Background:
Achievement of a normal peak bone mass is an especially important consideration in young adults with juvenile idiopathic arthritis (JIA), because interference with attainment of peak bone ...mass may not be repaired later in life. It has been suggested that children with JIA cortical appendicular skeletal bone is more affected by disease activity than axial trabecular bone. To evaluate bone mineral density (BMD) DXA measurements are widely used. Although, DXA is limited to two-dimensional evaluation of integral BMD and cannot differentiate between trabecular and cortical bone compartments. Alternatively, 3D-DXA analysis is a new method based on DXA scans of proximal femur that provides accurate estimates of trabecular and cortical volumetric BMD.
Objectives:
The aim of the study was to analyze the trabecular and cortical bone using 3D-DXA in young adults with JIA compared to age-matched healthy controls.
Methods:
This cross-sectional study was aimed to analyze the differences in 3D-DXA proximal femur compartments. The patients were recruited from the specialized transitional unit of the Vall d’Hebron University Hospital. JIA patients older than 18 yo without previous bisphosphonates intake were included. Age and sex-matched healthy controls were selected. DXA scans (Lunar Prodigy, General Electric Medical Systems, v.15) were acquired. OP was defined according to the WHO criteria. The 3D-DXA software was used to assess in 3D the trabecular density and cortical thickness from DXA scans as reported previously 1. The 3D-DXA and DXA measurements were compared using Student’s
t
test. Categorical variables were compared using Chi-squared test. All hypothesis tests with p value lower than 5% were considered significant.
Results:
Forty-six patients and 46 age and sex-matched controls were evaluated. The mean time of disease’s duration was 7.37 years, and the most frequent JIA subtype was Oligoarticular ANA positive. More than 21% of the patients had an active disease. OP was present in 5 patients and 1 control. Despite not finding significant differences in the prevalence of osteoporosis, JIA patients had lower aBMD at total hip by DXA and lower cortical sBMD by 3D-DXA than controls (Table 1).
Table 1.
Comparison of DXA and 3D-DXA measurements between JIA and healthy controls
Variable
JIA patients (n=46)
Healthy controls (n=46)
P value
DXA
Lumbar aBMD (g/cm
2
), mean ± sd
1.097 ± 0.02
1.155 ± 0.02
0.042
Right femoral neck aBMD (g/cm
2
), mean ± sd
0.930 ± 0.02
1.014 ± 0.01
0.041
Right total hip aBMD (g/cm
2
), mean ± sd
0.932 ± 0.02
1.001 ± 0.01
0.008
Lumbar T-score, mean ± sd
-0.716 ± 0.16
-0.212 ± 0.16
0.037
Right femoral neck T-score, mean ± sd
-0.563 ± 0.17
0.087 ± 0.12
0.036
Right total hip T-score, mean ± sd
-0.721 ± 0.18
-0.12 ± 0.13
0.008
3D-DXA
Trabecular vBMD (mg/cm
3
), mean ± sd
180.44 ± 5.54
194.57 ± 5.11
0.064
Cortical sBMD (mg/cm
2
), mean ± sd
147.23 ± 3.41
161.52 ± 2.89
0.002
Osteoporosis (WHO definition)
Yes, n (%)
5 (10.87)
1 (2.17)
0.091
No, n (%)
41 (89.13)
45 (97.83)
DXA
dual-energy X-ray absorptiometry
, sd
standard deviation
, aBMD
areal bone mineral density,
3D-DXA
three-dimensional dual-energy X-ray absorptiometry,
vBMD
volumetric bone mineral density,
sBMD
superficial cortical bone mineral density
Conclusion:
JIA patients do not have more OP than controls in our cohort but there are differences in BMD in the different locations and greater involvement of the cortical bone in JIA.
References:
1Brance ML, et al. Trabecular and cortical bone involvement in rheumatoid arthritis by DXA and DXA-based 3D modelling. Osteoporos Int, 2020 Sep 24.
Disclosure of Interests:
None declared
Evaluar el tratamiento con una pauta insulínica con análogo glargina en niños y adolescentes y valorar el grado de satisfacción de los pacientes y sus padres con esta nueva pauta.
Estudio prospectivo ...de 18 meses de duración con 42 pacientes con diabetes mellitus tipo 1, 27 mujeres y 15 varones, con edad media al inicio de 6,8 años (rango: 1,2-13,2), edad media al inicio del tratamiento con glargina 12,8 años (rango: 7,0-17,7), tiempo de evolución medio 6,1 años (rango: 2,0-11,9). Las indicaciones de la nueva pauta fueron mal control metabólico o hipoglucemias frecuentes con la pauta intensiva con 3 dosis de insulina de acción intermedia (NPH), que fueron sustituidas por una dosis de glargina. El nivel de hemoglobina A
1c (HbA
1c), la dosis diaria de insulina, el índice de masa corporal (IMC) y la satisfacción de pacientes y padres con el tratamiento de la diabetes se valoraron. Pruebas ANOVA (análisis de la varianza), t de Student, Mann-Whitney y Fisher.
Tras 18 meses de tratamiento con la nueva pauta, se observó una disminución del nivel de HbA
1c (7,65
±
0,74% frente a 8,03
±
0,69 %; p
=
0,001), sin cambios significativos en la dosis de insulina (1,03 ± 0,19 U/kg/día frente a 1,08
±
0,21
U/kg/día; p
=
0,052) ni en la EDE (escala de desviación estándar) del IMC (+0,51
±
0,96 frente a ±0,61
±
1,02; p
=
0,11), mientras que aumentó el grado de satisfacción con el tratamiento de los niños (+44,5
±
18,8 puntos en la escala frente a −9,9
±
26,8; p
=
0,001) y de sus padres (+42,0
±
17,9 puntos en la escala frente a −20,8
±
29,1; p
=
0,001).
1. La pauta insulínica con glargina mejora el control metabólico en niños y adolescentes con diabetes mellitus tipo 1 en tratamiento intensivo.
2. También mejora el grado de satisfacción de pacientes y padres con el tratamiento de la diabetes.
To evaluate the use of insulin glargine in intensively-treated children and adolescents. To assess the degree of patient and parent satisfaction with this treatment.
We studied 42 patients with type 1 diabetes. There were 27 girls and 15 boys. The mean age at diagnosis was 6.8 years (range 1.2-13.2), the mean age at initiation of glargine therapy was 12.8 years (range 7.0-17.7), and the mean duration of diabetes was 6.1 years (range 2.0-11.9). Glargine indications were poor metabolic control or frequent hypoglycemia with multiple daily injections of NPH insulin, which were substituted by one dose of glargine. Patient and parent satisfaction with diabetes treatment was assessed with the scale published by Boot. ANOVA, Student's t test, Mann-Whitney and Fisher tests were applied.
Variables are reported as mean ± standard deviation. After 18 months, glargine reduced hemoglobin A
1c levels (7.65%
±
0.74 vs. 8.03%
±
0.69; p
=
0.001), with no significant changes in insulin dose (1.03
±
0.19
U/kg/day vs. 1.08
±
0.21; p
=
0.052) or body mass index SDS (standard deviation score) (+0.51
±
0.96 vs. +0.61
±
1.02; p
=
0.11).Glargine also increased patient satisfaction (+44.5
±
18.8 points vs. −9.9
±
26.8; p
=
0.001) and parent satisfaction (+42.0
±
17.9 points vs. −20.8
±
29.1; p
=
0.001) with diabetes treatment.
1. Glargine insulin improves metabolic control in intensively- treated children and adolescents with type 1 diabetes.
2. Glargine also improves patient and parent satisfaction with diabetes treatment.
Background:
STING-associated vasculopathy with onset in infancy (SAVI syndrome) can mimic Juvenile Idiopathic Arthritis.
Objectives:
The aim of this study is to describe a detailed cohort of patients ...with SAVI syndrome and highlight the similarity, in some cases, of the phenotype of this disease with Juvenile Idiopathic Arthritis.
Methods:
3 patients diagnosed with SAVI syndrome from the institution Hospital Universitari Vall d’Hebron were recruited. Written informed parental consent was obtained for the use of clinical data and pictures reported. Demographic, clinical, analytical, lung function and previous and current treatment are described.
Results:
Patient 1, a 11-year-old boy, was identified to carry a de novo p.V155M mutation in TMEM173. He presented at first month of life with recurrent bronchial infection and skin vasculitis lesions in nose, cheeks and toes. Arthritis affected hands, toes and knees but no erosions were found at X-Ray. Fever was not reported. High-resolution computed tomography (HRCT) of the lungs identified a nonspecific interstitial pneumonia (NSIP) and a lung biopsy showed lymphoid hyperplasia. Elevated inflammatory markers were reported and rheumatoid factor (RF), ACPA antibodies and antinuclear antibodies (ANA) were also positive. At the age of 6 years Ruxolitinib (RX) was introduced at the initial dose of 5 mg twice daily with an improvement of skin disease and lung function. Arthritis was well controlled and RX was well tolerated.
Patient 2, a 17-year-old girl, was identified to carry a de novo p.V155 mutation in TMEM173. She presented at the age of 3 with a severe polyarthritis of large and small joints. No fever, skin or respiratory symptoms were reported at the beginning of the disease. Laboratory tests were positive for RF and ACPA antibodies. She was diagnosed with Polyarticular JIA and was treated with steroids and Methotrexate without improvement. Few months later she reported dyspnoea with recurrent bronchial infections. HRCT showed NSIP and lymphoid interstitial pneumopathy was found at the lung biopsy. RX was initiated at the age of 17 years but at this time lung fibrosis was stablished. Moreover, RX was not well tolerated due to headache. She requires continuous domiciliary oxygen and has been included to lung transplant.
Finally, patient 3, a 29-year-old man, was recently diagnosed with a de novo p.V155 mutation in TMEM173. He presented at the age of 7 years with symmetrical polyarticular arthritis after a bronchial infection that course with fever. No skin manifestations were objectified. Autoimmune lab test was positive for RF, ACPA, and ANA. With the diagnosis of Polyarticular JIA he received different treatments with no response. Due to recurrent bronchial infections a HRCT was performed showing an ILD at bases and follicular bronchiolitis with NSIP pattern in a lung biopsy. Functional tests were worsening without any response to different treatments. SAVI syndrome was suspected, and genetic test was performed with positive result. RX was initiated but compliance was not good
Conclusion:
SAVI syndrome is a rare monogenic autoinflammatory disease with few cases reported in the literature. Disease phenotype could be different in every patient, with no presence of skin vasculitic lesions or fever. Patient 2 and 3, in contrast with patient 1, had severe articular and lung manifestations with no skin involvement. Furthermore, lab tests were positive for RF and ACPA and were misdiagnosed as JIA so genetic test was performed later in the follow-up. Being aware of the distinct phenotype of the disease could help the clinicians to make a PRONTO diagnostic and reassess the patients with these presentations that not respond well to conventional treatments.
References:
1Liu Y, et al. Activated STING in a vascular and pulmonary syndrome. N Engl J Med. 2014 Aug 7;371(6):507-518.
Disclosure of Interests:
None declared
Malnutrition among hospitalized patients has clinical implications, and interest has arisen to find screening tools able to identify subjects under risk. At present, there is no consensus about the ...most suitable nutrition screening tool for pediatric patients.
To validate STAMP (Screening Tool for the Assessment of Malnutrition in Pediatrics) pediatric screening tool in Spain.
Descriptive cross-sectional study of patients admitted to a 3rd level children's hospital with both medical and surgical specialities. During the first 24 hours of admission, STAMP screening tool was applied. For its validation, results were compared with those obtained from a nutritional assessment performed by specialist staff, which included clinical, anthropometric and body composition data.
A sample of 250 children was studied. Nutritional assessment identified 64 patients (25.6%) under risk, 40 of whom were malnourished (16%). STAMP classified 48.4% of the patients as being under nutritional risk. This tool showed 75% sensitivity and 60.8% specificity when identifying patients under risk according to nutritional assessment. It showed 90% sensitivity and 59.5% specificity when identifying malnourished patients.
Malnutrition was less frequent than that reported in other European countries, although diagnosis technique was different. STAMP is a simple and useful tool for nutritional screening, avoiding the need to assess all patients on admission in order to identify those under nutritional risk.
Introducción: La malnutrición en los pacientes hospitalizados tiene implicaciones clínicas y evolutivas, por lo que existe interés en desarrollar métodos de cribado que identifiquen los individuos de ...riesgo. En la actualidad no existe consenso acerca de la herramienta de cribado nutricional más apropiada para aplicar en población pediátrica. Objetivo: Validar en España la herramienta de cribado nutricional pediátrico STAMP (Screening Tool for the Assessment of Malnutrition in Pediatrics). Métodos: Estudio descriptivo transversal en pacientes ingresados en un hospital pediátrico de tercer nivel con diferentes especialidades médicas y quirúrgicas. En las primeras 24 horas de ingreso se aplicó el método de cribado nutricional STAMP. Para la validación de sus resultados se llevó a cabo una valoración del estado nutricional que incluyó datos clínicos, antropométricos y de composición corporal realizada por personal especializado en nutrición. Resultados: Fueron estudiados 250 niños. La valoración nutricional detectó 64 pacientes (25,6%) considerados de riesgo, de los cuales 40 (16%) estaban ya malnutridos. STAMP clasificó un 48,4% de la muestra como de riesgo nutricional elevado. Dicho método mostró una sensibilidad del 75% y una especificidad del 60,8% para identificar los pacientes considerados de riesgo en la valoración nutricional, y una sensibilidad del 90% y especificidad del 59,5% para detectar los malnutridos. Comentarios: La frecuencia de malnutrición fue algo inferior a la de otros países de nuestro entorno, aunque el método diagnóstico fue diferente. El método STAMP es una herramienta sencilla y útil para el cribado nutricional, que evitaría la necesidad de valorar a todos los pacientes al ingreso para detectar los sujetos de riesgo.
BackgroundJuvenile psoriatic artritis (JPSA), a subtype of juvenile idiopathic arthritis (JIA), constitutes 5% of JIA. The literature is inconsistent regarding features of JPSA, and physicians debate ...whether it is a distinct entity within JA. Moreover, the criteria diagnosis for JPsA is currently in debate.There are two pediatric diagnostic classifications based on clinical criteria: ILAR classification criteria and Vancouver classification criteria. Classification of JIA has been a great debate since this entity disappeared. 1 The CASPAR criteria are standard in adults. These criteria have a specificity of 91.4% and a sensitivity of 98.7%, makingdiagnostic classification easier. However, the peculiarities of childhood cause some differences to consider.ObjectivesTo compare 3 diagnostic criteria for JPSA ILAR, Vancouver and CASPAR.MethodsMulticenter, cross-sectional study of data from children with JPsA (diagnosis by prescritor physician) who consecutivelly attended in ped-rheumatology outpatient clinic and enrolled from 9 centers within the last 1.5 years. Sociodemographic, clinical characteristics and family history of Psoriasis were collected to assess compliance with diagnostic classification criteria using using the Cohen’s Kappa statistic index.ResultsForty eight children were included with the following sociodemographics characteristics;(table1) Thirty eight children who met the VANCOUVER criteria (80,9%) while thirty two children met the ILAR criteria (68.1%). As for the CASPAR criteria, thirty eight children were diagnosed (80.9%). The diagnostic agreement between the ILAR and CASPAR criteria and ILAR and Vancouver criteria (diagnosed defined) was weak (K=0.67 and K=0.67). In contrast, the agreement was total (K=1 between the CASPAR and Vancouver criteria.ConclusionDespite minimal changes between the Vancouver and ILAR criteria, the ILAR exclusion criteria limit the diagnosis of PsA in childhood. Given that the CASPAR and Vancouver were able to detect a significant number of patients with PsA in our series (predominantly adolescents), the application of the CASPAR criteria in the subgroup of children with late onset could be considered.References1 Alberto martini, Angelo Ravelli, Tad Avcin, et al. Toward new Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, Pediatric Rheumatology International Trials Organization International Consensus; J Rheumatol. 2019: 46(2):190-197.Table 1.Clinical and demographic data of the study populationTotal (n=48)Sex, female (%)34(70.8)Age at inclusion (years), mean (SD)11.5(4)Age at articular onset (years), mean (SD)7.1(5)Age at psoriasis onset (years), mean (SD)10(3.2)Type of onset Oligoarthritis n (%)33(68.7) Poliarthritis n (%)12(25) No arthritis n (%)3(6.2)Family with Psoriasis39(81.2)Extra-articular involvement Uveitis n (%)(12.5) Entesitis n (%)7(14.6) Onicopathy n(%)14(29.2)---.Skin-Psoriasis26(54.2)Lab AAN: positive n (%)19(39.5) RF: negative n (%)48(100) HLAB27: negative n (%):43(89.6)Acknowledgements:NIL.Disclosure of InterestsNatalia Palmou-Fontana: None declared, Inmaculada Calvo Speakers bureau: ABVIEE, NOVARTIS, JANSEN, GSK, GISELLA DIAZ CORDOVES REGO: None declared, ADRIAN GARCIA-ROGERO: None declared, Juan Carlos Lopez Robledillo: None declared, Berta Paula Magallares López: None declared, Pablo Mesa del Castillo: None declared, ESTEFANIA MORENO RUZAFA: None declared, CARLOS REDONDO-FIGUERO: None declared, MARTINA STEINER: None declared, PAZ COLLADO: None declared.
Resumen Objetivos Calcular la prevalencia de obesidad y sobrepeso en niños y adolescentes de nuestra ciudad e investigar los factores asociados. Sujetos y métodos Estudio transversal de 1.317 niños y ...adolescentes de 2 a 16 años. Mediante muestreo probabilístico polietápico se seleccionaron 3 grupos: 411 de 12 a 16, 504 de 6 a 12 y 402 de 2 a 6 años. Se les calculó el índice de masa corporal y se definió obesidad y sobrepeso según la International Obesity Task Force. Se realizó un cuestionario de consumo de alimentos y de características clínicas y sociodemográficas. Los resultados se expresan como porcentajes (intervalos de confianza al 95%). Mediante regresión logística múltiple se estudió la asociación entre exceso de peso (obesidad y sobrepeso) y las distintas variables, calculando la odds ratio (OR) ajustada. Resultados El 9,5% (8,0-11,0) de los niños y adolescentes de 2 a 16 años son obesos y 22,4% (23,3-24,6) tienen sobrepeso. En el grupo de 12 a 16 años, el 8,5% (5,9-11,2) son obesos y el 20,5% (16,7-24,3) tienen sobrepeso, en el grupo de 6 a 12 años el 11,6% (8,9-14,3) y el 31,0% (27,0-35,0) y en el de 2 a 6 años el 8,0% (5,4-10,6) y el 13,6% (10,3-16,9), respectivamente. Se asocian con el exceso de peso la edad (OR 1,21; p < 0,001), la obesidad materna (OR 10,99; p = 0,008), el peso al nacer mayor de 4 kg (OR 2,91; p = 0,002) y la lactancia artificial exclusiva (OR 1,82; p = 0,005). Conclusión La obesidad y el sobrepeso infantil y juvenil son problemas extraordinariamente prevalentes en nuestra ciudad.
Introducción: La malnutrición en los pacientes hospitalizados tiene implicaciones clínicas y evolutivas, por lo que existe interés en desarrollar métodos de cribado que identifiquen los individuos de ...riesgo. En la actualidad no existe consenso acerca de la herramienta de cribado nutricional más apropiada para aplicar en población pediátrica. Objetivo: Validar en España la herramienta de cribado nutricional pediátrico STAMP (Screening Tool for the Assessment of Malnutrition in Pediatrics). Métodos: Estudio descriptivo transversal en pacientes ingresados en un hospital pediátrico de tercer nivel con diferentes especialidades médicas y quirúrgicas. En las primeras 24 horas de ingreso se aplicó el método de cribado nutricional STAMP. Para la validación de sus resultados se llevó a cabo una valoración del estado nutricional que incluyó datos clínicos, antropométricos y de composición corporal realizada por personal especializado en nutrición. Resultados: Fueron estudiados 250 niños. La valoración nutricional detectó 64 pacientes (25,6%) considerados de riesgo, de los cuales 40 (16%) estaban ya malnutridos. STAMP clasificó un 48,4% de la muestra como de riesgo nutricional elevado. Dicho método mostró una sensibilidad del 75% y una especificidad del 60,8% para identificar los pacientes considerados de riesgo en la valoración nutricional, y una sensibilidad del 90% y especificidad del 59,5% para detectar los malnutridos. Comentarios: La frecuencia de malnutrición fue algo inferior a la de otros países de nuestro entorno, aunque el método diagnóstico fue diferente. El método STAMP es una herramienta sencilla y útil para el cribado nutricional, que evitaría la necesidad de valorar a todos los pacientes al ingreso para detectar los sujetos de riesgo.Background: Malnutrition among hospitalized patients has clinical implications, and interest has arisen to find screening tools able to identify subjects under risk. At present, there is no consensus about the most suitable nutrition screening tool for pediatric patients. Aim: To validate STAMP (Screening Tool for the Assessment of Malnutrition in Pediatrics) pediatric screening tool in Spain. Methods: Descriptive cross-sectional study of patients admitted to a 3rd level children's hospital with both medical and surgical specialities. During the first 24 hours of admission, STAMP screening tool was applied. For its validation, results were compared with those obtained from a nutritional assessment performed by specialist staff, which included clinical, anthropometric and body composition data. Results: A sample of 250 children was studied. Nutritional assessment identified 64 patients (25.6%) under risk, 40 of whom were malnourished (16%). STAMP classified 48.4% of the patients as being under nutritional risk. This tool showed 75% sensitivity and 60.8% specificity when identifying patients under risk according to nutritional assessment. It showed 90% sensitivity and 59.5% specificity when identifying malnourished patients. Comments: Malnutrition was less frequent than that reported in other European countries, although diagnosis technique was different. STAMP is a simple and useful tool for nutritional screening, avoiding the need to assess all patients on admission in order to identify those under nutritional risk.
To evaluate the use of insulin glargine in intensively-treated children and adolescents. To assess the degree of patient and parent satisfaction with this treatment.
We studied 42 patients with type ...1 diabetes. There were 27 girls and 15 boys. The mean age at diagnosis was 6.8 years (range 1.2-13.2), the mean age at initiation of glargine therapy was 12.8 years (range 7.0-17.7), and the mean duration of diabetes was 6.1 years (range 2.0-11.9). Glargine indications were poor metabolic control or frequent hypoglycemia with multiple daily injections of NPH insulin, which were substituted by one dose of glargine. Patient and parent satisfaction with diabetes treatment was assessed with the scale published by Boot. ANOVA, Student's t test, Mann-Whitney and Fisher tests were applied.
Variables are reported as mean 6 standard deviation. After 18 months, glargine reduced hemoglobin A1c levels (7.65 % +/- 0.74 vs. 8.03 % +/- 0.69; p = 0.001), with no significant changes in insulin dose (1.03 +/- 0.19 U/kg/day vs. 1.08 +/- 0.21; p = 0.052) or body mass index SDS (standard deviation score) (+0.51 +/- 0.96 vs. 10.61 +/- 1.02; p = 0.11). Glargine also increased patient satisfaction (+44.5 +/- 18.8 points vs. -9.9 +/- 26.8; p < 0.001) and parent satisfaction (+42.0 +/- 17.9 points vs. -20.8 +/- 29.1; p < 0.001) with diabetes treatment.
1. Glargine insulin improves metabolic control in intensively-treated children and adolescents with type 1 diabetes. 2. Glargine also improves patient and parent satisfaction with diabetes treatment.
The structure of fish assemblages accounted for by different sampling methods (namely fyke net, seine nets, visual census) applied to vegetated and unvegetated lagoon habitats was investigated in ...terms of species composition, functional groups (ecological and trophic guilds), and fish size distribution. Significant differences were detected among methods, even among similar ones (seine nets). Visual census and fyke net detected more easily pelagic species, allowing the sampling of larger fish, whereas seine nets targeted more efficiently benthic-demersal species, with a dominance of 2–10 cm size classes in the fish catches. Differences were detected also among habitats, reflecting the different fish assemblages associated to vegetated and unvegetated habitats in coastal lagoons and transitional waters. However a different ability of discriminating between habitat-associated fish assemblages was recorded for the sampling methods. The different selectivity and functioning of the tested sampling methods confirm the importance of considering the targeted scale at which the research is being carried out, as well as the method that will be used to assess the ecological status of lagoon fish assemblages when choosing the most appropriate sampling method. A cross-validation of fish sampling methodologies in transitional waters is necessary to cope with the mandatory of the Water Framework Directive of standardization and comparability of monitoring methods.
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