Renal artery aneurysm and fibromuscular dysplasia.
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Biomedical subjects
Publications and source records attributed to U Blum.
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Medullary cystic kidney disease (MCD) is characterized by multiple renal cysts at the corticomedullary boundary area, by autosomal dominant inheritance, and by onset of chronic renal failure in the third decade of life. We report on a family with three affected individuals of both sexes in two generations presenting with end-stage renal failure at age 22-31 years. Primarily diagnoses considered included unclassified hereditary nephropathy and autosomal dominant polycystic kidney disease. Careful evaluation of all findings, initiated after investigation of renal morphology with CT, revealed features characteristic for MCD and led to the final diagnosis of MCD. We conclude that MCD is an important differential diagnosis for polycystic kidney disease in young adults with end-stage renal failure. Establishing the correct diagnosis has considerable impact for genetic counselling.
PURPOSE: To compare the diagnostic accuracy of magnetic resonance imaging (MRI) and immunoscintigraphy (IS) for detection of bone marrow infiltration in malignant lymphoma. PATIENTS AND METHODS: In 32 patients with Hodgkin's disease (HD) or non-Hodgkin's lymphoma (NHL), MRI of the axial skeleton and whole-body IS using technetium-99m (99mTc)-labeled monoclonal antibodies were reviewed and compared with iliac crest biopsies. Criterion for marrow infiltration was a positive biopsy or concordant positive results of MRI and IS. RESULTS: In 16 patients (50%), MRI, IS, and iliac crest biopsies were negative for marrow infiltration. Iliac crest biopsy showed infiltration in only four patients (13%). Infiltration was missed in two of 32 patients with IS and in one patient with MRI. In one additional patient, MRI was false-positive because of pelvic hematopoietic hyperplasia. A subset of nine patients (28%) with negative biopsies had bone marrow involvement according to MRI and IS with identical location and pattern of infiltration. In eight of these nine patients, diagnostic imaging indicated marrow involvement only in noncrest marrow. Subsequent biopsy confirmed infiltration in five patients. The clinical course suggested true-positive imaging results in the remaining four patients. Two patients (6%) remained equivocal. Overall concordance of MRI and IS for marrow infiltration was 88% (28 of 32 patients). CONCLUSION: Diagnostic imaging is essential for optimal staging in malignant lymphoma, as blind biopsies appear to have low sensitivity for bone marrow infiltration because of frequent involvement in noncrest marrow. both imaging modalities show a high rate of detection of bone marrow infiltration.
PURPOSE: To report the results of a two-center study of endovascular abdominal aortic aneurysm (AAA) exclusion using a polyester-covered nitinol stent-graft. METHODS: Candidates were evaluated with arteriography and computed tomography. Criteria for endovascular therapy were a proximal aortic neck > 10 mm in length and < 25 mm in diameter, no bilateral internal iliac artery involvement in the aneurysm, no markedly tortuous common iliac arteries (CIAs) or CIAs < 7 mm in diameter, and no superior mesenteric artery occlusive disease. Patients were treated with the Mialhe Stentor and Vanguard stent-grafts in either tube or bifurcated versions. RESULTS: Between August 1994 and November 1996, 149 patients (mean age 67 years, range 49 to 90) were admitted to the study. Overall primary technical success (aneurysm exclusion without endoleak) was 87% (130 patients): 78% (7 patients) for tube grafts and 88% (123 patients) for bifurcated endografts. The rate of local, remote, or systemic complications was 10.8%, with a 30-day mortality rate of 0.7%. During an average 13.5-month follow-up, there were no late deaths. Four of 20 endoleaks sealed spontaneously, 14 were treated with endoluminal techniques, and 2 remain untreated by patient request. Three graft limb thromboses occurred; one was treated surgically, one with lytic therapy, and one was untreated. Secondary patency was 96%. CONCLUSIONS: Endoluminal repair of infrarenal AAAs using straight or bifurcated grafts is a feasible alternative to conventional surgical repair. Longer follow-up and more experience with refined endograft models will elucidate the durability of this endovascular approach to treating AAAs.
Hepatocellular carcinoma (HCC) is a frequent complication of chronic liver disease. The major causes of chronic liver diseases are hepatitis B virus (HBV) or hepatitis C virus (HCV) infection, as well as chronic alcohol misuse. The clinical presentations of HCC patients are unspecific, with signs and symptoms of chronic liver disease. Early diagnosis of HCC in an asymptomatic and potentially curable stage is of highest priority. The present strategy for the detection of early HCC in patients with chronic liver disease is 6-monthly determination of alpha-fetoprotein (AFP) and ultrasound study of the liver. If these are abnormal, further diagnostic steps include computer tomography, magnetic resonance tomography, lipiodol-angiography and histopathology. By this sequential diagnostic strategy it should be possible to identify HCC in patients with chronic liver disease at an early and potentially curable stage.
The therapeutic modalities in patients with hepatocellular carcinoma (HCC) depend on the number, size and location of the lesions as well as the stage of the underlying liver disease and the physical condition of the patient. In patients with small and solitary lesions, resection, liver transplantation and in some cases percutaneous ethanol injection (PEI) can be curative. In more advanced stages of the disease with larger or multiple lesions, PEI and/or transarterial chemotherapy with or without embolization (TACE or TAC) can slow the progression of the disease. In disseminated disease, a radiotherapeutic approach can be taken in selected cases. The therapeutic strategy in patients with HCCs should be individualized, frequently involving a combination of therapeutic modalities. In contrast to the earlier dismal prognosis, for most HCC patients there is today a therapeutic strategy that results in prolongation of life and in some cases even cure.
HISTORY AND CLINICAL FINDINGS: Two patients were admitted to hospital for diagnosis of recurrent upper gastrointestinal bleeding. Both had chronic pancreatitis with alcohol abuse. Their general condition was satisfactory. Physical examination showed no diagnostic abnormalities other than mild epigastric pain on pressure in one patient. INVESTIGATIONS: In case 1 angiography revealed pseudoaneurysm of the splenic artery as a complication of chronic pancreatitis to be the cause of the bleeding. In case 2 sonography demonstrated multiple pancreatic pseudocysts after recurrent pancreatitis. Duplex sonography revealed one of the cyst to be a partly thrombosed pseudoaneurysm of the splenic artery and the source of the bleeding. TREATMENT AND COURSE: In both cases a fistula between splenic artery and pancreatic duct having been shown to be the source of the bleeding, transcatheter embolisation of the splenic artery with platinum coils was successfully undertaken. Both patients remained symptom-free 4 and 10 months later. CONCLUSION: Although haemosuccus pancreaticus is a rare cause of upper gastrointestinal bleeding, given certain features in the patient's history and the clinical findings, it should be included in the differential diagnosis.
The purpose of this prospective, angiographically controlled study was to determine the diagnostic value of color Doppler sonography with two differing approaches of scanning in patients with clinical clues for renal artery stenosis (RAS). In 135 hypertensive patients peak systolic velocity in the main renal arteries and the resistive index (RI) of the intrarenal arteries were measured. The RI side-to-side-difference (delta RI) greater than 0.05 or peak systolic velocity greater than 2 m/s were used to discriminate normal from stenotic renal arteries, defined as angiographically confirmed RAS > 50%. In 88 of 135 patients 107 RAS were demonstrated by selective arteriography. Five renal artery occlusions occurred, which were all diagnosed by color Doppler sonography. Seventy-six RAS were detected by increased peak systolic velocity, and an additional 19 stenoses were identified by delta RI. The combined analysis of peak systolic velocity and delta RI resulted in a sensitivity of 89% and specificity of 92%. The positive predictive value was 92% and the negative predictive value was 88%. Our data suggest that only the combination of intra- and extrarenal scanning with color Doppler sonography represents an effective screening method for significant renal artery stenosis in hypertensive patients.
PURPOSE: With a series of 31 transluminal stent-graft procedures, we assessed the feasibility and clinical effectiveness of a new stent-graft for the treatment of infrarenal abdominal aortic aneurysms (AAAs). METHODS: We treated the patients (male; mean age 71 years) with straight or bifurcated nitinol stents covered with woven dacron graft material for infrarenal eccentric saccular AAA (n = 4) or AAA involving the bifurcation and the common iliac arteries (n = 27). The 18-F delivery system was advanced via a surgical arteriotomy and the stent-graft was placed under fluoroscopic control. Follow-up period ranged from 8 days to 9 months. RESULTS: The implantation of the stent-grafts was technically successful in 30/31 cases. Procedure-related complications were acute hepatic failure due to gastric bleeding in a patient with liver cirrhosis, graft occlusion due to emboli originating from the left atrium (n = 1), macro-embolisation (n = 3), local haematoma (n = 1) and av-fistula (n = 1) requiring surgery. A post-implantation syndrome with leukocytosis and elevated C-reactive protein was observed in all patients. CONCLUSIONS: Endoluminal repair of infrarenal AAA with use of dacron covered nitinol stent-grafts is feasible, safe and clinically effective. However, careful long-term evaluation is necessary.
PURPOSE: To evaluate treatment of infrarenal abdominal aortic aneurysm (AAA) with a new endoluminal stent-graft. MATERIALS AND METHODS: In 26 male patients, straight or bifurcated nitinol stents covered with woven Dacron graft material were implanted to treat eccentric saccular AAA (n = 3) or AAA involving the bifurcation and common iliac arteries (n = 23), with follow-up from 8 days to 8 1/2 months. RESULTS: Implantation was technically successful in all but one of the 26 (96%) patients (leak of the stent-graft for more than 3 months necessitated implantation of an additional covered stent). In seven of the 26 patients, minor residual perfusion persisted immediately after implantation, but complete thrombosis occurred within 7 days. Five procedure-related complications occurred: distal embolization (n = 2); local hematoma, which necessitated surgery (n = 1); acute hepatic failure due to gastric bleeding, in a patient with liver cirrhosis (n = 1); and stent-graft occlusion due to emboli originating from the left atrium (n = 1). CONCLUSION: Exclusion of AAA from circulation was feasible, safe, and clinically effective with the new stent-graft.
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OBJECTIVE: Sacroiliitis is often difficult to diagnose in the absence of radiographic alterations. For the diagnosis of active sacroiliitis, plain radiography, scintigraphy, and contrast enhanced magnetic resonance imaging (MRI) were evaluated in a prospective study. METHODS: In 44 consecutive patients with complete clinical and laboratory evaluation, plain radiographs, quantitative sacroiliac (SI) scintigraphy, and MRI were performed to evaluate the contribution of these imaging techniques to the diagnosis of active sacroiliitis. Scintiscanning and MRI were done in 20 control subjects to define the normal range of imaging findings. We determined the sensitivity and specificity for each imaging method using a reference standard based on clinical symptoms of inflammatory low back pain with or without laboratory signs, and on clinical and radiographic followup during 1.5-2.5 years to confirm diagnosis. RESULTS: MRI was most sensitive (95%) and superior to quantitative SI scintigraphy (48%) or conventional radiography (19%) for the detection and confirmation of active sacroiliitis. For the assessment of inflammatory signs, MRI had higher specificity (100%) than scintigraphy (97%) or plain radiography (47%). At repeat MRI after 2-30 months, there was persistent pathologic signal intensity in the subchondral bone area despite clinically successful antiinflammatory drug therapy. CONCLUSION: For the assessment of active changes in the synovial portion and the subchondral bone marrow, contrast enhanced MRI is superior to quantitative SI scintigraphy or conventional radiography. MRI picks up an additional 75% of early cases not diagnosed by plain radiography. Scintigraphy is only of limited value. Persistent pathologic signal intensity in the subchondral bone marrow seems to be closely associated with previous inflammatory episodes, thus limiting specificity of MRI for active sacroiliitis. Based on our findings we suggest an algorithm for the evaluation of patients with suspected active sacroiliitis.
Intravascular stents have played an important role in improving early and long-term patency in the treatment of arterial occlusive disease, particularly in the iliac arteries after failed balloon angioplasty. The development of covered stents opened a new dimension for non-operative therapy of aneurysmal disease. Transluminally placed stent-grafts represent a blending of technologies with use of intravascular stents and prosthetic vascular grafts. Stent-graft combinations were first envisioned by Dotter in 1969 as devices that would ultimately be useful to treat aneurysms, pseudoaneurysms and arterio-venous fistulas. Animal studies of endovascular stented grafts demonstrated the potential feasibility of these devices to treat arterial lesions. Parodi et al. first implanted stent-grafts in patients with abdominal aortic aneurysms and demonstrated its clinical feasibility. Newly developed stent-grafts allowed to treat successfully a limited number of patients with AAA. Although much has been reported about the healing properties of prosthetic grafts in extraluminal locations, there are only little data concerning the arterial response to a prosthetic graft placed within the lumen or anchored into the wall of a human vessel. Therefore, long-term evaluation of these new devices will be crucial.
With a series of 34 transluminal stent-graft procedures, we assessed the feasibility and clinical effectiveness of a new stent-graft for the treatment of infrarenal abdominal aortic aneurysms (AAAs). We treated 34 male patients (mean age 71 years) with straight of bifurcated nitinol stents covered with woven Dacron graft material for infrarenal excentric saccular AAA (n = 3) or AAA involving the bifurcation and the common iliac arteries (n = 31). The 18-F delivery system was advanced via a surgical arteriotomy and the stent-graft was placed under fluoroscopic control. Follow-up period ranged from 8 days to 13 months. The implantation of the stent-grafts was technically successful with exclusion of AAA in 31/34 cases (91%). In 2 patients, there was a persisting leak at the distal end of the endoprosthesis after treatment; in another, marked coiling of the external iliac artery impeded the delivery system to be advanced and consecutive rupture resulted in conversion to surgical repair. Other procedure-related complications were acute hepatic failure due to gastric bleeding in a patient with liver cirrhosis, graft occlusion due to emboli originating from the left atrium (n = 1), local hematoma (n = 1), and AV-fistula (n = 1) requiring surgery. A post-implantation syndrome with leucocytosis and elevated C-reactive protein was observed in all patients. Endoluminal repair of infrarenal AAA with use of Dacron covered nitinol stent-grafts is feasible, safe and clinically effective. However, careful long-term evaluation is necessary before it will become clinical practice.
Breath-hold MR cholangio-pancreaticography using the RARE method was evaluated as a noninvasive alternative to direct cholangio-pancreaticography in 30 healthy volunteers and in 30 patients with various diseases of the respective duct systems. In contrast to 3D MR cholangiography, RARE MRCP generates projection images requiring a 4-s acquisition time and no postprocessing. Diagnostic information gained from the cholangio-pancreaticograms was fully equivalent to that of conventional x-ray imaging by invasive procedures. Additional information was gained whenever endoscopic access was impossible and/or cystic malformations were present.
We investigated the radiological findings of five patients with primary leiomyosarcoma of the inferior vena cava (IVC) comparing cavography, computed tomography (CT), and magnetic resonance imaging (MRI). The radiographic presentation ranged from an intraluminal lesion with obstruction of the IVC to a tumor mass extending from the media musculature into the surrounding perivascular tissue with only slight protrusion into the lumen. The lesions were associated with late or few symptoms. Pathognomonic radiological findings could not be established. In cases with an extraluminal growth a lobulated, well-defined, encapsulated tumor with an inhomogeneous contrast uptake is characteristic in CT studies. Additional information was provided by MRI. Leiomyosarcomas with extraluminal involvement showed homogeneous intermediate signal intensity (SI) on T1-, and mixed intermediate/high SI on T2-weighted images. In cases with predominantly intraluminal growth, both CT and MRI demonstrated the IVC to be dilatated and allowed to differentiate between tumor extent and obliterating thrombosis. Modern imaging modalities allow an early and accurate pre-operative diagnosis resulting in a higher rate of surgical resection and improvement of survival.
PURPOSE: To evaluate the use of the transjugular intrahepatic portosystemic shunt (TIPS) and local, low-dose thrombolysis in the treatment of complete, noncavernomatous portal vein occlusion. MATERIALS AND METHODS: TIPS implantation and portal vein recanalization was attempted in seven patients with noncavernomatous portal vein obstruction and recurrent variceal bleeding. TIPS placement was followed by thrombolytic therapy to restore portal venous blood flow. Hemodynamic effects and clinical characteristics after the procedure and during follow-up were assessed. RESULTS: The implantation of TIPS and the recanalization of the portal vein trunk were successful in all patients. The treatment reduced the portal venous pressure gradient and restored portal blood flow. No bleeding complications were observed. CONCLUSION: TIPS placement and recanalization of the main portal vein is a safe and effective treatment option for patients with liver cirrhosis and noncavernomatous portal vein occlusion.