A Cross-Sectional Study on the Quality of Life of Adults With Sickle Cell Disease Followed-Up in Outpatient Clinics: A Single-Center Experience.

Background Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin S, leading to red blood cell deformities, chronic hemolysis, and frequent vaso-occlusive crises (VOC). While advancements in medical care have improved survival rates, adults with SCD continue to face substantial challenges in their quality of life (QoL) due to chronic pain, recurrent VOCs, and various complications. This study aimed to evaluate the health-related quality of life (HRQoL) in adult patients aged 14 years and above with SCD and identify key factors influencing patient outcomes using the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me).

Early Detection of Renal Complication in Children With Sickle Cell Disease: A Single Center Prospective Study.

Introduction: This observational cross-sectional study aimed to identify predictors of renal complications in pediatric patients with sickle cell disease (SCD) at King Salman Armed Forces Hospital, Tabuk, Saudi Arabia, over six months from February 2023 to July 2023. The study evaluated microalbuminuria as an early indicator of renal injury and explored its correlations with clinical and laboratory parameters and abdominal ultrasound (US) findings.

Methods: Included were pediatric patients aged 1 to 14 years with confirmed SCD, excluding those with acute infections or pre-existing renal diseases.

Priapism as an Unusual Symptom of T-cell Acute Lymphoblastic Leukemia in a Pediatric Case.

Acute lymphoblastic leukemia (ALL) in pediatric patients typically presents with recognizable symptoms such as fever, pallor, and bone pain. However, atypical manifestations can complicate the diagnostic landscape. We present a unique case of a seven-year-old male with T-cell ALL whose presenting symptom was priapism.

Reduction of hospital bed cost for inpatient overstay through optimisation of patient flow.

Background: Overstay of inpatients is a big challenge to healthcare systems which interferes with the proper utilisation of the available resources and efficient delivery of care. Unnecessary days in the hospital may lead to patient complications including healthcare-associated infections, falls and delirium, which can negatively impact both patient and staff experience. This project aimed to reduce the cost of bed days of inpatient overstay through facilitating the discharge process using a multidisciplinary intervention approach.

Genotype and Phenotype of Adenosine Deaminase 2 Deficiency: a Report from Saudi Arabia.

Adenosine deaminase 2 deficiency (DADA2), a rare and potentially fatal systemic autoinflammatory disease, is characterized by low or lack of ADA2 activity due to ADA2 mutations. DADA2 symptoms are variable and include vasculitis, immunodeficiency, and cytopenia. Minimal data are available from Saudi Arabia.

Novel Adenosine Deaminase 2 (ADA2) Mutations Associated With Hematological Manifestations.

Recent progress in laboratory techniques, particularly, identification of novel disease-causing genes, has led to the detection of different gene mutations that might be implicated in the pathogenesis of different hematological disorders like pure red cell aplasia (PRCA) and neutropenia. An autoinflammatory disorder known as deficiency of adenosine deaminase 2 (DADA2) has been recently noticed to present with variable hematologic abnormalities. We report 2 patients who presented with hematologic abnormalities in which 2 ADA2 gene mutations were detected.

Outcomes of multidisciplinary team interventions in the management of sickle cell disease patients with opioid use disorders. A retrospective cohort study.

Objectives: To identify the magnitude of opioid use disorder (OUD) among sickle cell disease (SCD) patients; emphasize on multidisciplinary team (MDT) role; estimate cost-effectiveness following the proper use of therapeutic guidelines; and facilitate the reduction of emergency room (ER) visits and the length of stay (LOS).

Methods: This retrospective cohort study included SCD patients aged 14 years and above, who have OUD. Data was collected between January 2016 and December 2018.

Posterior reversible encephalopathy syndrome secondary to asymptomatic poststreptococcal glomerulonephritis in a child with sickle cell anemia: a case report.

Background: Posterior reversible encephalopathy syndrome is a neurotoxic condition that occurs as a result of the failure of posterior circulatory autoregulation in response to acute changes in blood pressure. Overperfusion with resultant disruption of the blood-brain barrier results in vasogenic edema, but not infarction. Posterior reversible encephalopathy syndrome can be the presenting feature of postinfectious glomerulonephritis, which has been reported in approximately 5% of hospitalized children, and it has been reported in very few cases of adult patients with sickle cell anemia.

Trends and outcomes of pancreaticoduodenectomy for periampullary tumors: A 25-year single-center study of 1000 consecutive cases.

Aim: To evaluate the evolution, trends in surgical approaches and reconstruction techniques, and important lessons learned from performing 1000 consecutive pancreaticoduodenectomies (PDs) for periampullary tumors.

Methods: This is a retrospective review of the data of all patients who underwent PD for periampullary tumor during the period from January 1993 to April 2017. The data were categorized into three periods, including early period (1993-2002), middle period (2003-2012), and late period (2013-2017).

Tumor histopathological response to neoadjuvant chemotherapy in childhood solid malignancies: is it still impressive?

The management of oncological malignancies has significantly improved over the last decades. In modern medicine, new concepts and trends have emerged paving the way for the era of personalized and evidence-based strategies adapted to the patients' prognostic variables and requirements. Several challenges do exist that are encountered during the management, including the difficulty to assess chemotherapy response with certainty.

Immune Thrombocytopenia and Obesity: Predictive Relationship.

Background: Chronic refractory immune thrombocytopenia (ITP) is defined as the failure of any modality to maintain the platelet count above 20 × 10/μL for an appreciable time without unacceptable toxicity. To date, certain predictive factors have been associated with refractory ITP. However, none of the published studies has declared the possible association between obesity and refractory ITP.

Clinicopathological features and surgical outcome of patients with fibrolamellar hepatocellular carcinoma (experience with 22 patients over a 15-year period).

Aim: To evaluate the clinicopathological features and the surgical outcomes of patients with fibrolamellar hepatocellular carcinoma (FL-HCC) over a 15-year period.

Methods: This is a retrospective study including 22 patients with a pathologic diagnosis of FL-HCC who underwent hepatectomy over a 15-year period. Tumor characteristics, survival and recurrence were evaluated.

Post-endoscopic retrograde cholangiopancreatography pancreatitis: Risk factors and predictors of severity.

Aim: To detect risk factors for post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis (PEP) and investigate the predictors of its severity.

Methods: This is a prospective cohort study of all patients who underwent ERCP. Pre-ERCP data, intraoperative data, and post-ERCP data were collected.

Iron Overload and Platelet Function Defects: Possible Correlation.

Acquired platelet function defect might be a consequence of iron overload. Even though there are various complications of iron overload, only few reports have indicated some correlations with platelets dysfunction. We report a child with Diamond-Blackfan anemia who has significant complications from iron overload due to chronic blood transfusion, and one of these complications is acquired platelet function defect that manifests with frequent episodes of epistaxis.

Outcomes of pancreaticoduodenectomy in elderly patients.

Background: Although the mortality and morbidity of pancreaticoduodenectomy (PD) have improved significantly over the past years, the concerns for elderly patients undergoing PD are still present. Furthermore, the frequency of PD is increasing because of the increasing proportion of elderly patients and the increasing incidence of periampullary tumors. This study aimed to analyze the outcomes of PD in elderly patients.

How does epidemiological and clinicopathological features affect survival after gastrectomy for gastric cancer patients-single Egyptian center experience.

Aim: To investigate the clinicopathological features and the significance of different prognostic factors which predict surgical overall survival in patients with gastric carcinoma.

Methods: This retrospective study includes 80 patients diagnosed and treated at gastroenterology surgical center, Mansoura University, Egypt between February 2009 to February 2013. Prognostic factors were assessed by cox proportional hazard model.

Predictors of long term survival after hepatic resection for hilar cholangiocarcinoma: A retrospective study of 5-year survivors.

Aim: To determine predictors of long term survival after resection of hilar cholangiocarcinoma (HC) by comparing patients surviving > 5 years with those who survived < 5 years.

Methods: This is a retrospective study of patients with pathologically proven HC who underwent surgical resection at the Gastroenterology Surgical Center, Mansoura University, Egypt between January 2002 and April 2013. All data of the patients were collected from the medical records.

Early Versus Late Cholecystectomy After Clearance of Common Bile Duct Stones by Endoscopic Retrograde Cholangiopancreatography: A Prospective Randomized Study.

Introduction: The time interval between endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic cholecystectomy (LC) is a matter of debate. This study was planned to compare early LC versus late LC.

Patients And Methods: This is a prospective randomized study on patients who are presented with concomitant gallbladder and common bile duct stone.

Insulin-Like Growth Factor-1 and Vascular Endothelial Growth Factor in Malignant and Benign Biliary Obstructions.

Background: Despite the presence of various diagnostic tools, the differential diagnosis between malignant and benign biliary obstructions is so difficult. This study aimed to evaluate the role of serum and biliary insulin-like growth factor-1 (IGF-1) and vascular endothelial growth factor (VEGF) in this differential diagnosis.

Materials And Methods: Patients (n = 109, 61 men and 48 women) with diagnosis of benign (n = 62) or malignant (n = 47) biliary obstruction were included.

Myelodysplastic Syndrome Presenting as Amegakaryocytic Thrombocytopenia in a Collodion Baby.

We report a rare case of myelodysplastic syndrome that presented early as amegakaryocytic thrombocytopenia in a collodion baby, which is a rare congenital disorder characterized by thick, taut membrane resembling oiled parchment or collodion, which is subsequently shed. To our knowledge, this is the first reported case of a collodion baby who presented with amegakaryocytic thrombocytopenia and who has a significant family history of the same condition. We document the rarity of this possible association and also the need for further study to establish whether a causal relationship exists.

Management and Outcome of Borderline Common Bile Duct with Stones: A Prospective Randomized Study.

Introduction: Management of common bile duct stones (CBDS) in patients with borderline CBD presents a surgical challenge. The aim of this study was to compare conservative treatment with endoscopic stone extraction for the treatment of borderline CBD with stones.

Patients And Methods: This prospective randomized controlled trial includes patients with CBDS in borderline CBD (CBD <10 mm) associated with gallbladder stones who were treated with conservative treatment or endoscopic stone extraction followed by laparoscopic cholecystectomy (LC) and intraoperative cholangiogram (IOC).