Biomedical subjects
Publications and source records attributed to A Ravelli.
A 10-month-old female presented with anuria due to bilateral cystine urolithiasis. Although bilateral obstruction of the ureters by calculi is very rare in children, it may be the presenting feature of cystinuria even in the first year of life.
A 9 year old girl presented with clinical manifestations of Henoch-Schönlein syndrome and macroscopic haematuria. Laboratory investigations showed selective IgA deficiency and renal biopsy showed mesangial proliferative glomerulonephritis with diffuse granular deposits of C3 on immunofluorescence. IgA deposits were absent.
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A case of transient stenosing ureteritis in a boy presenting with the clinical features of Henoch-Schoenlein purpura (diffuse articular swelling, petechiae, abdominal pain) complicated by macroscopic haematuria with blood clots is reported. On urography the right collecting system and the right ureter up to the distal lumbar portion were dilated owing to tight ureteral stenosis at that level. Above the stricture, numerous ring-like functional ureteral wall defects were detectable. On the left a stenosis was evident at the lumbo-sacral level with mild dilatation of the ureter. A small filling defect due to subephitelial haemorrhage was evident in the upper part of this ureter. Three months later, after prednisone therapy, the urography was normal on the right: residual mild stenosis was still evident on the left. Radiological findings in Henoch-Schoenlein purpura and the possible differential diagnosis of this condition are discussed.
Two cases of glomus tumour in unusual sites are described. Their indistinct symptomatology, site and histological polymorphism are made the basis of a general discussion of the histogenesis and possible ongoing pictures of glomus tumours, the classification of which is also felt to be unsatisfactory.
Lack of an universally accepted nomenclature of lumbo-sacral angles often leads to misunderstanding. As a basis for a future agreed nomenclature the descriptions of these angles are put in order, wherever possible without introducing new names. Only in one instance - for the differentiation between an anterior and posterior lumbo-sacral angle - was a new name essential. The series of angles also contains a point - the promontory - and a distance - the height of the promontory - which is the second new name.
The basic shape of the facies lunata shows at the inner margin of each of the three participating bones a dell into which the floor of the acetabulum progresses. These dells originate in nutritive vessels which enter the bone and claim this place. There always are groups of vessels and wide channels which may be visible on radiographs. The so far unexplained picture of a vascular canal in the roof of the acetabulum corresponds to the canal leading into the body of the ilium. Due to its position there is often restructuring leading to filling or covering of the canal. The entrance to this canal is shown as a gap in the arc of the acetabular cavity which would have to be added to that of the "tear figure" known so far. The closed arc, as always drawn, reults only when this entry has been filled up.
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Chronic iridocyclitis (CI) is among the most important extra-articular manifestations of juvenile chronic arthritis (JCA) and is most often observed in young girls with pauciarticular JCA and circulating antinuclear antibodies (ANAs). The frequency of CI found in 39 children with ANA-positive pauciarticular JCA was 56%. None of the patients developed ocular complications during follow-up. We think that this favourable outcome was primarily due to the early diagnosis and to our choice of using atropine as a mydriatic agent and to prolong atropine, after having spaced its administration further apart, up to one year or longer after the remission of ocular manifestations.
Wegener granulomatosis (WG) is a systemic disease that is particularly unusual in children. A limited form has been described without renal involvement. We report a 14-year-old girl in whom the disease started with acneiform nodular and papular lesions on the forehead. Later necrotic ulcers developed on her forehead, arms, and buttocks. The cutaneous lesions were associated with upper and lower respiratory tract involvement, low-grade fever and arthralgias. Subsequently clinical and laboratory evaluations (increased ESR; leukocytosis and presence of serum IgG antibodies cANCA = 1:160), with chest roentgenograms revealing pulmonary densities and parenchymal infiltration, suggested the diagnosis of WG. The histologic findings of a cutaneous biopsy specimen were ulceration of the epidermis with diffuse neutrophilic inflammatory infiltrate and a late-stage small vessel vasculitis in the dermis. Histopathology of the nasal mucosa was characterized by a granulomatous process with a dense lymphohistiocytic infiltrate with few giant cells, a finding that confirmed the diagnosis of WG. No renal involvement was present. One month of cyclophosphamide (125 mg/day) and prednisone (70 mg/day) therapy markedly improved the patient's clinical condition. At present, 1 year later, she is free from any signs of the disease. According to the literature, the frequency of cutaneous lesions in WG ranges from 16% to 46%. They are the presenting sign only in 6% of patients. Cutaneous lesions are even more uncommon in children. In particular, an "acneiform" presentation is a rare finding in WG.
A six-year-old boy with systemic lupus erythematosus developed insidiously a monolateral serous retinal detachment leading to severe visual loss. Serial determinations of anticardiolipin antibodies showed the presence of IgG anticardiolipin antibodies only at the time of ocular involvement. This case underlines the need for regular ophthalmologic evaluation in children with active systemic lupus erythematosus and supports the previous hypothesis of a relationship between antiphospholipid antibodies and retinal involvement in systemic lupus erythematosus.
OBJECTIVE: To investigate the efficacy of folinic acid in reducing the side effects associated with methotrexate (MTX) therapy in children with juvenile idiopathic arthritis (JIA) and to determine whether folate supplementation may reduce the benefit of MTX administration. METHODS: This was a retrospective, non-controlled study. Inclusion criteria were: 1) diagnosis of JIA according to the Durban 1997 criteria; 2) treatment with low to intermediate doses of MTX (10-20 mg/m2/week) as the sole second-line agent for at least 6 mos.; and 3) supplementation with folinic acid (2.5-7.5 mg) in a single weekly dose 24 hrs after MTX administration. All patients were started on folinic acid only after the development of a side effect. Exclusion criteria were: treatment with higher doses of MTX (> 20 mg/m2/week). The outcomes investigated were: hepatotoxicity (liver transaminase increase), gastrointestinal toxicity, disease flare, and clinical remission. The number of episodes per patient-year of MTX treatment of each outcome before and after folinic acid supplementation was compared by the Wilcoxon matched pairs test. RESULTS: A total of 43 children with JIA were included in the study. The mean duration of treatment before and after folinic acid supplementation was 1.1 years and 1.8 years, respectively. After the start of folinic acid supplementation, the mean number of episodes per patient-year of hepatotoxicity and gastrointestinal toxicity decreased from 2.30 to 0.32 (p < 0.001) and from 1.09 to 0.29 (p = 0.002), respectively. The mean number of disease flares and clinical remissions per patient-year did not change significantly. CONCLUSION: In our JIA patients, folinic acid supplementation resulted in a significant reduction in the most common side effects of MTX, without affecting the clinical efficacy of the drug.
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The aim of this project was to cross-culturally adapt and validate the American English version of the Childhood Health Assessment Questionnaire (CHAQ) and of the Child Health Questionnaire (CHQ) in the 32 different member countries of the Paediatric Rheumatology International Trials Organisation (PRINTO). This effort forms part of an international study supported by the European Union to evaluate the health-related quality of life in children with juvenile idiopathic arthritis (JIA) as compared to their healthy peers. A total of 6,644 subjects were enrolled from 32 countries: Argentina, Austria, Belgium, Brazil, Bulgaria, Chile, Croatia, the Czech Republic, Denmark, Finland, France, Georgia, Germany, Greece, Hungary, Israel, Italy, Korea, Latvia, Mexico, the Netherlands, Norway, Poland, Portugal, Russia, Slovakia, Spain, Sweden, Switzerland, Turkey, the United Kingdom, and Yugoslavia. A total of 3,235 patients had JIA (20% systemic onset, 33% polyarticular onset, 17% extended oligoarticular subtype, and 30% persistent oligoarticular subtype) while 3,409 were healthy children. This introductory paper describes the methodology used by all the participants. The results and the translated version of both the CHAQ and the CHQ for each country are fully reported in the following papers. The results of the present study show that cross-cultural adaptation is a valid process to obtain reliable instruments for the different socio-economic and socio-demographic conditions of the countries participating in the project.
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.