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What Are the Complications of Sickle Cell Trait?
Discussion Sickle cell disease (SCD) is a group of inherited blood disorders where there are abnormal hemoglobin molecules and the red blood cells take on a characteristic sickled shape instead of a rounded shape. Sickled hemoglobin provides an evolutionary advantage of giving the individual increased protection against severe and cerebral malaria. The sickled shape doesn’t move through the vascular system as well and therefore is more likely to aggregate in small vessels causing an increased risk of vaso-occlusive disease, acute chest syndrome, splenic sequestration, and priapism. There is also an increased risk of ...
Source: PediatricEducation.org - September 4, 2023 Category: Pediatrics Authors: Pediatric Education Tags: Uncategorized Source Type: news

MTHFR C677T Polymorphism, Plasma Homocysteine, and PDGF-AA Levels and Transcranial Doppler Velocity in Children with Sickle Cell Disease
CONCLUSION: PDGF-AA and tHcy levels could be used as predictive markers for stroke in SCD children. MTHFR Polymorphism contributes to elevated tHcy levels.PMID:37260067
Source: Indian Pediatrics - June 1, 2023 Category: Pediatrics Authors: Asmaa A Mahmoud Nahla M S Abd El Hady Mohammed S Rizk Ahmed M El-Hawwary Nagwan Y Saleh Source Type: research

Cerebral artery conditional blood velocity in sickle cell disease: a multicentre study and evidence for active treatment
Conclusion Since conditional blood velocity in cerebral arteries can progress to high values and predispose to stroke, the proportion of children with SCD who are affected (15.5%) raises the question of whether regular monitoring and proactive intervention ought to be the standard of care.
Source: Archives of Disease in Childhood - May 18, 2023 Category: Pediatrics Authors: Modebe, E., Nonyelu, C., Duru, A., Ezenwosu, O., Chukwu, B., Madu, A., Ezekekwu, C., Aneke, J., Izuka, M., Nri-Ezedi, C., Nnachi, O., Eze, A., Ajuba, I., Okwummuo, E., Chilaka, J., Onodugo, C., Fidelis-Ewa, U., Agwu, O., Anigbogu, I., Muoghalu, E., Okoye, Tags: ADC Global child health Source Type: research

Brain-derived neurotrophic factor and neuroimaging in pediatric patients with sickle cell disease
CONCLUSION: Serum BDNF levels were higher in sickle disease patients who had abnormal transcranial Doppler. BDNF had a significant diagnostic ability in the detection of SCD complications.IMPACT: Silent stroke is a very serious complication in children with sickle cell disease, so regular follow up should be every six months. BDNF is considered a potential biomarker for stroke risk prediction in patients unable to receive TCD.PMID:36774398 | DOI:10.1038/s41390-023-02513-5
Source: Pediatric Research - February 11, 2023 Category: Pediatrics Authors: Asmaa A Mahmoud Sameh A Abd El Naby Ahmed S Abdelgawad Marwa Sabry Rizq Nahla M S Abd El Hady Source Type: research

Sickle-Cell Disease and Stroke: Quality of Life of Patients in a Chronic Transfusion Regimen from the Caregivers ’ Perspective
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Source: Pediatric Hematology and Oncology - November 24, 2022 Category: Pediatrics Authors: Maria Aline Ferreira de Cerqueira Luciana Maria Fortes Magalh ães Castelo Branco Couto M írian Perpétua Palha Dias Parente Juan Clinton Llerena Source Type: research

Fifteen-minute consultation: Recognition of sickle cell crises in the paediatric emergency department
Children with sickle cell disease can develop life-threatening and painful crises that require prompt assessment and efficient management by healthcare professionals in the emergency or acute care setting. Due to migration patterns and improved survival rates in high-prevalence countries, there is an increased tendency to encounter these patients across the UK. These factors warrant regular revisions in sickle cell crisis management, along with education for medical personnel and patients to improve clinical care and patient management. The focus of this article is on the initial assessment and management of acute paediatr...
Source: Archives of Disease in Childhood - Education and Practice - May 19, 2022 Category: Pediatrics Authors: Patel, S., Dadnam, C., Hewitson, R., Thakur, I., Morgan, J. Tags: Best practice and Fifteen Minute Consultations Best practice and Fifteen-minute consultations Source Type: research

Polyuria, Polydipsia and Possible Diabetes Insipidus?
Discussion The body is smart. It has mechanisms for maintaining balances within the body in a closely controlled manner but allows for a variety of states. This is very true for fluid balance in the body which is highly controlled between almost all of the major body organ systems. When fluid is low, the sensor sends signals for us to drink, and conserves fluid until we can. When the fluid is high, the kidneys excrete the excess and sends signals not to drink. Usually it works very well. While there are many pathological states that can cause polyuria and/or polydipsia, the most common reason is excessive fluid intake beca...
Source: PediatricEducation.org - March 7, 2022 Category: Pediatrics Authors: Pediatric Education Tags: Uncategorized Source Type: news

Hydroxyurea Use Associated with Nonverbal and Executive Skills in Sickle Cell Anemia
ConclusionsThese results suggest that treatment with HU may not only reduce physical symptoms, but may also provide potential benefit to cognition in children with SCA, particularly in regard to attention/executive functioning and nonverbal skills. Replication with larger samples and longitudinal studies are warranted.
Source: Journal of Pediatric Psychology - March 11, 2021 Category: Pediatrics Source Type: research

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