Burkholderia Pseudomallei As The Predominant Cause Of Splenic Abscess In Kapit, Sarawak, Malaysian Borneo.

BACKGROUND: Splenic abscess is an uncommon condition, with autopsy studies estimating an incidence rate of 0.14-0.70%. Causative organisms can be extremely diverse. Burkholderia pseudomallei is the most common cause of splenic abscess in melioidosis-endemic areas. METHODS: We reviewed 39 cases of splenic abscesses in a district hospital in Kapit, Sarawak, from January 2017 to December 2018. The demographics, clinical characteristics, underlying diseases, causative organisms, therapeutic methods, and mortality rates were investigated. RESULTS: There were 21 males and 18 females (mean age, 33.7±2.7 years). Almost all patients (97.4%) had a history of pyrexia. Diabetes mellitus was present in 8 patients (20.5%). Splenic abscesses were diagnosed using ultrasonography and were multiple in all 39 cases. Positive blood cultures were obtained in 20 patients (51.3%), and all yielded B. pseudomallei. Melioidosis serology was positive in 9 of 19 patients (47.4%) with negative blood cultures. All patients were treated for melioidosis with antibiotics without the need for surgical intervention. All splenic abscesses resolved after anti-melioidosis treatment was completed. One patient died (2.6%) as a result of B. pseudomallei septicaemia with multiorgan failure. CONCLUSIONS: Ultrasonography is a valuable tool for diagnosing splenic abscesses in resource-limited settings. B. pseudomallei was the most common etiological agent of splenic abscesses in our study.

Sarcopenia, body composition and factors associated with variceal gastrointestinal bleeding and splenectomy in hepatosplenic schistosomiasis mansoni.

BACKGROUND: Sarcopenia is a common complication of cirrhosis and an important predictor of morbimortality. We aimed to determine the prevalence of sarcopenia and its associated factors in hepatosplenic schistosomiasis (HSS) as well as to evaluate whether muscle mass and function are associated with variceal upper gastrointestinal bleeding (VUGIB) and previous splenectomy in subjects without other liver diseases. METHODS: We conducted a cross-sectional study including adults with HSS who underwent clinical, biochemical, anthropometric, muscle strength and physical performance evaluations and were submitted to bioelectrical impedance analysis and abdominal ultrasound. Sarcopenia was diagnosed according to the 2019 European consensus criteria. RESULTS: A total of 66 patients with HSS (62.1% male; mean age 48.8±8.6 y) were included. Overall, six subjects (9.1%) were diagnosed with probable sarcopenia and none had confirmed sarcopenia. Fat-free body mass index (BMI) was independently associated with VUGIB (odds ratio 0.701 [95% confidence interval 0.51 to 0.96]; p=0.025). Compared with patients who did not undergo surgery, individuals who underwent esophagogastric devascularization combined with splenectomy (EGDS) had higher serum lipid levels, fat percentage and frequency of metabolic syndrome, with lower skeletal muscle mass index and hand grip strength. CONCLUSIONS: HSS mansoni seems not to cause sarcopenia. However, a lower fat-free BMI was associated with previous VUGIB and the subgroup of patients who underwent EGDS presented higher lipid levels, fat percentage and frequency of metabolic syndrome and lower muscle mass and function.

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

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

Hidatidosis esplénica: resultados de una serie de casos consecutiva intervenidos quirúrgicamente / Splenic hydatidosis: results of a series of consecutive cases undergoing surgery

INTRODUCCIÓN: La echinococcosis esplénica (EE), suele ser una enfermedad asintomática cuyo diagnóstico se plantea de forma incidental. OBJETIVO: Determinar morbilidad post-operatoria (MPO) y recurrencia en pacientes intervenidos quirúrgicamente por EE. Material y MÉTODO: Serie de casos con seguimiento, de pacientes con EE intervenidos de forma consecutiva, entre 2000 y 2018. Las variables resultado fueron MPO y recurrencia. Otras variables de interés fueron: diámetro y localización del quiste, tipo de cirugía, tiempo quirúrgico, estancia hospitalaria, y mortalidad. Los pacientes fueron seguidos de forma clínica y con imágenes por un mínimo de 18 meses. Se utilizó estadística descriptiva, con medidas de tendencia central y dispersión. RESULTADOS: Se intervinieron 26 pacientes (53,8% hombres), con una mediana de edad de 41,5 años. Las medianas del diámetro de los quistes, el tiempo quirúrgico y el tiempo de hospitalización fueron 14,5 cm; 65 minutos y 4,5 días, respectivamente. La MPO fue 11,5% (3 casos). No hubo mortalidad. Con una mediana de seguimiento de 94 meses, se verificó una recurrencia de 3,8%. CONCLUSIÓN: Los resultados verificados, en términos de MPO, mortalidad y recurrencia son apropiados en comparación a otras series publicadas.

BACKGROUND: Splenic echinococcosis (SE) is usually an asymptomatic disease whose diagnosis is made incidentally. Aim: To determine postoperative morbidity (POM) and recurrence in patients who underwent surgery for SE. METHODS: Case series with follow-up, of patients with SE operated on, consecutively, between 2000 and 2018. The outcome variables were POM and recurrence. Other variables of interest were diameter and location of the cyst, type of surgery, surgical time, hospital stay, and mortality. The patients were followed up clinically and with images for a minimum of 18 months. Descriptive statistics were used, with measures of central tendency and dispersion. RESULTS: Twenty-six patients (53.8% men), with a median age of 41.5 years, underwent surgery in this period. The medians of cysts diameter, surgical time and hospital stay were 14.5 cm, 65 min, and 4.5 days respectively. POM was 11.5% (3 cases). There was no mortality. With a median follow-up of 94 months, a recurrence of 3.8% was verified. CONCLUSION: Verified results, in terms of POM, mortality and recurrence are appropriate in comparison with those of series of similar size and follow-up.

Serological evidence of avian HEV antibodies in apparently healthy chickens in southwest Nigeria.

Avian hepatitis E virus (aHEV) is associated with hepatitis-splenomegaly syndrome, big liver and spleen disease and hepatic rupture haemorrhage syndrome. However, the knowledge about aHEV in commercial layer chickens in Nigeria is scarce. In this study, 460 serum samples obtained from 36 apparently healthy commercial layer chicken flocks in three states (Ogun, Osun and Oyo States) of southwestern Nigeria were analysed by enzyme linked immunosorbent assay for the presence of anti-aHEV immunoglobulin Y (IgY) antibodies. In total, the overall seroprevalence of anti-aHEV antibodies was 14.6%. The serological analysis revealed that 75% of the flocks examined were positive for anti-aHEV IgY antibodies from chickens of various ages in all three states. The percentage of the seropositive chickens in the three states varied from flock to flock ranging from 60% to 88.8% and seropositive chickens were detected at any age (24-52 weeks of age) without significant differences between the age groups. This is the first report assessing the presence of aHEV antibodies in chickens from Nigeria. The detection of anti-aHEV antibodies in commercial layer chickens in this study emphasizes the importance of serosurveillance in disease monitoring due to the economic threat posed by aHEV as a result of decreased egg production and increased mortality in affected commercial layer chicken farms. However, further studies are essential to reveal the clinical implications and to assess the real burden of aHEV in Nigeria.

Evaluation of systemic involvement of Coronavirus disease 2019 through spleen; size and texture analysis

Background/aim: To investigate the changes in the spleen size, parenchymal heterogeneity, and computed tomography (CT) texture analysis features of patients diagnosed with Coronavirus disease 2019 (COVID-19) Materials and methods: The size and parenchymal structure of the spleen in 91 patients who underwent thoracic CT examination due to COVID-19 were evaluated. For the evaluation of parenchymal heterogeneity, CT texture analysis was performed using dedicated software (Olea Medical, France). The texture analysis of each case consisted of 15 first-order intensity-based features, 17 gray level co- occurrence matrix-based features, and 9 gray level run length matrix-based features. Results: A total of 91 patients (45 males, 46 females) with a mean age of 54.31 ± 16.33 years (range: 18­81) were included in the study. A statistically significant decrease in spleen size was seen in the follow-up CT examinations (p < 0.001) whereas no statistically significant difference was found between the Hounsfield unit (HU) values. The radiomics consisted of first-order intensity-based features such as 90th percentile, maximum, interquartile range, range, mean absolute deviation, standard deviation, and variance, all of which showed statistically significant differences (p-values: < 0.001, < 0.001, 0.001, 0.003, 0.001, 0.001, and 0.004, respectively). "Correlation" as a gray level co-occurrence matrix-based feature and "gray level nonuniformity" as a gray level run length matrix-based feature showed statistically differences (p-values: 0.033 and < 0.001, respectively). Conclusions: Although COVID-19 manifests with lung involvement in the early stage, it can also cause systemic involvement, and the spleen may be one of its target organs. A decrease in the spleen size and parenchymal microstructure changes can be observed in the short follow-up time. It is hoped that the changes in the parenchymal microstructure will be demonstrated by a noninvasive method: texture analysis.

The spectrum of splenic complications in patients with sickle cell disease in Africa: a systematic review.

The majority of the global population of sickle cell disease (SCD) patients resides in Africa. Individuals with this condition are at great risk of serious infections and early mortality secondary to splenic dysfunction without preventative measures. This review investigated the spectrum of splenic complications encountered in SCD among populations in Africa. We systematically searched several databases for all articles published through March 3, 2020. We included 55 studies from 14 African countries. This review reveals the difference in frequency of splenic complications in SCD in Africa when compared with their counterparts in the United State and Europe. While several studies (n = 45) described splenomegaly with a prevalence of 12% to 73% among children, and 4% to 50% among adults with HbSS, the reported prevalence for acute splenic sequestration crisis (n = 6 studies) and hypersplenism (n = 4 studies) was <10% and <5% respectively. A total of 30 surgical splenectomy was reported across eight studies. Only two (3.7%) studies provided data on spleen function. A conflicting pattern was observed amongst studies that evaluated the relationship between splenomegaly and the presence of bacterial and malaria infections. This review reveals the paucity of studies describing the role of SCD-induced splenic dysfunction in morbidity and infection related mortality in Africa.

Splenic abscess in a tertiary care centre in India: clinical characteristics and prognostic factors.

BACKGROUND: Splenic abscess has been an uncommon entity which is now being encountered more frequently due to increased prevalence of immunodeficiency disorders and chronic illnesses. This study was aimed to audit our experience with splenic abscesses at a tertiary care centre in India highlighting usefulness of an algorithmic approach. METHODS: Retrospective analysis of data of patients (January 2014 to December 2019) with splenic abscess was done. Data were retrieved for clinical characteristics, radiological findings, organism spectra, abscess characteristics, therapeutic measures and clinical outcome. RESULTS: The mean age of the study population (n = 36) was 41.3 ± 19.0 years with 50% males. Comorbidities were identified in 17 (47.2%) patients, with diabetes mellitus being the commonest. Fever and abdominal pain were the most common presenting features. Multiple splenic abscesses were present in 21 (58.3%) patients. Extra-splenic abscesses in liver were seen in five (13.9%) patients while nine (25%) patients had ruptured splenic abscess. Microorganisms were identified in 24 (66.7%) patients, with Salmonella typhi being the commonest (n = 9, 25%) followed by Escherichia coli (n = 7, 19.4%) and Staphylococcus aureus (n = 4, 11.1%). Six patients received only antimicrobials, 24 were managed with percutaneous aspiration or catheter drainage and six required surgery. Five (13.9%) patients died, with highest mortality being seen in those who received only antimicrobial (50%), compared to percutaneous aspiration or catheter drainage (8.3%) and surgery (0%), P = 0.017. CONCLUSION: Using percutaneous aspiration or drainage in conjunction with antibiotics, followed by surgery in non-responder, patients with splenic abscesses can be managed successfully with acceptable mortality.

Detection of spleen abscesses facilitates diagnosis of melioidosis in Malaysian children.

OBJECTIVES: Melioidosis is associated with extremely high case fatality ratios. The aim of this study was to determine whether detection of abdominal visceral abscesses can facilitate diagnosis of melioidosis in children. METHODS: We conducted a retrospective analysis of all children who had liver and/or spleen abscesses on abdominal ultrasonography admitted to Bintulu Hospital in Sarawak, Malaysia, from January 2014 until December 2018. RESULTS: Fifty-three children had liver and/or spleen abscesses. Spleen abscesses were present in 48 (91%) cases; liver abscesses in 15 (28%). Melioidosis was confirmed by culture in 9 (17%) children; small occult splenic abscesses were present in all cases. In 78% of these cases, the lesions were detected before any positive culture (or serology) results were available. Four (8%) children had bacteriologically-confirmed tuberculosis. Two (4%) had Staphylococcus aureus infection. Of the remaining 38 (72%) culture-negative cases, 36 (95%) had clinical and imaging characteristics similar to that of children with culture-confirmed melioidosis and improved with empirical melioidosis antibiotic therapy. CONCLUSIONS: A large number of children in Bintulu Hospital in Sarawak, Malaysia, were found to have spleen abscesses. Melioidosis was the most common etiology identified in these children. Abdominal ultrasonography is extremely useful in facilitating the diagnosis of pediatric melioidosis.

Ultrasonographic honeycomb pattern of the spleen in cats: correlation with pathological diagnosis in 33 cases.

OBJECTIVES: The aim of this study was to evaluate the relationship between the ultrasonographic (US) diffuse honeycomb pattern (HCP) of the spleen and a pathological diagnosis in cats, and to assess the influence of transducer type on HCP visualisation. METHODS: Abdominal ultrasounds of cats with an HCP were reviewed and splenic size, shape, margination, other parenchymal alterations and splenic hilar lymphadenopathy were recorded. When applicable, images acquired with high-frequency linear and curvilinear transducers were compared to determine if an HCP was more frequently demonstrated on high-resolution images. A retrospective review of the corresponding splenic cytohistopathological samples was also performed. RESULTS: Thirty-three cats met the inclusion criteria. Five cases were diagnosed by histology and 28 by cytology, confirmed by PCR for antigen receptor rearrangements (PARR) in uncertain cases. There were 15 cases of lymphoid hyperplasia, eight cases of lymphoma (four B cell, three T cell and one large granular lymphocytes), six cats with splenitis, three with extramedullary haematopoiesis and one with histiocytic sarcoma. The prevalence of lymphoma in cats with an HCP of the spleen was 24%. Splenomegaly was the most frequent US feature associated with an HCP and was observed in all lymphoma cases. In the images obtained from both high-frequency linear and micro-convex transducers the visualisation of an HCP was enabled in all cases (24/24) and in 62.5% (15/24), respectively. CONCLUSIONS AND RELEVANCE: Based on our findings, a US HCP of the spleen in cats can be associated with benign and malignant disorders and is infrequently associated with lymphoma in comparison with dogs. Cytological or histological examination, possibly supplemented by PARR, should always be performed for diagnostic support. Use of high-frequency linear transducers is recommended to properly recognise an HCP or subtle changes in splenic parenchyma.

Prevalence and diagnostic value of the ultrasonographic honeycomb appearance of the spleen in cats.

OBJECTIVES: The aim of this study was to report the prevalence of a honeycomb appearance of the spleen in a population of referral cats presented for ultrasound examination, and to determine the diagnostic value of this finding vs the definitive diagnosis, the splenic cytological and haematological results. METHODS: Data were obtained from the medical records (2016-2018) of cats that had an ultrasonographic honeycomb appearance of the spleen, a splenic cytological diagnosis and a complete blood count. RESULTS: Twenty-five cats were included. Prevalence of the honeycomb pattern was 6.8%. None of the spleen was considered normal on cytology and four types of lesions were found: lymphoid hyperplasia (64%), neoplasia (16%), extramedullary haematopoiesis (12%) and splenitis (8%). A honeycomb pattern was successfully identified with a linear high-frequency probe in all cats, but only in 36% of cases with the micro-convex probe. Follow-up information was available for four cats, in which the honeycomb appearance persisted up to 105 days after the first examination; there was persistence of the honeycomb pattern in all cases. Cats with a splenic cytological diagnosis of extramedullary haematopoiesis had the lowest haemoglobin plasma concentration (P = 0.011). CONCLUSIONS AND RELEVANCE: Honeycomb appearance of the spleen is uncommon in cats and, in our study, was systematically associated with cytological alterations; most of the time it was benign (84%). The use of a high-frequency linear probe improves its detection rate. No epidemiological, ultrasonographic or clinical criteria allow differentiation between the different types of infiltration and fine-needle aspiration is therefore recommended.

Prevalence and predictive factors of splenic sequestration crisis among 423 pediatric patients with sickle cell disease in Tunisia.

This study was aimed to identify the predictors of splenic sequestration crisis (SSC) among pediatric patients with sickle cell disease (SCD). This prognosis study was carried out in the pediatric immuno-hematology unit, over 20 years (1998 to 2017), enrolling patients with SCD. The cox model was used in multivariate analysis. Among 423 patients with SCD (240 S/S phenotype, 128 S/B0, 30 S/B+, 14 S/O arab and 11 S/C), 150(35.4%) had at least one episode of SSC. The average age of patients at the first episode was 48.3 months ± 32.4(2-168). Recurrence of SSC was observed in 117 patients (78%). Spleen size ≥3 cm at baseline was the strongest predictor of SSC occurrence (HR = 7.27, CI: 4.01-13.20, p = 0.05) and recurrence (HR = 6.37, CI: 1,46-27.83, p = 0.01). Pallor revealing the disease, age at onset of symptoms <24 months and reticulocytosis ≥300,000/mm3 increased the risk of SSC. Pain crisis revealing the disease as well as neutrophilia was associated with a lower risk of SSC. In conclusion, this study confirmed the high prevalence of SSC in SCD and the high frequency of recurrence after a first episode. The SSC occurrence and recurrence were intimately linked to the presence of splenomegaly, chronic pallor revealing the disease as well as reticulocytosis.

Insights into determinants of spleen injury in sickle cell anemia.

Spleen dysfunction is central to morbidity and mortality in children with sickle cell anemia (SCA). The initiation and determinants of spleen injury, including acute splenic sequestration (ASS) have not been established. We investigated splenic function longitudinally in a cohort of 57 infants with SCA enrolled at 3 to 6 months of age and followed up to 24 months of age and explored the respective contribution of decreased red blood cell (RBC) deformability and increased RBC adhesion on splenic injury, including ASS. Spleen function was evaluated by sequential 99mTc heated RBC spleen scintigraphy and high-throughput quantification of RBCs with Howell-Jolly bodies (HJBs). At 6 and 18 months of age, spleen filtration function was decreased in 32% and 50% of infants, respectively, whereas the median %HJB-RBCs rose significantly (from 0.3% to 0.74%). An excellent correlation was established between %HJB-RBCs and spleen scintigraphy results. RBC adhesion to laminin and endothelial cells increased with time. Adhesion to endothelial cells negatively correlated with splenic function. Irreversibly sickled cells (ISCs), used as a surrogate marker of impaired deformability, were detected at enrollment and increased significantly at 18 months. %ISCs correlated positively with %HJB-RBCs and negatively with splenic uptake, indicating a relationship between their presence in the circulation and spleen dysfunction. In the subgroup of 8 infants who subsequently experienced ASS, %ISCs at enrollment were significantly higher compared with the asymptomatic group, suggesting a major role of impaired deformability in ASS. Higher levels of %HJB-RBCs were observed after the occurrence of ASS, demonstrating its negative impact on splenic function.

Prevalence of extramedullary hematopoiesis, renal cysts, splenic and hepatic lesions, and vertebral hemangiomas among thalassemic patients: a retrospective study from the Myocardial Iron Overload in Thalassemia (MIOT) network.

We determined the prevalence of incidental extracardiac findings (IEF) at Magnetic Resonance Imaging (MRI) potentially related to anemia and hypoxia in age- and sex-matched populations (N = 318) with thalassemia major (TM) and thalassemia intermedia (TI) enrolled in the Myocardial Iron Overload in Thalassemia network. Overall, IEFs were detected in 33.3% and 25.8% of patients with TI and TM, respectively (P = 0.114). TI and TM patients had elevated but comparable prevalence of renal, splenic and liver cysts, and vertebral hemangiomas while TI patients had a significant higher frequency of extramedullary hematopoiesis (EMH) (15.1% vs 4.4%; P = 0.002). The prevalence of total IEFs increased with advancing age. TI non-transfusion-dependent patients had a significantly lower frequency of renal cysts than TI transfusion-dependent patients (8.8% vs 26.4%; P = 0.005). The prevalence of renal cysts in the thalassemic population was significantly higher than that in the general population (19.2% vs 1.9%; P < 0.0001). Our data on renal cysts indicate a significant higher prevalence of these IEFs compared to the general population, suggesting the role of the inappropriate activation of the hypoxia-inducible factor system linked to the chronic hypoxia. The significant prevalence of IEF in thalassemia patients undergoing MRI for iron quantification should prompt the discussion of the inclusion of IEF in the MRI report.

Patient, Procedure, and Endoscopist Risk Factors for Perforation, Bleeding, and Splenic Injury After Colonoscopies.

BACKGROUND & AIMS: We investigated perforations, bleeding, and splenic injuries after screening or diagnostic colonoscopies to identify patient-, procedure-, endoscopist-, and facility-associated risk factors. METHODS: We analyzed data from the SNIIRAM-PMSI national claims databases in France. A total of 4,088,799 patients, 30 years or older, undergoing a first screening or diagnostic colonoscopy from 2010 through 2015 were identified. Rates of severe adverse events (SAEs) were estimated using stringent and broad definitions. Risk factors associated with perforations and major bleeding were estimated using multilevel logistic regression models, adjusted for patient, colonoscopy, and endoscopist characteristics. RESULTS: Perforation rates ranged from 3.5 (stringent definition) to 7.3 (broad definition) per 10,000 procedures, bleeding rates ranged from 6.5 to 23.1 per 10,000 procedures, and splenic injury rates ranged from 0.20 to 0.34 per 10,000 procedures. Rates of 30-day mortality were 13.2 per 1000 bleeds, 29.2 per 1000 perforations, and 36.1 per 1000 splenic injuries (stringent definitions). Patient characteristics associated with SAEs were increasing age (especially for perforation), cancer, and cardiovascular comorbidities. Procedure characteristics associated with SAEs included polypectomy-especially of polyps larger than 1 cm with an increased risk of perforation (odds ratio, 4.1; 95% CI, 3.4-5.0) and bleeding (odds ratio, 13.3; 95% CI, 11.7-15.1). Less-experienced endoscopists and endoscopists who performed a smaller number of colonoscopies were independently associated with a risk of SAEs. CONCLUSION: In an analysis of national claims databases in France, we found SAEs related to screening and diagnostic colonoscopies to be more frequent in older patients, in patients with comorbidities, and with less-experienced endoscopists. Patients at risk of SAE should be identified and colonoscopies should be performed or supervised by experienced endoscopists.

Morbidity pattern and interferon gamma level in sickle cell anemia patients with autosplenectomy.

INTRODUCTION: In sickle cell anemia patients (SCA), the spleen suffers multiple occlusion of its microvasculature causing ischemia and subsequently autosplenectomy. Among the functions of the spleen is the production of gamma interferon (IFN-γ) which has several immunological roles. This function could be impaired in these patients. Therefore, this study aimed at determining the extent to which autosplenectomy affects the immunity of SCA patients as reflected by the level of IFN-γ, frequency of infections, and crises (vaso-occlusive, hemolytic, aplastic) in these patients. MATERIALS AND METHODS: Forty SCA patients in steady state condition were recruited into the study. A self-administered questionnaire was completed by all patients, and venous blood sample was analyzed for IFN-γ levels. All patients underwent abdominal ultrasound scan. RESULTS: Autosplenectomy was observed in 20% of the sickle cell patients. Bone pain was the most frequent type of crisis in the study population; this occurred in 100% and 85% of patients with autosplenectomy and those without autosplenectomy, respectively. Infection rates of more than once a year was reported in 87.5% of SCA patients with autosplenectomy compared with 50% of SCA patients without autosplenectomy. There was no significant difference in IFN-γ plasma levels between SCA patients with autosplenectomy and those without autosplenectomy. CONCLUSION: This study showed a higher prevalence of infection and bone pain crisis among SCA patients with autosplenectomy than in SCA patients without autosplenectomy. It also showed comparable level of IFN-γ in the 2 groups of patients. Patients with autosplenectomy may benefit from early institution of drugs such as hydroxyurea to improve quality of life.

Clinical and hematological profile in a newborn cohort with hemoglobin SC / Perfil clínico e hematológico em uma coorte neonatal com hemoglobina SC

Abstract Objectives: Hemoglobin SC is the second most common variant of sickle-cell disease worldwide, after hemoglobin SS. The objectives of the study were to describe the clinical and laboratory characteristics of hemoglobin SC disease in children from a newborn screening program and treated at a blood center. Methodology: This study assessed a cohort of 461 infants born between 01/01/1999 and 12/31/2012 and followed-up until 12/31/2014. Clinical events were expressed as rates for 100 patient-years, with 95% confidence intervals. Kaplan-Meier survival curves were created. Results: The median age of patients was 9.2 years; 47.5% were female. Mean values of blood tests were: hemoglobin, 10.5 g/dL; reticulocytes, 3.4%; white blood cells, 11.24 × 109/L; platelets, 337.1 × 109/L; and fetal hemoglobin, 6.3%. Clinical events: acute splenic sequestration in 14.8%, blood transfusion 23.4%, overt stroke in 0.2%. The incidence of painful vaso-occlusive episodes was 51 (48.9-53.4) per 100 patient-years and that of infections, 62.2 episodes (59.8-64.8) per 100 patient-years. Transcranial Doppler ultrasonography (n = 71) was normal given the current reference values for SS patients. Hydroxyurea was given to ten children, all of whom improvement of painful crises. Retinopathy was observed in 20.3% of 59 children who underwent ophthalmoscopy. Avascular necrosis was detected in seven of 12 patients evaluated, predominantly in the left femur. Echocardiogram compatible with pulmonary hypertension was recorded in 4.6% of 130 children, with an estimated average systolic pulmonary artery pressure of 33.5 mmHg. The mortality rate from all causes was 4.3%. Conclusions: Clinical severity is variable in SC hemoglobinopathy. Several children have severe manifestations similar to those with SS disease.

Resumo Objetivos: A hemoglobinopatia SC é a segunda variante mais comum da doença falciforme no mundo, após a hemoglobinopatia SS. Os objetivos do estudo foram descrever as características clínicas e laboratoriais da hemoglobinopatia SC em recém-nascidos diagnosticados por programa de triagem neonatal e encaminhados para acompanhamento em hemocentro. Metodologia: Coorte de 461 recém-nascidos SC nascidos entre 01/01/1999 e 31/12/2012 e seguidos até 31/12/2014. A incidência de eventos clínicos foi expressa por taxas relativas a 100 pacientes-ano, com limites de confiança a 95%. Curvas de sobrevida foram construídas segundo Kaplan-Meier. Resultados: Mediana de idade, 9,2 anos; 47,5%, feminino. Médias dos valores hematológicos: hemoglobina 10,5 g/dL; reticulócitos 3,4%; leucometria 11,24 x 109/L; plaquetometria 337,1x109/L; hemoglobina fetal 6,3%. Eventos clínicos: sequestro esplênico agudo em 14,8%, hemotransfusão 23,4%, AVC isquêmico 0,2%. A incidência de episódios vaso-oclusivos dolorosos foi de 51 (48,9-53,4) por 100 pacientes-ano; a de infecções, 62,2 episódios (59,8-64,8) por 100 pacientes-ano. Doppler transcraniano (n = 71) foi normal, se usados os valores de referência de crianças SS. Dez pacientes usaram hidroxiureia, todos com melhoria das crises dolorosas. Retinopatia foi observada em 20,3% das 59 crianças que fizeram fundoscopia. Necrose avascular foi detectada em 7 de 12 pacientes avaliados, com predomínio no fêmur esquerdo. Ecocardiograma compatível com hipertensão pulmonar foi registrado em 4,6% de 130 crianças, com média estimada de 33,5 mm Hg de pressão arterial pulmonar. A taxa de mortalidade por todas as causas foi de 4,3%. Conclusões: A hemoglobinopatia SC tem gravidade variável; várias crianças apresentam manifestações clínicas intensas, semelhantes às da hemoglobinopatia SS.

Anatomic site and etiology of hemorrhage in small versus large dogs with spontaneous hemoperitoneum.

OBJECTIVE: To compare anatomic sources and underlying etiology of hemorrhage in small vs large dogs with spontaneous hemoperitoneum (SH). STUDY DESIGN: Retrospective cross-sectional study. ANIMALS: Client-owned dogs with SH at 2 academic institutions. METHODS: Medical records were reviewed for age, breed, sex, weight, and results of imaging, surgery, necropsy, cytology, and histopathology. Dogs were divided according to body weight (≤ 20 kg = small, > 20 kg = large). Confidence intervals were calculated to estimate rates of splenic and hepatic hemorrhage in small and large dog SH populations. Multivariable regression was used to compare prevalence of anatomic sources of hemorrhage and etiology in small vs large dogs. RESULTS: We identified 742 dogs with SH, including 637 in which the anatomic site of hemorrhage was investigated. Splenic hemorrhage was diagnosed in 43.2% (95% CI, 34.3-52.4) of small dogs and 61.3% (95% CI, 57.0-65.6) of large dogs. Small dogs had lower prevalence of splenic hemorrhage (prevalence ratio, 0.70; 95% CI, 0.58-0.87; P < .001) and higher prevalence of hemorrhage from liver (prevalence ratio, 1.72; 95% CI, 1.20-2.47; P = .003) or from another location such as retroperitoneal mass, kidney, or adrenal (prevalence ratio, 2.73; 95% CI, 1.66-4.47; P < .001) vs large dogs. Hemangiosarcoma was associated with splenic hemorrhage and occurred more frequently in large vs small dogs (P = .011). CONCLUSION: Small dogs had a lower rate of splenic hemorrhage and higher rates of hemorrhage from liver and other sites compared to large dogs. Etiologies other than splenic hemangiosarcoma were common, particularly among dogs weighing ≤ 20 kg. CLINICAL SIGNIFICANCE: Clinicians should perform diagnostics and consider body size before making presumptive diagnoses in dogs with SH.

Prenatal and postnatal evolution of isolated fetal splenic cysts.

OBJECTIVES: The aim of this study was to evaluate the prevalence, the prenatal, and postnatal evolution of isolated fetal splenic cysts. METHODS: All cases of suspected fetal splenic cyst or abdominal unidentified cyst discovered during routine ultrasound scan, from 2007 to 2017, and referred to a French tertiary care center, were retrospectively collected. For each case, several prenatal parameters and postnatal evolution were reported. RESULTS: Among 5450 cases of fetal anomalies, 14 patients (0.3%) had a prenatal diagnosis of fetal splenic cysts. Median gestational age at diagnosis was 30.1 weeks. A unique cyst was present in 78.6%, whereas 2 cysts were observed in 14.3% and 3 cysts in 7.1%. During the pregnancy, cysts remained the same (78.6%) or disappeared (21.4%). Ultrasound scans at 6 months of age found total disappearance of the cysts (36.4%), spontaneous reduction from 2 to 1 cyst (18.2%) or persistence of the cysts (45.4%). CONCLUSION: Fetal splenic cysts are rare images, always isolated, usually unique and mainly found during third trimester of pregnancy. Their evolution is to disappear spontaneously during pregnancy or at 6 months of age leaving only half of them to remain beyond that age but without any symptoms.