scholarly journals Optimal Hemoglobin Level for Pediatric Sickle Cell Patients Who Undergo Adenotonsillectomy

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4938-4938 ◽  
Author(s):  
Zhongbo Hu ◽  
Simone Megan Chang ◽  
Ramzi Younis ◽  
Ofelia A. Alvarez

Abstract Background:The prevalence of obstructive sleep apnea syndrome (OSA) in children with sickle cell disease (SCD) is higher than in the general pediatric population. Adenotonsillectomy involves significantly increased risks in patients with SCD because of the need for general anesthesia,which could induce a vaso-occlusive event. Various preoperative regimens have been suggested to reduce peri-operative risks for SCD patients. Objective: We retrospectively reviewed the perioperative management for pediatric SCD patients undergoing adenotonsillectomy. We sought to identify the optimal preoperative hemoglobin (Hb) level for patients to have lowest risk of post-operative complications. Methods: Children between 1 and 18 years old with SCD including HbSS, HbSC, HbS beta thalassemia (Sβ0 and Sβ+) who underwent adenotonsillectomy from 2007 to 2017 were identified at Holtz Children's Hospital. Patients without these diseases who underwent the procedures were collected as a control. The study was approved by the University of Miami Institutional Review Board (IRB). De-identified data were exported to GraphPad Prism version 5.02 to perform the statistical analyses. Means were analyzed by student t test, rates by Chi-squared test. p < 0.05 is considered statistically significant. Results: Thirty-four patients with SCD (mean 7.1 yrs) and 145 controls (mean 7.0 yrs) were identified with adenotonsillectomy (see Table 1). SCD patients had significant longer hospital stay (2.6 versus 0.9 days, p< 0.001), and higher postoperative complication rates than the control group (20.6% vs 4.0%, p= 0.02). Most of the sickle cell patients' complications were hypoxemia (5 out of 7), except one supraventricular tachycardia and one headache. Four out of 7 patients with pretransfusion (pretrx) Hb level above 10 g/dL received manual exchange transfusion to keep Hb over 10 g/dL. None had complication or PICU stay. When patients' pretrx Hb levels was 9-10 g/dL, 5/8 received transfusion without significant complications, with the goal of not increasing post transfusion Hb over 11.5 g/dL. Most patients with pretrx Hb level 7-9 g/dL received simple blood transfusion. For patients with preoperative Hb level 9-11.5 g/dL, the PICU transfer rate was significantly lower than ones with Hb <9 or >= 11.5 g/dL (11.5% vs 60%, p=0.04; see Figure 1). Complication rates were also lower in the Hb 9-11.5 g/dL group, but without statistical difference (19.2% vs 40%, p=0.3). Data is limited by small sample size in the SCD group. Conclusion(s): We concluded that for pediatric SCD patients planning for adenotonsillectomy, it is better to keep the posttransfusion Hb level at least above 9 mg/dL but less than 11.5 g/dL before procedure to avoid significant post operational complications. If pretransfusion Hb level is above 9 mg/dL, exchange transfusion by partial phlebotomy or simple transfusion (not to exceed 11.5 g/dL) may be considered. Disclosures No relevant conflicts of interest to declare.

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3472-3472
Author(s):  
Sarah Agamah

Background Sickle Cell Disease (SCD) is the most common lethal genetic blood disease in the world, impacting approximately 100,000 people in the US, and about 25 million people worldwide (WHO, CDC 2019). Some literature refers to the SCD patients' barriers to appropriate care as "biopsychospiritual," indicating the complexity of the disease, and hence, call for a multi-dimensional approach to care (Adegbola et al., 2012). Considering the role of energy, protein and Vitamin D deficiency in SCD patients, a multi-dimensional approach to care that includes nutritional supplementation is worth investigating (Hyacinth, Gee and Hibbert 2010). There is limited research that illuminates the relationship between nutrition, food insecurity and health outcomes of those with SCD. Patients with SCD experiencing challenges of undernutrition are more prone to growth retardation in childhood, disease exacerbation, impaired immune function, and poor ulcer healing among other complications (Mandese et al., 2015). In Chicago, Illinois on the South and West Side where many SCD patients live, it is estimated that 35.1%-57.8% of people are food insecure (Gunderson, Dewey, Crumbaugh,Kato & Engelhard, 2016). This study aims to better understand food insecurity for SCD patients at the University of Illinois Hospital and Health Science Systems (UIHHSS) in Chicago, the largest adult sickle cell center in the Midwest. Physicians are uniquely positioned to address health inequities among their patients when they practice patient centered-care (Law, Leung, Veinot, Miller & Mylopoulos, 2016). Research shows that healthcare providers who are aware of external factors such as food insecurity, can be better advocates for their patients, and help them find ways to gain access to healthy foods (Nesbitt and Palomarez, 2016). Methods An exploratory study involving data collection of patient demographics, food consumption, and ability to access food was conducted. All patients at the Sickle Cell Clinic at UIHHSS who were interested in participating received a full explanation of the purpose of the study.Twenty-seven adult patients (19 female, 8 male) were recruited in the Sickle Cell Center waiting room. Food intake by patients with SCD was documented using the validated Food Insecurity Experience Scale (FIES) from the Food and Agriculture Organization (FAO). Participants self-administered the FIES questionnaire, followed by a one-on-one discussion with the interviewer. Qualitative data gathered from the follow-up interviews was used to investigate themes and provide clarity. Demographic information was collected to supplement the screening tool, as it is not a sufficient stand alone measure. Patient zip codes were referenced with the Distressed Community Index (DCI), a comparative measure of community well-being using census bureau data from 2012-2016. Outcomes Results of the analysis indicated the nutritional status of sickle cell patients and identified barriers to food access especially economic factors. Of the twenty-seven patients interviewed, 40% screened moderate and 22% screened as severe for food insecurity. The remaining 48% screening negative for food insecurity, but 80% of all patients did not consume the daily recommended dietary allowance of vegetables and fruits, and 44% worried about having enough money for food, citing that the cost of healthy food was a barrier to eating healthy. Based on zip code, 80% of patients live in a high DCI zone. Discussion and Limitations This study utilizes convenience sampling with a small sample size, which limits external validity. Conclusions from the data should not be made, but rather used to design larger confirmatory studies. The information could aid in aligning undernourished patients with social support and provide potential insight for clinicians into the management of patients with SCD. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 13-13
Author(s):  
Susumu Inoue ◽  
Christina Anagonye

Background Thrombosis is one of the most common complications described in patients with sickle cell disease. However, little is known about the prevalence, variety and severity of thrombus particularly in sickle cell patients hospitalized with vaso-occlusive crisis. Methods We made a retrospective chart review of patients (age 21 years or older) admitted with sickle cell disease at Hurley Medical Center (Flint, Michigan) and was diagnosed with a thrombotic event between April 2012 and April 2020. To obtain a logistic model, we identified all patients with the the final discharge diagnosis of thrombosis (ICD-9 or ICD-10 code indicating any thrombosis, such as DVT, arterial thrombosis, catheter-related thrombus, pulmonary embolism, and stroke), combined with the diagnosis of sickle cell disease. Findings 37/137 (27%) sickle cell patients were found to have had at least one thrombotic event during their hospitalization. As predicted, patients with sickle cell disease appeared to have very high frequency of thrombotic complications, though we lack a control group to compare with. Genotypes of sickle cell disease was also measured in this study. Patients with the Hb-SS disease had a much higher rate of this complication(25/68=37%) compared with those with Hb SC disease patients (12/49=24%). There were no thrombotic events reported in patients with Hb S/beta thalassemia+, or 0 patients or in patients with Hb SS with HPFH. Interpretation The risk of a thromboembolic event in patients with SCD is high, Sickle cell anemia is a procoagulant condition, which is a risk factor for thrombosis. More research is required to determine if preventive modalities could reduce thrombotic risk. Disclosures No relevant conflicts of interest to declare.


2014 ◽  
pp. 77-86
Author(s):  
Anh Tien Hoang ◽  
Thi Y Nhi Nguyen ◽  
Luu Trinh Nguyen ◽  
Thi Hong Diep Phan ◽  
Huu Cat Nguyen ◽  
...  

Background : Sleep Apnea Syndrome (SAS) is a cause of hypertension, increasingcardiovascular risk and cardiovascular disease such as stroke, coronary artery disease, myocardial infarction, arrhythmias, heart failure, increasing the risk of death in patients with heart disease, independent of other causative factors. So far, in Vietnam there are very few studies on Obstructive Sleep Apnea Syndrome (OSAS) and cardiovascular risk factors . Self-making SASD07 is trustly for detecting OSAS with statistical significiant in comparision with StarDustII (gold criteria). Subjects and Methods: Cross sectional study, comparision with control group: 136 peoples (68 in disease group and 68 in control group). Patients were parallelly measured with StarDustII and SASD07 to detect OSAS and find the corellation with cardiovascular risk factors. Results: There is a positive correlation between SBP with the severity of OSAS (r = 0.459, p < 0.001), positive correlation between DBP with the severity of OSAS (r = 0.352, p < 0.003). No statistically significant differences between severe OSAS and fasting blood glucose, cholesterol, HDL Cholesterol, Non - HDL Cholesterol, LDL Cholesterol and TG median (p > 0.05). There is a positive correlation between AHI and neck circumference (r = 0.511, p < 0.001), waist circumference (r = 0.585, p < 0.001), BMI (r = 0.380, p < 0.01). SASD07 diagnostic value of detecting OSAS compared with StarDustII have Kappa = 0.72, (standard error 0.06, p <0.001). Conclusion: The risk factors related to OSAS in our study is neck circumference, waist circumference, systolic blood pressure, diastolic blood pressure. SASD07 have a good value in diagnosing of OSAS compared with polysomnography StarDustII. Key words: Sleep Apnea Syndrome, cardiovascular risk factor, SASD07.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Aleksandra Rajewska-Rager ◽  
Monika Dmitrzak-Weglarz ◽  
Pawel Kapelski ◽  
Natalia Lepczynska ◽  
Joanna Pawlak ◽  
...  

AbstractMood disorders have been discussed as being in relation to glial pathology. S100B is a calcium-binding protein, and a marker of glial dysfunctions. Although alterations in the S100B expression may play a role in various central nervous system diseases, there are no studies on the potential role of S100B in mood disorders in adolescents and young adults . In a prospective two-year follow-up study, peripheral levels of S100B were investigated in 79 adolescent/young adult patients (aged 14–24 years), diagnosed with mood disorders and compared with 31 healthy control subjects. A comprehensive clinical interview was conducted which focused on clinical symptoms and diagnosis change. The diagnosis was established and verified at each control visit. Serum S100B concentrations were determined. We detected: lower S100B levels in medicated patients, compared with those who were drug-free, and healthy controls; higher S100B levels in a depressed group with a family history of affective disorder; correlations between age and medication status; sex-dependent differences in S100B levels; and lack a of correlation between the severity of depressive or hypo/manic symptoms. The results of our study indicate that S100B might be a trait-dependent rather than a state-dependent marker. Due to the lack of such studies in the youth population, further research should be performed. A relatively small sample size, a lack of exact age-matched control group, a high drop-out rate.


2021 ◽  
pp. 112067212110065
Author(s):  
Murat Serkan Songur ◽  
Yavuz Selim İntepe ◽  
Seray Aslan Bayhan ◽  
Hasan Ali Bayhan ◽  
Ender Şahin ◽  
...  

Purpose: In the present study we evaluate the corneal endothelium using specular microscopy in patients with obstructive sleep apnea syndrome (OSAS). Methods: The study included a total of 100 patients including 35 patients with mild OSAS, 34 patients with moderate OSAS and 31 patients with severe OSAS, and the right eyes of 30 patients as a control group. Patients were examined to exclude the possibility of ocular diseases. Cellular density in the cornea epithelium (cell/mm2), corneal thickness (µ), percentage of hexagonal cells (%) and the coefficient of variation were evaluated using a specular microscope. Results: Corneal thickness was significantly decreased in all OSAS groups when compared to the control group ( p = 0.002), while no significant difference was identified among the OSAS groups. The corneal endothelial cell density, percentage of hexagonal cells and coefficient of variation were significantly different between the OSAS groups and the control group ( p < 0.001). Conclusion: More significant impairments were noted in the corneal endothelium of the patients in the OSAS group than in the control group, and specular microscopy is in valuable in the follow-up and treatment of such patients.


2009 ◽  
Vol 24 (8) ◽  
pp. 521-525 ◽  
Author(s):  
H. Hustig ◽  
R. Onilov

AbstractObjectiveThe aim of our naturalistic-observational study was to determine the efficacy and utility of electroconvulsive therapy (ECT) in clinical population of individuals with schizophrenia where pharmacological response was suboptimal.MethodsThe cohort comprised 27 patients suffering from schizophrenia with refractoriness to antipsychotic agents and with severe, disabling symptoms. They only interventional assessing tool was clinical global impression (CGI-S) performed at the baseline and at the end of the treatment.ResultsThe administration of ECT resulted in overall clinical improvement reflected in CGI scales and descriptions in clinical notes. On 12 months follow-up period, 10 patients (37.1%) maintained improvement and were able to continue with pharmacological therapy only, suggesting its rekindling effect, especially with Clozapine. Conversely, 17 patients (62.9%) deteriorated and required an additional course of ECT to maintain improvement. In some cases, maintenance ECT treatment was required. The group who engaged in self-harming behaviour at baseline demonstrated they were more likely to relapse into psychosis at the end of the first course of ECT, their self-harming behaviour abated, especially when maintenance ECT was undertaken.ConclusionsAlthough limited by lack of control group, small sample size, heterogeneous symptom profiles and various concurrent neuroleptic agents, the ECT proved as valuable and safe augmentative procedure when unsatisfactory response to pharmacological interventions had been demonstrated prior to interventions. This effect was evident despite the chronicity of the illness.


Author(s):  
Li Wang ◽  
Yiwen Zhang ◽  
Jiajun Zhong ◽  
Yuan Zhang ◽  
Shuisheng Zhou ◽  
...  

Objective: The efficacy of mesenchymal stem cell (MSC) therapy in acetaminophen-induced liver injury has been investigated in animal experiments, but individual studies with a small sample size cannot be used to draw a clear conclusion. Therefore, we conducted a systematic review and meta-analysis of preclinical studies to explore the potential of using MSCs in acetaminophen-induced liver injury. Methods: Eight databases were searched for studies reporting the effects of MSCs on acetaminophen hepatoxicity. The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines were used. SYRCLE’s risk of bias tool for animal studies was applied to assess the methodological quality. A meta-analysis was performed by using RevMan 5.4 and STATA/SE 16.0 software. Results: Eleven studies involving 159 animals were included according to PRISMA statement guidelines. Significant associations were found for MSCs with the levels of alanine transaminase (ALT) (standardized mean difference (SMD) − 2.58, p < 0.0001), aspartate aminotransferase (AST) (SMD − 1.75, p = 0.001), glutathione (GSH) (SMD 3.7, p < 0.0001), superoxide dismutase (SOD) (SMD 1.86, p = 0.022), interleukin 10 (IL-10) (SMD 5.14, p = 0.0002) and tumor necrosis factor-α (TNF-α) (SMD − 4.48, p = 0.011) compared with those in the control group. The subgroup analysis showed that the tissue source of MSCs significantly affected the therapeutic efficacy (p < 0.05). Conclusion: Our meta-analysis results demonstrate that MSCs could be a potential treatment for acetaminophen-related liver injury.


2010 ◽  
Vol 14 (8) ◽  
pp. 1337-1344 ◽  
Author(s):  
Sandra C Jones ◽  
Lisa Kervin

AbstractObjectiveThe present study sought to determine the feasibility of an experimental research design to investigate the effects of exposure to magazine advertising on children's food choices.DesignChildren were randomized to read either a magazine with food advertisements or a magazine with no food advertisements. They then chose two food items from the intervention ‘store’ to eat after the session. Data were also collected on attitudes to advertising and snack food preferences. Finally, participants’ parents were provided with a self-completion survey on food choices and other variables (n24).SettingThree vacation care centres in regional New South Wales, Australia.SubjectsChildren aged 5–12 years (n47).ResultsChildren in the experimental condition were more likely to choose advertised foods than those in the control group. Interestingly, the majority reported taste and healthiness as the most important factors in snack food choices; however, when faced with the actual food choice, they predominantly chose unhealthy foods (eighty-two unhealthy and only twelve healthy items were chosen).ConclusionsThis was the first study to assess the effects on children of exposure to food advertising within the context of reading a child-targeted magazine. Importantly, even with the small sample size and venue limitations, we found that exposure to magazine advertising influenced food choices. Children's magazines are an under-researched and poorly regulated medium, with considerable potential to influence children's food choices. The present study shows that the methodology is feasible, and future studies could replicate this with larger samples.


2020 ◽  
Vol 40 (4) ◽  
pp. 321-329
Author(s):  
Talal AlMaghamsi ◽  
Naeem Iqbal ◽  
Nabil Abdullrahman Al-Esaei ◽  
Muhsina Mohammed ◽  
Kamel Zein Eddin ◽  
...  

ABSTRACT BACKGROUND: Some mutations of the cystic fibrosis transmembrane regulator ( CFTR ) gene may impair spermatogenesis or cause a congenital absence of the vas deferens that manifests as isolated male infertility. OBJECTIVE: Assess the frequency and analyze the spectrum of CFTR gene variations in Saudi men with primary infertility. DESIGN: Prospective, cross-sectional. SETTING: Tertiary care specialist hospital in Jeddah. PATIENTS AND METHODS: Genomic DNA was extracted from peripheral blood samples of Saudi men who presented with primary infertility to the outpatient andrology clinic with either azoospermia or oligoasthenoteratozoospermia. Polymerase chain reaction and direct sequencing were used to identify all variants of the CFTR gene. MAIN OUTCOME MEASURES: Proportion of the patients with a mutant CFTR gene and the spectrum of CFTR gene variations. SAMPLE SIZE: 50 infertile Saudi men. RESULTS: This study identified 10 CFTR gene variants in 7 (14%) subjects (100 chromosomes). The detected variants and polymorphisms were: c.1408G>A, c.4389G>A, c.2562T>G, c.869+11C>T, c.2909-92G>A, c.3469-65C>A, c.1210-6delT, c.1210-6T>A, c.2988+1G>A, and c.1210-13GT>TG. CONCLUSION: We demonstrated that 14% of the study subjects had one or more CFTR mutations and these were compounded in most of the affected patients. The spectrum of CFTR gene mutations in these subjects was similar to the mutations reported in other studies throughout the world. LIMITATIONS: Small sample size and the lack of a control group. CONFLICTS OF INTEREST: None.


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