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G Ganser

Publications and source records attributed to G Ganser.

16 recordsLinked to original sources

Arthrosonography of hip and knee joints in the follow up of juvenile rheumatoid arthritis.

OBJECTIVE: To evaluate sensitivity of arthrosonography of hip and knee joints for monitoring disease activity in juvenile rheumatoid arthritis (JRA). METHODS: Twenty eight patients with JRA with active disease at entry in 15 hips and 38 knee joints were followed up three times in intervals of 4-6 weeks. Sonographic, clinical, and laboratory findings were documented at the same time in clinically active and inactive disease. As controls of the sonographic variables 10 children without a history of arthritis were examined by ultrasound. RESULTS: In active arthritis of the hip joint 19/31 (61%) examinations showed a pathological widening of the synovial joint space. There was no significant correlation between sonographic and clinical measures of disease activity in coxitis. Marked effusion within the suprapatellar pouch was seen in 87% and thickening of the synovial membrane in 92% of cases of active gonarthritis in patients with JRA. There was a significant difference in the number of patients with joint effusion and in the mean joint effusion between patients with clinically active gonarthritis at entry and inactive arthritis at follow up (p<0.001). In contrast, synovial thickening persisted in about 80% after induction of clinical remission. CONCLUSION: The data confirm the high sensitivity of arthrosonography in imaging changes in hip and knee joints of patients with JRA. Sonographic effusion of the knee provided the highest correlation with measures of clinical disease activity. Further prospective studies should evaluate whether persistent thickening of the synovial membrane detected by ultrasound in clinically inactive arthritis indicates residual inflammatory activity and an increased risk of relapse.

Arthritis, Juvenile↗

Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey.

OBJECTIVE: To increase the current knowledge of the outcome of juvenile systemic sclerosis (jSSc), which is currently limited. METHODS: In order to investigate the patient outcome and prognostic factors, starting October 1994, we distributed questionnaires to 324 paediatric rheumatology centres. RESULTS: Until 15 May 1998 responses from 46 centres were received, 34 of which returned completed questionnaires on a total of 135 patients. One hundred and twenty-two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (S.D. +/- 3.3 yr). The mean disease duration at the last follow-up was 5 yr(S.D. +/- 3.3 yr). At the last follow-up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per cent of the living patients were fully active in daily life at the last follow-up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7-15.8 yr). The median disease duration until death was 2 yr (range 1-8 yr). The causes of death were heart failure (five), renal failure (one), sepsis (one) and in one case the cause was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and the 4 yr was 95%. CONCLUSIONS: At a mean follow-up of 5 yr, the current results show a favourable outcome in most patients with childhood onset jSSc and a significantly better survival than in the adult SSc patients.

Adult↗

Decreased prorenin processing develops before autonomic dysfunction in type 1 diabetes.

It is well documented that diabetic patients with chronic complications have decreased renin secretion and elevations in the renin precursor prorenin. It is uncertain, however, whether the abnormal processing of prorenin is reflective of microvascular disease, hypertension, or autonomic neuropathy. Dechaux et al. (Transplant Proc. 18:1598-1599, 1986) observed abnormalities in prorenin processing in uncomplicated diabetes and suggested that it was the result of subclinical autonomic neuropathy. To test this hypothesis, we measured renin, prorenin, and autonomic function in early type 1 diabetes at a time when there is little or no microvascular disease or hypervolemia. Thirty-seven patients (10 males, 27 females) enrolled 2-22 months after diagnosis in a longitudinal study in which renin, prorenin, and autonomic function were measured annually for 3 years. Forty-one age-matched control subjects were also studied. PRA in the diabetic patients at the time of the second and third evaluations was 1.71 +/- 0.24 ng angiotensin I/mL x h and 1.67 +/- 0.24 ng angiotensin I/mL x h, respectively, significantly lower (P < 0.05) than that of the control subjects in whom PRA was 2.96 +/- 0.38 ng angiotensin I/mL x h. Prorenin was not different in the diabetic patients in comparison with controls. The renin to prorenin ratio in the diabetic patients at the time of the first, second, and third evaluations was 0.260 +/- 0.03, 0.235 +/- 0.05, and 0.227 0.05, respectively, significantly lower (P < 0.01) than in control subjects in whom the renin to prorenin ratio was 0.475 +/- 0.08. Despite this, at the time of the first and second evaluations, there was no evidence of autonomic dysfunction and no correlation between any test of autonomic function and the renin to prorenin ratio. At the time of the third evaluation, however, the intermediate frequency (0.04-0.15 Hz) power spectra while patients were supine (an index of sympathetic modulation of heart rate variability) showed a highly significant (P < .001) correlation with the renin to prorenin ratio. High frequency (0.15-0.40 Hz) spectra from supine patients at the third evaluation also correlated with the renin to prorenin ratio (P < 0.01). We conclude abnormal processing of prorenin develops in diabetic patients prior to microvascular disease, even before the first evidence of autonomic dysfunction. Although the latter may play a contributory role, additional as yet unidentified mechanisms seem to interrupt the processing of prorenin in early diabetes.

Adolescent↗

Antibodies to glutamic acid decarboxylase and peripheral nerve function in type 1 diabetes.

Autoimmune mechanisms have been implicated in the pathophysiology of diabetic neuropathy. We studied the association between glutamic acid decarboxylase (GAD65) and islet cell (IA-2) autoantibodies as well as autoantibodies to the autonomic nervous system and peripheral nerve function in recent onset type 1 diabetes. Thirty-seven patients (27 females and 10 males) enrolled 2-22 months after diagnosis. Humoral factors, glycemic control, and peripheral nerve function were measured annually for 3 yr. Patients with high GAD65Ab had worse glycemic control and higher insulin requirements. Patients with high GAD65Ab had slower motor nerve conduction velocities in the median, ulnar, and peroneal nerves (P < 0.025 for each nerve). The mean motor nerve conduction velocity Z scores at the time of the third evaluation was 0.341 +/- 0.25 for the low GAD65Ab patients and -0.600 +/- 0.25 for the high GAD65Ab patients (P < 0.01). Similar differences between the low and high GAD65Ab groups were observed for F wave latencies, thermal threshold detection, and cardiovascular autonomic function. The composite peripheral nerve function Z scores in the low GAD65Ab patients were 0.62 +/- 11, 0.71 +/- 0.19, and 0.21 +/- 0.14 at the first, second, and third evaluations, significantly different from those in the high GAD65Ab patients in whom they were -0.35 +/- 0.15, -0.46 +/- 0.18, and -0.42 +/- 0.16 (P < 0.001). In summary, GAD65Ab in patients with recent onset type 1 diabetes are associated with worse glycemic control and slightly worse peripheral nerve function. Although the latter remained within normal limits and none of the patients had clinical neuropathy, the GAD65Ab-related differences in composite peripheral nerve function were highly significant (P < 0.001) and could not be attributed to GAD65Ab-related differences in glycemic control.

Adolescent↗

Twenty-four hour intraesophageal pH monitoring in children and adolescents with scleroderma and mixed connective tissue disease.

OBJECTIVE: To investigate esophageal dysmotility and gastroesophageal reflux, which are important, but often occult features of gastrointestinal (GI) involvement in children and adolescents with mixed connective tissue disease (MCTD) and scleroderma. METHODS: We carried out intraesophageal 24 h pH monitoring of 14 patients with scleroderma and MCTD. RESULTS: We observed an elevated reflux index in 64% of the patients. An increased number of reflux events was found in 85%. Reflux events > 5 min were noted in 50% of the patients, which is indicative of possible development of esophagitis. Only 3 patients presented clinical symptoms. Four of 5 patients with localized scleroderma revealed a pathological reflux index. We found no relationship between gastroesophageal reflux, age of patients, duration of disease, and Raynaud's phenomenon. CONCLUSION: GI involvement in children with scleroderma or MCTD is more frequent than clinical symptoms indicate. An active diagnostic investigation for GI dysmotility and gastroesophageal reflux is necessary to detect these complications.

Adolescent↗

A subgroup-specific evaluation of the efficacy of intraarticular triamcinolone hexacetonide in juvenile chronic arthritis.

OBJECTIVE: To determine the subgroup-specific differences of intraarticular triamcinolone hexacetonide (TH) in the treatment of joint inflammation in patients with juvenile chronic arthritis (JCA). METHODS: A retrospective review of 194 children of all subgroups of JCA, treated by a single or repeated TH injection between 1989 to 1994. Efficacy and duration of benefit were evaluated after a mean duration of 3, 15, 30, and 64 weeks. RESULTS: In all, 1439 TH injections were given to 194 patients; 368 of these were reinjections. The median duration of improvement of all injections was 74 weeks. Responses were significantly different among subgroups (p = 0.0001): there were 121 weeks of efficacy in early-onset pauciarticular JCA type I (223 injections), 47 weeks in late-onset pauciarticular JCA type II (190 injections), 105 weeks in rheumatoid factor negative polyarticular JCA (445 injections), 63 weeks in rheumatoid factor positive polyarticular JCA (127 injections), and 36 weeks in systemic JCA (413 injections). Forty-one injections were done in other rheumatic diseases. In relation to this result there were also differences with regard to joint groups, antinuclear antibody (ANA) and HLA-B27 status, and sex. Side effects were rare: infections of skin or joints were not noted; skin and lipoatrophy were seen after 15 injections, necrosis of the hip in one case, luxation of 2 shoulders of one patient, and periarticular calcification in 3 patients. CONCLUSION: Intraarticular TH is an effective therapy for inflammatory joint disease in all subgroups of JCA. The risk of major complications is low. The median duration of improvement depends on the subgroup of the disease.

Administration, Topical↗

Diagnosis of paediatric Lyme arthritis using a clinical score.

UNLABELLED: Diagnosis of Lyme arthritis (LA) in children and adolescents may be difficult due to non-specific clinical manifestations and unreliable serological tests for antibodies to Borrelia burgdorferi. In a national prospective study, 186 children with arthritis were examined in whom the attending physicians had considered the diagnosis of LA. Ultimately, LA was confirmed in 87 patients and these were compared with the remaining 99 children in whom arthritis was attributable to other causes. In comparison to patients with other causes of arthritis, patients with LA had a higher frequency of episodic arthritis and initial knee joint arthritis, reported tick bites more frequently, were older, had a lower frequency of initial arthralgias, and there were fewer large joints involved. A score was developed in a group of these patients and tested in a second group. It enabled patients with LA to be distinguished from those with other causes of arthritis: within a range from 12 to -7 points, a score of 2.5 or less excluded LA whereas 6 or more points were highly indicative of LA. If only those children with a score result between 2.5 and 6 had been tested for antibodies to B. burgdorferi, the number of tests would have been reduced by 63%. CONCLUSION: Careful analysis of clinical presentation and use of a clinical score may help in distinguishing LA from other causes of arthritis and thus reduce unnecessary and expensive testing and uninterpretable test results.

Adolescent↗

Lyme arthritis in European children and adolescents. The Pediatric Rheumatology Collaborative Group.

OBJECTIVE: To evaluate and describe Lyme arthritis in European children and adolescents. METHODS: This was a prospective multicenter study. The diagnosis of Lyme arthritis required the exclusion of other diseases and positive findings on serology for IgG antibodies to Borrelia burgdorferi. Enzyme-linked immunosorbent assay, immunoblotting, and polymerase chain reaction techniques to identify infection by B burgdorferi were used. RESULTS: Among 62 children and adolescents with Lyme arthritis, only 1 had a preceding erythema migrans. Arthritis was episodic in 62% and was chronic at onset in 18%. The most common manifestation was monarthritis of the knee. Joint involvement in patients with oligoarthritis was predominantly unilateral or symmetric. Arthralgia was very rare. Treatment with 1 or 2 courses of different antibiotics resulted in disappearance of the arthritis in 77% of the patients. CONCLUSION: The clinical presentation of Lyme arthritis in children is different from that in adults. The calculated incidence of Lyme arthritis in persons under the age of 17 years (4/100,000) exceeds previous estimations.

Adolescent↗

Naproxen-induced pseudoporphyria: appearance of new skin lesions after discontinuation of treatment.

Non-steroidal antiinflammatory drugs (NSAIDs) are routinely used in the therapy of chronic inflammatory joint diseases in childhood. Recently the NSAID naproxen was recognized to induce pseudoporphyria. This rare photodermatitis is characterized by skin fragility and vesiculation, resulting in shallow scarring. We report 4 children with juvenile rheumatoid arthritis who developed naproxen-induced pseudoporphyria. All children had received naproxen for more than 5 months when pseudoporphyria occurred. A disorder of porphyrin metabolism was excluded by analysis of the urine, serum and erythrocytes. Previous reports on naproxen-induced pseudoporphyria described a rapid disappearance of blisters after discontinuation of treatment. However, in our patients, new lesions appeared for up to 5 weeks after discontinuation of the therapy and skin fragility was apparent for up to 6 months after cessation of treatment. Since naproxen is a widely used drug in the treatment of children with juvenile rheumatoid arthritis parents of fair-skinned children should be alerted to the possibility of this rare adverse effect.

Arthritis, Juvenile↗

[Acute focal bacterial nephritis in childhood].

The clinical findings and sonographic observations in four patients with AFBN in childhood are described. AFBN in childhood is an acute interstitial nephritis presenting with septicaemia. The patients show a rapid deterioration of condition, weight loss, flank pain and often leucocyturia without detection of bacteria. The diagnosis is confirmed by renal sonography, showing typical focal alterations. Sonographic follow-up is important to prove the diagnosis. Even without detection of bacteria intravenous broad-spectrum antibiotic therapy is required. The antibiotic should be active against gram-negative organisms and Staphylococci. Medication should be given for 2-3 weeks. After adequate treatment the clinical condition will improve within a few days whereas sonographic alterations return to normal after 2-4 weeks. In childhood, a bacterial infection of other organs preceding AFBN is more common than anomalies of the urinary tract as predisposing factors. Therefore in most cases a hemotogenous infection must be presumed and its focus discovered and eliminated by appropriate antibiotics since otherwise AFBN may recur.

Acute Disease↗

[Unilateral pseudotumor of the orbit--an autoimmune disease?].

An eleven-year-old boy with congenital paresis of the left superior rectus eye muscle developed symptoms of acute left orbital expansion: pain, ptosis, redness, extraocular muscle dysfunction. Computer assisted tomography of the orbit showed typical signs of inflammatory orbital pseudotumor and immunological screening cryoglobulinemia. Short-term treatment with prednisone (2 mg/kg bodyweight/day) rapidly improved symptoms. However several relapses occurred following discontinuation of therapy. Therefore a long-term treatment with corticosteroids was instituted. The simultaneous development of inflammatory orbital pseudotumor and cryoglobulinemia support the hypothesis that orbital pseudotumor is an autoimmune disease. The meaning of the congenital superior rectus muscle paresis in this case for the development of the inflammatory process remains uncertain.

Autoimmune Diseases↗

[Leg edema].

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Adult↗

[Body complaints and neuroticism in pediatric patients with rheumatism].

BACKGROUND: Pain and aches are part of subjective experience. Caring for chronically ill children and adolescents not only objective results but also the children s subjective perception of their illness should be taken into consideration. We have tried to record the extent of subjective suffering of a group of pediatric rheumatic patients and to correlate our findings with personality traits. PATIENTS AND METHOD: We interviewed thirty-one 8 to 18 years old children and adolescents (average age 12 years) by means of a standardized questionnaire (Giessener Beschwerdefragebogen) and of a standardized personality questionnaire (HANES,KJ). The tested group comprised of 9 boys and 22 girls. 14 patients suffered from a chronic oligoarthritis, 9 from a chronic polyarthritis, 8 from a other rheumatic disease. Percentiles of more than 89 percent have been considered significant compared to norm collectives. RESULTS: 28% of the patients showed augmented values on the scales "exhaustion" and "intestinal pain", 25% on the scale "pain in joints". Only 3 respectively 2 patients showed augmented values on the scales "heart" and "symptoms of cold". 28% showed augmented values on the overall scale "feeling of discomfort". 35% showed augmented values on neuroticism, 61% values > 89. percentile on the scale "extraversion". There is a significant connection between augmented strong feelings of discomfort and augmented values of neuroticism on the level 0.1%. A context to the duration of the illness was not found. CONCLUSION: Experience of physical pains is not only limited to isolated symptoms of the basic illness but also associated with general features of personality.

Abdominal Pain↗