Síndrome de Alagille. Experiencia clínica y sobrevida de un caso

Paciente masculino de 7 años de edad, padres y hermana de 11 años sanos. A los 15 días de nacido inicia con vómitos, ictericia y acolia. Al examen mostró rasgos particulares: frente ancha, puente nasal deprimido, mentón puntiagudo cara triangular y ojos profundos. Escleras y piel con tinte ictérico-verdoso, xantomas faciales. Soplo cardíaco sistólico III/VI en foco pulmonar. Hígado 4 cm debajo del reborde costal derecho. Perfil bioquímico: colestasis, hiperamonemia e hiperlipidemia. Radiografía de columna: hemivertebras. Ultrasonido: sin dilatación de vías biliares intrahepáticas, vesícula biliar atrófica. Ecocardiograma: estenosis de la arteria pulmonar y persistencia del conducto arterioso. Biopsia hepática: ductopenia, fibrosis leve. Los criterios de colestasis, facies peculiar, cardiopatía congénita y hemivertebras con ductopenia sustentan el diagnóstico de Síndrome de Alagille. Su evolución 7 años después demuestra que cursa con enfermedad renal estadio II, acidosis tubular renal, hipertensión arterial, hiperparatiroidismo secundario, anemia microcítica-hipocrómica, detención del crecimiento y retraso psicomotriz; complicaciones compensadas con el tratamiento médico, que le permite llevar una calidad de vida satisfactoria. El síndrome es una alteración autosómica dominante con expresión variable y el pronóstico de sobrevida depende del grado de compromiso y malformación de órganos y sistemas; la literatura reporta que la esperanza de vida a 20 años es entre 75-80%. Es importante reconocer este síndrome (incidencia mundial 1/100,000 nacidos vivos) dentro del diagnóstico diferencial de colestasis infantil para identificarlo y determinar su frecuencia

Reducing the environmental impact of surgery on a global scale: systematic review and co-prioritization with healthcare workers in 132 countries

Abstract Background Healthcare cannot achieve net-zero carbon without addressing operating theatres. The aim of this study was to prioritize feasible interventions to reduce the environmental impact of operating theatres. Methods This study adopted a four-phase Delphi consensus co-prioritization methodology. In phase 1, a systematic review of published interventions and global consultation of perioperative healthcare professionals were used to longlist interventions. In phase 2, iterative thematic analysis consolidated comparable interventions into a shortlist. In phase 3, the shortlist was co-prioritized based on patient and clinician views on acceptability, feasibility, and safety. In phase 4, ranked lists of interventions were presented by their relevance to high-income countries and low–middle-income countries. Results In phase 1, 43 interventions were identified, which had low uptake in practice according to 3042 professionals globally. In phase 2, a shortlist of 15 intervention domains was generated. In phase 3, interventions were deemed acceptable for more than 90 per cent of patients except for reducing general anaesthesia (84 per cent) and re-sterilization of ‘single-use’ consumables (86 per cent). In phase 4, the top three shortlisted interventions for high-income countries were: introducing recycling; reducing use of anaesthetic gases; and appropriate clinical waste processing. In phase 4, the top three shortlisted interventions for low–middle-income countries were: introducing reusable surgical devices; reducing use of consumables; and reducing the use of general anaesthesia. Conclusion This is a step toward environmentally sustainable operating environments with actionable interventions applicable to both high– and low–middle–income countries

Logística inversa para la optimización de procesos en la empresa Metrotech, S. A.

Realizar un análisis completo e integral de la cadena de distribución bajo la cual opera la empresa Metrotech, S. A., proponiendo una solución eficaz y directa a los principales problemas que limitan la rentabilidad de la misma, así mejorar los procesos y organización de la empresa con base en la aplicación de la logística inversa para detectar problemas e inconveniente

Caso probable de Zica. Reacción cruzada con infección previa por dengue

Paciente femenina de 5 años de edad, de Santa Lucía Cotzumalguapa, Escuintla. Consulta por fiebre, malestar general, cefalalgia, exantema maculopapular eritematoso, vómitos y dolor abdominal en región epigástrica de 5 días de evolución. Datos positivos al examen físico: Temperatura axilar 38°C, dolor abdominal difuso a la palpación profunda y dolor músculo esquelético en extremidades. Hematología: 9,290 leucocitos (45% de neutrófilos), hemoglobina 13.58 g/dL, plaquetas 26,400/cc, velocidad de sedimentación 3 mm/h, proteína C reactiva 5 mg/L, aspartato-aminotransferasa 148 U/L, panel viral de hepatitis negativo, albumina sérica 2.7 g/dL. Radiografía de tórax: derrame pleural derecho. Por proceder de área endémica y llenar criterios de caso probable de dengue se hizo serología; dengue IgM (-), IgG (+), chikungunya IgM (-), IgG (-), zika IgM (+), IgG (-). Se consideró como caso probable de zika y se solicitó pruebas confirmatorias. Antígeno para dengue (-), RT-PCR para chikungunya (-), RT-PCR para zika (-), con lo que se descartó la infección por zika. El tratamiento fue sintomático y la evolución fue satisfactoria, egresando al cuarto día. Al reevaluarla una semana después normalizó el valor de plaquetas y resolvió el derrame pleural. Es de recordar que los ensayos serológicos positivos son presuntivos, pudiendo existir reacción cruzada con infección previa por otros flavivirus (en este caso infección previa por dengue) y el diagnóstico deberá confirmarse o descartarse con pruebas de diagnóstico molecular

Síndrome de Alagille. Experiencia clínica y sobrevida de un caso

Paciente masculino de 7 años de edad, padres y hermana de 11 años sanos. A los 15 días de nacido inicia con vómitos, ictericia y acolia. Al examen mostró rasgos particulares: frente ancha, puente nasal deprimido, mentón puntiagudo cara triangular y ojos profundos. Escleras y piel con tinte ictérico-verdoso, xantomas faciales. Soplo cardíaco sistólico III/VI en foco pulmonar. Hígado 4 cm debajo del reborde costal derecho. Perfil bioquímico: colestasis, hiperamonemia e hiperlipidemia. Radiografía de columna: hemivertebras. Ultrasonido: sin dilatación de vías biliares intrahepáticas, vesícula biliar atrófica. Ecocardiograma: estenosis de la arteria pulmonar y persistencia del conducto arterioso. Biopsia hepática: ductopenia, fibrosis leve. Los criterios de colestasis, facies peculiar, cardiopatía congénita y hemivertebras con ductopenia sustentan el diagnóstico de Síndrome de Alagille. Su evolución 7 años después demuestra que cursa con enfermedad renal estadio II, acidosis tubular renal, hipertensión arterial, hiperparatiroidismo secundario, anemia microcítica-hipocrómica, detención del crecimiento y retraso psicomotriz; complicaciones compensadas con el tratamiento médico, que le permite llevar una calidad de vida satisfactoria. El síndrome es una alteración autosómica dominante con expresión variable y el pronóstico de sobrevida depende del grado de compromiso y malformación de órganos y sistemas; la literatura reporta que la esperanza de vida a 20 años es entre 75-80%. Es importante reconocer este síndrome (incidencia mundial 1/100,000 nacidos vivos) dentro del diagnóstico diferencial de colestasis infantil para identificarlo y determinar su frecuencia

Meningitis and pneumonia in Guatemalan children: the importance of Haemophilus influenzae type b and Streptococcus pneumoniae

OBJECTIVE: To determine the epidemiology of Haemophilus influenzae type b (Hib) and Streptococcus pneumoniae invasive infections in hospitalized Guatemalan children. This is an important issue since Hib vaccine has not been incorporated into the routine immunization program in Guatemala and information from hospital records in 1995 indicated a low incidence of Hib and S. pneumoniae as causes of meningitis and invasive infections. METHODS: Children who were hospitalized in Guatemala City with clinical signs compatible with bacterial infections were evaluated for evidence of Hib or S. pneumoniae infection. Normally sterile body fluids were cultured, and antigen detection was performed on cerebrospinal fluid (CSF) and pleural fluid. RESULTS: Of 1 203 children 1-59 months of age hospitalized over a 28-month period, 725 of them (60.3%) had a primary diagnosis of pneumonia, 357 (29.7%) of meningitis, 60 (5.0%) of cellulitis, and 61 (5.1%) of sepsis and other conditions. Hib was identified in 20.0% of children with meningitis and S. pneumoniae in 12.9%. The average annual incidence of Hib meningitis was 13.8 cases per 100 000 children under 5 years of age, and 32.4% of meningitides caused by Hib and 58.7% of S. pneumoniae meningitides occurred prior to 6 months of age. Case fatality rates were 14.1%, 37.0%, and 18.0%, respectively, for children with Hib, S. pneumoniae, and culture-negative and antigen-negative meningitis. Prior antibiotic therapy was common and was associated with significant reductions in CSF-culture-positive results for children with other evidence of Hib or S. pneumoniae meningitis. CONCLUSIONS: Improvements in case detection, culture methods, and latex agglutination for antigen detection in CSF resulted in identification of Hib and S. pneumoniae as important causes of severe disease in Guatemalan children. Using a cutoff of > 10 white blood cells per cubic millimeter in CSF would improve the sensitivity for detection of bacterial meningitis and help estimate the burden of bacterial meningitis in Guatemala and other developing countries

Global variation in postoperative mortality and complications after cancer surgery: a multicentre, prospective cohort study in 82 countries

© 2021 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY-NC-ND 4.0 licenseBackground: 80% of individuals with cancer will require a surgical procedure, yet little comparative data exist on early outcomes in low-income and middle-income countries (LMICs). We compared postoperative outcomes in breast, colorectal, and gastric cancer surgery in hospitals worldwide, focusing on the effect of disease stage and complications on postoperative mortality. Methods: This was a multicentre, international prospective cohort study of consecutive adult patients undergoing surgery for primary breast, colorectal, or gastric cancer requiring a skin incision done under general or neuraxial anaesthesia. The primary outcome was death or major complication within 30 days of surgery. Multilevel logistic regression determined relationships within three-level nested models of patients within hospitals and countries. Hospital-level infrastructure effects were explored with three-way mediation analyses. This study was registered with ClinicalTrials.gov, NCT03471494. Findings: Between April 1, 2018, and Jan 31, 2019, we enrolled 15 958 patients from 428 hospitals in 82 countries (high income 9106 patients, 31 countries; upper-middle income 2721 patients, 23 countries; or lower-middle income 4131 patients, 28 countries). Patients in LMICs presented with more advanced disease compared with patients in high-income countries. 30-day mortality was higher for gastric cancer in low-income or lower-middle-income countries (adjusted odds ratio 3·72, 95% CI 1·70–8·16) and for colorectal cancer in low-income or lower-middle-income countries (4·59, 2·39–8·80) and upper-middle-income countries (2·06, 1·11–3·83). No difference in 30-day mortality was seen in breast cancer. The proportion of patients who died after a major complication was greatest in low-income or lower-middle-income countries (6·15, 3·26–11·59) and upper-middle-income countries (3·89, 2·08–7·29). Postoperative death after complications was partly explained by patient factors (60%) and partly by hospital or country (40%). The absence of consistently available postoperative care facilities was associated with seven to 10 more deaths per 100 major complications in LMICs. Cancer stage alone explained little of the early variation in mortality or postoperative complications. Interpretation: Higher levels of mortality after cancer surgery in LMICs was not fully explained by later presentation of disease. The capacity to rescue patients from surgical complications is a tangible opportunity for meaningful intervention. Early death after cancer surgery might be reduced by policies focusing on strengthening perioperative care systems to detect and intervene in common complications. Funding: National Institute for Health Research Global Health Research Unit

Effects of hospital facilities on patient outcomes after cancer surgery: an international, prospective, observational study

© 2022 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 licenseBackground: Early death after cancer surgery is higher in low-income and middle-income countries (LMICs) compared with in high-income countries, yet the impact of facility characteristics on early postoperative outcomes is unknown. The aim of this study was to examine the association between hospital infrastructure, resource availability, and processes on early outcomes after cancer surgery worldwide. Methods: A multimethods analysis was performed as part of the GlobalSurg 3 study—a multicentre, international, prospective cohort study of patients who had surgery for breast, colorectal, or gastric cancer. The primary outcomes were 30-day mortality and 30-day major complication rates. Potentially beneficial hospital facilities were identified by variable selection to select those associated with 30-day mortality. Adjusted outcomes were determined using generalised estimating equations to account for patient characteristics and country-income group, with population stratification by hospital. Findings: Between April 1, 2018, and April 23, 2019, facility-level data were collected for 9685 patients across 238 hospitals in 66 countries (91 hospitals in 20 high-income countries; 57 hospitals in 19 upper-middle-income countries; and 90 hospitals in 27 low-income to lower-middle-income countries). The availability of five hospital facilities was inversely associated with mortality: ultrasound, CT scanner, critical care unit, opioid analgesia, and oncologist. After adjustment for case-mix and country income group, hospitals with three or fewer of these facilities (62 hospitals, 1294 patients) had higher mortality compared with those with four or five (adjusted odds ratio [OR] 3·85 [95% CI 2·58–5·75]; p<0·0001), with excess mortality predominantly explained by a limited capacity to rescue following the development of major complications (63·0% vs 82·7%; OR 0·35 [0·23–0·53]; p<0·0001). Across LMICs, improvements in hospital facilities would prevent one to three deaths for every 100 patients undergoing surgery for cancer. Interpretation: Hospitals with higher levels of infrastructure and resources have better outcomes after cancer surgery, independent of country income. Without urgent strengthening of hospital infrastructure and resources, the reductions in cancer-associated mortality associated with improved access will not be realised. Funding: National Institute for Health and Care Research