Neonatal diabetes mellitus (NDM) is a rare monogenic disorder presenting usually within the first 6 months of life, with an estimated incidence ranging from 1 in 90,000 to 1 in 400,000 live births. We report a case of a 2-month-old male infant initially presenting with respiratory symptoms, later diagnosed with diabetic ketoacidosis. He was initially managed with intravenous insulin and subsequently transitioned to oral sulfonylurea. Genetic analysis revealed a heterozygous p.Gln1019Arg mutation in the ABCC8 gene, known to confer sulfonylurea responsiveness. The child demonstrated excellent clinical response to glibenclamide.

BACKGROUND:Sepsis continues to be a leading cause of illness and mortality in children around the world. Various scoring systems have been devised to predict the outcome of pediatric sepsis. Pediatric sequential organ failure assessment (p SOFA) and lactate clearance are the two commonly used methods. OBJECTIVE:The aim of this study was to compare the p SOFA score with lactate clearance as predictors of morbidity and mortality in pediatric sepsis, to compare the initial plasma lactate level and lactate clearance, and to know which is better to predict outcomes in sepsis and septic shock. METHODS:This prospective observational study was conducted in a pediatric intensive care unit of a tertiary care teaching hospital from July 2022 to June 2024. The blood lactate level and p SOFA score were assessed at admission and at 24 and 48 hours, and lactate clearance was calculated at 24 and 48 hours of admission. The receiver operating characteristic (ROC) curve was plotted to predict deaths using p SOFA, lactate level, and lactate clearance. RESULTS:A total of 71 children were enrolled in the study. All children were divided into two groups, 58 (82%) survivors and 13 (18%) non-survivors. The most common diagnosis was pneumonia, observed in 31 (43.6%) children. Compared to survivors, non-survivors had a higher prevalence of multiple organ dysfunction syndrome (MODS). The most common organ system involved was the cardiovascular, in 50 (70%) cases. For predicting mortality, p SOFA scores were statistically significant at admission and at 24 and 48 hours with a high area under the curve (AUC) at 48 hours (0.985). Lactate clearance at 24 hours was a better predictor of mortality than at 48 hours with a higher AUC (0.958). CONCLUSION:Both p SOFA score at 48 hours and lactate clearance at 24 hours were significant predictors of mortality. Among both parameters, lactate clearance at 24 hours was superior in predicting mortality early.

Influenza, a significant cause of seasonal morbidity and mortality, can lead to severe complications affecting multiple organ systems. This case series highlights the diverse and severe manifestations of influenza-related organ dysfunction in children such as myocarditis, acute respiratory distress syndrome, acute kidney injury, acute necrotizing encephalopathy, acute liver failure, and influenza-associated encephalopathy. This series emphasizes the critical need for early recognition and aggressive management of severe influenza cases, given the potential for rapid deterioration and multiorgan involvement. The diversity in organ dysfunction emphasizes the importance of a multidisciplinary approach in managing severe influenza, particularly in children, where outcomes can be dire despite optimal care.

The most common self-limiting viral infection of the lower respiratory tract in the pediatric population is bronchiolitis, which most commonly affects infants. In most cases, respiratory syncytial virus (RSV) is the causative agent. In general, the recovery occurs within 5–7 days without any complications. Spontaneous pneumothorax is one of the rare and less-reported complications of bronchiolitis. We report a case of a male child aged 5 months with bilateral spontaneous pneumothorax (PNO) with subcutaneous emphysema following bronchiolitis. The nasopharyngeal swab was positive for RSV, and all other investigations ruled out any underlying illness causing spontaneous PNO. Although it is a rare complication of bronchiolitis, it may occur late during the disease course. Pediatricians must be aware of this life-threatening complication, which may develop during the disease course despite an early clinical improvement.

Sepsis continues to be a major contributor to illness and death in children, necessitating effective risk assessment tools. Incidence of pediatric sepsis in intensive care units is on increasing trend. Over the years, the concept of sepsis scoring systems has evolved to enhance the prediction of outcomes and mortality in pediatric age group. To better identify sepsis and septic shock status in the pediatric age group, various sepsis scores were developed. Properly applying these scores can significantly enhance timely decision making and ultimately reduce mortality rates. Selecting appropriate score should match the settings where they were designed. Scoring system is broadly categorized into two types - prognostic scores and descriptive or outcome scores. To improve care for critically ill children, it is important to develop tools that can better predict long-term mortality and morbidity and identify factors related to intensive care related events. This review article aims to discuss the evolution of various sepsis scoring systems, highlighting their development over time and their practical utility in clinical settings.

Sir, Pleuropericardial cysts (PPCs) are rare benign anomalies of the anterior and middle mediastinum. It accounts for 5%–10% of all mediastinal tumors with an incidence of 1:100,000 population.[1] Although all ages can be affected, they have been commonly reported in middle-aged adults and rarely in children.[2] It is usually congenital and most patients are asymptomatic (75%). However, two-thirds of children are symptomatic[3] due to compression or invasion of adjacent organs dominated by respiratory signs. A 3-year-old girl was admitted with complaints of cough, fever, and coryza for 7 days. The child had a history of recurrent lower respiratory tract infections in the past year. At admission, she was febrile, tachycardic, and tachypneic, with normal blood pressure and saturation at room air. On examination, there was bilateral wheeze and crepitations, and heart sounds were muffled. Hematologic investigation revealed leukocytosis with high C-reactive protein which was suggestive of infection, but the blood culture was negative, and biochemical profiles were within the normal limits. Chest X-ray showed mediastinal widening with no cardiomegaly [Figure 1]. The child was started on antibiotics with nebulization and other supportive management. Transthoracic echocardiography was suggestive of pyopericardium with adequate biventricular function and no signs of cardiac tamponade. Contrast-enhanced computed tomography (CT) of the chest revealed a hypodense lesion of 20–40 Hounsfield unit with a well-defined margin in the left paracardiac region closely adherent to the pericardium [Figure 2a and b] which was likely a pericardial cyst. The child was operated on for cyst removal and intraoperative findings were suggestive of an 8 cm × 8 cm cyst [Figure 3] containing greenish color fluid from the aortic arch up to the apex of the heart. Pericardial fluid was exudative and the histopathology report showed a cyst lined by flattened, attenuated denuded epithelium with no features suggestive of infection. The wall of the cyst also showed dilated lymphatic spaces lined by thinned to attenuated endothelial cells along with altered fibromuscular stroma which was suggestive of a benign cystic lesion. The child was discharged after an uneventful postoperative period and is doing well after 6 months of follow-up.Figure 1: Chest X-ray showing mediastinal wideningFigure 2: (a and b) Computed tomography scan appearance of the cyst well-defined hypodense lesion in left paracardiac region closely adherent to pericardium with extensions (marked as arrow)Figure 3: Histopathology image showing cyst lined by flattened, attenuated to cuboidal denuded epithelium focally showing microvilli. The wall of the cyst was fibrous with also showed congested capillaries, dilated lymphatics, and patchy lymphoid aggregates. Magnification value: 20–40Reports of pericardial cysts in children have been scarce with <20 cases reported worldwide in children <18 years.[4] The most frequent location of PPC is the right cardiophrenic angle (51%–75%), followed by the left but in this case, the cyst was located in the left paracardiac region. Most of cysts are diagnosed by transthoracic echocardiography; however, a CT scan is considered the best modality for the diagnosis. The therapeutic approach depends on the symptomatology and size, shape, and compressive nature of the cyst. In asymptomatic patients, management is conservative but symptomatic patients with large cysts need surgical resection, percutaneous aspiration of cyst, or video-assisted thoracoscopic surgery. The recurrence rate of pericardial cysts after aspiration is about 33%. The prognosis is excellent, even spontaneous resolution of lesions has been reported.[5] This case was a diagnostic dilemma because of its unusual presentation. Clinicians should be aware of this rare entity and its unusual presentation so that the diagnosis is not missed. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Inherited causes of cavernous venous sinus thrombosis (CVST) leading to stroke in infancy are rare. Methylenetetrahydrofolate reductase (MTHFR) gene mutation is one such cause. Polymorphism of MTHFR, an essential enzyme in Vitamin B12 metabolism, leads to an increase in homocysteine levels. This is a prothrombotic state and, therefore, causes pediatric stroke. Children with a mutation of the MTHFR gene have elevated homocysteine levels. The index case of MTHFR polymorphism with CVST leading to stoke had a normal homocysteine level. The association of MTHFR A1298C mutation with CVST can be confirmed only after further studies, as the role of heterozygous A1298C mutation in prothrombotic state is conflicting.

Metronidazole-induced encephalopathy is a rare cause of toxic encephalopathy in children. Although many cases have been reported in adults, it is rarely reported in the pediatric population. Here, we report a case of an 11-year-old boy who presented with acute-onset encephalopathy with slurring of speech after receiving metronidazole for treatment of acute gastroenteritis. Neuroimaging is the cornerstone in the diagnosis of this entity with typical involvement of cerebellum, brain stem, and splenium of the corpus callosum. In our case, magnetic resonance imaging of the brain revealed hyperintensity of the splenium of the corpus callosum on the fluid-attenuated inversion recovery sequence along with diffusion restriction in the diffusion-weighted imaging and apparent diffusion coefficient images. Rapid complete neurological and radiological recovery with supportive treatment is key in making the diagnosis. Although a safer and commonly used drug, new-onset encephalopathy after the use of metronidazole must be considered.

Introduction:The number of neonatal cerebrospinal fluid (CSF) samples sent from the neonatal intensive care unit (NICU) for cytologic examination is rising, warranting accurate analysis and interpretation of the same. This study was taken up to assess the usefulness of CSF cell count and cytology in NICU settings, as it can be used even in a resource-limited setting. Aim and Objective:1) To study the prevalence of cell count and cytologic changes in CSF from NICU and assess their usefulness in correlation to C-reactive protein, CSF neutrophil percentage, blood, CSF culture, and other biochemical parameters. 2) To correlate cell counts and cytology with age, period of gestation, presence, and absence of sepsis, seizures, intracranial hemorrhage, and their clinical follow-up. Materials and Methods:A retrospective study was done on neonatal CSF samples submitted for cytology over one year (January-December 2016) in the Department of Pathology. CSF cell counts were retrieved, and cytosmears were reviewed for cellularity, cell type, proportion, and background and correlated with the biochemical, microbiological, and clinicoradiological findings. Results:A total of 213 samples were included with 140 males and 73 females with an age range of 0-28 (mean: 7.3) days. The mean CSF cell count was 5.48/cu.mm (0-90 cells/cu.mm). The most frequent cytologic finding was occasional lymphocytes or acellular CSF (63.9%). The CSF leucocyte count and protein levels showed a significant correlation with s C-reactive protein. The CSF cytology showed a significant correlation between the age of the neonate and blood neutrophil percentage (P = 0.0158). History of intracranial hemorrhage showed a significantly higher frequency of the presence of red blood cells (P = 0.0147). Conclusion:Accurate cell counts, cytology of neonatal CSF, and biochemical and microbiological workup can help diagnose and manage neonates in intensive care.

This chapter discusses about IgA Vasculitis (Henoch- Schönlein purpura) among children. The IgA vasculitis, a primary vasculitis that mostly affects small blood vessels, is diagnosed based on the presence of characteristic purpura or petechiae, abdominal discomfort, and renal abnormalities. The Ig A vasculitis is an acute multisystemic disease with signs and symptoms of purpura or petechiae, arthralgia or arthritis, and abdominal pain. Approximately 50% to 75% of children have gastrointestinal symptoms, such as colicky abdominal discomfort, vomiting, paralytic ileus, and GI bleeding, often occurring between 1-4 weeks. An incomplete presentation of IgAV, particularly if the skin symptoms are initially missing or there is an atypical presentation, the diagnosis becomes very difficult. Under these conditions, it is necessary to take into account alternative factors that may contribute to purpura, arthritis, abdominal discomfort, and renal illness. Disease recurrence can occur in patients with IgA Vasculitis even after renal transplantation. But despite recent advances in molecular understanding and healthcare facilities over the last few decades, the renal complication of IgA vasculitis remains a concern and the outcome has not changed over time.

Dermatitis as an initial manifestation of cystic fibrosis (CF) is unusual. The eruption is usually first noted in the perineum anywhere from several days to few months after birth. It subsequently spreads to the extremities and trunk. We report a 2-month-old male baby who presented with failure to thrive, hypoproteinemia, anemia, and a cutaneous eruption resembling acrodermatitis enteropathica. Oral zinc supplementation resulted in temporary resolution of the dermatitis. A further workup revealed the diagnosis of CF. The rash was responsive to nutritional and pancreatic enzyme supplementation.

Fanconi anemia (FA) is a rare genetic disorder and one of the most common inherited forms of aplastic anemia. FA is an autosomal recessive or X-linked genetic disorder that is characterized by typical physical malformations and haematopoietic anomalies. In most cases of FA, patients harbor homozygous or double heterozygous mutations in the FANCA (60–65

Snakebite is a commonly seen problem in tropical countries like India. Early morning neuroparalytic syndrome and cranial nerve palsies are the usual presentations. Locked-in syndrome (LIS) is a rare presentation. We present a 1-year and 6-month-old female toddler with acute onset of weakness in all four limbs, who developed LIS. The patient was given anti-snake venom on day 3 of admission and fully recovered after 3 weeks of ventilation. Snakebite should be suspected in any child presenting with early-onset neuroparalytic syndrome or LIS even if there is no history of snakebite or any bite mark.

Background: Anemia can have severe implications on the health of children including motor development, behavioral and cognitive development. Furthermore, morbidity from infectious disease is higher in anemic children. Nutritional anemia is a major concern in rural India. Aim and Objective: We aimed to observe the percentage of megaloblastic anemia among the anemic children and their clinical and hematological parameters. Materials and Methods: After obtaining permission from the Institutional Ethics Committee, this cross-sectional study was conducted from September 2018 to August 2021. The setting was a tertiary care hospital in the eastern part of Odisha, India. Children of 1–14 years of age presenting with anemia were included in the study. Details clinical examination and blood tests namely mean corpuscular volume, red blood cell count, total leucocyte count, and platelet count were carried out from venous blood. Descriptive statistical analyses were conducted in STATA software version 15.1. Results: Among the total 150 anemic patients, the majority (126 [84%]) were in the 11–14 years of age group followed by 24 (16%) in 6–10 years of age (P < 0.0001). Girls were more (94 [62.7%]) than boys (56 [37.3%]), P < 0.0001. Anorexia was the most frequently encountered clinical symptom (99.3%) followed by pallor (94%), weakness (86%), fatigue (62%), and hyperpigmentation (37.4%). According to hemoglobin level, 40% were suffering from severe anemia, 38.7% was having moderate, and 21.3% was having mild anemia. The majority (74%) were having both Vitamin B12 and folic acid deficiency followed by 16.7% Vitamin B12, and 9.3% folic acid deficiency. Conclusion: Girls were presenting with megaloblastic anemia more than boys. Majority of them were suffering from both Vitamin B12 and folic acid deficiency. Children suffering from megaloblastic anemia present with anorexia, pallor, weakness, and fatigue. A proper health promotion program may be designed to aware the parents about the prevention of nutritional anemia. [Natl J Physiol Pharm Pharmacol 2022; 12(9.000): 1493-1496]

Atypical hemolytic–uremic syndrome (aHUS) is a form of thrombotic microangiopathy that occurs due to dysregulation of alternate pathway of complement system, which progressively causes systemic complications, end-stage renal disease, and death. As prognosis is poor compared to typical hemolytic–uremic syndrome, early diagnosis and treatment is crucial for favorable outcome. We came across seven patients of aHUS in our pediatric intensive care unit in the last 5 years. Plasma exchange (PE) along with immunosupressives was used for treatment. First child who did not receive PE died. Rest six patients underwent PE and attained hematological remission; however, one later on progressed to chronic kidney disease and expired. All others are on regular follow-up and doing well. A high index of suspicion is required to diagnose aHUS. Early PE can give a better prognosis.

Introduction In April 2020, a group of children with hyperinflammatory shock were reported in England. Now many cases have been reported from across the world. We here report a case of Multisystem Inflammatory Syndrome in Children (MIS-C) detected in the Odisha state of India. © 2021, Sri Lanka Journal of Child Health. All Rights Reserved.

Diabetic ketoacidosis is an acute life-threatening complication of type 1 diabetes. Sometimes it is the first presentation in an undiagnosed child. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) disease (COVID-19) and diabetes mellitus are very much interrelated as diabetes mellitus is associated with an increased risk of severe COVID19 at the same time, many cases of new-onset diabetes had been diagnosed. Hyperglycemia, metabolic acidosis, and ketonemia are classical presentations. It is essential to correct the acidosis and fluid correction and insulin therapy in these patients, leading to vital organ dysfunction. In refractory metabolic acidosis, renal replacement therapy may help

Introduction: Following an asymptomatic or mildly symptomatic coronavirus disease (COVID-19), otherwise healthy children may develop serious manifestations in the form of cardiac, neurological, respiratory, gastrointestinal, and dermatologic dysfunction. Many such cases were being observed in Odisha, an eastern state of India, and have been reported from different health-care facilities. We related these unexplained serious manifestations to multisystem inflammatory syndrome associated with COVID-19 (MIS-C) and planned this study. Methods: This retrospective observational study was carried out at the following three tertiary care centers: Kalinga Institute of Medical Sciences, Bhubaneswar; MKCG Medical College, Berhampur; and Jagannath Hospital, Bhubaneswar. The study population included all children aged from 1 month to 18 years admitted to the hospitals with MIS-C according to the WHO diagnostic criteria. All the data were analyzed by SPSS software. Results: A total of 21 children were included in our study. Majority of the cases were male (76.2%), and the predominant age group was 6–10 years (47.6%). Common symptoms and signs in our observation included fever, pain abdomen, seizure, and hypotension. Most of these cases were positive for severe acute respiratory syndrome coronavirus antibody (80.95%). Response to immunotherapy was dramatic. Mortality (9%) of our study was higher than 1.8%–3% from that of Western literature. None of our patients had coronary abnormality, while two patients had mild cardiac dysfunction at discharge comparable to that of other studies. Conclusion: MIS-C following exposure to COVID-19 infection in children is a clinical syndrome, which needs early suspicion and appropriate intervention to prevent mortality.

Multi-system inflammatory syndrome in children (MIS-C) associated with COVID-19 is a recently recognised potentially life-threatening entity. There is limited data on post-MIS-C sequelae. 21 children fulfilling the WHO criteria for MIS-C were included in our study. Data were collected at baseline and at 12-16 weeks post-discharge to look for any persistent sequelae mainly relating to the lungs or heart including coronary arteries. Fever was the most common presentation, found in 18 (85.7%) patients. All had a marked hyper-inflammatory state. Low ejection fraction (EF) was found in 10 (47.6%), but none had any coronary artery abnormality. All received corticosteroids, while 7 (33.3%) children required additional treatment with intravenous Immunoglobulins. 20 children improved while 1 left against medical advice. At discharge, 3 children had impaired left ventricular function. At median 15 weeks' follow-up, no persistent complications were found. EF had returned to normal and no coronary artery abnormalities were found during repeat echocardiography. Chest radiographs showed no fibrosis and all biochemical parameters had normalized. The children with MIS-C are extremely sick during the acute stage. Timely and adequate management led to full recovery without any sequelae at a median follow-up of 15 weeks.

The pandemic caused by COVID-19 has left few countries untouched. It is a far-reaching implication on humankind, with children and adolescents being no exception. Although the prevalence and fatality are negligible among children, a possible impact on their psychological and mental health cannot be disregarded. The unprecedented change in the way of living is bound to be having some psychological consequences on children and adolescents. The experiences gathered in childhood and adolescence are known to contribute to shaping the physical, emotional, and social well-being in adult life. Children are highly susceptible to environmental stressors. The present situation has the potential of adversely affecting the physical and mental well-being of children. To save the children from the long term consequences of this pandemic, a holistic approach integrating biological, psychological, social and spiritual methods of enhancing mental health have become essential. A concerted effort of government, non government organisations, parents, teachers, schools, psychologists, counselors and physicians are required to deal with the mental health issues of children and adolescents. This paper discusses the possible role of these agencies in the holistic intervention of this crisis.