1/42. Nocardia thyroiditis: unusual location of infection.nocardia asteroides complex is an important opportunistic agent in immunocompromised hosts. Usually, primary pulmonary infection occurs and is followed by dissemination of the pathogen to the central nervous system and soft tissues. As described in the literature, almost every organ can be infected, but to our knowledge, Nocardia has been described as a pathogen responsible for thyroid abscess in only one report, which was published in 1993. The present report is the second case report of Nocardia thyroiditis. The patient was under immunosuppressor treatment following a combined liver-kidney transplant and presented with a preexisting nodular goiter which was probably a predisposing factor to the start and development of the thyroid infection.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/42. adenoma of the ampulla of vater: a genetic condition?The etiology of adenoma of the ampulla of vater is not well understood. Previous authors reported the association of this neoplasm with polycystic kidney disease of two fraternal sisters. They concluded that these two conditions were somehow related. We describe a case of ampullary adenoma associated with polycystic kidney disease. This presentation raises again the question of a possible link between these two diseases.- - - - - - - - - - ranking = 0.817894140625keywords = neoplasm (Clic here for more details about this article) |
3/42. Bilateral nephrectomy, peritoneal dialysis and subsequent cadaveric renal transplantation for treatment of renal failure due to polycystic kidney disease requiring continuous ventilation.We report here on a newborn with end-stage renal failure due to autosomal recessive polycystic kidney disease, also causing ventilation-requiring respiratory distress. peritoneal dialysis was able to keep the newborn alive but not wean it from the respirator. After removal of both huge kidneys, dialysis became more effective and allowed the neonate to be extubated only 5 days later. It was decided to register the baby for a pediatric cadaveric kidney transplant when it reached 6 kg/body wt or to perform a living related transplant if no such kidney became available and the baby grew to 7 kg/body wt. At the age of 9 months and a weight of 6 kg a cadaveric kidney from a 20-month-old donor became available and was transplanted extraperitoneally. Prophylactic immunosuppression included cyclosporin, mycophenolate mofetil and steroids. pneumonia on post-operative day 10 required respiratory care for several days and acute rejection requiring peritoneal dialysis. Both complications were controlled with antibiotics and conversion from cyclosporin to tacrolimus and a temporary increase in steroids. Thirteen months later the child is alive and well with a serum creatinine of 0.6 mg%. From this experience we would recommend early removal of both polycystic kidneys causing end-stage renal failure and respiratory insufficiency, starting peritoneal dialysis and performing a renal transplant as soon as possible. This therapeutic strategy seems appropriate for this complex situation.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/42. An unbalanced submicroscopic translocation t(8;16)(q24.3;p13.3)pat associated with tuberous sclerosis complex, adult polycystic kidney disease, and hypomelanosis of Ito.We report on a familial submicroscopic translocation involving chromosomes 8 and 16. The proband of the family had a clinical picture suggestive of a large deletion in the chromosome 16p13.3 area, as he was affected with tuberous sclerosis complex (TSC) and had alpha thalassaemia trait, and his half brother, who also had TSC, may have suffered additionally from polycystic kidney disease (PKD). FISH studies provided evidence for a familial translocation t(8;16)(q24.3;p13.3) with an unbalanced form in the proband and a balanced form in the father and in a paternal aunt. The unbalanced translocation caused the index patient to be deleted for the chromosome 16p13.3-pter region, with the most proximal breakpoint described to date for terminal 16p deletions. In addition, FISH analysis showed a duplication for the distal 8q region. Since the index patient also had hypomelanosis of Ito (HI), either of the chromosomal areas involved in the translocation may be a candidate region for an HI determining gene. Furthermore, it is noteworthy that both carriers of the balanced translocation showed a nodular goitre, while the proband has hypothyroidism.- - - - - - - - - - ranking = 5keywords = complex (Clic here for more details about this article) |
5/42. focal nodular hyperplasia of the liver associated with portal vein agenesis: a morphological and immunohistochemical study of one case and review of the literature.Congenital absence of portal vein (CAPV) is a rare malformation. To our knowledge, sixteen cases are reported in western literature. All the cases are associated with other diseases, cardiac malformations (12/17 patients; 16 plus the present case) and hepatic neoplasms being the most frequent observations. We present the case of a girl with a complex malformative syndrome consisting of multicystic kidney dysplasia, CAPV and nodular tumor-like mass of the liver. angiography showed that the splenic vein and superior mesenteric vein joined to form a common trunk directly entering the inferior vena cava above the liver. A review of the CAPV cases of the literature and the clinical and pathological features of the hepatic lesion, classified as focal nodular hyperplasia (FNH), are extensively discussed.- - - - - - - - - - ranking = 1.817894140625keywords = complex, neoplasm (Clic here for more details about this article) |
6/42. Clinics in diagnostic imaging (46). Renal cell carcinoma in acquired cystic kidney disease.Acquired cystic disease of the kidney and renal cell carcinoma are associated with chronic renal failure. In recent years, there has been increased incidental detection of renal tumours through the liberal use of ultrasonography (US) and computed tomography (CT). A 40-year-old man suffering from chronic renal failure and who was being treated with haemodialysis for six years, was found to have a complex cystic lesion on US and CT. nephrectomy was performed and the lesion was confirmed to be renal cell carcinoma associated with acquired cystic kidney disease. The current role of imaging in the management of affected patients are discussed.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
7/42. Acute promyelocytic leukemia with additional chromosome abnormalities in a renal transplant case.Some cases of acute myeloid leukemia following organ transplant (PT-AML) have been published in the literature. We report the second case of acute promyelocytic leukemia (APL), which developed post-transplant and immunosuppressive treatment, in a 50-year-old male who had undergone a renal transplant. At diagnosis he presented typical t(15;17)(q12;q13) with additional abnormalities, including 8,t(13;22)(q12;q13) and an abnormal chromosome 1 which was better characterized by fluorescence in situ hybridization (FISH). He obtained cytological, karyotypic and molecular complete remission (CR) with induction treatment according to the all-trans retinoic acid idarubican (AIDA) protocol; after 12 months, he relapsed (molecular relapse) and achieved molecular remission with all-trans retinoic acid (ATRA) plus mitoxantrone and cytosine arabinoside. After a further 14 months, he was treated with arsenic trioxide for cytological relapse and obtained a third CR; at the cytological relapse the karyotype showed 47,XY, 8, t(15;17)(q22;q21),t(13;22)(q12;q13),der(22)t(1;22)(p22;q13). He is alive 3.3 years after diagnosis of APL. Cyclosporin A (CsA) was given during all cycles of chemotherapy. We did not observe any severe infections or kidney failure during treatments. The use of conventional cytogenetic analysis plus FISH may identify complex karyotype also in transplanted patients receiving immunotherapy, and may also contribute to a better assessment of PT-AL.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
8/42. Spiradenocylindroma of the kidney: clinical and genetic findings suggesting a role of somatic mutation of the CYLD1 gene in the oncogenesis of an unusual renal neoplasm.We describe the morphology and comparative genomic hybridization findings in a tumor for which we propose the term "spiradenocylindroma" of the kidney. The tumor arose in the wall of a renal cyst in an otherwise healthy male patient who had a favorable clinical course after nephrectomy. Tumor cells formed either large nodules exhibiting a solid or trabecular architecture with conspicuous perivascular spaces or cylindromatous small tumor cell islands arranged in a jigsaw pattern. Focally, there were interspersed tubular structures and tumor cell rosettes with central deposits of periodic acid-Schiff-positive material. A minor tumor component showed epidermoid differentiation. The tumor cells were strongly positive for cytokeratins 5/6, high molecular weight cytokeratins 34betaE12 and AE1/3, and E-cadherin, but only weakly positive for cytokeratins 7, 8, 18, 19, and epithelial membrane antigen. Focal reactivity for actin, vimentin, and S-100 protein or lysozyme and alpha 1 -antichymotrypsin within tubular and cylindromatous areas suggested myoepithelial and apocrine differentiation, respectively. By comparative genomic hybridization, the only abnormality was loss of the long arm of chromosome 16 and gain of genetic material on the short arm of chromosome 16, suggesting isochromosome i(16p). This finding is unique among renal neoplasms and implies loss of heterozygosity at 16q12-13 of the CYLD1 gene that is critically involved in the oncogenesis of familial cylindromatosis and some sporadic spiradenocylindromas. We conclude that somatic mutation of the CYLD1 gene outside the skin can have a role in the oncogenesis of tumors with cylindromatous features.- - - - - - - - - - ranking = 4.089470703125keywords = neoplasm (Clic here for more details about this article) |
9/42. Papillonodular type of cystic partially differentiated nephroblastoma: a case report.We report a case of the papillonodular type of cystic partially differentiated nephroblastoma (CPDN), an extremely rare renal neoplasm that occurs in newborns and infants. The papillonodular type of CPDN is a variant of the conventional form of CPDN. MRI clearly demonstrated the gross pathologic features, distinguishing it from other renal multilocular cystic tumors.- - - - - - - - - - ranking = 0.817894140625keywords = neoplasm (Clic here for more details about this article) |
10/42. Percutaneous antegrade pyelography in small infants and neonates.Percutaneous antegrade pyelography is a safe and useful alternative to retrograde pyelography in the investigation of urinary-tract malformations in the neonate or very young infant. It is preferred to arteriography in the infant with a loin mass. Satisfactory delineation of hydronephrosis or cysts is simply and directly accomplished by this method, so that more complex and less definitive investigations can often be avoided. In addition, a variety of ureteric abnormalities may be displayed.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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