Sickle-cell disease (SCD) is a worldwide distributed hemoglobinopathy, characterized by hemolytic anemia associated with vaso-occlusive events. These result in acute and chronic multiorgan damage. Bone is early involved, leading to long-term disability, chronic pain and fractures. Here, we carried out a retrospective study to evaluate sickle bone disease (SBD) in a cohort of adults with SCD. We assessed bone density, metabolism and turnover. We also evaluated the presence of fractures and the correlation between SCD severity and skeletal manifestations. A total of 71 patients with SCD were analyzed. The mean age of population was 39 ± 10 years, 56% of which were females. We found osteoporosis in a range between 7% and 18% with a high incidence of vertebral fractures. LDH and AST were predictive for the severity of vertebral fractures, while bone density was not. Noteworthy, we identified -1.4 Standard Deviations T-score as the cutoff for detecting the presence of fractures in patients with SCD. Collectively our data allowed us to develop an algorithm for the management of SBD, which may be useful in daily clinical practice to early intersect and treat SBD.

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A 35-year-old man with sickle cell disease (SCD, S/β 0 ) was admitted to our emergency department (ED) for right upper quadrant abdominal pain (RUQ) associated with fever (38 C) and jaundice (Charcot's triad; Total Bil. 3.19 mg/dL). Pain was constantly midepigastric, often radiated to the back. Laboratory tests showed increased CRP (93 mg/dL), ALT (71 U/L), and LDH (347 U/L); whereas, AST (32 U/ L), γGT (19 U/L), albumin (34.1 g/L), total protein (74.1 g/L), amylase, aPTT, PT were within normal range. CBC documented Hb 8.7 g/dL, circulating erythroblasts 510.000/mm 3 , WBC 11 800/mm 3 , PLTs 286 000/ mm 3 . The collection of patient history revealed: splenectomy at the age of 8 years and cholecistectomy at age of 19 years.He was taking hydroxyurea (20 mg/kg/d) therapy since the age of 17 years due to recurrent pain crisis, requiring hospitalization, and aspirin (100 mg/d) since splenectomy. Two previous episodes of superficial venous thrombosis were reported by the patient.RUQ pain occurs in almost 10% of SCD patients during acute vaso-occlusive crisis (VOC) and it is one of the first three causes of access to the ED admission. 1 RUQ pain is generally associated with increased AST/ALT ratio, which rapidly decreases at resolution of the VOC. Although RUQ pain is generally related to VOCs, a wide variety of conditions has been recognized to possibly sustain abdominal pain and jaundice in SCD (Table 1). 1-3 However, emergency physician rarely considers other causes of RUQ pain than VOC in SCD subjects (see also Table 1). This might expose SCD patients to the risk of undertreatment or misdiagnosis of RUQ pain and possibly mismanagement. Thus, it is mandatory to discriminate with the patient whether the abdominal pain episode has characteristics similar to previous once experienced by the patients in order to consider causes others than VOCs and to decide a more aggressive diagnostic approach. 4At the admission in the ED, patient VAS pain-score was 7. Multimodal analgesia with tramadol (7.2 mg/kg/d), ketorolac (0.86 mg/ kg/d) and metoclopramide associated with intravenous hydration (saline solution 20-30 mL/kg/d) and ceftriaxone (2 g/d) was started. 5,6 Abdominal ultrasound (US) was negative and portal vein velocity was normal at the admission to the ED and at 48 hours after hospitalization.Abdominal-US is generally used to exclude biliary obstruction and/or dilation (intrahepatic, extrahepatic, or both) or to document primary liver disorders, such as infections/abscesses or portal vein disease as well as pancreatic diseases such as acute or chronic pancreatitis or gallstone-related pancreatic disease. However, US does not allow the identification of choledocholithiasis, which requires MR cholangiopancreatography (MRCP). Multidetector Computed Tomography (MDCT) is, overall, the gold standard imaging approach for undifferentiated abdominal pain, especially in presence of fever and nonlocalized symptoms. MDCT can also better delineate complicated biliary disorders, such as gas-forming processes, abscesses, hemorrhage, ...