Evaluation of retinal and optic disc vascular structures in individuals before and after Pfizer-BioNTech vaccination.

INTRODUCTION: We conducted this study to detect possible changes in posterior segment structures using the optical coherence tomography angiography (OCTA) in individuals vaccinated with the Pfizer-BioNTech vaccine. MATERIALS AND METHODS: The study included healthcare professionals who presented to the Ophthalmology Clinic of Health Sciences University Antalya Training and Research Hospital, who were scheduled to receive the first dose of the Pfizer-BioNTech vaccine. The exclusion criteria were any eye pathology (e.g., glaucoma, uveitis, diabetic retinopathy, amblyopia), myopia with the absolute value of refractive error >6, axial length >26 mm, history of eye surgery, and presence of systemic disease.OCTA was performed to 40 healthcare professionals before vaccination and on the third day after vaccination. RESULTS: After Pfizer-BioNTech vaccination, there was a statistically significant decrease in the total vascular, foveal vascular, parafoveal vascular and perifoveal vascular density of the superficial capillary plexus and the perifoveal vascular density of the deep capillary plexus and a statistically significant increase in the retinal foveal thickness and total retinal parafoveal thickness compared to the pre-vaccination values (p < 0.0001, p = 0.009, p < 0.0001, p = 0.001, p = 0.04, p = 0.03, and p = 0.05, respectively). CONCLUSION: We consider that the decrease in the retinal vascular density may be due to vascular endothelial damage and inflammation in vaccinated people. It can be suggested that increased inflammation plays a role in the retinal thickness in vaccinated people similar to patients with a history of COVID-19. We also consider that spike protein may be effective in these processes.

Factors affecting the need for silicon tube implantation in cases of congenital dacriostenosis: time of delivery, type of delivery and age of operation.

PURPOSE: To evaluate the delivery time, type of delivery, age at the time of operation, and surgical methods applied in cases of congenital nasolacrimal duct obstruction (CNLDO). METHODS: This study retrospectively included a total of 207 eyes of 160 cases who underwent surgery for CNLDO between February 2012 and April 2021. According to their age at the time of operation, the cases were divided into 0-12, 12-24, 24-36, 36-48 and > 48 months groups. The cases were evaluated as term/preterm according to the delivery time and cesarean section/vaginal delivery according to the type of delivery. The surgical methods applied were examined as probing alone and probing plus silicone tube implantation. RESULTS: Of the cases, 146 (91.2%) were born at term and 14 (8.7%) were born preterm, and there was no statistically significant difference in the silicone tube implantation rates according to the time of delivery. The rate of silicone tube implantation was statistically significantly higher in the vaginal delivery group than in the cesarean section group (p = 0.001; p < 0.01). The rate of silicone tube implantation was higher in those who were older than the age of the operation. CONCLUSIONS: Although the rate of those who were born by cesarean section was higher in probing cases, those who required silicone intubation were more common in those who were born vaginally. This suggests that dacryostenosis in the vaginally born cases resulted from a persistent structural and anatomical obstruction despite the presence of a high intrauterine pressure increase and enzymatic lysis.

Assessment of hepatic involvement by two-dimensional shear wave elastography in paediatric patients with cystic fibrosis.

AIM: This study aimed to investigate parenchymal changes in the liver in paediatric patients with cystic fibrosis (CF) and to analyse diagnostic performance of two-dimensional shear wave elastography (2D-SWE) for the detection of hepatic involvement. METHODS: Patients with CF treated and followed at our centre were evaluated prospectively. All patients underwent liver tissue stiffness (TS) measurements by 2D-SWE, in addition to routine clinical assessments, laboratory work-up and abdominal ultrasound imaging. Data from patients with CF were compared with healthy control subjects. RESULTS: This study included 39 patients with CF and 37 healthy controls. Patients had a mean body weight of 29.9 (16.6-55) kg, mean age of 9 (5-17) years, mean height of 130 (107-172) cm and a mean body mass index of 16.1 (12.8-21.4) kg/m2 . Average SWE values of the liver were 1.02 (0.70-1.60) m/s in patients with CF (n = 39) and 0.89 (0.60-1.35) m/s in healthy controls (n = 37). Cystic fibrosis patients had significantly increased tissue stifness by liver elastography compared to controls (P = 0.005). CONCLUSION: Parenchymal liver changes may occur early in cystic fibrosis, which cannot be detected by conventional ultrasonography but may be demonstrated by 2D-SWE. Based on this cross-sectional study, 2D-SWE may be a promising, simple and non-invasive modality for objective monitoring of patients with cystic fibrosis who require lifelong follow-up, by providing numerical data for tissue stiffness early in the disease.

Evaluation of the Relationship Between Carotid Intima Media Thickness and Dietary Compliance in Pediatric Celiac Patients: A Single-Center Pilot Study.

OBJECTIVES: This study aimed to show the relationship between gluten-free diet (GFD) compliance in Celiac Disease (CD) and early atherosclerotic findings in pediatric patients and to test the effectiveness of carotid-intima-media-thickness (cIMT) to possibly predict long-term compliance to the GFD. METHODS: Patients from 6 to 18 years of age with a diagnosis of CD confirmed by endoscopic duodenal biopsy who were followed at our hospital's pediatric gastroenterology outpatient clinic between November 2020 and May 2021 were evaluated in this single-center, prospective study. The study patients were divided into two groups according to GFD compliance. Serologic and biochemical tests were conducted routinely during the follow-up period. cIMT was measured using ultrasound for both groups. RESULTS: A total of 80 patients (GFD-non-compliant: n = 35, GFD-compliant: n = 45) were evaluated. No significant differences were observed between the groups in terms of demographic data and pathology results. The mean cIMT value was 0.44 ± 0.028 mm for the GFD-compliant group and 0.54 ± 0.036 mm for the GFD-non-compliant group, with a statistically significant between-group difference (P < .001). The receiver operating characteristic curve analysis showed an area under the curve of 0.992 (95% confidence interval [CI]: 0.978-1, P < .001) for discrimination of the groups. In addition, a cutoff value of 0.486 mm for cIMT showed 96% (95% CI: 0.83-0.99) sensitivity and 94% (95% CI: 0.79-0.99) specificity for distinguishing GFD-compliant patients from non-GFD-compliant patients. CONCLUSION: In this study, the relationship between long-term GFD compliance and cIMT was demonstrated in CD. Currently used by some authors for the assessment of preclinical atherosclerosis, cIMT can also be used as a long-term indicator of dietary compliance as well as cardiovascular risk.

Is Henoch-Schönlein purpura a susceptibility factor for functional gastrointestinal disorders in children?

Henoch-Schönlein purpura (HSP), the most common childhood vasculitis is characterized by non-thrombocytopenic palpable purpura, arthritis/arthralgia, abdominal pain and renal involvement. Functional gastrointestinal disorders (FGIDs) are heterogeneous disease spectrum with unclear etiology and include the most common subtypes: functional dyspepsia, irritable bowel syndrome (IBS), functional abdominal pain and functional constipation. Formerly, FGIDs were known as non-organic disorders; however, recent advances revealed that low-grade inflammation may also play a role. We aimed to clarify whether HSP predisposes to FGIDs in pediatric population. Seventy-four children with HSP, diagnosed at least 6 months before the study and 78 healthy controls were enrolled to the study. Patients with red flag signs for organic GI disorders were excluded. Rome IV criteria were utilized for FGIDs diagnosis. We compared the frequencies of FGIDs between HSP patients and healthy subjects. We also examined the parameters including age, abdominal pain, arthralgia, bloody stool, renal involvement and treatment with corticosteroids and laboratory results at HSP diagnosis such as erythrocyte sedimentation rate, C-reactive protein, hemoglobin, leukocytes and platelet counts among patients with and without FGIDs. Overall FGIDs and IBS frequency were 35.1% (n = 26) and 10.8% (n = 8) in HSP patients, 19.2% (n = 15) and 2.6% (n = 2) in healthy controls, respectively. Disease characteristics and laboratory parameters at disease onset were similar between HSP patients with and without FGIDs. Overall FGIDs rate, particularly IBS were statistically higher in HSP patients. We speculate that children with preceding HSP may be predisposed to FGIDs. Since the FGIDs pathogenesis is still remains unclear, further studies are needed to confirm this hypothesis and clarify the etiology. Physicians also should pay attention to FGIDs in HSP patients with ongoing abdominal pain and thus prevent this comorbidity with dietary and psychologic measures.

Score-based diagnostic approach to celiac disease and bone mineral density.

BACKGROUND: The aim of this study was to assess the performance of a score-based diagnostic approach (SBDA) proposed in the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) 2012 guideline, and the usefulness of bone mineral density (BMD) measurement in SBDA as an objective finding in the diagnosis of celiac disease (CD). METHODS: The SBDA scores of 153 biopsy-proven celiac diagnosed children (derived from symptomatology, serology, human leukocyte antigen [HLA] analysis, histology) were calculated. Additionally, BMD Z scores obtained at diagnosis were also investigated. The diagnostic sensitivity of SBDA was tested in different scenarios in which low BMD was scored as a diagnostic finding. RESULTS: The mean age of children was 9.48 ± 3.59 years and 54.2% were female. All patients scored ≥4, which is the minimum score to diagnose CD in SBDA. Mean BMD Z score in 142 of 153 patients was -2.70 ± 1.16, and 73.9% of them were below -2. Moreover, different diagnostic scenarios without histology were tested. In one of them, BMD and HLA were not included and the sensitivity was 85.2%. In another one, low BMD was scored as an equivalent of malabsorption, HLA was not included and sensitivity was 97.2%. The sensitivities of these scenarios were significantly different (P = 0.001). CONCLUSION: In the absence of both HLA and histology, accepting low BMD as an equivalent of malabsorption drastically increased the diagnostic sensitivity, while SBDA had limited success. Therefore, BMD might be useful when HLA and biopsy are not available.

Trientine-induced Rhabdomyolysis in an Adolescent with Wilson's Disease.

BACKGROUND: Drugs are very important in the etiology of nontraumatic rhabdomyolysis. CASE DESCRIPTIONS: A 16-year-old male patient with Wilson's disease was admitted for myoclonic contractions. Oral trientine was started for neurological problems and tremor on the hands due to D-penicillamine 1 month ago. Patient was oligoanuric, and his creatine kinase level was 15197 U/L. Rhabdomyolysis was associated with trientine, and trientine treatment was stopped. Hemodiafiltration was performed. The patient began to urinate on the 24th day. CONCLUSION: This is the first pediatric patient with rhabdomyolysis induced by trientine. Drugs used should be questioned carefully in patients with rhabdomyolysis. HOW TO CITE THIS ARTICLE: Aslan N, Yavuz S, Yildizdas D, Horoz OO, Coban Y, Tumgor G, et al. Trientine-induced Rhabdomyolysis in an Adolescent with Wilson's Disease. Indian J Crit Care Med 2019;23(10):489-490.

Pemphigus herpetiformis-type drug reaction caused by erdosteine containing mucolytic in a child.

Drug-related pemphigus is very rare in children. Erdosteine is a thiol compound having mucoactive, antioxidant, anti-inflammatory, and antitussive effects and is reported to be safe for treatment of acute respiratory tract diseases in children. Herein, we report a 9-year-old boy presented with pemphigus herpetiformis associated with anti-desmoglein 1 antibodies due to erdosteine consumption.

Skin punch biopsy sectioning: before or after tissue processing?

BACKGROUND: We intended to study whether there is a meaningful difference in microscopic examination between dividing a biopsy section into two equal parts before tissue processing (first method) or after (second method). METHODS: A total of 400 cases were included in the study. Punch biopsies (PB) were cut into two pieces using the first method in 200 cases and just before paraffin embedding in another 200 cases using the second method. We microscopically evaluated the epidermal mesh view, the presence of a cross-cut hair follicle and bow shape because of epidermal angling, the presence of two pieces on the slide and if there was a difference of >2 mm between the parts, and the number of new sections and new slides. RESULTS: Cross-cut hair follicle (p = 0.018), epidermal mesh view (p = 0.036), difference of >2 mm between the parts (p = 0.008), the number of new sections (p < 0.001) and new slides (p < 0.001) were considerably higher when the first method was used compared with the second method. The presence of two pieces was less (p < 0.001) when using the first method. CONCLUSIONS: We noted a meaningful difference in the quality of microscopic evaluation between the first and second methods. Better sections were obtained with the second method. In addition, the decrease in the number of new slides will reduce workload, archival work and cost.

Evaluation of depression and anxiety in parents of children undergoing cardiac catheterization.

OBJECTIVE: This study aimed to determine pre-procedure depression and anxiety levels among a group of parents whose children had congenital heart disease and were undergoing angiography. METHODS: The study comprised parents of 73 congenital heart disease patients undergoing angiography. The Beck Depression Inventory (BDI) and the Beck Anxiety Inventory (BAI) were used to evaluate the depression and anxiety scores. RESULTS: Sixty-one patients (83.6%) had acyanotic congenital heart disease, and 25 patients (34.2%) were undergoing diagnostic angiography. BDI scores among the mothers determined that 8 (11%) had mild, 14 (19.2%) moderate, and 10 (13.7%) severe depression. Their BAI scores showed that 16 (21.9%) had mild, 8 (11%) moderate, and 13 (17.8%) severe anxiety. BDI scores for the fathers showed that 12 (16.4%) had mild, 10 (13.7%) moderate, and 8 (11%) severe depression. Their BAI scores showed that 12 (16.4%) had mild, 10 (13.7%) moderate, and 8 (11%) severe anxiety. A comparison of mothers of cyanotic patients and those of acyanotic patients in terms of depression and anxiety levels revealed a statistically significant difference (p=0.050 and 0.043, respectively). CONCLUSION: Angiography was associated with increased levels of depression and anxiety in parents of children with congenital heart diseases. In comparison to parents of patients with acyanotic congenital heart disease, mothers of patients with cyanotic congenital heart disease had significantly higher levels of depression and anxiety.

Comparison of the Quantitative Values of Peripapillary, Macular and Choriocapillary Microvascular Structures According to the Presence of Lupus Nephritis in Patients with Systemic Lupus Erythematosus.

PURPOSE: To determine whether there are quantitative changes in macular, choriocapillary, and peripapillary microvascular structures using optical coherence tomography angiography (OCTA) due to the presence of lupus nephritis (LN) in patients with systemic lupus erythematosus (SLE) and to investigate the correlation between these quantitative values and disease duration. METHODS: Fifty -five patients followed up in the rheumatology clinic with an SLE diagnosis were evaluated. As the control group, 61 eyes of 61 age- and gender-matched healthy individuals were included. The patients with SLE were further divided into two groups: those with LN (29 eyes) and those without LN (26 eyes). Macular, choriocapillary, and peripapillary microvascular structures were quantitatively analyzed with OCTA and compared between the three study groups. A correlation analysis of the measured quantitative values and disease duration was also performed. RESULTS: In macular microvascular (MMV) analysis, the vessel densities (VDs) of the superficial capillary plexus (SCP) decreased in both SLE groups, while those of the deep capillary plexus (DCP) decreased only in the SLE group with LN. The foveal density significantly decreased in the SLE group with LN compared to the control group, there were no significant differences in terms of the radial peripapillary capillary VDs or the choriocapillaris flow area. Disease duration was not correlated with any of the quantitative parameters measured by OCTA in either SLE group. CONCLUSIONS: Identifying differences in retinal microvascular circulation in SLE patients with kidney damage helps predict possible nephropathy and therefore guides the treatment process of this patient.

Do intravitreal anti-VEGF injections have an effect on corneal endothelial remodeling in children with premature retinopathy?

PURPOSE: This study was carried out to investigate the effects of retinopathy of prematurity (ROP) and intravitreal antivascular endothelial growth factor (VEGF) injections on the corneal endothelium in the childhood period of patients who have had ROP. METHODS: The material of comparative case-control clinical study consisted of patients followed up with ROP between February 2013 and February 2023. The eyes in the study group were divided into two subgroups consisting of those who received intravitreal anti-VEGF injections (subgroup 1) and those who were followed up only (subgroup 2). Central corneal endothelial cell density (ECD), coefficient of variation (CV), central corneal thickness (CCT), and pleomorphism parameters in the childhood period were evaluated by corneal specular microscopy and compared with age-matched healthy control subjects. RESULTS: There were 84 eyes of 42 patients with ROP in the study group and 80 eyes of 40 healthy children in the control group. Mean CCT was significantly higher in subgroup 1 and the control group than in subgroup 2 (p = 0.037), and mean ECD was significantly higher in subgroup 2 than in subgroup 1 and the control group (p < 0.001). There was no significant difference between subgroup 1 and the control group in mean ECD and CCT values (p = 1.000 for both cases). CONCLUSIONS: Considering that ROP patients who received intravitreal anti-VEGF injections had more advanced-stage ROP than ROP patients who were followed up only, these findings suggest that intravitreal anti-VEGF applications in ROP cases may lead to corneal endothelial parameters similar to those of healthy eyes.

Explainable Boosting Machine approach identifies risk factors for acute renal failure.

BACKGROUND: Risk stratification and outcome prediction are crucial for intensive care resource planning. In addressing the large data sets of intensive care unit (ICU) patients, we employed the Explainable Boosting Machine (EBM), a novel machine learning model, to identify determinants of acute kidney injury (AKI) in these patients. AKI significantly impacts outcomes in the critically ill. METHODS: An analysis of 3572 ICU patients was conducted. Variables such as average central venous pressure (CVP), mean arterial pressure (MAP), age, gender, and comorbidities were examined. This analysis combined traditional statistical methods with the EBM to gain a detailed understanding of AKI risk factors. RESULTS: Our analysis revealed chronic kidney disease, heart failure, arrhythmias, liver disease, and anemia as significant comorbidities influencing AKI risk, with liver disease and anemia being particularly impactful. Surgical factors were also key; lower GI surgery heightened AKI risk, while neurosurgery was associated with a reduced risk. EBM identified four crucial variables affecting AKI prediction: anemia, liver disease, and average CVP increased AKI risk, whereas neurosurgery decreased it. Age was a progressive risk factor, with risk escalating after the age of 50 years. Hemodynamic instability, marked by a MAP below 65 mmHg, was strongly linked to AKI, showcasing a threshold effect at 60 mmHg. Intriguingly, average CVP was a significant predictor, with a critical threshold at 10.7 mmHg. CONCLUSION: Using an Explainable Boosting Machine enhance the precision in AKI risk factors in ICU patients, providing a more nuanced understanding of known AKI risks. This approach allows for refined predictive modeling of AKI, effectively overcoming the limitations of traditional statistical models.

Prevalence and clinical significance of antithyroid antibodies in children with immune thrombocytopenic purpura.

BACKGROUND AND OBJECTIVE: To determine the prevalence and the clinical significance of thyroid autoantibodies and their influence on treatment response in children with idiopathic thrombocytopenic purpura (ITP). PATIENT AND METHOD: We retrospectively analyzed the antithyroglobulin (anti-TG) and antithyroid peroxidase (anti-TPO) antibodies from the records of 151 ITP patients who were admitted to the Pediatric Hematology Department of Gaziantep University between 2009 and 2012. RESULTS: Anti-TPO and/or anti-TG was found positive in 38 (36.8%) of 103 patients whose thyroid autoantibody levels were measured. The comparison of positivity ratios of autoantibodies between acute and chronic ITP patients showed no significant difference. However, the separate comparison of each group of ITP patients with control group showed significantly high positivity ratios of autoantibodies in ITP patients. The initial mean platelet count of anti-TPO positive patients at diagnosis was significantly less than that of the negative patients (P = .008). One month after treatment, platelet count of anti-TPO positive patients was significantly less than that of the negative patients (P = .01). Moreover, the mean platelet counts of anti-TPO positive patients were significantly less than those of the negative patients after intravenous immunoglobulin treatment (P < .001). CONCLUSION: We demonstrated that the thyroid-autoimmune-diseases-related autoantibodies are frequently found in childhood ITP. Although no recommendation is found in international guidelines regarding screening for thyroid autoantibodies in patients with ITP, in view of the high incidence of antithyroid antibodies and their potential negative effect on treatment response, screening these patients for such antibodies would be recommended.

Prevalence of appendicolith in children with acute appendicitis and its correlation with disease severity.

OBJECTIVE: Appendicitis typically develops secondary to obstruction of appendiceal lumen and one of the causes of obstruction is appendicolith. Appendicolith has become a relevant issue due to heightened interest in the treatment of uncomplicated appendicitis with antibiotics. This study aimed to determine the prevalence of appendicolith in pediatric patients with appendicitis and to investigate the association between the presence of appendicoliths and radiological disease severity. METHODS: Patients under the age of 18 diagnosed with appendicitis between March 2021 and April 2022 and had available preoperative computed tomography (CT) images were identified retrospectively. The presence of an appendicolith and if present, its longest diameter in the axial plane, its visibility on direct radiographs, appendiceal diameter, degree of inflammation, and the presence of perforation were evaluated. Radiological severity of inflammation was rated on a 3-point scale. RESULTS: CT scans were available in 77 (32.1%) of 240 patients with histopathologically confirmed diagnosis of acute appendicitis. 39% (n=30) of the patients were girls and the median age was 13 years. The prevalence of appendicoliths detected on CT scans was 32.5% (n=25) and the median size of appendicoliths was 6 mm. In only 1 patient, appendicolith was detected by direct radiography. The median appendiceal diameter was significantly greater in the group with appendicoliths (10 mm vs. 8 mm; p=0.001). A moderate correlation was found between appendicolith size and appendiceal diameter (r=0.407, p=0.043). Perforation was present in 10.4% (n=8) of the patients with appendicitis and 25% (n=2) of them had appendicoliths. The presence of appendicoliths was not significantly associated with the occurrence of perforation (p=0.485). Periappendiceal inflammation scores were 1.52±0.74 in the group with appendicoliths and 1.42±0.63 in the group without appendicoliths (p=0.591). CONCLUSION: The prevalence of CT-detected appendicoliths was 32.5% in pediatric patients with appendicitis. Patients with appendicoliths showed higher inflammation scores and greater appendiceal diameter than those without appendicoliths. These factors may be associated with poor outcomes in patients with appendicoliths treated with antibiotics. Therefore, knowledge of the prevalence of appendicoliths and questioning their presence may guide clinicians when deciding on the suitability of nonoperative treatment in a patient diagnosed with uncomplicated acute appendicitis.

OCT-A evaluation of the retinal and choroidal structures of patients with a history of radiotherapy due to nasopharyngeal carcinoma.

BACKGROUND: To perform the optical coherence tomography angiography (OCT-A) evaluation of the microvascular structures of the retina and choroidal tissue in asymptomatic patients who received radiotherapy for nasopharyngeal carcinoma and to compare the results to those of healthy individuals. METHODS: Ophthalmological examinations were performed in all asymptomatic patients without vascular or systemic diseases, or fundus findings who had received radiotherapy at least two years earlier. Then, OCT-A scans were obtained. Foveal, parafoveal, and whole retinal thicknesses, vessel densities in the superficial and deep capillary plexuses, subfoveal choroidal thickness, the non-flow area in the superficial capillary plexus, and the choriocapillaris flow area were measured and compared to the values of the healthy control group. RESULTS: The radiotherapy group had significantly lower deep capillary plexus vascular density and subfoveal choroidal thickness values and significantly higher choriocapillaris flow area values compared to the control group. CONCLUSIONS: We consider that OCT-A is useful in the early diagnosis of radiation retinopathy that may develop during follow-up in patients with nasopharyngeal carcinoma who have received radiotherapy.

Patient-Related Factors Associated with Adverse Outcomes Following Weaning from Veno-Arterial Extracorporeal Membrane Oxygenation.

BACKGROUND: Veno-arterial extracorporeal membrane oxygenation (vaECMO) removal reflects a critical moment and factors of adverse outcomes are incompletely understood. Thus, we studied various patient-related factors during vaECMO removal to determine their association with outcomes. METHODS: A total of 58 patients from a university hospital were included retrospectively. Demographic, clinical, and echocardiographic parameters were recorded while under vaECMO support, as well as the need for inotropic and vasoactive-inotropic scores (VIS). Successful weaning was defined as 28-day survival without reinitiation of vaECMO. RESULTS: Patient age differed significantly between patients with a successful and a failed vaECMO weaning (54 ± 14 vs. 62 ± 12 years, p = 0.029). In univariable logistic regression, age (OR 0.952 (0.909-0.997), p = 0.038), the necessities for inotropic agents at the time of echocardiography (OR 0.333 (0.113-0.981), p = 0.046), and vaECMO removal (OR 0.266 (0.081-0.877), p = 0.030) as well as the dobutamine dose during removal (OR 0.649 (0.473-0.890), p = 0.007), were significantly associated with a successful weaning from vaECMO. Age (HR 1.048 (1.006-1.091), p = 0.024) and the VIS (HR 1.030 (1.004-1.056), p = 0.025) at the time of vaECMO removal were independently associated with survival in bivariable Cox regression. In Kaplan-Meier analysis, a VIS of >5.1 at vaECMO removal was associated with impaired survival (log-rank p = 0.025). CONCLUSIONS: In this cohort, age and the extent of vasoactive-inotropic agents were associated with adverse outcomes following vaECMO, whereas echocardiographic biventricular function during vaECMO support was not.

The frequency of Duchenne muscular dystrophy/Becker muscular dystrophy and Pompe disease in children with isolated transaminase elevation: results from the observational VICTORIA study.

Introduction: Elevated transaminases and/or creatine phosphokinase can indicate underlying muscle disease. Therefore, this study aims to determine the frequency of Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) in male children and Pompe disease (PD) in male and female children with isolated hypertransaminasemia. Methods: This multi-center, prospective study enrolled patients aged 3-216 months with serum alanine transaminase (ALT) and/or aspartate transaminase (AST) levels >2× the upper limit of normal (ULN) for ≥3 months. Patients with a known history of liver or muscle disease or physical examination findings suggestive of liver disease were excluded. Patients were screened for creatinine phosphokinase (CPK) levels, and molecular genetic tests for DMD/BMD in male patients and enzyme analysis for PD in male and female patients with elevated CPK levels were performed. Genetic analyses confirmed PD. Demographic, clinical, and laboratory characteristics of the patients were analyzed. Results: Overall, 589 patients [66.8% male, mean age of 63.4 months (standard deviation: 60.5)] were included. In total, 251 patients (188 male and 63 female) had CPK levels above the ULN. Of the patients assessed, 47% (85/182) of male patients were diagnosed with DMD/BMD and 1% (3/228) of male and female patients were diagnosed with PD. The median ALT, AST, and CPK levels were statistically significantly higher, and the questioned neurological symptoms and previously unnoticed examination findings were more common in DMD/BMD patients than those without DMD/BMD or PD (p < 0.001). Discussion: Questioning neurological symptoms, conducting a complete physical examination, and testing for CPK levels in patients with isolated hypertransaminasemia will prevent costly and time-consuming investigations for liver diseases and will lead to the diagnosis of occult neuromuscular diseases. Trial Registration: Clinicaltrials.gov NCT04120168.

Hemophagocytic lymphohistiocytosis in 2 pediatric patients secondary to hepatitis A virus infection.

Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by uncontrolled activation of cytotoxic T cells and antigen-presenting cells. Common clinical manifestations include high fever, maculopapular rash, neurological symptoms, coagulopathy, and abnormal liver function tests [1]. HLH can be either primary, that is, due to an underlying genetic defect, or secondary, associated with malignancies, autoimmune diseases, or infections. The true incidence of secondary HLH is difficult to define. Infection associated HLH are most commonly associated with viral infections mainly of the herpes group, with Epstein-Barr virus (EBV) that is proposed to be the most common cause [2]. Despite the high incidence of hepatitis A virus (HAV) infection in the pediatric population in general, there are few pediatric case reports in the literature about HAV-associated hemophagocytic syndrome [3]. We encountered 2 patients with HAV-associated hemophagocytic syndrome.

Treatment With Balloon Angioplasty of Chronic Portal Vein Thrombosis.

The therapeutic options in portal vein thrombosis cases of young age and low weight, as in this case, are limited. Interventional radiologists also have minimal experience in pediatric patients. There are no reported cases anywhere worldwide, especially in this age group. However, we think that balloon angioplasty can be safely applied in cases in which esophageal variceal bleeding cannot be controlled using traditional treatment.