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Pediatric Nephrology (Berlin, Germany) Oct 2023This review provides updated knowledge on the long-term outcomes among children with antenatally diagnosed urinary tract dilatation (UTD), previously often referred to... (Review)
Review
This review provides updated knowledge on the long-term outcomes among children with antenatally diagnosed urinary tract dilatation (UTD), previously often referred to as antenatal hydronephrosis. Different definitions of UTD exist, which makes comparison between studies and generalized conclusions difficult. Roughly, one-third of antenatally diagnosed UTD, defined as a renal pelvis anterior posterior diameter (APD) of ≥ 4 mm in the second trimester and/or ≥ 7 mm in the third trimester, will resolve before birth, another third will resolve within the first years of life, and in the remaining cases, UTD will persist or a congenital abnormality (CAKUT) will be diagnosed postnatally. The risk of a postnatal CAKUT diagnosis increases with the degree of prenatal and postnatal dilatation, except for vesicoureteral reflux (VUR), which cannot be predicted from the degree of UTD. Urinary tract infections (UTIs) occur in 7-14% of children with UTD during the first years of life. The risk of UTI is higher in children with traditional risk factors for UTI, such as dilated VUR, hydroureteronephrosis, female gender, and intact foreskin. Continuous antibiotic prophylaxis may be considered in selected patients during the first years of life. In long-term follow-ups, permanent kidney damage is diagnosed in approximately 40% of children with moderate or severe UTD, but hypertension, proteinuria, and/or reduced eGFR are uncommon (0-5%). In children with mild UTD, the long-term outcome is excellent, and these children should not be subjected to unnecessary examinations and/or follow-up.
Topics: Child; Humans; Female; Pregnancy; Dilatation; Vesico-Ureteral Reflux; Hydronephrosis; Urinary Tract Infections; Dilatation, Pathologic; Kidney Pelvis; Urinary Tract
PubMed: 36920569
DOI: 10.1007/s00467-023-05907-z -
Cureus Aug 2023Alcoholic cardiomyopathy (ACM) is a cardiac ailment marked by impaired contraction and dilation of one or both ventricles of the heart. The extent of daily alcohol... (Review)
Review
Alcoholic cardiomyopathy (ACM) is a cardiac ailment marked by impaired contraction and dilation of one or both ventricles of the heart. The extent of daily alcohol intake and duration of alcohol abuse are linked to the development of ACM, although the exact thresholds and timeline for alcohol misuse to induce heart dysfunction remain uncertain. Thus, the objective of this systematic review is to comprehensively evaluate the existing knowledge on the specific disease entity, particularly in light of the ongoing issue of alcohol misuse, with the intention of determining if recent advancements and discoveries have significantly altered the understanding of this condition compared to the past century. This systematic review involved a literature search that was conducted on PubMed to identify suitable and appropriate literature for the study. The inclusion criteria encompassed articles that focused on ACM or the relationship between alcohol abuse and cardiac dysfunction, involved human subjects or relevant animal models, were written in the English language, and were published within the last 10 years. The exclusion criteria included duplicates, case reports, letters, editorials, and reviews not specifically addressing ACM. As a result, a total of 18 articles were included in this systematic review. The risk of bias was assessed through the use of the Cochrane risk-of-bias tool for clinical trials. The findings of this systematic review indicated that the likelihood of ACM occurrence significantly rose when the consumption of over 80 g of alcohol per day occurred for at least five years. The systematic review further revealed that ACM is associated with various detrimental changes in the cellular, structural, and histological aspects of the heart muscles, even though the specific clinical and histological characteristics of ACM have yet to be established. In individuals with an extensive history of excessive alcohol abuse, the diagnosis of ACM was reached through the exclusion of other potential causes of the condition. The fundamental approach to treatment lies in abstaining from alcohol. It is crucial to manage symptoms in individuals with secondary heart failure and address any related complications.
PubMed: 37664402
DOI: 10.7759/cureus.42886 -
Genome Medicine Sep 2023Dilated cardiomyopathy (DCM) is a life-threatening heart disease and a common cause of heart failure due to systolic dysfunction and subsequent left or biventricular...
BACKGROUND
Dilated cardiomyopathy (DCM) is a life-threatening heart disease and a common cause of heart failure due to systolic dysfunction and subsequent left or biventricular dilatation. A significant number of cases have a genetic etiology; however, as a complex disease, the exact genetic risk factors are largely unknown, and many patients remain without a molecular diagnosis.
METHODS
We performed GWAS followed by whole-genome, transcriptome, and immunohistochemical analyses in a spontaneously occurring canine model of DCM. Canine gene discovery was followed up in three human DCM cohorts.
RESULTS
Our results revealed two independent additive loci associated with the typical DCM phenotype comprising left ventricular systolic dysfunction and dilatation. We highlight two novel candidate genes, RNF207 and PRKAA2, known for their involvement in cardiac action potentials, energy homeostasis, and morphology. We further illustrate the distinct genetic etiologies underlying the typical DCM phenotype and ventricular premature contractions. Finally, we followed up on the canine discoveries in human DCM patients and discovered candidate variants in our two novel genes.
CONCLUSIONS
Collectively, our study yields insight into the molecular pathophysiology of DCM and provides a large animal model for preclinical studies.
Topics: Humans; Animals; Dogs; Cardiomyopathy, Dilated; Homeostasis; Models, Animal; Phenotype; Risk Factors
PubMed: 37723491
DOI: 10.1186/s13073-023-01221-3 -
Korean Journal of Radiology Dec 2023Dilated cardiomyopathy (DCM) is one of the most common types of non-ischemic cardiomyopathy. DCM is characterized by left ventricle (LV) dilatation and systolic... (Review)
Review
Dilated cardiomyopathy (DCM) is one of the most common types of non-ischemic cardiomyopathy. DCM is characterized by left ventricle (LV) dilatation and systolic dysfunction without coronary artery disease or abnormal loading conditions. DCM is not a single disease entity and has a complex historical background of revisions and updates to its definition because of its diverse etiology and clinical manifestations. In cases of LV dilatation and dysfunction, conditions with phenotypic overlap should be excluded before establishing a DCM diagnosis. The differential diagnoses of DCM include ischemic cardiomyopathy, valvular heart disease, burned-out hypertrophic cardiomyopathy, arrhythmogenic cardiomyopathy, and non-compaction. Cardiac magnetic resonance (CMR) imaging is helpful for evaluating DCM because it provides precise measurements of cardiac size, function, mass, and tissue characterization. Comprehensive analyses using various sequences, including cine imaging, late gadolinium enhancement imaging, and T1 and T2 mapping, may help establish differential diagnoses, etiological work-up, disease stratification, prognostic determination, and follow-up procedures in patients with DCM phenotypes. This article aimed to review the utilities and limitations of CMR in the diagnosis and assessment of DCM.
Topics: Humans; Cardiomyopathy, Dilated; Contrast Media; Magnetic Resonance Imaging, Cine; Gadolinium; Magnetic Resonance Imaging; Myocardial Ischemia; Predictive Value of Tests; Ventricular Function, Left
PubMed: 38016680
DOI: 10.3348/kjr.2023.0531 -
Medicina (Kaunas, Lithuania) Jul 2023Dilated cardiomyopathy (DCM) is a rare and severe condition characterized by chamber dilation and impaired contraction of the left ventricle. It constitutes a... (Review)
Review
Dilated cardiomyopathy (DCM) is a rare and severe condition characterized by chamber dilation and impaired contraction of the left ventricle. It constitutes a fundamental etiology for profound heart failure and abrupt cardiac demise, rendering it a prominent clinical indication for heart transplantation (HTx) among both adult and pediatric populations. DCM arises from various etiologies, including genetic variants, epigenetic disorders, infectious insults, autoimmune diseases, and cardiac conduction abnormalities. The maintenance of cardiac function involves two distinct types of immune cells: resident immune cells and recruited immune cells. Resident immune cells play a crucial role in establishing a harmonious microenvironment within the cardiac tissue. Nevertheless, in response to injury, cardiomyocytes initiate a cytokine cascade that attracts peripheral immune cells, thus perturbing this intricate equilibrium and actively participating in the initiation and pathological remodeling of dilated cardiomyopathy (DCM), particularly during the progression of myocardial fibrosis. Additionally, immune cells assume a pivotal role in orchestrating the inflammatory processes, which are intimately linked to the prognosis of DCM. Consequently, understanding the molecular role of various immune cells and their regulation mechanisms would provide an emerging era for managing DCM. In this review, we provide a summary of the most recent advancements in our understanding of the molecular mechanisms of immune cells in DCM. Additionally, we evaluate the effectiveness and limitations of immunotherapy approaches for the treatment of DCM, with the aim of optimizing future immunotherapeutic strategies for this condition.
Topics: Adult; Child; Humans; Cardiomyopathy, Dilated; Cytokines; Myocytes, Cardiac; Prognosis; Heart Failure
PubMed: 37512058
DOI: 10.3390/medicina59071246 -
JPMA. the Journal of the Pakistan... Oct 2023Urethral stricture disease is relatively common in Pakistan, constituting 4-5% of the urological workload. Despite the high prevalence, little is known about its... (Review)
Review
Urethral stricture disease is relatively common in Pakistan, constituting 4-5% of the urological workload. Despite the high prevalence, little is known about its epidemiology in the country. The current narrative review comprised search on PubMed, Pak MediNet and Google Scholar databases for studies done in Pakistan and published between January 1, 2000, and December 31, 2021. The search yielded 30 local publications on stricture urethra. Demographic data as well as causes and management pattern of male urethral stricture were noted and analysed. There were 5,021 patients, with 3850 (76.6%) being from the province of Sindh. The disease had the greatest impact on younger patients aged up to 40 years (n=1572), while after the age of 60 years, 248 (9%) patients had the disease. The common cause was trauma due to road traffic accidents in both anterior and posterior strictures compared to idiopathic cause reported in the West. Infection 170 (6.9%) and Lichen sclerosis 123(4.5%) as a cause was found to decline in our region. A clinic-based regular urethral dilatation was still in practice at some centres to manage such cases. Vast majority of stricture patients were being treated by endoscopic procedures, and only 1154 (23%) cases underwent urethroplasty.
Topics: Aged; Humans; Male; Middle Aged; Constriction, Pathologic; Dilatation; Pakistan; Retrospective Studies; Treatment Outcome; Urethra; Urethral Stricture; Urologic Surgical Procedures, Male; Adult
PubMed: 37876069
DOI: 10.47391/JPMA.7925 -
Clinical and Experimental Hypertension... Dec 2023This study aimed to explore whether circadian rhythm of blood pressure is associated with brachial-ankle pulse wave velocity (baPWV) and brachial artery flow-mediated...
OBJECTIVE
This study aimed to explore whether circadian rhythm of blood pressure is associated with brachial-ankle pulse wave velocity (baPWV) and brachial artery flow-mediated dilation (FMD) in patients with essential hypertension.
METHOD
This cross-sectional study included 4,217 patients with essential hypertension who completed 24-hour ambulatory blood pressure monitoring, baPWV, and FMD. BaPWV and FMD were measured to evaluate arterial stiffness and endothelial dysfunction. Participants were divided into dipper, non-dipper, and reverse dipping groups according to the nocturnal systolic blood pressure dipping percentage.
RESULTS
In this study, baPWV was highest in the reverse dipping groups, followed by non-dipper and dipper groups (1667.11 ± 327.90 vs. 1613.88 ± 325.11 vs. 1577.45 ± 306.15 cm/s, < .001) and FMD gradually increased (4.41 ± 2.87 vs. 4.70 ± 2.84 vs. 4.92 ± 2.79%, = .001). baPWV and FMD were significantly associated with declining nocturnal systolic blood pressure (SBP). Interestingly, FMD (β = 0.042, = .014) was only positively associated with a drop in nocturnal SBP decline in patients <65 years of age. Whereas baPWV was consistently negatively associated with nocturnal SBP decline regardless of age (β = -0.065, < .001, age <65 years; β = -0.149, = .002, age ≥ 65). Receiver operating characteristics (ROC) curves analysis showed areas under the curve (AUC) of baPWV/FMD for predicting circadian rhythm of blood pressure are 0.562/0.554 with a sensitivity of 51.7%/53.9% and specificity of 56.4%/53.4.
CONCLUSION
Impairment of baPWV and FMD were correlated with abnormal circadian rhythm of blood pressure in essential hypertension, suggesting a decrease in nighttime SBP may associate with endothelial function and arterial stiffness.
Topics: Humans; Aged; Blood Pressure; Hypertension; Ankle Brachial Index; Blood Pressure Monitoring, Ambulatory; Cross-Sectional Studies; Pulse Wave Analysis; Essential Hypertension; Circadian Rhythm; Dilatation, Pathologic; Vascular Stiffness
PubMed: 37358045
DOI: 10.1080/10641963.2023.2229535 -
Cureus Nov 2023Background Aortic root dilation is an increasingly recognized feature in repaired tetralogy of Fallot (TOF) patients. However, the dilation at the aortic root and...
Background Aortic root dilation is an increasingly recognized feature in repaired tetralogy of Fallot (TOF) patients. However, the dilation at the aortic root and ascending aorta in unrepaired TOF is rarely studied. This study aims to confirm whether aortic dilation is a common feature in unrepaired TOF and investigate the factors attributed to aortic dilation. Methodology Patients with an echocardiographic diagnosis of TOF undergoing computed tomography angiography were retrospectively studied. Diameters and z scores of aortic annular (Ao1), aortic sinotubular junction (Ao2), ascending aorta (Ao3), and distal transverse aortic arch (Ao4) were measured. Preoperative body surface area (BSA), hemoglobin (Hb), hematocrit (HCT), arterial oxygen saturation (SaO), and platelet (PLT) count were recorded. Results A total of 101 TOF patients aged 6.8 ± 9.5 years were included in this study, whose mean BSA (m), Hb (g/L), HCT, SaO (%), and PLT (10/L) were 0.7 ± 0.4, 162.1 ± 3.8, 0.5 ± 0.1, 85.1 ± 9.3, and 238.1 ± 101.1, respectively. The mean z score of Ao1, Ao2, Ao3, and Ao4 were 10.3 ± 3.5, 4.7 ± 2.9, 4.0 ± 2.7, and 4.1 ± 2.4, respectively. Age and BSA were positively correlated with the z scores of Ao1 and Ao2. Preoperative Hb and HCT were positively correlated with the z scores of Ao1, Ao2, Ao3, and Ao4. Preoperative SaO and PLT were negatively correlated with the z scores of Ao1, Ao2, Ao3, and Ao4. Conclusions Aortic dilation is common in unrepaired TOF patients. The dilation of different levels of the aorta was correlated with age, BSA, preoperative Hb, HCT, SaO, and PLT.
PubMed: 38143655
DOI: 10.7759/cureus.49212