testing (Schirmer-1 test, break-up time test and Rose-Bengal staining test) and estimation of serum cryoglobulins and autoantibodies. 80 healthy volunteers were included in the study as controls. RESULTS: 34 out of 93 HCV patients (36.6%) and eight out of 80 healthy subjects (10%) had at least two abnormal lacrimal function tests suggestive of KCS (p < 0.001), cryoglobulinemia was evident in 20 patients (21.5%), rheumatoid factor (RF) in 43 (46.2%), antinuclear antibodies (ANA) in 19 (20.4%), antinuclear antigens (anti-SS-A and anti-SS-B) in one (1.1%) and two (2.2%) patients, respectively. Reduced prevalence of KCS was found in patients with genotype 3a compared to those with other genotypes (5/30, 16.7% vs 20/42, 47.6%, p = 0.007), probably because of their younger age. In patients with KCS a higher staging score was noted in liver biopsy compared to those without KCS (4.50 +/- 1.65 vs 3.06 +/- 1.88, p = 0.005). CONCLUSION: Greek patients with chronic HCV infection have a high prevalence of KCS (36.6%). The low frequency of anti-SS-A and anti-SS-B antibodies in these patients denotes different pathogenetic associations from primary Sjogren's syndrome.