Mild Dilatation Research Articles

The effects of daily dose of intense exercise on cardiac responses and atrial fibrillation.

Atrial fibrillation (AF) is a supraventricular tachyarrhythmia that is strongly associated with cardiovascular (CV) disease and sedentary lifestyles. Despite the benefits of exercise on overall health, AF incidence in high-level endurance athletes rivals that of CV disease patients, suggesting a J-shaped relationship with AF. To investigate the dependence of AF vulnerability on exercise, we varied daily swim durations (120, 180 or 240minday-1 ) in 7-week-old male CD1mice. We assessed mice after performing equivalent amounts of cumulative work during swimming (i.e. ∼700L O2 kg-1 ), as determined from O2 consumption rates ( ). The mean during exercise increased progressively throughout the training period and was indistinguishable between the swim groups. Consistent with similar improvements in aerobic conditioning induced by swimming, skeletal muscle mitochondria content increased (P=0.027) indistinguishably between exercise groups. Physiological ventricular remodelling, characterized by mild hypertrophy and left ventricular dilatation, was also similar between exercised mice without evidence of ventricular arrhythmia inducibility. By contrast, prolongation of daily swim durations caused progressive and vagal-dependent heart rate reductions (P=0.008), as well as increased (P=0.005) AF vulnerability. As expected, vagal inhibition prolonged (P=0.013) atrial refractoriness, leading to reduced AF vulnerability, although still inducible in the 180 and 240min swim groups. Accordingly, daily swim dose progressively increased atrial hypertrophy (P=0.003), fibrosis (P<0.001) and macrophage accumulation (P=0.006) without differentially affecting the ventricular tissue properties. Thus, increasing daily exercise duration drives progressively adverse atrial-specific remodelling and vagal-dependent AF vulnerability despite robust and beneficial aerobic conditioning and physiological remodelling of ventricles and skeletal muscle. KEY POINTS: Previous studies have suggested that a J-shaped dose-response relationship exists between physical activity and cardiovascular health outcomes, with moderate exercise providing protection against many cardiovascular disease conditions, whereas chronic endurance exercise can promote atrial fibrillation (AF). We found that AF vulnerability increased alongside elevated atrial hypertrophy, fibrosis and inflammation as daily swim exercise durations in mice were prolonged (i.e. ≥180minday-1 for 6weeks). The MET-hweek-1 (based on O2 measurements during swimming) needed to induce increased AF vulnerability mirrored the levels linked to AF in athletes. These adverse atria effects associated with excessive daily exercise occurred despite improved aerobic conditioning, skeletal muscle adaptation and physiological ventricular remodelling. We suggest that atrial-specific changes observed with exercise arise from excessive elevations in venous filling pressures during prolonged exercise bouts, which we argue has implications for all AF patients because elevated atrial pressures occur in most cardiovascular disease conditions as well as ageing which are linked to AF.

Retroperitoneal lymphadenopathy: An underappreciated contributor to acute kidney failure in patients with cancer

Introduction: Retroperitoneal lymphadenopathy represents a unique and infrequent complication within various malignancies. This case report exemplifies the intricate diagnostic challenges that arise when identifying nondilated obstructive nephropathy in the context of retroperitoneal lymphadenopathy due to diffuse large B-cell lymphoma (DLBCL), particularly when conventional imaging fails to reveal the hallmark feature of hydronephrosis. Case description: We present a 66-year-old male with relapsed chronic lymphocytic leukemia post-allogeneic stem cell transplantation. His direct admission to the hematology/oncology service was due to failure to thrive. Despite the absence of hydronephrosis on imaging, the patient developed volume overload and increased serum creatinine levels. An ultrasound unveiled mild bilateral collecting system dilation, while laboratory findings did not provide valuable insight into the underlying etiology. A Lasix renal scan confirmed split renal function with minimal bilateral excretion. High clinical suspicion resulted in an MRI scan showing interval enlargement of retroperitoneal lymph nodes and pelvicaliectasis, which prompted urological and interventional radiology involvement. Conclusions: This case underscores the complexity of diagnosing retroperitoneal lymphadenopathy and pelvicaliectasis, in DLBCL, particularly when hydronephrosis is absent on imaging. It emphasizes the significance of clinical acumen, collaboration among specialties, and the use of advanced imaging techniques. Timely recognition of nondilated obstructive nephropathy in the setting of retroperitoneal lymphadenopathy is pivotal for guiding appropriate management strategies and optimizing patient outcomes in the intricate clinical setting.

Comparison of Chemoembolization Outcomes Using 70-150 µm and 100-300 µm Drug-Eluting Beads in Treating Small Hepatocellular Carcinoma: A Korean Multicenter Study.

To evaluate the outcomes of drug-eluting bead transarterial chemoembolization (DEB-TACE) according to the size of the beads for the treatment of small hepatocellular carcinoma (HCC). This retrospective study included 212 patients with a single HCC ≤5 cm from five tertiary institutions. One hundred and nine patients were treated with 70-150-µm doxorubicin DEBs (group A), and 103 patients received 100-300-µm doxorubicin DEBs (group B). The initial tumor response (assessed between 3 weeks and 2 months after DEB-TACE), time to local tumor progression (TTLTP), restricted mean duration of complete response (RMDCR), rate of complications, incidence of post-embolization syndrome, and length of hospital stay were compared between the two groups. Logistic regression was used to analyze prognostic factors for initial tumor response. The initial objective response rates were 91.7% (100/109) and 84.5% (87/103) for groups A and B, respectively (P = 0.101). In the subgroup analysis of tumors ≤3 cm, the initial objective response rates were 94.6% (53/56) and 78.0% (39/50) for groups A and B, respectively (P = 0.012). There was no significant difference in the TTLTP (median, 23.7 months for group A vs. 19.0 months for group B; P = 0.278 [log-rank], 0.190 [multivariable Cox regression]) or RMDCR at 24 months (11.4 months vs. 8.5 months, respectively; P = 0.088). In the subgroup analysis of tumors >3-cm, the RMDCR at 24 months was significantly longer in group A than in group B (11.8 months vs. 5.7 months, P = 0.024). The incidence of mild bile duct dilatation after DEB-TACE was significantly higher in group B than in group A (5.5% [6/109] vs. 18.4% [19/103], P = 0.003). DEB-TACE using 70-150-µm microspheres demonstrated a higher initial objective response rate in ≤3-cm HCCs and a longer RMDCR at 24 months in 3.1-5-cm HCCs compared to larger DEBs (100-300-µm).

Manifestações neurológicas da Síndrome de Ogden: relato de caso

INTRODUCTION: The syndrome related to a mutation in the N-alpha-acetyltransferase 10 (NAA10) gene, an X-linked recessive disorder, Ogden Syndrome, presents in boys with severe developmental delay, central hypotonia, dysmorphic features, cardiac malformations, and early death in the first months of life. CASE REPORT: Male infant with hypotonia and craniofacial dysmorphisms: prominent forehead, triangular facies, bilateral microphthalmia, superior oblique palpebral fissure and epicanthus, increased nasolabial filter, tapered upper lip, sparse blond hair, long and light eyelashes. Delay in neuropsychomotor development, brief episodes of oromasticatory movements, sialorrhea, horizontal nystagmus, excessive sleepiness, and hypo-responsiveness, with oxygen saturation drop and bradycardia. Electroencephalogram: bursts of diffuse slow waves. Echocardiogram and electrocardiogram without alterations. Cranial resonance: cortical thickening in the right frontal lobe, small encephalomalacic area; signs of previous ischemic insult in the territory of the right posterior cerebral artery; areas of signal alteration in the central and deep white matter adjacent to the lateral ventricles with mild compensatory dilation of the same; and diffuse thinning of the corpus callosum of undetermined nature, treatment initiated. Genome identified a likely pathogenic variant in the NAA10 gene. Under treatment with phenobarbital and oxcarbazepine, and multiprofessional follow-up. DISCUSSION/CONCLUSION: Many patients present neuroimaging alterations, mainly: corpus callosum hypoplasia and microcephaly. Epilepsy around 69%, generalized, described eyelid myoclonias or spasms, or as identified in this patient: oromasticatory automatism. There is a need to include Ogden Syndrome in differential diagnoses and investigate the NAA10 gene in epilepsy and intellectual disability panels, even without multisystemic manifestations.

Cardiovascular, autonomic symptoms and quality of life in children with hypermobile Ehlers-Danlos syndrome.

Hypermobile Ehlers-Danlos syndrome is a connective tissue disorder characterized by joint hypermobility and other systemic manifestations. Cardiovascular, autonomic symptoms and dysautonomia are frequently reported in adults with hypermobile Ehlers-Danlos syndrome and have been shown to have a negative impact on quality of life. However, there is scant literature on autonomic symptoms in pediatric patients with hypermobile Ehlers-Danlos syndrome. This study aims to characterize cardiovascular symptoms and diagnoses in pediatric patients with hypermobile Ehlers-Danlos syndrome and evaluate the impact of autonomic symptoms on quality of life. As part of a longitudinal study, a consecutive sample of 70 patients with Ehlers-Danlos syndromes were recruited at routine clinical care visits. Medical history was reviewed, demographics were obtained, and patient-reported outcomes were completed by the patients. The average age of 70 patients was 15.8 years, and the majority were females (89%) and Caucasian (89%). The most common cardiovascular diagnoses were orthostatic intolerance (59%), dysautonomia (47%), and postural orthostatic tachycardia syndrome (21%). Most patients had an echocardiogram (77%), that was normal (82%). No patients had mitral valve prolapse, and only one patient had mild aortic root dilation (2%). Patient-reported outcomes revealed decreased quality of life associated with autonomic symptoms. This study shows that most children with hypermobile Ehlers-Danlos syndrome have cardiovascular and autonomic symptoms, which have a negative impact on quality of life. Few patients with hypermobile Ehlers-Danlos syndrome have structural abnormalities on echocardiogram, which suggests that the cardiovascular symptoms experienced by patients are not due to structural cardiovascular disease and possibly reflective of autonomic pathology, though further studies will need to confirm this. This study confirms that cardiovascular and symptoms are prevalent and have a dramatic impact on quality of life in pediatric and young adult patients diagnosed with hypermobile Ehlers-Danlos syndrome.

Investigation of Bax and Phospho-Tau Protein Expression in Preeclampsia Placenta

Background: Preeclampsia is a pregnancy complication with a high morbidity and mortality rate.BAX is a proapoptotic protein and in recent years, 14-3-3 tau proteins are considered as key regulators of many processes, such as apoptosis. In this study, we investigated the expression of phospho-tau and Bax in preeclampsia apoptosis immunohistochemically. Materials and Methods: 25 healthy and 25 preeclamptic pregnant placentas were included in the study. Placental samples were fixed with 10% neutral buffered formalin. Routine paraffin wax tissue protocol was used. Results: According to histological micrograf, the trophoblastic cells in the villi were normal in the control group. Mild dilatation in blood vessels was seen. Hyalinized and necrotic areas in chorionic villi and an increase in fibrinoid tissue in root villi were detected in preeclampsia sections. Phospho-tau and Bax primary antibodys were used for immunohistochemical evaluation. The sections from of the preeclampsia group were highly positive for syncytiotrophoblasts and villous connective tissue. It was also statistically different from the control group (p&amp;lt;0.05). Conclusions: When we evaluate the results shows that Phospho-tau and Bax may be determinant proteins in the apoptosis pathway of preeclampsia.

Carotid-subclavian bypass and endovascular aortic repair of Kommerell’s diverticulum with aberrant left subclavian artery: A case report

BACKGROUND Kommerell’s diverticulum (KD) with aberrant left subclavian artery is a rare congenital deformity and also has very little research literature about it (35% of case study). There are three types of aortic arch diverticulum. Even literature concerning the treatment options are limited. CASE SUMMARY We present a case report of a 50-year-old male with KD in the right aortic arch with aberrant left subclavian artery. We conducted a total endovascular repair procedure, which is innovative and will spread more light in the medical world. Our patient has no past medical history and is a non-smoker and non-alcoholic. Patient presented with shortness of breath, chest pain and dizziness for six months. Blood tests were done and computerized tomography (CT) angiogram of the chest confirmed the diagnosis, illustrating showed a 3.9 cm KD. On Day 1, the CT angiogram showed mild dilatation of the thoracic aorta, adjacent esophagus, trachea was compressed and displaced. Surgery was planned as the treatment modality. Carotid-Subclavian artery bypass and endovascular aortic repair was conducted. We used prolene 5-0 C1 sutures to precisely anastomose a 6-mm Dacron graft to the left subclavian artery. Haemostasis was secured and wounds were closed. Protamine was administered and patient was shifted to intensive care unit. Post-operative, patient responded favorably and was discharged. Regular follow-up is done. CONCLUSION The procedure we performed is novel. This will help the cardio-thoracic surgeons a better insight about the full procedures we conducted, thereby bringing more light and better treatment options in managing KD with aberrant subclavian artery.

Genetic and phenotypic characterization of Nexilin (NEXN) related cardiomyopathy

Abstract Background Clinical and genetic features of Nexilin (NEXN) related cardiomyopathies (CMPs) are largely uncharacterized. At present, the strength of the gene-disease association for NEXN is currently moderate for Dilated Cardiomyopathy (DCM), limited for Hypertrophic Cardiomyopathy (HCM), and unevaluated for Arrhythmogenic Cardiomyopathy (ACM). More evidence is needed to establish a definite genotype-phenotype association. Purpose We sought to investigate 1) the causative role of NEXN gene in CMPs, and 2) its phenotypic expression and prognostic profile. Methods All patients carrying NEXN variants, recruited in 11 international referral centres, were classified according to reported phenotype and genetic variant. Variants were considered rare if below GnomAD Popmax Filtering Allele Frequency of 8x10-5. Burden enrichment testing of rare NEXN variants was performed in our case-cohort of patients with DCM/ACM (n=3053), in comparison with the GnomAD population of non-Finnish Europeans (NFE). Clinical phenotypes and outcomes of patients carrying validated variants were described. A previously established cohort of patients with Titin-related CMP was used for prognostic comparison. Results Data from 87 patients were collected. Forty patients were excluded due to being carriers of non-rare NEXN variants (n=22) and/or pathogenic variants in other genes (n=18). Of the remaining patients, only 2 had HCM diagnosis, precluding any further analysis on this phenotype. NEXN truncating variants (tv) resulted significantly enriched in DCM/ACM disease-cohort with a prevalence of 0.39% vs 0.09% in GnomAD NFE (p=0.0001), whereas non-truncating ones were not. Patients carrying NEXNtv (n=16), plus a further patient carrying a homozygous p.G650del variant were thus considered affected by NEXN-related CMP (NEXN-CMP). Out of 17 patients with NEXN-CMP, 82% were probands, 53% were males and median age at diagnosis was 46 (IQR 35-55). At baseline, the most prevalent phenotypes were DCM (53%) and ACM (41%) and NYHA I class was common (71%). Left ventricular (LV) indexed End Diastolic Volume was mildly increased (75mL, IQR 43-87) and ejection fraction mildly reduced (LVEF 41%±15). Myocardial fibrosis was reported for up to 67% of patients, median ventricular ectopic beats were 515/24h (IQR 43-5071). During a median follow of 42 months, 8 patients were implanted with ICD, 1 patient died and 3 (21%) had malignant ventricular arrythmias (MVA). When compared to a cohort of patients with TTN-CMP, patients with NEXN-CMP showed MVA earlier, and with higher LVEF. Conclusions NEXNtv showed significant disease association with DCM/ACM phenotypes. NEXN-CMP seems to be characterized by mild LV dilation and dysfunction, uncommon heart failure, but frequent myocardial fibrosis and ventricular arrythmias. These findings from the largest cohort of NEXN variants carriers strongly contribute to defining the causative role of this rare genotype and its related phenotype.

Shared and distinguishing phenotypic characteristics of filamin c and desmoplakin cardiomyopathy

Abstract Background Rare truncating variants in the genes Filamin C (FLNC) and Desmoplakin (DSP) are increasingly recognised causes of Dilated Cardiomyopathy and Arrhythmogenic Cardiomyopathy (1,2). A direct comparison of both genotypes is needed to aid in subclassification and genotype-specific risk modelling. Purpose We sought to identify shared and distinguishing clinical characteristics and imaging phenotypes between FLNC and DSP cardiomyopathy. Methods 41 patients with ‘pathogenic’ or ‘likely pathogenic’ FLNC or DSP variants by American College of Medical Genetics (ACMG) criteria were identified from a tertiary centre cardiovascular genetics laboratory. Clinical, genetic and imaging data were compiled. Continuous variables are presented as median [IQR] or mean±SD and analysed by nonparametric Mann Whitney U or t-test. Categorical variables were analysed by Chi-Squared or Fisher’s exact test. Results FLNC and DSP cohorts included a similar proportion of patients with truncating variants (91% vs 100%) and female sex (12/21, 57% vs 13/20, 65%). FLNC patients were younger (median age 30 [13-51] vs 57 [41-65] years, p&amp;lt;0.0008) and more likely to present with sustained ventricular arrhythmia (5/21 24% vs 3/20, 15%) (Table 1). Acute myocarditis episodes were exclusively linked to DSP cardiomyopathy (4/20, 20%) with no association to FLNC (0/21, 0%, p&amp;lt;0.05). FLNC cardiomyopathy patients had normal left ventricular (LV) systolic function (LVEF 57±9% SD vs 46±13% DSP, p&amp;lt;0.03) and normal left ventricular cavity side (mean indexed LV end diastolic volume (EDV) 85±12 vs 112±26ml/m2 DSP, p&amp;lt;0.02) (Table 2). Both FLNC and DSP cause exclusive left ventricular or biventricular late gadolinium enhancement (75% Vs 82%) on cardiac magnetic resonance imaging (MRI). Fibrosis was predominantly in a subepicardial distribution in both cohorts (66% FLNC vs 57% DSP). In FLNC, circumferential fibrosis was the most frequent pattern of distribution (5/9, 55%), whereas in DSP inferior and lateral wall fibrosis was the predominant feature (8/14, 57%). There were no cases of isolated RV involvement and no cases fulfilled ‘definite’ ARVC taskforce diagnostic criteria. Over a follow up of median 45months, there was no significant difference in the proportion of FLNC and DSP patients experiencing a composite end point of LVAD implantation, heart transplant, aborted sudden cardiac death or mortality (7/21, 33% FLNC Vs 7/20, 29% DSP p&amp;gt;0.99). Conclusion Normal systolic function with marked circumferential fibrosis is the predominant feature of FLNC cardiomyopathy whereas mild LV dilatation, mild systolic impairment and inferolateral fibrosis are more frequent in DSP cardiomyopathy. Myocarditis episodes are a distinguishing feature of DSP cardiomyopathy, suggesting a different underlying molecular mechanism driven by inflammation. The overall clinical trajectory of FLNC and DSP cardiomyopathy patients are comparable.

Abstract 18941: Pediatric Electrocardiogram-Based Deep Learning to Predict Left Ventricular Dysfunction and Remodeling

Background: Artificial intelligence-enhanced ECG algorithms show promise to detect a range of adult phenotypes, inclusive of ventricular dysfunction and remodeling. However, it remains unclear whether similar approaches provide meaningful and generalizable predictions in children. Methods: We trained a convolutional neural network on paired ECG-echos (≤ 2 days apart) from patients ≤ 18 years old without major congenital heart disease to detect human expert classified greater than mild left ventricular (LV) dysfunction, hypertrophy, and dilation (individually and as a composite outcome). Model performance was assessed by calculating area under the receiver operating curve (AUROC) in an internal test set and external setting (emergency department). Results: The main cohort comprised of 119,787 ECG-echo pairs (61,722 patients; median age 8.3 [IQR, 2.9-13.8] years; 55% male). Training, internal test, and external test sets comprised of 92,377, 24,343, and 3,067 ECG-echo pairs with 8.2%, 7.7%, and 10.0% composite outcome rates, respectively. During internal testing, AUROCs of 0.84 [95% CI, 0.83-0.85], 0.87 [95% CI, 0.85-0.88], 0.86 [95% CI, 0.84-0.87], and 0.84 [95% CI, 0.83-0.86] were achieved for the LV composite outcome, dysfunction, hypertrophy, and dilation, respectively (Figure). During external testing, AUROCs of 0.80 [95% CI, 0.77-0.83], 0.84 [95% CI, 0.80-0.88], 0.77 [95% CI, 0.72-0.82], and 0.86 [95% CI, 0.82-0.89] were achieved, respectively (Figure). Similar model performance was achieved when using quantitative z-score cutoffs for each outcome. Exploratory saliency mapping provides insight into notable ECG components that influence model predictions. Conclusions: Our artificial intelligence-enhanced pediatric ECG algorithm shows promise to inexpensively screen for and diagnose LV dysfunction or remodeling in children. Prospective trials to guide model implementation for supporting clinical decision making are warranted.

Abstract 19052: Interferon B1A-Induced Dilated Cardiomyopathy

Introduction: Congestive heart failure is a leading causes of morbidity and mortality. Interferon therapy is a rare cause of dilated cardiomyopathy (DCM) and Heart Failure with Reduced Ejection Fraction (HFrEF). Specifically, interferon Alpha has been associated with cardiotoxicity. Here we describe a case of interferon B1A - induced HFrEF and DCM in a patient who took subcutaneous Interferon B1A for 11 years before developing clinical signs of heart failure. To our knowledge, only four other cases have been reported. Case Description: A 70-year-old man with a history of multiple sclerosis managed with Interferon B1A presented with shortness of breath, orthopnea, and lower extremity edema for 10 days. Physical examination revealed jugular venous distension and pitting edema. Brain natriuretic peptide (BNP) was 1580 pg/mL, troponin was 49 ng/L. Lipid panel, complete blood count, complete metabolic panel, Hemoglobin A1c, and thyroid stimulating hormone were within normal limits. Urine drug screen was negative. Chest X-ray revealed pulmonary vascular congestion and small pleural effusions bilaterally. A Transthoracic echocardiogram revealed mild aortic root dilatation (4.1cm), significant four-chamber dilatation, severely reduced left ventricular (LV) systolic function with an estimated ejection fraction of 20%, diastolic LV dysfunction, and moderately reduced systolic RV function, consistent with HFrEF and DCM (LVIDD 6.1cm). Coronary angiography showed normal coronary anatomy. The patient was started on guideline-directed medical therapy for HFrEF. Close follow-up was arranged to track improvement in systolic function after cessation of interferon b1a and consideration for Cardiac MRI and possible RV biopsy to evaluate for other causes of cardiomyopathy. Discussion/Conclusions: Due to the patient’s negative ischemic workup and absence of risk factors for cardiovascular disease, we strongly suspect that the patient’s cardiomyopathy was due to interferon B1A. With only 4 other cases of interferon B1A-induced DCM reported, it is a rare cause of medication-induced DCM. Interferon B1A should be considered a potential risk factor for developing heart failure, especially in high-risk patients.

Acute psychosis as the presenting manifestation of lupus: case reports

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disorder that affects multiple organs with a wide range of clinical manifestations. Neuropsychiatric manifestation (NPSLE) is present among 28–40% of the SLE patients. Psychosis is rarely the only initial symptom of lupus, as neuropsychiatric lupus is often associated with systemic activity. We present two cases of young ladies presenting with acute psychosis as the predominant symptom and further diagnosed with SLE disease. Case reports: Case 1 A 20-year-old female, unmarried, presented to the Psychology Department with acute behavioral abnormality associated with staring, insomnia and, aggressive behavior, hearing voices for the last 3 days. No other psychiatric or neurological symptoms; she was taking 20 mg oral prednisolone prescribed according to a history of fatigue, low-grade fever, loss of appetite, pain in almost all joints, and myalgia for the last 1 month. There was no sign of focal neurological deficit or meningeal irritation. Examination of other systems did not find any clinical abnormality except for the presence of photosensitivity. A psychiatric consultation was ordered. Laboratory tests: white blood cells 3200 cell/mm3 (range=4000–11 000) with normal differentiation, hemoglobin 9.2 mg/dl (range=12.5–16), platelet 90 000/dl (range=150 000–400 000), C-reactive protein (CRP) 65 mg/l (n≤6), and erythrocyte sedimentation rate (ESR) 100 mm/h(range=0–20). anti-nuclear antibody (ANA) profile which was positive in 1:320 dilution, homogenous type, along with anti-ds-DNA positive (+), decreased C3 and C4. The diagnosis of SLE was done according to the European League Against Rheumatism/American College of Rheumatology Classification Criteria (EULAR/ACR Criteria). Case2 A 37-year-old married female with 3 children presented to the Psychology Department with disorientation, staring, hearing voices, seeing images of people who want to hurt her, refusing to respond to people, and insomnia for the last 10 days. She had complained of fatigue and low-grade fever for last month. A psychiatric consultation was ordered, and the patient was managed with an injection of haloperidol 5 mg BD. Laboratory tests: white blood cells 7300 cells/mm3 (range=4000–11 000) with normal differentiation, hemoglobin 8.7 mg/dl (range=12.5–16), platelet 90 000/dl (range=150 000–400 000), reticulocyte:1.4, CRP 7 mg/l (n≤6), and ESR 50mm/h (range=0–20). The ANA profile was positive in 1:320 dilution, homogenous type, along with the anti-ds-DNA negative (−), decreased C3, and normal C4. Brain magnetic resonance imaging (MRI) showed a high white matter signal around the lateral ventricles and in the semi-oval centers. Mild dilation of the lateral ventricles and slight elevation of the left insular and medial temporal lobe signal. Conclusion: Our case reports showed that organic disorders should be considered while evaluating acute psychosis, especially autoimmune disorders. The presence of laboratory features like anemia, elevated ESR, and elevated CRP should be taken with utmost importance.

Effects of chronic administration of some bouillon cubes on oxidative stress and liver function markers in female albino rats

Aim: This study evaluated the effects of chronic administration of some bouillon cubes on oxidative stress and liver function markers in female albino rats. Methodology: A total of thirty-five (35) female albino rats, weighing between 120 and 150 grams, were used for the study. The bouillon cubes, Star Maggi and Knorr were administered daily to the rats, using an oral gavage tube for 90days. Superoxide Dismutase (SOD) and Glutathione Peroxidase (GPx) level were determined using a rat-specific sandwich-enzyme linked immunosorbent assay (ELISA) method. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were estimated quantitatively using the Reitman and Frankel Method. Total protein (TP) was estimated quantitatively using the Biuret colorimetric method. Albumin (ALB) was estimated quantitatively following the Modified bromocresol green colorimetric method. Liver sections were stained using haematoxylin and eosin (H&amp;E) staining technique. Quantitative analysis of monosodium glutamate (MSG) content of the bouillon cubes was analyzed using ultraviolet (UV) spectroscopy while the sodium content was analyzed using atomic absorption spectrophotometry according to the method of the American Public Health Association. Results: There were no significant differences (p&gt;.05) in SOD and GPx levels in all the treatment groups, compared to the negative control. Group D (High Dose Maggi) had lower SOD and GPx levels than the negative control, though not significant. ALT level (5.01 ± 0.53 iu/L) in group E (High Dose Knorr) was significantly higher (p&lt;.05) when compared to the negative control. There were no significant differences (p&gt;.05) in AST levels in all groups, even though group E had the highest AST level. There were no significant differences (p&gt;.05) in TP and ALB levels, except for group E (High Dose Knorr), which was significantly lower (p&lt;.05), compared to the negative control. Liver sections of the negative control showed normal histoarchitecture. The treated groups showed histological changes in the architecture of the hepatocytes indicating moderate dilation of the central vein, moderate disruption of the hepatic lobules, and mild sinusoidal dilation, with associated inflammatory cell infiltration. Conclusion: Chronic administration of bouillon cubes did not have significant impact on the levels of the oxidative stress markers, superoxide dismutase (SOD) and glutathione peroxidase (GPx) in the rats. Chronic administration of Knorr cubes impacted the liver, with elevated alanine aminotransferase (ALT) levels, with a reduced total protein and albumin level. There were changes in the architecture of the hepatocytes of the treated rats, indicating moderate dilation of the central vein, moderate disruption of the hepatic lobules, and mild sinusoidal dilation. Bouillon cubes should be used in moderation, taking into account their potential effects on overall health and wellbeing when consumed for long periods.

Hypertension: An Important But Reversible Cause of Systolic Dysfunction in a Cohort of Pediatric Patients.

Cardiac dysfunction due to hypertension (CDHTN) in pediatrics is not well described. We aimed to describe the presentation and outcomes of pediatric CDHTN and identify clinical features associated with resolution of dysfunction. A single-center retrospective cohort study of patients ≤ 21years with CDHTN from January 2005-September 2020 was performed. Patients with systolic dysfunction without another cause, blood pressure > 95th percentile, and physician judgment that dysfunction was secondary to hypertension were included. Demographics, clinical characteristics, echocardiographic findings, and outcomes were examined using Fisher's exact and Mann-Whitney U tests. Multiple correspondence analysis was used to explore the relationship of resolution of dysfunction to clinical features. Thirty-four patients were analyzed at a median age of 10.9 (IQR 0.3-16.9) years. Patients were divided into groups < 1year (n = 12) and ≥ 1year (n = 22). Causes of hypertension were varied by age, with renovascular disease most common in infants (42%) and medical renal disease most common in older patients (77%). Echocardiography demonstrated mild LV dilation (median LV end-diastolic z-score 2.6) and mild LV hypertrophy (median LV mass z-score 2.4). Most patients (81%) had resolution of dysfunction, particularly infants (92%). One patient died and one patient was listed for heart transplant. None required mechanical circulatory support (MCS). No clinical features were statistically associated with resolution of dysfunction. Hypertension is an important but reversible cause of systolic dysfunction in children. Patients are likely to recover with low mortality and low utilization of MCS or transplantation. Further studies are needed to confirm features associated with resolution of dysfunction.

Internal circular suture annuloplasty for aortic valve repair.

Stabilizing the aorto-ventricular junction is integral in aortic valve repair. We report our technique of internal circular suture annuloplasty. We used a continuous horizontal mattress suture of a single thick expanded polytetrafluoroethylene suture (CV-3). We put 4 stitches per sinus, so the suture was below the cusp attachment line at the nadirs and passed through the interleaflet triangle at the upper aorto-ventricular junction level. The suture was reinforced with pericardial pledgets on both sides of each commissure. We used this technique in 12 patients. The diameter of aorto-ventricular junction was reduced from 25 ± 2mm to 22 ± 1mm (n = 11) and was 22 ± 1mm at the latest follow-up (4-74months, median 41, n = 10). In 2 patients with large aorto-ventricular junction (27mm or more), expected annular reduction was not achieved. Our modified technique is simple and seems durable. It may be useful for mild annular dilatation.

Mini Percutaneous Nephrolithotomy in Ectopic Lumber Kidney: A Case Report

The chance of the development of hydronephrosis and the formation of stones is more in the ectopic kidney. Treatment of stones in ectopic kidneys is challenging for urologists due to abnormal location and rotation of the kidney and unfavourable local anatomy. We herein report a case of stone in a lumber kidney successfully managed by mini percutaneous nephrolithotomy (PCNL) in a prone position. A 55-year-old male presented with right lower abdominal pain. His CT scan of the KUB reveals the right kidney is in an ectopic location and has a hyperdense structure measuring about 15 mm in length, seen in the right renal pelvis with mild dilatation of the pelvicalyceal system. The patient underwent right-sided prone mini PCNL under the subarachnoid block (SAB), and complete stone clearance was achieved. There was no adverse event during or after surgery or post-operative blood transfusion. Bangladesh J. Urol. 2022; 25(2): 122-125

FRI244 Autoimmune Polyglandular Syndrome Type 2 And Peripartum Cardiomyopathy: A Case Report

Abstract Disclosure: A.G. Jimenez: None. J.D. Christensen: None. P. Surampudi: None. S. Phatak: None. J. Yoon: None. Background: Peripartum cardiomyopathy (PPCM) is a diagnosis of exclusion with evolving understanding of pathophysiology. There is limited consideration of the autoimmune processes contributing to PPCM. Case Presentation: A 20-year-old female had a recent delivery complicated by placenta previa, hemorrhage requiring transfusion, and pelvic infection. She presented 2 weeks postpartum with altered mental status and abdominal pain, found to be in septic shock, acute respiratory failure, acute liver injury initially thought to be secondary to retained products of conception. Subsequently, she was diagnosed with moderate-to-severe PPCM as she developed signs of heart failure, low ejection fraction &amp;lt;35%, mild ventricular dilatation, cardiogenic shock, hemorrhagic pericardial effusion with tamponade. She had no other prior cardiovascular history. She was briefly started on bromocriptine for treatment of suspected prolactin-mediated PPCM, in addition to digoxin, as her blood pressure was unable to sustain other treatments for PPCM. However, she hemodynamically decompensated and required persistent pressor support, leading to evaluation for other causes. Due to her prior history of hypothyroidism, an adrenal evaluation with a cosyntropin stimulation test was done. It demonstrated an undetectable cortisol at 0, 30, 60 minutes, with an elevated ACTH (1256, n=7-63 ng/dL), consistent with primary adrenal insufficiency (PAI). Workup for PAI did not reveal any infectious, infiltrative, vascular, pharmacological, or neoplastic etiology. Lab workup for concomitant hypophysitis was significant for low IGF-1 (Z-score -5.8, n= -2.0 to +2.0), but otherwise had expected elevated prolactin (121 ng/mL, n=5.2-26.5 ng/mL), suppressed LH (0.3 mIU/mL) and FSH (2.4 mIU/mL), and normal TSH (3.87 mIU/mL, n=0.27-4.20 mIU/mL) on levothyroxine 100 mcg. She had normal parathyroid hormone and hemoglobin A1c levels with no history of chronic candidiasis. Further workup showed positive antibodies to adrenal 21-hydroxylase enzyme, thyroid peroxidase, and glutamic acid decarboxylase 65, suggestive of autoimmune polyglandular syndrome (APS) type 2. She was started on glucocorticoids and mineralocorticoids for adrenal insufficiency resulting in a significant clinical improvement. She was subsequently discharged in stable condition. Conclusion: Due to increased rates of mortality in PPCM (4-11%), thought should be given to nontraditional etiologies of cardiac failure. Although rare, adrenal insufficiency must be considered in those with moderate-to-severe PPCM who continue to decompensate despite treatments. Untreated adrenal insufficiency can result in fatal consequences if not immediately treated. APS type 2 should be considered even in those without any confirmed family history of autoimmune disease. Those with autoimmune tendencies may be at higher risk of PPCM. Presentation: Friday, June 16, 2023

In-vivo anti-inflammatory effects of Roman Chamomile (Chamaemelum nobile) aqueous extracts collected from the National Park of El-Kala (North-East, Algeria).

The aim of our study is to evaluate anti-inflammatory effect of Chamaemelum nobile. Aqueous extracts were administrated to Wistar rats in bronchial-inflammation experimentally induced by an allergen and ovalbumin, administered intraperitoneally / intranasally (20mg/kg/day). Experimentation showed disturbances in bronchoalveolar fluid with increased leukocyte and lymphocyte levels as well as IL-4 concentration in the lungs and erythrocytes associated with high lipid peroxidation. There were disturbances in enzymatic and non-enzymatic antioxidant defense system. Lungs histopathological showed an inflammatory lymphoplasmacytic infiltrate, moderate edema of alveoli, vascular congestion and suffusion hemorrhage. Administration of aqueous extract to OVA-sensitized rats caused a significant and very highly significant improvement of MDA levels in lungs, erythrocytes, GSH, GPx, GST, catalase and SOD. We notice a decrease in IL-4 in LBA and lungs alongside reduced inflammatory cell infiltration, mild bronchiolar dilation, mild alveolar edema and normal cell morphology allowing us to conclude on the effectiveness of anti-inflammatory activity of Roman chamomile.

Evaluating Variation in the Cardiac Management of Children with Hereditary Thoracic Aortic Disease in the United States.

Hereditary thoracic aortic diseases (HTAD) such as Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS), and vascular Ehlers-Danlos syndrome (VEDS) frequently result in complex cardiovascular pathology that can lead to premature death. However, given limited research and lack of detailed pediatric management guidelines, practice in the U.S. is largely guided by personal experience and/or advice from other professionals. A REDCap survey was composed that covered topics including genetic testing, imaging, and medication choice (all in children), among others. After piloting, the survey was distributed via email and advertised on PediHeartNet. Email addresses of providers were obtained through an established aortic research collaborative and a clinic directory offered through The Marfan Foundation. There were 64 survey responses (pediatric cardiologists 66%; geneticists 13%, genetic counselors 6%; the remaining 15% was comprised of a combination of cardiothoracic surgeons, adult cardiologists, adult congenital specialists, combined cardiology and genetics specialist, nurse practitioners, physician assistants, and nurse coordinators). The most supported indication for genetic evaluation in a child with mild aortic root dilation was family history of thoracic aortic dissection (100%), in contrast to mild root dilation with no other HTAD features (39% supported, 45% did not, 15% saying it would depend on other factors). The majority would start medical therapy in MFS at an aortic root z-score of 2, however differences existed regarding medication preferences for initiation (47% angiotensin receptor blockers, 36% beta blockers, 17% would not or cannot prescribe medication/defer medication choice to another provider). Variation existed for cross-sectional imaging indications and modality and for exercise restrictions, although on average respondents were more lenient than the Bethesda guidelines. While there are areas of general agreement in the cardiac management of children with HTAD, there are also several areas of considerable variation. This highlights the need for additional study in these areas with the ultimate goal of creating consensus guidelines.

Normothermic Ex Vivo Liver Machine Perfusion in Mouse.

This protocol presents an optimized erythrocytes-free NEVLP system using mouse livers. Ex vivo preservation of mouse livers was achieved by employing modified cannulas and techniques adapted from conventional commercial ex vivo perfusion equipment. The system was utilized to evaluate the preservation outcomes following 12 h of perfusion. C57BL/6J mice served as liver donors, and the livers were explanted by cannulating the portal vein (PV) and bile duct (BD), and subsequently flushing the organ with warm (37 °C) heparinized saline. Then, the explanted livers were transferred to the perfusion chamber and subjected to normothermic oxygenated machine perfusion (NEVLP). Inlet and outlet perfusate samples were collected at 3 h intervals for perfusate analysis. Upon completion of the perfusion, liver samples were obtained for histological analysis, with morphological integrity assessed using modified Suzuki-Score through Hematoxylin-Eosin (HE) staining. The optimization experiments yielded the following findings: (1) mice weighing over 30 g were deemed more suitable for the experiment due to the larger size of their bile duct (BD). (2) a 2 Fr (outer diameter = 0.66 mm) polyurethane cannula was better suited for cannulating the portal vein (PV) when compared to a polypropylene cannula. This was attributed to the polyurethane material's enhanced grip, resulting in reduced catheter slippage during the transfer from the body to the organ chamber. (3) for cannulation of the bile duct (BD), a 1 Fr (outer diameter = 0.33 mm) polyurethane cannula was found to be more effective compared to the polypropylene UT - 03 (outer diameter = 0.30 mm) cannula. With this optimized protocol, mouse livers were successfully preserved for a duration of 12 h without significant impact on the histological structure. Hematoxylin-Eosin (HE) staining revealed a well-preserved morphological architecture of the liver, characterized by predominantly viable hepatocytes with clearly visible nuclei and mild dilation of hepatic sinusoids.