scholarly journals Splenectomy in sickle cell haemoglobinopathies

2019 ◽  
Vol 6 (4) ◽  
pp. 1371
Author(s):  
Brajamohan Mishra ◽  
Malaya Krishna Nayak ◽  
Sandeep Mishra ◽  
Itishree Das
Keyword(s):  
Blood Transfusion ◽  
Sickle Cell ◽  
Hematological Parameters ◽  
Splenic Abscess ◽  
Operative Management ◽  
Preventive Strategy ◽  
High Morbidity ◽  
Minimal Risk ◽  
Safe Procedure ◽  
Surgery Department

Background: Sickle cell disease is one of the common haemoglobinopathies in the world. Among its various clinical presentations, splenic complications are associated with high morbidity and substantial mortality, the only effective preventive strategy for which is prophylactic splenectomy. The aim of the present study was to observe the safety and effectiveness of splenectomy to prevent frequent requirement of hospitalizations, blood transfusions and splenic complications in patients of sickle cell disease.Methods: The study was carried out in 72 patients of SCD with splenomegaly who underwent splenectomy for some indication in the surgery department of VSSIMSAR, Burla during the period from October 2016 to September 2018. Their preoperative baseline hematological parameters and frequency of transfusion requirement was compared with postoperative status.Results: Of these 72 SCD patients, 49 (68.7%) patients had repeated requirement of blood transfusion and hypersplenism, 22 (30.5%) patients had history of one or more episodes of splenic sequestration crises, one patient had splenic abscess. After splenectomy the mean increase in haemoglobin level, TLC and TPC was respectively 2.83±0.9 gm%, 1.7±0.8 lac/cmm and 2726±1618/cmm. Operative mortality was 0%. None of the patients required any blood transfusion and no major postoperative complications during 6 months follow up period.Conclusions: The morbidity of the patients of SCD in terms of repeated hospitalizations, blood transfusion, living with a huge spleen, accompanying symptoms and its complications can be effectively minimized by the elective splenectomy. With good preoperative preparation and post-operative management, splenectomy in SCD patients is a safe procedure with minimal risk of post-operative complications.

scholarly journals The Vasculopathy in the Bone Marrow Microenvironment of Humanized Sickle Cell Mice Is Reversible By Blood Transfusion

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2256-2256
Author(s):  
Shin-Young Park ◽  
Alessandro Mattè ◽  
Yookyung Jung ◽  
Jina Ryu ◽  
Wilson Anand ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Vascular Dysfunction ◽  
High Morbidity ◽  
Hematopoietic Stem ◽  
Vascular Niche ◽  
The Impact

Sickle cell disease (SCD) is a monogenic red cell disorder with high morbidity and mortality due to significant end-organ disease. Here we investigated for the first time the impact of SCD on the bone marrow (BM) vascular niche, which is critical for hematopoiesis. In SCD mice, we find a disorganized and structurally abnormal BM vasculature with highly tortuous arterioles and fragmented sinusoidal vessels, the latter filled with aggregates of sickle erythroid and myeloid cells. By in vivo imaging, sickle and control red blood cells exhibit significantly slow intravascular flow speeds in sickle cell BM but not in control BM. These abnormalities lead to increased ROS production in expanded erythroblast populations, and to up-regulation in the BM of HIF-1a and key growth factors required to support angiogenesis, e.g. VEGF-A, Ang1, Ang2. Moreover, increased levels of VCAM-1, a marker for vascular dysfunction, are detected. SCD also impacts the BM perivascular niche, i.e. the number of CXCL12-abundant reticular cells, is markedly decreased, and is associated with mobilization of hematopoietic stem/progenitor cells as well as mature leukocytes to the periphery. Strikingly, the distorted vascular network is completely reversed by blood transfusion, which highlights the plasticity of the BM vascular niche. Thus, we provide molecular and cellular features of the pathologic BM microenvironment, which suggest that the local hypoxia-induced angiogenic network combined with inflammatory vascular dysfunction is responsible for the vasculopathy. In addition, the findings may serve to develop new biomarkers and therapeutic targets of BM end-organ damage. Figure Disclosures No relevant conflicts of interest to declare.

Laparoscopic Splenectomy for Hematologic Disorders: A Safe Procedure.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5523-5523
Author(s):  
Christophe Ghys ◽  
Elie Capelluto ◽  
A. Baize ◽  
Nathalie Meuleman ◽  
Jalil Bennani ◽  
...  
Keyword(s):  
Laparoscopic Splenectomy ◽  
Hematological Parameters ◽  
Pneumococcal Vaccination ◽  
Invasive Procedure ◽  
High Morbidity ◽  
Safe Procedure ◽  
Portal Thrombosis ◽  
Perioperative Mortality ◽  
Thrombotic Complications ◽  
Autoimmune Cytopenia

Abstract Background : Splenectomy for hematological diseases remains controversial in many circumstances due to a high morbidity and mortality after a classical surgery. Laparoscopic surgery becoming more popular, spleen removal by this approach was more widely used and we retrospectively reviewed the outcome of 33 patients who underwent laparoscopic splenectomy in our institution. Population : Between 1998 and 2006, laparoscopic splenectomy was performed in 33 patients (F=6, M=27) by an anterior approach. Median age was 62 (40–90) y.o. Indications for splenectomy was hemolytic anemia or discomfort in malignant hemopathies (CML, MF, NHL, CLL, HD) and autoimmune cytopenia or peripheral cytopenia in non malignant hemopathies (AIHA, ITP,.). Spleen weights ranged from 100g to 5000g. All the patients received anti-pneumococcal vaccination 2 weeks before surgery. After surgery, all patients were covered by penicillin and LMWH until discharge from the hospital. Results : Perioperative mortality was 0% and major postoperative complications were observed in 3 patients (Pulmonary Embolism, DVT, Hemorrhage). Conversion was required in one patient. One patient died 4 years after splenectomy from extended deep portal thrombosis. Improvement in hematological parameters and/or performans status was observed in 95% of the patients. Mean duration of hospital stay was 5 days. Conclusion : Anterior laparoscopic splenectomy for splenomegaly is a safe and minimally invasive procedure in the hands of experienced surgeons. Howerer, a closed monitoring of the platelets count should be performed, even on an ambulatory basis, because of the risk of thrombotic complications.

scholarly journals Study of acute intestinal obstruction management and its outcome

2021 ◽  
Vol 8 (12) ◽  
pp. 3535
Author(s):  
Valarmathi M.
Keyword(s):  
Intestinal Obstruction ◽  
Clinical Presentation ◽  
Operative Management ◽  
Acute Intestinal Obstruction ◽  
High Morbidity ◽  
Common Symptom ◽  
Abdominal Emergency ◽  
Postoperative Therapy ◽  
Correct Technique ◽  
Surgery Department

Background: Acute intestinal blockage (AIO) is a common abdominal emergency with a high morbidity and mortality rate. Early blockage diagnosis, skilled operative management, correct technique during surgery and intense postoperative therapy yield superior results. The aim was to study the aetiology, clinical presentation, therapy and outcomes of patients with IO who presented in the emergency department.Methods: This prospective study was conducted at the general surgery department from June 2019 to May 2021. The study comprised 60 patients with IO who presented to the OPD or emergency room and the data was analysed.Results: Among 60 patients with IO, peak incidence was observed in patients above 50 years of age, predominantly among males. Abdominal pain turned out to be the most common symptom of IO in our patients. Patients obstructed in the small intestine were found to be predominant. Post-operative adhesions were the most common cause of IO.Conclusions: Early diagnosis, proper preoperative hydration, fast investigations, and early operational intervention were found to increase survival in patients with intestinal obstruction in the current study.

scholarly journals Splenectomy in Patients with Sickle Cell Disease in Tabuk

2016 ◽  
Vol 4 (1) ◽  
pp. 107-111
Author(s):  
Asmaa Ghmaird ◽  
Mohammad Mohammad Alnoaiji ◽  
Sawsan Al-Blewi ◽  
Shaimaa Zaki ◽  
Ahmad El-lewi ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Autosomal Recessive Disorder ◽  
Acute Chest Syndrome ◽  
High Morbidity ◽  
Cell Disease ◽  
North West ◽  
Life Threatening

BACKGROUND: Sickle cell disease is a common genetic disease in Saudi Arabia; it is an autosomal recessive disorder characterized by production of abnormal hemoglobin S and is associated with high morbidity and mortality. Acute splenic sequestration is a life-threatening complication for this disease. Prophylactic splenectomy is the only effective strategy for preventing future life-threatening episodes.AIM: The aim of this study was to study hospital records for all children aged 2 to 12 year old with Sickle cell disease who underwent splenectomy in Tabuk in Saudi Arabia.METHODS: Records of 24 children (13 males, 11 females) who underwent splenectomy in surgery department of King Salman North West Armed Hospital, Tabuk, Saudi Arabia between 2008 and 2015 were reviewed retrospectively and analyzed for age, sex, indications for splenectomy, surgical technique, preoperative and postoperative length of stay, operative and postoperative complications, acute chest syndrome, painful crises, blood transfusion and fever (preoperative and postoperative).RESULTS: We stressed on the information about the details of operation, the frequency of blood transfusion, fever, acute chest syndrome and painful crisis before and after operation.CONCLUSION: Here we found that blood transfusion frequency decreased after splenectomy.

scholarly journals Pathologic angiogenesis in the bone marrow of humanized sickle cell mice is reversed by blood transfusion

Blood ◽  
2020 ◽  
Vol 135 (23) ◽  
pp. 2071-2084 ◽  
Author(s):  
Shin-Young Park ◽  
Alessandro Matte ◽  
Yookyung Jung ◽  
Jina Ryu ◽  
Wilson Babu Anand ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
High Morbidity ◽  
Vascular Niche ◽  
Flow Speeds ◽  
Vessel Growth ◽  
And Control ◽  
The Impact

Abstract Sickle cell disease (SCD) is a monogenic red blood cell (RBC) disorder with high morbidity and mortality. Here, we report, for the first time, the impact of SCD on the bone marrow (BM) vascular niche, which is critical for hematopoiesis. In SCD mice, we find a disorganized and structurally abnormal BM vascular network of increased numbers of highly tortuous arterioles occupying the majority of the BM cavity, as well as fragmented sinusoidal vessels filled with aggregates of erythroid and myeloid cells. By in vivo imaging, sickle and control RBCs have significantly slow intravascular flow speeds in sickle cell BM but not in control BM. In sickle cell BM, we find increased reactive oxygen species production in expanded erythroblast populations and elevated levels of HIF-1α. The SCD BM exudate exhibits increased levels of proangiogenic growth factors and soluble vascular cell adhesion molecule-1. Transplantation of SCD mouse BM cells into wild-type mice recapitulates the SCD vascular phenotype. Our data provide a model of SCD BM, in which slow RBC flow and vaso-occlusions further diminish local oxygen availability in the physiologic hypoxic BM cavity. These events trigger a milieu that is conducive to aberrant vessel growth. The distorted neovascular network is completely reversed by a 6-week blood transfusion regimen targeting hemoglobin S to <30%, highlighting the plasticity of the vascular niche. A better insight into the BM microenvironments in SCD might provide opportunities to optimize approaches toward efficient and long-term hematopoietic engraftment in the context of curative therapies.

scholarly journals Importance of a Follow-Up Ultrasound Protocol in Monitoring Posttraumatic Spleen Complications in Children Treated with a Non-Operative Management

Medicina ◽  
2021 ◽  
Vol 57 (8) ◽  
pp. 734
Author(s):  
Ivona Djordjevic ◽  
Dragoljub Zivanovic ◽  
Ivana Budic ◽  
Ana Kostic ◽  
Danijela Djeric
Keyword(s):  
Splenic Abscess ◽  
Operative Management ◽  
High Energy ◽  
Significant Difference ◽  
Injury Grade ◽  
Grade Ii ◽  
The Mean ◽  
Non Operative Management ◽  
Grade Iii

Background and objectives: For the last three decades, non-operative management (NOM) has been the standard in the treatment of clinically stable patients with blunt spleen injury, with a success rate of up to 95%. However, there are no prospective issues in the literature dealing with the incidence and type of splenic complications after NOM. Materials and methods: This study analyzed 76 pediatric patients, up to the age of 18, with blunt splenic injury who were treated non-operatively. All patients were included in a posttraumatic follow-up protocol with ultrasound examinations 4 and 12 weeks after injury. Results: The mean age of the children was 9.58 ± 3.97 years (range 1.98 to 17.75 years), with no statistically significant difference between the genders. The severity of the injury was determined according to the American Association for Surgery of Trauma (AAST) classification: 7 patients had grade I injuries (89.21%), 21 patients had grade II injuries (27.63%), 33 patients had grade III injuries (43.42%), and 15 patients had grade IV injuries (19.73%). The majority of the injuries were so-called high-energy ones, which were recorded in 45 patients (59.21%). According to a previously created posttraumatic follow-up protocol, complications were detected in 16 patients (21.05%). Hematomas had the highest incidence and were detected in 11 patients (14.47%), while pseudocysts were detected in 3 (3.94%), and a splenic abscess and pseudoaneurysm were detected in 1 patient (1.31%), respectively. The complications were in a direct correlation with injury grade: seven occurred in patients with grade IV injuries (9.21%), five occurred in children with grade III injuries (6.57%), three occurred in patients with grade II injuries (3.94%), and one occurred in a patient with a grade I injury (1.31%). Conclusion: Based on the severity of the spleen injury, it is difficult to predict the further course of developing complications, but complications are more common in high-grade injuries. The implementation of a follow-up ultrasound protocol is mandatory in all patients with NOM of spleen injuries for the early detection of potentially dangerous and fatal complications.

scholarly journals Research Article GSTT1 null genotype in sickle cell anemia and blood transfusion recurrence – a case report

2019 ◽  
Vol 18 (2) ◽  
Author(s):  
O.S. Dias Neto ◽  
K.S.F. e Silva ◽  
A.M. Barbosa ◽  
D.A. Rodrigues ◽  
M.H. Lagares ◽  
...  
Keyword(s):  
Case Report ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Research Article

The Obesity Paradox in Emergency General Surgery Patients

2021 ◽  
pp. 000313482096852
Author(s):  
Sean R. Maloney ◽  
Caroline E. Reinke ◽  
Abdelrahman A. Nimeri ◽  
Sullivan A. Ayuso ◽  
A. Britton Christmas ◽  
...  
Keyword(s):  
General Surgery ◽  
Multivariable Analysis ◽  
Operative Management ◽  
Chronic Obstructive ◽  
High Morbidity ◽  
Emergency General Surgery ◽  
Common Procedure ◽  
Increased Risk ◽  
Social Security Death Index ◽  
The Impact

Operative management of emergency general surgery (EGS) diagnoses involves a range of procedures which can carry high morbidity and mortality. Little is known about the impact of obesity on patient outcomes. The aim of this study was to examine the association between body mass index (BMI) >30 kg/m2 and mortality for EGS patients. We hypothesized that obese patients would have increased mortality rates. A regional integrated health system EGS registry derived from The American Association for the Surgery of Trauma EGS ICD-9 codes was analyzed from January 2013 to October 2015. Patients were stratified into BMI categories based on WHO classifications. The primary outcome was 30-day mortality. Longer-term mortality with linkage to the Social Security Death Index was also examined. Univariate and multivariable analyses were performed. A total of 60 604 encounters were identified and 7183 (11.9%) underwent operative intervention. Patient characteristics include 53% women, mean age 58.2 ± 18.7 years, 64.2% >BMI 30 kg/m2, 30.2% with chronic obstructive pulmonary disease, 19% with congestive heart failure, and 31.1% with diabetes. The most common procedure was laparoscopic cholecystectomy (36.4%). Overall, 90-day mortality was 10.9%. In multivariable analysis, all classes of obesity were protective against mortality compared to normal BMI. Underweight patients had increased risk of inpatient (OR = 1.9, CI = 1.7-2.3), 30-day (OR = 1.9, CI = 1.7-2.1), 90-day (OR = 1.8, CI 1.6-2.0), 1-year (OR = 1.8, CI = 1.7-2.0), and 3-year mortality (OR = 1.7, CI = 1.6-1.9). When stratified by BMI, underweight EGS patients have the highest odds of death. Paradoxically, obesity appears protective against death, even when controlling for potentially confounding factors. Increased rates of nonoperative management in the obese population may impact these findings.

Abstract TP402: Sickle Cell Disease in Pediatric Stroke - Inpatient Resource Utilization in the Decade Following STOP

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
J. Michael Taylor ◽  
Paul Horn ◽  
Heidi Sucharew ◽  
Todd A Abruzzo ◽  
Jane Khoury
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Care Utilization ◽  
Cell Disease ◽  
Common Procedure ◽  
Transfusion Therapy ◽  
Stroke Population ◽  
Clinical Classification Software ◽  
Inpatient Database

Background: Sickle cell disease (SCD) is an important risk factor for stroke in children. Natural history studies demonstrate that greater than 10% of hemoglobin SS patients suffered ischemic stroke prior to age 20 years. In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) successfully demonstrated the role for routine transfusion therapy in reducing stroke in at risk SCD patients. Fullerton and colleagues then found that first time stroke in SCD decreased in Californian children in the 2 years following STOP. We investigated the stroke rate and health care utilization of children with SCD for two calendar years in the decade following publication of the STOP trial using a national inpatient database. Methods: The 2000 and 2009 Kids’ Inpatient Database (KID) were used for analysis. SCD and stroke cases were identified by ICD-9 codes 282.6x, 430, 431, 432.9, 434.X1, 434.9, 435.9. We queried the KID procedural clinical classification software for utilization of services pertinent to SCD and stroke; transfusion, MRI, and cerebral angio. Results: In 2000, SCD was a discharge diagnosis in 34,294 children and 158 (0.46%) children had SCD and stroke. By 2009, discharges with SCD rose to 37,082 children with 212 (0.57%) children carrying both diagnoses. In 2000 and 2009, AIS is the most common stroke type at 83%, males account for 53% of stroke and black race was reported by 92% of SCD and stroke subjects. Procedure utilization is higher in the SCD and stroke population than in SCD without stroke (Figure 1). Blood transfusion is the most common procedure in both study years, significantly higher in stroke subjects. Conclusion: For pediatric inpatients with SCD, blood transfusion and diagnostic cerebrovascular procedures were significantly more common in the cohort with comorbid stroke. In the decade after STOP, children hospitalized with SCD and stroke represented less than 0.6% of the total inpatient SCD population.

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