Pediatric Department Research Articles

LASSO-derived nomogram for early identification of pediatric monogenic lupus.

Monogenic lupus is defined as systemic lupus erythematosus (SLE)/SLE-like patients with either dominantly or recessively inherited pathogenic variants in a single gene with high penetrance. However, because the clinical phenotype of monogenic SLE is extensive and overlaps with that of classical SLE, it causes a delay in diagnosis and treatment. Currently, there is a lack of early identification models for clinical practitioners to provide early clues for recognition. Our goal was to create a clinical model for the early identification of pediatric monogenic lupus, thereby facilitating early and precise diagnosis and treatment for patients. This retrospective cohort study consisted of 41 cases of monogenic lupus treated at the Department of Pediatrics at Peking Union Medical College Hospital from June 2012 to December 2022. The control group consisted of classical SLE patients recruited at a 1:2 ratio. Patients were randomly divided into a training group and a validation group at a 7:3 ratio. A logistic regression model was established based on the least absolute shrinkage and selection operator to generate the coefficient plot. The predictive ability of the model was evaluated using receiver operator characteristic curves and the area under the curve (AUC) index. A total of 41 cases of monogenic lupus patients and 82 cases of classical SLE patients were included. Among the monogenic lupus cases (with a male-to-female ratio of 1:1.05 and ages of onset ranging from birth to 15years), a total of 18 gene mutations were identified. The variables included in the coefficient plot were age of onset, recurrent infections, intracranial calcifications, growth and developmental delay, abnormal muscle tone, lymphadenopathy/hepatosplenomegaly, and chilblain-like skin rash. Our model demonstrated satisfactory diagnostic performance through internal validation, with an AUC value of 0.97 (95% confidence interval = 0.92-0.97). We summarized and analyzed the clinical characteristics of pediatric monogenic lupus and developed a predictive model for early identification by clinicians. Clinicians should exercise high vigilance for monogenic lupus when the score exceeds - 9.032299.

Clinico-Laboratory Profile and Outcome of Kawasaki Disease in Children in a Tertiary Care Hospital in Kathmandu

Introduction: Kawasaki disease (KD) is one of the most common vasculitis of childhood. It contributes significantly to acquired heart disease in children and results in coronary artery abnormalities including aneurysm in 25% of untreated children. This study aims to retrospectively assess the clinical course, laboratory results, and presenting symptoms of patients with complete and incomplete KD treated at tertiary care hospital in Kathmandu.Method: This retrospective study was conducted in the Department of Pediatrics. Data from January 2020 to December 2020 were retrieved from medical records department of the hospital and analyzed using Microsoft excel.Result: The total number of cases evaluated in our study was 9. The median age at the time of the diagnosis of KD was 24 months (median age for incomplete KD-21 months, complete KD-37 months). Male to female ratio was 1.2:1. The peak season of KD occurrence was Spring (March – May). Mean duration of stay in the hospital was 7 days. All children presented with fever, with an mean fever duration of 7 days at the time of presentation. Fever was present in every case. The most common principal symptom was a polymorphous skin rash, observed in 88% of the cases. Elevated CRP levels (≥3 mg/dl) were noted in all 9 cases. Cardiovascular complications were present in 88% of the patients, with coronary artery aneurysm (CAA) detected in 11%, coronary artery dilation in 22%, and mild pericardial effusion documented in 66% of the cases. Eight patients (88%) received IVIG, all 9 patients (100%) received aspirin, and 2 patients received steroids. There were no fatalities.Conclusion: KD is a common vasculitis in children which do not have significant sex predilection in oppose to most of the studies. Majority of cases occurred in spring season. The most common symptom was polymorphous rash. A high level of suspicion for diagnosing KD is essential, and early treatment is crucial to prevent complications and reduce the future economic burden on families and the nation.

Investigation of Nursing Errors in Greek Pediatric Hospitals

Background: Adverse events are a prevalent occurrence across pediatric healthcare environments, and patient safety is intricately tied to nursing errors due to nurses’ consistent presence and interaction with patients, which surpasses that of any other healthcare professional. This research sought to explore the factors influencing errors as perceived by pediatric nurses in Greek hospital settings. Methods: Clinical pediatric nurses voluntarily and anonymously completed a specialized structured survey, utilizing the Taxonomy of Error, Root Cause Analysis, and Practice-responsibility (TERCAP) tool, which delineates the circumstances surrounding errors occurring during clinical practice. Results: Among the participants employed in the pediatric department, 80.8% (n = 84) reported experiencing an error at their workplace. Notably, in 48.7% (n = 38) of these instances, the error was attributed to themselves (personal responsibility), while in 78.9% (n = 56) of cases, it was linked to errors committed by other colleagues in the clinic. As reported by participants in pediatric departments, the primary factors contributing to potential error occurrence include the absence or inadequacy of orientation and training for new staff (43.2%), the absence of a standardized protocol for resolving disagreements (39%), insufficient ongoing training (38.3%), and breakdowns in interdisciplinary communication (21%). Conclusions: By classifying errors based on various criteria such as outcomes, processes, cognitive reasoning, ethical considerations, and importance, this study presents a holistic framework for examining pediatric nurses’ errors from diverse perspectives. Through this classification approach, the study establishes a foundation for tailored interventions targeting particular aspects of errors and their root causes in pediatric departments.

Outcome of Neonatal Registry Through Electronic Medical Registry at Neonatal Intensive Care Units, Children's Hospital of Ain Shams University

Abstract Background The neonatal mortality rate is 19 deaths per 1000 live births worldwide. Studies have shown that neonatal registry network systems can provide the necessary data for establishing evidence- based strategies to decrease the complications in high-risk neonates and consequently reduce neonatal mortality rate. Aim of the Work To determine the frequency and distribution of neonatal critical illnesses in Neonatal Intensive Care Unit (NICU) of Ain Shams University. Patients and Methods This descriptive study was conducted in NICU, Children's Hospital, Ain Shams University. The data collected from initiated neonatal registry through electronic medical record to reach sample size number (381) patients. The current study was conducted after approval by the Department of Pediatrics, and the local Ethics and Research Committee, Faculty of Medicine, Ain Shams University. Results The mean ± (SD) age at admission was 5.52 ± 6.9 days, gestational age was 36.19 ± 2.47 weeks, more than half of our patients were males (55.6%) and females represented in (44.4%). Two- thirds (64.2%) of our participants were full term, and preterm were only (35.7%). The main etiology of NICU admission was respiratory disease (35.7%), followed by neonatal sepsis (25.2%), neonatal jaundice (21%), congenital anomalies (12.3%), CNS diseases (6.8%), inborn errors of metabolism (1%) and mortality rate was 10.5%. Four cases had inborn errors of metabolism in the form of: Maple syrup urine disease (one case), organic academia (one cases) and 2 cases referred to genetic clinic for further assessment. Congenital heart diseases in our study was reported in (7.9%) of neonates. The main etiology among them was reported in following order: PDA / pulmonary hypertension (26.7%), VSD (13.3%), Fallot tetralogy (10%), transposition of great arteries (10%), supraventricular tachycardia (6.7%) then many etiologies represented in 3.3% as Ebstein anomaly, right single ventricle, heart block, pulmonary artery stenosis, arrhythmia, tricuspid atresia and left hypoplastic heart disease. Conclusion This study showed the ability of the electronic medical record to store large amounts of data which creates a potential for information overload in the intensive care (ICU) setting. This will stimulate and facilitate research on neonatal critical illness and its management, mortality and morbidity outcomes of neonates admitted to NICUs in Children's Hospital, Ain Shams University, Egypt.

A Study of Clinical Profile of Congenital Anomalies of Kidney and Urinary Tract (CAKUT) in a Tertiary Care Center.

Background Congenital anomalies of the kidney and urinary tract (CAKUT) encompass a diverse array of disorders arising from developmental irregularities in the renal parenchymal development, disrupted embryonic migration of the kidneys, and the urinary collecting system. This study aimed to investigate the clinical presentations, patterns of obstructive and non-obstructive CAKUT, and associated extrarenal manifestations in affected children. Methods This observational study was conducted in the Department of Pediatrics, Acharya Vinoba Bhave Rural Hospital, Wardha. Ethical clearance was obtained, and the study included 105 diagnosed CAKUT patients aged from birth to 18 years. Data collection spanned from June 2022 to May 2024. Clinical features, antenatal findings, associated anomalies, estimated glomerular filtration rate (eGFR), and serum creatinine levels were recorded. Descriptive and inferential statistical analyses were performed using Stata software. Results Among the 105 participants, 81 (77.14%) were males, with a male-to-female ratio of 3.37:1. The mean age was 42.49 months. Forty-two individuals (40%) were asymptomatic, while the most common symptomatic presentation was the ventral opening of the urethra (24.76%). Extrarenal malformations were present in 35 subjects (33.33%), with undescended testis (25.71%) and congenital heart disease (CHD) (20%) being the most common. The antenatal diagnosis was made in 63.8% of cases. Obstructive uropathy was present in 42.86% of subjects, with a significant association between antenatal diagnosis and bilateral hydronephrosis. Medical management was provided to 41.9% of subjects, while 58.1% underwent surgical interventions. Conclusion The study highlights the clinical variability and diverse presentations of CAKUT in children, with a substantial proportion being asymptomatic. Early detection through antenatal screening and prompt intervention can potentially prevent or delay the progression to ESRD. The findings underscore the importance of comprehensive evaluation and targeted management strategies to address both renal and extrarenal manifestations of CAKUT.

Evaluating Pediatric Appendicitis Score and Alvarado Scoring System for Detecting Acute Appendicitis in Pediatric Population

Abstract Background Acute appendicitis is the most common surgical condition of children. About 1 to 10% of children presenting in surgical emergencies with acute abdomen fall within the domain of acute appendicitis. In few of these cases, clinical signs and symptoms at presentation are such that acute appendicitis is a clear diagnosis while more often it is challenging for a surgeon to make a clinical diagnosis of acute appendicitis. In these circumstances, explorations reveal negative appendectomies and an organ with potential functions can be lost. Objective To evaluate the Pediatric Appendicitis Score and Alvarado Scoring System in Detecting Acute Appendicitis in Pediatric Population in terms of applicability, safety, and specificity in the diagnosis of Acute Appendicitis among the study group and to suggest improvements and/or modifications to increase the predictive values of Pediatric Appendicitis Score and Alvarado Scoring System, decrease the rates of false diagnosis or reduce costs. Methods a prospective cohort study that was conducted on 50 patients aged under 14 years admitted to pediatric surgery department in Ain Shams University Hospital within a period of 6 months with an expected diagnosis of Acute appendicitis. Each patient was subjected to a detailed medical history taking (with emphasis on The age, gender, duration of pain, complaint complaints ofabdominal pain and duration, fever, nausea-vomiting, diarrhea or constipation), Clinical examination:(right lower quadrant sensitivity,defense, rebound tenderness, percussion/ cough/ right lower quadrant sensitivity with jumping). Laboratory investigations:(complete blood count, the total white blood cell (WBC) and total neutrophil counts c-reactive protein ) and Ultrasound examination. Alvarado score as well as PAS were calculated for each patient . the results were then compared to the intra operative findings confirming or excluding the diagnosis of acute appendicitis. Results in the study group that was 38% males and 62% females with ages ranging from 3 to 14 years .Pediatric appendicitis score (PAS) was found to significantly predict acute appendicitis in the studied patients at cutoff 5.0 with sensitivity, specificity, PPV and NPV was 60.5%, 100%, 100% and 71.9% respectively (p < 0.001). Alvarado score can significantly predict acute appendicitis in the studied patients at cutoff 4.0 with sensitivity, specificity, PPV and NPV was 79.1%, 85.7%, 84.7% and 80.4% respectively (p < 0.001). Conclusion The results of this study showed the higher sensitivity of Alvarado score and more specificity of the Pediatric Appendicitis score. However, the sensitivity, specificity, PPV, and NPV of these criteria were not considered enough to confirm the diagnosis of acute appendicitis alone. There is a need for new parameters in the scoring systems. Such as including ultrasonagraphic findings and additional laboratory investigations such as CRP.

Insulin Resistance in Pediatric Migraine

Abstract Background Migraine is a common complaint in the pediatric population with a prevalence ranging from 3% to 10%. Several studies among adult population defined a link between migraine and insulin resistance (IR), however data among pediatric population is scarce, thus the aim of the current study is to assess the relation between IR and migraine among pediatric patients with migraine. Patients and Methods A case control study that was conducted on children aged between 4 and 16 years; 30 were patients with migraine who were recruited from the pediatric neurology department and 40 were healthy. The diagnosis of migraine was according to the criteria of the International Classification of Headache Disorders (ICHD) -II criteria. Anthropometric measurements of all patients were assessed. Insulin resistance was assessed by Homeostatic Model Assessment for Insulin Resistance (HOMA-IR) and Quantitative Insulin Sensitivity Check Index (QUICKI equation). Results The mean age of patients with migraine was 10.03 ± 2.16 years with male predominance. Most of the patients (80%) were pre-pubertal. Headache with aura was present in 13 (43.3%), family history was present in 24 (80%), bilateral headache was the most common among our patients (71.4%). There was significant increase in BMI among migraine group (P = 0.03). 50% of migraine patients were overweight, 20% were obese. Incidence of insulin resistance by HOMA-IR and by QUICKI was significantly higher among patients with migraine (P = 0.007 and 0.033) respectively. Conclusion based on our results, it can be concluded that migraine is associated with insulin resistance in 36.7% of patients.

Use of a new vertical traction device for early traction-assisted staged closure of congenital abdominal wall defects: a prospective series of 16 patients

PurposeAbdominal wall closure in patients with giant omphalocele (GOC) and complicated gastroschisis (GS) remains to be a surgical challenge. To facilitate an early complete abdominal wall closure, we investigated the combination of a staged closure technique with continuous traction to the abdominal wall using a newly designed vertical traction device for newborns.MethodsFour tertiary pediatric surgery departments participated in the study between 04/2022 and 11/2023. In case primary organ reduction and abdominal wall closure were not amenable, patients underwent a traction-assisted abdominal wall closure applying fasciotens®Pediatric. Outcome parameters were time to closure, surgical complications, infections, and hernia formation.ResultsTen patients with GOC and 6 patients with GS were included. Complete fascial closure was achieved after a median time of 7 days (range 4–22) in GOC and 5 days (range 4–11) in GS. There were two cases of tear-outs of traction sutures and one skin suture line dehiscence after fascial closure. No surgical site infection or signs of abdominal compartment syndrome were seen. No ventral or umbilical hernia occurred after a median follow-up of 12 months (range 4–22).ConclusionTraction-assisted staged closure using fasciotens®Pediatric enabled an early tension-less fascial closure in GOC and GS in the newborn period.

Comparative Study on the Effect of Different Modes of Administration of Ambroxol Hydrochloride in the Treatment of Pediatric Respiratory Diseases

Objective: To investigate the comparison of the effects of intravenous infusion of Ambroxol Hydrochloride and nebulized inhalation in the treatment of pediatric respiratory diseases. Methods: 188 children with respiratory diseases admitted to the pediatrics department of the hospital from September 2022 to March 2024 were selected as the study subjects, and were randomly divided into the control group (n = 41) and the observation group (n = 41) according to the random number table method. Both groups of patients were first treated with conventional treatment, and based on conventional treatment, the control group was treated with intravenous infusion of Ambroxol Hydrochloride, and the observation group was treated with nebulized inhalation of Ambroxol Hydrochloride. The general information, clinical symptom improvement time, oxygen therapy time, sputum aspiration frequency, hospitalization time, and the occurrence of adverse reactions were observed in the two groups. Results: The baseline data of the two groups of patients were not statistically significant (P > 0.05); the disappearance time of clinical symptoms such as cough, lung rales, and shortness of breath in the observation group was shorter than that in the control group (t = 8.739, t = 10.108, t = 19.448, P < 0.001); the time of oxygen therapy, number of sputum aspirations, and hospital stay of the patients in the observation group were significantly shorter than that of the control group (t = 10.593, t = 10.665, t = 14.673, all P < 0.001); the total incidence of adverse reactions in patients in the observation group (3/3.18%) was significantly lower than that in the control group (14/14.89%) (x2 = 7.825, P = 0.005 < 0.01). Conclusion: In the treatment of pediatric respiratory diseases with Ambroxol Hydrochloride, nebulized inhalation has better efficacy than intravenous drip, and can better improve the clinical symptoms and reduce the side effects in children.

Clinical and laboratory profile, disease activity and outcome of childhood systemic lupus erythematosus - A retrospective study in a tertiary care hospital in Assam

Background: Systemic lupus erythematosus in children (cSLE) represents 10-20% of all cases of SLE. However, this is still underreported from Assam. In this study, we report our experience with 25 patients of cSLE over the last 3.5 years. Materials and methods: This retrospective study was carried out at the Department of Pediatrics, Gauhati Medical College and Hospital, Guwahati, Assam. Data of patients with cSLE in the records of pediatric rheumatology and immunodeficiency clinic from April 2018 to October 2021 were collected and analysed. Results: In our cohort, the median age at presentation was 10 years (range, 4.6-14 years). The female-to-male ratio was 7:1. Hematological involvement, 21 (84%) was the most common manifestation followed by cutaneous, 19 (76%), and renal involvement, 17 (68%). Five patients also had associated HbE hemoglobinopathy. We had performed renal biopsy in 12/17 patients. Class IV lupus nephritis was the most common type in our patients. Twenty-four out of 25 patients had active disease at the time of the first presentation. There were four deaths in our cohort including two deaths that occurred due to congestive cardiac failure in patients with suspected myocarditis. Seventeen patients in our cohort had completed a median of 10 months (range, 2 – 30 months) follow-up and 6/9 biopsy-proven proliferative nephritis were in remission after a median of 9.5 months (range, 2-27 months) follow-up. Conclusion: Hematological involvement was the commonest manifestation in cSLE at our center. The majority of patients with proliferative LN were in remission with standard treatment. Keywords: hemoglobin E; lupus nephritis; myocarditis; vasculitis

Related factors of acute symptomatic seizures and epilepsy in children with myelin oligodendrocyte glycoprotein antibody-associated disease

Objective: To investigate the risk factors of acute symptomatic seizures (ASS) and epilepsy in children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods: A ambispective cohort study was used including 74 children with MOGAD who were admitted to the Department of Pediatrics of Peking University First Hospital from January 2013 to June 2023 and were followed up. Demographic information, clinical information, treatment status, ASS and epilepsy status were collected. The clinical phenotypes were classified. According to the presence or absence of ASS in the course of disease, the children and the course of disease were divided into groups with and without ASS. Chi-square test, Fisher exact test and Mann Whitney U test were used to analyze the correlation between symptoms and auxiliary examination characteristics and the occurrence of ASS in the two groups of children. Multivariate Logistic regression analysis was used for multivariate analysis. Results: The onset age of the 74 children with MOGAD was 6.58 (3.80, 9.67) years, including 38 females (51.4%) and 36 males (48.6%). The duration of the final follow-up was 2.67 (1.10, 4.12) years, with a total of 239 times acute clinical episodes. ASS occurred in 39.2% (29/74) children during the course of disease and in 29.3% (70/239) of attacks. The common phenotypes were ADEM (67 times (28.0%)), optic neuritis (37 times (15.4%)) and cerebral cortical encephalitis (31 times (13.0%)) in 239 times acute clinical episodes. The incidence of ASS in ADEM and cerebral cortical encephalitis phenotype was 28.4%(19/67) and 100.0% (31/31), respectively. Multivariate analysis showed that cortical involvement on magnetic resonance imaging during clinical attacks was an independent risk factor for ASS (β=-1.49, OR=0.23) after excluding attacks involving only optic nerve or spinal cord (49 episodes). During the follow-up, 5 children (6.8%) had epilepsy, and all children with epilepsy had multiple clinical attacks of MOGAD and previous ASS. Conclusions: Cortical involvement on magnetic resonance imaging during clinical episodes is an independent risk factor for ASS in children with MOGAD. All MOGAD children with epilepsy had ASS and multiple MOGAD clinical episodes in the past.

Therapeutic effectiveness of modified endoscopic retrograde appendicitis therapy for acute appendicitis of different severities in children

Objective: To investigate the effects of modified endoscopic retrograde appendicitis therapy (mERAT) on the treatment of children with different severities of acute appendicitis. Methods: This study was a case-control study. A total of 586 children with acute appendicitis, who were admitted to the Pediatric Department of Second Affiliated Hospital of Air Force Medical University between January 2019 and November 2023, were selected as the research subjects. According to the severity of the disease, the patients were divided into simple appendicitis group, suppurative appendicitis group and perforated appendicitis group. The baseline data, hospitalization treatment and costs, outcomes, and recurrence in each group were analyzed, and the difference in the effectiveness of mERAT between the groups were compared by Kruskal-Wallis H test and χ2 test. Results: Among 586 children, there were 338 males and 248 females. The age at onset was 7.0 (4.6, 9.4) years. There were 475 cases of simple appendicitis, 78 cases of suppurative appendicitis, and 33 cases of perforated appendicitis. There were no significant differences in age and gender among the three groups (F=0.59, χ2=3.31, both P>0.05). However, there were statistically significant differences in body temperature, white blood cell counts, neutrophil percentage, lymphocyte percentage, nausea or vomiting, right lower abdominal pain, umbilical pain, right lower abdominal tenderness, and right lower abdominal rebound pain (H=7.56, 161.52, 169.11, and 169.61, χ2=12.05, 13.82, 12.05, 7.74, 20.35, and 94.61, all P<0.05). Also, the treatment time, postoperative hospital stay, total hospital stay, and cost showed statistically significant differences (H=4.70, 33.66, 34.99, 30.37, all P<0.05). There was no significant difference in the initial treatment success rate (98.1% (466/475) vs. 98.7% (77/78) vs. 90.9% (30/33), P=0.057). During the 30 (23, 36) months of follow-up, the recurrence rate was 7.9% (35/433) in the simple appendicitis group, 20.8% (15/72) in the suppurative appendicitis group, and 30.0% (9/30) in the perforated appendicitis group, with a statistically significant difference (χ2=23.56, P<0.001). Among the children with recurrent appendicitis, 15 cases still chose mERAT, of them 11 cases (31.2%) had simple appendicitis, 2 cases (2/15) had suppurative appendicitis, and 2 cases (2/9) had perforated appendicitis.The latest time to recurrence in the 3 groups was 32, 35 and 10 months, respectively. Conclusion: Treatment with mERAT has a good effect in pediatric simple appendicitis, but has a higher recurrence rate despite a better initial treatment success rate in suppurative appendicitis and perforated appendicitis.

Clinical Profile of Hospitalized Varicella Cases From Pediatric Emergency Consultations: A Retrospective Cohort Study.

Varicella is a very common childhood infectious disease. It is generally benign, but it can lead to fatal complications. Our study aimed to describe theclinical and therapeutic profile of varicella based on consultations in the pediatric emergency department, to determine the incidence of hospitalized varicellacasesin the pediatric department for complementary management,and to specify the incidence of varicella complications in hospitalized patients. We conducted a retrospective descriptive cohort study over 12 months. It took place in the pediatrics and pediatric emergency departments of the Mother-Child Hospital of the Mohammed VI University Hospital, Mohammed I University, in Oujda, Morocco. We collected 120 cases of varicella. The mean age of patients was 4.5 years. The most common age range was 4-6 years (69%). Males predominated. The reason for consulting the pediatric emergency department wasa febrile rash in 65% of cases. Treatment in pediatric emergencies was mostly symptomatic. Antibiotic treatment for superinfection of lesions was used in 11% of cases. The number of hospitalizations due to complicated and/or severe varicella was 17 cases. The median age was 6.3 years. Most of the children (82%) were immunodeficient and 18% were immunocompetent.Sixteen patients had underlying risk factors. Infectious skin and soft tissue complications were noted in most hospitalized patients (47%). They were mainly presented by cutaneous reinfections with alteration of general health (41%). Neurological complications ranked second (23%). The majority were febrile convulsions (17%). One case of bronchopulmonary complication was noted. No hematological, digestive, renal, or cardiac complications were noted. Intravenous antiviral treatment was used in 88% of hospitalized cases. The drug of choice was acyclovir. Antibiotic therapy was used in 53% of cases. No patient received corticosteroid therapy.The median length of hospitalization for our patients was 14 days. The evolution was favorable in 100% of cases. Varicella remains a benign disease in children, rarely leading to hospitalization. However, complications may develop in cases of comorbidity or children with risk factors. The introduction of the varicella vaccine into the national immunization program could considerably reduce the number of children hospitalized in the near future.

Clinical spectrum of acute flaccid paralysis among pediatric patients at the National Institute of Child Health, Karachi, Pakistan.

Objective: To evaluate the clinical spectrum and immediate outcomes of acute flaccid paralysis (AFP) in children presenting at National Institute of Child Health, Karachi, Pakistan. Study Design: Cross-sectional study. Setting: Department of Pediatrics, NICH, Karachi, Pakistan. Period: August 2023 to January 2024. Methods: A total of 121 children of either gender, aged 1-15 years, and presenting with AFP were analyzed. AFP was diagnosed by nerve conduction study. Causes of AFP, like Guillian Barre Syndrome (GBS), transverse myelitis, traumatic neuritis were noted. Outcomes were noted in terms of discharged from hospital, left against medical advice, and mortality. Results: In a total of 121 children, 75 (62.0%) were boys. The mean age was 6.00±2.88 years, ranging between 1-12 years. There were 74 (61.2%) children who were fully vaccinated as per age. The most common cause of AFP were GBS, transverse myelitis, and hypokalemic paralysis, noted in 49 (40.5%), 19 (15.7%), and 15 (12.4%) children, respectively. Ninety (74.4%) children were discharged after the treatment, 10 (8.3%) left against medical advice, whereas mortality was noted in 21 (17.4%) children. Children leaving against medical advice were left out from the analysis to compared final outcomes with respect to various study variables. Incomplete vaccination status (p=0.0006), and presentation with sensory loss (p=0.0003) were found to have significant association with mortality. Conclusion: Guillian Barre Syndrome was found to be the most common cause behind acute flaccid paralysis in children. Incomplete vaccination history, and presenting with sensory loss were associated with poor outcomes.

Clinical Profile, Laboratory Characteristics and Treatment of Wilson’s Disease in Children from Western India

To study the clinical profile and laboratory characteristics and treatment of children with Wilson’s disease (WD). The current study was done at Department of pediatrics, Sir Padampat Institute of Neonatology and Pediatric Health, Sawai Man Singh Medical College, Jaipur. It was an observational study and institution ethics committee approved the study. Patients visiting the outpatient department or admitting in wards with clinical presentation suggestive of WD were enrolled in the study after obtaining a valid informed written consent. Patients subjected to detailed clinical history and physical examination. All patients subjected to routine blood count, biochemistry including liver function tests and specific laboratory investigations. They underwent ophthalmological examination. Ultrasonography abdomen and liver biopsy performed in enrolled patients. Magnetic resonance imaging brain carried out in patients with neurological WD. Ferenci score was calculated for each of the patients. Total 50 patients were included in the study. Mean age at the time of diagnosis was 9.4 years with delay of 11 months after onset of symptoms. Male is to female ratio was 2/1. Hepatic manifestation were seen in 76% patients and 24% patients presented with neurological disease. Kayser-Fleischer ring was seen in 44% patients with hepatic disease and 83% patients with neurological disease. Twenty-four hour urinary copper was more than 2 time of upper limit of normal in all patients. Fifty-four percent patients showed improvement with chelation therapy and 9 patients died during the study period. WD in children has varied clinical manifestation and early diagnosis is necessary for good prognosis. It requires wide range of tests as genetic testing is not easily available. Acute liver failure has high mortality. Early chelation therapy reverses the clinical and biochemical abnormalities.

Lifestyle Practices of Adolescents in a Tertiary Care Centre in India

Introduction: Adolescents are individuals in the 10–19year age group; there are 253 million adolescents in India. Growth and development are predominant during adolescence, a relatively healthy time. Good diet, physical activity, sleep, and screen time are pillars of good health. Aims &amp; Objectives: To identify lifestyle practices such as diet, physical activity, sleep, and screen time among adolescents in the outpatient and inpatient services, Departments of Paediatrics and Paediatric Surgery, Christian Medical College, Vellore. Methods: A descriptive study design employing a non-probability purposive sampling technique was used to recruit 1420 adolescents. Written consent and assent were obtained, demographic and clinical variables were collected using interviews, and the lifestyle practice was assessed using a modified YRBS questionnaire. Results: 70.8% of the adolescents were in the age group of 10-14 years; males and females were almost equal (46.2 and 53.8%), 73.6% lived in nuclear families, and 19.4% had a BMI of 23 adult equivalent. Regarding diet in the past 7 days, 41.3% did not consume green/vegetable salads, and 89.7% of the adolescents ate breakfast daily, but the meals of 81.4% did not contain 25% carbohydrates. 42.2% did not meet the recommended 60 minutes of daily physical activity, and 51% were not a part of any sports team in the past year. Most (33.8%) of the adolescents slept for about 8 hours during the school days, but 68% reported difficulty falling asleep. Most participants (58.7% and 59.2%) stopped screentime just before sleep on weekdays and weekends. Conclusion: Adolescence is when lifestyle behaviours that affect present and future health begin. Therefore, helping adolescents establish healthy lifestyles and avoid developing health risk behaviours is crucial and should be started before these behaviours are firmly established. Keywords: Adolescents, Lifestyle, Diet, Physical Activity, Sleep, Screen time.

Efficacy of 10% Dextrose versus Expressed Breast Milk in Relieving Procedural Pain in Neonates – A Randomized Controlled Trial

Aim: This study aims to compare the efficacy of 10% dextrose (10%D) and expressed breast milk (EBM) in relieving heel prick pain in neonates assessed by premature infant pain profile-revised (PIPP-R). Settings and Design: This study was done in the neonatal intensive care unit under the department of pediatrics, hospital-based prospective, randomized controlled trial. Subjects and Methods: The study includes neonates born between ≥34 weeks and ≤42 weeks of gestational age. The 72 enrolled neonates were randomized into two intervention groups (EBM and 10%D). One milliliter of test solution was given to the baby 30 s before the heel prick. Pain reactions were scored using the PIPP-R scoring system, and two independent observers were chosen, who were blinded to what intervention was given. Results: For the EBM solution, the mean PIPP-R at 0 s was 7.19 ± 2, 5.5 ± 1.5 at 30 s, and 4.28 ± 1.65 at 60 s. For the 10%D solution, the mean PIPP-R at 0 s was 4.97 ± 1.42, 2.36 ± 1.44 at 30 s, and 1.69 ± 1.53 at 60 s. The difference in the mean PIPP-R between the two groups was statistically significant, and the 10%D group majority had only minimal pain throughout the procedure. Conclusions: Compared to the EBM group, 10%D significantly lowered the mean PIPP-R score, giving good pain relief and less procedural discomfort.

Ewings sarcoma in pediatric population of North India: Demographics, outcome & prognostic factors

BackgroundEwings sarcoma is relatively rare and accounts for a 3–4% of childhood cancer. The data of Ewings sarcoma is scarce in North India and so the study was planned to find out the demographics, outcome and prognostic factors of Ewings sarcoma. MethodsThis is a retrospective study conducted in the Department of Pediatrics, Pediatric Oncology unit at a tertiary care centre (KGMC, Lucknow) in North India. All patients of age 1–16 years with biopsy and IHC proven Ewings sarcoma during 2015–2020 were included in the study. Parameters were noted and follow up was done till December 2021. Statistical analysis was done by Microsoft Excel and GraphPad Prism 10. Overall survival was assessed using Kaplan Meir Survival Analysis. P value < 0.05 was considered significant. Results75 patients i.e.4.1 % of the total were diagnosed with ES. Median age of patients was 10 years. 37.35 % had metastasis at presentation. 32 % were defaulters (abandoned and not taking treatment). Extremity ES constituted 46.7 % of the patient. 29.4 % patients relapsed. The overall survival of the whole cohort was 23.5 % and OS of localised disease was 42.3 %. OS of patients with respect to various sites failed to show any significance. Staging of disease have shown to be affecting outcome. ConclusionProportion of Ewings sarcoma was in accordance with the world's data but the survival outcome was low at our centre primarily due to delayed presentation translating to metastatic disease at diagnosis and abandonment issues along with defaults in treatment. Conducting focussed group discussions, awareness sessions, caregivers counselling and educating them about disease might help in curbing these factors.

Correlation between Serum Ferritin and Thyroid Hormones Level in Children with Thalassemia

Background: Thalassemia is a group of hereditary blood disorder characterized by reduced or absent globin chain synthesis resulting in transfusion dependent anaemia and iron overload. Due to regular blood transfusion and inadequate chelation therapy patient develop multiorgan dysfunction. Objectives: To find any correlation between serum ferritin and thyroid hormones level in children with transfusion dependent thalassemia. Methods: This cross-sectional study was carried out in the department of Paediatrics and department of Medicine, Sylhet MAG Osmani Medical College Hospital between June, 2021 to May, 2022, on one hundred children with transfusion dependent thalassemia who were on more than 12 times blood transfusion but did not take any chelation therapy and whose serum ferritin level were &gt; 1000 ng/ml. Assessment of their thyroid hormones level and statistically its correlation with serum ferritin were done. Result: A total of 53 (53%) male and 47 (47%) female were included in this study. The mean age of the patients was 9.47 ±3.63 years. Mean serum ferritin was 1980±1126.19 ng/ml, mean serum TSH was 3.55±2.08 µIU/ml, mean serum free T4 was 1.10±0.18 ng/ml, mean serum free T3 was 3.64±0.37 pg/ml. Subclinical hypothyroidism and euthyroid were found in 20 patients (20%) and 80 patients (80%) respectively. No overt hypothyroidism or hyperthyroidism were found. Mean serum ferritin in subclinical hypothyroid and euthyroid were 2407±995.37 ng/ml and 1875.59±1131.18 ng/ml respectively which difference was statistically significant (P value &lt;0.05). Scattered diagram was done and it showed linear correlation between serum ferritin and serum TSH of subclinical hypothyroid group. No linear correlation was found between serum ferritin and F.T3 and F.T4 on scattered diagram. Positive (Pearson) correlation was found between serum ferritin and serum TSH of subclinical hypothyroid group and strong positive (Pearson) correlation (r=0.74, P=0.02) was found between them. Conclusion: This study showed that good percentages of children with transfusion dependent thalassemia are suffering from subclinical hypothyroidism. There is positive correlation between serum ferritin level and subclinical hypothyroidism.

Prevalence of Iron Deficiency Anemia and Iron Deficiency Without Anemia Among Moderate and Severely Acute Malnourished Children.

Iron deficiency remains one of the globally recognized leading causes of morbidity and mortality in children, among developing countries like India as compared to the Western world. To estimate the prevalence of iron deficiency anemia (IDA) and iron deficiency without anemia (IDWA) among malnourished children. This cross-sectional study was conducted in the Department Of Pediatrics, Tertiary Care Hospital, Western Rajasthan. Demographic data and serum samples were collected and analyzed. Hematological and biochemical values were determined for 300 children aged 6 months to 59 months. A total of 93.9% of severe acute malnutrition (SAM) children and 83.24% of moderate acute malnutrition (MAM) children had anemia as perWHO definition, with moderate anemia (47.66%) being the predominant type of anemia. About 64% of children showed iron deficiency with a prevalence of IDA and IDWA being 94.27% and 5.72%, respectively. The mean values of serum ferritin, serum iron, serum total iron binding capacity (TIBC), and transferrin saturation in children with IDWA were 8.34±2.85, 17.43±7.57, 454.09±40.76, and 4.09±1.44, respectively. The proportion of anemic children in both SAM and MAM groups was very high. Our study shows that more than 60% of the MAM and SAM children were iron deficient. We recommend future measures for the prevention and control of anemia, including increased coverage of nutritional supplementation, fortification programs, and supplement iron in this sub-group (IDWA) to take care of their symptoms due to iron deficiency even before the development of overt IDA.