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J Ludwig

Publications and source records attributed to J Ludwig.

341 records · Page 19Linked to original sources
PubMedDate not supplied

Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis.

Histologic, ultrastructural, chemical, and statistical methods were used to study liver biopsy and autopsy specimens from 43 patients who had primary sclerosing cholangitis (PSC), with or without chronic ulcerative colitis (CUC), and from 19 patients who had CUC without PSC. In all study groups, essentially the same abnormalities were found in the hepatic parenchyma outside the major bile ducts, although nondiagnostic tissue samples were observed also. Specimens from patients with extrahepatic PSC were indistinguishable from those patients with combined extra- and intrahepatic PSC. Common findings included periductal fibrosis and inflammation, portal edema and fibrosis, focal proliferation of bile ducts and ductules, focal bile duct obliteration and loss of bile ducts, copper deposition, and cholestasis. Proliferation of bile ducts in some portal tracts and obliteration or absence of bile duct in others were the most characteristic changes. In most specimens, inflammatory changes appeared mild, yet biliary cirrhosis had developed in 34% of the patients. Specimens from patients with PSC, with or without CUC, more often contained bile and strikingly increased stainable copper (Grades 2 and 3) than did specimens from patients with CUC without PSC. Hepatic copper contents, measured by atomic absorption spectrophotometry, also were higher in specimens from patients with PSC. Study of PCS specimens by transmission electron microscopy and by energy-dispersive X-ray microanalysis revealed that most copper was sequestered in lipolysosomes. The recognition of strikingly similar morphologic features in many liver specimens from patients with either PSC or CUC or both suggests that the causes of these conditions are closely related.

Cholangitis↗
PubMedDate not supplied

Elevated circulating immune complexes in primary sclerosing cholangitis.

Primary sclerosing cholangitis (PSC) is a syndrome of unknown etiology characterized by an association with inflammatory bowel disease in 50% or more cases. Since altered immunity, including circulating immune complexes, has been implicated in the pathogenesis of inflammatory bowel disease, we postulated that humoral immune mechanisms might also be important in the development of PSC. Therefore, as an initial step in testing this hypothesis, we examined sera of patients with PSC for the presence of circulating immune complexes by two independent methods: C1q binding and Raji cell assays. Twenty-four patients with PSC, 16 of whom had coexisting chronic ulcerative colitis, were prospectively selected by predefined biochemical, histologic, and radiographic criteria. Sixteen patients with inflammatory bowel disease and normal liver tests as well as six patients with extrahepatic biliary obstruction served as disease controls. Sera were positive for circulating immune complexes by at least one method in 80% (16/20) of patients with PSC; 70% (14/20 were positive by the Raji cell assay, 58% (14/24) by the C1q binding assay, and 45% (9/20) by both methods. Levels of circulating immune complexes by each assay were higher in sera from patients with PSC than in sera from healthy controls or patients with inflammatory bowel disease alone (p less than 0.01). There were no differences in the levels of circulating immune complexes or in the frequency of positive tests in PSC patients with or without associated inflammatory bowel disease. In addition, there was no difference between the Raji cell binding of sera from six patients with extrahepatic biliary obstruction and six healthy controls tested concurrently. These data are consistent with the hypothesis that immunologic mechanisms may be important in the pathogenesis of PSC.

Antigen-Antibody Complex↗
PubMedDate not supplied

Complete resolution of inflammatory activity following corticosteroid treatment of HBsAg-negative chronic active hepatitis.

To assess the frequency and significance of complete resolution of inflammatory activity following corticosteroid therapy, 115 patients with severe HBsAg-negative chronic active hepatitis were followed regularly for 84 +/- 5 months. Of 83 patients eligible to revert to normal liver tissue, 18 did so after 56 +/- 8 months. Five of the 18 relapsed after treatment withdrawal. Only patients who improved spontaneously after cessation of treatment from histologic features of chronic persistent hepatitis to normal invariably sustained the improvement. Of 32 patients with cirrhosis at presentation, 17 reverted to inactive cirrhosis after 48 +/- 10 months, but only 3 remained inactive after discontinuation of treatment. Mortality was similar in patients with and without reversion to normal tissue (0 vs. 14%, p greater than 0.2), but the frequency of relapse was less after complete resolution (28 vs. 76%, p less than 0.001). Reversion to inactive cirrhosis did not improve survival or reduce relapse frequency after remission and treatment withdrawal. Findings prior to therapy did not predict outcome. We conclude that complete resolution of inflammatory activity is possible, but that it occurs slowly, infrequently and unpredictably after therapy. In patients without cirrhosis, reversion to normal liver tissue decreases the likelihood of relapse and the requirement for retreatment. In patients with cirrhosis at presentation, elimination of inflammatory activity is rarely sustained and does not improve prognosis after remission and treatment withdrawal.

Adolescent↗
PubMedDate not supplied

Intrahepatic cholangiectases and large-duct obliteration in primary sclerosing cholangitis.

We studied intrahepatic bile ducts of five patients with chronic ulcerative colitis and primary sclerosing cholangitis. The livers had been obtained at the time of orthotopic liver transplantation. After specimen cholangiography and perfusion fixation, sequential blocks and sections from portal tracts were studied, combining light microscopy with scanning electron microscopy. In vivo cholangiograms were studied also. The specimens revealed: absence of normal bile ducts; presence of thin-walled tubular or saccular cholangiectases with semicircular and annular fibrous crests, without evidence of superinfection; cholangiectases with secondary acute or chronic-cellular cholangitis, with or without cholangitic abscesses; fibrous cholangitis without ductal dilatation; transformation of bile ducts into fibrous cords which were either solid or contained remnants of bile duct epithelium, and complete loss of bile ducts. The shape and distribution of the cholangiectases suggested that these lesions were manifestations of the disease process and not passively dilated normal ducts. Fibrous-obliterative cholangitis with formation of fibrous cords was found not only at the level of interlobular and adjacent septal bile ducts but also at the level of segmental bile ducts that normally would have been demonstrable in cholangiograms. The "pruned-tree" appearance in cholangiograms appears to result from the transition between patent and often cholangiectatic ducts, and duct obliteration. At present, intrahepatic cholangiectases in association with duct obliteration can be considered pathognomonic morphologic features of primary sclerosing cholangitis.

Adolescent↗
PubMedDate not supplied

The acute vanishing bile duct syndrome (acute irreversible rejection) after orthotopic liver transplantation.

The acute vanishing bile duct syndrome can be defined as an irreversible, rejection-related condition that affects hepatic allografts within 100 days after orthotopic liver transplantation and whose presence requires retransplantation. We have observed the acute vanishing bile duct syndrome in 5 of 48 consecutive patients (approximately 10%) who underwent orthotopic liver transplantation. In 4 cases, the condition progressed relentlessly within approximately 7 to 11 weeks after orthotopic liver transplantation from mild rejection to severe rejection to acute vanishing bile duct syndrome. A fifth patient had severe rejection in the first week and required retransplantation after 17 days because of thrombotic venoocclusive disease complicating the acute vanishing bile duct syndrome. Clinically, signs of impending acute vanishing bile duct syndrome included abrupt onset of fever and jaundice and marked elevation of serum bilirubin and alkaline phosphatase levels which persisted despite antirejection treatment. Biopsy specimens revealed destructive cholangitis (rejection cholangitis), ductopenia, and, if retransplantation was delayed, presence of noninflammatory, "burnt-out" portal tracts without bile ducts. We recommend to base the diagnosis of acute vanishing bile duct syndrome on documentation of severe ductopenia in at least 20 portal tracts which may require several consecutive needle biopsies. Rejection arteriopathy which was found in 3 of our 5 cases might have been another important diagnostic clue but could not be recognized prior to retransplantation. The pathogenesis of acute vanishing bile duct syndrome is not clear; until the condition had manifested itself, we found no qualitative differences between acute reversible and irreversible rejection.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMedDate not supplied

Clinical and statistical analyses of new and evolving therapies for primary biliary cirrhosis.

Primary biliary cirrhosis is a chronic, progressive, cholestatic liver disease thought to be related to abnormalities in immune regulation. The disease is associated with granulomatous bile duct destruction, cholestasis, hepatic copper overloading and the development of hepatic fibrosis or cirrhosis or both. There have been numerous therapeutic trials evaluating immunosuppressive, antifibrotic and cupruretic agents. Prednisolone, D-penicillamine, azathioprine, colchicine and chlorambucil have been evaluated in controlled clinical trials, and biochemical improvement of liver function has been noted with all of the agents, except D-penicillamine. Improved survival has also been reported in patients treated long-term with azathioprine and colchicine. However, none of the therapeutic agents has been demonstrated to halt histologic progression of the disease or to induce a complete clinical, biochemical and histologic remission as has been reported in patients with autoimmune chronic active hepatitis treated with corticosteroids. Many of the trials did not use a double-blind design, failed to use the "intent to treat" rule or failed to define an objective time to analyze results. Many of the studies involved small numbers of patients with short-term follow-up and thus potentially were inadequate to appreciate drug effects that might be of clinical benefit. Currently, there is no totally effective therapy for primary biliary cirrhosis. We believe that well-designed clinical trials can provide important information to better understand this disease until a totally effective therapy is available. New clinical trials should use well-established methodologic guidelines in study design and well-accepted statistical standards in the analysis and interpretation of results.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Diagnosis of cytomegalovirus hepatitis by histopathology and in situ hybridization in liver transplantation.

Hematoxylin-eosin staining (H&E) of cytomegalovirus (CMV) inclusion bodies was compared with in situ hybridization using a biotinylated DNA probe for the detection of CMV. Of 29 biopsy specimens selected from 23 patients with clinical CMV hepatitis and typical CMV inclusions on histopathologic examination, 23 (79%) were positive by DNA probe and 17 (59%) were detected in cell cultures. The mean number of CMV foci per tissue section was higher by DNA probe (12) compared with H&E (5). We do not recommend in situ hybridization in microbiology laboratories as a replacement for histopathology for the diagnosis of CMV in tissue specimens.

Biopsy, Needle↗
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Hepatic steatosis as a potential risk factor for major hepatic resection.

Hepatic steatosis is a recognized risk factor for primary nonfunction of hepatic allografts, but the effect of steatosis on postoperative recovery after major liver resection is unknown. Our aim was to determine if hepatic steatosis is associated with increased perioperative morbidity and mortality in patients undergoing major resection. A retrospective review of medical records of 135 patients who had undergone major hepatic resection from 1990 to 1993 was performed. Histopathology of the hepatic parenchyma at the resection margin was reviewed for the presence of macro- or microvesicular steatosis. The extent of steatosis was graded as none (group 1), mild with less than 30% hepatocytes involved (group 2), or moderate-to-severe with 30% or more hepatocytes involved (group 3). Outcome of patients was correlated with extent of steatosis. Patients with moderate-to-severe steatosis were obese (body mass index = 25.8 +/- 0.5 vs. 26.5 +/- 1.0 vs. 33.4 +/- 2.9; P< 0.05 groups 1, 2, and 3, respectively) and had an increased serum bilirubin concentration preoperatively. Hepatectomy required a longer operative time for group 3 (290 +/- 9 minutes vs. 287 +/- 13 minutes vs. 355 +/- 24 minutes; P </=0.05 groups 1, 2 and 3, respectively). Likelihood of blood transfusion was 51% in group 1, 52% in group 2, and 71% in group 3. Mortality was 14% in group 3 vs. 3% in group 1, and 7% in group 2; and liver failure occurred in 14% of patients in group 3 compared to 4% and 9% in groups 1 and 2, respectively. Patients in group 3 also had increased postoperative bilirubin levels compared to preoperative values. Moderate-to-severe hepatic steatosis may be associated with increased perioperative morbidity and mortality, and preoperative identification of steatosis warrants caution prior to major resection.

Bilirubin↗
PubMedDate not supplied

[Occupational diseases of the spine].

Since 1993 occupational influences of the spine are included into the German workman's compensation law (Berufskrankheitenverordnung). Because it is difficult to differentiate degenerative from occupational disc diseases only 3 % of the proposals have been accepted in the last years. Unfortunately most of the epidemiologic studies only consider morphologic changes of the spine but not discogenic diseases. More efforts have to be made to prevent spine diseases in the working place by exercises and back training. Our results of medical opinions in 256 cases of occupational spine diseases are presented and analysed.

Adult↗
PubMedDate not supplied

[Value of the basophil stimulation test in the diagnosis of juvenile bronchial asthma].

The diagnostic value of histamine liberation from basophil granulocytes (BST) was investigated in asthmatic children with house dust, house dust mite or moulds allergy. BST corresponded at about 80% to skin prick test (PRICK) and radio-allergo-sorbens test (RAST) in 178 patients. The relevance of the results of BST, PRICK and RAST for the asthmatic symptoms was proved by bronchial provocation in 77 children. BST corresponded clearly better to the inhalation test than PRICK and RAST. Correspondence of BST in house dust mite allergy amounted to 94%, in house dust allergy to 100%, and in moulds allergy to 77%. Overall correspondence of BST was 92%. Even when RAST and PRICK showed different results correspondence of BST attained 91%. In cases with identical results of BST, PRICK and RAST the bronchial provocation test corresponded in 98%. It is concluded, that if history fits to BST, RAST and PRICK bronchial provocation is no longer essential for the diagnosis of relevant allergens in perennial childhood asthma. This seems to hold also in cases of house dust or house dust mite allergy, where the result of PRICK or RAST is different. Especially in childhood the use of BST in the diagnostic procedure provides the following advantages: risks of bronchial provocation and the necessary hospitalisation can be avoided, cooperation of the patient is not needed, and costs can be reduced.

Allergens↗
PubMedDate not supplied

[Pediatric mono-arthritis as initial manifestation of X-linked agammaglobulinemia (M. Bruton)].

INTRODUCTION: There are different causes for knee joint effusions in children. Rheumatoid diseases and hematogenous septic arthritis are the most important differential diagnoses. It is less known that immunodeficiency syndromes may cause serous monarthritis. RESULTS: A case of a five years old boy with monarthritis of the knee is presented. Blood assay and specific immunologic analysis revealed a so far unknown X-linked agammaglobulinemia (M. Bruton). CONCLUSIONS: In case of a monarthritis in children the orthopaedic surgeon or the pediatrician is primarily involved. For differential diagnosis X-linked agammaglobulinemia should be considered as reason for monarthritis, especially, if there are other clues to that disease in the medical history. In the case of agammaglobulinemia effusion and swelling will decrease soon after treatment with intravenous gammaglobulin.

Agammaglobulinemia↗
PubMedDate not supplied

Does long-term home parenteral nutrition in adult patients cause chronic liver disease?

Sixty patients with gut failure were treated with home parenteral nutrition for 2000 patient months. Fifty-one of these 60 patients had either no abnormalities or mild and transient elevations of their liver chemistries and did not have liver biopsies. Nine (15%) of 60 patients had abnormalities of liver tests that persisted from 8 to 95 months (median, 18 months) which prompted one or more liver biopsies per patient. Three patients had prolonged jaundice, one died of hepatic encephalopathy, and another with protracted intrahepatic cholestasis died following a biliary tract exploration. A third patient remains ill with signs and symptoms of chronic liver disease. Steatohepatitis was found in eight of the nine patients and was characterized by centrilobular and midzonal microvesicular and macrovesicular fatty changes with fat cysts, focal necrosis, and mixed inflammatory infiltrates. Centrilobular fibrosis was present in three patients and evidence of nodular regeneration in one. In the three patients demonstrating cholestasis, bile pigment was identified both in hepatocytes and canaliculi. Ceroid pigment in Kupffer cells was a consistent finding and much more severe than expected from the mildness of the hepatitis. Persistent abnormalities of liver chemistries in nine patients and progressive liver disease while receiving home parenteral nutrition in three patients are quite worrisome and suggest that home parenteral nutrition-associated steatohepatitis with or without cholestasis may progress to chronic liver disease.

Adult↗