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Section 1
Published in Padmanabhan Ramnarayan, MCQs in Paediatrics for the MRCPCH, Part 1, 2017
1.8 Usual indications for an exchange transfusion in a child with sickle cell disease include:Painful vaso-occlusive crisisSplenic sequestration crisisAcute chest syndromeDactylitisPriapism
Gastrointestinal and hepatobiliary
Published in Dave Maudgil, Anthony Watkinson, The Essential Guide to the New FRCR Part 2A and Radiology Boards, 2017
Dave Maudgil, Anthony Watkinson
Splenic angiosarcoma is a very rare tumour with a poor prognosis. Iron deposits in spleen or liver cause low T2 signal. Splenic sequestration crisis is an early manifestation of sickle cell disease which presents occasionally. Post-mortems demonstrate a massively enlarged spleen with an accumulation of sickled erythrocytes trapped within the splenic sinusoids.
Case Report of Acute Splenic Sequestration Crisis in an Adult Patient with Hb S Disease and Suspected Hereditary Persistence of Fetal Hemoglobin
Published in Hemoglobin, 2021
Ian R. Sigal, Christine A. Ciunci
Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication of sickle cell disease that is characterized by acute splenic enlargement and a drop in hemoglobin (Hb) level of >2.0 g/dL as the spleen becomes engorged with blood. Acute splenic sequestration crisis is predominantly a disease of childhood and rarely occurs beyond 6 years of age due to autoinfarction of the spleen in patients with homozygous Hb S (HBB: c.20A>T) disease [1,2]. However, patients with Hb S/Hb C (HBB: c.19G>A), Hb S/β-thalassemia (Hb S/β-thal), and with hereditary persistence of fetal Hb (HPFH), can avoid splenic autoinfarction into adulthood and consequently are at-risk of ASSC. Due to its rarity in adult populations, ASSC may be misdiagnosed, putting patients at-risk of morbidity or mortality. Historically, ACSS has a mortality rate of up to 15.0% in the pediatric population [3], and several case reports document adult mortality secondary to ASSC [1,4], thus, prompt recognition is essential for successful treatment.
Distribution of sickle cell disease and assessment of risk factors based on transcranial Doppler values in the Gulf region
Published in Hematology, 2020
Yasser Wali, Vishwanatha Kini, Mohamed A. Yassin
The most common type of the first presentation (related to the history of SCD) was VOC (237 [57.8%]), followed by anemia (98 [23.9%]). VOC with anemia was reported in an additional 10 patients (2.4%), while 12 patients (2.9%) were asymptomatic. Of the 410 patients with SCD, 59 (14.4%) did not report any VOC, and 346 (84.4%) reported having a VOC at least once in the past 3 years (Figure 2A). Other clinical manifestations of SCD were reported in 222 patients (54.1%) within the past 3 years. The most common clinical manifestations (> 25%) included splenic sequestration crisis (40.5%), acute chest syndrome (33.3%), and hemolytic crisis (25.2%). Aplastic crisis was reported in 3.6% (8 of 222) of patients, stroke was reported in 1.8% (4 of 222), and silent stroke in another 1.8% (4 of 222) of patients.
The clinical characteristics of Congolese children and adolescents suffering from sickle-cell anemia are marked by the high frequencies of epistaxis compared to Western series
Published in Pediatric Hematology and Oncology, 2019
Aimé L. Kazadi, René M. Ngiyulu, Jean L. Gini-Ehungu, Jean M. Mbuyi-Muamba, Michel N. Aloni
Acute splenic sequestration crisis (ASSC) is an unpredictable life-threatening complication of SCA in sickle cell pediatric population. This complication was noted with a frequency of 7% in our series, and was found to be negligible in our cohort. Similar prevalence was previously reported in Senegal, India, Saudi Arabia and Kuwait.19-21 However, this prevalence is slightly low in comparison with others worldwide series where the prevalence varies from 20% to 35%.22-28 Hepatic sequestration was not found in our series. Similar findings were reported in other series.8,13