Chacko Saran, Jadhav Ulhas, Ghewade Babaji, Wagh Pankaj, Prasad Roshan, Wanjari Mayur B
Respiratory Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Community Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Cureus. 2023 Apr 4;15(4):e37113. doi: 10.7759/cureus.37113. eCollection 2023 Apr.
Sickle cell disease (SCD) consists of a variety of hereditary hemoglobinopathies linked to alterations in the beta component of the hemoglobin (Hb) molecule. Acute SCD manifestations include stroke, acute chest syndrome (ACS), and pain, whereas chronic manifestations include avascular necrosis, chronic renal disease, and gallstones. This case report describes a rare instance of SCD-related pulmonary arterial hypertension (PAH) and cholelithiasis (CL). Following investigations, such as high-resolution CT scan thorax, chest X-ray, two-dimensional echocardiography, and ultrasonography of the abdomen and pelvis, PAH and CL were confirmed. The medical intervention mainly involved oxygenation, IV fluids, IV antibiotics, simple packed red blood cell transfusion (SBCT), folic acid, calcium supplementation, hydroxyurea, chest physiotherapy, and respiratory muscle strengthening exercises. The surgical intervention for CL was planned. Hence, the learning point from this case is that early multidisciplinary approach should be taken in order to control the progression of SCD.
镰状细胞病(SCD)由多种与血红蛋白(Hb)分子β成分改变相关的遗传性血红蛋白病组成。急性SCD表现包括中风、急性胸部综合征(ACS)和疼痛,而慢性表现包括无血管性坏死、慢性肾病和胆结石。本病例报告描述了1例罕见的SCD相关肺动脉高压(PAH)和胆石症(CL)。经过胸部高分辨率CT扫描、胸部X线、二维超声心动图以及腹部和骨盆超声等检查,确诊为PAH和CL。医疗干预主要包括给氧、静脉输液、静脉使用抗生素、单纯浓缩红细胞输血(SBCT)、叶酸、补钙、羟基脲、胸部物理治疗以及呼吸肌强化锻炼。计划对CL进行手术干预。因此,该病例的经验教训是,应尽早采取多学科方法以控制SCD的进展。