Epicardial mass causing cardiac compression: an unusual involvement in lymphomatoid granulomatosis

Lymphomatoid granulomatosis is a rare, diffuse, large B-cell lymphoma that is positive for Epstein-Barr virus. A multiorgan process, it manifests itself chiefly in the lungs but can also affect the skin, nervous system, and kidneys. Cardiac involvement and pericardial effusion are very unusual. We report the case of a 62-year-old man with lymphomatoid granulomatosis involving the heart and lungs. Diagnosis was confirmed with wedge biopsy at pericardiotomy, and the patient was treated with cyclophosphamide, prednisolone, and vincristine. Although the patient was still symptomatic at 6-month follow-up, he was in partial remission with improved functional capacity.     

A rare cause of refractory ascites in a child: familial Mediterranean fever

Familial Mediterranean fever (FMF) is hereditary episodic febrile syndrome characterized by acute attacks of fever and serosal inflammation, generally lasting 1–3 days and resolves spontaneously. Apart from abdominal pain, patients may present with variety of abdominal manifestations such as acute peritonitis, mechanical intestinal obstruction, diarrhea, bowel infarction, amyloidosis and small amounts of peritoneal fluid during the acute attacks. A 6-year-old boy was admitted with massive ascites. After extensive laboratory investigations, no causative agent could be identified. On subsequent days, he developed fever and skin eruptions. Acute-phase reactants were increased. A second tomography revealed cystic fluid collection near the anterior side of spleen that invades the stomach. An exploratory was performed and histopathological examination of the all resected specimens revealed mix inflammatory cell infiltrate associated with severe myofibroblast proliferation suggesting chronic inflammatory process on the mesenteric region. A diagnosis of FMF was suspected based on the clinical, laboratory and histopathological findings, and a trial of colchicine therapy initiated. Ascites and other serosal inflammations improved within 1 week without any recurrence during the next 12-month period on colchicine treatment. Atypical presentations of FMF have been increasingly reported. Pediatricians should keep FMF in mind in the differential diagnosis of massive ascites especially in regions where hereditary inflammatory disease are common.     

Granulomatous hepatitis after intravesical BCG treatment for bladder cancer.

Intravesical bacillus Calmette-Guerin (BCG) is used in patients with urinary bladder carcinoma. Although it is generally well tolerated, granulomatous hepatitis is a rare but serious complication. We report a 42-year-old man and a 56-year-old man who developed granulomatous hepatitis following intravesical BCG. One of them was treated successfully with antitubercular therapy; the other died because of BCG sepsis and multi-organ failure.     

Decreased ratio of CD4/CD8 lymphocytes might be predictive for successful interferon alpha and lamivudine combined therapy in childhood chronic hepatitis B infection: A preliminary study

BACKGROUND: In the development of chronic hepatitis with hepatitis B virus infection and in response to therapy, the immune status of the infected host plays a critical role. In this study, immunological variables were assessed in patients before interferon alpha and lamivudine therapy to determine if any pretreatment immunological parameter could be an indicator of response to therapy in childhood chronic hepatitis B infection. METHODS: Forty-four patients with chronic hepatitis B virus infection, aged 9.0 +/- 3.9 years, were enrolled in the study. The pretreatment clinical features, biochemical test results, histological activity indexes and immunological parameters were evaluated. All patients received interferon alpha for 6 months and lamivudine for 52 weeks. Four patients who could not be followed up were excluded from the study. The other 40 patients have been followed for a mean period of 27.5 +/- 9.7 months after therapy discontinuation. RESULTS: Seventeen patients showed loss of hepatitis B early antigen (HBeAg) with appearance of anti-HBe (42.5%) and six of those who responded also showed loss of hepatitis B surface antigen (HBsAg) with the presence of anti-HBs (15%). Except elevated aspartate aminotransferase levels, there was no significantly correlation between response and sex, age, pretreatment duration of disease and histological activity indexes. Pretreatment immunoglobulins (Ig), IgG subclasses, complement C3, C4 and secretory IgA levels were also not found to be significantly related to response. The evaluation of lymphocyte subsets showed that therapy responders had significantly reduced pretreatment ratios of CD4/CD8+ lymphocytes due to prominent increased percentages of CD8+ cells. The other cellular immunity parameters and some cell surface adhesion molecules were similar in both groups. CONCLUSION: This study emphasizes the importance of increased pretreatment CD8+ lymphocyte percentages leading to a significant decrease in CD4/CD8 ratio in chronic hepatitis B virus infection of childhood as an immunological factor predicting response to treatment.     

Failure to detect hepatitis B virus in vitreous by polymerase chain reaction

PURPOSE: To assay the vitreous of asymptomatic hepatitis B virus (HBV) carriers for the presence of HBV DNA using polymerase chain reaction (PCR). METHODS: Vitreous and serum specimens were collected from 13 carriers of HBV. The presence of HBV DNA was investigated by using PCR and Digene's Hybrid Capture System. The presence of hepatitis B surface antigen (HBsAg) in vitreous was also investigated by using the enzyme immunoassay (EIA). RESULTS: The serum was positive for HBV DNA in3 of the 13 asymptomatic carriers using PCR. Vitreous samples of all the patients, including 3 patients who were positive for HBV DNA in serum, were negative for HBV DNA with PCR and were negative for HBsAg with EIA. CONCLUSION: There is no evidence of HBV in the vitreous of asymptomatic HBV carriers.     

Surgical considerations and safety of cochlear implantation in otitis media with effusion

Objective

To evaluate the effects of otitis media with effusion on surgical parameters, patient safety, perioperative and postoperative complications.

Polymorphisms of the ICAM-1 Gene Are Associated with Biliary Atresia

Inflammation is an important feature of biliary atresia, and recent studies suggest that its occurs in a genetically susceptible host. The intercellular adhesion molecule-1 (ICAM-1) is of paramount importance for the initiation and propagation of various inflammatory conditions. AIM: To determine whether the Glu241Arg polymorphism in the ICAM-1 gene, which impairs inflammatory responses, is associated with biliary atresia. METHODS: Between February 2002 and November 2004, 19 patients (mean age 1 +/- 0.4 years) diagnosed as biliary atresia were included in the study. Thirty-eight children with chronic liver disease and a group of unrelated healthy controls (n = 123) included in this study. After informed consent, blood was collected and genomic DNA was obtained. Genotyping was performed by amplification-refractory mutation system polymerase chain reaction (ARMSPCR). Associations were assessed by using Fischer's exact test. RESULTS: ICAM G242R A allele frequency was significantly higher in the BA group than in both the CLD and healthy control groups (OR = 4.4, 95 CI% 1.3-15.1, P = 0.03 and OR = 4.8 CI% 1.5-15.6, P = 0.01, respectively). Univariate analysis showed that polymorphism of ICAM G241R polymorphism was significantly related to biliary atresia. There was not significant correlation between PELD score and ICAM-1 genotypes both in BA and CLD groups. CONCLUSION: These findings provide evidence for the possible role of ICAM-1 241R polymorphism in BA pathogenesis.