Juvenile idiopathic arthritis is a heterogeneous group of diseases characterized by arthritis with poorly known causes, including monogenic disorders and multifactorial etiology. 22q11.2 proximal deletion syndrome is a multisystemic disease with over 180 manifestations already described. In this report, the authors describe a patient presenting with a short stature, neurodevelopmental delay, and dysmorphisms, who had an episode of polyarticular arthritis at the age of three years and eight months, resulting in severe joint limitations, and was later diagnosed with 22q11.2 deletion syndrome. Investigation through Whole Genome Sequencing revealed that he had no pathogenic or likely-pathogenic variants in both alleles of the MIF gene or in genes associated with monogenic arthritis (LACC1, LPIN2, MAFB, NFIL3, NOD2, PRG4, PRF1, STX11, TNFAIP3, TRHR, UNC13DI). However, the patient presented 41 risk polymorphisms for juvenile idiopathic arthritis. Thus, in the present case, arthritis seems coincidental to 22q11.2 deletion syndrome, probably caused by a multifactorial etiology. The association of the MIF gene in individuals previously described with juvenile idiopathic arthritis and 22q11.2 deletion seems unlikely since it is located in the distal and less-frequently deleted region of 22q11.2 deletion syndrome.

UNASSIGNED: This study aimed to determine if the presence of a pericardial effusion is associated with adverse outcomes among children admitted with juvenile idiopathic arthritis.
UNASSIGNED: The multicenter, retrospective cohort study was conducted with 4,332 patients (1,554 males, 2,778 females; median age: 12 years; IQR, 7, 15 years) using the Pediatric Health Information System. Data from hospital admissions between January 1, 2004, and September 15, 2015, were obtained for patients with an International Disease Classification, Ninth Revision code for juvenile idiopathic arthritis. Pericardial effusion was the primary predictor variable; the outcomes of interest were length of stay, hospital costs, and readmission within 90 days. Multivariate models were created to evaluate associations between pericardial effusion and adverse outcomes. We also analyzed factors associated with increased odds of having pericardial effusion in juvenile idiopathic arthritis.
UNASSIGNED: One hundred twenty (3%) patients had a code for pericardial effusion. Children with pericardial effusion had a longer median length of stay (7 days (IQR 3, 12) vs. 3 days (IQR 2,6), p<0.001), higher median costs ($17,688 (IQR 8,657, 40,623) vs. $8,456 (IQR 4,865, 16,302), p<0.001), and greater rates of readmission (22% vs. 15%, p=0.045). Multivariate analysis demonstrated no significant association between pericardial effusion and outcomes of interest. Black race and male sex were associated with increased odds of having pericardial effusion.
UNASSIGNED: Pericardial effusion is rare among children admitted with juvenile idiopathic arthritis but is associated with significant morbidity; its presence may be a marker of disease severity. Black children and males admitted with juvenile idiopathic arthritis warrant special consideration and may benefit from screening echocardiography.

Purpose: To report a case of ocular toxoplasmosis following long-term treatment with adalimumab and review the literature on ocular toxoplasmosis following anti-Tumour necrosis factor-α therapy. Method: A retrospective chart review of A 21-year-old male who developed retinochoroiditis in his left eye following adalimumab therapy combined with oral methotrexate. Result: A known patient of juvenile idiopathic arthritis (JIA) on adalimumab and oral methotrexate for the last four years presented to us with a blurring of vision for the last 15 days. Fundus examination of the left eye revealed severe vitritis and two patches of retinochoroiditis in the inferior part of the fundus. Subsequent investigations confirmed it to be a case of toxoplasma retinochoroiditis, and he responded to anti-toxoplasma treatment. A review of literature on a similar topic revealed five such cases, and the index case was the first such report in patients with JIA. Conclusion: The index case highlights the importance of early recognition and management of opportunistic infections in patients receiving biologicals.

OBJECTIVE: The prevalence of juvenile idiopathic arthritis (JIA) is estimated to be 16-150 per 100,000 children worldwide. The hip joint may be involved in over 50% of children leading to significant morbidity which may require surgical intervention in the form of arthroplasty. The literature lacks a concise overview of the outcomes, including complication and implant survival of total hip arthroplasty (THA) in juvenile idiopathic arthritis (JIA). The aim of this study is to systematically analyze the literature and report the outcomes of THA in JIA.
METHODS: Search was conducted in the online databases PubMed, Embase and Cochrane database. It included all original studies which evaluated clinical and/or radiological outcomes of THA in JIA with a minimum sample size of 5 patients and published in English. The level of evidence of the included studies was graded according to the Oxford Centre for Evidence Based Medicine. The Institute of Health Economics checklist was used to assess the quality of the studies included.
RESULTS: The nine studies included were retrospective in nature with all being Level IV according to Oxford Centre for Evidence Based Medicine. 475 hips in 304 patients with majority of them being females (241/304, 79.2%) were included in this review. All the studies reported the outcome objectively using various scores. The proportion of revision surgeries (92/378), either femoral or acetabular, noted was 22% (95% CI 10-33%). The proportion of acetabular revisions (72/378) was 16% (95% CI 8-25%) as compared to 4% (95% CI 1-6%) for femoral revisions (20/378). There was no difference in survivorship when cemented and uncemented implants were compared.
CONCLUSIONS: JIA patients with advanced hip disease represent a unique population with need for extra-long implant longevity. THA in patients of JIA leads to improved pain relief as well as mobility but the conversion of the same outcomes to functional activity is not proportionally improved. The current trend is the use of uncemented and ceramic-on-ceramic implants. Acetabular implants require earlier revision as compared to femoral implants. Age at surgery can be delayed by early institution of methotrexate which indirectly improves implant survival.
METHODS: IV.

Naproxen is a widely used nonsteroidal anti-inflammatory drug (NSAID) in pediatric population, used for mild-to-moderate pains, arthritis, and other immune-mediated disorders. It rarely causes clinically apparent liver injury in the adult population taking high doses of the drug over a prolonged period and is reported even rarer in pediatric population. We present a case of drug-induced liver injury (DILI) in a 13-year-old girl taking naproxen in therapeutic doses for juvenile rheumatoid arthritis. There was a complete recovery of liver function following discontinuation of naproxen therapy.

A diverse group of idiopathic inflammatory arthritis that affects children under the age of 16 and lasts six weeks or longer is known as juvenile idiopathic arthritis (JIA) or juvenile rheumatoid arthritis (JRA). It causes joint pain and morning stiffness, and the affected joints swell and become difficult to move. During flares, patients may experience flu-like symptoms such as muscle aches and fatigue. Disease-modifying antirheumatic drugs (DMARDs) are the mainstay of rheumatoid arthritis (RA) treatment. If necessary, immunosuppressive medications can be administered in stages. Nonsteroidal anti-inflammatory drugs (NSAIDs) are pain relievers for joints. Physical therapy treatment aims to reduce pain, improve joint range, correct movement patterns, strengthen weak structures, improve cardiovascular endurance, and improve patients\' quality of life. We present a case of a 12-year-old boy who presented to the hospital with knee pain and an inability to walk without assistance after falling on the ground one month prior. Medical history revealed that the patient had juvenile rheumatoid arthritis, which was confirmed on investigations. The consultant physician referred the patient for pre-rehabilitation physiotherapy before corrective surgery.

An iris cyst is a nonkeratinized squamous epithelial-lined space involving a layer of iris. The presentation of iris cyst can be variable-innocuous or presenting with secondary complications. The identification of whether the cyst is primary or secondary is important. The prognosis of primary iris cysts is good, as the majority does not require treatment and that of secondary iris cysts is much more capricious depending on their presentation. Their optimal management often poses a challenge for ophthalmologists. Here, we will present two cases of iris cysts with diverse presentation along with a review of the literature of this rare clinical entity.

We identified an unusual subtype of a Cryptosporidium sp. horse genotype as the cause of cryptosporidiosis in a 13-year-old girl in Poland who was undergoing immunosuppressive treatment for juvenile rheumatoid arthritis and Crohn\'s disease. The same subtype was identified in a horse the girl had ridden.

Multicentric carpotarsal osteolysis syndrome (MCTO; MIM #166300) is a rare skeletal disorder characterized by osteolysis affecting particularly the carpal, metacarpal, and tarsal bones, although other bones might be involved. MCTO is an autosomal dominant disease caused by heterozygous variants in the MAFB gene, frequently misdiagnosed as juvenile rheumatoid arthritis due to similar clinical manifestations. This study reports the first Brazilian family diagnosed with MCTO with progressive osteolysis of the carpal and tarsal bones, presenting a c.161C>T (p.Ser54Leu) heterozygous variant in the MAFB gene, describing the clinical, radiological, and molecular findings, compared with literature data, and discussing the different clinical and molecular diagnosis, as well as the natural history of the disease. Since MCTO is a disorder with progressive symptoms, an early diagnosis is important to avoid unnecessary investigations and treatments and to provide the proper follow-up.

Case report.
Juvenile rheumatoid arthritis (JRA) typically presents with fever, rash, anterior uveitis, and/or joint pain. We present three cases with initial torticollis due to rotatory subluxation of C1-C2 as an initial sign of JRA.
Three girls, ages 5-9, presented with C1-2 rotatory subluxation. Traction was able to reduce the atlanto-axial joint in all cases. Based on imaging, history, exam, and laboratory results, they were diagnosed with JRA. After reduction of the atlantoaxial joint, they were transitioned to a halo vest and disease-modifying antirheumatic drugs (DMARDs). The older 2 children underwent C1-2 fusion. The younger child has minimal symptoms and has not undergone surgical intervention 4 years from initial presentation.
Rotatory subluxation can be the first presenting sign of JRA. Younger children may be able to be treated conservatively with traction and medication, while older children may require occiput to C2 fusion due to bony destruction and basilar invagination.
IV.