Intravenous mercury poisoning is a rare but severe medical emergency, often resulting from accidental exposure or intentional self-harm. We present the case of a 30-year-old male with a history of sickle cell anemia who presented with high-grade fever, vomiting, giddiness, and breathlessness following intravenous mercury self-administration. Diagnostic challenges included distinguishing symptoms of acute mercury toxicity from those of his chronic condition of sickle cell trait. Markedly elevated serum mercury levels confirmed the diagnosis, with high-resolution computed tomography (HRCT) imaging studies revealing mercury deposits and alveolar lung injury. Management involved antidote of mercury poisoning, including non-invasive ventilation and transfusions, with consultations from multiple specialties. The patient demonstrated significant clinical improvement and was discharged with scheduled follow-ups. This case underscores the complexity of diagnosing and managing intravenous mercury poisoning, highlighting the importance of a comprehensive multidisciplinary approach for optimal patient outcomes.

UNASSIGNED: There is growing recognition of the benefits of nutraceuticals in the management of sickle cell disease but a scarcity of reports on their use. Nutraceuticals are food or parts of food that provide medical or health benefits. They include botanicals, functional foods and medicinal foods. This is a preliminary report on the use of nutraceuticals in the management of a small cohort of children with sickle cell disease in Nigeria.
UNASSIGNED: The retrospective audit of clinical outcomes was carried out at a brain center located within the Federal Capital Territory, Abuja, Nigeria. Children, aged 1 to 12 years, presenting with sickle cell anaemia were evaluated at baseline and at six months after the commencement of a cocktail of nutraceuticals using an objective grading tool. Changes in weight, haematocrit and frequency of sickle cell crises were determined. On account of the small sample size, Wilcoxon sign test was used to determine the similarity / difference between the average values of weight, PCV, and crises obtained at enro7lment and six months.
UNASSIGNED: Ten children with sickle cell anaemia were placed on nutraceuticals therapy. The average age of the children was 8.5 (range 1 - 12) years. Aroga immune support was the commonest component of the nutraceutical cocktail given to the children. There was a rise in average weight (from 21.8 to 23.0 Kg, p-value 0.236) and average haematocrit (22.8 to 27.2, p-value 0.011) at six months compared to values obtained at baseline. There was also a fall in the average frequency of sickle cell crises at six months compared to values obtained at baseline (from 7.4 to 3.2, p-value 0.011). Overall, eight out of the ten children showed moderate to good clinical improvement. There was no documentation of any adverse reaction to the medications in any of the children.
UNASSIGNED: The results suggest that the use of nutraceuticals may be beneficial in the management of sickle cell anaemia in children. However, the results are preliminary and will require stronger evidence. There is a need for controlled clinical trials for using larger sample sizes. Such clinical trials of unconventional therapies should be conducted with great care and concern for the safety of the participants.

Sickle cell disease (SCD) has been identified as one of the most prevalent genetic conditions. It alters the shape and function of red blood cells. This brief case report presents a case of a five-year-old male with sickle cell disease who complained of pain in the left mandibular region due to deep proximal caries. Before dental management, a complete fitness evaluation was performed with the help of a pediatrician, followed by informed consent. Dental management includes pulpectomy followed by stainless steel crown placement and Glass ionomer cement (GIC) restoration for superficial caries. Other oral manifestations were observed, including a smooth tongue and mucosal pallor. It was concluded that dentists and health professionals should be knowledgeable of the general and oral anomalies that can be present in individuals with sickle cell anemia in order to take preventive action and implement effective management.

Red cell exchanges (RCE) help in the treatment of complications of sickle cell anemia (SCA) by reducing the viscosity of blood and improving the oxygen-carrying capacity. We present a case of sickle cell crisis (SCC) managed with automated RCE and also reviewed the literature to assess the utilization and clinical efficiency of this therapy in India. A 19-year-old gentleman diagnosed with SCA presented with acute chest syndrome. Hemoglobin (Hb) was 8.8 g%, hematocrit (HCT) was 24%, and HbS was 90%. As there was worsening of symptoms with conventional management, the patient underwent two procedures of automated RCE. The clinical condition of the patient was improved, HbS was reduced to 16% and HCT was remained at 21% postprocedure. Articles on automated RCE in SCA conducted in India were reviewed and four articles were analyzed based on the search strategy. All the included articles concluded automated RCE as an effective procedure for complications of SCA. Common indication in India was SCA patients undergoing surgery as a prophylactic measure. Automated RCEs are promising as an acute treatment for indicated sickle cell complications. This therapy is underutilized in the Indian scenario, especially in patients with SCC.

BACKGROUND: Sickle cell disease is one of the most common hemoglobinopathies in Africa. Tanzania alone accounts for about 11,000 sickle cell births annually making it one of the most common disorders in eastern Africa. The affected individuals are prone to several complications since childhood as a result of the defective hemoglobin structure, these include neurological complications such as ischemic stroke due to hypercoagulability state caused by the disease. Spontaneous intracranial hemorrhage such as subdural hemorrhage in the absence of predisposing factors such as trauma, anticoagulant use, or recent blood transfusions is rare. As reported in the previous literature.
METHODS: We report a rare case of acute spontaneous subdural hemorrhage in an adolescent sickle cell patient of African descent.
CONCLUSIONS: Initial management including early referral and medical treatment is crucial for cases that are suspicious of intracranial hemorrhage. These cases are more common to be missed in resource-limited settings where there are a limited number of neurosurgery interventions.
CONCLUSIONS: Although few reported cases of spontaneous intracranial hemorrhage in sickle cell patients are reported, it is important to be vigilant as a clinician wherever a sickle cell patient presents with signs of increased intracranial pressure without a history of trauma such as in our patient and order an urgent brain imaging to rule out spontaneous hemorrhagic events which may lead to fatal consequences if missed out.

OBJECTIVE: To report a rare case of spontaneous bilateral epidural hematoma (EDH) in a 10-year-old Nigerian child with sickle cell disease (SCD) and review the literature regarding this unusual complication.
METHODS: We present a case of a pediatric patient with SCD who developed a spontaneous bilateral EDH and discuss the potential underlying mechanisms, management approaches, and outcomes. We also conducted a literature review of existing cases of spontaneous EDH in patients with SCD.
RESULTS: Our patient initially presented with a subgaleal hematoma and underlying bilateral EDH, but she was sent home without any neurosurgical consultation. Two years later, she returned with altered consciousness and left-sided weakness, revealing an increased size of the EDH with a noticeable mass effect. She underwent a successful emergency bilateral craniotomy, with noticeable improvement in her level of consciousness and left-sided weakness post-operatively. In our literature review, we found 40 reported cases of spontaneous EDH in SCD patients, with a male predominance (82.5%). The average age of patients was 15.282 years. The most common hematoma location was bifrontal (20%) and the most reported symptom was headache (47.5%). Most patients (97.5%) were already known cases of SCD. Among those treated, 77.5% survived.
CONCLUSIONS: Spontaneous bilateral EDH in SCD patients is an uncommon complication, with a variety of proposed pathophysiological mechanisms. Prompt recognition and appropriate management, either conservative or surgical, are crucial to improve outcomes. Our case and literature review underscore the importance of considering spontaneous EDH in SCD patients presenting with neurological symptoms, even in the absence of trauma. Further research is needed to elucidate the precise etiology, identify risk factors, and optimize management approaches for this rare complication in SCD patients.

UNASSIGNED: Sickle cell disease (SCD) rarely presents with acute soft head syndrome (ASHS) often posing a diagnostic dilemma. Recovery is typically spontaneous, however, in the context of lack of awareness and limited brain imaging it could potentially lead to poor outcome.
UNASSIGNED: ASHS is a rare complication of SCD, invariably occurring near puberty with hitherto elusive pathogenic mechanisms. ASHS often resolves spontaneously on conservative management, however, lack of awareness in the context of limited access to brain imaging could pose diagnostic challenges resulting in inappropriate management and untoward outcome. We present a case of a teenager who presented with subtle symptoms for which the diagnosis of sickle cell anemia (SCA) was delayed until he developed ASHS. LTM was a 16 years old boy with a history of recurrent joints pain since the age of 6 years, with a family history of SCA, but had initial negative sickling test. He presented with episodes of multiple joints pain, unprovoked scalp and left orbital swelling, low-grade fever and mild headache without any evidence for bleeding diathesis. The diagnosis of SCA was confirmed by hemoglobin electrophoresis. Computed tomography (CT) scan of the head revealed subgaleal heamatoma (SGH) and intraorbital haematoma without intracranial hemorrhage (ICH). He was managed conservatively with analgesics and hydration together with antibiotics for associated sepsis with complete resolution of clinical symptoms within 2 weeks. This case represents a rare scenario for a relatively mild SCA phenotype presenting with ASHS whose diagnosis poses an enigma in the resource-limited contex. It is therefore, prudent to recognize ASHS to avoid judicious interventions which could potentially result in untoward clinical outcome.

BACKGROUND: Puberty may be impaired in children with sickle cell anemia (SCA). Therefore, we aimed to explore the clinical and hormonal features of puberty in Cameroonian children.
METHODS: In a case-control study, we included 64 children aged 8-18 years with SCA matched to healthy controls. We assessed height, weight, body mass index, body composition, and Tanner stages. Hormonal measurements included anti-mullerian hormone, follicle-stimulating hormone, luteinizing hormone, and sex hormones (estrogens/testosterone). We used the Mann-Whitney Wilcoxon test to compare the median values between cases and controls. We looked for associations between the severity criteria of SCA and delayed puberty through multivariate analysis.
RESULTS: Delayed puberty was reported in 27.3% of girls and 10% of boys with SCA. The median age of menarche was delayed by 2 years compared to controls. SCA patients had a low lean body mass compared to controls (p = 0.03). Anti-mullerian hormone levels were significantly higher in boys with SCA than those of controls (45.9 ng/mL vs. 17.65 ng/mL; p = 0.018). A history of severe infection, acute chest syndrome, and low hemoglobin level was associated with delayed sexual maturation in children with SCA.
CONCLUSIONS: Our study revealed delayed puberty in children with SCA. Moreover, puberty is affected by the severity of the disease. This highlights the importance of regular monitoring of puberty during the follow-up of these children.

Sickle cell anemia is a chronic and debilitating hemoglobinopathy characterized by the presence of abnormal hemoglobin, resulting in the formation of sickle-shaped red blood cells. This case report presents an unusual case of a 32-year-old female patient with sickle cell anemia who had not experienced any previous sickle cell crises since her diagnosis at the age of four years. Despite a stable clinical history, the patient\'s condition rapidly deteriorated, leading to septic shock, multiorgan failure, and atypical complications such as neurological impairment and acute kidney injury. Intensive management strategies, including blood exchange, mechanical ventilation, and aggressive antibiotic therapy, were implemented but unfortunately failed to reverse the progressive clinical deterioration. This case underscores the importance of early recognition and a multidisciplinary approach in managing atypical sickle cell crises to optimize patient outcomes. Further research is needed to improve our understanding and management of such presentations.

Sickle cell anemia (SCA) is an autosomal recessive disorder caused by a mutation in beta globin gene. Hepatobiliary system is affected in 10-40% of patients with SCA and has a multifactorial etiology. The authors present a child with SCA and conjugated hyperbilirubinemia due to biliary obstruction. He underwent endoscopic retrograde cholangiopancreatography (ERCP) and biliary stenting, had complications of post sphincterotomy bleed, retroperitoneal hematoma and post laparoscopic cholecystectomy sepsis with acute sickle hepatic crisis. He was managed successfully and is doing well on follow-up. Here authors discuss a stepwise approach in management of jaundice in a patient with SCA. Patients with SCA are prone to develop vaso-occlusive crisis (VOC) during periods of stress. VOC affects the liver as acute sickle hepatic crisis, acute hepatic sequestration or sickle cell intrahepatic cholestasis and is collectively termed as sickle cell hepatopathy. Hemolysis due to sickling results in cholelithiasis with its associated complications. These patients are vulnerable to viral hepatitis and hemochromatosis due to multiple blood transfusions. There may be a concomitant acute viral hepatitis, drug induced liver injury, Budd-Chiari syndrome or other chronic liver diseases. These conditions have considerable clinical overlap and may coexist, making the evaluation more challenging. Detailed history, examination and investigations are required for differentiation of etiology. Periods of stress must be tackled with proper hydration, oxygen supplementation, maintaining hemoglobin >10 g/dL, and a low hemoglobin S fraction. Patients with SCA and conjugated hyperbilirubinemia are \"high-risk\" and best managed by a multidisciplinary team. Preventive strategies like timely vaccinations, chelation, etc. must be practised.