BACKGROUND Asplenia,the lack of a spleen,can be congenital and increases susceptibility to severe infections caused by encapsulated bacteria,such as Streptococcus pneumoniae(S.pneumoniae).We report two cases of severe...BACKGROUND Asplenia,the lack of a spleen,can be congenital and increases susceptibility to severe infections caused by encapsulated bacteria,such as Streptococcus pneumoniae(S.pneumoniae).We report two cases of severe pneumococcal infection in two asplenic family members living in the same household.CASE SUMMARY Patient 1,a 38-year-old man with a history of congenital hepatitis B infection and hypospadias,was brought to our emergency department with complaints of cyanosis,cough,and edema of his limbs.He was clinically diagnosed as hyposplenic with overwhelming pneumococcal sepsis.He was admitted to the intensive care unit and was administered antibiotics and catecholaminergic therapy but died 2 h after admission.Patient 2,a 63-year-old woman with a history of type 2 diabetes,was brought to our emergency department one month after admission of Patient 1.She was diagnosed as asplenic with overwhelming pneumococcal sepsis.History-taking revealed that she was the mother of Patient 1 and the two had lived in the same household.She was admitted to the intensive care unit and was rapidly provided antibiotics and catecholaminergic intervention but died one day after admission.CONCLUSION Pneumococcal bacteremia caused by virulent S.pneumoniae may be transmitted within households.All residents of households where individuals with pneumococcal bacteremia are living should be educated about the risk of transmissibility.Family members of patients with congenital asplenia/hyposplenia,all family members should be examined to assess their splenic function.展开更多
BACKGROUND Asplenia-type heterotaxy syndrome(HS)is rare and refers to visceral malposition and dysmorphism.It is associated with a high infant mortality rate due to cardiac anomalies,and related digestive endoscopic i...BACKGROUND Asplenia-type heterotaxy syndrome(HS)is rare and refers to visceral malposition and dysmorphism.It is associated with a high infant mortality rate due to cardiac anomalies,and related digestive endoscopic interventions are poorly understood.With the improved long-term prognosis of these individuals after modern cardiac surgery,intra-abdominal anomalies have become increasingly significant.CASE SUMMARY Herein,we report successful endoscopic retrograde cholangiopancreatography(ERCP)in a 14-year-old male with asplenia-type HS that involved unique imaging findings and technical difficulties.His anatomic anomalies included complex congenital heart disease,midline liver placement,an absent spleen,a left-sided inferior vena cava,and dextroposition of the stomach and pancreas.He suffered from choledocholithiasis with obstructive jaundice,and the stone was successfully extracted with a basket following endoscopic papillary balloon dilation.CONCLUSION Although anatomic anomalies in HS increase the degree of technical difficulty when performing ERCP,they can be safely managed by experienced endosco-pists,as illustrated by the present case.Identifying these variations with imaging modalities and being aware of them before initiating an invasive intervention are crucial to preventing potential complications.展开更多
Background:Asplenia vaccination is employed before ABO-incompatible(ABOi)transplantation in case splenectomy is needed.Pneumococcal vaccines have been reported,in different patient groups,to increase anti-A/B titers,t...Background:Asplenia vaccination is employed before ABO-incompatible(ABOi)transplantation in case splenectomy is needed.Pneumococcal vaccines have been reported,in different patient groups,to increase anti-A/B titers,through cross-reactivity to shared polysaccharide epitopes.We investigated the impact of pretransplant asplenia vaccinations on anti-A/B antibody titers in prospective ABOi renal transplant recipients.Methods:Published asplenia vaccine microbial structures were reviewed to assess expression of A/B antigens.All patients who underwent ABOi transplantation at Monash Medical Centre with anti-A/B titers taken either side of asplenia vaccination were included in a retrospective cohort study between 2007 and 2021.Patients with paired titers without intervening vaccination were included as controls.Paired titers were compared within groups.Results:A and B antigens were found to be expressed by vaccine specific pneumococcal serotypes.Thirty-nine ABOi renal transplant recipients received vaccination including at least one pneumococcal vaccine.The most common donor to recipient combination was A1 to O.The median pre-and postvaccination anti-A/B titers were 1:32 and there was no significant change in titers following vaccination(median change in titer 0 dilutions,range–2 to 3,P=0.43).The same findings were apparent in the control group(n=20).There was no significant change in titer by donor blood group or vaccine type.No transplants were canceled or delayed by a rise in anti-A/B titers postvaccination.Conclusion:Pneumococcal vaccination had no clinically relevant impact on anti-A/B titers before ABOi transplantation in this cohort.These results provide reassurance regarding the safety of asplenia vaccination before ABOi transplantation.展开更多
Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal organs.We aimed ...Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal organs.We aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism,seeking to determine whether it was feasible to assess complexity on this basis,in this regard taking note of the potential value of bronchial as opposed to appendage morphology.Methods and Results:We studied 78 patients known to have isomerism of the bronchuses,43 with right and 35 with left isomerism.Appendage anatomy could be determined in 49 cases(63%),all but one of these being concordant with bronchial anatomy.When assessing abdominal features,in only 59 cases(76%)was splenic morphology in keeping with the thoracic findings.As expected,right isomerism was associated with greater complexity of cardiac malformations,with an odds ratio of 6.53,with confidence intervals from 2.2–19.3(p<0.001).The odds were slightly decreased with thoraco-abdominal disharmony,when lesions shown to carry higher risk were then found in the setting of left isomerism.Conclusion:Harmony is excellent between bronchial and appendage isomerism,but less so with the arrangement of the abdominal organs.Right isomerism in our cohort,was indicative of a sixfold increase in intracardiac complexity.When discordance was found between the systems,however,the cardiac anomalies were less typical of the anticipated findings for right vs.left isomerism of the appendages.展开更多
Coronavirus disease 2019(COVID-19),caused by the novel severe acute respiratory syndrome coronavirus 2(SARS-CoV-2),is an acute infectious disease that spreads mainly through the respiratory route.Besides interstitial ...Coronavirus disease 2019(COVID-19),caused by the novel severe acute respiratory syndrome coronavirus 2(SARS-CoV-2),is an acute infectious disease that spreads mainly through the respiratory route.Besides interstitial pneumonia,a number of other clinical manifestations were noticed in COVID-19 patients.In particular,liver and spleen dysfunctions have been described both as complications of COVID-19 and as potential predisposing factors for severe COVID-19.Liver damage is rather common in COVID-19 patients,and it is most likely multifactorial,caused by the direct insult of SARS-CoV-2 to the liver by the cytokine storm triggered by the virus,by the use of hepatotoxic drugs,and as a consequence of hypoxia.Although generally mild,liver impairment has been found to be associated with a higher rate of intensive care unit admission.A higher mortality rate was reported among chronic liver disease patients.Instead,spleen impairment in patients with COVID-19 has been poorly described.The main anatomical changes are the architectural derangement of the B cell compartment,white pulp atrophy,and reduction or absence of lymphoid follicles,while,from a functional point of view,the IgM memory B cell pool is markedly depleted.The outcome of COVID-19 in asplenic or hyposplenic patients is yet to be defined.In this review,we will summarise the current knowledge regarding the impact of SARS-CoV-2 on the liver and spleen function,as well as the outcome of patients with a pre-existent liver disease or defective spleen function.展开更多
文摘BACKGROUND Asplenia,the lack of a spleen,can be congenital and increases susceptibility to severe infections caused by encapsulated bacteria,such as Streptococcus pneumoniae(S.pneumoniae).We report two cases of severe pneumococcal infection in two asplenic family members living in the same household.CASE SUMMARY Patient 1,a 38-year-old man with a history of congenital hepatitis B infection and hypospadias,was brought to our emergency department with complaints of cyanosis,cough,and edema of his limbs.He was clinically diagnosed as hyposplenic with overwhelming pneumococcal sepsis.He was admitted to the intensive care unit and was administered antibiotics and catecholaminergic therapy but died 2 h after admission.Patient 2,a 63-year-old woman with a history of type 2 diabetes,was brought to our emergency department one month after admission of Patient 1.She was diagnosed as asplenic with overwhelming pneumococcal sepsis.History-taking revealed that she was the mother of Patient 1 and the two had lived in the same household.She was admitted to the intensive care unit and was rapidly provided antibiotics and catecholaminergic intervention but died one day after admission.CONCLUSION Pneumococcal bacteremia caused by virulent S.pneumoniae may be transmitted within households.All residents of households where individuals with pneumococcal bacteremia are living should be educated about the risk of transmissibility.Family members of patients with congenital asplenia/hyposplenia,all family members should be examined to assess their splenic function.
文摘BACKGROUND Asplenia-type heterotaxy syndrome(HS)is rare and refers to visceral malposition and dysmorphism.It is associated with a high infant mortality rate due to cardiac anomalies,and related digestive endoscopic interventions are poorly understood.With the improved long-term prognosis of these individuals after modern cardiac surgery,intra-abdominal anomalies have become increasingly significant.CASE SUMMARY Herein,we report successful endoscopic retrograde cholangiopancreatography(ERCP)in a 14-year-old male with asplenia-type HS that involved unique imaging findings and technical difficulties.His anatomic anomalies included complex congenital heart disease,midline liver placement,an absent spleen,a left-sided inferior vena cava,and dextroposition of the stomach and pancreas.He suffered from choledocholithiasis with obstructive jaundice,and the stone was successfully extracted with a basket following endoscopic papillary balloon dilation.CONCLUSION Although anatomic anomalies in HS increase the degree of technical difficulty when performing ERCP,they can be safely managed by experienced endosco-pists,as illustrated by the present case.Identifying these variations with imaging modalities and being aware of them before initiating an invasive intervention are crucial to preventing potential complications.
文摘Background:Asplenia vaccination is employed before ABO-incompatible(ABOi)transplantation in case splenectomy is needed.Pneumococcal vaccines have been reported,in different patient groups,to increase anti-A/B titers,through cross-reactivity to shared polysaccharide epitopes.We investigated the impact of pretransplant asplenia vaccinations on anti-A/B antibody titers in prospective ABOi renal transplant recipients.Methods:Published asplenia vaccine microbial structures were reviewed to assess expression of A/B antigens.All patients who underwent ABOi transplantation at Monash Medical Centre with anti-A/B titers taken either side of asplenia vaccination were included in a retrospective cohort study between 2007 and 2021.Patients with paired titers without intervening vaccination were included as controls.Paired titers were compared within groups.Results:A and B antigens were found to be expressed by vaccine specific pneumococcal serotypes.Thirty-nine ABOi renal transplant recipients received vaccination including at least one pneumococcal vaccine.The most common donor to recipient combination was A1 to O.The median pre-and postvaccination anti-A/B titers were 1:32 and there was no significant change in titers following vaccination(median change in titer 0 dilutions,range–2 to 3,P=0.43).The same findings were apparent in the control group(n=20).There was no significant change in titer by donor blood group or vaccine type.No transplants were canceled or delayed by a rise in anti-A/B titers postvaccination.Conclusion:Pneumococcal vaccination had no clinically relevant impact on anti-A/B titers before ABOi transplantation in this cohort.These results provide reassurance regarding the safety of asplenia vaccination before ABOi transplantation.
文摘Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal organs.We aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism,seeking to determine whether it was feasible to assess complexity on this basis,in this regard taking note of the potential value of bronchial as opposed to appendage morphology.Methods and Results:We studied 78 patients known to have isomerism of the bronchuses,43 with right and 35 with left isomerism.Appendage anatomy could be determined in 49 cases(63%),all but one of these being concordant with bronchial anatomy.When assessing abdominal features,in only 59 cases(76%)was splenic morphology in keeping with the thoracic findings.As expected,right isomerism was associated with greater complexity of cardiac malformations,with an odds ratio of 6.53,with confidence intervals from 2.2–19.3(p<0.001).The odds were slightly decreased with thoraco-abdominal disharmony,when lesions shown to carry higher risk were then found in the setting of left isomerism.Conclusion:Harmony is excellent between bronchial and appendage isomerism,but less so with the arrangement of the abdominal organs.Right isomerism in our cohort,was indicative of a sixfold increase in intracardiac complexity.When discordance was found between the systems,however,the cardiac anomalies were less typical of the anticipated findings for right vs.left isomerism of the appendages.
文摘Coronavirus disease 2019(COVID-19),caused by the novel severe acute respiratory syndrome coronavirus 2(SARS-CoV-2),is an acute infectious disease that spreads mainly through the respiratory route.Besides interstitial pneumonia,a number of other clinical manifestations were noticed in COVID-19 patients.In particular,liver and spleen dysfunctions have been described both as complications of COVID-19 and as potential predisposing factors for severe COVID-19.Liver damage is rather common in COVID-19 patients,and it is most likely multifactorial,caused by the direct insult of SARS-CoV-2 to the liver by the cytokine storm triggered by the virus,by the use of hepatotoxic drugs,and as a consequence of hypoxia.Although generally mild,liver impairment has been found to be associated with a higher rate of intensive care unit admission.A higher mortality rate was reported among chronic liver disease patients.Instead,spleen impairment in patients with COVID-19 has been poorly described.The main anatomical changes are the architectural derangement of the B cell compartment,white pulp atrophy,and reduction or absence of lymphoid follicles,while,from a functional point of view,the IgM memory B cell pool is markedly depleted.The outcome of COVID-19 in asplenic or hyposplenic patients is yet to be defined.In this review,we will summarise the current knowledge regarding the impact of SARS-CoV-2 on the liver and spleen function,as well as the outcome of patients with a pre-existent liver disease or defective spleen function.
