Neurodevelopmental Outcomes in Preterm Children with Sickle Cell Disease

2021 ◽  
pp. 1-11
Author(s):  
Sarah E. Bills ◽  
Jeffrey Schatz ◽  
Erin Hunt ◽  
Sreya Varanasi ◽  
Julia Johnston ◽  
...  
Keyword(s):  
Preterm Birth ◽  
High Risk ◽  
Sickle Cell Disease ◽  
Cognitive Functioning ◽  
Sickle Cell ◽  
Full Term ◽  
Neurocognitive Deficits ◽  
Cell Disease ◽  
Neurodevelopmental Outcomes ◽  
Increased Risk

ABSTRACT Objectives: To explore the combined effect of pediatric sickle cell disease (SCD) and preterm birth on cognitive functioning. Methods: Cognitive functioning was examined in children ages 6–8 with high risk SCD genotypes born preterm (n = 20) and full-term (n = 59) and lower risk SCD genotypes/no SCD born preterm (n = 11) and full-term (n = 99) using tests previously shown to be sensitive to SCD-related neurocognitive deficits. Factorial ANOVAs and log linear analyses were conducted to examine the relationship between SCD risk, preterm birth status, and cognitive outcomes. Continuous scores were examined for specific tests. Children were categorized as having an abnormal screening outcome if at least one cognitive score was ≥1.5 standard deviations below the population mean. Results: Children with elevated risk due to high risk SCD and preterm birth performed worse than other groups on a test of expressive language but not on tests that emphasize processing speed and working memory. There was a three-way interaction between preterm status, SCD risk, and abnormal screening outcome, which was largely driven by the increased likelihood of abnormal cognitive scores for children with high risk SCD born preterm. Conclusions: The combination of SCD and preterm birth may confer increased risk for language deficits and elevated rates of abnormal cognitive screenings. This suggests that neurodevelopmental risk imparted by comorbid SCD and preterm birth may manifest as heterogenous, rather than specific, patterns of cognitive deficits. Future studies are needed to clarify the domains of cognitive functioning most susceptible to disease-related effects of comorbid SCD and preterm birth.

scholarly journals Responsive Parenting Behaviors and Cognitive Function in Children With Sickle Cell Disease

2019 ◽  
Vol 44 (10) ◽  
pp. 1234-1243 ◽  
Author(s):  
Janet Yarboi ◽  
Kemar V Prussien ◽  
Heather Bemis ◽  
Ellen Williams ◽  
Kelly H Watson ◽  
...  
Keyword(s):  
Cognitive Function ◽  
Sickle Cell Disease ◽  
Cognitive Functioning ◽  
Sickle Cell ◽  
Parental Stress ◽  
Cell Disease ◽  
Targeted Interventions ◽  
Responsive Parenting ◽  
Increased Risk ◽  
Cognitive Problems

Abstract Objective Children with sickle cell disease (SCD) are at increased risk for cognitive impairment as a result in part from biological characteristics of the disease; however, limited research has explored possible social and contextual factors associated with risk for cognitive problems. The primary aim of the present study was to examine the relation between children’s cognitive functioning and responsive parenting, a potentially important contextual factor in children with SCD, accounting for family socioeconomic disadvantage, child disease severity, and caregivers’ perceived stress. Methods Forty-eight children completed standardized cognitive assessments and caregivers provided self-reports of general and disease-related stress. Parent–child dyads completed a video recorded puzzle-solving task and observed parenting was quantified using two coding systems. Bivariate Pearson correlations were used to assess preliminary hypotheses, and linear multiple regression analyses were used to assess the primary hypothesis. Results Results suggested that increased levels of parental stress were related to fewer observations of responsive parenting and provided evidence of an association between children’s cognitive function and responsive parenting. Specifically, increased disease-related parent stress and reduced parental use of expansive language were associated with significantly lower cognitive functioning in children with SCD. Conclusions Findings suggest that social environmental factors along with disease characteristics are sources of risk for cognitive problems with children with SCD. Further, these findings highlight the need to develop targeted interventions for parents of children with SCD to decrease levels of stress and enhance parenting skills, with the aim improving cognitive functioning in youth.

Transcranial Doppler Measures In Patients with Sickle Cell Disease at High Risk for Stroke and Receiving Hydroxyurea: The HyRetro Ancillary Study.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1620-1620 ◽  
Author(s):  
Thomas Adamkiewicz ◽  
Nadine Odo ◽  
Abdullah Kutlar ◽  
Janet L Kwiatkowski ◽  
Robert J Adams
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Hemoglobin F ◽  
Transfusion Therapy ◽  
Ancillary Study ◽  
Increased Risk

Abstract Abstract 1620 Children with sickle cell disease (SCD) and increased Transcranial Doppler (TCD) sonography velocity measures are at increased risk for stroke. Although chronic transfusion decreases this risk ten fold, this form of therapy is burdensome, and includes risk of iron overload. Although it has been established that the risk of stroke is still present even after 30 months of transfusion therapy, the total length of transfusion therapy required is not known. Hydroxyurea (HU) therapy is effective in preventing SCD complication, although its effect in preventing SCD central nervous system complications is less clear and a matter of current investigation. During one of the clinical trials (STOP2), an ancillary study was conducted to examine TCD velocities in 76 patients with SCD receiving hydroxyurea (HU) for a variety of indications. TCD measures were defined as the highest time-averaged maximum mean velocity of one of 8 measures (from the following cerebral arteries: LMCAVM, LM1VM, LBIFVM, LDICAVM, RMCAVM, RM1VM, RBIFVM and RDICAVM), as per STOP protocol. Results from 10 patients at high risk for stroke (defined as one or more TCD >=200 cm/sec) were evaluated for the present analysis. Average age at start of HU was 10.7 +/− 3.2 standard deviation (SD) years, 6 were female. Eight did not receiving chronic transfusion in STOP (randomized observation arm STOP n=4; observed non randomized in STOP n=4), and 2 were on transfusion (randomized transfusion arm STOP n=1; STOP/STOP2 with cross over n=1). Reasons for HU therapy included primary stroke prevention (n=6); secondary stroke prevention (TIA n=1; overt ischemic stroke n=1), vaso-occlusive or acute chest episodes (n=2). Averaged HU dose (available in 8) was 15 +/− 3 SD mg/kg. Averaged measures, off and on HU for each patient, were used to calculate means. Averaged hematological indices on treatment were as follows: white blood cell count 10.2 +/− 1.8 SD × 10^3/mm^3, hemoglobin 8.1 +/− 0.7 gm/dL; Mean Corpuscular Volume 105.6 +/− 6.7 cu μm; reticulocyte count 11.1 +/− 2.3 %. Averaged hemoglobin F, available in 8, was 16.1 +/− 5.7 %. Results are in table: Table Groups Treatment Months TCDs (n) cm/sec Not transfused in STOP (n=8) Pre HU 44+/−23 5.3+/−2.5 203+/−28 HU 33+/−24 5.0+/−3.6 179+/−48* Transfused in STOP (n=2) Pre HU 64+/−19 10.5+/−0.7 160+/−0.5 HU 19+/−7 4.5+/−0.7 180+/−1.1 * Wilcoxon signed-rank test p=0.008 One patient receiving HU for secondary stoke prevention suffered an overt stroke. This patient had a first overt stroke 24 months prior to start of HU therapy. MRI showed right frontal watershed and bilateral lacunar infarcts. Severe stenosis of the left MCA was noted. Patient had a repeat stroke 13 months after start of HU. MRI showed ischemia with watershed distribution of the left frontal lobe, stenosis of A2 and occlusion of A1 segments. Three TCD before and two after start of HU were all > 220 cm/sec. Overall, decreasing TCD velocities were noted in 60% prior to HU (STOP transfusion, n=2) and in 50% on HU (STOP transfusion, n=0). In conclusion, TCD velocities decreased significantly in high risk patients receiving HU, that were not transfused in STOP. However, these results require cautious interpretation, as numbers of patients are small, and length of observation varied. Patients with very high TCD measures remain at risk. Further studies may elucidate if there is a role for HU in patients with abnormal TCDs. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Sickle Cell Anemia Presenting with Vaso-Occlusive Pain: Should We Screen for COVID-19?

2021 ◽  
pp. 1-4
Author(s):  
Mohammad Ali ◽  
Lina Okar ◽  
Nabil E. Omar ◽  
Jabeed Parengal ◽  
Ashraf Soliman ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Signs And Symptoms ◽  
Risk Groups ◽  
Position Statement ◽  
Interesting Case ◽  
International Federation ◽  
Cell Disease ◽  
The World

Despite the widespread of coronavirus disease-19 (CO­VID-19) infection around the world, there are very scarce reported literature about the care of patients with a known diagnosis of hemoglobin disorders such as sickle cell disease (SCD) or thalassemia and confirmed COVID-19 infection. Thalassemia International Federation issued a position statement to include patients with thalassemia and SCD among the high-risk groups of patients. Here, we present an interesting case of a 42-year-old patient know to have SCD presenting with Vaso-occlusive (VOC) pain episode in the absence of COVID-19 signs and symptoms, who tested positive for COVID-19 infection and had a smooth recovery. This case highlights the importance of screening SCD patients presenting with VOC-related events even in the absence of COVID-19 signs and symptoms.

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 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 Ischemic Stroke in Children and Young Adults with Sickle Cell Disease (SCD) in the Post-STOP Era

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 68-68 ◽  
Author(s):  
Janet L. Kwiatkowski ◽  
Julie Kanter ◽  
Heather J. Fullerton ◽  
Jenifer Voeks ◽  
Ellen Debenham ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Clinical Series ◽  
The Mean

Abstract Background: The Stroke Prevention Trial in Sickle Cell Anemia (STOP) and Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) established routine transcranial Doppler ultrasound (TCD) screening with indefinite chronic red cell transfusions (CRCT) for children with abnormal TCD as standard of care. To identify children at high-risk of stroke, annual TCD screening is recommended from ages 2 to 16 years, with more frequent monitoring if the result is not normal. A reduction in stroke incidence in children with SCD has been reported in several clinical series and analyses utilizing large hospital databases when comparing rates before and after the publication of the STOP study in 1998. We sought to determine the rate of first ischemic stroke in a multicenter cohort of children who had previously participated in the STOP and/or STOP 2 trials and to determine whether these strokes were screening or treatment failures. Subjects and Methods: Between 1995 and 2005, STOP and STOP 2 (STOP/2) were conducted at 26 sites in the US and Canada. These studies included 3,835 children, ages 2 to 16 y with SCD type SS or S-beta-0-thalassemia. Participation in STOP/2 ranged from a single screening TCD to randomization. STOP 2 also had an observational arm for children on CRCT for abnormal TCD whose TCD had not reverted to normal. The Post-STOP study was designed to follow-up the outcomes of children who participated in one or both trials. 19 of the 26 original study sites participated in Post-STOP, contributing a total of 3,539 (92%) of the STOP/2 subjects. After exit from STOP/2, these children received TCD screening and treatment according to local practices. Data abstractors visited each clinical site and obtained retrospective data from STOP/2 study exit to 2012-2014 (depending on site) including follow-up TCD and brain imaging results, clinical information, and laboratory results. Two vascular neurologists, blinded to STOP/2 status and prior TCD and neuroimaging results, reviewed source records to confirm all ischemic strokes, defined as a symptomatic cerebral infarction; discordant opinions were resolved through discussion. For the first Post-STOP ischemic stroke, prior TCD result and treatment history subsequently were analyzed. Results: Of the 3,539 subjects, follow-up data were available for 2,850 (81%). Twelve children who had a stroke during STOP or STOP2 were excluded from these analyses resulting in data on 2,838 subjects. The mean age at the start of Post-STOP was 10.5 y and mean duration of follow-up after exiting STOP/2 was 9.1 y. A total of 69 first ischemic strokes occurred in the Post-STOP observation period (incidence 0.27 per 100 pt years). The mean age at time of stroke was 14.4±6.2 (median 13.8, range 3.5-28.9) y. Twenty-five of the 69 patients (36%) had documented abnormal TCD (STOP/2 or Post-STOP) prior to the stroke; 15 (60%) were receiving CRCT and 9 (36%) were not (treatment data not available for 1 subject). Among the 44 subjects without documented abnormal TCD, 29 (66%) had not had TCD re-screen in the Post-STOP period prior to the event; 7 of these 29 (24%) were 16 y or older at the start of Post-STOP, which is beyond the recommended screening age. Four of the 44 (9%) patients had inadequate TCD in Post-STOP (1 to 10.7 y prior to event). Six (14%) had normal TCD more than a year before the event (1.2 - 4 y); all but one of these children were younger than 16 y at the time of that TCD. Only 5 (11%) had a documented normal TCD less than 1 year prior to the event. Conclusions: In the Post-STOP era, the rate of first ischemic stroke was substantially lower than that reported in the Cooperative Study of Sickle Cell Disease, prior to implementation of TCD screening. Many (39%) of the Post-STOP ischemic strokes were associated with a failure to re-screen according to current guidelines, while only 11% occurred in children who had had recent low-risk TCD. Among those known to be at high risk prior to stroke, treatment refusal or inadequate treatment may have contributed. While TCD screening and treatment are effective at reducing ischemic stroke in clinical practice, significant gaps in screening and treatment, even at sites experienced in the STOP protocol, remain to be addressed. Closing these gaps should provide yet further reduction of ischemic stroke in SCD. Disclosures No relevant conflicts of interest to declare.

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.

High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease

1991 ◽  
Vol 118 (3) ◽  
pp. 377-382 ◽  
Author(s):  
Winfred C. Wang ◽  
Edward H. Kovnar ◽  
Ina L. Tonkin ◽  
Raymond K. Mulhern ◽  
James W. Langston ◽  
...  
Keyword(s):  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Recurrent Stroke ◽  
Cell Disease ◽  
Transfusion Therapy

Sickle cell trait and the risk of venous thromboembolism among blacks

Blood ◽  
2007 ◽  
Vol 110 (3) ◽  
pp. 908-912 ◽  
Author(s):  
Harland Austin ◽  
Nigel S. Key ◽  
Jane M. Benson ◽  
Cathy Lally ◽  
Nicole F. Dowling ◽  
...  
Keyword(s):  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Odds Ratio ◽  
Sickle Cell Trait ◽  
Coagulation System ◽  
Cell Disease ◽  
Hemoglobin C ◽  
Increased Risk ◽  
S Allele

Abstract People with sickle cell disease have a chronically activated coagulation system and display hemostatic perturbations, but it is unknown whether they experience an increased risk of venous thromboembolism. We conducted a case–control study of venous thromboembolism that included 515 hospitalized black patients and 555 black controls obtained from medical clinics. All subjects were assayed for hemoglobin S and hemoglobin C genotypes. The prevalence of the S allele was 0.070 and 0.032 for case patients and controls, respectively (P < .001). The odds that a patient had sickle cell trait were approximately twice that of a control, indicating that the risk of venous thromboembolism is increased approximately 2-fold among blacks with sickle cell trait compared with those with the wild-type genotype (odds ratio = 1.8 with 95% confidence interval, 1.2-2.9). The odds ratio for pulmonary embolism and sickle cell trait was higher, 3.9 (2.2-6.9). The prevalence of sickle cell disease was also increased among case patients compared with controls. We conclude that sickle cell trait is a risk factor for venous thromboembolism and that the proportion of venous thromboembolism among blacks attributable to the mutation is approximately 7%.

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