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Background : Open-heart surgery is challenging in preterm neonates and infants, and its feasibility in low-resource settings has not been defined. We describe our institutional experience with open-heart surgeries performed on consecutive preterm infants. Materials, Methods and Results : This was a single-center retrospective cohort from a tertiary hospital in Southern India and included consecutive preterm neonates (<37 weeks) admitted for open-heart surgery. This report is limited to babies who were <3 months at the surgery. The salient features of the 15 preterm included twin gestation: 7 (46.7%); median gestational age at birth: 35 weeks (28-36 weeks); median corrected gestational age at surgery: 37 weeks (33-40 weeks); birth weight: 1.75 kg (1.0-2.6 kg); weight at surgery: 1.8 kg (1.2-2.9 kg); and small for gestational age: 12 (80%). The heart defects included transposition of the great arteries (7), total anomalous pulmonary venous return (3), large ventricular septal defect (VSD) (1), and VSD with coarctation of the aorta (4). Eleven (73%) were mechanically ventilated preoperatively and five had preoperative sepsis. The mean cardiopulmonary bypass time was 169.7 ± 61.5 min, and cross-clamp time was 99.7 ± 43.8 min. There was no inhospital mortality; one baby expired during follow-up at 1 month. Postoperative mechanical ventilation duration was 126.50 h (84.25-231.50 h), and intensive care unit stay was 13.5 days (9-20.8). The total hospital stay was 39 days (11-95 days). Two children (13.3%) had postoperative sepsis. Conclusion : Through collaborative multidisciplinary management, excellent outcomes are feasible in low-resource environments for selected preterm neonates undergoing corrective open-heart operations. [ABSTRACT FROM AUTHOR]

Introduction: The treatment of late-presenting Perthes disease with extrusion is controversial and debatable. One of the options available is the labral shelf acetabuloplasty (LSA). Aim: The aim of the study was to evaluate the results of LSA in late-presenting Perthes disease in terms of clinic-radiological outcome measures. Materials and Methods: A retrospective analysis of prospectively collected data of patients with late presenting Perthes disease (Elizabethtown stage 2B onwards) treated by LSA by 2 experienced paediatric orthopaedic surgeons was performed. Data was collected of clinical parameters such as hip range of motion(ROM) and Harris Hip score and radiological parameters such as acetabular height, width and volume, shelf width, Centre Edge angle(CEA) and the lateral extrusion. Results: Thirty-five patients (28 males and 7 females) treated between 2012 to 2019 were analyzed. Majority were in Elizabethtown stage 3A (23) followed by 2B and 3B (12 each). At a mean follow up of 36 months, the hip ROM and the Harris Hip Score (from 65 ± 3.5 to 81.33 ± 7.12) improved significantly and there was a statistically significant improvement in terms of all radiological parameters. Majority of the hips were in Stulberg grade 3 (20) followed by grade 1 and 2 (7 each) and Stulberg 4 (1). There were no major complications in any of the patients of the series. Conclusion: Labral support shelf acetabuloplasty is a valuable surgery for late presenting Perthes disease and helps in maintenance of good coverage and allows restoration of range of motion over time. [ABSTRACT FROM AUTHOR]

Introduction: Malrotation is a congenital anatomical anomaly that affects the normal positioning of the intestines. Traditional management of malrotation, as described by Ladd, consists of detorsion of the volvulus if present, division of Ladd's bands, widening of the mesenteric root, proper positioning of the small and large bowels, and a prophylactic appendectomy. This study was done to determine whether appendectomy should be an integral part of the Ladds procedure or if it can be avoided. Materials and Methods: This retrospective observational study was conducted in one pediatric surgical unit in the tertiary care center of North India. All the cases of malrotation of the gut managed from January 2008 to December 2018 were reviewed. The details of the patients were recovered from the electronic data recording system of the hospital and manual operation theater records. The cases that have a follow-up of <5 years were not included in the study. The details were charted in an Excel Sheet for the analysis. No statistical test was performed because there was no event in patients in whom prophylactic appendectomy was not done. Results: The data analysis revealed that a total of 66 malrotation patients were managed during this period, those who fulfilled the inclusion criteria. Among 66 cases included in the study, in 41 cases, prophylactic appendectomy was done, whereas in the rest 25, prophylactic appendectomy was not done. During the follow-up, none of the patients in whom the appendix was preserved presented with signs or symptoms related to appendicitis or any other complications. Conclusion: Ladds procedure without appendectomy can be performed in view of the potential use of the appendix in the future, and with the fact that in the current era of advanced medicine, appendicitis can be diagnosed early due to the advancement of imaging and better record keeping. [ABSTRACT FROM AUTHOR]

Background: Psoriasis begins in childhood in around one-third of the cases. There has been conflicting evidence regarding the association of paediatric psoriasis with comorbidities. Aims and Objectives: The objective of this study was to find out various comorbidities (abnormal body mass index, metabolic syndrome, lipid abnormalities, diabetes mellitus, and raised blood glucose) associated with paediatric psoriasis. Materials and Methods: All patients of psoriasis (age <18 years) who visited the Paediatric Psoriasis clinic from January 2017 to September 2021 were recruited in this record-based study. Results: Records of 100 patients were analysed, with female to male ratio of 1:1. The age group ranged from 11 months to 18 years. The average body surface area involved was 5.43%. The average psoriasis area and severity index was 2.47. Joint involvement was seen in 7% of patients in our study. A total of 52 (52%) patients had abnormal body mass index, out of which 21 patients were overweight and 31 were obese. At least one lipid abnormality was found in 66 (66%) patients. The most common lipid abnormality was decreased high-density lipoprotein, followed by raised total cholesterol, raised total triglycerides and raised low-density lipoprotein. 8 (8%) patients were found to have raised blood glucose levels. Out of which, six had impaired fasting glucose and two were diagnosed with diabetes mellitus. 5 (5%) patients were diagnosed with metabolic syndrome. Conclusion: Paediatric psoriasis is a chronic disfiguring disease and may have profound emotional and psychological effects. There is a lack of studies from India on the prevalence of these comorbidities in paediatric psoriasis. More studies are required from different parts of the world for a better understanding of paediatric psoriasis. [ABSTRACT FROM AUTHOR]

Introduction: Peutz--Jeghers Syndrome (PJS) is an autosomal dominant disease presenting with hamartomatous polyps in the gastrointestinal tract and hyperpigmented macules on lips and oral mucosa. The incidence of this syndrome is approximately 1 in 1,20,000 births. Materials and Methods: In this article, we are presenting 11 cases of PJS which were misdiagnosed and patients were compelled to visit hospital repeatedly. All these cases were diagnosed based on clinical suspicion, family history, and histopathological examination of specimens. Most of the cases presented with intussusception and required emergency surgical management. Results: PJS can be diagnosed by the presence of microscopically confirmed hamartomatous polyps and a minimum of two of the following clinical criteria: Family history, mucocutaneous melanotic spots, and small bowel polyps with bleeding per rectally. The diagnosis can be missed if the melanotic spots on the face are missed. Routine investigations, imaging, and endoscopy were done in all cases. PJS patients need regular follow-up due to chance of recurrence of symptoms and susceptibility to cancer. Conclusion: PJS needs a high index of suspicion for diagnosis in cases of recurrent abdominal pain with bleeding per rectum. Proper family history and meticulous clinical examination for melanosis are very important to prevent the misdiagnosis of these cases. [ABSTRACT FROM AUTHOR]

Introduction and Aims : Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome. Methods and Results : We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5--9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up (n = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias (P value-- 0.05). The 5-year survival rate of DCM patients was 59%. Conclusion : A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts. [ABSTRACT FROM AUTHOR]

Introduction: Highly active antiretroviral therapy (HAART) is used to treat human immunodeficiency virus type 1 (HIV-1). Introduction of antiretroviral therapy (ART) has reduced the HIV/AIDS associated morbidity and mortality significantly. But 25% of all patients discontinue treatment because of adverse drug reactions (ADRs). Adverse cutaneous drug reactions (ACDR) are very common with ART regimens, which may range from mild pruritus, maculopapular rash to serious Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN). ACDRs comprise 10%-30% of all reported ADRs. Aims and Objectives: To assess the different types of cutaneous adverse drug reactions in immunocompromised children of less than 18years. Materials and Methods: This is a retrospective record-based study, conducted at department of Dermatology, Venereology and Leprosy, Government Medical College (GMC)/Government General Hospital (GGH), Suryapet, Telangana, India. Data was collected from the records available at ART centre, from November 2018 to October 2021 GGH, Suryapet. All the HIV infected children =18 years who were on ART, were included in this study. Patients of more than 18 years and on other medications were excluded. Demographic data, socio economic status, vaccination status, height, weight, complete blood analysis, complete urine analysis, erythrocyte sedimentation rate, liver and renal function tests and CD4 counts were recorded before initiation of ART. Results: A total of 330 children of less than 18 years were initiated for ART, at ART centre, Government General Hospital, Suraypet. Out of 330 children, 27.8% (92) children developed ACDRs. 58.7% (54) were males and 41.3% (38) were females. Maculopapular rash was seen in 65.2% (60) cases, urticaria was seen in 15.3% (14) cases, Steven Johnson Syndrome (SJS) was seen in 9.8% (9) cases, SJS/TEN overlap was seen in 6.5% (6) cases and toxic epidermal necrolysis (TEN) was seen in 3.2% (3) case. CD4 count was below 300 in 65.3% (60) cases above 300 in 34.7% (32) cases. Gap between initiation of the treatment and onset of reaction was less than one month in 65.3% (60) cases, and more than one month in 34.7% (32) cases. [ABSTRACT FROM AUTHOR]

Background: Glomerular diseases vary with age, and it is important to investigate the spectrum of glomerular diseases in pediatric patients to help in a more precise clinical diagnosis and optimize the management of patients. We aimed to study the clinicopathologic pattern of pediatric glomerular diseases in North India. Methods: This is a 5-year retrospective, single-center cohort study. The database was searched to identify all pediatric patients with glomerular diseases in their native kidney biopsies. Results: About 2890 native renal biopsies were studied, of which 409 were pediatric glomerular diseases. The median age was 15 years with a male preponderance. Nephrotic syndrome was the most common presentation (60.8%), followed by non-nephrotic proteinuria with hematuria (18.5%), rapidly proliferative glomerulonephritis (7%), isolated hematuria (5.3%), acute nephritic syndrome (3.4%), non-nephrotic proteinuria (1.9%), and advanced renal failure (0.7%). Minimal change disease (MCD) was the most common histological diagnosis, followed by focal segmental glomerulosclerosis (17.4%), IgA nephropathy (IgAN; 10%), membranous nephropathy (6.6%), lupus nephritis (5.9%), crescentic glomerulonephritis (2.9%), and C3 glomerulopathy (2.9%). Diffuse proliferative glomerulonephritis (DPGN) was the most common histological diagnosis in patients with hematuria and non-nephrotic as well as nephrotic range proteinuria. The most common histological diagnoses for isolated hematuria and acute nephritic syndrome were IgAN and postinfectious glomerulonephritis (PIGN), respectively. Conclusions: MCD and lupus nephritis are the most common pediatric primary and secondary histopathologic diagnoses, respectively. The adolescent-onset glomerular diseases have a higher frequency of IgAN, membranous nephropathy, and DPGN. PIGN is still an important differential in our pediatric patients presenting with acute nephritic syndrome. [ABSTRACT FROM AUTHOR]

Background: Slightly more than half the total number of childhood leprosy cases worldwide are from India. Aim: To analyze the clinical and epidemiological trends of childhood leprosy over 20 years in a tertiary care hospital. Methods: We retrieved the medical records of all children less than 15 years of age registered in the leprosy clinic between April 1998 and March 2018. We tabulated and analyzed data pertaining to demographic details along with clinical findings such as cutaneous lesions, nerves involved, sensory loss, deformities, reactions, smear status, histopathology and treatment. Results: Out of total 1548 leprosy cases registered during the study period, 55 (3.55%) cases of childhood leprosy were diagnosed. Thirty five (63.6%) children were in the age group of 11–15 years and 83.7% were migrants from other states. Thirteen (23.6%) children reported contact with a diagnosed case of leprosy, mainly in close contacts. Fifty three (96.4%) children presented with cutaneous lesions while 2 (3.6%) had pure neural involvement. Borderline tuberculoid leprosy was the most common clinical presentation in 27 (49.1%) followed by borderline lepromatous leprosy in 11 (18%). Thickened peripheral nerve trunks were detected in 42 (76.4%), most commonly the ulnar nerve. Reactional episodes occurred in 12 (21.8%) cases (Type 1 reaction, 10 (18.2%); Type 2 reaction, 2 (3.6%)). Grade 2 disability was detected in 4 (7.3%). Multidrug therapy was started in all patients, multibacillary (MB) regimen in 42 (76.3%) patients and paucibacillary (PB) regimen in 13 (23.7%). Twenty five (45.4%) children defaulted from the treatment. On comparing the data of 2008-18 with that of the previous decade (1998–2007), there was a higher proportion of migrant cases as compared to local cases (3:1–11:1) and MB cases as compared to PB cases (2:1–6:1).T he proportion of treatment defaulters declined from 60% to 36%. Limitations: Relapse rate could not be calculated due to inadequate follow-up period. As it is a hospital-based retrospective study with no active surveys, these findings may not reflect trends in the community. Conclusion: Childhood leprosy continues to be a significant problem. There is a clear need to strengthen early detection, treatment and regular follow-up of these cases in both high and low endemic settings. [ABSTRACT FROM AUTHOR]

Background: Most centers in developing countries prefer chelation therapy with D-penicillamine for the management of Wilson's disease (WD) because of its easy availability and affordability. Neurological worsening following treatment with D-penicillamine is not uncommon. However, there is a paucity of Indian data on the incidence of neurological worsening in children and adolescents with WD following chelation therapy. Our study objectives were to identify the prevalence of neurological worsening in children and adolescents with WD following chelation with D–penicillamine therapy and to describe the management options and outcomes in these patients. Materials and Methods: In this retrospective chart review, children and adolescents with an established diagnosis of WD from 2010 to 2020 were identified from the hospital electronic database. Among these patients, data of children and adolescents with neurological worsening following D–penicillamine therapy were extracted and analyzed. Results: Neurological worsening was observed in 27/122 (22.1%) children and adolescents with WD on chelation therapy with D-penicillamine. Fifteen patients with neurological worsening following D-penicillamine therapy were managed with zinc monotherapy. Four patients were managed with a combination therapy of zinc and trientine. Five patients were treated with trientine monotherapy. Re-challenging with D-penicillamine at a lower dose followed by a slow dose escalation was attempted in three patients. Gradual clinical and functional status improvement was observed in 24 cases while one patient succumbed to pneumonia. Conclusion: Children and adolescents with WD who had neurological worsening on D-penicillamine therapy may be managed with trientine. Zinc monotherapy with copper restricted diet was also found to be effective in non-affordable patients. [ABSTRACT FROM AUTHOR]

There is limited information on the feeding practices of 9.42 million children living within institution‐based care (IBC) worldwide. Poor feeding practices can predispose or exacerbate malnutrition, illness and disability. Here we describe the feeding practices of children living within IBC based on a retrospective analysis of records from 3335 children, 0–18 years old, participating in Holt International's Child Nutrition Program (CNP), from 36 sites in six countries. Data analysed included demographic information on age, sex, feeding practices, disabilities and feeding difficulties. Descriptive statistics were produced. A generalised linear model explored associations between feeding difficulties and disability and 2 × 2 tables examined feeding difficulties over time. An additional set of feeding observations with qualitative and quantitative data was analysed. At baseline, the median age of children was 16 months (0.66–68 months) with 1650/3335 (49.5%) females. There were 757/3335 (22.7%) children with disabilities; 550/984 (55.9%) were low birth weight; 311/784 (39.7%) were premature; 447/3113 (14.4%) had low body mass index and 378/3335 (11.3%) had feeding difficulties. The adjusted risk of having a feeding difficulty was 5.08 ([95% confidence interval: 2.65–9.7], p ≤ 0.001) times greater in children with disabilities than those without. Many children saw their feeding difficulties resolve after 1‐year in CNP, 54/163 (33.1%) for children with disabilities and 57/106 (53.8%) for those without disabilities. Suboptimal hygiene, dietary and feeding practices were reported. In conclusion, feeding difficulties were common in IBC, especially among children with disabilities. Supporting safe interactive mealtimes for children living within IBC should be prioritised, to ensure overall health and development. Key points: Feeding difficulties are common among children living in institution‐based care (IBC), particularly but not exclusively among those children with disabilities.Suboptimal feeding practices were common in IBC and encompassed inadequate hygiene, limited support for self‐feeding, reading children's feeding cues (especially around pacing and satiety), addressing feeding difficulties, such as difficulty chewing or swallowing. These should be prioritised in training and supervision for caregivers.Addressing the needs of this vulnerable group should include support for safe feeding techniques. These should be prioritised to help ease the transition into eventual family‐based care if we are to move towards deinstitutionalizing children and strengthening families. [ABSTRACT FROM AUTHOR]

Background: To describe the clinical profile, risk factors, and outcomes that are associated with candida infection among critically ill children. Patients and methods: A retrospective case-control study wherein 109 children admitted to the pediatric intensive care unit (PICU) in the years between 2015 and 2017 with the growth of candida from blood, urine, endotracheal (ET) aspirate, and pus swabs were included and compared to 97 age and sex-matched controls chosen from the same time period. Results: Of the 124 candida isolates from 109 children, 37% were from blood, 24% from urine, and 14% in pus; 40% of the isolates were from ET aspirate. Candida non-albicans types (70%) predominated with Candida tropicalis causing 50% of the infections. Risk factors for candida infection were neutropenia [OR 20.01, 95% CI (0.94-422.32)], mechanical ventilation [OR 5.97, 95% CI (2.44-14.62)], peritoneal dialysis [OR 5.81, 95% CI (1.27-26.50)], institution of amino acids [OR 5.41, 95% CI (0.85-34.13)], presence of central venous catheter [OR 3.83, 95% CI (1.59-9.19)], antibiotic use >5 days [OR 3.58, 95% CI (1.38-9.29)]. Candida Cases (95.4%) had a septic shock with acute kidney injury in 34% and had significantly lower survival than controls [72 (66%) of 109 vs. 74 (80%) of 92] (p = 0.023). Conclusions: The rate of candida infection in our PICU was 4.2% of PICU admissions. The most common species was C. tropicalis. The independent risk factors for candida infection were neutropenia, antibiotic duration >5 days, peritoneal dialysis, amino acid administration, mechanical ventilation, and presence of a central venous catheter (CVC). [ABSTRACT FROM AUTHOR]

Background: The purpose of this study was to examine the evolution of Legg–Calvé–Perthes' disease (LCPD) among children from British Columbia (BC), Canada who were treated non-operatively and to compare the results to a previously conducted study in India. Methods: This was a retrospective review of patients treated non-operatively for LCPD in BC between 1990 and 2006 compared with a cohort from India. Demographic and treatment information were collected from medical records. Radiographs were assigned modified Waldenstrom, Catterall, Salter–Thompson and Herring classifications and intra- and interobserver reliability were assessed. We evaluated epiphyseal extrusion (EE) and metaphyseal width (MW), and assessed radiographs using the Mose and modified Stulberg classifications. Results: 102 hips (90 patients) had radiographs available for evaluation. 95% of the BC cohort presented as Waldenstrom stages I and II, whereas, 90% of the Indian cohort presented as IIIa. Final EE was similar for both groups (BC 26.8%, India 27.3%) and final MW was 119% in both groups. Modified Waldenstrom and Herring classifications had substantial intra- and interobserver reliability, while Salter–Thompson and Catterall classifications had moderate agreement at best. Most hips were Catterall IV (80%) and Herring C (89%) for the BC cohort compared to only 44% and 43% of Indian hips, respectively. Most hips were irregular according to the Mose classification (BC 43%, India 52%) and aspherical according to the Stulberg classification (BC 78%). Conclusions: We found similar radiographic progression and final radiographic appearances of LCPD in India and BC though differences in the distribution of the classification systems warrant further study. [ABSTRACT FROM AUTHOR]

Background: Nutritional rehabilitation centers (NRCs) were established with the objective of providing institutional care to malnourished children and building the capacity of the primary caregivers of these children. Objectives and Methodology: The objective of this study was to assess the effect of nutritional interventional measures undertaken at NRCs in improving the nutritional status of admitted children; a retrospective facility-based descriptive study was conducted using the monthly performance reports (March 2015 to November 2019) of all NRCs in Dhamtari district, Chhattisgarh. Results: Of the 3171 children registered in the NRCs, the majority were 1--3 years of age (59.22%), female children (55.50%), and from Other Backward Class caste category (56.79%). The district records a cure rate of 56.12%, death rate of 0.03%, and defaulter rate of 4.71%, with an average length of stay being 16.5 days. NRCs of Dhamtari district had less than acceptable level of recovery/cure rate though death rate and defaulter rate were within acceptable standards. Conclusion: Lower cure rate along with lower defaulter rate and death rate with more than recommended length of stay reflects the below-average performance of the NRCs in the district. [ABSTRACT FROM AUTHOR]

Background: Acute respiratory distress syndrome (ARDS) associated with COVID-19 in children is not well described in the literature, so this study was designed to assess the severity, clinical course, different treatment measures, and outcome of this group of patients. Patients and methods: This descriptive study was performed by retrospective chart review of children admitted in pediatric intensive care unit (PICU) in the age-group of 1 month to 12 years over the period of 6 months (July--December 2020) in a tertiary care pediatric COVID facility in eastern India. Severity of ARDS, ventilator settings, oxygenation and laboratory parameters, and outcomes were documented. Predictors associated with severe ARDS were evaluated. Results: Among 128 laboratory-confirmed pediatric COVID-19 cases admitted in PICU, 18 (14%) developed ARDS, 6 (33.3%) had severe ARDS, and 3 (16.6%) succumbed to death. Outcome was measured by median hospital stay [20 days (IQR 19, 21)], PICU stay [13 days (IQR 10, 16)], and 28-day ventilator-free days [14 days (IQR 13, 22)]. Half (n = 9) of our study cohort had different comorbidities and congenital heart disease being the most common (4, 22.2%). Median positive end-expiratory pressure requirement was 10 cm H2O (9, 11) for invasively ventilated children (n = 13, 72.2%) along with peak inspiratory pressure of 24 cm H2O (20, 29) and mean airway pressure of 17 cm H2O (14, 20). Median oxygenation index was 13.3 (10.5, 18.6). Nine (69.2%) out of 13 intubated children had undergone prone ventilation. C-reactive protein (CRP) and D-dimer levels were significantly high in children with severe ARDS alongside pSOFA and lung USG score. Conclusion: Incidence of ARDS in pediatric COVID-19 though less but is not rare. Elevated CRP, D-dimer values, and high lung USG scores were associated with severe ARDS. Those who died had significant comorbidity. [ABSTRACT FROM AUTHOR]

Aim: The aim of the study was o define the preoperative diagnostic clinical and radiological features in girls with congenital pouch colon (CPC). Materials and Methods: Records of 47 girls with CPC, managed from 1996 to 2018, were reviewed. There were two age groups: Group A (newborn to 12 months; n = 26) and Group B (>12 months to 20 years; n = 21). The important clinical and radiologic features to help in a preoperative diagnosis were noted. Results: The most common subtype was Type II (57.4%), followed by Type I (23.4%) and Type III (12.8%). The features common to both the groups were abdominal distension (A = 53.8%; B = 9.52%), severe perineal excoriation (A = 19.2%; B = 23/8%), and urinary incontinence (A = 30.7%; B = 85.7%). In addition, in Group B, fecaloma on abdominal palpation was noted in 28.6% of patients. The characteristic appearance of the perineum including external genitalia and findings on plain abdominal X-ray (AXR) were 100% accurate and hence diagnostic. These unique features helped us formulate an algorithm for preoperative diagnosis of this uncommon form of anorectal malformation in girls seen in North India. Conclusions: The characteristic features on clinical examination should alert one to the presence of CPC in the outpatient clinic. The AXR was diagnostic in 100% of cases and is mandatory. If any doubt persists, examination of the genitalia under anesthesia with more retraction of the labial folds and endoscopy can be performed for confirmation. These measures should enable a clinician to make an accurate preoperative diagnosis in every girl with CPC. [ABSTRACT FROM AUTHOR]

Background: Benign renal tumors are extremely rare and were studied here. This series also includes a renal teratoma in a horseshoe kidney, probably only the second in the pediatric literature. Materials and Methods: Retrospective review of children with benign renal tumors operated between 2006 and 2018 at one center. Results: Twelve patients (M:F ratio 10:2), age range 3 weeks (31-week gestation) to 13 years presented with large palpable renal swelling (n = 12) and hematuria (n = 3). Computed tomography (CT) scan showed features typical of the tumor. Final histopathology (age group [mean]) showed: multilocular cystic nephroma (MLCN) - n = 5 (41.7%), (11–16 months [13.6]); congenital mesoblastic nephroma (CMN) - n = 4 (33.3%) (classic 1, cellular 3) (0.75–5 months [2.125]); mature cystic teratoma - n = 1 (8.3%): (48 months, in a horseshoe kidney), and angiomyolipoma (AML) - n = 2 (16.7%) (144 months [sporadic] and 156 months [tuberous sclerosis]) One patient with cystic teratoma with no calcification on CT scan received pre-operative chemotherapy as fine-needle aspiration cytology (FNAC) reported malignant small blue cell tumor. Nephroureterectomy with Gerota's fascia could be done easily in all without intraoperative complications. Delay in presentation in MLCN and CMN led to increased symptoms and CT scan changes. All patients did well in 1.5–12 years (median 3 years) follow-up including cellular mesoblastic nephroma. Conclusions: Benign renal tumors often occur in specific age groups but may overlap that of Wilms tumor. Proper interpretation of clinical presentation, CT scan, and FNAC findings help in avoiding preoperative chemotherapy. Upfront nephroureterectomy is curative. Histopathological findings decide further treatment. Children with AML and tuberous sclerosis need lifelong follow-up. [ABSTRACT FROM AUTHOR]

Background: Multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a new entity affecting a small percentage of children during the COVID-19 pandemic. Materials and methods: Demography, clinical, and laboratory variables of children admitted from April to September 2020 with MIS-C were studied retrospectively at eight hospitals in Delhi, India. Results: We identified 120 patients [median age: 7 years (interquartile range (IQR): 4-10)] with male-to-female ratio of 2.3:1. Overall, 73 out of 120 children (60.8%) presented with shock, 63 (52.5%) required inopressor support, and 51 (43%) required respiratory support. We categorized the cohort into three observed clinical phenotypes: MIS-C with shock (n = 63), MIS-C with Kawasaki disease (KD) (n = 23), and MIS-C without shock and KD (n = 34). Atypical presentations were hypothermia, orchitis, meningoencephalitis, demyelination, polyneuropathy, pancreatitis, and appendicitis. Ninety-four percent had laboratory evidence of SARS-CoV-2 (78.3%, seropositive and 15.8%, RT-PCR positive). The median C-reactive protein (CRP) was 136 mg/L (IQR, 63.5-212.5) and ferritin was 543 ng/mL (IQR, 225-1,127). More than 90% received immunomodulatory therapy (intravenous immunoglobulins and/or steroids) with an excellent outcome (96% survived). CRP and absolute neutrophil count (ANC) were correlated statistically with severity. Conclusion: MIS-C data from Delhi are presented. Rising CRP and ANC predict the severe MIS-C. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to review the clinical profile, management, and outcome of solid tumors in the head-and-neck region in children at our institute. Methods: We retrospectively reviewed children with head-and-neck solid tumors who were treated jointly under the departments of Pediatric Surgery and Otorhinolaryngology at our institute between 2016 and 2019. Results: In the study period, 10 children (6 males, 4 females) with a median age of 9.5 years (range 5 days–16 years) were treated by our unit. The patients had four tumors arising from the parotid, 2 from nose/naso-pharynx, 1 each from the tongue, submandibular gland, para-pharyngeal space, and infratemporal fossa. A majority (90%) of the patients underwent complete surgical excision of the tumor, preserving the loco-regional neurovascular structures to minimize postoperative morbidity. Malignant lesions were seen in 4 patients (2 rhabdomyosarcoma, 1 primitive neuro-ectodermal tumor, 1 mucoepidermoid carcinoma) and 6 patients had benign pathology (3 pleomorphic adenoma, 2 mature teratoma, 1 schwanomma). Additional therapy with chemotherapy and local radiotherapy was required in 3 out of 4 patients with malignant pathology. The median follow-up duration is 15 months. At last follow-up, all patients are alive and 9 out of 10 patients (90%) are disease-free. Conclusion: Our experience highlights rare and difficult tumors in the head-and-neck region in children. These tumors are not commonly managed by the pediatric surgeon in routine practice. The paper outlines the multi-modality management of these tumors, which is essential for an optimal outcome. [ABSTRACT FROM AUTHOR]

Context: Adnexal masses in girls represent a wide pathological spectrum. We performed a retrospective analysis of clinical and diagnostic aspects of adnexal masses in girls. Aims: To analyze the clinical characteristics and surgical management of adnexal masses in children and adolescents. Settings and Design: Retrospective observational analysis from 2000 to 2020. Subjects and Methods: This analysis was performed on 28 children under the age of 17 years with adnexal masses who were managed by us at tertiary care centers of Delhi-NCR. Patients were analyzed based on their age at the time of admission, their presenting complaints, clinical and radiological findings, tumor markers, management, and follow-up. Results: The age ranged between newborn and 17 years. Abdominal pain, nausea with vomiting, and abdominal lump was the most common presenting complaints. Pelvic ultrasound with or without color Doppler done in all patients and computed tomography, magnetic resonance imaging was performed in seven patients preoperatively. Adnexal masses were unilateral in 26 (92.85%) patients and bilateral in 2 (7.15%) patients. Five babies had antenatal diagnosis of ovarian cysts. Serum alpha fetoprotein (AFP) and beta-human chorionic gonadotropin (b-HCG) were normal in all 19 tested patients. Cancer antigen 125 (CA-125) was raised in three ovarian tumors out of 9 tested patients. Laparoscopy was performed in 23 out of 28 patients (30 ovaries). Ovarian cyst torsion was present in 50% patients. Ovarian cystectomy was done in 10 (33.3%) patients. Six (20%) ovaries were saved by detorsion of the ovary and deroofing of the cyst with or without ovarian biopsy. Oophorectomy was done in five (16.7%) patients. Salpingo-oopherectomy had to be done in seven (23.3%) patients. Histopathology revealed functional nonneoplastic lesions in 18 cases (66.7%), benign neoplastic lesions in 8 cases (29.6%), and malignant neoplastic lesion in 1 case (3.7%). Conclusions: Adnexal masses in children are generally benign. AFP, b-HCG, and CA-125 in ovarian torsion are within normal limits. Urgent surgery is required if torsion is established. The ovary should be preserved wherever possible. [ABSTRACT FROM AUTHOR]

Background and Design: Leprosy is a chronic granulomatous, infectious disease that primarily affects the skin and peripheral nerves. Leprosy in children is an indicator of active transmission in the community. This study aimed to analyze the prevalence and clinical spectrum of childhood leprosy and highlight the importance of examining close contacts and the impact of detection and treatment of leprosy in the community. Materials and Methods: This retrospective study included patients presenting to the dermatology department of a tertiary care hospital attached to a medical college during the years 2011-2019. Results: Two hundred and twenty-three patients with leprosy were analyzed, of which 201 were adults and 22 were children. Children constituted 9.87% of the patients (approx. one child with leprosy for every 10 adults with leprosy). Of 22 children with leprosy, 13 (59.09%) were boys and nine (40.90%) were girls, with a ratio of 1.4:1. Twelve of twenty-two child patients (54.54%) had a single patch, while 10 of 22 (45.45%) had more than one skin lesion. Fourteen patients (63.63%) were diagnosed clinically with borderline tuberculoid (BT) leprosy, and six patients (27.27%) had borderline lepromatous leprosy. Of the fourteen patients with BT leprosy, 10 patients (71.4%) had a single lesion. Eleven patients (50%) were diagnosed with multibacillary leprosy, and eleven patients (50%) were diagnosed with paucibacillary leprosy. A clinicopathological correlation was noted in 16 patients (72.72%). Family contact was observed in four children with leprosy. Conclusion: A high proportion of childhood leprosy cases indicates active transmission and warrants awareness among the community members and healthcare workers in terms of early detection and treatment of childhood and adult leprosy. This helps in preventing transmission, development of grave deformities, and associated social stigma of leprosy among the community members. [ABSTRACT FROM AUTHOR]

Background: Outcome data of children with heart disease who acquired COVID-19 infection are limited. Aims : We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. Settings and Design: This is a retrospective, multicentric, observational study. Materials and Methods: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. Results: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, P = 0.046). Conclusions: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes. [ABSTRACT FROM AUTHOR]

Background: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID-19 on pediatric cardiac care in India. Aims: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. Settings and Design: This is a retrospective, multicentric, observational study. Methods: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. Results : The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). Conclusions: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic. [ABSTRACT FROM AUTHOR]