Splenic Dysfunction in Children With Sickle Cell Disease: A Single Centre Experience From Central India.

OBJECTIVE: To assess the incidence and predictors of splenic dysfunction in children with sickle cell disease (SCD). METHODS: A cross-sectional study was conducted between June 2019 and December 2020 where children aged 1 to 15 years of age with SCD were screened for splenic dysfunction. Children who were splenectomised, those with other diseases known to affect splenic function like congenital malformations, immunodeficiencies, and chronic diseases like tuberculosis, nephrotic syndrome, diabetes mellitus, chronic liver disease, celiac disease or malignancy were excluded. Splenic size was assessed by clinical examination and ultrasonography. Splenic dysfunction was assessed using splenic scintigraphy using Technetium-99m (99mTc) labeled autologous RBCs and by the presence of Howell Jolly bodies in the peripheral smear. Laboratory and clinical predictors of splenic dysfunction were assessed by multiple logistic regression. RESULTS: We evaluated 66 children with SCD with a mean (SD) age of 7.41 (3.3) years. Impaired and absent splenic function as assessed by 99mTc scintigraphy was found in 13 (19.7%), and 3 (4.6%) children, respectively. Howell Jolly bodies in peripheral smear were found in 5 (7.5%) children; 3 of them had abnormal uptake on scintigraphy; all five had splenomegaly. Age > 5 years, > 4 episodes of vaso-occlusive crisis (VOC), > 3 hospitalization events in the past, > 5 blood transfusions, children not receiving hydroxyurea, reticulocyte count > 4%, and HbS > 70% were independent predictors of splenic dysfunction. CONCLUSION: The prevalence of splenic dysfunction in children with SCD in Central India is lower than that reported from the West. The decision to start antibiotic prophylaxis can be individualized in these children.

Profile of Acute Kidney Injury in the Pediatric Age Group in a Tertiary Care Hospital: A Prospective Observational Study.

BACKGROUND AND OBJECTIVES: Acute kidney injury (AKI) is a menace in the pediatric intensive care unit (PICU) and is responsible for significant morbidity and mortality all over the world. There are limited data available on pediatric AKI in central India. Our primary objective is to determine the clinical, etiological, and outcome profile of AKI in the pediatric age group of 3 months to 15 years admitted to the All India Institute of Medical Sciences (AIIMS), Raipur. The secondary objective(s) is to predict the association of mortality in children diagnosed with AKI and to estimate the number of patients developing chronic kidney disease (CKD) at three-month follow-up. MATERIALS AND METHODS: This observational study was conducted in the Department of Pediatrics at AIIMS Raipur, Chhattisgarh, from September 2021 to February 2023. All patients aged 3 months to 15 years of age satisfying the Kidney Disease: Improving Global Outcomes (KDIGO) criteria for AKI and presenting to the hospital were included, and those refusing consent or having CKD stage ≥3 were excluded. A total of 66 children were assessed for eligibility. Out of these 66 patients, 2 were excluded as they had AKI on CKD, and a total of 64 patients were included. For all included patients, details of their demography, clinical features, etiology workup, and hospital stay were collected. Their outcome was observed and categorized into complete response, partial response, no response, left against medical advice (LAMA), or death. Patients who were discharged were followed up for three months and observed for the recovery or development of CKD. RESULTS: The incidence of AKI in the PICU was 15.48% (64/413). Ventricular septal defect with pneumonia and pneumonia (12.5%, 8/64 each) were the most common diagnoses at presentation, resulting in AKI. The most common clinical presentations were fever (54.7%, 35/64) and respiratory distress (43.8%, 28/64). Out of them, 73.4% (47/64) had sepsis, and 62.5% (40/64) had shock. About 56.2% (36/64) of children had non-oliguric AKI as compared to 43.8% (28/64) who had oliguric AKI. Among total children with AKI, 54.7% (35/64) of patients had prerenal AKI, 43.8% (28/64) had renal AKI, and 1.6% (1/64) had postrenal AKI. Of all the children included, 32.8% (21/64) experienced complete resolution of AKI, while 18.8% (12/64) showed partial resolution, and 1.6% (1/64) remained unresolved. Among them, 3.1% (2/64) LAMA, and 43.8% (28/64) died. The median duration of the hospital stay in our study was 16.5 days. Out of them, 59.4% (38/64) of patients required renal replacement therapy (60.5% required peritoneal dialysis (PD), 36.8% required hemodialysis (HD), and 2.6% required both). Among survivors, 19.35% (6/31) developed CKD on a three-month follow-up. CONCLUSION: The incidence of AKI was seen in critically ill children in the PICU, and it was associated with high mortality.

Knowledge, Awareness, and Understanding of Pediatric Triage Among Nursing Officers in India: A Multicenter Study.

INTRODUCTION:  Triage is crucial in patient screening within emergency departments (EDs) worldwide. It is one of the essential and standard medical practices in many developed countries. However, in India, there is a need for improvement in triage utilization, as it is predominantly performed by resident doctors or medical officers, leading to an uneven distribution of clinical skills among healthcare providers (HCPs). A comprehensive analysis incorporating literature review and data collection revealed that while mandatory screening is conducted in most Indian EDs, the formal implementation of standardized triage protocols remains limited. Like in developed countries, registered nurses or nursing officers (NOs) can be effectively trained and directed to play the role of dedicated triage personnel in EDs of most of the healthcare facilities in India. METHOD AND MATERIALS:  This study aimed to examine the current state of triage utilization and its impact on the distribution of responsibilities among HCPs in Indian EDs. Through this online survey, the investigators assessed the knowledge and practical understanding of clinical triaging among NOs, working at various hospitals nationwide. RESULTS:  The participants included 5,029 NOs working in various parts of India, predominantly nursing graduates (82.52%), the majority being employed in government healthcare settings (84.01%) and most having over five years of cumulative working experience in the ED (70.77%). Nurses showed inadequate knowledge and awareness about the Pediatric Assessment Triangle (PAT) used for quick initial evaluation (62.18% among all participants). Concerning the complete triage process applicable, especially in pediatric ED settings, they had even less satisfactory knowledge and understanding, e.g., identifying primary (28.27%) and secondary (22.69%) survey components via focused history and examination, properly using temperature assessment (23.32%) and instant blood glucose level assessment (22.95%) in triage, and knowing various types of internationally accepted triage systems for ED-based health facilities such as the Emergency Severity Index (ESI), Canadian Triage and Acuity Scale (CTAS), and Australasian Triage Scale (ATS) (15.87%). ANOVA and post hoc analysis revealed that the intergroup performance of the study participants with maximum correct responses to the knowledge-determining specified subset of the questionnaire depicts the significantly higher role of graduate nursing degree over diploma such as General Nursing and Midwifery (GNM)/Auxiliary Nursing and Midwifery (ANM) qualification, working in government hospital versus private setup, and ED working experience of >5 years over that of <5 years. CONCLUSIONS:  Of the participants in the study, 50% were not evaluated for cognitive or psychomotor domains during their assessment examinations. The research illuminated a significant disparity in knowledge and proficiency levels among Indian nurses concerning pediatric triage, especially with the ability to effectively apply the PAT for initial patient evaluations, discern components of primary and secondary surveys, and comprehend various triage systems. This study underscores the importance of comprehensive reform in the Indian healthcare system and teaching curriculum by emphasizing clinical triage training and interprofessional collaboration, and establishing guidelines and regulations to ensure consistent and standardized triage practices across all EDs.

A Novel Mutation in Cystic Fibrosis Presenting as Pseudo Bartter Syndrome: A Case Report.

Pseudo-Bartter's (PB) syndrome is characterized by hypokalemic metabolic alkalosis and failure to thrive which constitutes a rare but typical presentation of cystic fibrosis (CF) in children. The most common mutation of CF is F508del, due to loss of 3 base pairs, causing deletion of phenylalanine, at position 508. We present a case of CF presenting with failure to thrive, dehydration, PB syndrome associated with urosepsis and primo-colonization with Escherichia coli suggesting the role of epigenetic factors. The heterozygous state for Phe508del mutation in Exon 11 combination with Glu92Ala in Exon 4 resulted in epigenetic effect on atypical phenotype as PBS, a novel mutation identified in our case.

"Liver Failure in an Infant of Late-Onset Glutaric Aciduria Type II": Case Report.

Glutaric aciduria type II, also known as Multiple acyl-CoA Dehydrogenase Deficiency, results from a defect in the mitochondrial electron transport chain resulting in an inability to break down fatty-acids and amino acids. There are three phenotypes- type 1 and 2 are of neonatal onset and severe form, with and without congenital anomalies, respectively, and presents with acidosis, severe hypotonia, cardiomyopathy, hepatomegaly, and non-ketotic hypoglycemia. Type 3 or late-onset Multiple acyl-CoA Dehydrogenase Deficiency usually presents in the adolescent or adult age group with phenotype ranging from mild forms of myopathy and exercise intolerance to severe forms of acute metabolic decompensation on its chronic course. Type 3 Multiple acyl-CoA Dehydrogenase Deficiency rarely presents in infancy and in liver failure. We present a five-month-old developmentally normal female child with acute encephalopathy, hypotonia, non-ketotic hypoglycemia, metabolic acidosis, and liver failure, with a history of sibling death of suspected inborn error of metabolism. The blood acyl-carnitine levels in Tandem Mass Spectrometry and urinary organic acid analysis through Gas Chromatography-Mass Spectrometry were unremarkable. The patient initially responded to riboflavin, CoQ, and supportive management but ultimately succumbed to sepsis with shock and multi-organ dysfunction. The clinical exome sequencing reported a homozygous missense variation in exon 11 of the ETFDH gene (chr4:g.158706270C > T) that resulted in the amino acid substitution of Leucine for Proline at codon 456 (p.Pro456Leu) suggestive of Glutaric aciduria type IIc (OMIM#231,680).

Trivial Blunt Chest Trauma Leading to Acute Respiratory Distress Syndrome in a Child.

Both blunt and penetrating chest trauma in children are less common than in adults but cause severe acute morbidity and mortality. As the literature suggests, pulmonary contusion is the most common chest injury in children, occurring in more than half of all blunt chest trauma cases. Even patients with blunt injuries are likely to have a longer hospital stay. The difference in physiological and anatomical variations in children compared to adults makes it more difficult from the diagnosis, management, and monitoring perspectives. A thorough physical examination is needed with close clinical monitoring, and additional vigilance is important during the management of a child. The physiologic consequences, such as the dreaded complication of alveolar hemorrhage and pulmonary parenchymal destruction, usually manifest within a few hours of the trauma and can take up to seven days to recover. Hence, timely diagnosis is crucial during the emergency evaluation. The clinical diagnosis can be supported by a special imaging modality in the form of chest computed tomography (CT), which confirms the radiological parenchymal destruction with high sensitivity. Management is mostly supportive to start with and includes high-flow oxygen, ventilatory pressure support as needed for the severity of acute lung injury (ALI) or acute respiratory distress syndrome (ARDS), judicious fluid administration, control of the pain associated with bony and thoracic soft tissue injuries, and careful hemodynamic monitoring for other complications and sequelae likely to develop. Here, we report an interesting case of a 10-year-old male child presenting to the Pediatric Emergency Department with acute moderate-to-severe respiratory distress that developed after two days of a few vomiting episodes along with non-specific lower chest and substernal pain following blunt trauma to the chest. The injury was trivial in nature as described by the father caused by an accidental fall on a small pile of bricks while playing near his home. After triaging under the red category, the child was managed in line with acute respiratory distress. We ruled out pneumothorax, hemorrhagic pleural effusion or pericardial effusion, and other evidence of invasive chest as well as gross abdominal injuries by comprehensive but focused history and clinical examinations, including adjuncts such as point-of-care ultrasound) and chest X-ray (CXR). Although the initial arterial blood gas analyses were suggestive of a mild form of ARDS or ALI by the criteria based on the P:F ratio (PaO2 to FiO2 ratio, which was between 200 and 300 for the case), the CXR and the chest CT revealed that the child had significant lung parenchymal injury in the form of bilateral fluffy pulmonary infiltrates. This case indicates that even a trivial blunt trauma can induce certain mechanisms of lung injury, leading to severe manifestations and sometimes fatal complications such as pulmonary contusion, hemorrhage, and ARDS.

Social and Demographic Factors Associated With Obese Children in the Age Group of 6-12 Years Attending a Tertiary Care Institute in Central India and the Prevalence of Depression in These Children: An Observational Study.

INTRODUCTION: Childhood obesity in India is on the rise and is rarely raised as a concern. In the central Indian states, focus is largely on undernutrition. Thus, studies related to risk factors for being overweight and obese and the impact of obesity on the psychology of children are lacking. Hence, a hospital-based study with objectives to identify social and demographic factors associated with obesity and the estimation of the prevalence of depression among these children was conducted. METHODS: This observational study was conducted in a tertiary care institute in the state of Chhattisgarh from July 2020 to October 2021. Children with obesity as per the WHO (> 95th percentile for the BMI) in the age group of 6-12 years were included after due consent. A proforma was administered targeting the objectives of the study and the Childhood Depression Rating Scale (CDR-S) and clinical evaluation identified the depressed. RESULTS: Among the 5,019 children screened during the study period, 54 met the inclusion criteria (1.07%). Fifty percent of children were from the upper middle class as per the Kuppuswamy scale. Seventy percent consumed junk food more than three times a week. Twenty-three children (42.6%) engaged in physical activity of > 1 hour and 49(90.7%) had a screen time of > 2 hours. The prevalence of depression among these children stood at 5.6% with the mean age being 11.67 years. Multiple logistic regression showed an inverse association of depression with physical activity. CONCLUSION: Obesity is prevalent in higher socioeconomic groups. Many risk factors like screen time, junk food consumption, and physical activity are modifiable. The prevalence of depression increased with age. Physical activity showed an inverse relation to depression in obese children.

Fluid Bolus: How Much More?

Fluid bolus in critically ill children is always a matter of concern and has to be balanced between benefits and harms. While optimizing pre-load is important in the golden hour period, fluid overload is a concern in ICU stay. Various dynamic parameters both clinical and device-guided assessment can help in optimizing fluid therapy. How to cite this article: Venkatesan DK, Goel AK. Fluid Bolus: How Much More? Indian J Crit Care Med 2023;27(4):296.

FOCUS more on POCUS.

How to cite this article: Venkatesan DK, Goel AK, Pratyusha, K. FOCUS more on POCUS. Indian J Crit Care Med 2023;27(3):225.

Association between anthropometric criteria and body composition among children aged 6-59 months with severe acute malnutrition: a cross-sectional assessment from India.

A multicentric study is being conducted in which children with severe acute malnutrition (SAM) aged 6-59 months are identified with only weight-for-height z-score (WHZ) < - 3 criteria. The present study aimed to assess associations of anthropometric parameters and body composition parameters, to improve treatment of SAM. We conducted a cross-section assessment using the enrolment data of children who participated in a multi-centric longitudinal controlled study from five Indian states. Fat-free mass (FFM) and fat mass (FM) were determined by bio-electrical impedance analysis (BIA). Six hundred fifty-nine children were enrolled in the study using WHZ < -3 criteria. Available data shows that WHZ, WAZ and BMIZ were significantly associated with FFMI while MUACZ was significantly associated with both FMI and FFMI. Children with both severe wasting and severe stunting had significantly lower FFMI compared to those who were only severely wasted. All forms of anthropometric deficits appear to adversely impact FFM and FM.Trial registrationThe study is registered with Clinical Trial Registration of India (Registration No.: CTRI/2020/09/028013 dated 24/09/2020).

A Novel SCN8A mutation in a case of early-onset infantile epileptic encephalopathy: A Case Report.

SCN8A gene encodes sodium channel alpha subunit Nav1.6, and its mutation is associated with Early Infantile Epileptic Encephalopathy-13 (EIEE-13). The mean age of onset is 4-5 months. The phenotype of SCN8A mutation varies from benign epilepsy syndromes, movement disorder, intellectual disability to severe epileptic syndromes with different types of seizures. We hereby report a case of a one-year old female who had an onset of infantile spasms on the seventeenth day of life, which gradually progressed to focal, multifocal, GTCS, and epileptic encephalopathy by one year of age associated with global developmental delay and hypotonia. All metabolic workup, TMS, GCMS, and MRI brain were normal. EEG at 2.5 months was suggestive of epileptic discharge arising from the left frontal region, evolving into generalized discharges. Whole exome sequencing revealed a heterozygous mutation in the SCN8A gene at exon 16 (p.Val892Ala) suggestive of Early Infantile Epileptic Encephalopathy-13 (EIEE-13). This is a novel mutation in the SCN8A gene which has not been reported previously in the literature.

Effectiveness of community-based treatment programs for treatment of uncomplicated severe acute malnourished children aged 6-59 months using locally produced nutrient dense foods: protocol for a multicentric longitudinal quasi-experimental study.

BACKGROUND: Severe acute malnutrition (SAM) is a major underlying cause of mortality among children. Around one third of the world's acutely malnourished children live in India. The WHO recommends community-based management of acute malnutrition (CMAM) for managing children with SAM. In India, different states are implementing community-based SAM treatment programme, hereinafter called CSAM, using varieties of locally produced nutrient dense food items with different nutrient compositions. The study will assess the effectiveness of these state specific CSAM interventions. METHODS: The longitudinal quasi-experimental study will be undertaken in two purposively selected blocks of one district each in the four intervention states and one comparison state. From each state, 200 SAM children identified using weight-for-length/height z-score (WHZ) < - 3 criteria will be enrolled in the study. Their anthropometric data and skinfold thickness will be taken on admission, at sixth week and at discharge by trained field investigators. Other child details, incidence of morbidity and socio-economic details will be collected on admission. To assess food consumption pattern including consumption of locally produced nutrient dense food supplements, dietary assessment, using 24-h dietary recall will be conducted on admission, at sixth week and at discharge. In addition, body composition parameters will be assessed for a sub-set of children using bio-electrical impedance analysis on admission and at discharge to analyse changes in total body water, fat-free mass, and fat mass. Post discharge, all study participants will be followed up monthly until 6 months. Atleast 10% of the sample will be checked for quality assessment. The study's primary outcome is cure rate defined as children attaining WHZ ≥ -2. Secondary outcomes include mean weight gain, mean length of stay, body composition parameters, relapse and mortality rates. Additionally, process evaluation and cost effectiveness analysis will be conducted. DISCUSSION: There is a shortage of robust evidence regarding the effectiveness of locally produced nutrient dense food supplements provided as part of the CSAM intervention in India. This study will contribute to evidence on effective strategies to manage children with uncomplicated SAM in India. The study protocol has all necessary ethical approvals. Written informed consent will be obtained from caregivers of the children. TRIAL REGISTRATION: The study is registered with Clinical Trial Registration of India (Registration No.: CTRI/2020/09/028013 ) Date of registration 24/09/2020.

Reversal of Acute Lead Encephalopathy in a Child.

Lead poisoning, fairly common in the 20th century, has decreased drastically in the last decade. Severe lead poisoning in the form of encephalopathy has a fatality rate of 28% to 45% and neurological sequelae in about 82%. We present the management of a case of lead encephalopathy that recovered without any significant neurological sequelae in a resource-limited setting. A previously healthy seven-year-old boy presented with complaints of falling unconscious on the ground while playing, followed by multiple episodes of seizures, vomiting, and altered sensorium. The patient had pallor, Glasgow coma score of E2V3M3, with features of raised intracranial pressure. Lead poisoning was suspected as the patient had four months of exposure to a battery recycling factory. Management of seizures and raised intracranial pressure was done. X-ray long bones showed lead lines at the metaphysis. Blood lead levels were highly elevated (139.96 mcg/dL). Investigations revealed iron deficiency anemia, vitamin D deficiency, and renal tubular injury in the form of proteinuria. D-penicillamine with supplements was started due to unavailability of other chelating agents. Encephalopathy improved, but patient had psychiatric symptoms of hallucinations and delusions. On the 12th day, CaNa2 EDTA was started, which resulted in significant improvement in the psychiatric symptoms. The patient had near-complete recovery in another one month, the patient being able to read, write, recite and speak as the pre-illness state. In conclusion, lead poisoning remains a significant health problem even today. Early recognition and management are of paramount importance in its outcome.

Cutaneous manifestations associated with COVID-19 in children: A systematic review.

Cutaneous manifestation of COVID 19 in children has not yet been reviewed systematically. Hence, this review gives the clinicians a future direction to be vigilant for skin presentations during pandemics. The Pubmed database used for literature search with keywords COVID 19, children, and skin in different combinations. Articles published in English with cases of age one month to 18 years were eligible. The outcome included varied aspects of cutaneous and COVID 19 infection. The authors did not register review protocol. Of 51 publications identified, 13 studies containing 149 children met the eligibility criteria. Acrally located erythematous maculopapular lesion was the most common finding in 138 children. The researcher reported Erythema multiforme, varicella like exanthem, and Kawasaki disease like presentations in the rest of the cases. The duration of the skin lesion was 1 2 weeks in 43%. Skin biopsy done in 18 patients revealed superficial and deep perivascular and peri eccrine lymphocytic infiltrate and lymphocytic vasculitis. RT PCR was positive13.8% cases. Serological markers for HSV, parvovirus B19 analyzed across various studies, were negative, except positive mycoplasma pneumonia in 2 of 20 cases tested. Clinicopathologic analysis established chilblains like lesion in 43% cases with no confirmed etiology like cold exposure, autoimmune dysfunction, drug reaction, or viral infection. The usual cephalo caudal spread of a viral exanthem was also missing. However, a low number of discussed cases was a limitation of the study. The absence of any confirmed etiology for such cutaneous manifestations, the possibility of COVID 19, should be explored and thoroughly evaluated and isolated during such a pandemic.

Sickle Cell Trait Presenting as Chronic Calcific Pancreatitis with Pseudocyst- A Case Report.

Sickle cell disease is known to cause acute pancreatitis either due to gall stones obstructing the pancreatic duct or by vaso-occlusive mechanism. However chronic pancreatitis is a very rare complication in sickle cell anemia. We report a case of sickle cell trait presenting with chronic pancreatitis with pseudo cyst. USG abdomen and CT abdomen confirmed the diagnosis of chronic calcific pancreatitis with pseudocyst. Etiological work up for other causes did not reveal anything except sickle cell trait. This case represents a rare association between chronic calcific pancreatitis and sickle cell trait.

Rotavirus Diarrhea and its Determinants Among Under-Five Children Admitted in a Tertiary Care Hospital of Southern Haryana, India.

OBJECTIVE: To estimate the prevalence of rotavirus diarrhea and its demographic, social, and clinical characteristics among children less than five years of age admitted in a rural tertiary care institute. METHODS: This prospective hospital-based observational study was carried out during February 2016 to June 2019. Diarrheal admissions of children aged 0-59 mo were screened and those who met the inclusion criteria were included in the study. Sociodemographic and clinical information was collected using a case report form. Stool samples were collected within 48 h of admission and transported in cold chain every month to the referral laboratory situated at Christian Medical College for testing. RESULTS: Among the children admitted with acute diarrhea, 148 (11.02%) were positive for rotavirus in the study. As per Vesikari scoring system, around three fourth (76.2%) of children were having severe or very severe diarrhea. Severity of diarrhea was more among rotavirus positive cases as assessed by the Vesikari scoring system. The rotavirus diarrhea showed a peak during November to February. CONCLUSION: Rotavirus diarrhea is an issue of public health importance, particularly due to its association with the severe diarrhea. As evidenced from similar settings in the world, rotavirus vaccine introduction and increased coverage is the most important strategy towards prevention and control of rotavirus diarrhea.

Nutritional Assessment and its Association with Rotavirus Positivity Among Under Five Children Admitted with Diarrhea in a Tertiary Care Hospital of Southern Haryana, India.

OBJECTIVE: To estimate the burden of undernutrition and its association with rotavirus positivity among under-five children admitted with diarrhea. METHODS: This prospective observational study was carried out in hospital-setting from February 2016 to January 2020. For all cases who met the eligibility criteria, an informed written consent was obtained from parents/caregivers. A case report form was used to collect sociodemographic, anthropometric and clinical data. The anthropometric measurements of children were performed according to World Health Organization (WHO) guidelines. RESULTS: Nutritional assessment revealed that 74.1% study subjects were underweight [Weight-for-Age (WAZ) < -2], 59% were stunted [Height-for-Age (HAZ), < -2] and 52.3% were wasted Body Mass Index (BMIZ) < -2]. Stunting was found to be significantly higher among boys as compared to girls and rotavirus positive diarrhea was significantly less prevalent among stunted children. CONCLUSION: There exists a very high prevalence of underweight, stunting, and wasting among hospitalized children with diarrhea. As the better nutrition has not been shown to guard against rotavirus diarrhea, coverage scale-up of rotavirus vaccination, improved hygiene and sanitation, and focussed nutrition programmes are the need of the hour in India.