BACKGROUND Ulcerative colitis(UC)and systemic lupus erythematosus(SLE)are both systemic immunoreactive diseases,and their pathogenesis depends on the interaction between genes and environmental factors.There are no re...BACKGROUND Ulcerative colitis(UC)and systemic lupus erythematosus(SLE)are both systemic immunoreactive diseases,and their pathogenesis depends on the interaction between genes and environmental factors.There are no reports of UC with SLE in China,but six cases of SLE with UC have been reported in China.The combination of these two diseases has distinct effects on the pathogenesis of both diseases.CASE SUMMARY A female patient(30 years old)came to our hospital due to dull umbilical pain,diarrhea and mucous bloody stool in August 2018 and was diagnosed with UC.The symptoms were relieved after oral administration of mesalazine(1 g po tid)or folic acid(5 mg po qd),and the patient were fed a control diet.On June 24,2019,the patient was admitted for treatment due to anemia and tinnitus.During hospitalization,the patient had repeated low-grade fever and a progressively decreased Hb level.Blood tests revealed positive antinuclear antibody test,positive anti-dsDNA antibody,0.24 g/L C3(0.9-1.8 g/L),0.04 g/L C4(0.1-0.4 g/L),32.37 g/L immunoglobulin(8-17 g/L),and 31568.1 mg/24 h total 24-h urine protein(0-150 mg/24 h).The patient was diagnosed with SLE involving the joints,kidneys and blood system.Previously reported cases of SLE were retrieved from PubMed to characterize clinicopathological features and identify prognostic factors for SLE.CONCLUSION The patient was discharged in remission after a series of treatments,such as intravenous methylprednisolone sodium succinate,intravenous human immunoglobulin,cyclophosphamide injection,and plasma exchange.After discharge,the patient took oral prednisone acetate tablets,cyclosporine capsules,hydroxychloroquine sulfate tablets and other treatments for symptoms and was followed up regularly for 1 month,after which the patient's condition continued to improve and stabilize.展开更多
Introduction: Moringa oleifera was a medicinal plant generally used by populations in the food and therapeutic fields. It’s used to treat anemia has been observed in the Djougou Zone in northern Benin. To our knowled...Introduction: Moringa oleifera was a medicinal plant generally used by populations in the food and therapeutic fields. It’s used to treat anemia has been observed in the Djougou Zone in northern Benin. To our knowledge, there were no scientific data available that have evaluated its efficacy in the treatment of haemolytic anemia. This was what justifies this research work in which the phytochemical analysis, extraction and evaluation of the anti-anemic effect were carried out. Methods: Five groups of five Wistar rats each were formed. All the rats were rendered anemic by injection of phenylhydrazine hydrochloride on the first two days D0 and D1 except those in the negative control group. From the second day, the anemic groups were force-fed either with the aqueous extract of Moringa oleifera leaves at 200 or 300 mg/kg body weight/day, or with vitafer, the reference drug against anemia. The positive control group (anemia) was not treated. Blood samples were taken from all the rats on different days: D0, D2, D7, D10 and D15 to evaluate the data of the hemogram and the osmotic resistance of the red blood cells. Results: Phytochemical analysis revealed the presence of tannins, flavonoids, leucoanthocyanins, saponosides, triterpenes and mucilages. A good yield was obtained at the extraction. Both the extract and the reference drug vitafer completely corrected anemia within two weeks after stimulating hemoglobin synthesis and early release of immature red blood cells into the bloodstream. Its effect seemed dose-dependent and specific. Conclusion: Moringa oleifera leaves showed good therapeutic efficacy and can be considered and exploited for transformation into improved traditional medicines (ITM) in the treatment of anemia.展开更多
Introduction: Acute Promyelocytic Leukemia (APL) is highly associated with hemostasis alterations. The atypical hemolytic uremic syndrome (aHUS) is a rare type of Thrombotic Microangiopathy (TMA) due to an overactivat...Introduction: Acute Promyelocytic Leukemia (APL) is highly associated with hemostasis alterations. The atypical hemolytic uremic syndrome (aHUS) is a rare type of Thrombotic Microangiopathy (TMA) due to an overactivation of the alternative complement pathway. Case Presentation: A 48-years-old woman was diagnosed with APL and achieved molecular remission after induction therapy. During the second consolidation cycle she presented with TMA. She began treatment with plasma exchange plus corticotherapy but due to aggravation of symptoms Eculizumab was initiated. Thrombotic thrombocytopenic purpura, infections and drug toxicity causes were ruled out. There was no evidence of relapse of the APL. Genetic studies of the hereditary anomalies of the alternative complement pathway were negative and the decision of stopping Eculizumab was made. During maintenance therapy for the APL she presented a severe relapse of the aHUS, requiring dialysis. She re-started treatment with Eculizumab with a progressive hematologic recovery and improvement of renal function. She completed APL treatment without relapse of the leukemia for the moment and continues to be treated with Eculizumab. Conclusion: This is the first published case of coexisting aHUS and APL successfully treated with Eculizumab.展开更多
BACKGROUND Anti-D antibody is not the common cause of Rh-isoimmunization in Chinese neonatal jaundice.Recent change in national population policy has followed by an increase in Rh-isoimmunization related hemolytic dis...BACKGROUND Anti-D antibody is not the common cause of Rh-isoimmunization in Chinese neonatal jaundice.Recent change in national population policy has followed by an increase in Rh-isoimmunization related hemolytic disease of the newborn(HDN).Unfortunately,regional status of Rh-HDN is unavailable.We hypothesize that Rh-HDN in our region is most commonly due to anti-E antibody.AIM To investigate the prevalence of hemolytic disease of the newborn due to Rhisoimmunization in Hefei City.METHODS Retrospective review of data obtained from Children’s Hospital of Anhui and Hefei Blood Center between January 2017 and June 2019.Status of minor blood group antibody was studied in the corresponding mothers.RESULTS Totally 4138 newborns with HDN admitted during the study period and 116(2.8%)received blood exchange transfusion(BET).Eighteen newborns(0.43%)with proven Rh-incompatible HDN were identified.All were not the first-born baby.Thirteen mothers were RhD(+)(72%)and five were RhD(-).The distribution of Rh-related antibodies in mothers was ten anti-E(55%),five anti-D(27%),and for one anti-C,anti-c,and anti-E/c(6%)each.Thirteen(72.2%)were qualified for BET,relative risk for BET was 28.9 as compared to other types of HDN,but only 10 received due to parenteral refusal.All(100%)RhD related HDN received BET which is not significantly different from RhE related HDN(81.8%).CONCLUSION As expected,all Rh-incompatible HDN newborns were not the first-born.Contrary to the Caucasian population,anti-D induced HDN is not the most common etiology.In our region,anti-E(11/18,61%)is the most common cause of Rh-HDN.展开更多
AIM: To clarify the incidence of congenital hemolytic anemias (CHA) in young cholelithiasis patients and to determine a possible screening test based on the results. METHODS: Young cholelithiasis patients (< 35 yea...AIM: To clarify the incidence of congenital hemolytic anemias (CHA) in young cholelithiasis patients and to determine a possible screening test based on the results. METHODS: Young cholelithiasis patients (< 35 years) were invited to our outpatient clinic. Participants were asked for comorbidities and family history. The number of gallstones were recorded. Blood samples were obtained to perform a complete blood count, standard Wright-Giemsa staining, reticulocyte count, hemoglobin (Hb) electrophoresis, serum lactate dehydrogenase and bilirubin levels, and lipid profile. RESULTS: Of 3226 cholecystectomy patients, 199 were under 35 years, and 190 with no diagnosis of CHA were invited to take part in the study. Fifty three patients consented to the study. The median age was 29 years (range, 17-35 years), 5 were male and 48 were female. Twelve patients (22.6%) were diagnosed as thalassemia trait and/or ?ron-deficiency anemia. Hblevels were significantly lower (P = 0.046), and mean corpuscular volume (MCV) and hematocrit levels were slightly lower (P = 0.072 and 0.082, respectively) than normal. There was also a significantly lower number of gallstones with the diagnosis (P = 0.007). CONCLUSION: In endemic regions, for young cholelithiasis patients (age under 35) with 2-5 gallstones, the clinician/surgeon should pay attention to MCV and Hb levels as indicative of CHA.展开更多
BACKGROUND Thrombocytopenia associated with acute kidney injury is a challenging disorder. Thrombotic microangiopathy (TMA) is a potentially life- or organ-threatening syndrome that can be induced by several disorders...BACKGROUND Thrombocytopenia associated with acute kidney injury is a challenging disorder. Thrombotic microangiopathy (TMA) is a potentially life- or organ-threatening syndrome that can be induced by several disorders or medical interventions. There is overlap between the clinical presentation and pathophysiology of thrombotic thrombocytopenia purpura and hemolytic uremic syndrome (HUS), and to a lesser extent, disseminated intravascular coagulation (DIC). We describe a case to illustrate the potential diagnostic difficulty, especially at initial presentation. CASE SUMMARY We reported a case of a 44-year-old woman that presented with diarrhea, thrombocytopenia, schistocytes, elevated serum lactate dehydrogenase (LDH) level and acute kidney injury. While the clinical presentation resembled that of Shiga toxin–induced HUS, the disease course was more consistent with gastrointestinal infection-related DIC. To aid in the accurate diagnosis of TMA and other associated disorders, we have undertaken a review and provided a clear interpretation of some typical biomarkers including schistocytes, LDH and platelet count, coagulation profile and more specific indexes of ADAMTS13, complement profile, and the isolation of Shiga toxin-producing Escherichia coli (commonly referred to as STEC). CONCLUSION The use and correct interpretation of classical indexes of schistocyte, LDH, and platelet count is vital in diagnosing TMA and associated disorders. Understanding the characteristics of these biomarkers in the context of thrombocytopenia purpura, HUS and DIC will facilitate the accurate diagnosis and early initiation of appropriate treatment.展开更多
BACKGROUND Microangiopathic hemolytic anemia(MAHA) with thrombocytopenia and organ failure caused by tumor-associated thrombotic microangiopathy(TMA) is a lifethreatening oncological emergency. Rapid diagnosis and pre...BACKGROUND Microangiopathic hemolytic anemia(MAHA) with thrombocytopenia and organ failure caused by tumor-associated thrombotic microangiopathy(TMA) is a lifethreatening oncological emergency. Rapid diagnosis and precise distinction from other forms of TMA is crucial for appropriate therapy, which aims at treating the underlying malignancy. However, the prognosis of patients with cancer-related(CR)-MAHA is limited. To date, less than 50 patients with gastric cancer and CRMAHA have been reported, mainly as single case reports, and detailed information on treatment strategies and outcome are scarce. We analyzed the characteristics and outcomes data of CR-MAHA patients with gastric cancer treated at our center between 2012 and 2019.AIM To gain knowledge about CR-MAHA and the course of disease.METHODS We retrospectively analyzed patients using an institutional prospectively maintained database. Patients who had CR-MAHA but other cancer types or cancer of unknown primary were excluded. The basic requirements for inclusion were: Histologically proven gastric adenocarcinoma;and clinical diagnosis of hemolytic anemia with schistocytes with or without thrombocytopenia. The observation period for each patient started with the first day of documented symptoms. The follow-up period for this analysis ended on February 1, 2020.RESULTS We identified eight patients with a median age of 54 years. Histologically, all patients had(partial) diffuse subtypes of gastric adenocarcinoma with partial or complete signet cell morphology. All patients had metastatic disease and one patient had a microsatellite instability-high(MSI-H) tumor. In three patients, clinical signs of MAHA preceded the diagnosis of cancer, and in two patients, CRMAHA indicated recurrent disease. All patients had severe hemolytic anemia and thrombocytopenia. Six patients experienced severe bone pain, and five patients had dyspnea. Systemic, 5-fluorouracil-based combination chemotherapy was initiated in six patients, which resulted in rapid initial response with significant improvement of clinical symptoms and blood values. Progression-free survival(PFS) of the whole cohort was 1.9 wk and median overall survival(OS) was 1.9 wk. For patients with chemotherapy, PFS was 9.0 wk and OS was 10.3 wk. The patient with the MSI-H tumor has been undergoing immunotherapy for more than 3 years.CONCLUSION The benefit of chemotherapy in CR-MAHA patients is limited. Immunotherapy for patients with MSI-H tumors may lead to long-term tumor control even in CRMAHA patients.展开更多
BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.Ho...BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.展开更多
Pyelonephritis is an inflammation of the kidney tissue, calyces, and renal pelvis. It is commonly caused by bacterial infection that has spread up the urinary tract. Common bacteria are Escherichia coli and Enterococc...Pyelonephritis is an inflammation of the kidney tissue, calyces, and renal pelvis. It is commonly caused by bacterial infection that has spread up the urinary tract. Common bacteria are Escherichia coli and Enterococcus faecalis. Beta hemolytic streptococci also cause pyelonephritis. Beta hemolytic streptococcus such as Streptococcus agalactiae, and S. dysgalactiae subsp. equisimilis, when grown on blood-agar, produce complete hemolysis around each colony, usually cause severe infections including many cases of cellulitis, pneumonia, meningitis and sepsis. However the analysis of pyelonephritis causing beta hemolytic streptococcus has seldom been investigated. In this study, we tried to clarify the relationship between beta hemolytic streptococcus and pyelonephritis patient in Japan. We investigated beta hemolytic streptococcus isolated from pyelonephritis patient at two tertiary care hospitals in the central region of Japan by analyzing the background and antimicrobial susceptibility of beta hemolytic streptococcus. We recovered total sixteen beta hemolytic streptococcus in nine years. In pyelonephritis patients, beta hemolytic streptococcus was isolated from 9 S. agalactiae and 7 S. dysgalactiae subsp. equisimilis, respectively. The numbers of streptococci from female patients were more than those from male patients. Bacteria were not isolated from under 30 year patients. All S. dysgalactiae subsp. equisimilis were isolated from 60-year-age patients. About one fifth of beta hemolytic streptococcus had erythromycin and minocycline-resistant ability. Four beta hemolytic streptococci that were all S. agalactiae, had levofloxacin-resistant ability. Only one S. agalactiae had trimethoprim-sulfamethoxazole-resistant ability. Ampicillin was effective against all beta hemolytic streptococci. In our results, beta hemolytic streptococcus caused pyelonephritis in aged people and the quarter of these bacteria had some antibiotics resistance.展开更多
BACKGROUND Wilson’s disease(WD)is a rare autosomal recessive inherited disorder of copper metabolism.Acute liver failure(ALF)and hemolytic anemia represent the most severe presentation of WD in children.No clear geno...BACKGROUND Wilson’s disease(WD)is a rare autosomal recessive inherited disorder of copper metabolism.Acute liver failure(ALF)and hemolytic anemia represent the most severe presentation of WD in children.No clear genotype-phenotype correlations exist in WD.Protein-truncating nonsense,frame-shift,or splice-site variants may be associated with more severe disease.In contrast,missense variants may be associated with late-onset,less severe disease,and more neurological manifestations.Recently,a gene variant(HSD17B13:TA,rs72613567)with a possible hepatic protective role against toxins was associated with a less severe hepatic phenotype in WD.AIM To analyze the possible genotype-phenotype correlations in children with WD presented with ALF and non-immune hemolytic anemia.METHODS The medical records of children with WD diagnosed and treated in our hospital from January 2006 to December 2020 were retrospectively analyzed.The clinical manifestations(ALF with non-immune hemolytic anemia or other less severe forms),laboratory parameters,copper metabolism,ATP7B variants,and the HSD17B13:TA(rs72613567)variant were reviewed to analyze the possible genotype-phenotype correlations.RESULTS We analyzed the data of 51 patients with WD,26 females(50.98%),with the mean age at the diagnosis of 12.36±3.74 years.ALF and Coombs-negative hemolytic anemia was present in 8 children(15.67%),all adolescent girls.The Kayser-Fleisher ring was present in 9 children(17.65%).The most frequent variants of the ATP7B gene were p.His1069Gln(c.3207A>G)in 38.24% of all alleles,p.Gly1341Asp(c.4021G>A)in 26.47%,p.Trp939Cys(c.2817G>T)in 9.80%,and p.Lys844Ter(c.2530A>T)in 4.90%.In ALF with hemolytic anemia,p.Trp939Cys(c.2817G>T)and p.Lys844Ter(c.2530A>T)variants were more frequent than in other less severe forms,in which p.His1069Gln(c.3207A>G)was more frequent.p.Gly1341Asp(c.4021G>A)has a similar frequency in all hepatic forms.For 33 of the patients,the HSD17B13 genotype was evaluated.The overall HSD17B13:TA allele frequency was 24.24%.Its frequency was higher in patients with less severe liver disease(26.92%)than those with ALF and hemolytic anemia(14.28%).CONCLUSION It remains challenging to prove a genotype-phenotype correlation in WD patients.In children with ALF and hemolytic anemia,the missense variants other than p.His1069Gln(c.3207A>G)and frame-shift variants were the most frequently present in homozygous status or compound heterozygous status with site splice variants.As genetic analysis is usually time-consuming and the results are late,the importance at the onset of the ALF is questionable.If variants proved to be associated with severe forms are found in the pre-symptomatic phase of the disease,this could be essential to predict a possible severe evolution.展开更多
Fluorine-doped hydrogenated amorphous carbon (a-C:H:F) film was deposited on a flow-straightener, impeller and diffuser surface (SUS 304) of an enclosed-impeller type flow blood pump using the ionization deposition me...Fluorine-doped hydrogenated amorphous carbon (a-C:H:F) film was deposited on a flow-straightener, impeller and diffuser surface (SUS 304) of an enclosed-impeller type flow blood pump using the ionization deposition method with a source gas of C6F5H. The surface characteristics of the a-C:H:F film were examined using atomic force microscopy, X-ray photoelectron spectroscopy, and measurements of surface roughness, friction and surface potential. The a-C:H:F film tends to increase surface roughness and the negative surface charge. In addition, the surface energy and friction decrease with fluorine dopant in the a-C:H film. To estimate the hemolytic performance of a blood pump with the a-C:H:F film coating, the amount of hemolysis was measured using a mock circulatory system (in vitro test) with 500 mL of pig blood containing sodium citrate. In vitro test was conducted for 180 min with the blood flow and pump head maintained at 5 L/min and 100 mmHg, respectively. The a-C:H:F film coating reduced the amount of hemolysis and improved the hemolytic performance. Decreasing the surface energy and negative surface charge of the a-C:H:F film contributes to the improvement of the hemolytic performance. The a-C:H:F film coating is thus expected to be utilized in medical technology as a surface coating technology for artificial heart blood pumps.展开更多
Drug-induced immune hemolytic anemia (DIIHA) is commonly attributed to cephalosporins. Ceftriaxone is the most frequently administered cephalosporin in patients with sickle cell disease. We present a pediatric patient...Drug-induced immune hemolytic anemia (DIIHA) is commonly attributed to cephalosporins. Ceftriaxone is the most frequently administered cephalosporin in patients with sickle cell disease. We present a pediatric patient with severe DIIHA (hemoglobin < 2 g/dl) who survived. Since DIIHA often goes undiagnosed until late in the course, vigilance of DIIHA minimizes unnecessary diagnostic tests and therapies. DIIHA likely remains under-recognized in all patient subpopulations due to its rarity and overlapping presentations with other conditions. Distinction between exacerbation of chronic hemolysis and new onset of acute hemolysis poses a unique challenge in patients with sickle cell disease. A thorough analysis is warranted to better identify factors within the pediatric sickle cell population that may increase the predisposition for DIIHA, particularly due to ceftriaxone.展开更多
A Chinese woman of blood group B,D-and her husband of blood group AB,CCDeewere examined.The woman had not been transfused before.Their first two babiesdied.Anti-Hro and anti-e were found in the mother’s serum.During ...A Chinese woman of blood group B,D-and her husband of blood group AB,CCDeewere examined.The woman had not been transfused before.Their first two babiesdied.Anti-Hro and anti-e were found in the mother’s serum.During her third pregnancy,the titer of antibodies went up quickly,approximately one titer per month.After 36 weeksof pregnancy,the baby was delivered by Caesarean section.The cord blood Hb was 88g/L,his red blood cell count 2.7×10<sup>12</sup>/L,and total biIirubin 114.6 mol/L.The baby was ofblood group AB,and CDe-D-genotype.Exchangetransfusion was begun 2.5 hours afterbirth.O,ccDEE washed red cells together with group AB plasma were used.Two dayslater,7Oml washed O,ccDEE concentrated red cells were administered.The baby is aliveand in good health.展开更多
Objective:To decrease or delay the major un-wanted clinical consequences to improve the quality of life in the involved patients.Methods:A retrospective case series study has been made on the forty five pediatric pati...Objective:To decrease or delay the major un-wanted clinical consequences to improve the quality of life in the involved patients.Methods:A retrospective case series study has been made on the forty five pediatric patients admitted to nephrology department of Ali-Asghar Hospital during a period of nearly 10 years.The patients have been divided into two groups of good and poor prognoses according to their clinical outcomes.The routine laboratory records and clinical manifestations extracted and statistically analyzed as independent variables both by univariate and multivariate methods.Results:Forty three patients have been managed successfully with only two deaths occurred.According to clinical findings,nineteen patients were classified as poor prognosis and the rest were categorized as good prognosis.Multivariate statistical analyses showed that lesser age at the time of admission(age【46 months,P【0.015) and the higher initial WBC count(count】15 000,P【0.226) were well-interrelated to ominous clinical consequences like convulsion,coma and peritonitis and statistically different between the two groups of patients.Conclusion:Despite the importance of predictive variables in the course of Hemolytic uremic syndrome(HUS) in children and their critical influence on the clinical outcome,many aspects of these parameters have been remained to be elucidated comprehensively.Our study showed that simultaneous low age of child at the time of admission with simultaneous high WBC count will result in the poorer prognoses of the patients.This may warn the clinicians to provide more supportive cares for this group of patients.展开更多
Giant cell hepatitis(GCH)with autoimmune hemolytic anemia is a rare entity,limited to young children,with an unknown pathogenesis.We report the case of 9-mo old who presented with fever,diarrhea and jaundice four days...Giant cell hepatitis(GCH)with autoimmune hemolytic anemia is a rare entity,limited to young children,with an unknown pathogenesis.We report the case of 9-mo old who presented with fever,diarrhea and jaundice four days before hospitalization.Physical examination found pallor,jaundice and hepatosplenomegaly.The laboratory workup showed serum total bilirubin at 101 μmol/L,conjugated bilirubin at 84 μmol/L,hemolytic anemia,thrombocytopenia and immunoglobulin G(IgG)and anti-C3d positive direct Coombs' test.The antinuclear,anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies,antiendomisium antibodies were negative.Serological assays for viral hepatitis B and C,cytomegalovirus,herpes simplex and Epstein Barr virus were negative.The association of acute liver failure,Evan's syndrome,positive direct Coomb's test of mixed type(IgG and C3)and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH.The diagnosis was confirmed by a needle liver biopsy.The patient was treated by corticosteroids,immunomodulatory therapy and azathioprine but died with septicemia.展开更多
Blooms of Phaeocystis globosa have been reported accountable for massive fi sh mortality worldwide.The toxigenic mechanisms of P.globosa,however,remain largely unclear due to the multiple structures and/or synergistic...Blooms of Phaeocystis globosa have been reported accountable for massive fi sh mortality worldwide.The toxigenic mechanisms of P.globosa,however,remain largely unclear due to the multiple structures and/or synergistic or antagonistic ef fects of hemolytic compounds.External stressors could lead to the regulation of photoprotective or antioxidative defense system,as well as the potential hemolytic activity.Therefore,the light-induced photosynthetic system,including the accessory photosynthetic growth,the relative electron transfer rate(ETR),photosynthetic e ffi ciency(F_(v)/F_(m)),quantum yield of photosystem II(Yield),together with the hemolytic activity of P.globosa were investigated under variable environmental conditions in the present study.Results confirmed that hemolytic activity of P.globosa was initiated by the light,but inhibited by low temperature(16℃),high light intensity(>100μmol/(m^(2)·s)),and iron-limited conditions.Interestingly,the hemolytic activity was not impacted by photosynthetic electron inhibitors(Diuron,atrazine,paraquat,and dibromothymoquinone),which signifi cantly inhibited the photosynthetic activity of P.globosa.The correlated response of hemolytic and photosynthetic activity of P.globosa under those environmental factors suggested that the hemolytic compounds of P.globosa would be involved in the photosynthetic process but not in the electron transfer chain of P.globosa.展开更多
Objective MicroRNAs are fine regulators for gene expression during the post-transcriptional stage in many autoimmune diseases.HypoxamiRs(miR-210 and miR-21)play an important role in hypoxia and in inflammation-associa...Objective MicroRNAs are fine regulators for gene expression during the post-transcriptional stage in many autoimmune diseases.HypoxamiRs(miR-210 and miR-21)play an important role in hypoxia and in inflammation-associated hypoxia.Systemic lupus erythematosus(SLE)is a chronic systemic autoimmune disease that would potentiate many pathological complications,including hemolytic anemia.This study aimed to investigate the role of hypoxamiRs in SLE/hemolytic anemia patients.Methods This work was designed to analyze the circulating levels of↱the miR-210 and miR-21 expressions and hypoxia-inducible factor-1α(HIF-α)in SLE/hemolytic anemia patients.SLE activity was evaluated for all patients by SLE Disease Activity Index(SLEDAI).Clinical manifestations/complications and serological/hematological investigations were reported.HIF-αconcentration was assayed by ELISA and expression of miR-21 and miR-210 was analyzed by qRT-PCR.Results The results indicated that the fold change of the miR-210/miR-21 expressions in plasma was significantly elevated in SLE/hemolytic anemia patients.A strong positive correlation between the miR-210 and miR-21 expression levels was also recorded.Among the associated-disease complications,hypertension,arthritis,oral ulcers,and serositis were associated with a high circulating miR-210 expression,while the occurrence of renal disorders was associated with the increased miR-21 expression.Furthermore,the HIF-αlevel was remarkably elevated in SLE/hemolytic anemia patients.A high positive correlation was recorded between the HIF-αconcentration and miR-210/miR-21 expression levels.The occurrence of oral ulcers,arthritis,and hypertension was associated with the increased HIF-αconcentration.On the other hand,SLEDAI and white blood cell count were positively correlated with miR-21/miR-210.The erythrocyte sedimentation rate was positively correlated with miR-21.Conclusion The dysregulation of the circulating miR-210/miR-210/HIF-1αlevels in SLE/hemolytic anemia patients advocated that the hypoxia pathway might have an essential role in the pathogenesis and complications of these diseases.展开更多
BACKGROUND The occurrence of Streptococcus pneumoniae-associated hemolytic uremic syndrome(SP-HUS)is increasing.Thomsen-Friedenreich antigen activation is highly involved in the pathogenesis of SP-HUS,and T-antibody-n...BACKGROUND The occurrence of Streptococcus pneumoniae-associated hemolytic uremic syndrome(SP-HUS)is increasing.Thomsen-Friedenreich antigen activation is highly involved in the pathogenesis of SP-HUS,and T-antibody-negative plasma exchange(PE)may be effective in the treatment of severe cases of SP-HUS.CASE SUMMARY We retrospectively reviewed two pediatric patients with SP-HUS.Both clinical features and laboratory examination results of the children were described.Tantibody-negative PE was performed in both cases.Both children made a full recovery after repeated PE and remained well at a 2 year follow-up.CONCLUSION Streptococcal pneumonia continues to be an uncommon but important cause of HUS.The successful treatment of the presented cases suggests that T-antibodynegative PE may benefit patients with SP-HUS.展开更多
BACKGROUND Severe refractory anemia during pregnancy can cause serious maternal and fetal complications.If the cause cannot be identified in time and accurately,blind symptomatic support treatment may cause serious ec...BACKGROUND Severe refractory anemia during pregnancy can cause serious maternal and fetal complications.If the cause cannot be identified in time and accurately,blind symptomatic support treatment may cause serious economic burden.Thalassemia minor pregnancy is commonly considered uneventful,and the condition of anemia rarely progresses during pregnancy.Autoimmune hemolytic anemia(AIHA)is rare during pregnancy with no exact incidence available.CASE SUMMARY We report the case of a 30-year-oldβ-thalassemia minor multiparous patient experiencing severe refractory anemia throughout pregnancy.We monitored the patient closely,carried out a full differential diagnosis,made a diagnosis of direct antiglobulin test-negative AIHA,and treated her with prednisone and intravenous immunoglobulin.The patient gave birth to a healthy full-term baby.CONCLUSION Coombs-negative AIHA should be suspected in cases of severe hemolytic anemia in pregnant patients with and without other hematological diseases.展开更多
Giant cell hepatitis(GCH)is characterized by large and multinucleated(syncytial)hepatocytes in the context of liver inflammation.Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of...Giant cell hepatitis(GCH)is characterized by large and multinucleated(syncytial)hepatocytes in the context of liver inflammation.Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity.The etiology is unknown;concomitant viral infections(as potential trigger factors)have been identified in a few patients.The pathogenesis reportedly relies upon immune-mediated/autoimmune mechanisms.This condition should be considered in any infant developing Coombs-positive anemia;indeed,anemia usually precedes the development of hepatitis.The clinical course is usually aggressive without the appropriate immunosuppressive therapy,which may include steroids,conventional immunosuppressors(e.g.,azathioprine and cyclophosphamide as first-line treatments),intravenous immunoglobulin,and biologics(rituximab).Improvements in medical management(including the availability of rituximab)have significantly reduced the mortality of this condition in the last decade.展开更多
文摘BACKGROUND Ulcerative colitis(UC)and systemic lupus erythematosus(SLE)are both systemic immunoreactive diseases,and their pathogenesis depends on the interaction between genes and environmental factors.There are no reports of UC with SLE in China,but six cases of SLE with UC have been reported in China.The combination of these two diseases has distinct effects on the pathogenesis of both diseases.CASE SUMMARY A female patient(30 years old)came to our hospital due to dull umbilical pain,diarrhea and mucous bloody stool in August 2018 and was diagnosed with UC.The symptoms were relieved after oral administration of mesalazine(1 g po tid)or folic acid(5 mg po qd),and the patient were fed a control diet.On June 24,2019,the patient was admitted for treatment due to anemia and tinnitus.During hospitalization,the patient had repeated low-grade fever and a progressively decreased Hb level.Blood tests revealed positive antinuclear antibody test,positive anti-dsDNA antibody,0.24 g/L C3(0.9-1.8 g/L),0.04 g/L C4(0.1-0.4 g/L),32.37 g/L immunoglobulin(8-17 g/L),and 31568.1 mg/24 h total 24-h urine protein(0-150 mg/24 h).The patient was diagnosed with SLE involving the joints,kidneys and blood system.Previously reported cases of SLE were retrieved from PubMed to characterize clinicopathological features and identify prognostic factors for SLE.CONCLUSION The patient was discharged in remission after a series of treatments,such as intravenous methylprednisolone sodium succinate,intravenous human immunoglobulin,cyclophosphamide injection,and plasma exchange.After discharge,the patient took oral prednisone acetate tablets,cyclosporine capsules,hydroxychloroquine sulfate tablets and other treatments for symptoms and was followed up regularly for 1 month,after which the patient's condition continued to improve and stabilize.
文摘Introduction: Moringa oleifera was a medicinal plant generally used by populations in the food and therapeutic fields. It’s used to treat anemia has been observed in the Djougou Zone in northern Benin. To our knowledge, there were no scientific data available that have evaluated its efficacy in the treatment of haemolytic anemia. This was what justifies this research work in which the phytochemical analysis, extraction and evaluation of the anti-anemic effect were carried out. Methods: Five groups of five Wistar rats each were formed. All the rats were rendered anemic by injection of phenylhydrazine hydrochloride on the first two days D0 and D1 except those in the negative control group. From the second day, the anemic groups were force-fed either with the aqueous extract of Moringa oleifera leaves at 200 or 300 mg/kg body weight/day, or with vitafer, the reference drug against anemia. The positive control group (anemia) was not treated. Blood samples were taken from all the rats on different days: D0, D2, D7, D10 and D15 to evaluate the data of the hemogram and the osmotic resistance of the red blood cells. Results: Phytochemical analysis revealed the presence of tannins, flavonoids, leucoanthocyanins, saponosides, triterpenes and mucilages. A good yield was obtained at the extraction. Both the extract and the reference drug vitafer completely corrected anemia within two weeks after stimulating hemoglobin synthesis and early release of immature red blood cells into the bloodstream. Its effect seemed dose-dependent and specific. Conclusion: Moringa oleifera leaves showed good therapeutic efficacy and can be considered and exploited for transformation into improved traditional medicines (ITM) in the treatment of anemia.
文摘Introduction: Acute Promyelocytic Leukemia (APL) is highly associated with hemostasis alterations. The atypical hemolytic uremic syndrome (aHUS) is a rare type of Thrombotic Microangiopathy (TMA) due to an overactivation of the alternative complement pathway. Case Presentation: A 48-years-old woman was diagnosed with APL and achieved molecular remission after induction therapy. During the second consolidation cycle she presented with TMA. She began treatment with plasma exchange plus corticotherapy but due to aggravation of symptoms Eculizumab was initiated. Thrombotic thrombocytopenic purpura, infections and drug toxicity causes were ruled out. There was no evidence of relapse of the APL. Genetic studies of the hereditary anomalies of the alternative complement pathway were negative and the decision of stopping Eculizumab was made. During maintenance therapy for the APL she presented a severe relapse of the aHUS, requiring dialysis. She re-started treatment with Eculizumab with a progressive hematologic recovery and improvement of renal function. She completed APL treatment without relapse of the leukemia for the moment and continues to be treated with Eculizumab. Conclusion: This is the first published case of coexisting aHUS and APL successfully treated with Eculizumab.
文摘BACKGROUND Anti-D antibody is not the common cause of Rh-isoimmunization in Chinese neonatal jaundice.Recent change in national population policy has followed by an increase in Rh-isoimmunization related hemolytic disease of the newborn(HDN).Unfortunately,regional status of Rh-HDN is unavailable.We hypothesize that Rh-HDN in our region is most commonly due to anti-E antibody.AIM To investigate the prevalence of hemolytic disease of the newborn due to Rhisoimmunization in Hefei City.METHODS Retrospective review of data obtained from Children’s Hospital of Anhui and Hefei Blood Center between January 2017 and June 2019.Status of minor blood group antibody was studied in the corresponding mothers.RESULTS Totally 4138 newborns with HDN admitted during the study period and 116(2.8%)received blood exchange transfusion(BET).Eighteen newborns(0.43%)with proven Rh-incompatible HDN were identified.All were not the first-born baby.Thirteen mothers were RhD(+)(72%)and five were RhD(-).The distribution of Rh-related antibodies in mothers was ten anti-E(55%),five anti-D(27%),and for one anti-C,anti-c,and anti-E/c(6%)each.Thirteen(72.2%)were qualified for BET,relative risk for BET was 28.9 as compared to other types of HDN,but only 10 received due to parenteral refusal.All(100%)RhD related HDN received BET which is not significantly different from RhE related HDN(81.8%).CONCLUSION As expected,all Rh-incompatible HDN newborns were not the first-born.Contrary to the Caucasian population,anti-D induced HDN is not the most common etiology.In our region,anti-E(11/18,61%)is the most common cause of Rh-HDN.
文摘AIM: To clarify the incidence of congenital hemolytic anemias (CHA) in young cholelithiasis patients and to determine a possible screening test based on the results. METHODS: Young cholelithiasis patients (< 35 years) were invited to our outpatient clinic. Participants were asked for comorbidities and family history. The number of gallstones were recorded. Blood samples were obtained to perform a complete blood count, standard Wright-Giemsa staining, reticulocyte count, hemoglobin (Hb) electrophoresis, serum lactate dehydrogenase and bilirubin levels, and lipid profile. RESULTS: Of 3226 cholecystectomy patients, 199 were under 35 years, and 190 with no diagnosis of CHA were invited to take part in the study. Fifty three patients consented to the study. The median age was 29 years (range, 17-35 years), 5 were male and 48 were female. Twelve patients (22.6%) were diagnosed as thalassemia trait and/or ?ron-deficiency anemia. Hblevels were significantly lower (P = 0.046), and mean corpuscular volume (MCV) and hematocrit levels were slightly lower (P = 0.072 and 0.082, respectively) than normal. There was also a significantly lower number of gallstones with the diagnosis (P = 0.007). CONCLUSION: In endemic regions, for young cholelithiasis patients (age under 35) with 2-5 gallstones, the clinician/surgeon should pay attention to MCV and Hb levels as indicative of CHA.
文摘BACKGROUND Thrombocytopenia associated with acute kidney injury is a challenging disorder. Thrombotic microangiopathy (TMA) is a potentially life- or organ-threatening syndrome that can be induced by several disorders or medical interventions. There is overlap between the clinical presentation and pathophysiology of thrombotic thrombocytopenia purpura and hemolytic uremic syndrome (HUS), and to a lesser extent, disseminated intravascular coagulation (DIC). We describe a case to illustrate the potential diagnostic difficulty, especially at initial presentation. CASE SUMMARY We reported a case of a 44-year-old woman that presented with diarrhea, thrombocytopenia, schistocytes, elevated serum lactate dehydrogenase (LDH) level and acute kidney injury. While the clinical presentation resembled that of Shiga toxin–induced HUS, the disease course was more consistent with gastrointestinal infection-related DIC. To aid in the accurate diagnosis of TMA and other associated disorders, we have undertaken a review and provided a clear interpretation of some typical biomarkers including schistocytes, LDH and platelet count, coagulation profile and more specific indexes of ADAMTS13, complement profile, and the isolation of Shiga toxin-producing Escherichia coli (commonly referred to as STEC). CONCLUSION The use and correct interpretation of classical indexes of schistocyte, LDH, and platelet count is vital in diagnosing TMA and associated disorders. Understanding the characteristics of these biomarkers in the context of thrombocytopenia purpura, HUS and DIC will facilitate the accurate diagnosis and early initiation of appropriate treatment.
文摘BACKGROUND Microangiopathic hemolytic anemia(MAHA) with thrombocytopenia and organ failure caused by tumor-associated thrombotic microangiopathy(TMA) is a lifethreatening oncological emergency. Rapid diagnosis and precise distinction from other forms of TMA is crucial for appropriate therapy, which aims at treating the underlying malignancy. However, the prognosis of patients with cancer-related(CR)-MAHA is limited. To date, less than 50 patients with gastric cancer and CRMAHA have been reported, mainly as single case reports, and detailed information on treatment strategies and outcome are scarce. We analyzed the characteristics and outcomes data of CR-MAHA patients with gastric cancer treated at our center between 2012 and 2019.AIM To gain knowledge about CR-MAHA and the course of disease.METHODS We retrospectively analyzed patients using an institutional prospectively maintained database. Patients who had CR-MAHA but other cancer types or cancer of unknown primary were excluded. The basic requirements for inclusion were: Histologically proven gastric adenocarcinoma;and clinical diagnosis of hemolytic anemia with schistocytes with or without thrombocytopenia. The observation period for each patient started with the first day of documented symptoms. The follow-up period for this analysis ended on February 1, 2020.RESULTS We identified eight patients with a median age of 54 years. Histologically, all patients had(partial) diffuse subtypes of gastric adenocarcinoma with partial or complete signet cell morphology. All patients had metastatic disease and one patient had a microsatellite instability-high(MSI-H) tumor. In three patients, clinical signs of MAHA preceded the diagnosis of cancer, and in two patients, CRMAHA indicated recurrent disease. All patients had severe hemolytic anemia and thrombocytopenia. Six patients experienced severe bone pain, and five patients had dyspnea. Systemic, 5-fluorouracil-based combination chemotherapy was initiated in six patients, which resulted in rapid initial response with significant improvement of clinical symptoms and blood values. Progression-free survival(PFS) of the whole cohort was 1.9 wk and median overall survival(OS) was 1.9 wk. For patients with chemotherapy, PFS was 9.0 wk and OS was 10.3 wk. The patient with the MSI-H tumor has been undergoing immunotherapy for more than 3 years.CONCLUSION The benefit of chemotherapy in CR-MAHA patients is limited. Immunotherapy for patients with MSI-H tumors may lead to long-term tumor control even in CRMAHA patients.
基金Supported by the Lanzhou Science and Technology Plan Project,No.2018-3-48Lanzhou Talent Innovation and Entrepreneurship Project,No.2019-RC-35and 2019 Special Fund for Doctoral Training of Lanzhou University Second Hospital,No.YJS-BD-15.
文摘BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.
文摘Pyelonephritis is an inflammation of the kidney tissue, calyces, and renal pelvis. It is commonly caused by bacterial infection that has spread up the urinary tract. Common bacteria are Escherichia coli and Enterococcus faecalis. Beta hemolytic streptococci also cause pyelonephritis. Beta hemolytic streptococcus such as Streptococcus agalactiae, and S. dysgalactiae subsp. equisimilis, when grown on blood-agar, produce complete hemolysis around each colony, usually cause severe infections including many cases of cellulitis, pneumonia, meningitis and sepsis. However the analysis of pyelonephritis causing beta hemolytic streptococcus has seldom been investigated. In this study, we tried to clarify the relationship between beta hemolytic streptococcus and pyelonephritis patient in Japan. We investigated beta hemolytic streptococcus isolated from pyelonephritis patient at two tertiary care hospitals in the central region of Japan by analyzing the background and antimicrobial susceptibility of beta hemolytic streptococcus. We recovered total sixteen beta hemolytic streptococcus in nine years. In pyelonephritis patients, beta hemolytic streptococcus was isolated from 9 S. agalactiae and 7 S. dysgalactiae subsp. equisimilis, respectively. The numbers of streptococci from female patients were more than those from male patients. Bacteria were not isolated from under 30 year patients. All S. dysgalactiae subsp. equisimilis were isolated from 60-year-age patients. About one fifth of beta hemolytic streptococcus had erythromycin and minocycline-resistant ability. Four beta hemolytic streptococci that were all S. agalactiae, had levofloxacin-resistant ability. Only one S. agalactiae had trimethoprim-sulfamethoxazole-resistant ability. Ampicillin was effective against all beta hemolytic streptococci. In our results, beta hemolytic streptococcus caused pyelonephritis in aged people and the quarter of these bacteria had some antibiotics resistance.
文摘BACKGROUND Wilson’s disease(WD)is a rare autosomal recessive inherited disorder of copper metabolism.Acute liver failure(ALF)and hemolytic anemia represent the most severe presentation of WD in children.No clear genotype-phenotype correlations exist in WD.Protein-truncating nonsense,frame-shift,or splice-site variants may be associated with more severe disease.In contrast,missense variants may be associated with late-onset,less severe disease,and more neurological manifestations.Recently,a gene variant(HSD17B13:TA,rs72613567)with a possible hepatic protective role against toxins was associated with a less severe hepatic phenotype in WD.AIM To analyze the possible genotype-phenotype correlations in children with WD presented with ALF and non-immune hemolytic anemia.METHODS The medical records of children with WD diagnosed and treated in our hospital from January 2006 to December 2020 were retrospectively analyzed.The clinical manifestations(ALF with non-immune hemolytic anemia or other less severe forms),laboratory parameters,copper metabolism,ATP7B variants,and the HSD17B13:TA(rs72613567)variant were reviewed to analyze the possible genotype-phenotype correlations.RESULTS We analyzed the data of 51 patients with WD,26 females(50.98%),with the mean age at the diagnosis of 12.36±3.74 years.ALF and Coombs-negative hemolytic anemia was present in 8 children(15.67%),all adolescent girls.The Kayser-Fleisher ring was present in 9 children(17.65%).The most frequent variants of the ATP7B gene were p.His1069Gln(c.3207A>G)in 38.24% of all alleles,p.Gly1341Asp(c.4021G>A)in 26.47%,p.Trp939Cys(c.2817G>T)in 9.80%,and p.Lys844Ter(c.2530A>T)in 4.90%.In ALF with hemolytic anemia,p.Trp939Cys(c.2817G>T)and p.Lys844Ter(c.2530A>T)variants were more frequent than in other less severe forms,in which p.His1069Gln(c.3207A>G)was more frequent.p.Gly1341Asp(c.4021G>A)has a similar frequency in all hepatic forms.For 33 of the patients,the HSD17B13 genotype was evaluated.The overall HSD17B13:TA allele frequency was 24.24%.Its frequency was higher in patients with less severe liver disease(26.92%)than those with ALF and hemolytic anemia(14.28%).CONCLUSION It remains challenging to prove a genotype-phenotype correlation in WD patients.In children with ALF and hemolytic anemia,the missense variants other than p.His1069Gln(c.3207A>G)and frame-shift variants were the most frequently present in homozygous status or compound heterozygous status with site splice variants.As genetic analysis is usually time-consuming and the results are late,the importance at the onset of the ALF is questionable.If variants proved to be associated with severe forms are found in the pre-symptomatic phase of the disease,this could be essential to predict a possible severe evolution.
文摘Fluorine-doped hydrogenated amorphous carbon (a-C:H:F) film was deposited on a flow-straightener, impeller and diffuser surface (SUS 304) of an enclosed-impeller type flow blood pump using the ionization deposition method with a source gas of C6F5H. The surface characteristics of the a-C:H:F film were examined using atomic force microscopy, X-ray photoelectron spectroscopy, and measurements of surface roughness, friction and surface potential. The a-C:H:F film tends to increase surface roughness and the negative surface charge. In addition, the surface energy and friction decrease with fluorine dopant in the a-C:H film. To estimate the hemolytic performance of a blood pump with the a-C:H:F film coating, the amount of hemolysis was measured using a mock circulatory system (in vitro test) with 500 mL of pig blood containing sodium citrate. In vitro test was conducted for 180 min with the blood flow and pump head maintained at 5 L/min and 100 mmHg, respectively. The a-C:H:F film coating reduced the amount of hemolysis and improved the hemolytic performance. Decreasing the surface energy and negative surface charge of the a-C:H:F film contributes to the improvement of the hemolytic performance. The a-C:H:F film coating is thus expected to be utilized in medical technology as a surface coating technology for artificial heart blood pumps.
文摘Drug-induced immune hemolytic anemia (DIIHA) is commonly attributed to cephalosporins. Ceftriaxone is the most frequently administered cephalosporin in patients with sickle cell disease. We present a pediatric patient with severe DIIHA (hemoglobin < 2 g/dl) who survived. Since DIIHA often goes undiagnosed until late in the course, vigilance of DIIHA minimizes unnecessary diagnostic tests and therapies. DIIHA likely remains under-recognized in all patient subpopulations due to its rarity and overlapping presentations with other conditions. Distinction between exacerbation of chronic hemolysis and new onset of acute hemolysis poses a unique challenge in patients with sickle cell disease. A thorough analysis is warranted to better identify factors within the pediatric sickle cell population that may increase the predisposition for DIIHA, particularly due to ceftriaxone.
文摘A Chinese woman of blood group B,D-and her husband of blood group AB,CCDeewere examined.The woman had not been transfused before.Their first two babiesdied.Anti-Hro and anti-e were found in the mother’s serum.During her third pregnancy,the titer of antibodies went up quickly,approximately one titer per month.After 36 weeksof pregnancy,the baby was delivered by Caesarean section.The cord blood Hb was 88g/L,his red blood cell count 2.7×10<sup>12</sup>/L,and total biIirubin 114.6 mol/L.The baby was ofblood group AB,and CDe-D-genotype.Exchangetransfusion was begun 2.5 hours afterbirth.O,ccDEE washed red cells together with group AB plasma were used.Two dayslater,7Oml washed O,ccDEE concentrated red cells were administered.The baby is aliveand in good health.
文摘Objective:To decrease or delay the major un-wanted clinical consequences to improve the quality of life in the involved patients.Methods:A retrospective case series study has been made on the forty five pediatric patients admitted to nephrology department of Ali-Asghar Hospital during a period of nearly 10 years.The patients have been divided into two groups of good and poor prognoses according to their clinical outcomes.The routine laboratory records and clinical manifestations extracted and statistically analyzed as independent variables both by univariate and multivariate methods.Results:Forty three patients have been managed successfully with only two deaths occurred.According to clinical findings,nineteen patients were classified as poor prognosis and the rest were categorized as good prognosis.Multivariate statistical analyses showed that lesser age at the time of admission(age【46 months,P【0.015) and the higher initial WBC count(count】15 000,P【0.226) were well-interrelated to ominous clinical consequences like convulsion,coma and peritonitis and statistically different between the two groups of patients.Conclusion:Despite the importance of predictive variables in the course of Hemolytic uremic syndrome(HUS) in children and their critical influence on the clinical outcome,many aspects of these parameters have been remained to be elucidated comprehensively.Our study showed that simultaneous low age of child at the time of admission with simultaneous high WBC count will result in the poorer prognoses of the patients.This may warn the clinicians to provide more supportive cares for this group of patients.
文摘Giant cell hepatitis(GCH)with autoimmune hemolytic anemia is a rare entity,limited to young children,with an unknown pathogenesis.We report the case of 9-mo old who presented with fever,diarrhea and jaundice four days before hospitalization.Physical examination found pallor,jaundice and hepatosplenomegaly.The laboratory workup showed serum total bilirubin at 101 μmol/L,conjugated bilirubin at 84 μmol/L,hemolytic anemia,thrombocytopenia and immunoglobulin G(IgG)and anti-C3d positive direct Coombs' test.The antinuclear,anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies,antiendomisium antibodies were negative.Serological assays for viral hepatitis B and C,cytomegalovirus,herpes simplex and Epstein Barr virus were negative.The association of acute liver failure,Evan's syndrome,positive direct Coomb's test of mixed type(IgG and C3)and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH.The diagnosis was confirmed by a needle liver biopsy.The patient was treated by corticosteroids,immunomodulatory therapy and azathioprine but died with septicemia.
基金Supported by the National Key R&D Program of China(Nos.2016YFC1402104,2019YFC1407900)the Special Foundation for National Science and Technology Basic Research Program of China(No.2018FY100201)+3 种基金the Hainan Provincial Joint Project of Sanya Yazhou Bay Science and Technology City(No.420LH004)the National Natural Science Foundation of China(No.41276091)the Program of Bureau of Science and Technology of Zhoushan(No.2019C8103)a Tang scholarship provided to Mengmeng TONG。
文摘Blooms of Phaeocystis globosa have been reported accountable for massive fi sh mortality worldwide.The toxigenic mechanisms of P.globosa,however,remain largely unclear due to the multiple structures and/or synergistic or antagonistic ef fects of hemolytic compounds.External stressors could lead to the regulation of photoprotective or antioxidative defense system,as well as the potential hemolytic activity.Therefore,the light-induced photosynthetic system,including the accessory photosynthetic growth,the relative electron transfer rate(ETR),photosynthetic e ffi ciency(F_(v)/F_(m)),quantum yield of photosystem II(Yield),together with the hemolytic activity of P.globosa were investigated under variable environmental conditions in the present study.Results confirmed that hemolytic activity of P.globosa was initiated by the light,but inhibited by low temperature(16℃),high light intensity(>100μmol/(m^(2)·s)),and iron-limited conditions.Interestingly,the hemolytic activity was not impacted by photosynthetic electron inhibitors(Diuron,atrazine,paraquat,and dibromothymoquinone),which signifi cantly inhibited the photosynthetic activity of P.globosa.The correlated response of hemolytic and photosynthetic activity of P.globosa under those environmental factors suggested that the hemolytic compounds of P.globosa would be involved in the photosynthetic process but not in the electron transfer chain of P.globosa.
基金supported by the Taif University Researchers Supporting Project(No.TURSP-2020/103).
文摘Objective MicroRNAs are fine regulators for gene expression during the post-transcriptional stage in many autoimmune diseases.HypoxamiRs(miR-210 and miR-21)play an important role in hypoxia and in inflammation-associated hypoxia.Systemic lupus erythematosus(SLE)is a chronic systemic autoimmune disease that would potentiate many pathological complications,including hemolytic anemia.This study aimed to investigate the role of hypoxamiRs in SLE/hemolytic anemia patients.Methods This work was designed to analyze the circulating levels of↱the miR-210 and miR-21 expressions and hypoxia-inducible factor-1α(HIF-α)in SLE/hemolytic anemia patients.SLE activity was evaluated for all patients by SLE Disease Activity Index(SLEDAI).Clinical manifestations/complications and serological/hematological investigations were reported.HIF-αconcentration was assayed by ELISA and expression of miR-21 and miR-210 was analyzed by qRT-PCR.Results The results indicated that the fold change of the miR-210/miR-21 expressions in plasma was significantly elevated in SLE/hemolytic anemia patients.A strong positive correlation between the miR-210 and miR-21 expression levels was also recorded.Among the associated-disease complications,hypertension,arthritis,oral ulcers,and serositis were associated with a high circulating miR-210 expression,while the occurrence of renal disorders was associated with the increased miR-21 expression.Furthermore,the HIF-αlevel was remarkably elevated in SLE/hemolytic anemia patients.A high positive correlation was recorded between the HIF-αconcentration and miR-210/miR-21 expression levels.The occurrence of oral ulcers,arthritis,and hypertension was associated with the increased HIF-αconcentration.On the other hand,SLEDAI and white blood cell count were positively correlated with miR-21/miR-210.The erythrocyte sedimentation rate was positively correlated with miR-21.Conclusion The dysregulation of the circulating miR-210/miR-210/HIF-1αlevels in SLE/hemolytic anemia patients advocated that the hypoxia pathway might have an essential role in the pathogenesis and complications of these diseases.
文摘BACKGROUND The occurrence of Streptococcus pneumoniae-associated hemolytic uremic syndrome(SP-HUS)is increasing.Thomsen-Friedenreich antigen activation is highly involved in the pathogenesis of SP-HUS,and T-antibody-negative plasma exchange(PE)may be effective in the treatment of severe cases of SP-HUS.CASE SUMMARY We retrospectively reviewed two pediatric patients with SP-HUS.Both clinical features and laboratory examination results of the children were described.Tantibody-negative PE was performed in both cases.Both children made a full recovery after repeated PE and remained well at a 2 year follow-up.CONCLUSION Streptococcal pneumonia continues to be an uncommon but important cause of HUS.The successful treatment of the presented cases suggests that T-antibodynegative PE may benefit patients with SP-HUS.
文摘BACKGROUND Severe refractory anemia during pregnancy can cause serious maternal and fetal complications.If the cause cannot be identified in time and accurately,blind symptomatic support treatment may cause serious economic burden.Thalassemia minor pregnancy is commonly considered uneventful,and the condition of anemia rarely progresses during pregnancy.Autoimmune hemolytic anemia(AIHA)is rare during pregnancy with no exact incidence available.CASE SUMMARY We report the case of a 30-year-oldβ-thalassemia minor multiparous patient experiencing severe refractory anemia throughout pregnancy.We monitored the patient closely,carried out a full differential diagnosis,made a diagnosis of direct antiglobulin test-negative AIHA,and treated her with prednisone and intravenous immunoglobulin.The patient gave birth to a healthy full-term baby.CONCLUSION Coombs-negative AIHA should be suspected in cases of severe hemolytic anemia in pregnant patients with and without other hematological diseases.
文摘Giant cell hepatitis(GCH)is characterized by large and multinucleated(syncytial)hepatocytes in the context of liver inflammation.Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity.The etiology is unknown;concomitant viral infections(as potential trigger factors)have been identified in a few patients.The pathogenesis reportedly relies upon immune-mediated/autoimmune mechanisms.This condition should be considered in any infant developing Coombs-positive anemia;indeed,anemia usually precedes the development of hepatitis.The clinical course is usually aggressive without the appropriate immunosuppressive therapy,which may include steroids,conventional immunosuppressors(e.g.,azathioprine and cyclophosphamide as first-line treatments),intravenous immunoglobulin,and biologics(rituximab).Improvements in medical management(including the availability of rituximab)have significantly reduced the mortality of this condition in the last decade.
