Cholelithiasis and its complications in sickle cell disease in a university hospital

Abstract

IntroductionThe clinical manifestations of sickle cell disease are related to the polymerization of hemoglobin S. The chronic hemolysis caused by this condition often causes the formation of gallstones that can migrate and block the common bile duct leading to acute abdomen.ObjectiveThis study aimed to evaluate the profile of patients with sickle cell disease and cholelithiasis.MethodsPatients with sickle cell disease were separated into groups according to the presence or absence of cholelithiasis. Socioepidemiological and clinical characteristics, such as gender, age, use of hydroxyurea and the presence of other hemoglobinopathies were researched in the medical records of patients.ResultsA hundred and seven patients with sickle cell anemia were treated at the institution. Of these, 27 (25.2%) had cholelithiasis. The presence of cholelithiasis was higher in the 11–29 age group than in younger than 11 years and over 29 years. No association was found for the presence of cholelithiasis with gender, use of hydroxyurea or type of hemoglobinopathy (hemoglobin SS, hemoglobin SC or sickle beta-thalassemia). Sixteen of the patients had to be submitted to cholecystectomy with 14 of the surgeries being performed by laparoscopy. Complications were observed in three patients and one patient died for reasons unrelated to the surgery.ConclusionA quarter of patients with sickle cell disease had gallstones, more commonly in the 11- to 29-year age range. Patients should be monitored from childhood to prevent cholelithiasis with preoperative, intra-operative and postoperative care being crucial to reduce the risk of complications in these patients.

Prevalence and Risk Factors for Microalbuminuria in Children with Sickle Cell Disease at King Abdulaziz University Hospital: A Retrospective Cross-sectional Study

“…Our findings were consistent with those of Alexander-Reindorf et al [26] and Bond et al [27] who reported significantly higher morbidity and more hospital admissions among SCD patients with gallbladder stones. Additionally, the mean age in our microalbuminuria group was 13.74 years, consistent with the findings of Martins et al [28] who reported patients of ages 11 and 29 years, with a higher prevalence of cholelithiasis and gallbladder stones respectively.…”

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

“…None of the patients presented here were on hydroxyurea or chronic transfusions prior to presentation; both may alter the onset and severity of liver damage in SCD . When primary prevention of sickle hepatopathy is not possible, early diagnosis and aggressive management may limit morbidity and mortality.…”

“…2 None of the patients presented here were on hydroxyurea or chronic transfusions prior to presentation; both may alter the onset and severity of liver damage in SCD. 20 When primary prevention of sickle hepatopathy is not possible, early diagnosis and aggressive management may limit morbidity and mortality. Studies are needed to clarify the pathophysiology of sickle hepatopathy and to define whether hepatic complications in childhood predict recurrent problems in adulthood.…”

Fisiopatogenia e métodos diagnósticos das anemias hemolíticas: uma revisão integrativa.

“…Essa mutação é causada pela substituição de uma adenina por uma timina no sexto códon do gene, acarretando na formação de drepanócitos rígidos que não conseguem atravessar a microcirculação dos tecidos e acabam sofrendo hemólise. Devido a isso, observa-se um aumento da viscosidade sanguínea, facilitando a formação de micro trombos, oclusão dos pequenos vasos e resultando em isquemia e micro infartos 44,41 . O formato de "foice" é um fator determinante do quadro hemolítico, em função do aumento da fragilidade mecânica, perda da elasticidade e plasticidade.…”

“…No diagnóstico inicial de detecção da hemoglobina S, utiliza-se uma análise eletroforética a fi m de identifi car preliminarmente a presença de amostras normais de patológicas. Assim, dependendo do resultado, busca-se técnicas laboratoriais complementares 43,44 . Dentre as técnicas de eletroforese, encontra-se a de gel de agarose, na qual a mais comum é a HYDRASYS ® acompanhada do teste de solubilidade 45 .…”