Characteristics of the initial dengue outbreaks in a region without dengue prior to mid-2009 in a dengue-endemic country.

INTRODUCTION: The present study evaluated the characteristics of the initial dengue outbreaks in the Jaffna peninsula, a region without dengue prior to mid-2009 in dengue-endemic Sri Lanka, a tropical island nation. METHODOLOGY: This is a cross-sectional study conducted using a total of 765 dengue patients' clinical data and samples collected from the Teaching Hospital, Jaffna during the initial dengue outbreaks. Clinical, non-specific, and specific virological laboratory characteristics including the platelet count, NS1 antigen, and anti-DENV IgM/IgG were evaluated as correlates of dengue virus (DENV) infection in the two initial outbreaks of 2009/2010 and 2011/2012 in Northern Sri Lanka. RESULTS: Firstly, affected age and clinical characteristics were significantly different between the outbreaks (p < 0.005). Secondly, NS1 antigen detection in patients with fever days < 5 was statistically significant (p < 0.005). Thirdly, platelet count, detection of NS1 antigen, and anti-DENV IgM/IgG profiles were adequate to diagnose 90% of the patients; hepatomegaly and platelet count of < 25,000/mm3 were identified as predictors of severe disease. Fourthly, secondary DENV infections were detected in the early stages of the illness in many patients. Finally, infecting DENV serotypes were different between the two outbreaks. CONCLUSIONS: Clinical and non-specific laboratory characteristics and the infecting DENV serotypes between the two initial outbreaks in Northern Sri Lanka were significantly different. NS1 antigen, anti-DENV IgM/IgG, and platelet counts were identified 90% of the dengue patients. Hepatomegaly and platelet count of < 25,000/mm3 were able to predict the disease severity in this study.

Abdominal wall hernia is a frequent complication of polycystic liver disease and associated with hepatomegaly.

BACKGROUND AND AIM: Polycystic liver disease (PLD) is related to hepatomegaly which causes an increased mechanical pressure on the abdominal wall. This may lead to abdominal wall herniation (AWH). We set out to establish the prevalence of AWH in PLD and explore risk factors. METHODS: In this cross-sectional cohort study, we assessed the presence of AWHs from PLD patients with at least 1 abdominal computed tomography or magnetic resonance imaging scan. AWH presence on imaging was independently evaluated by two researchers. Data on potential risk factors were extracted from clinical files. RESULTS: We included 484 patients of which 40.1% (n = 194) had an AWH. We found a clear predominance of umbilical hernias (25.8%, n = 125) while multiple hernias were present in 6.2% (n = 30). Using multivariate analysis, male sex (odds ratio [OR] 2.727 p < .001), abdominal surgery (OR 2.575, p < .001) and disease severity according to the Gigot classification (Type 3 OR 2.853, p < .001) were identified as risk factors. Height-adjusted total liver volume was an independent PLD-specific risk factor in the subgroup of patients with known total liver volume (OR 1.363, p = .001). Patients with multiple hernias were older (62.1 vs. 55.1, p = .001) and more frequently male (22.0% vs. 50.0%, p = .001). CONCLUSION: AWHs occur frequently in PLD with a predominance of umbilical hernias. Hepatomegaly is a clear disease-specific risk factor.

Relation of Liver Volume to Adverse Cardiovascular Events in Adolescents and Adults With Fontan Circulation.

Elevated central venous pressure in those with Fontan circulation causes liver congestion and hepatomegaly. We assessed if liver volume by magnetic resonance imaging (MRI) is associated with adverse cardiovascular outcomes. Retrospective study of 122 patients with Fontan circulation who were >10 years old and had a liver MRI with magnetic resonance elastography. Liver volume (ml) was measured by manual segmentation from axial T2-weighted images and was indexed to body surface area. The composite outcome included death, heart transplant, ventricular assist device placement, or nonelective cardiovascular hospitalization. The median age at the time of MRI was 18.9 (interquartile range 15.8 to 25.9) years, and 47% of the patients were women. The mean indexed liver volume was 1,133 ± 180 ml/m2. Indexed liver volume was not significantly associated with age, years since Fontan, or with liver stiffness (r = 0.15, p = 0.10), but was positively correlated with Fontan pressure (r = 0.32, p = 0.002). Over a median follow-up of 2.1 (0.8 to 4.2) years, 32 patients (26%) experienced the composite outcome. Higher indexed liver volume was associated with a greater hazard for the composite outcome (hazard ratio per 1 SD increase = 1.74, 95% confidence interval 1.27 to 2.35, p = 0.0004) but increased liver stiffness was not significantly associated with the composite outcome (hazard ratio per 1 SD increase 1.44, 95% confidence interval 0.90 to 2.21, p = 0.11). In conclusion, greater liver volume indexed to body surface area is associated with unfavorable hemodynamics and adverse outcomes in patients with Fontan circulation. Liver volume may be a useful, simple imaging biomarker in adolescents and adults with Fontan circulation.

Characteristics of severe malaria in hospitalized children in Ho Chi Minh City from 2012 to 2019.

In Vietnam, severe malaria is currently rare but is a life-threatening disease. It may be misdiagnosed with other common diseases. This descriptive study aimed to characterize severe malaria and its clinical aspects, as well as outcomes of infected pediatric patients to improve case management. The case-series study was carried out based on medical records of children aged between one month and 15 years with malaria diagnosed by blood smear or rapid diagnostic test. Chi-squared test with the p values less than 0.05 were considered statistically significant. There were 47 cases enrolled in the study. The prevalence of severe malaria was 29.8% (57.1% in children under five). The morbidity was 71.4% in male and 28.6% in female. Common clinical signs of severe malaria were fever (100%), severe anemia (21.4%), hepatomegaly (85.7%), and splenomegaly (71.4%). Common biological abnormalities in severe malaria were anemia, thrombocytopenia, increased liver enzymes, and high CRP level. The severe malaria was mainly caused by P. falciparum (100%). The age range for those infected with P. falciparum was 6.5 ± 4.5 years (min 0.3; max 14.9). The successful rate of treatment was 92.9% with artesunate. Antimalarial treatment time was 9.0 (6 - 12) days for severe malaria, which was twice as many as that for non-severe malaria (p = 0.067). The current clinical and biological findings of severe malaria are different from those in previous times, which make it easy to be overlooked. Therefore, it's important to perform malaria diagnostic tests when there're clinical suggestions of severe malaria, including fever, hepatomegaly or splenomegaly.

Liver disease in children and adolescents with type 1 diabetes mellitus: A link between glycemic control and hepatopathy.

AIM: The aim of this study was to assess the prevalence of liver disease in children and adolescents with type-1 diabetes mellitus (T1DM) by detection of elevated liver transaminases, confirmed by fibroscan and ultrasound. The secondary objective was to assess the effect of glycemic control on improvement of liver functions. METHODS: One hundred and seven children and adolescents with T1DM were investigated by liver transaminases, mean HbA1c and pelviabdominal ultrasound while fibroscan was done for those with elevated liver transaminases only. Patients with elevated liver enzymes were reassessed after one year. RESULTS: Only nine (8.4%) of the studied patients have exhibited liver dysfunction in the form of elevated liver transaminases with median ALT 140 U/L and AST 191 U/L and hepatomegaly by ultrasound; The HbA1c (median = 10.8%) and fibroscan abnormalities (median fibrosis score 1) were significantly higher in patients with elevated liver transaminases (p < 0.001). Adequate glycemic control resulted in a significant decrease in liver transaminases (median ALT = 25 U/L and AST = 29 U/L), fibroscan fibrosis score (median = 0) and HbA1c (median = 9%) (p = 0.003), (p = 0.01) and (p = 0.003) respectively. CONCLUSION: Adequate glycemic control was associated with improvement of liver disease in children and adolescents with diabetes.

Cystic fibrosis-associated liver disease in children.

As improvements in nutritional and pulmonary care increase the life expectancy of cystic fibrosis (CF) patients, CF-associated liver disease (CFLD) is emerging as a cause of mortality. CFLD is the third leading cause of death in CF patients. We performed a search on PubMed and Google Scholar for published articles on CFLD. We reviewed the articles found in the literature search and gave priority to recent publications and studies with larger sample sizes. The prevalence of CFLD in the CF population is around 23% with a range of 2-62% and that prevalence increases linearly with age from 3.7% at age 5 to 32.2% at age 30. CFLD can present clinically in various ways such as hepatomegaly, variceal hemorrhage, persistent elevation of liver enzymes, and micro-gallbladder. Due to the focal nature of fibrosis in majority cases of CFLD, liver biopsies are sparsely performed for diagnosis or the marker of liver fibrosis. Although the mechanism of CFLD development is still unknown, many potential factors are reported. Some mutations of CFTR such as having a homozygous F508del mutation has been reported to increase the risk of developing CFLD and its severity. Having the SERPINA1 Z allele, a history of pancreatic insufficiency, a history meconium ileus, CF-related diabetes, or being male increases the risk of developing CFLD. Environmental factors do not appear to have significant effect on modulating CFLD development. Ursodeoxycholic acid is commonly used to treat or prevent CFLD, but the efficacy of this treatment is questionable.

Clinical, ocular motor, and imaging profile of Niemann-Pick type C heterozygosity.

OBJECTIVE: To characterize subclinical abnormalities in asymptomatic heterozygote NPC1 mutation carriers as markers of neurodegeneration. METHODS: Motor function, cognition, mood, sleep, and smell function were assessed in 20 first-degree heterozygous relatives of patients with Niemann-Pick disease type C (NPC) (13 male, age 52.7 ± 9.9 years). Video-oculography and abdominal ultrasound with volumetry were performed to assess oculomotor function and size of liver and spleen. NPC biomarkers in blood were analyzed. 18F-fluorodesoxyglucose PET was performed (n = 16) to detect patterns of brain hypometabolism. RESULTS: NPC heterozygotes recapitulated characteristic features of symptomatic NPC disease and demonstrated the oculomotor abnormalities typical of NPC. Hepatosplenomegaly (71%) and increased cholestantriol (33%) and plasma chitotriosidase (17%) levels were present. The patients also showed signs seen in other neurodegenerative diseases, including hyposmia (20%) or pathologic screening for REM sleep behavior disorder (24%). Cognitive function was frequently impaired, especially affecting visuoconstructive function, verbal fluency, and executive function. PET imaging revealed significantly decreased glucose metabolic rates in 50% of participants, affecting cerebellar, anterior cingulate, parieto-occipital, and temporal regions, including 1 with bilateral abnormalities. CONCLUSION: NPC heterozygosity, which has a carrier frequency of 1:200 in the general population, is associated with abnormal brain metabolism and functional consequences. Clinically silent heterozygous gene variations in NPC1 may be a risk factor for late-onset neurodegeneration, similar to the concept of heterozygous GBA mutations underlying Parkinson disease.

An ultrasound-based referential of body height-adjusted normal liver organometry in school children from Bokito in rural Cameroon.

The grading system for ultrasonographic assessment of Schistosoma mansoni morbidity is crucial for evaluation of control programs. This requires prior definition of normal liver organometric ranges in the population from the endemic area. A cross-sectional study was conducted in a S. mansoni endemic area in rural Cameroon. 1002 Participants were screened and 234 of them, free from all common liver-affecting diseases in the area (schistosomiasis, malaria, hepatitis B and C) and with no ultrasonographic signs of liver disease were selected and their liver parameters measured by ultrasonography. All statistics were considered significant for p-values < 0.05. Normal dimensions of livers lobe sizes, portal vein wall thickness and portal vein diameters are reported. The liver organometric data are presented for the entire study population as a whole and separately for males and females as prediction plots, with observed values and fitted regression line with 95% confidence. Reference ranges for liver parameters (size, portal vein thickness and diameter) adjusted for body height established in the current study are novel for Cameroon. The prediction plots generated should improve the accuracy of the assessment of liver morbidity by ultrasonography in the region.

Evaluation and characterisation of Chronic myeloid leukemia and various treatments in Saudi Arabia: A retrospective study.

BACKGROUND: Chronic myeloid leukemia (CML) is a clonal BCR-ABL1-positive myelo-proliferative disorder resulting from an acquired genetic mutation, characterized by the presence of the Philadelphia (Ph) chromosome. CML is associated with significantly high granulocyte numbers in the bone marrow and peripheral blood. MATERIALS AND METHODS: This retrospective study conducted at the Hematology Unit of King Saud University Medical City aimed to evaluate the incidence and characteristics of CML and the various treatments in Saudi Arabia. We have evaluated the demographic, clinical, and hematological data of 56 consecutive patients who visited the hospital from Jan 2012 to Jan 2018. RESULTS: The diagnosis and stage of CML were determined based on the World Health Organization criteria, following polymerase chain reaction analysis of bone marrow aspirates. Our study group had equal numbers of genders with a age mean of 43.3+18.1 years. The predominance of younger patients and equal incidence in males and females could be due to the racial and socioeconomic disparities among our patients compared to those in previous studies. While the most predominant symptom was fatigue and bone pain, the most common clinical sign was hepato-splenomegaly, followed by remarkable weight loss, and epistaxis. CONCLUSION: A patient with an increased WBC count, abdominal pain, left side distension, and hepato-splenomegaly should clearly be evaluated for CML.

Intestinal schistosomiasis of Ijinga Island, north-western Tanzania: prevalence, intensity of infection, hepatosplenic morbidities and their associated factors.

BACKGROUND: Intestinal schistosomiasis is highly endemic in Tanzania and mass drug administration (MDA) using praziquantel is the mainstay of the control program. However, the MDA program covers only school aged children and does not include neither adult individuals nor other public health measures. The Ijinga schistosomiasis project examines the impact of an intensified treatment protocol with praziquantel MDA in combination with additional public health interventions. It aims to investigate the feasibility of eliminating intestinal schistosomiasis in a highly endemic African setting using an integrated community-based approach. In preparation of this project, we report about baseline data on S.mansoni prevalence, intensity of infection, related hepatosplenic morbidities and their associated factors. METHODS: A cross sectional study was conducted among 930 individuals aged 1-95 years living at Ijinga Island, north-western Tanzania in September 2016. Single stool and urine samples were collected from each study participant and processed using Kato Katz (KK) technique and point-of-care Circulating Cathodic (POC-CCA) antigen test for detection of S.mansoni eggs and antigen respectively. Ultrasonographical examination for S.mansoni hepatosplenic morbidities was done to all participants. For statistical analyses Fisher's exact test, chi-square test, student-t-test, ANOVA and linear regression were used where applicable. RESULTS: Overall based on KK technique and POC-CCA test, 68.9% (95%CI: 65.8-71.8) and 94.5% (95%CI: 92.8-95.8) were infected with S.mansoni. The overall geometrical mean eggs per gram (GMepg) of faeces was 85.7epg (95%CI: 77.5-94.8). A total of 27.1, 31.2 and 51.9% of the study participants had periportal fibrosis (PPF-grade C-F), splenomegaly and hepatomegaly. Risk factors for PPF were being male (aRR = 1.08, 95%CI: 1.02-1.16, P < 0.01), belong to the age group 16-25 years (aRR = 1.23, 95%CI: 105-1.44, P < 0.01), 26-35 years (aRR = 1.42, 95%CI: 1.21-1.67, P < 0.001), 36-45 years (aRR = 1.56, 95%CI:1.31-1.84, P < 0.001) and ≥ 46 years (aRR = 1.64, 95%CI:1.41-1.92, P < 0.001). The length of the left liver lobe was associated with being female (P < 0.03), belong to the age group 1-5 years (P < 0.013), 6-15 years (P < 0.04) and S.mansoni intensity of infection (P < 0.034). Male sex (aRR = 1.15, 95%CI:1.06-1.24, P < 0.001) and belonging to the age groups 16-25 years (aRR = 1.27, 95%CI:1.05-1.54, P < 0.02) or 26-35 years (aRR = 1.32, 95%CI:108-1.61, P < 0.01) were associated with splenomegaly. CONCLUSION: Schistosoma mansoni infection and its related morbidities (hepatomegaly, splenomegaly, periportal fibrosis) are common in the study area. Age, sex and intensity of infection were associated with periportal fibrosis. The prevalence of S.mansoni was above 50% in each age group and based on the observed prevalence, we recommend MDA to the entire community.

Complete or partial split in associating liver partition and portal vein ligation for staged hepatectomy: A systematic review and meta-analysis.

BACKGROUND: Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) has been adopted by liver surgeons in recent years. However, high morbidity and mortality rates have limited the promotion of this technique. Some recent studies have suggested that ALPPS with a partial split can effectively induce the growth of future liver remnant (FLR) similar to a complete split with better postoperative safety profiles. However, some others have suggested that ALPPS can induce more rapid and adequate FLR growth, but with the same postoperative morbidity and mortality rates as in partial split of the liver parenchyma in ALPPS (p-ALPPS). AIM: To perform a systematic review and meta-analysis on ALPPS and p-ALPPS. METHODS: A systematic literature search of PubMed, Embase, the Cochrane Library, and ClinicalTrials.gov was performed for articles published until June 2019. Studies comparing the outcomes of p-ALPPS and ALPPS for a small FLR in consecutive patients were included. Our main endpoints were the morbidity, mortality, and FLR hypertrophy rates. We performed a subgroup analysis to evaluate patients with and without liver cirrhosis. We assessed pooled data using a random-effects model. RESULTS: Four studies met the inclusion criteria. Four studies reported data on morbidity and mortality, and two studies reported the FLR hypertrophy rate and one study involved patients with cirrhosis. In the non-cirrhotic group, p-ALPPS-treated patients had significantly lower morbidity and mortality rates than ALPPS-treated patients [odds ratio (OR) = 0.2; 95% confidence interval (CI): 0.07-0.57; P = 0.003 and OR = 0.16; 95%CI: 0.03-0.9; P = 0.04]. No significant difference in the FLR hypertrophy rate was observed between the two groups (P > 0.05). The total effects indicated no difference in the FLR hypertrophy rate or perioperative morbidity and mortality rates between the ALPPS and p-ALPPS groups. In contrast, ALPPS seemed to have a better outcome in the cirrhotic group. CONCLUSION: The findings of our study suggest that p-ALPPS is safer than ALPPS in patients without cirrhosis and exhibits the same rate of FLR hypertrophy.

Clinical presentation and hematological profile among young and old chronic lymphocytic leukemia patients in Sudan.

OBJECTIVE: To assess the clinical presentation and hematological profile among young (≤ 55 years) and old (> 55 years) chronic lymphocytic leukemia patients in Sudan. RESULT: In the present cross-sectional descriptive study, out of 110 cases studied, among them 31 (28.2%) were young (≤ 55 years) patients with mean age 48 years, and 79 (71.8%) were elder patients (> 55 years) with mean age 66 years, the overall mean age was 62.97 ± 12.06 with range (22-85 years), and 79 (71.8%) were males and 31 (28.2%) were females (M:F = 2.6:1) (P = 0.000). (7.3%) were asymptomatic, 61 (55.5%) presented with nonspecific complains. Generalized lymphadenopathy was seen in 52 (47.27%) with elder predominance (P = 0.03). Splenomegaly, hepatomegaly, thrombocytopenia and anemia were seen in 54 (49.1%), 14 (12.7%), 43 (39.1%) and 38 (34.5%) of patients respectively with male predominance. 54 (49.1%) and 42 (38.18%) of patients presented at Rai high risk and Binet C stages respectively with nearly same age and sex distribution. CLL in Sudan is a disease of elders, same as seen in literature, with high male to female ratio. In general hematological parameters means were noted to be distributed equally according to age and sex groups. Majority of patients were presented with nonspecific symptoms and nearly half of patients presented at late stages as reported in most developing countries.

Genotyping of Cytomegalovirus from Symptomatic Infected Neonates in Iraq.

Among all other viruses, human cytomegalovirus (HCMV) is the most frequent cause of congenital infection worldwide. Strain variation in HCMV may predict severity or outcome of congenital HCMV disease. Previous studies have associated a particular genotype with specific sequelae or more severe illness, but the results were contradictory. There are no previous studies addressing the genotype of HCMV in Iraq. Therefore, the present study is aimed at molecular detection and genotyping of HCMV isolated from symptomatic congenitally/perinatally infected neonates. This prospective study comprised 24 serum samples from symptomatic neonates with congenital/perinatal infection. Viral DNA was extracted from these serum samples; nested polymerase chain reaction was used to amplify the HCMV gB (UL55) gene. Polymerase chain reaction products of the second round of amplification were subjected to direct Sanger sequencing. Bioedit and MEGA5 software (EMBL-EBI, Hinxton, Cambridgeshire, UK) were used for alignment and construction of a phylogenetic tree. Human cytomegalovirus DNA was detected in 23 of 24 samples (95.8%). According to the phylogenetic analysis, three genotypes of the virus were identified; gB1, gB2, and gB3 genotypes. However, the gB4 genotype was not detected. Human cytomegalovirus gB3 was the most frequent genotype: 14 of 24 (58.33%) among symptomatic infected infants, followed by gB1 (6/24; 25%) and gB2 (4/24; 16.67%). A mixed HCMV infection with gB3/gB1 was detected in only one case. Human cytomegalovirus gB3 was the most predominant genotype among symptomatic congenitally/perinatally HCMV-infected neonates. No association was found between B3 genotype and specific clinical presentation. Jaundice was the most common clinical feature among symptomatically infected neonates, followed by hepatosplenomegaly.

Blood transfusion therapy for ß-thalassemia major and hemoglobin E ß-thalassemia: Adequacy, trends, and determinants in Sri Lanka.

BACKGROUND: Regular blood transfusion therapy still remains the cornerstone in the management of ß-thalassemia. Although recommendations are clear for patients with ß-thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E ß-thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with ß-thalassemia major and hemoglobin E ß-thalassemia. METHODS/PROCEDURE: This cross-sectional study was performed among all regularly transfused patents with ß-thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka. Data were collected using an interviewer-administered questionnaire, perusal of clinical records, and physical examination of patients by trained doctors. RESULTS: A total of 328 patients (male 47%) were recruited; 83% had ß-thalassemia major, whereas 16% had hemoglobin E ß-thalassemia. Sixty-one percent of patients had low pretransfusion hemoglobin levels (< 9.0 g/dL) despite receiving high transfusion volumes (> 200 mL/kg/year) by a majority (56%). Median pretransfusion hemoglobin was significantly lower in patients with hemoglobin E ß-thalassemia compared with ß-thalassemia major (P < 0.001); however, there was no difference in requirement for high transfusion volumes over 200 mL/kg/year in both groups (P = 0.14). Hepatomegaly and splenomegaly were more common in hemoglobin E ß-thalassemia and were associated with lower pretransfusion hemoglobin. Transfusion requirements were higher among patients with hepatitis C and in those who are underweight. CONCLUSIONS: Over 60% of regularly transfused patients with ß-thalassemia have low pretransfusion hemoglobin levels despite receiving large transfusion volumes. Patients with hemoglobin E ß-thalassemia are undertransfused and specific recommendations should be developed to guide transfusions in these patients.

[Characteristics of chronic lymphocytic leukemia in Togo]. / Caractéristiques de la leucémie lymphoïde chronique au Togo.

The epidemiological, clinical and biological characteristics of chronic lymphocytic leukemia (CLL) are little studied in Togo. The purpose of this study was to describe these characteristics at the time of diagnosis. We conducted a retrospective and descriptive study of patients diagnosed at the University Hospital Campus from January 1999 to December 2018. Over the past two decades, 87 patients were seen for CLL (20% of patients with hematological malignancies) with an annual prevalence of 4.35 new cases. The average age of patients was 61 +/- 12,48 years (ranging from 17-85 years); 55 women and 32 men (sex ratio M/F 0.58) were enrolled. Clinically, 16 patients (18%) had no tumor syndrome, 33 patients (38%) had lymphadenopathy, 62 patients (71%) splenomegaly and 23 patients (26%) hepatomegaly. Biologically, the mean blood and medullary lymphocyte count was 87188/mm3 (ranging from 7000-481780/mm3) and 75.75% +/- 12,88 (ranging from 44,5-96,5%) respectively; 65 patients (75%) had haemoglobin less than 10g/dl and 20 patients (23%) had platelet count below 100000/mm3. At the time of diagnosis, 67 patients (77%) had Binet stage C, 7 patients (8%) stage B and 13 patients (15%) stage A. The study of biological prognostics factors showed that 66% of cases had ß2-microglobulin level higher than normal and 95% of cases had LDH higher than normal. CLL is a reality in Togo with a predominance of women and an average age of 61 years. Most patients are seen at Binet stage C and their assessment has revealed huge tumor mass with increased LDH and ß2-microglobulin. The current follow-up of these patients will enable us to assess their overall survival.

Transition of Spleen Volume Long After Pediatric Living Donor Liver Transplantation for Biliary Atresia.

PURPOSE: After undergoing the Kasai procedure for biliary atresia (BA), most patients develop severe splenomegaly that tends to be improved by liver transplantation. However, fluctuations in splenic volume long after transplantation remain to be elucidated. PATIENTS AND METHODS: Seventy-one consecutive patients who had undergone pediatric living donor liver transplantation (LDLT) for BA were followed up in our outpatient clinic for 5 years. They were classified into 3 groups according to their clinical outcomes: a good course group (GC, n = 41) who were maintained on only 1 or without an immunosuppressant, a liver dysfunction group (LD, n = 18) who were maintained on 2 or 3 types of immunosuppressants, and a vascular complication group (VC, n = 11). Splenic and hepatic volumes were calculated by computed tomography in 464 examinations and the values compared before and after the treatment, especially in the VC group. RESULTS: Splenic volume decreased exponentially in the GC group, with splenic volume to standard spleen volume ratio (SD) being 1.59 (0.33) 5 years after liver transplantation. Splenic volume to standard spleen volume ratios were greater in the VC and LD groups than in the GC group. Patients in the VC group with portal vein stenosis developed liver atrophy and splenomegaly, whereas those with hepatic vein stenosis developed hepatomegaly and splenomegaly. Interventional radiation therapy tended to improve the associated symptoms. CONCLUSIONS: Fluctuations in splenic volume long after pediatric LDLT for BA may reflect various clinical conditions. Evaluation of both splenic and hepatic volumes can facilitate understanding clinical conditions following pediatric LDLT.

Autoimmune hepatitis in 847 childhood-onset systemic lupus erythematosus population: a multicentric cohort study.

OBJECTIVE: To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: This retrospective multicenter study included 847 patients with cSLE, performed in 10 Pediatric Rheumatology services of São Paulo state, Brazil. AIH was defined according to the International Autoimmune Hepatitis Group criteria (IAHGC). The statistical analysis was performed using the Bonferroni's correction (p < 0.0033). RESULTS: AIH in cSLE patients confirmed by biopsy was observed in 7/847 (0.8%) and all were diagnosed during adolescence. The majority occurred before or at cSLE diagnosis [5/7 (71%)]. Antinuclear antibodies were a universal finding, 43% had concomitantly anti-smooth muscle antibodies and all were seronegative for anti-liver kidney microsomal antibodies. All patients with follow-up ≥18 months (4/7) had complete response to therapy according to IAHGC. None had severe hepatic manifestations such as hepatic failure, portal hypertension and cirrhosis at presentation or follow-up. Further comparison of 7 cSLE patients with AIH and 28 without this complication with same disease duration [0 (0-8.5) vs. 0.12 (0-8.5) years, p = 0.06] revealed that the frequency of hepatomegaly was significantly higher in cSLE patients in the former group (71% vs. 11%, p = 0.003) with a similar median SLEDAI-2 K score [6 (0-26) vs. 7 (0-41), p = 0.755]. No differences were evidenced regarding constitutional involvement, splenomegaly, serositis, musculoskeletal, neuropsychiatric and renal involvements, and treatments in cSLE patients with and without AIH (p > 0.0033). CONCLUSIONS: Overlap of AIH and cSLE was rarely observed in this large multicenter study and hepatomegaly was the distinctive clinical feature of these patients. AIH occurred during adolescence, mainly at the first years of lupus and it was associated with mild liver manifestations.

Childhood Visceral Leishmaniasis: Distinctive Features and Diagnosis of a Re-emerging Disease. An 11-year Experience From a Tertiary Referral Center in Athens, Greece.

BACKGROUND: Visceral leishmaniasis (VL) remains a public health issue in Greece. The aim of this study was to describe the clinical and epidemiologic characteristics of pediatric VL in our region as well as to evaluate the laboratory findings and the diagnostic techniques that are applied. METHODS: We retrospectively reviewed the medical records of all children diagnosed with VL in an 11-year period at a tertiary public hospital in the region of Athens. Demographic features, clinical information and laboratory findings were accessed. RESULTS: A total of 43 cases were recorded during 2005-2015. Median age of the patients was 3.7 years. Pallor (100%), fever (98%), hepatosplenomegaly (55.8%) and appetite loss (32.6%) were the most common presentations of the disease. The predominant laboratory abnormalities were anemia (100%), thrombocytopenia (90.7%), elevated inflammatory markers (86.1%) and decreased albumin/globulin (A/G) ratio (72.1%). Four patients developed secondary hemophagocytic lymphohistiocytosis syndrome, whereas in 3 others abdominal ultrasound showed splenic nodules. Bone marrow aspiration detected Leishmania parasites in 92.7% of cases and the rapid rK39 strip test indicated anti-Leishmania antibodies in 97.1% of children. In addition, all patients in whom indirect immunofluorescent antibody test was implemented had positive results. CONCLUSIONS: VL still affects children in our area. Fever, splenomegaly, anemia and appetite loss are the typical findings in children. Noninvasive techniques (immunofluorescent antibody test, rK39) in combination with bone marrow microscopy are useful in the diagnosis of pediatric VL.

Prevalence of Trypanosoma cruzi and organ alterations in Virginia opossums (Didelphis virginiana) from western Mexico - short communication.

Small populations of Virginia opossum (Didelphis virginiana) in western Mexico are endangered by hunting and natural predators as well as by different kinds of diseases. After two serological analyses using Serodia® latex particle agglutination and indirect haemagglutination (IHA) tests, 35 (53.03%) of 66 collected opossums in two small towns in western Mexico were positive for the presence of Trypanosoma cruzi. Twenty-eight of the 35 seropositive opossums had pathological lesions: 11 had changes in only one organ, 13 in two organs, and four had pathological changes in three organs. Splenomegaly was the most common finding in the examined opossums, followed by hepatomegaly. These potentially fatal pathological changes could contribute to the scarcity of the opossum population, even leading to the extinction of this species in western Mexico.

Schistosoma mansoni Infection and Its Related Morbidity among Adults Living in Selected Villages of Mara Region, North-Western Tanzania: A Cross-Sectional Exploratory Study.

Schistosoma mansoni is highly endemic in Tanzania and affects all age groups at different degrees. However, its control approach does not include adult individuals who are equally at risk and infected. To justify the inclusion of adult individuals in MDA programs in Tanzania, the present study focused on determining the prevalence of S. mansoni infection and its related morbidities among adult individuals. This was a cross sectional study conducted among 412 adult individuals aged 18-89 years living in selected villages of Rorya and Butiama districts located along the shoreline of the Lake Victoria. A pretested questionnaire was used to collect socio-demographic and socio-economic information of participants. Ultrasonographic examinations were conducted for all study participants using the Niamey protocol. A single stool sample was obtained from all study participants and examined for S. mansoni using the Kato-Katz technique. The study revealed a high prevalence of S. mansoni (56.3%), and the majority of infected individuals had a light intensity of infection. Ultrasonographic findings revealed that 22.4% of adult individuals had periportal fibrosis (PPF) (grade C-F), with 18.4% having grade C and D and 4% having grade E and F. Males had the highest prevalence of PPF (31.7% vs 10.8%, P<0.001). Organomegaly was common with 28.5% and 29.6% having splenomegaly and hepatomegaly, respectively. S. mansoni infection and its related morbidities included PPF, hepatomegaly, and splenomegaly were common among adult individuals. To reduce the level of transmission of S. mansoni infection, planned mass drug administration campaigns should include adult individuals living in these villages.