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Biomedical subjects

I Foeldvari

Publications and source records attributed to I Foeldvari.

17 recordsLinked to original sources
PubMed2001

[Naproxen-induced pseudoporphyria].

A 12 year old boy developed scars at light-exposed areas following long-term therapy with naproxen for rheumatoid arthritis. Erythrocyte and urine porphyrin levels were not increased, and there was no evidence of increased photosensitivity. Pseudoporphyria is reported in 10-20% of those treated with naproxen for > 4 weeks. As compared to other nonsteroidal anti-inflammatory agents, the specific risk for naproxen is increased about 6fold. While the underlying abnormality has not been elucidated, formation of phototoxic metabolites in a subgroup of genetically predisposed individuals has been suggested as the most likely mechanism. Both dermatologists and rheumatologists should be aware of the risk of naproxen-induced pseudoporphyria and discontinue therapy early in order to avoid scar formation in light-exposed areas.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2001

Recognition and management of scleroderma in children.

Juvenile scleroderma is a rare disease of childhood. The 2 main forms are localised and systemic scleroderma, although the pathogenesis of both forms is still unclear. The main clinical characteristic is the presence of skin sclerosis. Localised scleroderma can be divided into 3 different subtypes: morphoea, linear scleroderma and 'coup de sabre' lesions. The incidence is 0.2 to 0.4 per 100,000. 25 to 40% of patients are rheumatoid factor positive and 23 to 67% antinuclear antibody positive. An increased number of eosinophils occur in 31% of patients. Systemic sclerosis is an even rarer disease; approximately 10% of patients evolve the disease before the age of 18 years. The clinical presentation and disease course differ from the adult disease. This is reflected in the much better outcome. With regard to therapeutic options, there is still no gold standard for either form of the disease.

Child, Preschool↗
PubMed2000

Neonatal onset of rash in Still's disease.

We report a neonate with a rash that appeared on the second day of life in association with elevated body temperatures and increased C-reactive protein levels. The rash was evanescent and recurred on a daily basis during the first year of life. At 15 months, the infant developed swelling of the right knee joint. Early-onset systemic juvenile rheumatoid arthritis can mimic congenital infections and should be considered in the differential diagnosis of neonatal exanthemas.

Age of Onset↗
PubMed2000

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↗
PubMed2000

Diffuse and limited cutaneous systemic scleroderma.

Juvenile systemic scleroderma (jSSc) is a rare disease. Based on the first large data collection on this patient group, the disease course was demonstrated to differ from that in adults. The concept of persistence of maternal cells in patients with SSc remains pathogenetically fascinating, as does the resemblance with graft-versus-host-disease. In view of new therapeutic options, controlled trials have not established a gold standard for treatment, but autologous bone marrow transplantation may be considered a rescue therapy for selected patients. Palliative therapies have also improved markedly in recent years. The first controlled trials for patients with jSSc are being proposed.

Adult↗
PubMed2000

Validation of the proposed ILAR classification criteria for juvenile idiopathic arthritis. International League of Associations for Rheumatology.

OBJECTIVE: The new International League of Associations for Rheumatology (ILAR) classification criteria were proposed to facilitate communication among pediatric rheumatologists. Before they are applied in daily practice they should be clinically validated. METHODS: We retrospectively applied the proposed criteria on our pediatric rheumatology patient population seen between June 1 and August 31, 1998. RESULTS: We saw 67 patients with oligoarticular (oJRA), 6 with polyarticular/RF negative (pJRA), and 8 with systemic juvenile rheumatoid arthritis (sJRA), all classified according to American College of Rheumatology criteria, 5 with juvenile psoriatic arthritis (PsA) according to the Vancouver criteria, and 11 with juvenile spondyloarthritis (SP) according to the European Spondylarthropathy Study Group preliminary criteria. Of the 97 patients, 85 could be clearly classified according to the ILAR criteria. Twelve patients (12%) were classified as "other." Six patients could not be classified as "oligo" because of a family history of psoriasis, and did not fulfill the criteria for PsA either. All 6 "other" patients fulfilled criteria for 2 different categories. CONCLUSION: With this ILAR proposed classification criteria 88% of patients could be classified. In patients classified as "other," the psoriatic trait caused the most difficulty in classification.

Arthritis, Juvenile↗
PubMed1998

Rapid development of severe osteoarthritis associated with osteoid osteoma in a young girl.

In a 14-year-old girl with a 5 month history of left sided thigh pain, X-rays demonstrating osteoarthritis and a bone scintigraphy showing increased focal uptake in the femoral neck led to the diagnosis of an osteoid osteoma. The protuberant bone at the femoral neck was removed en-bloc and the diagnosis histologically confirmed. The synovium was extensively infiltrated with lymphocytes. Postoperatively the girl experienced neither the expected pain relief nor improvement of her hip function over the next 4 months. MRI and CT results indicated development of a severe osteoarthritis even though no residual lesion could be found.

Adolescent↗
PubMed1995

Systemic capillary leak syndrome in a child.

A child with idiopathic systemic capillary leak syndrome had four episodes between 3 and 3 years 9 months of age, all characterized by the sudden onset of hypovolemic shock without any obvious triggering factor. Between the attacks she is well and free of symptoms. Early establishment of intravenous access facilitates volume replacement during the acute episodes.

Acute Disease↗
PubMed1991

A single amino acid substitution in a common African allele of the CD4 molecule ablates binding of the monoclonal antibody, OKT4.

The CD4 molecule is a relatively non-polymorphic 55 kDa glycoprotein expressed on a subset of T lymphocytes. A common African allele of CD4 has been identified by non-reactivity with the monoclonal antibody, OKT4. The genetic basis for the OKT4- polymorphism of CD4 is unknown. In the present paper, the structure of the CD4 molecule from an homozygous CD4OKT4- individual was characterized at the molecular level. The size of the CD4OKT4- protein and mRNA were indistinguishable from those of the OKT4+ allele. The polymerase chain reaction (PCR) was used to map the structure of CD4OKT4- cDNAs by amplifying overlapping DNA segments and to obtain partial nucleotide sequence after asymmetric amplification. PCR was then used to clone CD4OKT4- cDNAs spanning the coding region of the entire, mature CD4 protein by amplification of two overlapping segments followed by PCR recombination. The nucleotide sequence of CD4OKT4- cDNA clones revealed a G----A transition at bp 867 encoding an arginine----tryptophan substitution at amino acid 240 relative to CD4OKT4+. Expression of a CD4OKT4- cDNA containing only this transition, confirmed that the arginine----tryptophan substitution at amino acid 240 ablates the binding of the mAb OKT4. A positively charged amino acid residue at this position is found in chimpanzee, rhesus macaque, mouse and rat CD4 suggesting that this mutation may confer unique functional properties to the CD4OKT4- protein.

Alleles↗
PubMedDate not supplied

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

We report the results of the cross-cultural adaptation and validation into the German 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 German CHAQ was fully validated with 3 forward and 3 backward translations, while the CHQ has already been published and therefore it was revalidated. A total of 197 subjects were enrolled: 142 patients with JIA (5% systemic onset, 13% polyarticular onset, 8% extended oligoarticular subtype, and 74% persistent oligoarticular subtype) and 55 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the 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 polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the German versions of the CHAQ-CHQ are reliable, and valid tools for the functional, physical and psychosocial assessment of children with JIA.

Adolescent↗
PubMedDate not supplied

Delayed diagnosis of juvenile rheumatoid arthritis in a girl with Turner's syndrome.

Although increased risk for autoimmune diseases has been documented in Turner's syndrome (TS), the involvement of juvenile rheumatoid arthritis (JRA) has rarely been reported. A detailed case description of JRA associated with Turner's syndrome is presented as the second such case report in the literature. The arthritis was diagnosed 8 years after its onset due to the confounding of its symptoms with those of TS.

Arthritis, Juvenile↗