Outcomes of transitional care programs on adolescent chronic inflammatory systemic diseases: systematic review and meta-analyses.

BACKGROUND: Patients with juvenile chronic inflammatory systemic diseases (jCID) are vulnerable to many circumstances when transitioning to adult-centered healthcare; this increases the burden of disease and worsen their quality of life. METHODS: MEDLINE, Embase, Web of Science and Scopus were searched from inception to March 16th, 2021. We included observational, randomized controlled trials and quasi-experimental studies that evaluated a transitional care program for adolescents and young adults with jCIDs. We extracted information regarding health-related quality of life, disease activity, drop-out rates, clinical attendance rates, hospital admission rates, disease-related knowledge, surgeries performed, drug toxicity and satisfaction rates. RESULTS: Fifteen studies met our inclusion criteria. The implementation of transition programs showed a reduction on hospital admission rates for those with transition program (OR 0.28; 95% CI 0.13 to 0.61; I 2 = 0%; p = 0.97), rates of surgeries performed (OR 0.26; 95% CI 0.12 to 0.59; I 2 = 0%; p = 0.50) and drop-out rates from the adult clinic (OR 0.23; 95% CI 0.12 to 0.46; I 2 = 0%; p = 0.88). No differences were found in other outcomes. CONCLUSION: The available body of evidence supports the implementation of transition programs as it could be a determining factor to prevent hospital admission rates, surgeries needed and adult clinic attendance rates.

Esophageal symptoms and their lack of association with high-resolution manometry in systemic sclerosis patients / Falta de asociación de síntomas esofágicos con la manometría de alta resolución en pacientes con esclerosis sistémica

Background: The esophageal involvement in systemic sclerosis (SSc) causes impact in the morbidity and mortality. High resolution manometry assesses esophageal involvement. Our aim was to categorize esophageal motor disorder in patients with SSc by HRM. Methods: We carried out an observational, descriptive and cross-sectional study. All patients underwent HRM as well as semi-structured interviews to assess frequency and severity of upper GI symptoms. Patients also completed the gastroesophageal reflux questionnaire (Carlsson-Dent). Results: We included 19 patients with SSc, 1 with morphea, and 1 with scleroderma sine scleroderma. Dysphagia and heartburn were the most frequent symptoms (61% each). We found an abnormal HRM in 15 (71.4%) patients. We found no statistically significant association between clinical or demographic variables and an abnormal HRM, or between any upper GI symptom and HRM findings. Conclusion: We observed a high prevalence of esophageal symptoms and of HRM abnormalities. However, there was no clear association between symptomatology and HRM findings. HRM does not seem to accurately predict upper GI symptomatology

Antecedentes: La afectación esofágica en la esclerosis sistémica causa impacto en la morbimortalidad. La manometría de alta resolución evalúa la afectación esofágica. Nuestro objetivo fue categorizar el trastorno motor esofágico en pacientes con esclerosis sistémica por HRM. Métodos: Se realizó un estudio observacional, descriptivo y transversal. Todos los pacientes se sometieron a HRM, así como a entrevistas semiestructuradas para evaluar la frecuencia y la gravedad de los síntomas gastrointestinales. Los pacientes también completaron el cuestionario de reflujo gastroesofágico (Carlsson-Dent). Resultados: Se incluyeron 19 pacientes con esclerosis sistémica, uno con morfea y uno con esclerodermia seno esclerodermia. La disfagia y la pirosis fueron los síntomas más frecuentes (un 61% cada uno). Encontramos una HRM anormal en 15 (71,4%) pacientes. No se halló ninguna asociación estadísticamente significativa entre las variables clínicas o demográficas y una HRM anormal, o entre cualquier síntoma GI superior y los hallazgos de HRM. Conclusión: Se observó una alta prevalencia de síntomas esofágicos y de anomalías de la HRM. Sin embargo, no hubo asociación clara entre la sintomatología y los hallazgos de HRM. La HRM no parece predecir con precisión la sintomatología gastrointestinal

Esophageal symptoms and their lack of association with high-resolution manometry in systemic sclerosis patients.

BACKGROUND: The esophageal involvement in systemic sclerosis (SSc) causes impact in the morbidity and mortality. High resolution manometry assesses esophageal involvement. Our aim was to categorize esophageal motor disorder in patients with SSc by HRM. METHODS: We carried out an observational, descriptive and cross-sectional study. All patients underwent HRM as well as semi-structured interviews to assess frequency and severity of upper GI symptoms. Patients also completed the gastroesophageal reflux questionnaire (Carlsson-Dent). RESULTS: We included 19 patients with SSc, 1 with morphea, and 1 with scleroderma sine scleroderma. Dysphagia and heartburn were the most frequent symptoms (61% each). We found an abnormal HRM in 15 (71.4%) patients. We found no statistically significant association between clinical or demographic variables and an abnormal HRM, or between any upper GI symptom and HRM findings. CONCLUSION: We observed a high prevalence of esophageal symptoms and of HRM abnormalities. However, there was no clear association between symptomatology and HRM findings. HRM does not seem to accurately predict upper GI symptomatology.

Imagen de «tiro al blanco» en una paciente con dolor abdominal y lupus eritematoso sistémico tratada con recambio plasmático / Target sign in woman with abdominal pain and systemic lupus erythematosus treated with plasma exchange

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Wernicke encephalopathy presenting in a patient with severe acute pancreatitis.

CONTEXT: Acute pancreatitis can lead to prolonged fasting and malnutrition. Many metabolic changes, including thiamine deficiency, may lead to the well know pancreatic encephalopathy. In this condition however the thiamine deficiency is rarely suspected. CASE REPORT: We report the case of a 17-year-old woman with severe acute pancreatitis who developed mental status changes and ophthalmoplegia. A magnetic resonance image showed hyperintensive signals in periventricular areas, medial thalamus, and mammillary bodies, findings consistent with the diagnosis of Wernicke encephalopathy. Thiamine treatment reversed neurological complications. CONCLUSION: Wernicke encephalopathy secondary to thiamine deficiency should be considered as a possible cause of acute mental status changes in patients with acute pancreatitis and malnutrition. Prophylactic doses of thiamine could be considered in susceptible patients.