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Biomedical subjects

R Cimaz

Publications and source records attributed to R Cimaz.

At least 55 records · Page 3Linked to original sources
PubMed2000

Systemic onset juvenile idiopathic arthritis: a retrospective study of 80 consecutive patients followed for 10 years.

OBJECTIVE: To investigate the relationships between systemic onset juvenile idiopathic arthritis disease activity, course of the disease, and functional class according to Steinbrocker. METHODS: The records of all children with systemic onset juvenile arthritis (JA) according to the American College of Rheumatology criteria attending our center since 1971 with a minimum followup period of 3 years were reviewed. A cohort of 80 consecutive patients entered the study: 42 males, 38 females, mean age at onset 6.3 years (range 0.7-16), mean followup period 10.7 years (range 3-33). The cumulative duration of the active periods (CDAP) in months was calculated for every patient. RESULTS: Three patterns of disease course were apparent: monocyclic (subtype I), intermittent (subtype II), and persistent (subtype III). At the last control the functional class and disease activity status were evaluated. In all subtype I patients (9 cases) the disease was in remission and no patient was in class II, III, or IV. In subtype II patients (27 cases), 16 were inactive or in remission and 6 in class III. In subtype III (44 cases) 21 were inactive or in remission and 17 were in class III or IV. The equation relating the Steinbrocker class to the CDAP was calculated considering the functional outcome as the dependent variable. The linear regression equation y = 0.0083 x + 1.266 was found with a correlation coefficient r = 0.586 (p < 0.0001). The majority of our patients were treated with disease modifying antirheumatic drugs, which in many cases were effective in reducing the duration of the active phases of disease. CONCLUSION: Systemic onset JA may present with different clinical courses; the functional outcome is always good in subtype I (monocyclic), but can be poor in subtypes II and III. The severity of disability evaluated according to Steinbrocker classes is dependent on the cumulative duration of the active periods of the disease.

Adolescent↗
PubMed1999

Bone mass change during methotrexate treatment in patients with juvenile rheumatoid arthritis.

Thirty-two children affected by juvenile rheumatoid arthritis (JRA) were studied with serial measurements of bone mass for an average of 18 months, to evaluate the effects of long-term methotrexate (MTX) treatment on bone. Bone mineral density (BMD) was measured on lumbar spine and total body. During MTX therapy some increase in BMD was observed, though this was smaller than in a control group of healthy children. Axial (spine and trunk) and appendicular (upper and lower limbs) BMD showed similar increases. BMD, either as an absolute value or as a percent variation from baseline, did not correlate with either MTX dose or length of therapy. In children treated also with corticosteroids, these drugs negatively influenced bone mass increase. The main determinant of absolute spine BMD value appeared to be weight, while height and lean mass seemed to be the determinants of total body BMD. Pubertal stage and disease activity significantly influenced the yearly change in BMD. In conclusion, our data suggest that long-term, low-dose therapy with MTX does not induce osteopenia in children with JRA.

Adolescent↗
PubMed1999

Reflex sympathetic dystrophy in children.

Reflex sympathetic dystrophy is a syndrome characterized by pain in one or more extremities, usually associated with vasomotor changes. Its occurrence in childhood has long been thought to be rare. We describe six cases of pediatric reflex sympathetic dystrophy and suggest that this syndrome could be underdiagnosed in children and adolescents. Psychologic problems frequently play a role in this disorder, which often can be treated conservatively. We also point out that the diagnosis is mainly clinical. An early diagnosis can avoid unnecessary tests and potentially can improve response to treatment, and prognosis.

Adolescent↗
PubMed1999

Are Angiotensin Converting Enzyme and von Willebrand factor circulating levels useful surrogate parameters to monitor disease activity in Kawasaki disease?

To verify if Angiotensin Converting Enzyme (ACE) and von Willebrand factor (vWF) may be used as a laboratory marker for the follow-up of endothelial derangement and therapeutic efficacy in Kawasaki disease (KD), circulating ACE, vWF routine hematological tests and cardiac involvement were assessed in 32 children with established diagnosis of KD before and up to six months after intravenous gamma-globulins (i.v.IG) treatment. I.v.IG treatment normalized progressively all the hematological parameters to levels comparable with healthy controls within 30 days. At baseline, ACE levels resulted significantly lower (1.8 +/- 1.3 pmol/ml/min), and vWF levels significantly increased (210.3 +/- 35.2%) when compared with controls (respectively 7.0 +/- 0.9 pm/ml/min and 99 +/- 17.9%). Seven days after the treatment vWF levels were decreased (188 +/- 18.4%) but still significantly higher than controls, and fully normalized after 15 days (104.8 +/- 14.3%). ACE levels were found progressively increased at 7, 15, and 30 days after the treatment (respectively 2.7 +/- 1.0, 3.7 +/- 0.4, 5.04 +/- 0.9 pm/ml/min) and reached the range of normality only after two months (7.74 +/- 2.46 pm/ml/min). The present study shows that ACE and vWF circulating levels are significantly modified during the acute phase of the disease.

Child, Preschool↗
PubMed1999

Elbow arthritis: a rare inaugural manifestation of acute leukemia.

We report four cases of swelling of the elbow in children in whom the final diagnosis was acute leukemia. We believe that elbow arthritis in children, especially when isolated, is an unusual finding that is unlikely to indicate juvenile idiopathic arthritis and should suggest alternative diagnoses including neoplastic disease.

Adolescent↗
PubMed1998

Arthritis as a presenting feature of non-Hodgkin's lymphoma.

Leukaemia can present with joint swelling in the absence of abnormal haematological findings. Arthritis as a presenting sign of lymphoma, however, is extremely rare. Three children with non-Hodgkin's lymphoma who had joint swelling at the onset of their disease are reported. Two cases showed histological features of anaplastic large cell lymphoma (Ki-1/CD30 positive), and one of angioimmunoblastic T cell lymphoma. In all patients the unusual presentation delayed correct diagnosis.

Arthritis↗
PubMed1997

Early onset sarcoidosis: not a benign disease.

There are 2 distinct forms of sarcoidosis in childhood. We describe the clinical picture of 6 patients with sarcoidosis of the early onset type. All patients had their disease onset during the first 4 years of life. In contrast to the black predominance reported in adult patients, our series comprised 5 whites and one Latin-American. The triad of rash, arthritis, and uveitis seems to be characteristic for this form. The symptoms in our patients were comparable to those in the literature. However, a long followup (9-23 yrs) allowed us to recognize severe complications of the disease, previously not well known, such as blindness (4 patients), growth retardation (3 patients), heart involvement (2 patients), renal failure (1 patient), and even death in one patient. Pulmonary involvement was present only in one patient late in the disease course. This condition may be overlooked, and is sometimes misdiagnosed, due to its rarity and to its similarities with juvenile arthritis. However, the clinical characteristics of the 2 diseases are different. Early and correct diagnosis is important in light of the severe disease prognosis, and the possible therapeutic options.

Blindness↗
PubMed1997

Idiopathic limb edema in children with chronic arthritis: a multicenter report of 12 cases.

Lymphedema, a well known extraarticular manifestation of rheumatoid arthritis, has been rarely described in children with idiopathic chronic arthritis. We describe 12 cases of lymphedema and idiopathic arthritis of childhood seen at 4 different pediatric rheumatology centers. Eight patients were girls, 4 boys; the age at appearance of lymphedema ranged from 2.3 to 17 years. In all patients except one, lymphedema was localized to the lower limbs. The outcome of lymphedema was variable, but not always related to the arthritis course, and was mostly independent of any specific therapy. Lymphography was performed in only one patient, and revealed lack of lymphatic drainage in the affected leg. We conclude that the association of lymphedema and idiopathic arthritis of childhood is not rare; this association is unlikely to be coincidental, even though the pathogenetic mechanisms are currently not well understood.

Adolescent↗
PubMed1995

Nonsteroidal antiinflammatory drug-induced gastroduodenal injury in children.

OBJECTIVE: To determine the incidence of abdominal pain and gastroduodenal injury in children with arthritis taking nonsteroidal antiinflammatory drugs (NSAIDs). METHODS: A retrospective review of the records of all children (570 patients) receiving followup care in an academic rheumatology clinic between 1991 and 1993 was performed. RESULTS: There were 344 patients who used NSAIDs during the study period. Abdominal pain was recorded in 27.9% of patients taking NSAIDs and 14.6% of patients not taking NSAIDs. Abdominal pain in 47 patients (49%) taking NSAIDs and 14 patients (42%) not taking NSAIDs was evaluated radiographically and/or endoscopically. Among those patients evaluated, gastric or duodenal injury was found in 16 (34.0%) who were taking NSAIDs and 1 (7.1%) who were not. This represented a relative risk for gastroduodenal injury of 4.8 for patients taking NSAIDs (P = 0.09). The incidence of injury did not change when analyses were controlled for prednisone or slow-acting antirheumatic drug use. None of the children were hospitalized or died as a result of gastroduodenal injury during the 3-year period. CONCLUSION: We conclude that NSAID use in children with arthritis frequently leads to gastroduodenal injury, with an estimated incidence and relative risk that are comparable to the rates found in adults with arthritis taking NSAIDs, but that hospitalization or death as a result of this injury is uncommon.

Abdominal Pain↗
PubMed1994

Levels of circulating intercellular adhesion molecule 1 and E-selectin in serum and synovial fluid of juvenile chronic arthritis patients.

OBJECTIVE: To measure the levels of two adhesion molecules (AM), soluble intercellular adhesion molecule 1 (sICAM-1) and soluble E-selectin (sE-selectin), in serum and synovial fluid (SF) of patients with juvenile chronic arthritis (JCA). METHODS: Both soluble AM levels were tested, in serum and synovial fluid (SF) samples, with an enzyme-linked immunosorbent assay (ELISA) method. RESULTS: Serum levels of sICAM-1 and sE-selectin in JCA patients were not significantly different from those of a control group. Synovial fluid levels of sICAM-1, but not of sE-selectin, assayed significantly higher (p < 0.05) in JCA patients than in controls. Moreover SF levels of both molecules correlated negatively with disease duration, being higher in the earliest phases. No significant correlations were found between JCA sICAM-1 and sE-selectin levels and leukocyte count or ESR. CONCLUSIONS: These observations may signify a more important role of ICAM-1 than E-selectin in the migration of inflammatory cells into JCA SF. The negative correlation of both AMSF levels in JCA patients with disease duration could reflect a higher expression of ICAM-1 and E-selectin during the earliest phases of the disease.

Adolescent↗
PubMedDate not supplied

Prevention of recurrences of corticosteroid-dependent idiopathic pericarditis by colchicine in an adolescent patient.

A case of an adolescent patient with idiopathic recurrent pericarditis is reported. On several occasions during a period of 3. 5 years he was shown to be dependent on corticosteroid therapy, and he became cushingoid. After corticosteroids were substituted with colchicine, no further relapses occurred during a period of 29 months. In accordance with the first published pediatric results, colchicine represents an effective and well-tolerated alternative therapy for recurrent idiopathic pericarditis and might replace prolonged administration of corticosteroids.

Adolescent↗