Department of Pediatric Surgery, Salmaniya Medical Complex, Manama, Kingdom of Bahrain.
Department of Postgraduate Surgical Studies, Royal College of Surgeons in Ireland, Dublin, Ireland.
Afr J Paediatr Surg. 2024 Jul 1;21(3):151-154. doi: 10.4103/ajps.ajps_133_22. Epub 2023 Apr 10.
Sickle cell disease (SCD) is a haemoglobinopathy that leads to the formation of distorted sickle-shaped red blood cells that are prone to vaso-occlusion. This may lead to vaso-occlusive crises that may affect any organ. Acute pancreatitis (AP) in SCD patients may be mimicked by a vaso-occlusive crisis in the abdomen. The objective of this article is to analyse the clinical profiles of SCD patients with AP and understand the differences in the presentation of AP compared to an abdominal vaso-occlusive crisis and the difference between its presentation in SCD patients in comparison to other patients.
Twenty-eight SCD patients who were diagnosed with AP during their admission to the paediatric department at a tertiary hospital between January 2012 and December 2020 were retrospectively studied. Patients aged older than 14 years were excluded. The data collected concerned: demographics, the clinical course and the hospital course. The diagnosis and severity protocols followed the revised Atlanta Criteria.
The patients were aged with a mean of 9.61 years. There were 15 males and 13 females. Demographics were not significantly correlated to complication rates (P > 0.05). The mean duration of hospitalisation was 6.43 days. The most common clinical presentations were abdominal pain, fever, then vomiting and nausea. Three patients experienced complications and they were all cases of cholangitis (10.71%). There were no cases of pseudocysts, acute necrotic collections, pancreatic or peripancreatic necrosis or walled-off necroses. All of the cases of AP in SCD children were mild according to the revised Atlanta classification. Leucocytosis was present in 29.29% of patients and 17.8% of patients had high C-reactive proteins (CRPs). There was no significant correlation between leucocyte counts, CRP levels, serum or urinary amylase levels and complications (P > 0.05). All patients had haemoglobin (Hb) levels above 7 g/dL. The levels of sickle Hb ranged from 40 to 70 g/dL and reticulocyte counts averaged at 3.57%. Haematologic parameters were not significantly correlated with complication rates (P > 0.05). There were no recurrences.
AP in SCD patients presented with classic signs and symptoms. There were no associations between demographics and complications. The levels of leucocytes, CRP counts and serum and urinary amylase were not correlated with complications. The level of Hb and sickle cell Hb was not associated with complication rates. Reticulocytes were slightly elevated in SCD patients with AP. More studies are needed to demarcate factors distinguishing AP in SCD from abdominal vaso-occlusive crises.
镰状细胞病(SCD)是一种血红蛋白病,导致形成易发生血管阻塞的扭曲镰状红细胞。这可能导致血管阻塞危象,可能影响任何器官。SCD 患者的急性胰腺炎(AP)可能被腹部的血管阻塞危象模拟。本文的目的是分析 SCD 患者中伴有 AP 的临床特征,并了解 AP 与腹部血管阻塞危象的表现差异,以及与其他患者的表现差异。
对 2012 年 1 月至 2020 年 12 月期间在一家三级医院儿科住院期间被诊断为 AP 的 28 名 SCD 患者进行回顾性研究。排除年龄大于 14 岁的患者。收集的数据包括:人口统计学、临床过程和住院过程。诊断和严重程度方案遵循修订后的亚特兰大标准。
患者年龄平均为 9.61 岁。其中 15 名男性,13 名女性。人口统计学与并发症发生率无显著相关性(P>0.05)。平均住院时间为 6.43 天。最常见的临床表现为腹痛、发热,然后是呕吐和恶心。3 例患者出现并发症,均为胆管炎(10.71%)。无假性囊肿、急性坏死性积聚、胰腺或胰周坏死或包裹性坏死。根据修订后的亚特兰大分类,SCD 儿童的所有 AP 病例均为轻度。白细胞增多见于 29.29%的患者,17.8%的患者 C 反应蛋白(CRP)升高。白细胞计数、CRP 水平、血清或尿淀粉酶水平与并发症之间无显著相关性(P>0.05)。所有患者的血红蛋白(Hb)水平均高于 7g/dL。镰状血红蛋白水平范围为 40 至 70g/dL,网织红细胞计数平均为 3.57%。血液学参数与并发症发生率无显著相关性(P>0.05)。无复发。
SCD 患者的 AP 表现出典型的症状和体征。人口统计学与并发症之间无关联。白细胞、CRP 计数和血清及尿淀粉酶水平与并发症无关。Hb 水平和镰状细胞 Hb 水平与并发症发生率无关。SCD 患者的网织红细胞略有升高。需要更多的研究来划定区分 SCD 中 AP 与腹部血管阻塞危象的因素。