The Italian version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report herein the results of the cross-cultural adaptation and validation into the Italian language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Italian CHAQ was already published in the literature and was therefore revalidated while the Italian CHQ was fully cross culturally adapted with 3 forward and 3 backward translations, and than validated. A total of 1,192 subjects were enrolled: 404 patients with JIA (16% systemic onset, 31% polyarticular onset, 21% extended oligoarticular subtype, and 32% persistent oligoarticular subtype) and 788 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Italian version of the CHAQ-CHQ are reliable, and valid tools for the functional, physical and psychosocial assessment of children with JIA.

Oral versus intramuscular methotrexate in juvenile chronic arthritis. Italian Pediatric Rheumatology Study Group.

OBJECTIVE: To compare the efficacy and safety of methotrexate (MTX) after oral and intramuscular administration in children with juvenile chronic arthritis (JCA). METHODS: Pediatric rheumatology centers in Italy participated in this short-term, prospective, open trial. Each investigator was allowed to choose the oral or intramuscular route of administration according to his personal preference in everyday clinical practice. Patients enrolled by each center were given MTX through the same method of administration. All patients received 10 mg/m2 of MTX each week for six months. RESULTS: A total of 257 patients with JCA (127 treated orally and 130 intramuscularly) were enrolled in the trial by 11 Italian centers. The response rate after 6 months of MTX therapy was 58% in the oral and 61% in the intramuscular cohort. The frequency of adverse side effects did not differ significantly between the two treatment groups. CONCLUSION: The results of this study suggest that MTX at the conventional dose regimen is equally effective and has a similar safety profile in children with JCA when administered orally or by intramuscular injections.

Growth retardation in non-steroid treated juvenile rheumatoid arthritis.

Growth in height was studied in 58 patients with juvenile rheumatoid arthritis (JRA) followed for 4.9 +/- (SD) 2.8 years, who had never received steroids. Height measurements were converted to Height Z Scores. Height Z scores at first and at last visit were respectively 0.7 +/- 1 and 0.7 +/- 0.9 (NS) in pauciarticular, 0 +/- 1.6 and -0.55 +/- 1.6 (p = 0.045) in systemic, 0.29 +/- 0.8 and -0.4 +/- 1 (p = 0.0001) in polyarticular JRA patients. In systemic and polyarticular patients a significant negative relation was found between the duration of disease and the delta Height Z score (p = 0.0008) as well as between the sum of the periods of active disease and the delta Height Z score (p = 0.0001). The patients with functional class = 1 lost 0.01 +/- 0.19 Height Z score per year while those with functional class > or = 2 lost 0.16 +/- 0.13 Height Z score per year (p = 0.005). The loss of Height Z score in systemic and polyarticular subjects observed during pubertal age (-0.71 +/- 0.67 Height Z score) was significantly (p = 0.02) greater than in those observed before puberty (-0.1 +/- 0.72). The longer duration of disease, the higher degree of functional joint involvement, and the age of puberty appear to be risk factors for height growth impairment in systemic and polyarticular JRA.

Middle ear involvement in children with chronic rheumatoid juvenile arthritis.

In a selected sample of patients affected by juvenile rheumatoid arthritis (JRA) little is known about middle ear involvement, even though many synovial joints are affected. Multifrequency tympanometry was used to measure admittance, conductance, susceptance and phase angle at different probe frequencies and resonant frequencies. In all, 35 children with JRA and a control group (30 children) were studied. Findings showed that mean resonant frequency values in all children with JRA were greater than in the control children. The multifrequency tympanometry parameters measured in acute JRA subjects are not different from parameters of remission JRA subjects except for a change in the phase angle. The changes found are due to involvement of the incudomalleolar and incudostapedial joints.

Altered body water distribution in subjects with juvenile rheumatoid arthritis and its effects on the measurement of water compartments from bioelectric impedance.

OBJECTIVE: To assess the reliability of bioelectric impedance analysis (BIA) for predicting total body water (TBW) and extracellular water (ECW) in children affected by juvenile rheumatoid arthritis (JRA). SUBJECTS: Thirty-nine children affected by JRA and 23 healthy children of similar age (11.0 +/- 3.6, range 3.0-19.0 y) were recruited for the study. METHODS: TBW and ECW were measured by deuterium oxide and bromide dilution, respectively. Bioelectric impedance (Z) was measured at frequencies of 5, 50 and 100 kHz. The prediction of TBW and ECW from BIA was based on the impedance index (ZI = height2/Z, cm2/omega). RESULTS: TBW standardized per kg of body weight and ECW standardized per litre of TBW were significantly higher in JRA as compared to control patients (59.7 +/- 2.4 vs 57.7 +/- 2.7% and 44.5 +/- 4.6 vs 38.1 +/- 7.9%, with P < 0.005 and P < 0.0001, respectively). Moreover, intracellular water standardized per litre of TBW was significantly lower in JRA than in control subjects (55.5 +/- 4.6 vs 62.5 +/- 8.1, with P < 0.0001). In both controls and patients, the use of ZI at 5kHz offered the more accurate prediction of ECW. However, the use of ZI at 100 kHz did not offer a better prediction of TBW as compared to its value of 50 kHz. Control-generated formulae for predicting water compartments from BIA [TBW = 0.716 x ZI at 100 kHz-1.504, r = 0.934, s.e.e. = 2.2 l;:ECW = 0.430 x ZI5-3.652, r = 0.869(7) s.e.e. = 1.7 l] underestimated TBW and ECW in JRA patients. However, population-specific formulae [TBW (1) = 0.766 x ZI at 100 kHz-0.053, r = 0.939, s.e.e. = 2.8 l; ECW (l) = 0.399 x ZI at 5 kHz-0.283, r = 0.886, s.e.e. = 1.7 l] allowed an accurate prediction of TBW and ECW in JRA patients, taking into account their altered body water distribution. CONCLUSIONS: Altered water distribution impedes the use of formulae developed on healthy children to predict TBW and ECW from BIA and JRA patients. It is hypothesized that chronic inflammation and subclinical malnutrition may be responsible for the altered body water distribution of JRA patients. Traditional body composition models may require adjustments for use in JRA children due to their altered body hydration and water distribution.

Nutritional status in active juvenile chronic arthritis not treated with steroids.

Nutritional status and nutrient intake were assessed in 17 children with active juvenile chronic arthritis (JCA) who never received steroids and in 17 controls matched for age and sex. Five patients had systemic, seven polyarticular and five oligoarticular JCA. Values significantly below those of the controls were found in systemic patients for height (p<0.05), upper arm circumference (p<0.05) and arm muscle area (p<0.01), and in polyarticular subjects for arm muscle area (p<0.01). All patients had unremarkable anthropometric fat measurements. All anthropometric measurements were normal in oligoarticular patients. Twelve JCA patients had reduced serum iron (Fe), 6 reduced serum zinc (SZn), 14 reduced intra-erythrocytic zinc (EZn) and 2 reduced serum copper (SCu). SZn was inversely correlated with erythrocyte sedimentation rate (ESR) (p=0.023). EZn was inversely related to lymphocyte count (p=0.022). SCu was related to ESR (p=0.037) and to lymphocyte count (p=0.016). No significant difference in nutrient intake was found between patients and controls. Active JCA was associated with reduced muscular mass, Fe, SZn, EZn. These alterations did not depend on reduced nutrient intake.

Reduced bone mineral content and normal serum osteocalcin in non-steroid-treated patients with juvenile rheumatoid arthritis.

OBJECTIVES: To distinguish the effects of juvenile rheumatoid arthritis (JRA) on bone mineralisation from those possibly caused by steroid therapy. METHODS: Bone mineral status was evaluated in 20 children (five boys and 15 girls) with active JRA who never received steroids. Seven had oligoarticular, nine had polyarticular, and four had systemic JRA. Bone mineral content (BMC) was assessed by single beam photon absorptiometry and expressed as a Z score relative to normal values in healthy children. Serum calcium, phosphate, and alkaline phosphatase were measured by colorimetric methods. Whole parathyroid hormone was assayed by Immuno Radiometric Assay. Serum osteocalcin was measured by specific radioimmunoassay. Nutrient intake was assessed by a 24 hours dietary recall. BMC and nutrient intake were also assessed in an age and sex matched control group. RESULTS: BMC was -1.5 (SEM 0.8) Z scores in patients and 0.4 (0.3) in the control group (p = 0.02). BMC averaged -4.9 (2) Z scores in the systemic JRA group, -1 (0.6) in the polyarticular group and 0.3 (0.7) in oligoarticular JRA patients. Serum calcium, phosphate and osteocalcin values were normal in all patients. No significant difference was found between JRA patients and controls in calcium, phosphate, energy, and protein intake. CONCLUSION: JRA subjects have significantly reduced BMC even in the absence of any steroid therapy. Bone demineralisation appears to depend more on disease activity and on reduced motility than on reduced nutrient intake.

Statural growth of children with chronic renal failure on conservative treatment.

Statural growth was studied in 20 prepubertal children with chronic renal failure on conservative treatment followed-up 1.1 to 8.9 years (average 3.9). Five children reached end-stage renal failure during the follow-up period and underwent dialysis or transplantation. Most patients grew at a normal rate. During the observation period only 1 out of 20 children lost more than 0.5 height standard deviation score whereas 9 gained 0.5 to 3.1. A growth velocity above the 97th percentile for at least 1 year was observed in 6 patients. A normal growth rate and even catch-up growth is possible in children with chronic renal failure regardless of the degree of reduction of glomerular function.

[Juvenile chronic arthritis]. / L'artrite cronica giovanile.

Juvenile chronic arthritis is the most common connective tissue disease in children. It is of great social and clinical interest for its chronicity, for the often unpredictable response to pharmacological treatment; for the spontaneous evolution toward infirmity and often blindness. The English classification of the disease is here been adopted. There are 3 different types of onset: systemic, poliarticular and pauciarticular. Large joints such as the knees, wrists and ankles are involved more often than small joints. Also the cervical spine is frequently affected. Systemic disease is accompanied by high spiking fever, rash, lynphoadenopathy, pericarditis and hepatosplenomegaly. Chronic uveitis is a feature of JCA, more frequently observed in pauciarticular than in the other types of onset, and it is almost always associated with antinuclear antibody seropositivity. Rheumatoid factor (RF) and subcutaneous nodules are unusual in JCA. Diagnosis is often not easy and it is essentially clinical. The diagnostic criteria adopted have been proposed by ARA in 1977. In the majority of children treatment with ASA is successful. Sometimes other types of more toxic drugs such as gold salts or penicillamine are needed. Their use is best confined to reference centers. Orthopedical and physiotherapic treatments are complementary to the pharmacological one. Multidisciplinary centers are therefore necessary for the total management of these children also to stress the importance of furthering physical and psychological growth.

[Must renal biopsy be still performed in "lipoid nephrosis"? (author's transl)]. / Esiste ancora indicazione all biopsia renal nella "nefrosi lipoidae"?

In 52 out of 272 children with one of the following diseases: nephrotic or nephritic syndrome, persistent humaturia and/or proteinuria, renal biopsy was performed on the basis of commonly accepted indications. 18 biopsies were from patients with a clinical picture of lipoid nephrosis and clinical course of "non responders" or "frequent relapsers" types. All of them showed the histologic pattern of minimal changes and had a value of Y index according to ISKCD greater than 0,85. Moreover all patients with minimal changes nephrotic syndrome had a Y index greater than 0.85. If these observations will be confirmed renal biopsy would not later be indicated in children with "lipoid nephrosis".