The aim of this study is to evaluate the Nicaraguan screening program for congenital hypothyroidism in terms of coverage and effectiveness of detection and confirmation of cases with the condition throughout a decade. Thyrotropin was quantified in cord-blood samples by a validated ELISA and a cut-off of 20 mU/l was applied. Coverage, positive predictive value, recall rate and prevalence were retrospectively analysed. Babies with positive screening results were contacted for confirmation by means of determination of thyrotropin and thyroid profile in serum samples. 272,338 babies were screened during the period 2005-2015. The mean coverage reached by the program in the participating departments was 71%, with a positive predictive value of 83% and a recall rate of 0.055%. Eighty cases of congenital hypothyroidism were identified, representing an incidence of 1 in 3229 live births, most of them (81%) being severe. The performance of the Nicaraguan screening program is comparable to those in Latin America also using cord-blood samples. The incidence of congenital hypothyroidism is within the low range of other countries worldwide. Strategies are needed to expand the program to the whole country, improve recall rates and achieve earlier treatment of babies, with the condition.
BackgroundThe Health Assessment Questionnaire (HAQ) is an instrument administered to patients to self-report functional status originally in rheumatoid arthritis (RA). In Argentina, it has been translated and validated for RA in 2004. For diffuse SSc, HAQ has been associated to morbidity and mortality.ObjectivesTo adapt S-HAQ into Spanish and to assess its validity in SSc patients in Argentina.MethodsS-HAQ was translated following a forward-backward translation procedure of the original English version, and transcultural adaptation was performed by a comprehension test reaching the final Spanish version. SSc patients that fulfilled ACR 80 criteria and early Systemic Sclerosis according to Le Roy and Medsger criteria were included. Patients with overlap were excluded. Cronbach's alpha and item-item item-total correlations were used to assess internal consistency. Construction validity was analyzed through factor analysis with Varimax rotation. Continuous variables were compared by t-test, Mann-Whitney or Kruskal-Wallis test, and categorical variables by chi-square or Fisher's test. A value of p<0.05 was considered significant.Resultsl9An adapted Argentine-Spanish version of S-HAQ was developed. One hundred patients were surveyed; 84% were female, mean age 54±12.8 years and disease duration 8.8±9.1 years. Limited SSc was more frequent (63%), followed by diffuse SSc (36%). Serologically, 89% were ANA positive, 27% had anti Scl 70 and 41% had anti centromere antibodies. Median Rodnan score (mRSS) was 9.8 (0–40.5) and median activity measured by EUSTAR was 1.25 (0–6). Median S-HAQ was 0.62 (0–2.5), Cronbach's alpha 0.89, and when removing questions one by one the coefficient decreased. Median VAS (visual analogue scale) was 0.57 (0–2.8). Factor analysis identified two factors for the S-HAQ: factor 1: dressing (0.61), arising (0.68), reach (0.63), and personal hygiene (0.70); factor 2: eating (0.68), grip (0.72), walking (0.49), usual activities (0.62). For questions, three factors were identified through VAS: factor 1: overall disease severity (0.63) and gastro-intestinal symptoms (0.57); factor 2: Raynaud's (0.66), digital ulcers (0.56); factor 3: respiratory symptoms (0.43). There was a statistically significant association between higher values of S-HAQ and higher values of mRSS (1.1±0.74 vs. 0.64±0.5 p=0.002) and also with seropositivity for anti-Scl 70 (p=0.003). Higher values of total VAS were associated to female gender (0.75±0.5 vs. 0.49±0.71, p=0.01). There was a significant association between S-HAQ and MEDSGER (p=0.04) and EUSTAR (p=0.03) scores; likewise, between VAS and MEDSGER (p=0.00) and EUSTAR (p=0.00) scores.ConclusionsA Spanish version of S-HAQ was developed, showing an acceptable reliability and validity.Disclosure of InterestNone declared
BackgroundHand function in scleroderma patients can be assessed by specific instruments such as the Cochin scale and the Scleroderma Health assessment questionnaire (SHAQ), both self administrated. Sanqvist and Eklun designed the Hand Mobility in Systemic Sclerosis test (HAMIS), which is performed by the evaluation of a health professional. The original instrument was designed in English.ObjectivesThe aim of this study was to adapt into Spanish the HAMIS test in order to assess hand function in patients with systemic sclerosis and to evaluate its reliability and validity.MethodsThe study included 28 patients that met ACR/EULAR 2013 classification criteria for systemic sclerosis, who attended the Rheumatology Service from March 2014 to March 2015; 21 were diffuse and 7 limited. Patients with overlapping and sclerodermiform syndromes were excluded. Demographic data, clinical manifestations, laboratory studies including ANA by IIF (Hep2) were recorded. Different scores were performed: modified Rodnan, Medsger and Cochin. HAMIS was translated into Spanish and back translated into English. Four translators did not know the original English version of the test. A pilot test was conducted in 5 systemic sclerosis patients and adaptations were performed in order to obtain the final Spanish version. HAMIS test was assessed by an occupational therapist. Two weeks later, the study was repeated in 10 patients chosen randomly to assess stability (test-retest) and concordance according to Bland&Altman. Values of HAMIS and Cochin test were correlated (Spearman's Rank correlation coefficient).ResultsAll 28 patients (27 female) had mean age at diagnosis of 49.6±13 years and median disease duration of 5 years (range=0–25). All patients developed Raynaud's phenomenon (mean age at onset of Raynaud of 45±17 years), 3 patients had puffy hands, 11 presented digital ulcers, 9 pitting scars and 23 telangiectasia. Through capillaroscopic evaluation, early pattern was found in 11 patients, active in 7 and late in 8 patients. When analyzing severity by Medsger score, such was normal in 1 patient, 11 presented mild involvement, 13 moderate and 3 severe. Median for Rodnan score was of 6 (range=2–27) and 8 for Cochin (range=0–38). Median for HAMIS was of 6 (range=0–21) with Cronbach's alpha of 0.81; in test-retest: mean of the difference was of 0.091 (range= -5 to +5). Correlation between HAMIS and Cochin was significant (r=0.65; p=0.0002)ConclusionsAn adapted Spanish version of HAMIS was developed, showing an acceptable reliability (Cronbach and stability) and criterion validity (correlation with Cochin test).Disclosure of InterestNone declared
Nuestro objetivo fue describir la frecuencia de úlceras digitales en una población de pacientes con Esclerosis Sistémica y comparar las características clínicas de los que desarrollaron úlceras de los que no lo hicieron. Se incluyeron en forma retrospectiva pacientes que cumplían criterios ACR para Esclerosis Sistémica. Se recolectaron datos demográficos, clínicos y serológicos de las historias clínicas. Se clasificó a los pacientes en dos grupos: un Grupo A con úlceras digitales y Grupo B aquellos pacientes sin antecedentes de úlceras digitales. Se compararon ambos grupos. Se estudiaron 60 pacientes con diagnóstico de Esclerosis Sistémica, 33% subtipo difuso, edad promedio al diagnóstico de 50,75 ± 14,75 años, el 15% (9 pacientes) eran de sexo masculino.
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