Hodgkin disease in children: clinicoepidemiologic and viral (Epstein-Barr virus) analyses.
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Publications and source records attributed to O Uluoglu.
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The tumor suppressor gene p53 is known to be involved in the negative regulation of cell growth. Proliferating cell nuclear antigen (PCNA), which is a nuclear protein and a component of the DNA replication process, is also involved in growth regulation. Both have been studied as progression markers in various tumors including hepatocellular carcinoma. In the present study, the aberrant p53 protein and PCNA expressions in non-tumoral liver diseases were investigated. Using monoclonal antibodies anti-p53 (D07-DAKO) and anti-PCNA (PC10-DAKO), 149 samples were stained, including 10 normal and 10 tumoral control liver tissues. p53 Overexpression was detected in 52 specimens (35%) whereas PCNA positivity was found in 96 (64%). There were 21 different pathological entities but most of the positive samples could be grouped into four types of diseases; namely, non-specific reactive hepatitis, steatohepatitis, chronic hepatitis and cirrhosis. Statistical analyses performed on these groups revealed that p53 positivity was found to be significantly higher in steatohepatitis (P < 0.05), while PCNA positivity did not show any statistical significance. The number of samples showing both p53 and PCNA positivity was 42 but their coexistence was not found to be significant. Certain cytological alterations like nuclear pleomorphism, steatosis and cholestasis, in addition to necroinflammatory activity, were evaluated for their possible impact on p53 and/or PCNA positivity. Necroinflammatory activity in steatohepatitis and steatosis in chronic hepatitis was found to be significant for p53 positivity (P < 0.05). In contrast, nuclear pleomorphism in non-specific reactive hepatitis was found to be significant for PCNA positivity (P < 0.05).
Calcium hydroxyapatite (HA) ceramics are widely used in orthopaedic surgery and are known to be biocompatible, biodegradable, and osteoinductive. Recent studies of HA have identified early stage inflammatory reactions and third-body wear following hip replacement surgery. The aim of this study was to investigate the early stage inflammatory response toward HA by means of conventional radiography, histopathology, and scanning electron microscopy (SEM). HA was implanted into the medullary area of the tibiae of rabbits and followed from one week to six months. Radiography revealed local osteoporosis on Week 6 and Month 6 in some, but not, all rabbits. SEM presented favorable bonding between the implant and the cortical bone. Eosinophilic deposition, non-specific local inflammation, and bone marrow depletion were the histopathological findings. Multinuclear cells were identified in the inflammation area. Although bone ingrowth into the pores of the HA was demonstrated, there is no doubt that HA may cause an early inflammatory reaction in the bone marrow. This inflammatory reaction is followed by bone marrow depletion and may be the early sign and the cause of HA-coated implant failure.
Burkitt's lymphoma (BL) in Turkish children is commonly associated with Epstein-Barr virus (EBV) infection. The C-terminus of the latent membrane protein 1 (LMP-1) of EBV is essential for transformation and the 30-bp deletion detected in this region has been implicated to be associated with a more aggressive malignant phenotype. To understand the molecular mechanisms underlying EBV pathogenesis in BL of Turkish children, we analyzed 30-bp deletion and 33-bp variable repeat regions of the LMP-1 gene from paraffin-embedded tumor tissues of 30 BL patients (mean age 5.9 years). Primer pairs spanning the 30-bp deletion and 33-bp repeat regions were designed for amplification by polymerase chain reaction (PCR). The PCR-amplified products were analyzed by gel electrophoresis, Southern blot hybridization, and DNA sequencing. Twenty-eight (93%) of 30 BL biopsy samples were EBV positive as determined by PCR. Variable copy numbers (ranging from 4.5 to 7) of the 33-bp repeat of LMP-1 gene were detected in these EBV-containing tumor samples. To determine the frequency of the 30-bp deletion of the LMP-1 gene, we sequenced the amplimers encompassing this region. Analyses of DNA sequence of 28 Turkish BLs have disclosed four patterns: the first (32% (9/28)) is identical to B95-8 with no deletion, the second (11% (3/28)) is identical to Asian NPC CAO strain with 30-bp deletion, the third (46% (13/28)) is prevalent in Turkish BLs with a longer deletion (69 bp), and the fourth (11% (3/28)) consists of a mixture of 30-bp and 69-bp deletion. The occurrence of high frequency of the 69-bp deletion appears to have no correlation with the disease site. Mutations found in the CAO strain were also detected in the Turkish BL clustering at the amino acids 322, 334, 338 and 342; whereas mutations specific for Turkish BL were clustered at amino acids 326, 352 and 361. To assess the EBV genotype with the changes in C-terminus of LMP-1 gene, we performed genotyping by PCR to differentiate type A and B strain. All 28 patients were infected by type A EBV. Such a high frequency of the larger size (69 bp) deletion has never been reported. Ascertaining the role of this deletion in BL pathogenesis will require further study.
The concept of endotoxin-mediated rather than direct liver injury in biliary obstruction was investigated using the experimental rat model of bile duct ligation (BDL) and small bowel bacterial overgrowth (SBBO). Small identical doses of intravenous endotoxin (bacterial LPS) caused a significantly more severe liver injury in rats with BDL, compared with sham-operated rats, suggesting the possible contribution of LPS in this type of liver damage. BDL was then combined with surgically created jejunal self-filling blind loops, which resulted in SBBO. Plasma LPS level increased significantly, and once again a more severe liver injury, determined by liver histology and serum gamma-glutamyl transpeptidase levels, was observed compared with the control group of rats with BDL+self-emptying blind loops. The data presented suggest that small amounts of exogenous LPS and/or the ordinarily innocous amounts of LPS constantly absorbed from the intestinal tract may be critical in the hepatic damage caused by obstruction of the biliary tract.
The effects of Tween 80, a surface active agent which is frequently used for dispersion of water-insoluble substances, on endothelial function in rabbit aorta, were investigated in a cascade superfusion bioassay system by using a deendothelized precontracted rat aorta ring as a bioassay tissue. Prior incubation of rabbit thoracic aorta segments with higher concentrations of Tween 80, caused total inhibition of acetylcholine-induced endothelium derived relaxing factor (EDRF) release, while moderate concentrations had no significant effect. Perfusion of the donor aortae segments with 10 (-1) - 10 (-3) ml/1 Tween 80 also inhibited the EDRF release from donor aorta dose-dependently. Histopathological examination of the vasculary wall and endothelial structures revealed a significant desquamation of vasculary endothelium with the highest concentration of Tween 80 used in this study. These data suggest that the mechanism underlying the reduction in the release of EDRF from donor aorta segments may be the destruction by Tween 80 of endothelial lining.
The authors present an infant with left ventricular and mitral valve thrombi diagnosed by cross-sectional echocardiography. Thrombosis was due to acquired transient protein C deficiency, which was caused by impaired liver function due to sepsis. Because the thombi were very large and mobile, urgent surgery was performed. Eight weeks later, the patient's protein C level returned to normal ranges. The authors suggest that in all cases with intracardiac thrombosis, protein C deficiency should be investigated.
Eighty-one Turkish children with Burkitt's lymphoma (BL) were observed during a period of 24 years (1968-1992). The diagnosis was established histologically according to WHO criteria. BL represented 48.5% of NHL in this series. The median age of patients was 5 years with a sex (M/F) ratio of 2.3/1. The most common primary site of tumor involvement at initial presentation was the abdomen (70.4%), which was followed by facial tumors, in particular the jaw and orbit (45.7%). The majority of the patients (84.0%) were in advanced stages (C and D) at initial diagnosis. Facial tumors observed in Turkish children with BL were more similar to African Burkitt's lymphoma than American or European cases. High titers of antibodies against VCA and EA of EBV were also observed in 32 recent cases of BL. Preliminary molecular and immunologic studies revealed EBV-DNA (type I) and T cell deficiency. The clinical presentation, median age, and association with EBV revealed that BL appears to be inbetween African and non-African types in Turkish children. This will be further elucidated in the future by direct examination of tumor cells for EBV and investigation of the molecular characteristics in these cases.
Primary malignant tumours of the great saphenous vein are extremely rare with only 15 cases reported in the English literature since 1919. We report a patient who survived 5.5 years after treatment with surgery and chemotherapy before dying from lung metastases.
The rare hungry bone syndrome was encountered in a 15-year-old child after the removal of a parathyroid adenoma. Contrary to the hypocalcemias caused by the removal of all parathyroid glands or transient ischemia after parathyroid surgery, in which the serum inorganic phosphorus level is usually normal, both serum calcium and inorganic phosphorus levels are decreased in hungry bone syndrome in the early postoperative period. Vigorous calcium supplementation and vitamin D are required for prolonged periods.
A case of congenital dermoid cyst in the floor of the mouth in a newborn is presented. The presence of microcyst formation with intestinal-type epithelial lining and mucin histochemistry are discussed.
Metastases from basal cell carcinoma (BCC) are very rare. Some clinical and histologic features have been demonstrated in these cases without corroborative evidence. Treatment of these cases has been disappointing. In this article, we report a patient with metastatic BCC who was treated successfully by surgical resection of lung metastases.
Tricuspid regurgitation (TR) due to chordal rupture is a rare condition in newborns. We present a newborn with critical TR. Cross-sectional echocardiography demonstrated a dense, mobile mass localized at the tricuspid valve and resembling an atrial thrombus or myxoma. At operation, ruptured chordae tendineae of the tricuspid valve were found, forming a tangled mass. Histopathologic examination revealed collagen degeneration and calcification in the ruptured chordae, producing the hyperechoic image. It should be emphasized that ruptured chordae of the tricuspid valve might mimic a tricuspid valve myxoma or a thrombus on cross-sectional echocardiography.
Pachydermoperiostosis in three males of seven siblings from a village of mid-Anatolia is presented. Although X-linkage could not have been ruled out, the autosomal recessive inheritance was considered most likely. It differs from the autosomal dominant form by growth retardation, early ulcers, and acrolysis of the distal parts of the extremities with secondary contractures.
Retroperitoneal teratomas are rare in adults. These tumours may be benign or malignant and are composed of multiple tissues foreign to the part in which they arise. We report a malignant teratoma of retroperitoneal space. In searching the literature, we have been able to find 39 cases reported since 1937. Of the forty cases reported (including this case), only ten (25%) were malignant.