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Biomedical subjects

G Kreuzpaintner

Publications and source records attributed to G Kreuzpaintner.

At least 19 recordsLinked to original sources

[Fever and exanthema after trip to the Mediterranean area--Mediterranean spotted fever (Boutonneuse fever)].

HISTORY AND CLINICAL FINDINGS: In connection with a period of residence in the Mediterranean basin (Portugal, Spain, Sicily) one woman and two men (age: 28, 31 and 40 years) got sick with fever in August and in the midst of October. In addition, they complained about headache in two cases and myalgias, arthralgia and vomiting in one case, respectively. Two of the vacationers (cases 2 and 3) had been accompanied by their dogs. One of them (case 2) was bitten of a tick. The woman (case 3) removed few days after vacation-beginning ticks from her dogs. Patients 1 and 2 appeared severely ill and presented with high fever and generalized maculopapular rash which included also the soles and palms. Patients 2 and 3 had a primarily-lesion ("eschar"). In patient 3 the eschar was pustular and associated with regional lymphadenitis. INVESTIGATIONS: All three patients showed an increased erythrocyte sedimentation rate and elevated liver enzymes. Cases 1 and 2 presented with a significantly elevated activity of lactate dehydrogenase, leukocyturia and microhematuria. Indirect immunofluorescent assay for specific antibodies directed against Rickettsia conorii revealed titers between 1:40 and 1:640 (normal: negative). DIAGNOSIS, TREATMENT AND COURSE: Diagnosis was based on the triad fever, rash and eschar as well as on epidemiological data which include a recent period of residence in the Mediterranean basin during the summer and contact with a dog. In case 1 diagnosis was difficult because of the absence of an eschar. This patient had been treated with penicillin for two days without success. Only the parenteral administration of ciprofloxacin caused complete defervescence and clinical improvement within two days. A complete defervescence within two days was reached with doxycycline also in case 2. In comparison to these cases, the course in the third patient was mild, and the patient defervesced spontaneously. CONCLUSION: Mediterranean spotted fever should be considered in the case of unclear fever and rash following a period of residence in the Mediterranean basin during summer time. An eschar may confirm this diagnosis, and early start of therapy may shorten the course of the disease and prevent complications.

Adult↗

Two large liver abscesses complicating Crohn's disease.

A 20-year-old woman with active Crohn's disease had an abrupt onset of high fever accompanied by an elevation of serum gamma-glutamyltransferase and alkaline phosphatase. Her past medical history included a course of corticosteroid therapy for 7.5 months and a resection of the terminal ileum and the cecum 2 months before admission. At that time an ileoascendostomy had been performed, revealing a walled-off perforation of the colon into the retroperitoneal space. Sonography revealed 2 large abscesses in the right lobe of the liver. After beginning antibiotics, ultrasound-guided percutaneous aspiration and drainage with a pigtail catheter were performed for both abscesses leading to a rapid reduction of their size and an improvement in the patients general condition. Liver abscess represents a rare complication of Crohn's disease. A review of the literature is presented.

Adult↗

Eosinophils, eosinophil cationic protein and eosinophil-derived neurotoxin in serum and urine of patients with onchocerciasis coinfected with intestinal nematodes and in urinary schistosomiasis.

Eosinophils, eosinophil cationic protein (ECP), eosinophil-derived neurotoxin (EDN/EPX), myeloperoxidase (MPO) and IgE were measured in blood, serum and/or urine in Schistosoma haematobium- and Onchocerca volvulus-infected Guineans and O. volvulus- and S. haematobium-negative Guineans coinfected or infected with intestinal nematodes. The number of eosinophils and levels of eosinophil granule proteins but not of MPO were found to be strongly elevated in all Africans as compared to European controls. The highest serum ECP and serum and urinary EDN/EPX levels were observed in the hyperreactive form of onchocerciasis (sowda). Onchocerciasis patients and O. volvulus-negative Africans coinfected or infected with intestinal nematodes (hookworm and/or Ascaris lumbricoides) revealed higher serum granule protein concentrations and/or absolute eosinophil counts and urinary ECP than those without nematode infections. Statistical differences between both sections were found for the absolute eosinophil counts and for serum EDN/EPX and IgE in generalized onchocerciasis, and for urinary ECP in sowda, indicating stimulation of the eosinophil potential of O. volvulus-positive patients by coexistent hookworm infection. This worm species, in contrast to A. lumbricoides, causes especially high eosinophil counts and EDN/EPX and IgE levels. From these results it is concluded that in nematode diseases, ECP and EDN/EPX levels reflect the degree of antigenic stimulation, eosinophil activation and eosinophil turnover rates. Serum ECP and serum and urinary EDN/EPX may, therefore, serve as parameters to monitor helminth infection. Urinary ECP may be a marker of eosinophiluria secondary to urogenital manifestation of S. haematobium. It is elevated in hyperreactive onchocerciasis activated by intestinal nematodes.

Adolescent↗

[Recurrent immune thrombocytopenia: a rare complication after contrast medium injection].

HISTORY AND CLINICAL FINDINGS: In a 66-year-old woman with unstable angina, treated with 20,000 IU heparin daily for 6 days, platelet count fell dramatically from 310,000 to 1000/microliters 8 hours after injection of 90 ml of contrast medium (Iopromide) during coronary angiography. In addition to a marked tendency towards spontaneous bleeding she developed a large haematoma at the site of the arterial puncture, with a fall in haemoglobin to 9 g/dl, and acute renal failure. TREATMENT AND COURSE: Red blood cell and platelet infusions were given, together with cortisone, 1000 mg, and immunoglobulins. Platelet count returned to within normal limits after 8 days. Two haemodialyses initiated polyuria, followed by rapid normalization of kidney function. No antibodies against iopromide, iopamidol, heparin or heparinoids were found. At an emergency coronary balloon angioplasty 3 weeks later iopamidol was injected (45 ml). Again there was a profound fall in platelets to 1000/microliters, associated with acute renal failure. Treatment identical to that after the first episode brought about complete normalization. CONCLUSION: The reported reactions were most likely due to immune thrombocytopenia after administration of contrast medium.

Acute Kidney Injury↗

Mycobacteria of Runyon groups I, II and IV do not play an aetiological role in Crohn's disease.

OBJECTIVE: In addition to Mycobacterium paratuberculosis, other slow-growing as well as rapid-growing mycobacteria were isolated from mucosa or full thickness samples of intestine from patients with Crohn's disease. The meaning of these data remained unclear. To investigate the possible aetiological role of these rapid- and slow-growing mycobacteria, serosa and mesenteric lymph nodes were also cultivated in the present study. DESIGN AND METHODS: Mucosa, lymph nodes and serosa of 23 patients with Crohn's disease and 23 patients with other intestinal afflictions were incubated at 37 degrees C on Löwenstein-Jensen medium and Herrold egg yolk medium. These methods allow the cultivation of most atypical mycobacteria, including Mycobacterium paratuberculosis. In addition, full thickness samples of some of these patients were analysed for mycobacterial DNA using polymerase chain reaction. RESULTS: Despite lack of decontamination of 60% of lymph nodes and serosa, no mycobacterial growth was observed over a long incubation period (an average of 718 days in Crohn's disease and 552 days in controls) on Löwenstein-Jensen medium. Polymerase chain reaction was based on the amplification of 16S ribosomal DNA sequences specific for mycobacteria of tissues derived from four patients with Crohn's disease and one control was negative. CONCLUSIONS: These findings suggest that mycobacteria, such as M. fortuitum and M. chelonei, which are widespread in the environment, are not involved in the aetiology of Crohn's disease but, rather, should be considered as environmental opportunists.

Adolescent↗

Effect of intestinal resection on serum antibodies to the mycobacterial 45/48 kilodalton doublet antigen in Crohn's disease.

Interest in the role of mycobacterial infection in Crohn's disease has been revived by the cultural detection of Mycobacterium paratuberculosis in patients with Crohn's disease. This hypothesis was examined serologically using assays with high specificity for Crohn's disease. The effect of intestinal resection on serum antibodies specific for Crohn's disease was investigated with an immunoblot assay and an enzyme linked immunosorbent assay using the 45/48 kilodalton doublet antigen of Mycobacterium tuberculosis. Antibodies were detected in 64.7% of patients with Crohn's disease (n = 17), 10% of patients with ulcerative colitis (n = 10), 5% of patients with carcinoma of the colon (n = 20), and none of 10 healthy subjects with the immunoblot assay. Statistical comparison of the Crohn's disease patients with each control group resulted in p = 0.0000236. Immunoglobulin G was essentially unchanged 75 days (mean) after surgery. After more than 180 days, however, the antibody response was reduced in all of five patients studied, and was no longer demonstrable in two of them (40%). Simultaneously, the Crohn's disease activity index (CDAI) decreased. Both the high specificity of this assay for Crohn's disease and the diminished antibody response after intestinal resection in parallel with decreased CDAI support a mycobacterial aetiology of Crohn's disease.

Adolescent↗

[A hemolytic crisis with liver failure as the first manifestation of Wilson's disease].

An 18-year-old woman developed an acute haemolytic anaemia, acute transient renal failure and progressive hepatic failure. Coeruloplasmin and serum copper concentration were normal; a Kayser-Fleischer ring and any neurological symptoms were absent initially. Liver biopsy was contraindicated because of increased bleeding tendency. Wilson's disease was diagnosed only after the acute renal failure had regressed, on the basis of the urinary copper excretion (2890 micrograms/d, rising to 7330 micrograms/d after D-penicillamine administration). Progressive liver failure required transplantation. After it the patient quickly recovered and is now, two years later, free of disease. -This case demonstrates that Wilson's disease may be difficult to diagnose at the time of initial acute manifestation. But it can be recognized early from the pathognomonic low alkaline phosphatase and by calculation of free serum copper.

Acute Disease↗

Increased risk of bacterial endocarditis in inflammatory bowel disease.

PURPOSE: The purpose of this retrospective as well as prospective case-control study was to analyze a possible overrepresentation of inflammatory bowel diseases among patients with native valve endocarditis as well as the factors that predispose patients with inflammatory bowel disease to infective endocarditis. PATIENTS AND METHODS: Among 213 consecutive patients treated for proven native valve endocarditis, six (2.8%) had inflammatory bowel diseases (three with ulcerative colitis and three with Crohn's disease). Three patients with inflammatory bowel disease were from the retrospective group, and three were from the prospective group. The prevalence of inflammatory bowel diseases has been determined to be 0.0641% in the Düsseldorf area. RESULTS: On the basis of these data, a 44-fold overrepresentation of inflammatory bowel diseases among the 213 patients with endocarditis was calculated with a statistical significance of p much less than 0.001. CONCLUSIONS: Inflammatory bowel disease may be considered an independent risk factor for bacterial endocarditis. Reasons may be more frequent bacteremias as a result of the higher incidence of diagnostic and therapeutic interventions, as well as increased permeability of the damaged mucosa for bacteria and the therapeutic immunosuppression in patients with active inflammatory bowel disease. Prophylaxis for bacterial endocarditis should be carefully considered before expected bacteremias in patients with highly active inflammatory bowel disease even in the absence of cardiac factors predisposing to bacterial endocarditis.

Adolescent↗

[An increased incidence of bacterial endocarditis in chronic inflammatory bowel diseases].

Of 92 consecutive patients treated for proven native valve endocarditis three had ulcerative colitis and 2 Crohn's disease. All 5 patients developed severe complications; three had to undergo emergency valve replacement. With a prevalence of 64.1/10(5) cases of inflammatory bowel disease the calculated incidence (5/92) revealed a significant over-representation of inflammatory bowel disease among patients with proven endocarditis (p less than 5.08 x 10(-9)). Possible explanations may be the suppression of cellular immune defense by therapeutic interventions, high frequency of bacteremia caused by increased permeability of the damaged mucosa for bacteria and a higher incidence of diagnostic and therapeutic interventions in this patient population. Therefore, prophylaxis for bacterial endocarditis should be carefully considered before expected bacteremias in patients with highly active inflammatory bowel disease even in the absence of cardiac factors predisposing to bacterial endocarditis.

Adult↗

[Kidney function of patients with healthy kidneys during cyclosporin treatment].

Long-term administration of ciclosporin has been complicated by side-effects, the predominant being nephrotoxicity. We performed renal function studies on 20 patients treated with ciclosporin (group 1) and on 12 patients serving as controls (group 2). Only patients with serum creatinine less than 1.3 mg/dl entered the study. The renal function studies consisted of: Inulin clearance, PAH clearance, sodium sulphate loading, sodium bicarbonate loading. Plasma renin activity (PRA), inactive renin (IR) and aldosterone (ALDO) were measured basally and after stimulation with 40 mg furosemide i.v. Serum creatinine was not significantly impaired under ciclosporin with 1.1 +/- 0.1 mg/dl vs 0.9 +/- 0.1 mg/dl in the control group (ns). Glomerular filtration rates as measured by creatinine and inulin clearance were significantly impaired in group 1 as compared to group 2. Inulin clearance was impaired by ciclosporin with 93.5 +/- 4.4 ml/min/1.73 m2 as compared to 121 +/- 6.6 ml/min/1.73 m2 (p less than 0.05) in patients of group 2. The PAH clearance in ciclosporin treated patients was impaired, with 379 +/- 22.1 ml/min/1.73 m2 in group 1 as compared to 605 +/- 39 ml/min/1.73 m2 (p less than 0.001) in group 2. Mean arterial pressure and renovascular resistance were significantly increased in ciclosporin treated patients. We demonstrated, by means of sodium sulphate and bicarbonate loading, incomplete distal tubular acidosis in 3 patients from group 1 but in none of group 2. There was no difference in basal plasma renin activity (PRA), but during volume contraction induced by furosemide there was only blunted response by PRA in patients receiving ciclosporin with 2.7 +/- 0.3 ng/ml/h as compared to 7.7 +/- 0.5 ng/ml/h in controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Acid-Base Equilibrium↗

Wilson disease: clinical presentation, treatment, and survival.

OBJECTIVE: To evaluate the diagnostic features, clinical course, and overall long-term survival of patients with Wilson disease. DESIGN: Retrospective cohort study with a mean follow-up period of 14.2 years. SETTING: A university medical center and a community hospital. PATIENTS: Fifty-one consecutive patients with Wilson disease were evaluated between 1957 and 1989. INTERVENTIONS: Patients were treated with D-penicillamine (600 to 1800 mg/d). Two patients with end-stage liver disease had liver transplantation. MAIN RESULTS: Initial symptoms occurred at a mean age of 15.5 years. At diagnosis, the most common neurologic signs were dysarthria, tremor, writing difficulties, and ataxia followed by hypersalivation and headache. Somatic symptoms included abdominal pain, hepatomegaly, splenomegaly, cirrhosis of the liver, and thrombocytopenia. The mean serum concentrations of ceruloplasmin and copper were 44 mg/L and 4.7 mumol/L, respectively. The mean basal urinary copper excretion was 5.5 mumol/d, and the mean hepatic copper concentration was 19.6 mumol/g dry weight. Free serum copper concentration (mean, 2.7 mumol/L) was a reliable indicator of disease and was useful in assessing the effectiveness of therapy (values less than 1.6 mumol/L). Treatment with D-penicillamine improved most of the hematologic and neurologic abnormalities but had little effect on hepatomegaly and splenomegaly and did not reverse cirrhosis. Two patients died of fulminant hepatic failure during the observation period, whereas two others with end-stage liver disease had successful liver transplantation and remain asymptomatic. Long-term survival of patients with Wilson disease was similar to that of age- and sex-matched controls. CONCLUSION: Our results suggest that long-term treatment of patients with Wilson disease with D-penicillamine can relieve symptoms and improve prognosis.

Adolescent↗

Does orthotopic liver transplantation heal Wilson's disease? Clinical follow-up of two liver-transplanted patients.

Two patients with Wilson's disease (WD) underwent orthotopic liver transplantation, one for subacute liver failure and the other for severe oesophageal haemorrhage. After transplantation both patients fully recovered within five months, and copper metabolism returned to normal. Follow-up examinations were continued for 4 and 6 years. Clinical as well as electrophysiological testing in these two patients yielded better results than in most of 12 WD-patients being conventionally treated for a similar period or even longer.

Adult↗

[Twin pregnancy after liver transplantation].

Orthotopic liver transplantation was performed in a 29-year-old woman because of increasing decompensation of HBs-antigen positive post-hepatitic cirrhosis. Postoperatively she developed a mild rejection reaction and diabetes mellitus. Thirteen months after the transplant she conceived twins. This high risk pregnancy was complicated by a febrile viral infection with purulent tracheobronchitis at 9 weeks and a threatened abortion at 11 weeks. At 33 weeks there was a sudden drop in haemoglobin due to a minor uterine rupture which necessitated cesarean section. The female infants--of development in keeping with the dates--showed no clinical or ultrasound evidence of any malformations. Apart from initial difficulties--asphyxia (second twin), fluctuating glucose and calcium levels, an episode of neonatal jaundice which required phototherapy, reluctance to suck and hypotonia--the further development of both twins proceeded normally. The maternal diabetes disappeared after delivery, HBs-antigen remained negative and the HBs-antibody titre rose. The patient has remained in good condition, both mentally and physically.

Abortion, Threatened↗

Cushing's syndrome due to ACTH-production of an ovarian carcinoid.

The case of a 56-year old woman with severe Cushing's syndrome due to ovarian ACTH-production is described. Both clinical picture and biochemical pattern were consistent with the ectopic ACTH syndrome. ACTH was found by specific immunohistochemical staining in a carcinoid tumor of the patient's right ovary. In contrast, pituitary cells exhibited immunoreactive ACTH to only a minimum extent.

ACTH Syndrome, Ectopic↗

[Orthotopic liver transplantation in Wilson's disease and acute liver failure].

Liver histology demonstrated progressive cirrhosis in a 19-year-old girl with a subacute form of Wilson's disease. Despite D-penicillamine administration her liver functions rapidly deteriorated further. Orthotopic liver transplantation was performed. Postoperatively there were two mild rejection episodes, an organic psychiatric syndrome and generalized tremor. Copper metabolism and clinical symptoms became normal postoperatively. Five months after the transplantation she was in a good general condition, able to continue her education.

Acute Disease↗

Inactivation of C3a by a monocarboxypeptidase present in culture supernatants of stimulated guinea pig peritoneal macrophages.

Hog C3a, as well as its derivative C3a-desArg were not found to act cytotoxically on starch gel-induced guinea pig peritoneal macrophages. Likewise, neither peptide significantly modified the secretion of N-acetyl-beta-D-glucosaminidase from these cells. However, C3a rapidly lost its spasmogenic activity during incubation in serum-free macrophage cultures and less rapidly in cellfree supernatants collected from cultured macrophages. The following results indicate that C3a is converted into its spasmogenically inactive derivative C3a-desArg by a macrophage-derived monocarboxypeptidase. The inactivated C3a product does not differ from native C3a in sodium dodecyl sulfate-polyacrylamide gel electrophoresis; it elutes from CM cellulose in the same position as purified C3a-desArg; and it is devoid of the carboxyl-terminal arginyl residue of C3a, but still contains the carboxyl-terminal sequence of C3a-desArg as determined by analysis after treatment with carboxypeptidases B or Y. Furthermore, inactivation of C3a in supernatants of macrophage cultures is completely blocked by the specific carboxypeptidase inhibitors guanidinopropylsuccinic acid and 2-mercaptomethyl-3-guanidinoethylthiopropanoic acid in final concentrations of 10 mM and 2.1 mM, respectively. The monocarboxypeptidase is apparently supplied by biosynthesis of new material but is not stored as a preformed enzyme because cycloheximide markedly inhibits its expression.

Acetylglucosaminidase↗

Effect of cycloheximide and of anti-C3 Fab' on the intrinsic synthesis and secretion of lysosomal enzyme and of complement components by guinea-pig peritoneal macrophages.

Without any additional stimulus, the lysosomal enzyme N-acetyl-beta-D-glucosaminidase (beta-GLU) is secreted immediately from starch gel induced guinea-pig peritoneal macrophages. A more than three-fold increase of the total enzyme activity in the course of 3 days and the reversible inhibition of this enzyme increase by cycloheximide prove the synthesis of beta-GLU. The synthesis rate of beta-GLU remains rather constant after the second day, despite rapid cell death in the same culture period which could be explained by heterogeneity of the macrophage population. Cycloheximide immediately inhibits secretion of C3 whereas inhibition of beta-GLU secretion is only observed after a lag phase of 24 h. Secretion of factor D and of beta-GLU is not altered if secreted C3 is totally neutralized by the addition of anti-C3 Fab' to macrophage cultures. Thus, endogenous C3 as well as its endogenously generated fragments do not influence these macrophage functions. In the same cultures with anti-C3 Fab', conversion of secreted factor B into its fragments is inhibited as indicated by the detection of functional B activity. These results indicate that the secretion of factor D and of beta-GLU is also independent of endogenous B-derived fragments such as Bb. Finally, the detection of functional B in cultures with anti-C3 Fab' proves that C3b is required for factor B activation. The apparent interaction of secreted C3 and factors D and B in regular macrophage cultures suggests the constant formation of the labile C3 convertase C3bBb of the alternative pathway.

Acetylglucosaminidase↗

Functional active complement components secreted by guinea pig peritoneal macrophages.

In our studies on complement secretion functional C1, C4, C2, C3, P, D and B were clearly identified in the same cultures. Functional assays did not allow the detection of C5 to C9. Spontaneous C3 activation occurred at a very low level in culture supernatants. The responsible enzyme was identified as a metallo-enzyme. Upon addition of antibody-coated sheep erythrocytes (EA) to culture supernatant it was possible to induce C3 activation as indicated by the apparent formation of EAC1423. Zymosan was also able to activate C3 in culture supernatant after addition of purified functional factor B indicating efficient cooperation of factors of the alternative pathway. Thus in this in vitro system macrophages not only provide C3 but also all factors for spontaneous and induced C3 activation. If these secretory functions reflect in vivo properties of macrophages, our results may indicate that C3 and its activating systems are most relevant for local cooperation between macrophages and the complement system in inflammation and antimicrobial defense. Therefore availability of these essential factors at any time is secured by local production.

Animals↗