Early detection of amyloidosis in renal allografts: electron microscopic, histochemical, immunohistochemical findings and relationship with graft survival.
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Biomedical subjects
Publications and source records attributed to B Demirhan.
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Hepatocyte growth factor (HGF) is a multipotent growth factor that is a powerful stimulator of DNA synthesis in a variety of cell types, especially hepatocytes. This factor is produced by nonparenchymal cells in the liver, presumably Kupffer cells, Ito cells, and sinusoidal endothelial cells. Research has shown that HGF is involved in tissue regeneration, wound healing, normal tissue growth, tumor progression, embryogenesis, and tumor invasion. Elevated serum levels of HGF have been documented in patients with acute renal failure and in renal transplant recipients; however, the importance of HGF in liver transplantation is unknown. The aim of this study was to determine whether induction of HGF expression is associated with acute rejection in liver transplants. We assessed the presence and density of HGF in hepatic allograft biopsy specimens compared with those from a control group of healthy patients.
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Sclerozing stromal tumor of the ovary is an extremely rare neoplasm occurring predominantly in the second and third decades of life. Most patients have menstrual irregularities and pelvic pain. Infertility and endometrial pathology have also been described. A 34-year-old woman presented with hirsutism and oligomenorrhea of three months duration. Ultrasound examination showed a heterogeneous right ovarian tumor consisting of predominantly solid tissue with several loculated cysts. On T2-weighted pelvic MR images, signal intensities of the cystic components were high and those of the solid components were heterogeneous, ranging from intermediate-high to high. Dynamic MRI marked early enhancement of solid components in the right ovary. The specimen obtained from endometrial curettage showed proliferative endometrium. Preoperative serum levels of tumor markers were in normal range: preoperative serum levels of testosterone (T) (2.42 ng/ml; normal for adult females 0.1-0.8 ng/ml) and dehydroepiandrosterone-sulphate (DHEA-S) (232.4 microg/dL; normal for adult female, 35-430 microg/dL) were measured and the T value was found increased. At laparotomy, a left ovarian mass was found attached to the right infundibulopelvic ligament and a left oophorectomy was performed. The mass was described as benign by frozen analysis. Definitive histopathological diagnosis was sclerozing stromal tumor of the ovary (SST). The histologic features included a pseudolobular pattern with focal areas of sclerosis and a two-cell population of spindled and polygonal cells. Immunohistochemical studies showed positive smooth muscle actin and negative cytokeratin, keratin, S100 and desmin. The T value decreased postoperatively (0.57 ng/ml).
Primary carcinoma of the fallopian tube is a very unusual gynecologic malignancy that accounts for less than 1% of all malignancies of the female genitalia. A 55-year-old, gravida 7, para 3 woman presented with no gynecologic complaints other than backache. TVS demonstrated a 35 x 25 mm heterogeneous mass that was not clearly separated from the left ovary, and another 31 x 14 mm cystic septated lesion in the left ovary region. Pelvic MRI demonstrated a 35 x 35 x 20 mm left adnexal mass that enhanced with contrast and a neighboring tubular-cystic mass. Upper and lower gastrointestinal endoscopy revealed no malignancy. Serum CA 125-level was merkedly elevated at 369 U/ml (normal < 35 U/ml). Laparotomy revealed left hydrosalpinx and a papillary-fimbrial mass. Pelvic lymph node metastases were observed. Frozen-section analysis identified the mass as a serous adenocarcinoma. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, appendectomy, omentectomy, pelvic and para-aortic lymph node dissection, and peritoneal washing were performed. The definitive histopathological diagnosis was primary serous adenocarcinoma of the fallopian tube with six of 25 lymph node biopsies showing metastasis. Six cycles of paclitaxel (175 mg/m2) plus cisplatin (75 mg/m2) combinatin chemotherapy were administered with 3-week intervals between cycles. Second-look laparotomy was performed; there was no evidence of disease. At the time of writing 12 months after the second-look laparotomy, she was still disease-free.
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Pachymeningitis is a rare complication of rheumatoid arthritis. The case of a 52-year-old male rheumatoid arthritis patient with pachymeningitis and optic neuritis who was successfully treated with intravenous cyclophosphamide is described.
UNLABELLED: Occurrence of extrarenal Wilms' tumour (WT) is very exceptional and the diagnosis is almost always made after surgical intervention. The tumour can be located in the retroperitoneum, uterus, cervix, testes, skin and even in the thorax. The exact mechanism whereby a WT occurs in extrarenal tissues is not known. The presence of ectopic metanephric blastema cells or the WT gene that cause transformation of extrarenal primitive mesonephric or pronephric remnants into WT are both considered in the embryogenesis. Although ultrasonography and CT scan are both helpful in the definition of retroperitoneal tumours, there is no characteristic finding to diagnose an extrarenal WT before surgery. However the histological characteristics are the same as in intrarenal WT, a retroperitoneal teratoma should be clearly investigated for a possible admixture of WT cells. Patients with extrarenal WT are given the same treatment protocol as patients with stage III WT. In this paper, a 5-year-old female patient with an extrarenal WT located in the lumbosacral region is presented. CONCLUSION: as a rule, diagnosis of extrarenal Wilms' tumour is made after surgery. Surgical excision is the treatment of choice and the same general therapeutic rules should be followed as when the kidney were affected. Stage III guidelines for chemotherapy and radiotherapy are appropriate in these patients.
Jeune syndrome is a rare autosomal recessive disease characterized by narrow thoracic cage and short-limbed dwarfism. Seventy percent of affected individuals die in early childhood from pulmonary hypoplasia and respiratory distress due to the small size of the thorax. Growth retardation and chronic renal insufficiency due to nephronophthisis may occur in patients who survive the respiratory failure. We report a family that exhibited clinically heterogeneous features of Jeune syndrome. The 6-year-old male proband presented with skeletal deformities and chronic renal failure. A kidney biopsy revealed that nephronophthisis was the cause of the patient's kidney failure, and we diagnosed Jeune syndrome. A retrospective diagnosis of Jeune syndrome was also established for the proband's older sister, who had died of renal failure at 8 years of age. The oldest female child in the family also had thoracic deformity, and the father and paternal uncle were both of short stature and exhibited brachydactyly. Their renal function and blood pressure were normal. The findings in this family are important in that they demonstrate the clinical heterogeneity of Jeune syndrome and underline the association of renal disease with this syndrome.
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OBJECTIVE: To assess the significance of atypical squamous cells of undetermined significance (ASCUS) in predicting the presence of underlying squamous intraepithelial lesion (SIL) of the uterine cervix, and to determine the best follow-up method for these patients. STUDY DESIGN: We reviewed 76 cases with cervical smears that had been labeled ASCUS over a 41-month period, 64 of which were also examined colposcopically. RESULTS: The 76 reported ASCUS cases represented 1.2% of all gynecologic cases diagnosed at our center during the same time period. Of the 64 patients who underwent colposcopy, 9 (14%) had low-grade SIL and 7 (11%) had high-grade SIL. CONCLUSION: Based on this study, ASCUS on a cervical smear is a good marker for detecting underlying SIL, and immediate colposcopy and directed biopsy are the most appropriate follow-up procedures.
Biliary atresia is defined as partial or total obliteration of the extra-hepatic bile ducts. In advanced cases, liver transplantation (LTx) is considered the most appropriate treatment. This report describes a female patient whose biliary atresia and subsequent cirrhosis required LTx at 1 yr of age. Macroscopic inspection of the hilar region of the native liver post-Tx revealed the formation of a pouch in the hepatic duct and a stone in the lumen of the portal vein. X-ray diffraction analysis showed that the stone was composed of cholesteryl cinnamate, gluconic acid phenylhydrazide, Na beta broma-allyl mercaptomethyl penicillinate, and Al2O3 crystals. While the cholesterol component is a known element of gallstones, we attributed the Na beta broma-allyl mercaptomethyl penicillinate to the patient's drug therapy. Our literature search revealed no previous record or crystallographic analysis of portal vein phlebolithiasis. In this report we describe this rare finding.