RESUMEN
BACKGROUND: Sickle cell disease (SCD) is a disorder that results in increased hospitalizations and higher mortality. Advances in management have resulted in increases in life expectancy and led to increasing awareness of sickle cell hepatopathy (SCH). However, its impact in patients on the natural history and outcomes of SCD is not known. Our study aims to describe the prevalence of extreme hyperbilirubinemia (EH), one form of SCH, its effect on morbidity and mortality, and correlations between sickle cell genotype and SCH type. We hypothesize that EH is associated with higher morbidity and mortality. AIM: To investigate the effects of EH on morbidity and mortality among patients with SCD. METHODS: This retrospective cohort study was performed using a database of patients with SCD treated at Grady Memorial Hospital between May 2004 and January 2017. Patients with EH (defined as total bilirubin above 13.0 mg/dL) were identified. A control group was identified from the same database with patients with total serum bilirubin ≤ 5.0 mg/dL. Electronic medical records were used to extract demographic information, laboratory values, radiology results, current medications, need for transfusions and mortality data. Two samples T-test, chi-squared test and Fisher's exact test were then used to compare the parameters between the two groups. RESULTS: Out of the database, fifty-seven charts were found of patients with bilirubin > 13 mg/dL. Prevalence of severe SCH as defined by EH was 4.8% (57/1172). There were no demographic differences between patients with and without EH. Significant genotypic differences existed between the two groups, with hemoglobin SS SCD being much higher in the EH group (P < 0.001). Patients with severe EH had a significant elevations in alanine aminotransferase (157.0 ± 266.2 IU/L vs 19.8 ± 21.3 IU/L, P < 0.001), aspartate aminotransferase (256.5 ± 485.9 U/L vs 28.2 ± 14.7 U/L, P < 0.001) and alkaline phosphatase (218.0 ± 176.2 IU/L vs 85.9 ± 68.4 IU/L, P < 0.001). Patients with EH had significantly higher degree of end organ failure measured with quick Sequential Organ Failure Assessment scores (0.42 ± 0.68 vs 0.01 ± 0.12, P < 0.001), increased need for blood products (63% vs 5%, P < 0.001), and exchange transfusions (10.5% vs 1.3%, P = 0.022). CONCLUSION: Among patients with SCD, elevated levels of total bilirubin are rare, but indicative of elevated morbidity, mortality, and need for blood transfusions. Large differences in sickle cell genotype also exist, but the significance of this is unknown.
RESUMEN
Sickle cell disease (SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as "sickle cell hepatopathy". This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis. In this review, we detail the pathophysiology, clinical presentation and biochemical features of various acute and chronic hepatobiliary manifestations of SCD and present and evaluate existing evidence with regards to management of this disease process. We also discuss recent advances and controversies such as the role of liver transplantation in sickle cell hepatopathy and highlight important questions in this field which would require further research. Our aim with this review is to help increase the understanding, aid in early diagnosis and improve management of this important disease process.
RESUMEN
El presente escrito recoge algunas ideas específicas con las cuales se propone guiar la articulación entre el lenguaje y la constitución psíquica; así mismo, destacar algunas otras que, consideramos, se convierten en hitos esenciales a la hora de disertar acerca de las manifestaciones del habla en la dimensión clínica. Tomamos para la elaboración la guía freudiana según la cual la asociación libre permite el acceso a lo inconsciente, vía por la cual el psicoanálisis se aleja de otro tipo de terapéuticas y posiciona al lenguaje del mismo modo que lo hacen autores como Octavio Paz como un asunto en íntima relación con la existencia. En esa perspectiva, retomamos ideas de Jacques-Alain Miller, quien, siguiendo a Lacan, otorga al lenguaje una simetría con la noción de estructura.
This paper presents some specific ideas with which it proposes to guide the articulation between language and psychic constitution. It also highlights some of the ideas that we consider to become essential milestones when discussing the manifestations of speech in the clinical dimension. We take the Freudian guide, according to which the free association allows access to the unconscious, and thus psychoanalysis gets away from other therapeutics and positions language in the same way that some authors like Octavio Paz do as a matter closely related to existence. In this perspective, we go over Jacques-Alain Miller's ideas. Following Lacan, he gives language a symmetry with the notion of structure.
Ce texte recueille quelques idées spécifiques qui seront la base sur laquelle s'orientera l'articulation entre le langage et la constitution psychique. De même, il a pour but de souligner quelques idées qui, selon nous, deviennent des événements marquants au moment de disserter sur les manifestations de la parole dans la dimension clinique. La thèse freudienne selon laquelle l'association libre permet l'accès à l'inconscient sera notre point de départ. Grâce à cette voie, la psychanalyse s'éloigne d'autres thérapeutiques et place le langage ainsi que des auteurs comme Octavio Paz le font comme un sujet lié intimement à l'existence. Sous cet angle, nous avons repris des idées de Jacques-Alain Miller qui, suivant Lacan, confère au langage une symétrie avec la notion de "structure".
