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1.
Teach Learn Med ; : 1-15, 2023 Dec 11.
Artículo en Inglés | MEDLINE | ID: mdl-38083811

RESUMEN

Phenomenon: Effective communication between team members is essential during the resuscitation of critically-ill patients. Failure of junior doctors to speak up and challenge erroneous clinical decisions made by their senior doctors is a serious communication failure which can result in catastrophic outcomes and jeopardize patient safety. Crisis resource management (CRM) and conflict resolution tools have been increasingly employed in the healthcare setting to reduce communication failure among healthcare providers and improve patient safety during crisis situations. The aims of our study were to: 1) evaluate the factors affecting junior doctors' ability to speak up on medical errors, 2) examine the effectiveness of CRM and conflict resolution tools, and 3) formulate a communication framework directed at training junior doctors in appropriate intellectual questioning of authority. Approach: From January to April 2019, we recruited twenty-five second-year postgraduate junior doctors working in an Emergency Department in Singapore. We provided training in CRM and conflict resolution communication for participants in the intervention arm. Participants underwent a high-fidelity simulated resuscitation scenario which was standardized to include faculty misdirection in the form of erroneous instructions given by a role-played senior doctor. We observed if participants appropriately challenged the erroneous instructions. We subsequently interviewed participants on their response during the simulation to elicit their barriers and motivations toward challenging authority. Video recordings were analyzed by an independent panel of investigators. Findings: Participants employed various non-verbal and verbal approaches when challenging erroneous decisions. We uncovered multiple personal, interpersonal, and situation-based factors influencing the junior doctor's willingness to challenge erroneous decisions made by seniors. From their responses, we conceptualized a theoretical model designed as a "weighing scale" to demonstrate how junior doctor's eventual response is the outcome of a delicate interplay of multiple barriers and motivations. Our intervention did not significantly increase the participants' likelihood of challenging authority (69% in control arm vs 75% in intervention arm, p = 1.00). Insights: Our study provides insights into the mindset of junior doctors when faced with the dilemma of challenging authority on medical errors. Established CRM training may not be effective in addressing the challenges junior doctors face when communicating against the hierarchal gradient. We propose strategies to further develop and optimize CRM training to enhance its value for junior doctors. Drawing from our findings, we formulated a "SAFE" communication tool (State the safety concern, suggest Alternative course of action, Support with Facts, Engage via Enquiry) directed at helping junior doctors in appropriate intellectual questioning of authority.

3.
Cochrane Database Syst Rev ; 11: CD013634, 2022 11 30.
Artículo en Inglés | MEDLINE | ID: mdl-36448638

RESUMEN

BACKGROUND: Chronic arthropathy is a potentially debilitating complication for people with haemophilia - a genetic, X-linked, recessive bleeding disorder, characterised by the absence or deficiency of a clotting factor protein. Staging classifications, such as the Arnold-Hilgartner classification for haemophilic arthropathy of the knee, radiologically reflect the extent of knee joint destruction with underlying chronic synovitis. Management of this highly morbid disease process involves intensive prophylactic measures, and chemical or radioisotope synovectomy in its early stages. However, failure of non-surgical therapy in people with progression of chronic arthropathy often prompts surgical management, including synovectomy, joint debridement, arthrodesis, and arthroplasty, depending on the type of joint and extent of the damage. To date, management of people with mild to moderate chronic arthropathy from haemophilia remains controversial; there is no agreed standard treatment. Thus, the benefits and disadvantages of non-surgical and surgical management of mild to moderate chronic arthropathy in people with haemophilia needs to be systematically reviewed.  OBJECTIVES: To assess the efficacy and safety of surgery for mild to moderate chronic arthropathy in people with haemophilia A or B. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register, CENTRAL, MEDLINE, Embase, CINAHL, and two trial registers to August 2022. We also handsearched relevant journals and conference abstract books. SELECTION CRITERIA: Randomized controlled trials (RCTs) and quasi-RCTs comparing surgery and non-surgical interventions, for any joint with chronic arthropathy, in people with haemophilia, who were at least 12 years old. DATA COLLECTION AND ANALYSIS: The review authors did not identify any trials to include in this review. MAIN RESULTS: The review authors did not identify any trials to include in this review. AUTHORS' CONCLUSIONS: The review authors did not identify any trials to include in this review. Due to a lack of research in this particular area, we plan to update the literature search every two years, and will update review if any new evidence is reported. There is a need for a well-designed RCT that assesses the safety and efficacy of surgical versus non-surgical interventions for chronic arthropathy in people with haemophilia.


Asunto(s)
Hemofilia A , Artropatías , Niño , Humanos , Hemofilia A/complicaciones , Artropatías/etiología , Artropatías/cirugía , Articulación de la Rodilla , MEDLINE , Ensayos Clínicos Controlados Aleatorios como Asunto
4.
Blood Cells Mol Dis ; 88: 102547, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33607590

RESUMEN

INTRODUCTION: Transfusion-dependent thalassaemia is associated with complications related to iron overload from frequent red cell transfusions which affect quality of life. We collected data on the clinical outcomes, complications, socioeconomic status and health-related quality of life (HRQoL) of transfusion-dependent thalassaemia patients in Singapore, and analysed the associations between clinical and socioeconomic factors with development of transfusion-related complications and HRQoL scores. MATERIALS AND METHODS: This was a cross-sectional study of transfusion-dependent thalassaemia patients treated at four major public hospitals in Singapore. Clinical information was obtained from retrospective reviews of medical records. Socioeconomic data and patient-reported compliance to iron chelators were obtained from prospective interviews of patients or caregivers using a questionnaire. A validated, disease-specific HRQoL instrument, the TranQOL, was administered to patients and caregivers during a routine clinic or transfusion visit. RESULTS: Liver iron loading was the most common transfusion-related complication and occurred in 79% of patients. Cardiac iron loading was noted in 28.3% and endocrine complications were present in 34.2%. Liver iron loading was significantly associated with higher mean ferritin level. Cardiac iron loading was significantly associated with increasing age, higher mean ferritin level and type of iron chelator. Endocrine complications were associated with increasing age, higher mean ferritin level, type of iron chelator and poorer patient-reported compliance to iron chelators. The lowest TranQOL scores were reported by caregiver parents of patients aged less than 18 years. Lower TranQOL scores were significantly associated with increasing age, especially in the 31-50 age cohort, and with reception of social assistance. CONCLUSION: The main morbidities noted in transfusion-dependent thalassaemia patients in Singapore are from complications associated with iron loading. The cohort of older thalassaemia patients aged 31-50 experienced significantly higher rates of cardiac iron loading, endocrine complications and lower TranQOL scores compared to younger age cohorts.


Asunto(s)
Transfusión Sanguínea , Calidad de Vida , Talasemia/terapia , Adolescente , Adulto , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Lactante , Quelantes del Hierro/uso terapéutico , Masculino , Persona de Mediana Edad , Singapur/epidemiología , Factores Socioeconómicos , Talasemia/complicaciones , Talasemia/epidemiología , Reacción a la Transfusión , Adulto Joven
5.
Artículo en Inglés | MEDLINE | ID: mdl-32647033

RESUMEN

OBJECTIVES: Death is a significant event that affects healthcare providers emotionally. We aimed to determine internal medicine (IM) and paediatric (PD) residents' responses and the impact on the residents following patient deaths, and to compare any differences between IM and PD residents. We also aimed to determine whether sufficient resources and measures were in place to support residents through their grief process. METHODS: This is a single-centre, cross-sectional study involving residents from IM and PD programmes from an academic tertiary hospital in Singapore. The residents completed a questionnaire regarding their responses and emotions after experiencing patient deaths. RESULTS: A total of 122 residents (85 IM and 37 PD, equally distributed between year 1 to year 4 of residency training) participated, with 100% response rate. Only half (57%) felt they would be comfortable treating a dying patient and 66.4% reported feeling sad following their patient's death. Most (79.5%) were not aware of support resources that were available and 82% agreed that formal bereavement training should be included in the residency curriculum. PD residents had more negative symptoms than IM residents, with poor concentration (PD 35.1% vs IM 16.5%, p=0.02) and lethargy (PD 35.1% vs IM 9.4%, p<0.01) being the most common. CONCLUSION: In our Asian context, residents are negatively affected by patient deaths, especially the PD residents. There is a need to incorporate relevant bereavement training for all residents.

6.
Pediatr Transplant ; 24(6): e13740, 2020 09.
Artículo en Inglés | MEDLINE | ID: mdl-32447823

RESUMEN

Pediatric organ donation represents only a low proportion of overall organ donation in many parts of world, unable to match the needs for pediatric organ transplantation. Pediatric organ donation after circulatory determination of death (DCD) is increasingly explored in pediatric transplantation, as it increases the availability of organ grafts. A 6-year-old Caucasian boy with a history of arteriovenous malformation presented with a catastrophic intracranial bleed, resulting in severe brainstem dysfunction despite maximal medical and surgical measures. He did not fulfill the criteria for brain death, which must be met for pediatric organ donation in Singapore. Due to parental request, his organs were donated after withdrawal of life support and determination of death by circulatory criteria. Pediatric organ DCD poses many challenges in the pediatric population, especially in the absence of a local practice guideline. We present the first case of a pediatric organ DCD that has occurred in Singapore. Further work is needed, particularly in establishing a national policy for pediatric organ DCD and increasing overall awareness and acceptance toward pediatric organ donations.


Asunto(s)
Donantes de Tejidos , Recolección de Tejidos y Órganos/métodos , Obtención de Tejidos y Órganos/legislación & jurisprudencia , Obtención de Tejidos y Órganos/métodos , Malformaciones Arteriovenosas/complicaciones , Malformaciones Arteriovenosas/cirugía , Muerte Encefálica , Niño , Muerte , Humanos , Hemorragias Intracraneales/mortalidad , Masculino , Trasplante de Órganos/métodos , Pediatría/legislación & jurisprudencia , Pediatría/métodos , Guías de Práctica Clínica como Asunto , Singapur
8.
J Pediatr Hematol Oncol ; 41(1): e51-e53, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-29668551

RESUMEN

We report a rare case of severe congenital dyserythropoietic anemia type 1 with fetal onset. Our patient presented with fetal hydrops from 19 weeks of gestation, requiring multiple intrauterine transfusions. At birth, she had severe hemolytic anemia with severe jaundice, and was subsequently transfusion dependent. She eventually developed severe iron overload and fulminant liver failure before her demise at 5 months of age. Genetic testing revealed a novel mutation in CDAN1.


Asunto(s)
Anemia Diseritropoyética Congénita , Colestasis Intrahepática , Glicoproteínas/genética , Hidropesía Fetal , Sobrecarga de Hierro , Mutación , Índice de Severidad de la Enfermedad , Anemia Diseritropoyética Congénita/genética , Anemia Diseritropoyética Congénita/patología , Colestasis Intrahepática/genética , Colestasis Intrahepática/patología , Femenino , Humanos , Hidropesía Fetal/genética , Hidropesía Fetal/patología , Lactante , Sobrecarga de Hierro/genética , Sobrecarga de Hierro/patología , Proteínas Nucleares
9.
Ann Acad Med Singap ; 46(2): 44-49, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-28263341

RESUMEN

INTRODUCTION: This study aimed to investigate the risk factors associated with mortality in haematopoietic stem cell transplant (HSCT) patients admitted to our paediatric intensive care unit (PICU) over an 8-year period. MATERIALS AND METHODS: A retrospective chart review was conducted of all HSCT patients requiring PICU admission at our centre (a tertiary care university hospital in Singapore) from January 2002 to December 2010. Chief outcome measures were survival at the time of PICU discharge and survival at 6 months after initial PICU admission. RESULTS: Ninety-eight patients underwent HSCT during this period; 18 patients (18%) required 24 PICU admissions post-HSCT. The overall survival to PICU discharge was 62.5%. Of those who survived discharge from the PICU, 33% died within 6 months of discharge. Non-survivors to PICU discharge had a higher incidence of sepsis (89% vs 33%, P = 0.013) and organ failure as compared to survivors (cardiovascular failure 100% vs 20%, P = 0.0003; respiratory failure 89% vs 20%, P = 0.002; and renal failure 44% vs 7%, P = 0.047). Mortality rates were higher in patients requiring mechanical ventilation (70% vs 14%, P = 0.010) and inotropic support (70% vs 14%, P = 0.010). Mortality in all patients with renal failure requiring haemodialysis (n = 4) was 100%. Presence of 3 or more organ failures was associated with 80% mortality (P = 0.003). CONCLUSION: Sepsis, multiple organ failure and the need for mechanical ventilation, inotropes and especially haemodialysis were associated with increased risk of mortality in our cohort of HSCT patients.


Asunto(s)
Cardiotónicos/uso terapéutico , Trasplante de Células Madre Hematopoyéticas , Unidades de Cuidado Intensivo Pediátrico , Insuficiencia Multiorgánica/mortalidad , Sepsis/mortalidad , Adolescente , Niño , Preescolar , Femenino , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/epidemiología , Insuficiencia Cardíaca/mortalidad , Mortalidad Hospitalaria , Humanos , Lactante , Masculino , Insuficiencia Multiorgánica/epidemiología , Pronóstico , Diálisis Renal/estadística & datos numéricos , Insuficiencia Renal/epidemiología , Insuficiencia Renal/mortalidad , Insuficiencia Renal/terapia , Respiración Artificial/estadística & datos numéricos , Insuficiencia Respiratoria/epidemiología , Insuficiencia Respiratoria/mortalidad , Insuficiencia Respiratoria/terapia , Estudios Retrospectivos , Factores de Riesgo , Sepsis/epidemiología , Singapur/epidemiología
10.
J Crit Care ; 38: 57-61, 2017 04.
Artículo en Inglés | MEDLINE | ID: mdl-27863269

RESUMEN

OBJECTIVES: We investigated whether diagnostic categories and presence of infections were associated with increased mortality or length of stay (LOS) in patients admitted to a pediatric intensive care unit (PICU). METHODS: A retrospective study of all PICU admissions between October 2002 and April 2016 was performed. Oncologic vs nononcologic, trauma/injuries vs nontraumatic, infectious (gram-positive, gram-negative, fungal bloodstream infections, common respiratory viruses) vs noninfectious diagnoses were evaluated for survival and LOS. RESULTS: Pediatric intensive care unit admissions (n = 2211) were associated with a mortality of 5.3%. Backward binary logistic regression showed that nonsurvival was associated with leukemia (odds ratio [OR], 4.81; 95% confidence interval [CI], 2.2-10.10; P < .0005), lymphoma (OR, 21.34; 95% CI, 3.89-117.16; P < .0005), carditis/myocarditis (OR, 7.91; 95% CI, 1.98-31.54; P = .003), encephalitis (OR, 6.93; 95% CI, 3.27-14.67; P < .0005), bloodstream infections with gram-positive organisms (OR, 5.32; 95% CI, 2.67-10.60; P < .0005), gram-negative organisms (OR, 8.23; 95% CI, 4.10-16.53; P < .0005), fungi (OR, 3.93; 95% CI, 1.07-14.42; P = .039), and pneumococcal disease (OR, 3.26; 95% CI, 1.21-8.75; P = .019). Stepwise linear regression revealed that LOS of survivors was associated with bloodstream gram-positive infection (B = 98.2; 95% CI, 75.7-120.7; P < .0005). CONCLUSIONS: Patients with diagnoses of leukemia, lymphoma, cardiomyopathy/myocarditits, encephalitis, and comorbidity of bloodstream infections and pneumococcal disease were significantly at risk of PICU mortality. Length of stay of survivors was associated with bloodstream gram-positive infection. The highest odds for death were among patients with leukemia/lymphoma and bloodstream coinfection. As early diagnosis of these childhood malignancies is desirable but not always possible, adequate and early antimicrobial coverage for gram-positive and gram-negative bacteria might be the only feasible option to reduce PICU mortality in these patients. In Hong Kong, a subtropical Asian city, none of the common respiratory viruses were associated with increased mortality or LOS in PICU.


Asunto(s)
Tiempo de Internación , Infecciones del Sistema Respiratorio/epidemiología , Sepsis/epidemiología , Niño , Servicios de Salud del Niño , Preescolar , Cuidados Críticos , Femenino , Hong Kong/epidemiología , Hospitalización , Humanos , Lactante , Unidades de Cuidado Intensivo Pediátrico , Modelos Logísticos , Masculino , Oportunidad Relativa , Infecciones del Sistema Respiratorio/complicaciones , Infecciones del Sistema Respiratorio/microbiología , Infecciones del Sistema Respiratorio/mortalidad , Estudios Retrospectivos , Factores de Riesgo , Sepsis/complicaciones , Sepsis/microbiología , Sepsis/mortalidad , Análisis de Supervivencia
11.
Asia Pac J Clin Nutr ; 25(4): 676-696, 2016 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-27702711

RESUMEN

BACKGROUND AND OBJECTIVES: Current practices and available resources for nutrition therapy in paediatric intensive care units (PICUs) in the Asia Pacific-Middle East region are expected to differ from western countries. Existing guidelines for nutrition management in critically ill children may not be directly applicable in this region. This paper outlines consensus statements developed by the Asia Pacific-Middle East Consensus Working Group on Nutrition Therapy in the Paediatric Critical Care Environment. Challenges and recommendations unique to the region are described. METHODS AND STUDY DESIGN: Following a systematic literature search from 2004-2014, consensus statements were developed for key areas of nutrient delivery in the PICU. This review focused on evidence applicable to the Asia Pacific-Middle East region. Quality of evidence and strength of recommendations were rated according to the Grading of Recommendation Assessment, Development and Evaluation approach. RESULTS: Enteral nutrition (EN) is the preferred mode of nutritional support. Feeding algorithms that optimize EN should be encouraged and must include: assessment and monitoring of nutritional status, selection of feeding route, time to initiate and advance EN, management strategies for EN intolerance and indications for using parenteral nutrition (PN). Despite heterogeneity in nutritional status of patients, availability of resources and diversity of cultures, PICUs in the region should consider involvement of dieticians and/or nutritional support teams. CONCLUSIONS: Robust evidence for several aspects of optimal nutrition therapy in PICUs is lacking. Nutritional assessment must be implemented to document prevalence and impact of malnutrition. Nutritional support must be given greater priority in PICUs, with particular emphasis in optimizing EN delivery.


Asunto(s)
Cuidados Críticos/métodos , Terapia Nutricional/métodos , Pediatría , Algoritmos , Australia , Preescolar , Consenso , Enfermedad Crítica/terapia , Proteínas en la Dieta/administración & dosificación , Nutrición Enteral , Humanos , India , Indonesia , Lactante , Recién Nacido , Unidades de Cuidado Intensivo Pediátrico , Malasia , Evaluación Nutricional , Estado Nutricional , Apoyo Nutricional , Nutricionistas , Arabia Saudita , Singapur , Tailandia , Emiratos Árabes Unidos
12.
Simul Healthc ; 10(4): 245-8, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-26098493

RESUMEN

INTRODUCTION: Bone marrow aspirate and trephine (BMAT) biopsy is a commonly performed procedure in hematology-oncology practice. Although complications are uncommon, they can cause significant morbidity and mortality. Simulation models are an excellent tool to teach novice doctors basic procedural skills before performing the actual procedure on patients to improve patient safety and well-being. METHODS: There are no commercial BMAT simulators, and this technical report describes the rationale, technical specifications, and construction of a low-cost, easily constructed, reusable BMAT simulator that reproduced the tactile properties of tissue layers for use as a teaching tool in our resident BMAT simulation course. Preliminary data of learner responses to the simulator were also collected. RESULTS: From April 2013 to November 2013, 32 internal medicine residents underwent the BMAT simulation course. Eighteen (56%) completed the online survey, 11 residents with previous experience doing BMAT and 7 without experience. Despite the difference in operative experience, both experienced and novice residents all agreed or strongly agreed that the model aided their understanding of the BMAT procedure. All agreed or strongly agreed that this enhanced their knowledge of anatomy and 16 residents (89%) agreed or strongly agreed that this model was a realistic simulator. CONCLUSIONS: We present a novel, low-cost, easily constructed, realistic BMAT simulator for training novice doctors to perform BMAT.


Asunto(s)
Médula Ósea/patología , Medicina Interna/educación , Internado y Residencia/métodos , Modelos Biológicos , Entrenamiento Simulado/métodos , Biopsia con Aguja , Humanos , Maniquíes
13.
Pediatr Nephrol ; 28(5): 819-22, 2013 May.
Artículo en Inglés | MEDLINE | ID: mdl-23306707

RESUMEN

BACKGROUND: Initiating continuous renal replacement therapy (CRRT) in infants exposes them to the dual hemodynamic challenges of high circuit extracorporeal volumes and potential membrane reactions, in the case of acrylonitrile AN69 membranes. The use of the new Prismaflex HF20 membrane in hemodynamically unstable low-body-weight infants on inotropic support has not been reported. TREATMENT: We describe the use of the HF20 (Gambro Lundia AB, Lund, Sweden) membrane in four low-body-weight infants (2.3 to 5.4 kg) with multi-organ dysfunction syndrome who were critically ill in the Pediatric Intensive Care Unit (PICU), hemodynamically unstable, and on inotropes. We were able to achieve target volume loss in all infants without compromising their hemodynamic status. Mean arterial pressures were maintained between 39 and 57 mmHg. The relatively low circuit volume of the HF20 set (60 ml) obviated the need for blood prime in the majority; however, when blood prime was required, there was no adverse reaction with the polyarylethersulfone (PAES) membrane. Solute clearance in these small infants was efficient with correction of metabolic acidosis and electrolyte abnormalities. Excellent circuit lifespan (56.3 ± 32.3 h) was observed. CONCLUSIONS: CRRT using the HF20 membrane is safe and hemodynamically well tolerated in high-risk, unstable low-body-weight infants with cardiac dysfunction on multiple inotropes.


Asunto(s)
Lesión Renal Aguda/terapia , Peso Corporal , Cardiotónicos/uso terapéutico , Hemodiafiltración/instrumentación , Hemodinámica/efectos de los fármacos , Membranas Artificiales , Insuficiencia Multiorgánica/terapia , Polímeros , Sulfonas , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/fisiopatología , Presión Arterial/efectos de los fármacos , Cardiotónicos/efectos adversos , Enfermedad Crítica , Diseño de Equipo , Oxigenación por Membrana Extracorpórea , Hemodiafiltración/efectos adversos , Humanos , Lactante , Unidades de Cuidado Intensivo Pediátrico , Insuficiencia Multiorgánica/diagnóstico , Insuficiencia Multiorgánica/fisiopatología , Resultado del Tratamiento
14.
Transfus Med Hemother ; 39(2): 139-150, 2012 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-22670132

RESUMEN

BACKGROUND: Uncontrolled bleeding continues to be a major cause of mortality in trauma, cardiac surgery, postpartum hemorrhage and liver failure. The aim of this paper is to assess the evidence supporting the efficacy of activated recombinant factor VII (rFVIIa) administration in these settings. METHODS: Electronic literature search. RESULTS: Numerous retrospective trials have mostly shown a decrease in blood transfusion requirements with no increase in thromboembolic events (TEE), but major limitations in trial design make generalization difficult. In most retrospective reports rFVIIa has been administered as a last-ditch attempt to control bleeding, when acidosis, hypothermia and coagulation factor depletion may not allow optimal rFVIIa effect. Prospective randomized controlled trials have not shown any effect of rFVIIa on mortality or TEE, although some have shown a reduction in RBC requirement. CONCLUSION: Stipulated transfusion protocols in prospective trials have reduced anticipated mortality among controls and make future trials for mortality effect unlikely in view of large sample size requirements. Establishment of these protocols and rapid hemostasis are likely to have greater benefits than administration of a single agent.

15.
J Neurol Sci ; 209(1-2): 101-4, 2003 May 15.
Artículo en Inglés | MEDLINE | ID: mdl-12686410

RESUMEN

A 25-year-old Chinese man presented with a 2-year history of recurrent coma. His plasma ammonia level was extremely elevated, with raised citrulline level and absence of argininosuccinic acid. Adult-onset citrullinaemia, a condition rarely reported outside the Japanese population, was diagnosed. Serial magnetic resonance (MR) imaging, including diffusion-weighted (DW) studies, showed initial involvement of the insula cortex and cingulate gyrus, changing to a pattern of multiple small lesions in the depths of the cortical sulci. This changing pattern of lesions over time on DW MR imaging has not previously been described in adult-onset citrullinaemia.


Asunto(s)
Citrulinemia/diagnóstico , Adulto , Amoníaco/sangre , Ácido Argininosuccínico/sangre , Encéfalo/patología , Citrulina/sangre , Citrulinemia/sangre , Citrulinemia/complicaciones , Citrulinemia/dietoterapia , Coma/etiología , Diagnóstico Diferencial , Progresión de la Enfermedad , Hígado Graso/diagnóstico por imagen , Hígado Graso/etiología , Humanos , Imagen por Resonancia Magnética , Masculino , Recurrencia , Remisión Espontánea , Trastornos Relacionados con Sustancias/diagnóstico , Ultrasonografía
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