Cross-cultural adaptation and initial validation of the Brazilian-Portuguese version of the pediatric automated neuropsychological assessment metrics.

Automated neuropsychiatric batteries have been used in research and clinical practice, including for chronic diseases, such as Systemic Lupus Erythematosus. The Pediatric Automated Neuropsychological Assessment Metrics battery (Ped-ANAM), originally developed for use in American-English speaking individuals, allows tracking of cognitive functions. It can be applied to people over 9 years old. The aim of this study was to translate and present initial validation data from the Ped-ANAM into Brazilian-Portuguese. We translated the battery according to Beaton's guidelines. Psychometric properties were tested, internal consistency was analyzed by Cronbach's alpha coefficient, test-retest reliability by the intraclass correlation coefficient (ICC). Further, we measured the test execution speed at both times as a temporal stability. Principal component analysis (PCA) was used for structural validity. Evidence of construct validity was assessed through assessment of the relationships with the Wechsler Intelligence Scales. All participants prior to the start of study related activities signed an informed consent form approved by the local ethics committee. A sample of 230 individuals [mean (range) of age: 23 (9 to 60) years; 65% females] was included; a subset of 51 individuals [mean (range) of age: 18 (9 to 57) years, 59% female] completed the Ped-ANAM twice to assess test-retest reliability, and another subset of 54 individuals [mean (range) of age: 20.4 (7 to 62) years; 67% female] completed the Wechsler Intelligence Scales for Children and Adult for assessment of the Ped-ANAM's construct validity. Our results suggest that the internal consistency of the Ped-ANAM (Cronbach's α = 0.890) and its subtest test-retest reliability were excellent (ICC: 0.59 to 0.94). There was no clustering in the Principal Components Analysis, suggestive of non-grouping of the evaluated variables. Construct validity assessment to the Wechsler Scales showed expected ranges of low to strong correlations (Spearman correlations: ρ = 0.40 to ρ = 0.69). We concluded that, based on the results of this study, a cross-culturally validated Brazilian-Portuguese version of the Ped-ANAM has been developed and it is a reliable tool for the screening cognitive function.

Hydroxychloroquine blood levels predicts flare in childhood-onset lupus nephritis.

OBJECTIVE: Low hydroxychloroquine (HCQ) blood levels are predictors of flare in adult lupus. Childhood-onset systemic lupus erythematosus (cSLE) has high morbidity with renal involvement in up to 80% of cases. The aim of this study is to determine the HCQ cut-off levels which predicts flare in childhood-onset lupus nephritis (LN). METHODS: Sixty LN patients on HCQ use for at least 6-months were prospectively evaluated at baseline (BL) and about 6-months later for cSLE flare and HCQ blood levels (ng/mL) measured by liquid chromatography-tandem mass spectrometry. RESULTS: There were 19 patients (32%) with flare, during the study with median SLEDAI increase of 4 (0-8). Median (IQR) BL HCQ levels of the flare group were lower compared to stable patients [557.5 (68.6-980.3) vs. 1061.9 (534.8-1590.0 ng/mL); p=0.012]. ROC curve analysis demonstrated that HCQ levels≤1075 ng/mL were associated with a 5.08 (95%CI 1.28-20.13; p=0.021) times increased risk of flare. Six-month HCQ levels revealed that most patients 24/54 (44%) had persistently low levels (≤1075) during follow-up. Among those, 11/24 (46%) had flare. Multiple logistic regression analysis including prednisone use, baseline SLEDAI-2K, adherence based on pharmacy refill and BL HCQ blood levels as possible predictors of flare revealed that only HCQ blood level was independently associated with flare (OR 0.999, 95%CI 0.998-1.0, p=0.013). CONCLUSIONS: We demonstrated that HCQ blood cut-off level under 1075 ng/mL predicts flare in childhood-onset LN patients under prescribed HCQ dose of 4.0-5.5 mg/kg/day. We further observed that most of these patients have compliance issues reinforcing the need for a close surveillance particularly in those with levels below the defined cut-off.

Taxonomy for systemic lupus erythematosus with onset before adulthood.

OBJECTIVE: To propose a common nomenclature to refer to individuals who fulfill the American College of Rheumatology classification criteria for systemic lupus erythematosus (SLE) during childhood or adolescence. METHODS: The medical literature was reviewed for studies conducted in the target population between 1960 and December 2011 to obtain information about the terms used to refer to such children and adolescents. We reviewed the threshold ages used and disease features considered to discriminate these individuals from patients with onset of SLE during adulthood. Furthermore, the nomenclature used in other chronic diseases with onset during both childhood and adulthood was assessed. RESULTS: There was an astonishing variability in the age cutoffs used to define SLE onset prior to adulthood, ranging from 14-21 years, but most studies used age 18 years. The principal synonyms in the medical literature were SLE without reference to the age at onset of disease, childhood-onset SLE, juvenile SLE, and pediatric (or paediatric) SLE. CONCLUSION: Based on the definition of childhood, in analogy with other complex chronic diseases commencing prior to adulthood, and given the current absence of definite genetic variations that discriminate adults from children, the term childhood-onset SLE is proposed when referring to individuals with onset of SLE prior to age 18 years.