Management of juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is a relatively uncommon disorder in childhood. Expertise however should be the corner stone of care of children with JIA, as early appropriate treatment is mandatory to ensure best possible short and longterm outcome for children with JIA. Therefore comprehensive treatment centers (with multi disciplinary teams) should be based in tertiary level academic centers. This article deals with both specific and generic issues encountered in managing children with JIA.     

MR spectrum in spinal dysraphism

Spinal dysraphism is a general term which encompasses a wide variety of anomalies of the spine, all of which result from imperfect midline fusion of the embryonic neural tube. This term refers to large defects that involve the spine and not to small vertical clefts commonly seen within the spinal process of L5 or S1. We present a spectrum of MR imaging findings selected from a retrospective review of 100 patients of spinal dysraphism evaluated at our institution. Received: 18 May 2000 Revised: 13 July 2000 Accepted: 13 July 2000     

The coeliac iceberg in Italy. A multicentre antigliadin antibodies screening for coeliac disease in school-age subjects

Background : Recent studies suggest that coeliac disease (CD) is one of the commonest, life-long disorders in Italy. The aims of this multicentre work were: (a) to establish the prevalence of CD on a nationwide basis; and (b) to characterize the CD clinical spectrum in Italy. Patients and methods : Fifteen centres screened 17201 students aged 6–15 years (68.6% of the eligible population) by the combined determination of serum IgG- and IgA-antigliadin antibody (AGA) test; 1289 (7.5%) were IgG and/or IgA-AGA positive and were recalled for the second-level investigation; 111 of them met the criteria for the intestinal biopsy: IgA-AGA positivity and/or AEA positivity or IgG-AGA positivity plus serum IgA deficiency. Results : Intestinal biopsy was performed on 98 of the 111 subjects. CD was diagnosed in 82 subjects (75 biopsy proven, 7 not biopsied but with associated AGA and AEA positivity). Most of the screening-detected coeliac patients showed low-grade intensity illness often associated with decreased psychophysical well-being. There were two AEA negative cases with associated CD and IgA deficiency. The prevalence of undiagnosed CD was 4.77 × 1000 (95% CI 3.79–5.91), 1 in 210 subjects. The overall prevalence of CD, including known CD cases, was 5.44 × 1000 (95% CI 4.57–6.44), 1 in 184 subjects. The ratio of known to undiagnosed CD cases was 1 in 7. Conclusions : These findings confirm that, in Italy, CD is one of the most common chronic disorders showing a wide and heterogeneous clinical spectrum. Most CD cases remain undiagnosed unless actively searched.     

Reduced upper airway nitric oxide in cystic fibrosis

PURPOSE: Incidence data for severe aplastic anaemia (SAA) in children are scanty and vary. Few population based studies have been reported. A retrospective and prospective study was conducted to determine the incidence and course of SAA. PATIENTS AND METHODS: All children with a diagnosis of SAA in the Nordic countries from 1982 through 1993 were registered and have been followed up since 1987. RESULTS: A total of 101 children were diagnosed with SAA. The mean annual child population was 4.31 million. A constant incidence of 1.95/million children/year was found: 2.4 for boys and 1.5 for girls. A non-significant increase of cases occurred from November to March. Possible aetiological agents were noted in 29%. The actuarial survival was 79% after one year and 68% after five years without significant difference between boys and girls. CONCLUSION: The incidence of SAA in the Nordic countries remains stable with a preponderance among boys. SAA has still a high initial mortality and a risk of late deaths.     

Actinomycin D induces apoptosis and inhibits growth of pancreatic cancer cells

Pancreatic cancer cells are usually resistant to apoptosis induced by cytotoxic drugs, by activation of surface receptors such as Fas and TNF receptor or by serum or growth factor withdrawal. Actinomycin D (actD) is an inhibitor of RNA synthesis and acts as a potent inducer of apoptosis in several cell lines. In the present study, we investigated the effects of actD on PANC-1 pancreatic cancer cells. ActD caused apoptosis in PANC-1 cells in a dose-dependent manner, as determined by cell growth assays, DNA laddering and TUNEL assays. Induction of apoptosis correlated with activation of the JNK/SAPK pathway and increased expression of Bax but not Bad or p53. PANC-1 cells were completely resistant to Fas antibody and TNF-alpha. In contrast, TRAIL decreased the growth of PANC-1 cells by 22%. Low concentrations of actD (10 ng/ml) enhanced the cytotoxic effects of all 3 cytokines. EGF, FGF-2 and IGF-I did not protect PANC-1 cells from actD-mediated apoptosis. ActD (10 ng/ml) also inhibited the growth of CAPAN-1 and T3M4 pancreatic cancer cells but not MiaPaCa-2 cells. Our observations suggest that actD may act via JNK/SAPK and Bax to promote apoptosis in PANC-1 cells and that it may inhibit the growth of other pancreatic cancer cell lines.     

Pregnancy in patients with systemic lupus erythematosus

We reviewed the obstetrical performance and outcome of 15 pregnancies in patients with systemic lupus erythematosus (SLE) (study group) and compared them with 45 age and parity-matched normal pregnancies (control group). Eleven women (73.8%) were in remission phase and 4 (26.7%) had active disease at the time of conception. The time interval between disease diagnosis and the index pregnancy was 4.2 +/- 2.5 years. Two patients with renal involvement had lupus flare-up during the antenatal period. There was no case of lupus flare-up in the postpartum period. Gestational age at delivery was significantly lower in SLE patients (35.9 +/- 2.5 weeks) compared to the control group (37.4 +/- 2.2 weeks). The incidence of intrauterine growth retardation was significantly higher in the SLE patients (40%). There was no case of neonatal lupus or congenital heart block.