The Association Between Dental Caries and Salivary Buffering Capacity in Syrian Patients Diagnosed with Sickle Cell Disease.

Background Sickle cell disease (SCD) is a genetic disorder caused by mutations in the HBB gene, resulting in the abnormal shape of red blood cells. This condition is accompanied by various oral manifestations including salivary gland dysfunction leading to a heightened susceptibility to dental caries. This disorder is primarily treated with hydroxyurea. This study aims to assess dental caries utilizing the decay, missing, filling teeth (DMFT) index and evaluate salivary buffering capacity in patients diagnosed with SCD (HbSS type). The study also aims to assess the relationship between DMFT and salivary buffering capacity. Additionally, the study aimed to find a correlation between treatment with hydroxyurea and changes in both dental caries and salivary buffering capacity. Methods This case-control study enrolled a total of 100 participants aged between 20 and 50 years. The participants were divided into two groups: the study group, which comprised 70 individuals diagnosed with SCD (HbSS type), who were asked to report their current use of hydroxyurea, and the control group, which included 30 healthy individuals. Dental caries were assessed using the DMFT index, while salivary buffering capacity was measured using a pH meter model 420A device. Results The study group exhibited a mean DMFT index value of 6.39 compared to 5.20 in the control group. This difference was statistically significant (P-value=0.037), indicating higher DMFT values among patients with SCD. Salivary buffering capacity was significantly lower in the study group compared to the control group, with average values of 6.47 and 6.88, (P-value=.022). Interestingly, the administration of hydroxyurea impacted salivary buffering capacity, resulting in lower values for individuals using the drug (P-value=0.039). Conversely, hydroxyurea did not have a significant effect on DMFT values (P-value=0.317). Conclusion SCD increases susceptibility to dental caries and is associated with significant changes in salivary composition. At the same time, the potential negative impact of hydroxyurea is acknowledged.

The Curious Case of a Painful Leg Ulcer.

Sickle cell disease is a condition that can involve numerous organ systems secondary to vascular occlusion. Herein, we present a case of a 21-year-old male with sickle cell disease requiring long-term hydroxyurea therapy. Upon migrating to the United States from Yemen, the patient developed a rapidly progressive, exquisitely painful ulcer on his right lower extremity. Given his country of origin, a broad differential, including select infectious diseases, was essential. Moreover, establishing the unequivocally correct diagnosis was crucial to determine proper and safe therapy. Ultimately, a lesional biopsy demonstrated numerous sickled red blood cells occluding blood vessels, leading to the diagnosis of sickle cell disease-related leg ulceration.

Unusual Distribution of Cerebral Venous Thrombosis in a Patient With Sickle Cell Disease: A Case Study.

This study presents the case of a 29-year-old Bahraini woman with a known history of sickle cell disease who exhibited acute neurological symptoms. Advanced imaging, specifically CT and MRI, identified cerebral venous thrombosis (CVT). The patient was managed with fluid therapy and anticoagulation, and received a packed red blood cell transfusion, leading to a complete recovery. Notably, this case was marked by the patient's positive anti-double stranded DNA (anti-dsDNA) status, typically linked with systemic lupus erythematosus (SLE), adding a potential pro-coagulant factor. The occlusion pattern, particularly involving the internal cerebral veins, was unique compared to other reviewed CVT cases in patients with sickle cell disease. This case emphasizes the significance of early diagnosis and intervention in CVT, especially in patients with sickle cell disease and other predisposing factors.

Jk(a) (Kidd-A) Variant in a Sickle Cell Disease Patient.

Sickle cell disease (SCD) is a chronic and prevalent hemoglobin disorder with various manifestations and complications depending on the organs involved. Red cell transfusion either simple or exchange is crucial due to its prophylactic and therapeutic roles. We present a case showing serologic discrepancy between the red cells phenotype and the developed alloantibodies to emphasize the crucial role of molecular testing in SCD patients requiring chronic blood transfusion.

A Case of Right Ventricular Failure Secondary to Acute Chest Syndrome Managed With Early Red Cell Exchange Transfusion.

Patients with sickle cell disease are at risk of vaso-occlusive crises including acute chest syndrome (ACS) and pulmonary hypertension. ACS is a life-threatening complication of sickle cell disease and is associated with increased morbidity and mortality. It is known that pulmonary pressures increase during episodes of acute chest syndrome and may lead to acute right ventricular failure leading to increased morbidity and mortality. Given the paucity of randomized controlled trials, the management of ACS and pulmonary hypertension in the setting of a sickle cell crisis largely relies on expert opinion. We present a case of acute chest syndrome complicated by acute right ventricular failure that was managed with prompt red cell exchange transfusion with favorable clinical outcomes.

Deep Compartment Syndrome Without Myonecrosis: A Case Report on a Rare Complication of Sickle Cell Disease.

Compartment syndrome is a rare manifestation of vaso-occlusive crisis, a serious complication of sickle cell disease (SCD), which is an inherited hemoglobinopathy. During a visit to Norway, an otherwise healthy, 20-year-old male from Ghana was admitted to Oslo University Hospital (Day 1) because of increasing pain in the hip and thighs that did not respond adequately to non-opioid painkillers. Despite initial treatment with intravenous fluids and opioids, his pain intensified. Careful clinical inspection supported by an MRI examination revealed focal, high-signal-intensity muscle edema of the anterior compartment of the thigh, almost exclusively limited to the vastus intermedius muscles. There were no MRI findings or blood biochemistry evidence for myonecrosis or rhabdomyolysis, and a diagnosis of deep compartment syndrome appeared to be the most likely explanation for his pain. We decided to continue with a conservative treatment approach, and the patient did not undergo a fasciotomy or blood transfusion therapy. On Day 7 after admission, his condition improved markedly, and he was discharged on Day 11 whereupon he returned to Ghana. This case is a reminder that, although rare, deep compartment syndrome can be a severe manifestation of vaso-occlusive crisis in SCD and should be considered in patients with severe, deep muscular pain in the absence of other explanatory factors.

Sickle Cell Disease and Its Respiratory Complications.

Sickle cell disease (SCD) is a hematological disorder that is inherited in an autosomal recessive (AR) fashion. It is caused by mutations in the genes encoding for the globin apoprotein of hemoglobin (Hb), leading to diminished oxygen-carrying ability. Its pathophysiologic mechanism affects multiple organ systems, making it crucial to understand the complications of SCD and find the best ways to prevent and treat them. Some important ways that SCD manifests in the respiratory system are acute chest syndrome (ACS), pulmonary hypertension (PH), asthma, and venous thromboembolism (VTE). This article summarizes their salient features, including pathogenesis related to the adverse outcomes, screening practices, and management guidelines, with the intent to provide greater insight into forming better practices that increase the quality of life in SCD patients.

The Efficacy of Marijuana Use for Pain Relief in Adults With Sickle Cell Disease: A Systematic Review.

Sickle Cell Disease (SCD) is a disease that affects many around the world and often accounts for frequent hospital admissions every year, secondary to uncontrolled pain. Marijuana is increasingly being used for its medicinal ability to treat pain in chronic medical conditions. Therefore, it is imperative to determine how effective it would be in providing pain relief to patients with SCD. We systematically screened five databases for relevant data: PubMed, Medline, PubMed Central (PMC), Cochrane Library, and Google Scholar. The inclusion and exclusion criteria were implemented. A quality appraisal was then done using the Cochrane Bias assessment for randomized controlled trials (RCTs), Newcastle-Ottawa tool for observational studies, and Scale for the Assessment of Narrative Review Articles (SANRA) checklist for traditional review articles. From seven articles, information was gathered; one systematic review, one RCT, two surveys, one cross-sectional study, one retrospective study, and one questionnaire-based study. Our review concluded that based on the literature assessed, marijuana use in SCD patients either worsened their painful crises or offered little to no help compared to opioids or hydroxyurea usage. There were limited RCTs published in addition to papers investigating the long-term effects of marijuana use in SCD. We hope that further data is gathered in these areas to sufficiently address whether cannabis use is efficacious for pain relief in patients with SCD.

A Huge Subcapsular Splenic Cyst Like Hematoma in Sickle Cell Anemia.

Nontraumatic splenic rupture and hematoma are rare in sickle cell disease. We present a case of a 22-year-old Saudi male with sickle cell disease. He presented to our hospital with a history of nontraumatic abdominal pain, hemodynamic instability, and abdominal tenderness, with a large mass extending to the umbilicus. A computed tomography (CT) examination showed splenomegaly and a spleen infarction. The patient was admitted to the intensive care unit (ICU) and stabilized. He was transferred to the regular ward and discharged against medical advice (DAMA). Later on, he presented again with persistent abdominal pain. He underwent splenectomy with cholecystectomy. The patient did well postoperatively and was discharged in good condition. While conservative management is common, operative management should be considered in patient with persistent pain. Splenic rupture has a high mortality rate.

Incidence and predictors of bacterial infections in febrile children with sickle cell disease.

INTRODUCTION: Sickle cell disease (SCD) is an autosomal recessive disorder. The incidence of bacterial infection in children with SCD globally is 16% compared 3-14% in general children. Bacterial infection in children is a severe problem and is considered to be a life-threatening condition. To reduce antibiotic overuse, the following factors might be associated with bacterial infection could help: age, C-reactive protein (CRP), white blood cells (WBCs) count, absolute neutrophil count (ANC), and genotype. Therefore, this study is designed to evaluate the CRP, ANC, WBCs, and platelet count levels as predictors for bacterial infection in febrile children with sickle cell anemia over a six-year period in a tertiary center in Jeddah, Saudi Arabia. METHODS: This study was a retrospective record review that included all SCD patients below the age of 18 years who presented with a febrile episode at any hospital's department from 2017 to 2019. Data were extracted from patient files that included culture result and the causative organism, CRP level, WBCs, ANC, and platelet count. RESULTS: The study included 62 children diagnosed with SCD who presented with 89 febrile episodes. There was no statistically significant difference in the median of CRP and ANC between the bacterial and nonbacterial febrile episodes (P = .314, .735, respectively). However, the level of WBC> 20 K/µL was statistically significant at P = .025. CONCLUSION: WBCs significantly associated with a bacterial infection in SCD febrile children along with clinical assessments. This parameter can guide the physicians to determine the children at high risk of bacterial infection.

Cardiac Chamber Quantification by Echocardiography in Adults With Sickle Cell Disease: Need Attention to Eccentric Hypertrophy.

Introduction and aim Sickle cell anemia (SCA) is the most common hemoglobinopathy worldwide, and cardiovascular diseases are the most common causes of death. In these patients, cardiac remodeling begins from childhood and leads to sickle cell cardiomyopathy in the following years. Concentric hypertrophy and eccentric hypertrophy are known to predict early cardiac events. This study aims to reveal the relationship between cardiac remodeling types and survival in patients with SCA and investigate the factors that may affect left ventricular mass. Materials and methods A total of 146 patients with SCA were included in the study, and the left ventricular mass index (LVMI) and relative wall thickness (RWT) of the patients were calculated according to echocardiographic measurements, and the patients were categorized into normal, concentric remodeling (CR), concentric hypertrophy (CH), and eccentric hypertrophy (EH) groups. Results The median age of the patients is 32 (18-72). In logistic regression analysis, hemoglobin S (HbS) and ferritin levels were independent predictors for LVMI (p = 0.01 and p < 0.001, respectively). It was observed that 56 (38.4%) of the patients had normal left ventricles, 24 (16.4%) had CR, 21 (14.4%) had CH, and 45 (30.8%) had EH. 31 (21.2%) of the patients died. When we look at the survival curves, there was a statistically significant difference between the four groups (log-rank p < 0.001). It was observed that patients with EH were the group with the lowest probability of survival. Conclusion Cardiac death is one of the most common causes of death in patients with SCA. Early detection of cardiac disorders and starting treatment may be important in reducing mortality in these patients.

Acute Liver Failure in Sickle Cell Disease: A Perfect Storm.

Sickle cell hepatopathy is a well-described but uncommonly seen complication of sickle cell disease and is usually caused by multiple overlapping processes. A more acute liver complication is hepatic sequestration which is important to recognize in order to initiate life-saving treatment. A 33-year-old woman with sickle cell disease complicated by painful crises, splenic infarction and significant alcohol abuse presented with gastrointestinal distress, pain crisis, acute-on-chronic anemia, and hyperbilirubinemia in the setting of greater than baseline alcohol consumption. She was found to have hepatomegaly, encephalopathy, severe jaundice, and severe hyperbilirubinemia. She was treated with red cell exchange and supportive care which resulted in an improvement in her symptoms as well as hyperbilirubinemia. She was discharged with plans for monthly red cell exchange, iron chelation therapy, and close monitoring of liver disease was planned upon discharge. This case illustrates that chronic liver disease can occur in sickle cell disease (Hgb SS) especially in the setting of acquired iron overload. More acutely, sequestration is a serious and life-threatening complication of sickle cell disease that can culminate in acute liver failure. Primary treatment for hepatic sequestration is red cell exchange along with management of contributing comorbidities, and symptomatic management of encephalopathy. In end-stage liver disease, transplantation may be considered in the context of the patient's clinical status.

Spontaneous Epidural and Corpus Callosum Hemorrhage in Sickle Cell Disease - An Unusual Presentation in a Ghanaian Patient.

Spontaneous intracranial bleed in sickle cell disease is a rare presentation and complication of the disease, with a few cases presenting with epidural hematoma. We present an 18-year-old boy with sickle cell, hemoglobin FS, who presented with non-traumatic scalp swelling, headaches, and vomiting six days following an episode of vaso-occlusive crisis with bone pain. A head CT scan showed extensive epidural hematoma with mass effect and acute corpus callosum bleed. The patient, however, had a cardiac arrest with unsuccessful resuscitation before neurosurgical interventions could be instituted. Of all reported cases, none has reported associated bleeding in the corpus callosum, making our case the firstwith such a combination, possibly worsening the outcome.

Association between eNOS Gene Polymorphism (T786C and VNTR) and Sickle Cell Disease Patients in Ghana.

Endothelial nitric oxide synthase (eNOS) variants have been found to be associated with several vascular disorders as well as the pathogenesis of sickle cell disease (SCD) complications such as vaso-occlusive crises (VOC). Studies on eNOS gene variants among SCD patients are rare in Ghana and several other African countries. The current study aimed to determine a possible association between variants of the eNOS gene (variable number of tandem repeats in intron 4 and T786C) in SCD complications among Ghanaian patients. This was a cross-sectional study involving 89 HbSS patients with complications and 46 HbSS patients without complications. Genomic DNA was extracted from leukocytes in the buffy coat and separated from collected whole blood samples of the study participants. PCR amplification, followed by restriction fragment length polymorphism (RFLP) was used to genotype T786C (rs2070744) variants. Variable number of tandem repeats (VNTR) in intron 4 was genotyped by PCR and direct electrophoresis. There was a significant difference in the genotype frequency of the T786C variant between HbSS patients with complications and those without complications (p = 0.0165). However, there was no significant difference in the VNTR intron 4 variant of the eNOS gene between patients with complications and those without complications (p > 0.05). The study shows an association between the eNOS gene variant (T786C) and complications in SCD.

A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease.

Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Patients with SCD were investigated by MDCT during ACS. A logistic regression was performed to determine independent risks factors for PAT and to build the PAT-ACS risk score. A total of 43 episodes (11·9%) of PAT were diagnosed in 361 episodes of ACS. Multivariate analysis identified four risk factors, which were included in the PAT-ACS risk score: a baseline haemoglobin >82 g/l, the lack of a triggering factor for ACS, a platelet count >440 × 109 /l and a PaCO2 <38 mmHg at ACS diagnosis. The area under the receiver operating characteristic curve for the PAT-ACS risk score was 0·74 (95% confidence interval [CI] 0·69-0·79) and differed from that of the revised Geneva score (0·63 (95% CI 0·58-0·69); P = 0·04). The negative predictive value of a PAT-ACS risk score ≥2 was 94%. In conclusion, we propose a simple clinical risk score to identify SCD patients at high risk of PAT during ACS.