scholarly journals Comparison of a Portable and Non-Portable Ultrasound Machine in the Evaluation of Children with Sickle Cell Disease: A Pilot Study

2021 ◽  
Vol 11 (4) ◽  
pp. 735-740
Author(s):  
Mohammed Alsaadi ◽  
Joanne Widdup ◽  
Mohammed Aslam ◽  
Subarna Chakravorty
Keyword(s):  
Pilot Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Stroke Risk ◽  
Screening Program ◽  
Cell Disease ◽  
Ultrasound Machine ◽  
Increased Risk ◽  
Flow Phantom ◽  
Risk Categorization

Background: Transcranial color Doppler (TCCD) ultrasound is used to identify children with sickle cell disease (SCD) at high risk of developing stroke. There is anecdotal evidence to suggest that different ultrasound equipment can give different blood flow velocities. The purpose of this study was to compare two different TCCD ultrasound machines. Methods and Results: A flow phantom was used to compare PSV measurements from a Philips IU-22 and Zonare Z-One ultrasound machine. Twenty-five children with SCD (aged between 2 and 16 years) attending the outpatient clinic at St. Mary’s Hospital, Imperial College Healthcare NHS Trust, as part of the NHS Sickle Cell & Thalassaemia (SC&T) screening program were studied. The two ultrasound machines compared the TAMM velocities in the middle cerebral artery and stroke risk categorization. PSV measurements using a flow phantom were underestimated by Philips IU-22 (31%) and Zonare Z-One (53%). TAMM velocities varied considerably between machines, with a poor agreement in stroke risk categorization. As a result, three children identified at increased risk of stroke by Philips IU-22 were not identified by Zonare Z-One. Conclusion: Two ultrasound machines were found to underestimate PSV using a flow phantom. The two ultrasound machines were shown to positively correlate, and this was statistically significant. However, there was variation in the TAMM velocities recorded by the machines which resulted in the different categorization of the stroke risk of a small number of the subjects. This pilot study confirms the feasibility and clinical significance of this investigation.

Computerized cognitive training in pediatric sickle cell disease: A randomized controlled pilot study.

2020 ◽  
Vol 8 (4) ◽  
pp. 390-401 ◽  
Author(s):  
Taryn M. Allen ◽  
Lindsay M. Anderson ◽  
Samuel M. Brotkin ◽  
Jennifer A. Rothman ◽  
Melanie J. Bonner
Keyword(s):  
Pilot Study ◽  
Sickle Cell Disease ◽  
Cognitive Training ◽  
Sickle Cell ◽  
Cell Disease ◽  
Computerized Cognitive Training ◽  
Randomized Controlled ◽  
Pediatric Sickle Cell Disease

Lead Neuropathy and Sickle Cell Disease

PEDIATRICS ◽  
1974 ◽  
Vol 54 (4) ◽  
pp. 438-441
Author(s):  
Gerald Erenberg ◽  
Steven S. Rinsler ◽  
Bernard G. Fish
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Lead Poisoning ◽  
Cell Disease ◽  
Hemoglobin S ◽  
Increased Risk ◽  
Rare Manifestation ◽  
Poisoning In Children

Four cases of lead neuropathy in children with hemoglobin S-S or S-C disease are reported. Neuropathy is a rare manifestation of lead poisoning in children, and only ten other cases have been well documented in the pediatric literature. The last previous case report of lead neuropathy was also in a child with hemoglobin S-S disease. The neuropathy seen in the children with sickle cell disease was clinically similar to that seen in the previously reported cases in nonsicklers, but differed in both groups from that usually seen in adult cases. It is, therefore, postulated that children with sickle cell disease have an increased risk of developing neuropathy with exposure to lead. The exact mechanism for this association remains unknown, but in children with sickle cell disease presenting with symptoms or signs of peripheral weakness, the possibility of lead poisoning must be considered.

scholarly journals VEGF Promoter Region 18-bp Insertion-Deletion Polymorphism in Sickle Cell Disease Patients with Microalbuminuria: A Pilot Study

2018 ◽  
Vol 35 (2) ◽  
pp. 278-283
Author(s):  
Dnyanesh B. Amle ◽  
Rachana L. Patnayak ◽  
Varsha Verma ◽  
Gajendra Kumar Singh ◽  
Vijaylakshmi Jain ◽  
...  
Keyword(s):  
Pilot Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Promoter Region ◽  
Cell Disease ◽  
Deletion Polymorphism

scholarly journals A pilot study of the effect of atorvastatin on endothelial function and albuminuria in sickle cell disease

2019 ◽  
Vol 94 (11) ◽  
Author(s):  
Kenneth I. Ataga ◽  
David Wichlan ◽  
Laila Elsherif ◽  
Vimal K. Derebail ◽  
Adane F. Wogu ◽  
...  
Keyword(s):  
Pilot Study ◽  
Sickle Cell Disease ◽  
Endothelial Function ◽  
Sickle Cell ◽  
Cell Disease

scholarly journals A case of severe multi-system disease in a sickle cell SC patient caused by parvovirus B19 infection

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Mohamed Almuqamam ◽  
◽  
Swetha Madhavarapu ◽  
Nataly Apollonsky ◽  
◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Acute Infection ◽  
Organ Injury ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Increased Risk ◽  
Parvovirus B19 Infection

Sickle Cell Disease (SCD) is an inherited hemoglobinopathy, which results in production of abnormal hemoglobin S. HbSC disease is a variant of SCD, which shares a similar clinical complication profile to HbSS disease, but often thought to be a milder condition. In patients with SCD, Hb S in deoxygenated state undergoes polymerization, leading to hemolysis, vaso-occlusive events, and eventually end-organ damage. Among other complications in patients with SCD is increased risk of complications caused by parvovirus B19. We present a case of a 14-year-old female with HbSC disease who presented to the emergency room with complaint of abdominal pain and found to have splenic sequestration. Splenic sequestration progressed rapidly, Hemoglobin (hb) dropped to 4.6 g/dl and acute chest syndrome (ACS) developed. She was treated following the ACS protocol, received 4 units of Packed Red Blood Cells (PRBC) and subsequently underwent a single volume PRBC exchange transfusion. Considering her unusual presentation, with severe ARDS from alveolar hemorrhage requiring mechanical ventilation and multi-organ injury, several autoimmune and infectious conditions with a cytokine storm component including COVID-19 disease, were considered. Results of viral testing revealed parvovirus B19 IgM antibodies signifying an acute infection. She fully recovered with supportive care and was discharged home. Multisystem involvement simulating connective tissue disorders or malignancies with acute parvovirus B19 infection has been reported and is considered extremely rare. To our knowledge, there were no reports of pediatric patients with SC disease presenting with splenic sequestration and ACS in the setting of parvovirus B19 multisystem disease. Keywords: sickle cell disease; acute respiratory distress syndrome; acute chest syndrome; parvovirus B19.

scholarly journals Review of Sickle Cell Disease and Spinal Pathology

2018 ◽  
Vol 9 (7) ◽  
pp. 761-766 ◽  
Author(s):  
Hayeem L. Rudy ◽  
David Yang ◽  
Andrew D. Nam ◽  
Woojin Cho
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vertebral Osteomyelitis ◽  
Compression Fracture ◽  
Symptomatic Therapy ◽  
Cell Disease ◽  
Blood Disorder ◽  
Increased Risk ◽  
Adequate Hydration ◽  
Surgical Treatments

Study Design:Sickle cell disease (SCD) is a relatively common blood disorder that has profound implications on the musculoskeletal system and particularly the spine; however, there is a paucity of data in the literature discussing this important topic.Objectives:(1) To elucidate common spinal pathologies affecting patients with SCD, as well as the medical and surgical treatments available for these patients. (2) To discuss indications for surgical management of spinal complications of SCD and important for orthopedic surgeons when taking patients with SCD to the operating room.Methods:A narrative review of the literature was performed.Results:Patients with SCD have a significantly higher risk of developing spinal pathologies including vertebral osteomyelitis, compression fracture, vertebral vaso-occlusive crises, and osteoporosis, among others. In addition, patients with sickle cell disease are particularly susceptible to developing perioperative and post-operative complications including surgical site infection, implant malfunction, and vertebral body compression fracture. Postoperatively patients with SCD are prone to developing complications and adequate hydration is necessary in order to reduce complications of SCD.Conclusions:Several spinal pathologies may arise secondary to SCD and distinguishing these pathologies from one another may be challenging due to similarities in symptoms and inflammatory markers. Although most patients with SCD can benefit from conservative treatment involving rest, symptomatic therapy, antibiotic therapy, and/or orthosis, surgical intervention may be indicated in certain cases. It is preferable to avoid surgery in patients with SCD due to an increased risk of complications such as wound infection and vaso-occlusive crisis.

Screening for Obstructive Sleep Apnea in Children With Sickle Cell Disease: A Pilot Study

2020 ◽  
Author(s):  
Andrew J. Maroda ◽  
Matthew N. Spence ◽  
Stephen R. Larson ◽  
Jeremie H. Estepp ◽  
M. Boyd Gillespie ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Pilot Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Obstructive Sleep

scholarly journals Evaluation of Technical Issues in a Pilot Multicenter Newborn Screening Program for Sickle Cell Disease

2018 ◽  
Vol 5 (1) ◽  
pp. 2
Author(s):  
Maddalena Martella ◽  
Giampietro Viola ◽  
Silvia Azzena ◽  
Sara Schiavon ◽  
Andrea Biondi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Newborn Screening ◽  
Molecular Analysis ◽  
Sickle Cell ◽  
Screening Program ◽  
Globin Gene ◽  
False Negative ◽  
Confirmatory Test ◽  
Cell Disease ◽  
Technical Issues

A multicenter pilot program for universal newborn screening of Sickle cell disease (SCD) was conducted in two centres of Northern Italy (Padova and Monza). High Performance Liquid Chromatography (HPLC) was performed as the first test on samples collected on Guthrie cards and molecular analysis of the β-globin gene (HBB) was the confirmatory test performed on the HPLC-positive or indeterminate samples. 5466 samples of newborns were evaluated. Of these, 5439/5466 were submitted to HPLC analysis and the molecular analysis always confirmed in all the alteration detected in HPLC (62/5439 newborns); 4/5439 (0.07%) were SCD affected, 37/5439 (0.68%) were HbAS carriers and 21/5439 (0.40%) showed other hemoglobinopathies. Stored dried blood spots were adequate for HPLC and β-globin gene molecular analysis. Samples were suitable for analysis until sixteen months old. A cut-off of A1 percentage, in order to avoid false negative or unnecessary confirmation tests, was identified. Our experience showed that several technical issues need to be addressed and resolved while developing a multicenter NBS program for SCD in a country where there is no national neonatal screening (NBS) program for SCD and NBS programs occur on a regional basis.

scholarly journals Perceptions and Practice of Early Diagnosis of Sickle Cell Disease by Parents and Physicians in a Southwestern State of Nigeria

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Oladele Simeon Olatunya ◽  
Adefunke Olarinre Babatola ◽  
Ezra Olatunde Ogundare ◽  
Babatunde Ajayi Olofinbiyi ◽  
Olubunmi Adeola Lawal ◽  
...  
Keyword(s):  
Genetic Counseling ◽  
Sickle Cell Disease ◽  
Early Diagnosis ◽  
Newborn Screening ◽  
Sickle Cell ◽  
Screening Program ◽  
Universal Coverage ◽  
Cell Disease ◽  
Cross Sectional ◽  
Southwestern State

Background. Early sickle cell disease (SCD) diagnosis has shown promise in combating SCD in many countries. The aim of this study was to assess the practice and perception of early SCD diagnosis among a group of parents and physicians in Nigeria. Patients and Methods. This was a cross-sectional descriptive study conducted to assess the opinions and practice of early diagnosis of SCD among 135 physicians caring for SCD patients and 164 mothers of children with SCD in a southwestern state of Nigeria. Results. Most physicians 132 (97.8%) were aware of prenatal SCD diagnosis, but only 51 (37.8%) would recommend it. Most physicians 129 (95.6%) routinely recommend premarital SCD genetic counseling and testing, and 89 (65.1%) were aware of the national government newborn screening program but lesser proportion 75 (55.6%) were willing to recommend it. Amongst the mothers, 154 (94%) and 158 (96%) had encountered genetic counseling for SCD and were willing to offer newborn screening to their children, respectively. On the contrary, fewer mothers 42 (25%) were aware of prenatal SCD diagnosis, 28 (17%) were willing to partake in it, and 44 (26%) were undecided. There were discrepancies in the willingness by physicians to practice early SCD diagnosis and its uptake by mothers (p<0.0001). The commonest reason given by both the physicians and mothers for not practicing SCD prenatal diagnosis was the high cost of the procedure. Conclusion. The perceptions and practice of early SCD diagnosis was suboptimal in the study locality. Scaling up awareness and universal coverage are required.

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