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  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric radiology 24 (1994), S. 145-146 
    ISSN: 1432-1998
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract A 7-year-old girl with an unremarkable family history was admitted with complaints of diarrhea containing mucus and blood, abdominal pain, weight loss and fever for 10 months. Although her symptoms, physical examination and laboratory results were highly suggestive of inflammatory bowel disease, radiologic studies and colonoscopy demonstrated multiple colonic polyps. A bilateral and symmetric lamellar periosteal reaction and osteopenia were present on her extremity radiographs. The chest radiograph and thoracic CT scan were normal. The histologic nature of the polyps was determined as hamartoma. One month after subtotal colectomy, the patient's symptoms resolved and she gained 7kg in weight. The association of generalized juvenile polyposis and hypertrophic osteoarthropathy has previously been described in five cases; arteriovenous malformations were present in four of them. In our case the polyps were hamartomatous and localized in the colon, without associated arteriovenous malformation.
    Materialart: Digitale Medien
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  • 2
    ISSN: 1432-1076
    Schlagwort(e): Key words Familial Mediterranean fever ; Childhood ; Amyloidosis ; Prognosis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Familial Mediterranean fever (FMF) is a genetically transmitted disease characterized by recurrent attacks of fever and serositis. The most important complication of this disease is the development of amyloidosis. We present our analysis of 425 FMF patients without and 180 with amyloidosis (123 FMF having amyloidosis type I and 57 FMF having amyloidosis type II). The male/female ratio was higher in the amyloidosis population (111/69) when compared to the FMF population (225/200) (P = 0.048). Consanguinity rate was the same among FMF and amyloidosis groups. However, a family history of amyloidosis was significantly more frequent in the amyloidosis group (P = 0.00001). Multivariate analysis has revealed that in FMF patients, the presence of a family history of amyloidosis plus consanguinity has a 6.04 fold increased risk of amyloidosis (P 〈 0.0001). The 5-year chronic renal failure free survival was 43.1% and 18.7% in type I and type II amyloidosis, respectively. The time interval to develop chronic renal failure after the development of amyloidosis was 4.8 in type I and 3.0 years in type II, respectively. We found ten cases of Henoch-Schönlein Purpura and nine of polyarteritis nodosa among our patients. The significance of the association between FMF and vasculitis awaits to be clarified. Among the FMF patients put on colchicine therapy (435), only 10 (2.3%) have developed amyloidosis confirming that this drug protects from amyloidosis. Conclusion Since the presence of a familial history of amyloidosis has been defined as the most important risk factor in the development of amyloidosis, we suggest that additional genetic factors may be operative in the development of amyloidosis.
    Materialart: Digitale Medien
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  • 3
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric nephrology 10 (1996), S. 40-40 
    ISSN: 1432-198X
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric nephrology 14 (2000), S. 325-327 
    ISSN: 1432-198X
    Schlagwort(e): Key words Renal involvement ; Polyarteritis nodosa
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract  Renal involvement is common in childhood polyarteritis nodosa (PAN). We report a retrospective analysis of the presentation and clinical course of 26 patients with PAN and renal involvement. The mean age was 9.3 years (range 1–14 years) and there were 12 boys and 14 girls. Renal symptoms at presentation were as follows: 3 had isolated proteinuria, 9 had nephritic syndrome, 2 had nephritic and nephrotic components, and 10 had renal failure with one of the above features. Two patients with isolated hypertension were diagnosed by angiography and classified as classical PAN. Patients either received prednisone p.o. alone (n=9), or prednisone plus cyclophosphamide p.o. (n=11), or pulse steroids with prednisone p.o. and cyclophosphamide (n=2); 4 did not receive any treatment. Patients who were given cyclophosphamide had a significantly better outcome than those who did not. We suggest that oral cyclophosphamide therapy and corticosteroids are effective in the treatment of PAN. The overall 1-year and 5-year survival rates of the patients were 72.5% and 60%, respectively. In conclusion, renal disease is a serious manifestation of PAN necessitating prompt and aggressive treatment.
    Materialart: Digitale Medien
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  • 5
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric cardiology 15 (1994), S. 151-153 
    ISSN: 1432-1971
    Schlagwort(e): Echocardiography ; Juvenile rheumatoid arthritis ; Valvular involvement
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Valvar heart disease is a rare complication of juvenile rheumatoid arthritis (JRA), the aortic valve being most commonly affected. Reported cases with symptomatic mitral involvement are rare. We describe a 13-year-old boy with seronegative, polyarticular onset of JRA in whom mitral and aortic valve insufficiency was diagnosed by clinical and laboratory investigations. Two-dimensional and continuous-wave Doppler echocardiography confirmed mild pericardial effusion with moderate mitral and mild aortic insufficiency. Cardiac assessment and echocardiogrphic follow-up are recommended in all patients with JRA.
    Materialart: Digitale Medien
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  • 6
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric radiology 15 (1985), S. 255-258 
    ISSN: 1432-1998
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Two cases of carpal-tarsal osteolysis are described. It is believed that clinical and radiological findings of the patients are compatible with the dominant type of the disease. Although case 2 does not have the indications of nephropathy, it is rather too early to decide whether he has it or not, because of his age.
    Materialart: Digitale Medien
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  • 7
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 153 (1994), S. 469-469 
    ISSN: 1432-1076
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 8
    ISSN: 1432-1076
    Schlagwort(e): Key words Complement  ;  C1q deficiency  ;  Point mutation  ;  Systemic lupus erythematosus
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract  A 7-year-old male with recurrent erythematous and desquamated skin lesions and respiratory infections was diagnosed as selective complete C1q deficiency following detailed studies of the complement system. His asymptomatic sister also had selective complete C1q deficiency. During a follow up period of 3 years, his skin lesions persisted, he suffered from recurrent bronchopneumonias and glomerulonephritis developed. Renal function deteriorated with the appearance of anti-DNA antibodies. Renal biopsy was consistent with systemic lupus erythematosus. The patient was treated with immunosuppressive drugs, but died of renal failure. It is postulated that in this patient defective clearance of antigen-antibody complexes by the reticulo- endothelial system resulted in progressive renal disease as observed in other complement deficiencies. A retrospective molecular study disclosed a point mutation in the ClqA chain gene in a heterozygous state in parents and two siblings; in a homozygous state in the asymptomatic sister. The reason why some individuals with this defect are asymptomatic is not known at present. Diagnosis of heterozygotes by molecular studies is extremely important to give genetic counselling to the family. Conclusion Patients with recurrent infections, ery-thematous desquamative skin lesions, malar rash and oral mucosal involvement should be screened for complement Clq deficiency.
    Materialart: Digitale Medien
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  • 9
    Digitale Medien
    Digitale Medien
    Springer
    Clinical rheumatology 15 (1996), S. 341-345 
    ISSN: 1434-9949
    Schlagwort(e): Juvenile Chronic Arthritis ; Lipoprotein Levels
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Altered levels of high density lipoprotein (HDL), low density lipoprotein (LDL), and very-low density lipoprotein (VLDL), as well as apolipoproteins have been previously described in rheumatoid arthritis patients. We have attempted to evaluate the serum triglyceride, total cholesterol, cholesterol in DHL, LDL, apolipoprotein A1 (apo-A1) and apolipoprotein B (apo-B) levels in juvenile chronic arthritis (JCA) and to correlate them with CRP and ESR in the active and non-active stages of JCA. A total of 37 children who fulfilled ARA criteria for the diagnosis of JCA were studied. There were 18 girls and 19 boys. Age range was 2.5–16 years with a mean of 9.5. The mean duration of disease was 1.8 years. Nineteen patients were accepted to have active disease. Eighteen age and sex matched healty children served as controls. Apo-A1 was significantly lower in the active JCA group when compared to inactive patients and healthy controls (both p〈0.05). There were significant inverse correlations between apo-A1 and both ESR and CRP levels in these patients (r=0.67, p〈0.05 and r=−0.61, p〈0.–05, respectively). Although mean LDL levels were numerically lower in the JCA patients (67.2 mg/dl in the active and 68.6 mg/dl in the inactive patients) the difference with healthy controls (91.7 mg/dl) was not statistically significant. There was no significant differences in regard to triglyceride, total cholesterol, cholesterol in HDL, and apo-B levels between neither of the groups. We conclude that JCA patients have a dyslipoproteinaemic state with already altered metabolism of lipids at different stages of the chronic inflammation from active to inactive disease.
    Materialart: Digitale Medien
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  • 10
    Digitale Medien
    Digitale Medien
    Springer
    Clinical rheumatology 16 (1997), S. 173-178 
    ISSN: 1434-9949
    Schlagwort(e): Juvenile Chronic Arthritis ; Interleukin-1 ; Interleukin-6 ; Interleukin-8
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The immunoinflammatory pathogenesis of juvenile chronic arthritis (JCA) involves the activation of many pathways including various cytokines. We have evaluated the levels of interleukin (IL)-1, IL-6 and IL-8 in 29 JCA patients. The age range was 1–16 with a mean of 10.1. A disease activity score was developed on the basis of: 1.constitutional symptoms and/or morning stiffness, 2.presence of joint swelling, 3.warmth, 4.limited range of motion, and 5.joint pain. This score correlated very significantly with laboratory disease activity markers such as erythrocyte sedimentation rate (ESR) and CRP (both p=0.006) and also correlated with IL-1 and IL-6 levels. The levels of IL-1 decreased in four of the five patients with improved disease activity. IL-6 but not IL-1 correlated significantly with the number of inflamed joints (p=0.013); IL-6 also strongly correlated with rheumatoid factor supporting this cytokine's role in B cell induction (p=0). Haemoglobin values correlated negatively with the activity index, ESR, CRP, IL-1 and IL-6. IL-8 did not correlate with disease activity markers. In the systemic patients all cytokines tended to be higher. Our data suggest that interleukins 1 and 6 are effective in the pathogenesis of JCA. Whether cytokines may be used for monitoring therapy may be clarified with further studies.
    Materialart: Digitale Medien
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