Morbidity associated with sickle cell trait carriers

Background: Sickle cell trait carriers has long considered asymptomatic. This affirmation is now challenged because many patients complain of osteoarticular pain and several organic degenerative complications in particular; renal, eye and sudden death have been described. The objective of this study was to evaluate the morbidity of sickle cell trait and identify risk factors associated.Methods: This is a prospective study with duration of 16 months including 50 patients with sickle cell trait received regular visits (every 6 months) for painful events. Biological assessment was carried out systematically to eliminate rheumatic disease (CRP, ASLO, latex Waler Rose) or metabolic disorders (serum calcium, serum magnesium, and serum uric acid). A correlation between clinical and laboratory data was performed to study the relationship between morbidity observed and biological abnormalities.Results: Mean age of patients was 32 years (12-59) and mean age at diagnosis was 24 years (12-55 years). Sex ratio M/F was 0.16. Clinical symptoms were osteoarticular pain (88%), headache (86%), abdominal pain (76%), muscle cramps (70%), dizziness (56%), biliary lithiasis (6%), femoral head osteonecrosis (2%) and gross haematuria (2%). Seventeen patients (34%) had abnormal metabolic or rheumatic analysis. No risk factor associated with morbidity of patients was identified.Conclusions: This work has allowed us to find that the symptoms presented by sickle cell trait patients are dominated by painful events. This morbidity associated with porting sickle cell trait was not secondary to inflammatory or metabolic disorders or physical activity.

Download Full-text

SALMONELLA SPONDYLITIS IN NON-SICKLE CELL PATIENTS

Journal of Musculoskeletal Research ◽

10.1142/s0218957701000593 ◽

2001 ◽

Vol 05 (04) ◽

pp. 253-260

Author(s):

PO-QUANG CHEN ◽

SHU-HUA YANG ◽

CHANG-CHOU YEN ◽

WEN-JER CHEN ◽

TIFFANY T. F. SHIH

Keyword(s):

Antibiotic Treatment ◽

Sickle Cell ◽

Clinical Symptoms ◽

Laboratory Data ◽

Clinical Manifestations ◽

Positive Culture ◽

Surgical Debridement ◽

Treatment Modalities ◽

Endemic Region ◽

Treatment Comparison

This article was a retrospective study of Salmonella spondylitis in seven non-sickle cell anemic patients. The objective of this study was to clarify the clinical manifestations of spinal infection by Salmonella, and to select the proper method of treatment. Comparison with the reported 25 cases in the literature was also attempted. The clinical symptoms and signs, laboratory data, bony images, treatment modalities and outcomes were documented, and compared with the 25 cases in the English literatures. The patients all suffered from insidious onset of low back pain, with progressive increasing intensity. Leucocytosis (>9000) and body temperature elevation (>37°C) were noted in four cases. Positive cultures were mainly obtained from blood (5 cases) and surgical specimens (one case) before treatment. One patient also had positive culture in the stool and urine. Six patients underwent surgical debridement of the infected foci, while one patient received antibiotic treatment only. The outcome was good in the uncomplicated cases. One patient with concurrent aortic aneurysm died of sepsis. We stress that Salmonella infection can be found in the apparently healthy persons. In the endemic region, high suspicion is needed. Surgical debridement, bone grafting and two months of antibiotic treatment are recommended.

Download Full-text

Sickle Cell Trait Induces Oxidative Damage on Plasmodium falciparum Proteome at Erythrocyte Stages

International Journal of Molecular Sciences ◽

10.3390/ijms20225769 ◽

2019 ◽

Vol 20 (22) ◽

pp. 5769

Author(s):

Alber Díaz-Castillo ◽

Neyder Contreras-Puentes ◽

Ciro Alvear-Sedán ◽

Carlos Moneriz-Pretell ◽

Erika Rodríguez-Cavallo ◽

...

Keyword(s):

Sickle Cell ◽

Clinical Symptoms ◽

Sickle Cell Trait ◽

Dot Blot ◽

Hemoglobin A ◽

Sds Page ◽

Erythrocytic Cycle ◽

Dot Blot Assay ◽

High Production ◽

Oxidative Effects

The presence of hemoglobin A-S (HbAS) in erythrocytes has been related to the high production of reactive oxygen species (ROS) and an increased in intracellular oxidative stress that affects the progress of Plasmodium erythrocytic cycle life and attenuates its serious clinical symptoms. Nevertheless, oxidative effects on P. falciparum proteome across the intraerythrocytic cycle in the presence of HbAS traits have not been described yet. Here, an immune dot-blot assay was used to quantify the carbonyl index (C.I) on P. falciparum 3D7 proteome at the different asexual erythrocytic stages. Protein carbonylation on parasites cultivated in erythrocytes from two donors with HbAS increased 5.34 ± 1.42 folds at the ring stage compared to control grown in hemoglobin A-A (HbAA) red blood cells. Whereas at trophozoites and schizonts stages were augmented 2.80 ± 0.52 and 3.05 ± 0.75 folds, respectively. Besides proteins involved in processes of the stress response, recognition and invasion were identified from schizonts carbonylated bands by combining SDS-PAGE with MALDI-TOF-TOF analysis. Our results reinforce the hypothesis that such oxidative modifications do not appear to happen randomly, and the sickle cell trait affects mainly a small fraction of parasite proteins particularly sensitive to ROS.

Download Full-text

Splenic sequestration crisis as an index manifestation of heterozygous hemoglobinopathy in an adult

Oxford Medical Case Reports ◽

10.1093/omcr/omz069 ◽

2019 ◽

Vol 2019 (7) ◽

Cited By ~ 1

Author(s):

Eseosa Edo-Osagie ◽

Ikponmwosa Enofe ◽

Hisham Hakeem ◽

Manoj Rai ◽

Emmanuel Adomako ◽

...

Keyword(s):

Sickle Cell ◽

Clinical Symptoms ◽

Sickle Cell Trait ◽

Beta Thalassemia ◽

Beta Globin ◽

Gradual Improvement ◽

Splenic Sequestration ◽

Dna Test ◽

Pathology Specimen ◽

Tomography Scan

Abstract Sickle β+-thalassemia rarely manifests with acute splenic sequestration crisis in adults. We report a case of a 20-year-old female who presented with fever and left upper quadrant abdominal pain. Laboratory studies revealed hemolytic anemia. Tests for autoimmune hemolysis and hemolytic diseases were negative except for Hemoglobin (Hb) electrophoresis, which revealed sickle cell trait (Hb AS). Infectious workup was unremarkable. Computed tomography scan of the abdomen showed marked splenomegaly. The patient received blood transfusions and empiric antibiotics with no improvement; thus, splenectomy was performed. Pathology specimen revealed peripheral serpiginous infarcts alternating with surrounding acute inflammation and small capillaries plugged with sickle cell shaped red blood cells consistent with splenic sequestration. DNA test later revealed beta-globin mutations consistent with sickle cell-beta+ thalassemia. Post-splenectomy, there was a gradual improvement in her clinical symptoms with concomitant rise in Hb to 10.6 g/dl at discharge.

Download Full-text

Sickle cell trait and the eye

Archives of Internal Medicine ◽

10.1001/archinte.137.3.281a ◽

1977 ◽

Vol 137 (3) ◽

pp. 281a-281

Author(s):

P. E. Mickelson

Keyword(s):

Sickle Cell ◽

Sickle Cell Trait

Download Full-text

Microsurgery in the Sickle Cell Trait Population: Is It Actually Safe?

Surgical Case Reports ◽

10.31487/j.scr.2020.04.02 ◽

2020 ◽

pp. 1-2

Author(s):

Michael Alperovich ◽

Eric Park ◽

Michael Alperovich ◽

Omar Allam ◽

Paul Abraham

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Free Flap ◽

Near Infrared ◽

Sickle Cell Trait ◽

Cell Disease ◽

Flap Transfer ◽

Free Flap Transfer ◽

Patient Reported ◽

Deep Inferior Epigastric Perforator

Although sickle cell disease has long been viewed as a contraindication to free flap transfer, little data exist evaluating complications of microsurgical procedures in the sickle cell trait patient. Reported is the case of a 55-year-old woman with sickle cell trait who underwent a deep inferior epigastric perforator (DIEP) microvascular free flap following mastectomy. The flap developed signs of venous congestion on postoperative day two but was found to have patent arterial and venous anastomoses upon exploration in the operating room. On near-infrared indocyanine green angiography, poor vascular flow was noted despite patent anastomoses and strong cutaneous arterial Doppler signals. Intrinsic microvascular compromise or sickling remains a risk in the sickle cell trait population as it does for the sickle cell disease population. Just like in sickle cell disease patients, special care should be taken to optimize anticoagulation and minimize ischemia-induced sickling for patients with sickle cell trait undergoing microsurgery.

Download Full-text

COVID-19 as a trigger for splenic infarction in a patient with sickle cell trait: a case report

Thrombosis Update ◽

10.1016/j.tru.2021.100047 ◽

2021 ◽

pp. 100047

Author(s):

Álvaro Alejandre-de-Oña ◽

Jaime Alonso-Muñoz ◽

Pablo Demelo-Rodríguez ◽

Jorge del-Toro-Cervera ◽

Francisco Galeano-Valle

Keyword(s):

Case Report ◽

Sickle Cell ◽

Sickle Cell Trait ◽

Splenic Infarction

Download Full-text

Acute Pancreatitis as a Complication of Antiepileptic Treatment: Case Series and Review of the Literature

Pediatric Reports ◽

10.3390/pediatric13010014 ◽

2021 ◽

Vol 13 (1) ◽

pp. 98-103

Author(s):

Agnieszka Pawłowska-Kamieniak ◽

Paulina Krawiec ◽

Elżbieta Pac-Kożuchowska

Keyword(s):

Valproic Acid ◽

Acute Pancreatitis ◽

Metabolic Disorders ◽

Genetic Factors ◽

Clinical Symptoms ◽

Gallstone Disease ◽

Young Patients ◽

Case Series ◽

Review Of The Literature ◽

Acid Therapy

Acute pancreatitis (AP) appears to be rare disease in childhood. In children, it has a different aetiology and course, and requires different management than in adult patients. The diagnosis of AP is based on at least two of the three criteria, which include typical clinical symptoms, abnormalities in laboratory tests and/or imaging studies of the pancreas. There are many known causes leading to AP in children including infections, blunt abdominal trauma, genetic factors, gallstone disease, metabolic disorders, anatomical defects of the pancreas, systemic diseases, as well as drugs, including antiepileptic drugs, and especially preparations of valproic acid. In our study, we present four cases of young patients diagnosed with acute pancreatitis as a complication of valproic acid therapy and we present a review of the literature. We believe that the activity of pancreatic enzymes should be monitored in children treated with valproate preparations in the case of clinical symptoms suggesting AP.

Download Full-text

Anti-IH Related Hemolytic Reaction in a Sickle Cell Patient and Further Management While Avoiding Transfusion

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqaa161.360 ◽

2020 ◽

Vol 154 (Supplement_1) ◽

pp. S165-S166

Author(s):

N Yurtsever ◽

K Wilson-Sandberg ◽

J Dikeman ◽

J Louie ◽

K Fomani

Keyword(s):

Sickle Cell ◽

Clinical Symptoms ◽

Acute Chest Syndrome ◽

Reference Laboratory ◽

Cold Temperatures ◽

Donor Cells ◽

Thermal Amplitude ◽

Local Reference ◽

Hemolytic Transfusion Reaction ◽

Group B

Abstract Introduction/Objective H antigen is a precursor for A and B antigens and is mostly converted except for the O blood group, which has the highest amount of H antigen. I is present on all adult RBCs. Anti-IH is usually an IgM antibody active at cold temperatures, and rarely demonstrates a wide thermal amplitude and can cause a significant hemolytic transfusion reaction. Methods Data was collected from patient information and transfusion management systems. Results 38 year old female with sickle cell disease presented to the emergency room with dizziness, tachypnea and Hgb: 5.9 g/dl Hct:19.1%. Upon further review, patient chart showed that she had received an emergency RBC exchange transfusion 21 days prior to this admission for acute chest syndrome. She was B positive. The units for RBC exchange consisted of 5 group B and 1 group O units and an additional 1 group O unit later. All RBC units were matched for her phenotype; Rh, K, Duffy & Kidd, except anti-S which was ruled out. At the time of discharge, Hgb was 9.3 g/dl and Hct was 27.7%. The drop in Hgb between discharge and the present admission prompted a suspicion for delayed hemolytic reaction/hyperhemolysis. The sample sent to the local Reference Laboratory came back as follows: DAT/Coombs Positive, DAT C3 positive; positive for cold auto-anti-IH antibody. A thermal amplitude test indicated that the anti-IH was reactive at 30 C and therefore had the potential to be of clinical significance. Her Hgb continued to drop and 3 days later Hb=3.7 g/dl with instructions not to transfuse unless clinically emergent. With treatment of IVIG and steroids, reducing further blood draws and monitoring the patient for clinical symptoms only, her Hgb/Hct started to rise and the patient was discharged 4 days later with Hgb: 6.4 g/dl, and no symptoms of anemia. Conclusion Our case study is important in two ways: Firstly, it raises awareness of the severity of a cold autoantibody, i.e. anti-IH, with a wide thermal amplitude. Specifically, in this case, our attempt to provide phenotypically similar RBCs resulted in the destruction of all the type O donor cells as well as some of the B donor cells. Secondly, even with Hgb counts as low as 3.7, treating the patient and not the number proved to be better clinical practice. In conclusion, a good monitoring protocol for sickle cell patients is required to transfuse less and avoid serious complications.

Download Full-text

Zika RNA and Flavivirus-Like Antigens in the Sperm Cells of Symptomatic and Asymptomatic Subjects

Viruses ◽

10.3390/v13020152 ◽

2021 ◽

Vol 13 (2) ◽

pp. 152

Author(s):

Hernan Vanegas ◽

Fredman González ◽

Yaoska Reyes ◽

Edwing Centeno ◽

Jayrintzina Palacios ◽

...

Keyword(s):

Clinical Symptoms ◽

Time Frame ◽

Sperm Cells ◽

Human Semen ◽

Zikv Infection ◽

Virus Shedding ◽

One Year ◽

Asymptomatic Subjects ◽

A Prospective Study ◽

Male Subjects

Zika virus (ZIKV) RNA has been found to remain in human semen for up to one year after infection, but the presence of Flavivirus antigens in the different compartments of semen has been largely unexplored. Following the introduction of ZIKV in Nicaragua (2016), a prospective study of patients with clinical symptoms consistent with ZIKV was conducted in León to investigate virus shedding in different fluids. ZIKV infection was confirmed in 16 male subjects (≥18 years of age) by RT-qPCR in either blood, saliva or urine. Of these, three provided semen samples at 7, 14, 21, 28, 60 and 180 days postsymptom onset (DPSO) for Flavivirus antigens and RNA studies. These cases were compared with 19 asymptomatic controls. Flavivirus antigens were examined by immunofluorescence (IF) using the 4G2 Mabs, and confocal microscopy was used to explore fluorescence patterns. The three (100%) symptomatic subjects and 3 (16%) of the 19 asymptomatic subjects had Flavivirus antigens and viral RNA in the spermatozoa fraction. The percentage of IF Flavivirus-positive spermatozoa cells ranged from 1.9% to 25% in specimens from symptomatic subjects, as compared with 0.8% to 3.8% in specimens from asymptomatic controls. A marked IF-pattern in the cytoplasmic droplets and tail of the spermatozoa was observed. The sperm concentrations (45 × 106/mL vs. 63.5 × 106/mL, p = 0.041) and the total motility percentage (54% vs. 75%, p = 0.009) were significantly lower in specimens from ZIKV-positive than in those of ZIKV-negative. In conclusion, this study demonstrated the presence of Flavivirus antigens and RNA within a time frame of 28 DPSO in sperm cells of symptomatic and asymptomatic subjects during the ZIKV epidemic. These findings have implications for public health, in terms of nonarthropod-born, silent transmission facilitated by sperm cells and potential transmission from asymptomatic males to pregnant women, with consequences to the fetus.

Download Full-text

Metabolism of Heterogenic Hemoglobins

Blood ◽

10.1182/blood.v22.3.334.334 ◽

1963 ◽

Vol 22 (3) ◽

pp. 334-341 ◽

Cited By ~ 10

Author(s):

RICHARD D. LEVERE ◽

HERBERT C. LICHTMAN ◽

Joan Levine

Keyword(s):

Pulmonary Tuberculosis ◽

Sickle Cell ◽

Sickle Cell Trait ◽

Hemoglobin S ◽

Active Pulmonary Tuberculosis ◽

Hemoglobin A ◽

Abnormal Hemoglobin

Abstract The relative rates of incorporation of Fe59 into heterogenic hemoglobins was studied in four patients with sickle cell trait. Three of the patients were free of superimposed disease, while one had active pulmonary tuberculosis. In all subjects there was a significantly greater incorporation of radioiron, per milligram of hemoglobin, into hemoglobin S than into hemoglobin A. The data indicate that in sickle cell trait the rates of synthesis of the heterogenic hemoglobins are not proportional to their circulating concentrations. Two interpretations appear possible. Since the size of the intra-marrow pool of hemoglobin S was not known, it is possible that there exists a smaller preformed pool of the abnormal hemoglobin, with the isotope making its appearance first in hemoglobin S. However, it is also possible that hemoglobin S is synthesized at a rate which is greater than that reflected by its circulating concentration. This implies that the relative concentrations of hemoglobin S and hemoglobin A vary from erythrocyte to erythrocyte, and that those cells with the greatest proportion of hemoglobin S are selectively destroyed.

Download Full-text