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.     

Comparison of clinical characteristics in good and poor insight obsessive-compulsive disorder

Beginning with DSM-III-R, the condition of an intact insight towards obsessive-compulsive symptoms, which was essential for the classical definition of obsessivecompulsive neurosis, has been removed, permitting inclusion of cases with poor insight. A total of 94 cases who met DSM-III-R criteria for obsessive-compulsive disorder were included in this study. The Structured Clinical Interview for DSM-III-R (SCID-P), YaleBrown Obsessive Compulsive Scale (Y-BOCS), Hamilton Rating Scale for Depression (HRSD), Hamilton Rating Scale for Anxiety (HRSA), and State-Trait Anxiety Inventory (STAI) were administered to each patient. Two subgroups determined by DSM-IV item "poor insight" were compared for demographic variables and the scores obtained on the scales. Scores on the Y-BOCS, HRSA, HRSD and STAI-state were significantly higher in the poor insight group. Current and past major depression were also more frequent. Among personality disorders (PDs), avoidant PD was more common in the good insight group and borderline and narcissistic PDs were more common in the poor insight group. HRSA, HRSD, and STAI-state scores had weak to moderate but significant correlations with insight as defined by the item 11 of Y-BOCS. Findings are discussed in view of previous reports.     

Impact of liver transplantation on renal function of patients with congenital hepatic fibrosis associated with autosomal recessive polycystic kidney disease

Congenital hepatic fibrosis (CHF) is an uncommon autosomal recessive malformation. It may be associated with extrahepatic manifestations such as polycystic kidney disease. The main consequence is portal hypertension and bleeding from varices. Despite liver transplantation as a therapeutic option for this patient, long-term impact of liver transplantation on renal functions of patients with autosomal recessive polycystic kidney disease with associated liver disease is not well known. In this study, we aimed to analyze the patient's renal function after liver transplantation by creatinine clearance, glomerular filtration rate, and renal resistive indexes. Between March 1997 and September 2002, three of 50 orthotopic liver transplantation (OLT) were performed because of CHF associated with ARPKD at Ege University Organ Transplantation and Research Center. Baseline immunosuppression consisted of prednisone and cyclosporine A (CSA). The mean follow-up of the patients was 2.1 yr. Blood urea and creatinine levels were decreased after operation in all patients and remained within the normal range at the sixth and 12th month, whereas the level of the third patient were increased at the 18th month. RRI values of patients were not found different at the sixth month whereas, RRI values of patients were decreased at the 12th month and remained unchanged at the 18th month of follow-up. During the study period hypertension developed in one patient at the 16th month and resolved with antihypertensive treatment and decreasing dosage of CSA. Kidney function has remained satisfactory in all of the patients despite the use of cyclosporine. OLT can provide good survival in patients with CHF associated with ARPKD.     

Current therapeutic approaches in childhood chronic hepatitis B infection: a multicenter study

BACKGROUND AND AIM: The aim of the present study was to compare the therapeutic efficacy of three different regimens in childhood chronic hepatitis B (CHB) infection. METHODS: A total of 182 children with CHB infection were prospectively allocated to three random groups. Sixty-two patients in the first group received high-dose interferon (IFN)-alpha 2b (10 MU/m2) thrice/weekly alone for 6 months. In the second (n = 60) and third groups (n = 60), IFN-alpha was used for 6 months (5 MU/m2) thrice/weekly in combination with lamivudine (LAM) (4 mg/kg, maximum 100 mg/day) for 12 months. Lamivudine was started simultaneously with IFN in the second group, while it was started 2 months prior to IFN injections in the third group. RESULTS: The initial mean alanine aminotransferase (ALT) values for the first, second and third groups were 109 +/- 93 IU/L, 101 +/- 64 IU/L and 92 +/- 42 IU/L, respectively (P > 0.05). At the end of the therapy, ALT values decreased to 82 +/- 111 IU/L, 38 +/- 41 IU/L and 29 +/- 16 IU/L in groups 1, 2 and 3, respectively. The mean ALT value of the first group was significantly different to the second and third groups (P = 0.046 and P = 0.002, respectively) at the end of the therapy and these differences were found to be sustained after 18 months. However, results in the second and third groups were similar (P > 0.05). There were no significant differences in HBeAg clearance and anti-HBe seroconversion at the initial stage, 12 months and 18 months between the three groups (P > 0.05). Hepatitis B virus (HBV) DNA clearance in the first group was different from the second and third groups, while the second and third groups had similar HBV DNA clearance ratios at 12 and 18 months. No significant difference was found in the complete response (normalization of ALT, clearance of HBV DNA and seroconversion of anti HBe) ratios of all groups (at 12 months: 28.8, 45.5, 35.8% and at 18 months 33.3, 49 and 34% in groups 1, 2 and 3, respectively, P > 0.05). CONCLUSIONS: Although the ALT normalization and HBV DNA clearance ratios of IFN plus LAM combination groups were better than the high-dose IFN-alpha monotherapy group, no significant difference was found in the complete response ratios of all three groups.     

Methods used for reconstruction in aggressive bone tumours: an early experience

Improvements in the overall treatment of patients with aggressive, large tumours involving the bone have made it possible to preserve and salvage limbs instead of amputating them. Each patient is unique in his clinical presentation and social circumstance. The different reconstructive options available allow us to choose the most appropriate method suited to the particular patient and with minimal delay, even when resources are limited. The patient and the relatives actively participate in the choice. The early experience of the different techniques for reconstructing these bone defects at our hospital are presented in this paper.     

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.