Search PubMed⌕ Search

Biomedical subjects

U Blum

Publications and source records attributed to U Blum.

At least 55 records · Page 3Linked to original sources

Budd-Chiari syndrome: technical, hemodynamic, and clinical results of treatment with transjugular intrahepatic portosystemic shunt.

PURPOSE: To evaluate use of the transjugular intrahepatic portosystemic shunt (TIPS) as a nonsurgical approach for the management of Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: Twelve patients with fulminant (n = 2), subacute (n = 5), or chronic (n = 5) BCS underwent TIPS placement. Hepatic venous obstruction was demonstrated at computed tomography and color duplex sonography. BCS was confirmed histologically in all patients. Hemodynamic parameters and clinical characteristics were assessed. RESULTS: TIPS creation was successful in all patients. Treatment reduced the portal venous pressure gradient by 75% and resulted in a mean shunt flow of 2,300 mL/min +/- 650 (standard deviation). No serious procedure-related complications were observed. The two patients with fulminant BCS died of septicemia or progressive liver failure despite intervention. The other 10 patients showed clinical improvement with reduction or disappearance of ascites. During follow-up, shunt dysfunction occurred in five of 10 patients with recurrence of ascites requiring repeat intervention. CONCLUSION: TIPS placement is safe and effective in patients with portal hypertension caused by subacute or chronic BCS.

Acute Disease↗

Hepatic portal venous gas. Imaging modalities and clinical significance.

In a prospective investigation the sensitivity of conventional radiography, sonography, color Doppler flow imaging (CDFI) and CT in the detection of hepatic portal venous gas (HPVG) was compared in 7 patients with different diagnoses. For the identification of HPVG sonography, CDFI and CT have a higher sensitivity than conventional radiography. CT, however, was the most suitable method to identify the underlying cause of HPVG. Patients with iatrogenic HPVG as a result of diagnostic or therapeutic intervention had a good prognosis. In contrast, in all cases with a sudden appearance of HPVG and a noniatrogenic cause, exitus ensued within 1 week.

Abdomen↗

Tuberous sclerosis complex with end-stage renal failure.

Renal angiomyolipoma is common in the tuberous sclerosis complex (TSC), the classic features of which are facial angiofibroma, seizures, and mental retardation. We report a family with three affected members demonstrating the wide spectrum of TSC-associated lesions ranging from asymptomatic findings to life-threatening complications. The predominant symptoms of the index patient were hypertension and mild renal insufficiency at age 48, resulting in end-stage renal failure at age 63 due to giant bilateral angiomyolipoma of the kidneys. The two TSC-affected siblings had died years previously, one from pulmonary lymphangioleiomyomatosis and the other during an epileptic state; the latter had situs inversus totalis as another remarkable finding. The diagnosis of TSC may be overlooked if CNS symptoms are absent and if cutaneous lesions are masked by cosmetic procedures, as occurred in the index case. Chronic renal failure due to angiomyolipoma is not widely known to clinical nephrologists, but develops in approximately 15% of TSC patients. Displacement of functional renal parenchyma by abnormal tissue appears to be the major pathogenetic mechanism leading to end-stage renal failure. Angiomyolipomas can be diagnosed from this characteristic sonographic pattern and the demonstration of fatty tissue in CT or MRI. Multiple renal cysts are also common in TSC. Therefore TSC should be considered in the differential diagnosis of polycystic kidney disease.

Angiomyolipoma↗

The transjugular intrahepatic portosystemic stent-shunt procedure for variceal bleeding.

BACKGROUND: Transjugular placement of an intrahepatic stent is a new technique to establish a portosystemic shunt for treatment of portal hypertension. A puncture needle is advanced in a catheter through the inferior vena cava into a hepatic vein; then an intrahepatic branch of the portal vein is punctured and an expandable stent of metallic mesh is implanted to establish the shunt. METHODS: We attempted the stent-shunt procedure in 100 of 112 consecutive patients with variceal bleeding due to cirrhosis, who were then followed for a mean (+/- SD) of 12 +/- 6 months. Of the 100 patients, 22 had Child-Pugh class C cirrhosis, 10 were treated on an emergency basis, and 68 had alcoholic cirrhosis. The shunt was established with use of Palmaz stents expanded to 8 to 12 mm in diameter. RESULTS: Technical success was achieved in 93 percent of the patients. The mean (+/- SD) time for the procedure was 1.2 +/- 0.3 hours. The shunt reduced the portal venous pressure gradient by 57 percent. Major complications were hemorrhage (intraabdominal bleeding in six patients, biliary bleeding in four, and bleeding in the liver capsule in three) and migration of the stent into the pulmonary artery (in two patients). At follow-up, stenosis of the shunt was evident in 21 patients and occlusion in 10 patients; 10 of these 31 patients had variceal rebleeding. Stenoses and occlusions of the shunt were all treated successfully by redilation, thrombolysis, or implantation of an additional stent. Hepatic encephalopathy (stages I to III) developed in 25 percent of the patients. The proportion of patients with shunts who remained free of variceal rebleeding was 92 percent at six months and 82 percent at one year. The 30-day mortality was 3 percent. The cumulative one-year survival was 85 percent. CONCLUSIONS: These results suggest that the transjugular placement of an intrahepatic portosystemic stent is an effective and safe treatment for variceal hemorrhage in patients with portal hypertension due to cirrhosis.

Esophageal and Gastric Varices↗

[Angiography detection and embolization of a hemodynamically significant hemorrhage from the right dorsal penile artery in symphysis rupture].

Pelvic fractures are frequently complicated by concomitant vascular lacerations involving, particularly, branches originating from the internal iliac arteries. Massive hemorrhage may be difficult to be treated surgically. In such cases interventional radiology can be helpful and able to treat definitely extensive hemorrhage caused by pelvic trauma.

Adult↗

[Radiologic differential diagnosis of inflammatory round pulmonary infiltrates in immunocompromised patients. A prospective study using CT and MRT].

In a prospective study we examined the diagnostic ranking of CT and MR in 52 immunocompromised patients with nodular pulmonary lesions and clinical suspicion of invasive pulmonary aspergillosis (IPA). For early diagnosis of IPA (clinical symptoms having existed for less than 10 days) the CT halo sign proved highly sensitive and specific. MRT showed at this time a comparatively high sensitivity but only low specificity that could not be improved upon after Gd-DTPA. At a later stage of the aspergillosis infection (clinical symptoms manifested for more than 10 days) MR identified aspergillus-specific lesions with on-target characteristics (marked enhancement of margins after Gd-DTPA) or the so-called "reverse" target phenomenon (T2-weighted sequences). Such lesions were never seen in the early stage of the disease in patients with nodular pulmonary lesions of different aetiology (pseudomonal or staphylococcal pneumonia).

Adult↗

Invasive pulmonary aspergillosis. MRI, CT, and plain radiographic findings and their contribution for early diagnosis.

A prospective study was conducted in 38 patients with nodular lesions on plain chest radiographs and the clinical suspicion of invasive pulmonary aspergillosis (IPA) to assess the diagnostic accuracy of magnetic resonance imaging (MRI) and computed tomography (CT). For early diagnosis of IPA (clinical signs and symptoms < 10 days), CT scans with demonstration of the halo sign had a high sensitivity (16/22) and specificity (8/8). Magnetic resonance imaging performed at the same time revealed a relatively higher sensitivity (22/22), but a very poor specificity (0/8). Gadolinium-diethylene-triamine-pentaacetic acid (Gd-DTPA) enhanced images did not improve specificity. In the later course of infection (clinical signs and symptoms > 10 days), MRIs showed typical nodular target-like lesions with Gd-DTPA enhancement of the rim area that was not seen in the early course of the disease or in patients with Pseudomonas or staphylococcal infection. In conclusion, MRI findings are not as characteristic as the CT halo sign in diagnosing IPA in the early course of the disease, but the MRI target sign with Gd-DTPA enhancement of the rim area and the "reverse target" on T2-weighted images are strongly suggestive of IPA at a later stage of the disease.

Aspergillosis↗

[Total cavopulmonary anastomosis: risk factors and results in patients under 4 years of age].

In recent years, an increasing number of modified Fontan-operations has been performed in children younger than 4 years of age. The purpose of this study was to identify preoperative risk factors in this age group. From February 1990 until February 1993, we performed in our center a modified Fontan-operation using the technique of total cavopulmonary anastomosis (TCPA) in 37 consecutive patients (17 pts. < 4 years = group I, 20 pts. > 4 years = group II). Early postoperative mortality occurred in patients of group I only (n = 3 pts.). All of these patients had additional preoperative risk factors. Pulmonary vascular resistance (PVRI) > 2 U x m2 was a significant risk factor for the younger patients while pulmonary artery size alone (expressed as the McGoon-ratio or Nakata-index) could not be identified as a separate risk factor. Using two additional indices (McGoon-ratio/PVRI and Nakata-index/PVRI), we were able to identify patients with unfavorable postoperative hemodynamics as high-risk patients. In our experience, TCPA can be performed in patients younger than 4 years of age with a low mortality, if there are no additional preoperative risk factors. For high-risk patients we recommend either a bidirectional Glenn-anastomosis as a first step procedure or a TCPA with fenestration of the intraatrial tunnel-patch.

Adolescent↗

[Differential diagnostic aspects of lethal midline granulomas (granuloma gangraenescens].

The lethal midline granuloma and limited Wegener's granulomatosis show clinical similarity, although they are of different etiology. The following case of a 53-year-old woman shows how difficult it is to establish the precise diagnosis of lethal midline granuloma. The diagnosis depends on the pathological finding of a lymphoma. The lymphoma can be differentiated in a T- or a B-cell lymphoma by immunostaining. However, the diagnostic yield of biopsies from the nose is not perfect. It would be, therefore, important to find other diagnostic criteria. The presence of the c-ANCA is a helpful tool, but in the case of limited Wegener's granulomatosis, it has a sensitivity of 50%. The prognosis of the lethal midline granuloma is poor even if an adequate radiation therapy is instituted.

Diagnosis, Differential↗

Early and long-term results of cardiac surgery in dialysis patients.

Cardiac surgery in patients receiving dialysis has a high mortality and morbidity. Early long-term results have been analysed following various cardiac operative procedures. Between 1981 and 1992, 40 patients, of mean age 55.7 (range 31-71) years underwent cardiac surgery. Procedures included coronary artery bypass grafting (20 patients), aortic valve replacement (six), mitral valve replacement (four), aortic valve or mitral valve plus coronary artery bypass grafting (one each), aortic plus mitral valves (two), heart and renal transplantation (one each), atrial septal defect closure (one) and pericardial decortication (three). Before surgery, patients were in the New York Heart Association (NYHA) classes II (5%), III (52.2%) and IV (42.5%). The operative mortality rate was 15%; survivors were in NYHA classes I (3%), II (88.2%) and III (8.8%). There were 11 late deaths. Follow-up at a mean of 35 (range 1-93) months showed actuarial survival rates of 91% (1 year) and 69% (5 years). After coronary artery bypass grafting, survival rates were 95% (1 year) and 72% (5 years). Survivors were in NYHA classes I (4.7%), II (85.5%) and III (9.5%). Cardiac surgery in dialysis patients is associated with an acceptable hospital mortality; quality of life in long-term survivors is increased. Hence, surgical treatment is advocated in patients with symptomatic heart disease. Early diagnosis and surgical intervention in NYHA class II patients may lead to a lower perioperative mortality.

Adult↗

Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease.

BACKGROUND: Pheochromocytoma is a feature of two disorders with an autosomal dominant pattern of inheritance--multiple endocrine neoplasia type 2 (MEN-2) (with medullary thyroid carcinoma and hyperparathyroidism) and von Hippel-Lindau disease (with angioma of the retina, hemangioblastoma of the central nervous system, renal-cell carcinoma, pancreatic cysts, and epididymal cystadenoma). The frequency of these syndromes in patients with pheochromocytoma is not known. METHODS: In an unselected group of patients with pheochromocytoma, we performed pentagastrin tests, parathyroid hormone assays, computed tomography (CT) or magnetic resonance imaging (MRI) of the brain, ophthalmoscopy, CT imaging of the abdomen, and ultrasonography of the testes. We also screened members of families with MEN-2 or von Hippel-Lindau disease for pheochromocytoma by measuring plasma and urine catecholamines and plasma chromogranin A and by performing abdominal ultrasonography, CT and MRI, and metaiodobenzylguanidine scintigraphy. RESULTS: Nineteen of 82 unselected patients with pheochromocytomas (23 percent) were carriers of familial disorders; 19 percent had von Hippel-Lindau disease and 4 percent had MEN-2. Prospectively, in 36 of 79 subjects at risk for pheochromocytoma (46 percent), 42 unsuspected pheochromocytomas were found. Overall, there were 130 patients with 185 pheochromocytomas; 43 had von Hippel-Lindau disease, 24 had MEN-2, and 63 had sporadic tumors. The patients with familial and those with sporadic pheochromocytomas differed in mean age at diagnosis (32 vs. 46 years, P < 0.001), multifocal localization (55 vs. 8 percent, P < 0.001), and cancer (0 vs. 11 percent, P = 0.005); but not in the frequency of extraadrenal tumors (24 vs. 16 percent). Thirty-eight percent of carriers of von Hippel-Lindau disease and 24 percent of carriers of MEN-2 had pheochromocytoma as the only manifestation of their syndrome. CONCLUSIONS: All patients with pheochromocytomas should be screened for MEN-2 and von Hippel-Lindau disease to avert further morbidity and mortality in the patients and their families. All patients in families with MEN-2 or von Hippel-Lindau disease should be screened for pheochromocytoma, even if they are asymptomatic.

Adolescent↗

Permanent epicardial and transvenous single- and dual-chamber cardiac pacing in children.

Between January 1974 and November 1991 33 children received a permanent single- or dual-chamber pacing system, mainly because of postoperative high-degree AV block. The children were followed up retrospectively for pacemaker- and lead-related complications, and for differences between epi- and endocardial stimulation. The overall rate of lead related complications was 35% and did not differ significantly between the epi- and endocardially paced groups, although it tended to be somewhat higher in the epicardially paced children, mainly due to a higher rate of exit blocks in the latter. The epicardially stimulated patients exhibited a significantly higher rate of pacemaker-related complications, which was primarily accounted for by a higher frequency of battery depletions in the epicardial systems. The most impressive differences between both groups, however, was seen with respect to subacute and chronic energy consumption. Chronic energy drain in the epicardially paced patients amounted to almost the sixfold of that seen in the endocardially stimulated children. This resulted in a significantly shorter cumulative pacemaker survival in the epicardial group. Therefore, it is concluded that, whenever possible, the transvenous approach be used in children and small infants too. However, as a rule, in the latter transvenous dualchamber pacing is usually not feasible. In these cases rate-adaptive single-chamber pacing has evolved as a reasonable alternative for improving hemodynamics as well as quality of life. In epicardial pacing the use of pulse generators allowing bidirectional telemetry is advisable. In this way monitoring of lead impedance and battery status can be performed noninvasively, thus permitting individualization of pulse widths and amplitude setting, which is important with respect to energy conservation.

Adolescent↗

Total cavopulmonary anastomosis: selection criteria related to postoperative results.

In this study we review our experience with total cavopulmonary anastomosis (modified Fontan procedure) which was performed in 31 patients. One patient died from postoperative cardiac low-output syndrome, the other died from a neurological complication (early mortality 6.5%). During a mean follow-up period of 15 months there was no late mortality. Preoperative assessment showed that one, two, or three of the Choussat criteria (defining the ideal Fontan candidate) were not fulfilled by 39%, 16% and 6% of our patients. In the 2 patients who died 2 and 3 of those criteria were not fulfilled. Poor outcome could not be predicted based on assessment of the pulmonary artery size (expressed as the McGoon-ratio or the Nakata-index) alone. In order to assess the relationship of pulmonary artery size and pulmonary arteriolar resistance (PVR) as a predictor of outcome, we introduced two new indices (McGoon-ratio/PVR and Nakata-index/PVR). The patient who died from poor postoperative hemodynamics had the lowest values of all patients. Among the 29 survivors we did not observe significant acquired postoperative arrhythmias. In our experience the total cavopulmonary anastomosis can be performed with a low mortality and good postoperative results. Patients who do not fulfill at least 8 of the Choussat criteria and children with a low ratio of pulmonary artery size to PVR are high-risk patients. In these children we recommend either a bidirectional Glenn anastomosis as a first-step procedure or a total cavopulmonary anastomosis with a fenestration of the intraatrial tunnel.

Adolescent↗

Percutaneous recanalization of iliac artery occlusions: results of a prospective study.

PURPOSE: To evaluate the long-term results of recanalization of occluded iliac arteries with local low-dose thrombolysis, angioplasty, and, if necessary, stent implantation. MATERIALS AND METHODS: Forty-seven patients with acute or chronic occlusions of the common or external iliac artery, or both, underwent local low-dose thrombolysis (n = 47), percutaneous transluminal angioplasty (PTA) (with balloon dilation [n = 43] and rotational angioplasty [n = 30] in the patients in whom a retrograde recanalization was performed), and, if needed, intravascular stent placement (n = 18). Follow-up lasted 3-53 (mean, 21) months. RESULTS: The primary recanalization rate was 98% (46 of 47). The mean ankle-brachial index increased from 0.33 to 0.81 within 14 days after treatment and was 0.76 at the most recent follow-up. Two early (< 14 days) and two late reocclusions (after 24 and 30 months) occurred; one restenosis detected with duplex sonography and angiography was observed after 19 months. CONCLUSION: This therapy represents a true alternative to vascular surgery and a first-line treatment for acute or chronically occluded iliac arteries.

Adult↗