Aortic stent-grafts: Endoleak surveillance.

Endoleaks have been referred to as the "Achilles heel" of endovascular aortic aneurysm repair (EVAR) and are the most common complication of this procedure. An endoleak can maintain a high systemic blood pressure within the aneurysm sac, potentially leading to rupture. Follow-up is therefore mandatory to detect and classify possible endoleaks. Computed tomography (CT) remains the gold standard for follow-up, but provides no hemodynamic information on endoleaks and has the disadvantages of exposing patients to iodine contrast and X-ray radiation. Exposure to radiation could be reduced in various ways, by simplifying the triphasic protocol using dual-energy CT imaging, limiting the amount of radiation per slice using iterative reconstruction, and reducing the follow-up schedule that could be altered to include non-ionizing radiation imaging techniques. Contrast-enhanced ultrasound (CEUS) is an interesting alternative to CT, as is magnetic resonance (MR) imaging that can be used as an alternative or for complementary imaging. Long-term follow-up schedules are currently based on repeated CT. However, more recently alternative follow-up protocols have been proposed for patients with no endoleaks nor increase in aneurysmal sac size. These new protocols consist of CT imaging at 1month and 1year after treatment, subsequently followed by CEUS. Nevertheless, the mechanical structure of the stent-graft must still be verified by CT. The use of patient-specific risk-adjusted follow-up protocols, based on preoperative imaging and the first postoperative results, is gradually becoming more and more widespread.

GM2 gangliosidosis in an adult pet rabbit.

A 1.5-year-old neutered male rabbit was presented with chronic nasal discharge and ataxia. Rapid progression of neurological signs was noted subsequent to general anaesthesia and the rabbit was humanely destroyed due to the poor prognosis. At necropsy examination there were no gross changes affecting the brain or spinal cord. Microscopical examination revealed that the perikarya of numerous neurons in the brain and spinal cord were distended by the intracytoplasmic accumulation of pale, finely granular to vacuolar material. Transmission electron microscopy showed this to be composed of concentric membranous cytoplasmic bodies. Thin layer chromatography revealed elevation of GM2 ganglioside in the brain of this rabbit compared with that of an unaffected control rabbit. Enzymatically, there was markedly reduced activity of tissue ß-hexosaminidase A in brain and liver tissue from the rabbit. This was a result of an almost complete absence of the enzymatic activity of the α-subunit of that enzyme. These findings are consistent with sphingolipidosis comparable with human GM2 gangliosidosis variant B1.

Hypersplenism due to portal hypertension: retrospective evaluation of 17 patients treated by splenic embolization.

PURPOSE: The objective of this retrospective study was to analyze the efficacy and morbidity associated with splenic artery embolization for hypersplenism due to portal hypertension (PHT), as a function of the volume of the splenic parenchyma embolized and the type of PHT (due to intrahepatic block or segmental PHT). PATIENTS AND METHODS: This study retrospectively included 17 patients with hypersplenism secondary to PHT (intrahepatic block, n=14; segmental, n=3) treated by splenic artery embolization. The splenic volume embolized was estimated by computed tomography (CT) one month after embolization. A clinical assessment and platelet count took place at 7 days, 1 month and 6 months after the embolization. RESULTS: In the group with PHT due to intrahepatic block, the mean volume of embolized splenic parenchyma was 63% of the initial volume (range: 30-95%). Six months later, the platelet level had increased by an average of 232%. All patients with fewer than 80,000 platelets/mL at 6 months had an embolization volume less than 50%. In the segmental PHT group, the mean volume of the embolized parenchyma was 62% of the initial volume (range: 20-95%), bleeding symptoms had disappeared in all patients, and the platelet level exceeded 80,000/mL. Six patients (6/17, 35%) had complications, two minor and four major: two splenic abscesses, one respiratory distress with ascites, and one pancreatitis with ascites. Five of the six complications were observed in patients with a volume of embolized splenic parenchyma more than 70%. CONCLUSION: Our results show that splenic embolization of more than 50% of the parenchyma is effective in the treatment of hypersplenism due to PHT, but that when the embolized volume exceeds 70%, the procedure is associated with considerable morbidity.

Cinacalcet treatment of primary hyperparathyroidism.

Although parathyroidectomy remains the only curative approach to most primary hyperparathyroidism cases, medical treatment with cinacalcet HCl has been proven to be a reasonable alternative for several patient subgroups. Cinacalcet almost always controls hypercalcemia and hypophosphatemia sufficiently. PTH levels are lowered, and cognitive parameters improve. While an increase in bone mineral density DEXA scan scores was not demonstrated in cinacalcet trials, the same applies to more than half of patients after parathyroidectomy. Medical therapy should be first choice in patients with hyperplasia in all glands rather than an isolated adenoma (10-15%), patients with persisting HPT following unsuccessful surgery or inoperable cases due to comorbidities, and patients detected in lab screens for hypercalcemia before developing symptoms who should be treated early but are usually reluctant to undergo surgery. Nephrolithiasis was not found to occur more frequently in cinacalcet trial groups, but urine calcium excretion as one major risk factor of this complication of primary HPT may increase on cinacalcet. Patients carrying the rs1042636 polymorphism of the calcium-sensing receptor gene respond more sensitively to cinacalcet and have a higher risk of calcium stone formation. Cinacalcet is usually administered twice daily but three or four doses per day should be discussed to mimic the beneficial pulsatile PTH-pattern.

Podocyte expression of the CDK-inhibitor p57 during development and disease.

BACKGROUND: The mature podocyte is a terminally differentiated cell with a limited proliferative capacity. The precise cell cycle proteins necessary for establishing podocyte quiescence during development or permitting podocyte cell cycle re-entry in disease states have not been fully defined. Accordingly, we studied the role of the cyclin dependent kinase (CDK)-inhibitor p57Kip2 (p57) in modulating these processes. METHODS: The expression of p57 protein in relation to markers of DNA synthesis was examined in developing mouse kidneys, and in the passive Heymann nephritis (PHN) and anti-glomerular antibody models of glomerular disease by immunohistochemistry. The role of p57 in glomerulogenesis was explored by examining renal tissue from embryonic p57-/- mice, and the expression of p21, p27 and p57 protein and mRNA was examined in podocytes in vitro. RESULTS: The de novo expression of p57 during glomerulogenesis coincides with the cessation of podocyte proliferation, and the establishment of a mature phenotype, and p57 is expressed exclusively in podocytes in mature glomeruli. However, p57 knockout mice have normal glomerular podocyte development. In addition, mRNA but not protein levels of p57 increased upon differentiation of podocytes in vitro. There was a marked decrease in p57 expression in both animal models of podocyte injury. This was diffuse in PHN, whereas in the murine model, loss of expression of p57 occurred predominantly in proliferating podocytes, expressing proliferating cell nuclear antigen (PCNA). CONCLUSION: Despite the de novo expression of p57 protein coinciding with the cessation of primitive podocyte proliferation during glomerulogenesis, embryonic p57-/- mice glomeruli were histologically normal. Cultured podocytes did not require changes in p57 protein levels to undergo differentiation. These data suggest that p57 alone is not required for podocyte differentiation, and that other cell cycle regulators may play a role. Furthermore, although injury to mature podocytes in experimental glomerular disease is associated with a decrease in p57, the levels of all three members of the Cip/Kip family of CDK inhibitors appear to determine the capability of podocytes to proliferate.

Sweet's syndrome in a patient with acute Crohn's colitis and longstanding ankylosing spondylitis.

Acute neutrophilic dermatosis, also referred to as Sweet's syndrome according to the first description in 1964, occurs not only as an isolated phenomenon but also in the context of neoplastic and inflammatory diseases, occasionally including arthritides. Recently Sweet's syndrome has been reported in a small number of patients with chronic inflammatory bowel disease, mostly in advanced stages of the disease. Here, we describe the sudden outbreak of acute neutrophilic dermatosis in coincidence with the onset of severe Crohn's disease (CD) in a patient with long-standing ankylosing spondylitis (AS). This condition has not been described before and therefore Sweet's syndrome should be added to the spectrum of skin manifestations the rheumatologist has to think about in the context of the spondylarthropathies (SpA). Furthermore, this case report is of interest because the skin lesions of Sweet's syndrome are somewhat similar to psoriasis, which is a rather frequent feature of the spondylarthropathies. This article intends to clarify the clinical and histological differentiation between Sweet's syndrome, psoriatic skin lesions and erythema nodosum for the rheumatologist and stresses that these conditions must each be treated in a completely different manner.

Familial hemolytic-uremic syndrome in three generations.

The hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are complex entities characterized by microangiopathic hemolytic anemia, thrombocytopenia, and variable impairment of renal function, occasionally complicated by neurological symptoms. In both syndromes, rare instances of familial forms have been reported. We present the case of a family in which signs and symptoms of HUS/TTP appeared in three generations. We also briefly review the literature on inherited forms of HUS/TTP and discuss the outcome of renal transplantation in adult patients with this syndrome.

Triple therapy and incidence of de novo cancer in renal transplant recipients.

Some 27 (5.5 per cent) of 492 renal transplant recipients developed de novo cancer between January 1975 and December 1991. Patients administered triple therapy of prednisolone, cyclosporin A and azathioprine had a significantly higher incidence of cancer (seven of 40 patients; 17.5 per cent) than those given prednisolone with cyclosporin (14 of 319; 4.4 per cent) and azathioprine with prednisolone (six of 133; 4.5 per cent) (P = 0.005). In a prospective study between January 1989 and December 1992, 110 renal transplant patients were randomized into three immunosuppressive regimens at the time of transplantation. The incidence of cancer in patients receiving low-dose cyclosporin, azathioprine and prednisolone was three of 45, in those given high-dose cyclosporin and prednisolone none of 23 and in those administered high-dose cyclosporin, nifedipine and prednisolone one of 29. The addition of azathioprine to ongoing maintenance cyclosporin and prednisolone therapy is useful in a subgroup of patients with graft dysfunction, but there are possibly higher risks in the development of de novo carcinoma.

Polymerase chain reaction and focal contact formation indicate integrin expression in mesangial cells.

Cultured kidney glomerular mesangial cells (MCs) allow the role of extracellular matrix (ECM) and growth factors in glomerular inflammatory disease to be studied. To investigate the potential of MCs to interact with matrix components, the expression of integrin mRNA in cultured MCs was examined by polymerase chain reaction (PCR), by Northern blotting and by immunofluorescence. In addition, the effect of matrix substrates on mRNA expression was assessed by PCR. Northern blots with cDNA probes to integrin alpha-chains revealed that MCs expressed alpha 1, alpha 3 and alpha 5 integrin mRNA. alpha 1 and alpha 3 were the major messages. No alpha 2, alpha 4 or alpha 6 were detectable. RT-PCR revealed that alpha 2 and alpha 6 were also expressed at low levels. The control cells, HT1080, expressed alpha 2, alpha 3, alpha 4, alpha 5 and alpha 6 mRNA, and Rugli expressed alpha 1, alpha 3 and alpha 5, supporting previous studies. Immunocytochemistry confirmed that alpha 1 beta 1, alpha 2 beta 1, and alpha 5 beta 1 integrins were expressed and that they were concentrated into focal adhesions (alpha 1 beta 1 on type I collagen and laminin; alpha 2 beta 1 on type I collagen; alpha 3 beta 1 on type I collagen, laminin and fibronectin; alpha 5 beta 1 on fibronectin). alpha 6 beta 1 was not detected in focal contacts. Attachment, spreading, and formation of talin and integrin containing focal contacts still occurred when endogenous protein synthesis was blocked with 30 micrograms.ml-1 cycloheximide.(ABSTRACT TRUNCATED AT 250 WORDS)

[On adaptation of adolescents to the vocational training in the point of view of schooldoctors (author's transl)]. / Zur Adaptation Jugendlicher an die Berufsausbildung aus jugendärztlicher Sicht.

Since 1969 bibliographie informes that growing numbers of apprentices and young workers are absent from work in spite of their obviously good health-state. All reported results of several studies and investigation-programms show that in most of the described cases problems of juvenile adaptability are linked with. Difficulties arise when young people change over from school education to vocational training. At first the paper deals with the differently used terminus adaptation. Three empirical principles from available studies may be deduced. They are interpreted under the point of view of school-doctors, works-doctors and vocational-school-instructors. Recommendations for school-doctors finish the paper.