asplenia

Capnocytophaga canimorsus infection is frequently associated with dog and cat bites or scratches in patients who have risk factors such as immunosuppression, asplenia, and alcohol abuse. However, rare instances of C. canimorsus  infection in patients without typical risk factors have been reported. Here, we present such a rare and unusual case of C. canimorsus  bacteremia in a patient without animal wounds or risk factors. Chronic sinusitis may have contributed to mucosal disruption and served as an entry point for C...

This report describes the unique case of a completely patent Double Aortic Arch (DAA) combined with Kommerell Diverticulum (KD), absence of celiac trunk and congenital asplenia. The anatomical variants described were identified and assessed in a 51-year old female from a computed-tomography angiography (CTA) images with 3D-volume rendered reconstructions during her follow-up after hysterectomy. The reconstructed CTA images showed a DAA with the left common carotid artery stemming from the hypoplastic left aortic arch while the left subclavian artery originated from a KD in the descending thoracic aorta...

We describe severe post-treatment episodes that complicate Plasmodium falciparum malaria in patients with hyposplenism, characterized by prolonged circulation of red cells containing dead parasites (hearse red cells), persistent or recurrent fever, profound anaemia, and inflammation. Asplenia-related Post-antimalarial Inflammation & Hemolysis (APIH) mimics concomitant infection and parasite resistance to drugs.

A single common pulmonary vein is a rare anomaly, mostly occurring with asplenia. We wanted to report our case because it co-exists with polysplenia and ciliary dyskinesia.A baby girl about 2 months old with respiratory arrest was admitted to the emergency room of our hospital. She responded to pulmonary resuscitation. We ascertained from her medical history that she had been hospitalised twice because of recurrent lung infections. After the initial check and treatment, we applied imaging modalities to detect underlying problems of recurrent lung infection...

Sickle cell disease (SCD) is an inherited autosomal recessive hemoglobin disorder caused by the presence of hemoglobin S, a mutant abnormal hemoglobin caused by a nucleotide change in codon 6 of the β-globin chain gene. SCD involves a chronic inflammatory state, exacerbated during vaso-occlusive crises, which leads to end-organ damage that occurs throughout the lifespan. SCD is associated with premature mortality in the first years of life. The process of sickling provokes asplenia in the first years of life with an increased risk of infection by encapsulated germs...

We present a unique case of a fetus with dextrocardia, asplenia and a right bilobed lung in a primigravida woman in her 20s at 21 weeks' gestation. Prenatal ultrasound examination revealed dextrocardia and other anomalies such as atrioventricular septal defect and situs ambiguous with the gallbladder on the left, leading to termination of the pregnancy. Fetal autopsy confirmed the diagnosis, detected additional findings such as asplenia and right bilobed lung missed on ultrasound and highlighted the importance of autopsy in prenatal diagnosis...

PURPOSE: To determine whether a novel intervention improves the adherence to guideline-based preventive measures in asplenic patients at risk of post-splenectomy sepsis (PSS). METHODS: We used a prospective controlled, two-armed historical control group design to compare a novel, health action process approach (HAPA)-based telephonic intervention involving both patients and their general practitioners to usual care. Eligible patients were identified in cooperation with the insurance provider AOK Baden-Wuerttemberg, Germany...

Sickle cell disease patients are prone to infection and overwhelming sepsis because an immunedeficient state arises from asplenia (autosplenectomy/surgical splenectomy) and functional hyposplenism. The common pathogen encountered in sepsis with asplenic/hyposplenism patients is encapsulated organism, gram-negative bacilli, but in developing countries like India, there are many possibilities of infection by an uncommon organism that make it difficult to diagnose. Here, we have described a case of sickle cell disease presented with persistent fever and later, found to have an atrophic spleen with involvement of respiratory system and osteoarticular system...

Immunocompromised patients (IPs) are at high risk for infections, some of which are vaccine-preventable. The Israeli Ministry of Health recommends pneumococcal conjugate vaccine 13 (PCV13) and pneumococcal polysaccharide vaccine 23 (PPSV23) for IP, but vaccine coverage is suboptimal. We assessed the project's effectiveness in improving the pneumococcal vaccination rate among IP. An automated population-based registry of IP was developed and validated at Maccabi Healthcare Services, an Israeli health maintenance organization serving over 2...

The spleen is located in the left upper quadrant of the abdominal cavity and has both immunological and haematological functions. A variety of quite different entities may be associated with sudden death due to splenic involvement. These range from simple traumatic disruption of the parenchyma of a normal spleen with marked intra-abdominal haemorrhage, to conditions such as malaria and infectious mononucleosis where splenomegaly predisposes to rupture. Haematological diseases such as malignancies and haemoglobinopathies may causes sudden death due to haemorrhage or red cell sequestration...

Nuclear receptor subfamily 2 group F member 2 (NR2F2 or COUP-TF2) encodes a transcription factor which is expressed at high levels during mammalian development. Rare heterozygous Mendelian variants in NR2F2 were initially identified in individuals with congenital heart disease (CHD), then subsequently in cohorts of congenital diaphragmatic hernia (CDH) and 46,XX ovotesticular disorders/differences of sexual development (DSD); however, the phenotypic spectrum associated with pathogenic variants in NR2F2 remains poorly characterized...

BACKGROUND: To explore the diagnostic clues and abnormality spectrum of heterotaxy syndrome by prenatal ultrasonography and postnatal verification. METHODS: The prenatal ultrasonic data of 88 heterotaxy syndrome fetuses were analyzed retrospectively as left isomerism (LI) and right isomerism (RI). Prenatal ultrasound compared with the anatomical casting of the fetal body after labor induction, and the confirmatory postnatal diagnosis after delivery. RESULTS: Fetal LI showed typical malformations of gastric vesicles on different sides from the heart, absence of hepatic segment of the inferior vena cava (IVC), abdominal aorta (AO) parallel with the azygos vein (AV), bilateral left bronchus, bilateral left atrial appendages, and polysplenia; intracardiac malformations of AV septal defects (AVSD), single atrium (SA), left ventricular outflow tract obstruction (LVOTO), and double-outlet right ventricle (DORV); and cardiac conduction abnormalities of sinus bradycardia and AV blockage...

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BACKGROUND: In settings with universal conjugate pneumococcal vaccination, invasive pneumococcal disease (IPD) can be a marker of an underlying inborn error of immunity. The aim of this study was to determine the prevalence and characterize the types of immunodeficiencies in children presenting with IPD. METHODS: Multicenter prospective audit following the introduction of routinely recommended immunological screening in children presenting with IPD. The minimum immunological evaluation comprised a full blood examination and film, serum immunoglobulins (IgG, IgA and IgM), complement levels and function...

A 44-year-old male with history of asplenia, provoked PE, and hyperlipidemia presented with ascending paralysis, bowel and bladder incontinence and altered mental status, and progressively developed acute hypoxic respiratory failure. Initial workup including CT head, magnetic resonance imaging (MRI) brain, and lumbar puncture which was concerning for herpes simplex virus (HSV) meningoencephalitis; out of caution he was started on multiple antibiotics consequently resulting in the development of Clostridium difficile (C...

In this study, we aim to explore the outcomes of Covid-19 infection in patients with Hairy cell leukemia (HCL). The cohort is based on data obtained from electronic medical records. It includes 218 consecutive patients diagnosed with HCL between 16 June 1998, and 20 September 2022, out of which the coronavirus has infected 85 patients during the Omicron surge. Out of 85 patients with HCL who were infected by Covid-19; 7 patients (8.2%) have been hospitalized, and the mortality rate was 2.3% (two patients). Thirteen of the 85 patients had been infected by Covid-19 in previous waves, including 9/13 after vaccination, and none of them developed a severe disease...

BACKGROUND: The development of evidence-based guidelines for the prevention of infectious diseases through vaccination requires an understanding of populations that most likely may obtain an infection, severe illness or disease. Targeted vaccination recommendations are made possible by identifying risk groups, as is the case with meningococcal infections. Despite falling case numbers, meningococcal sepsis and meningococcal meningitis remain a major health problem. METHOD: A systematic literature search was carried out on the research platform Ovid...

The spleen is responsible for blood filtration and mounting an immune response against pathogens. In some people the spleen must be surgically removed because of traumatic events or oncological and hematological conditions. These patients are at higher risk of developing diseases caused by encapsulated bacteria throughout their lives. Thus, immunisations are advised for splenectomised persons to prevent infection caused by Streptococcus pneumoniae, Neisseria meningitidis and Haemophilus influenzae type b (Hib)...

Pyomyositis is an infection of skeletal muscles, commonly affecting deep longitudinal muscles of the lower extremities. Primary pyomyositis is uncommon in the United States. Staphylococcus aureus is the most common cause of pyomyositis, but Streptococcus pneumoniae is the most common cause of life-threatening bacterial infection in asplenic patients. Most cases of S. pneumoniae pyomyositis occur in immunocompromised patients. We describe a 31-year-old man with S. pneumoniae pyomyositis whose diagnosis and hospital course were complicated by an immunocompromised state from asplenia and an underlying connective tissue disease, Stickler syndrome...

OBJECTIVE: Patients with asplenia have an increased lifelong risk of severe infections especially post splenectomy sepsis with hospital mortality rates of 30-50%. Adherence to existing guidelines for preventive measures is low. Objective of the study is the evaluation of a novel intervention to increase health psychological outcomes in patients with asplenia resulting in better adherence to preventive measures. METHODS: The intervention was evaluated by conducting a prospective, two-armed historical control group design via propensity score analysis...