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Introduction: Difficult coronary sinus (CS) anatomy may lead to difficulty in optimal left ventricular (LV) lead placement and lead displacements leading to nonresponse to cardiac resynchronization therapy (CRT). Methods: In this retrospective study, we studied the CRT parameters of devices implanted by single operator during the time period from January 2014 till December 2021, where different off-label techniques were used to place/stabilize LV lead. The technique used to stabilize LV lead, CRT parameters at baseline and follow up were noted for each patient. Results: Out of 133 CRTs implanted during the study period, 23 patients (17.29 %) required off-label techniques. Stylet and guidewire retaining techniques were used in 11/23 (47.82 %) and 7/23 (30.43 %) patients respectively. In two patients, LV lead was jailed using coronary stent to prevent displacement. Two patients had CS stenosis and required balloon dilation while one patient had tortuous posterolateral vein which was straightened using a coronary stent. There was technical failure of 6/23 LV leads (26.08 %) with loss of capture, at a median follow up of 44 months (Range: 06–114 months). Out of these 6 patients, stylet and guidewire retaining techniques were used in 4 and 2 patients respectively. Conclusion: Despite having acceptable parameters at implantation, these techniques particularly stylet and guidewire retention, may lead to non-capture of LV lead on long term follow ups. Better LV leads like active fixation leads and conduction system pacing (His Bundle/left bundle branch pacing) should be preferred in difficult CS anatomy.

Summary: Background: Only limited information exists regarding the epidemiology of Kawasaki disease (KD) in low-income and middle-income countries. The present study provides the incidence of KD during 2015–2019 in Chandigarh, north India. Our centre follows the largest KD cohort in India. Methods: Children with KD at Chandigarh diagnosed during January 2015–December 2019 were enrolled in the study. Annual incidence rates were determined using decadal growth rates of the National Census 2011. We computed the incidence of KD in children aged

Abstract Background Portsmann and co. performed the first PDA device closure in 1967. The technique and the devices used have evolved since then and are the first choice in managing anatomically feasible patent ductus arteriosus (PDA) for the last 20 years. Though catheter-based closure of PDA is generally a simple procedure, there are instances when the interventionist faces challenges, especially in smaller children, with syndromic features and venous anomalies even when defects are small and pulmonary artery pressures are normal. Although the femoral vein is the relatively risk-free standard access, internal jugular vein, femoral artery, and transhepatic IVC can be used to close the PDA in different anomalies. The rare venous anomaly of infrahepatic interruption of the IVC with azygous continuation poses technical challenges when percutaneous closure of PDA was attempted through the standard femoral access. Case presentation We report a rare case of PDA device closure in a syndromic child with a short neck having interrupted IVC via femoral-azygous venous approach. Conclusion Knowledge of the IVC course and its anomalies should be known to the operator before the percutaneous closure of PDA. Although other approaches are available, femoral vein approach can be used in case of interrupted IVC for percutaneous closure of PDA.

Management of pulmonary atresia with VSD is a difficult task negotiating varied anatomical and physiological obstacles for establishing adequate pulmonary blood flow. In underweight infants where surgical palliation has its own challenges, pulmonary valve perforation and RVOT stenting offers a physiological pathway for maintaining adequate pulmonary blood flow in selected subset with membranous pulmonary atresia. We present a case of successful pulmonary valve perforation with RVOT stenting to establish the antegrade pulmonary flow in a sick underweight infant.

The modern-day surgical techniques and strategies have changed the outlook of patients with dextro-transposition of great arteries (d-TGA). The survival of an unrepaired d-TGA into late adulthood is difficult to explain. Even when large intracardiac shunts are present, it still remains a lethal cyanotic congenital heart disease if it is not surgically corrected soon after birth. Here, we report an extremely rare case of d-TGA presenting at 40 years of age, with moderately elevated pulmonary artery pressures and relatively stable symptoms.

The objective of this work is to optimize the geometry of the heat sink, which improves the performance. The geometrical parameter chosen for optimization is fin gap. The heat sink is under the condition of natural convection. The optimization method used is particle swarm optimization (PSO). Entropy generation minimization is the objective function. The constraints are set according to the design requirements. The idea of entropy generation minimization is employed to combine the effects of thermal resistance and pressure drop within the heat sink. The optimization problem is solved with the help of MATLAB. The optimized fin gap obtained is b ​= ​6.5 ​mm. The value of entropy generation is 21.4365 ​W/K. Keywords: Heat sink, Plate fin, Particle swarm optimization, Entropy generation, Thyristor

In patients with rheumatic mitral valve stenosis having atrial fibrillation, the “Ball valve thrombus” is an uncommonly encountered complication. A case of 42-year-old male with rheumatic severe mitral stenosis, mild aortic stenosis with moderate regurgitation presented with recurrent episodes of non-exertional syncope for seven days as chief symptom. He was in atrial fibrillation with controlled ventricular rate and echocardiography, showed a large ball valve thrombus in the left atrium. Thus, echocardiography facilitated for rapid diagnosis and earliest surgical intervention as a life saving measure.

Background: Tuberculosis is probably the most important infectious disease of human beings. Early diagnosis of tuberculosis and initiating prompt treatment has been the mainstay in interrupting the transmission of the infection to others in the community. Aim: The present study was aimed at evaluating and comparing the different tests which are available for the diagnosis of tuberculosis and at determining their feasibility as diagnostic tests in terms of their effectiveness and affordability. Materials and Methods: 100 sputum samples, 50 samples which were positive for acid fast bacilli (AFB) and 50 samples which were negative for AFB on Ziehl-Neelsen (ZN) staining were included in the study. The sputum was decontaminated and concentrated by the modified Petroff’s method and the deposit which was obtained was inoculated onto plain Lowenstein –Jensen (LJ) slants and the rapid slide culture (RSC) medium. Results: Of the 100 sputum samples which were inoculated onto the RSC and LJ mediums, 64 samples showed growth of Mycobacterium tuberculosis on RSC after a mean incubation period of 7 days and 63 samples showed a positive growth on LJ medium after a mean incubation period of 34 days. Conclusion: RSC is more sensitive as compared to the direct smear examination and as sensitive as the LJ medium for the diagnosis of tuberculosis, but RSC had an advantage of a shorter turn around a period of 7 days.

Pemphigus is an autoimmune blistering disorder characterized by the presence of intraepidermal blisters and erosions, primarily affecting the mucosa and/or skin. There are no established Indian guidelines for the management of pemphigus, and Western guidelines cannot be directly applied due to differences in clinicodemographic profiles, comorbidities, and resource limitations. These guidelines aim to provide Indian dermatologists with evidence-based and consensus-driven recommendations for the management of pemphigus vulgaris (PV) and pemphigus foliaceous (PF), taking into account the unique challenges posed by the Indian healthcare setting. The guidelines focus on the comprehensive management of PV and PF, addressing diagnosis, treatment, monitoring, and follow-up. It is intended for dermatologists working in both outpatient and inpatient settings across India. The first draft of the guidelines was prepared by the writing group and then reviewed by 19 national experts in pemphigus management, including inputs from allied specialties. Areas with limited evidence or anticipated variation in recommendations were subjected to rounds of voting, with responses categorized as “strongly agree,” “agree,” “neutral,” “disagree,” or “strongly disagree.” Suggestions were incorporated, and statements were revised until a mean agreement score of 4 or higher was reached across 16 key areas after four voting rounds. These guidelines offer a structured approach to managing pemphigus in India, addressing the need for region-specific recommendations that account for unique challenges such as resource constraints and specific comorbidities in Indian patients. They serve as a valuable resource for dermatologists treating pemphigus in a range of clinical settings.

Introduction: 22q11.2 deletion is associated with conotruncal anomalies and immunological aberrations. Given the common embryonic origin of conotruncus and thymus, conotruncal anomalies may be associated with immunological aberrations irrespective of 22q11.2 deletion. We planned to study the prevalence of immunological aberrations and 22q11.2 deletion among patients with conotruncal anomaly to understand the impact of their interplay. Patients and Methods: Preoperative children (age

Total anomalous pulmonary venous connection (TAPVC) is anomalous drainage of all pulmonary veins into systemic circulation. The intracardiac type typically entails the drainage of all the pulmonary veins into the right atrium, via the coronary sinus. The connection of the pulmonary veins directly into the right atrium is exceptionally rare and has been primarily reported with right atrial isomerism. Herein, we presented a remarkable case of TAPVC in a 10-year-old male child, distinguished by an unconventional drainage of all the pulmonary veins directly into the right atrium, with normal coronary sinus and absent right atrial isomerism. Intriguingly, computed tomography imaging revealed a combination of incredibly rare coexistent pulmonary, vascular, and skeletal anomalies. These anomalies included absence of pulmonary fissures in the right lung, presence of left circumflex aortic arch with bovine branching pattern, bilateral cervical ribs, and C7 vertebral fusion anomalies. To our knowledge, this unique combination of coexistent anomalies has not been previously reported in scientific literature in the background of rare drainage pattern of TAPVC.

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disorder affecting pilosebaceous units causing painful nodules, abscesses, and scarring. Despite global studies linking HS to metabolic syndrome, insights specific to Indian patients are limited. Materials and Methods: This study aimed to describe the clinicodemographic characteristics and study frequency of metabolic syndrome in Indian HS patients. In this cross-sectional study, 100 HS patients were evaluated for clinical characteristics, comorbidities, pain and suppuration scores, quality of life, and ophthalmological and dental abnormalities. Metabolic parameters, including anthropometry and blood markers, were performed in all cases and 100 age- and gender-matched controls. Results: The mean age was 29.47 years, with a male predominance of 57%. HS predominantly affected a combination of two or more sites (60%), with nodules as primary lesions (95%). Quality of life was significantly impaired, with 49% experiencing moderate impact as per Dermatology Life Quality Index. Metabolic derangements, represented by an increased body mass index, waist circumference, blood pressure, fasting blood sugar, and fasting insulin, were significantly more frequent in HS cases compared to controls (P < 0.001). The frequency of metabolic syndrome in HS patients was 42%, significantly higher than controls (8%, P < 0.001) with a relative risk of 5.25 (95% CI 2.68–10.58). Dental and ophthalmological abnormalities were observed in 35% and 21.7% of screened patients, respectively. Limitations: Cross-sectional nature, no prospective assessment to determine evolution with time, and limited patient number are the limitations of the study. Conclusion: A substantial association with metabolic syndrome and considerable impact on quality of life is seen in Indian HS patients, emphasizing the need for metabolic screening and holistic management strategies.

Pemphigus in pregnancy is a special clinical scenario that has potential consequences on both maternal and fetal outcomes. Being an autoimmune disease with Th2 preponderance, pemphigus is expected to flare in pregnancy, especially in the first two trimesters. Fetal outcomes like stillbirth and neonatal pemphigus have been reported, the latter being a consequence of a transient transplacental transfer of autoantibodies. Management needs to be individualized keeping the risk/benefit ratios of therapies in mind while optimizing maternal and fetal health. It is crucial to have appropriate counseling regarding conception for women with pemphigus in the child-bearing period because the probability of adverse materno-fetal outcomes is higher if the disease is severe.

Background: Subclinical involvement of nerves may sometimes be present much before the overt clinical manifestations become apparent. Protein gene product (PGP) 9.5, a ubiquitin-C-terminal hydrolase, has been widely used as a marker to study the involvement of peripheral nerve fibers in many diseases. Aim and Objectives: To evaluate the change in cutaneous nerve fiber staining and distribution from pre-treatment and post completion of multidrug therapy through the expression of PGP9.5 and to assess PGP9.5 as a marker of treatment response. Materials and Methods: In this prospective single-center observational study, skin biopsy was taken in patients with leprosy, having areas of nerve function impairment (NFI), based on findings of nerve conduction studies (NCSs), but not having lesions or impaired tactile or thermal impairment clinically. The thin nerve fiber density in the clinically normal skin in areas supplied by nerve showing changes of sensory neuropathy was evaluated to study the density of the fibers. A second biopsy was taken at the end of treatment from a site near the previous site to assess the changes in intra-epidermal nerve fiber staining and distribution. Results: Thirty-three patients were recruited in the present study (24 males and 9 females). Pre-treatment, 27 patients had abnormal NCSs, while six patients did not have any evidence of neuropathy on NCSs. Staining for nerve fibers using PGP9.5; in the epidermis was positive in five patients pre-treatment and 11 patients post treatment (P = 0.181). Staining in the dermis revealed positivity in 14 pre-treatment, which increased to 18 post treatment (P = 0.342). Adnexae showed positivity in five patients pre-treatment and increased to 17 post treatment (P = 0.005). Conclusion: A reduced PGP9.5 staining in the epidermal, dermal, and adnexal regions was seen in leprosy patients, which improved post treatment. Thus, PGP9.5 may serve as a marker of NFI and treatment response.

Epidermolysis bullosa (EB) is a heterogeneous group of genetic disorders characterized by the formation of blisters either spontaneously or at the sites of trauma. These heal with post-inflammatory hypopigmentation, scarring, or milia formation. We hereby present a child who presented with widespread hypopigmented atrophic areas, blistering at trauma-prone sites, and nail dystrophy. The significance of this particular case lies in the challenge of distinguishing between epidermolysis bullosa and bullous extragenital lichen sclerosus et atrophicus.

Tracheobronchial compression due to cardiovascular disease is a rare cause of respiratory distress in children, and common causes include vascular slings and rings, abnormal origin of major vessels, and left atrial enlargement. Intrathoracic airway compression due to pericardial effusion is a rare cause of respiratory distress in children. Here, we present a 6-month-old male child who presented with respiratory distress due to extraluminal compression of the left main bronchus by large pericardial effusion leading to left lung collapse, which improved after pericardiocentesis.

Mosaicism has long been considered the underlying mechanism of segmental infantile hemangiomas (SIH). This was a prospective pilot case-control study conducted with the objective to quantify the percentage overlap of silhouettes of facial SIH with those of Blaschko lines (the most well studied archetypical pattern of mosaicism on face) as compared to other mosaic disorders on face.Lesional silhouettes of 8 patients with SIH (Group A) and 6 patients with other facial dermatosis known to have blaschkoidal distribution (Group B), were overlapped on a standardized template with Blaschkoidal lines on the frontal view of face. The alignment was done via the auto align tool of Photoshop and the percentage of overlap was calculated with an online image comparison software (IMGonline.com.ua). There was a significant difference in mean overlap in Group A (72.92 ± 15.6 %) as compared to Group B (90.1 ± 4.3%; P=0.018). Hence, we concluded that facial SIH do not follow lines of Blaschko.

Background: Congenital ichthyoses are a rare Mendelian group of disorders affecting the integument with a heterogeneous clinical presentation amongst which scaling is a constant feature. There is scanty epidemiologic data regarding the clinical profile and histologic patterns of inherited ichthyosis from resource-poor countries. Aims and Objectives: The study was aimed at assessing the clinic-epidemiologic characteristics associated with the different forms of non-syndromic congenital ichthyosis. Materials and Methods: This was a retrospective chart review of ichthyosis patients that presented between July 2016 and Jun 2020. Details including demographic profile, clinical characteristics along with any relevant investigations done were included. Results: During the study period of 4 years, 107 patients with congenital non-syndromic ichthyosis were seen. The most frequent diagnosis was of common ichthyoses, followed by autosomal recessive congenital ichthyosis, epidermolytic ichthyosis and erythrokeratoderma, in decreasing order. Conclusion: Important clinical findings like erythema and the type of scales as well as histological differences including an absent or reduced granular layer in ichthyosis vulgaris can help differentiate among the clinical phenotypes of inherited non-syndromic ichthyosis especially in resource-poor settings. Also, there is a high prevalence of vitamin D deficiency and hence a need for screening for the same in all patients of congenital ichthyosis including the milder phenotypes.

Previous studies have raised concerns about the effects of oral propranolol on the central nervous system in infants, the exact measure and mechanism and the long-term follow-up of which is less well studied. This was an ambispective comparative study of children with infantile haemangioma (IH) followed by a repeat visit 4–10 years after completion of propranolol therapy. Parents were asked about psychologic functioning along with an initial screening examination. All patients were evaluated by a paediatric psychiatrist. After evaluation by the Strength and Difficulties Questionnaire, and subsequently by the paediatric psychiatrist, 2 of 12 patients (16.67%) showed features of attention deficit hyperactivity disorder in comparison to 0 of 40 subjects in the control group (0.0498; α = 0.05). These results indicate an increased risk of neuropsychiatric illnesses such as attention deficit hyperactivity disorder (ADHD) in patients given propranolol for IH, as supporting evidence to previous claims.

Congenital anomalies of inferior vena cava are increasingly being recognized with the technical advancements and increased utilization of cross-sectional imaging techniques. Duplication of inferior vena cava classically involves duplication of the infrarenal segment, where both inferior vena cava ascend on either side of the abdominal aorta until they form a confluence at the level of the renal veins. It has been extensively described in literature with few reports of more complex variation in the form of duplicated infrarenal inferior vena cava with azygos or hemiazygos continuation. This article describes extremely rare complete duplication of inferior vena cava involving both suprarenal and infrarenal segments. Moreover, the complete duplication of inferior vena cava is seen in association with concomitant double superior vena cava, in a patient with visceroatrial situs solitus and associated congenital heart disease, which to the best of our knowledge, has not been reported so far in literature. This study also highlights the utility of multidetector computed tomography in accurate identification of such anomalies.

Partial anomalous pulmonary venous drainage is a congenital cardiac disorder characterized by abnormal drainage of one or more pulmonary veins into the systemic circulation. It can be isolated or associated with other congenital cardiac anomalies, most commonly atrial septal defect and patent ductus arteriosus. The clinical presentation is variable and depends on the degree of shunting and associated cardiac anomalies. Many patients usually remain asymptomatic until late in life. In this article, we presented a complex case of obstructive partial anomalous pulmonary venous drainage with dual drainage of bilateral accessory pulmonary veins with intact interatrial septum in conjunction with a patent ductus arteriosus and a ventricular septal defect. This pattern is incredibly rare and to the best of our knowledge has not been previously reported. Computed tomography played a pivotal role in precisely elucidating the intricate anatomy in this case with a complex pattern of anomalous pulmonary venous drainage.

Changes in heat shock factor 1 (HSF1) levels have significant impacts on living organism survival after chemical and thermal stress. The levels activate nuclear-localized heat shock factor binding protein 1 (HSBP1), which negatively affects DNA-binding and represses transactivation in mammalian cells. Tests on Caenorhabditis elegans protein demonstrated the interaction between HSF1 levels and HSBP1 as its negative regulator.

Background: Nodulocystic acne is a severe type of acne that is known to improve after treatment with isotretinoin. Melnik has hypothesized a unifying concept on the mechanism of acne pathogenesis involving altered expression of Forkhead box O transcription factor (FoxO1) and role of isotretinoin in improving acne via modulating this pathway. Aim: To evaluate the pathway proposed by Melnik in acne pathogenesis by analysing the difference in the expression of FoxO1, peroxisome proliferator-activated receptor (PPARγ), and androgen receptor (AR) between acne patients and non-acne controls and the effect of treatment with isotretinoin on change in expression of these genes in acne patients. Results: The gene expression of FoxO1 was non significantly higher in acne patients as compared to controls. After treatment with isotretinoin, a significant decrease in FoxO1 expression in acne patients at mRNA (P = 0.05) level was observed. There was a significant decrease in grade 3 positivity of FoxO1 at protein level (P = 0.0009). A decrease in androgen receptor positivity (P = 0.055) at protein level was also observed. Conclusion: Reduction in FoxO1 expression appears to be an important mechanism of action of isotretinoin in acne.