BACKGROUND:Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with a strong female predisposition. pSLE often results in a worse prognosis compared to adult SLE. Studies on pSLE from the Indian subcontinent are scarce. OBJECTIVE:This study aims to describe the clinical manifestations, laboratory and serological parameters, management, and outcomes of pSLE patients from a premier tertiary care institute in Eastern India. METHODS:This prospective observational study was conducted at Kalinga Institute of Medical Sciences, Bhubaneswar, from September 2020 to October 2023. Children aged 1-14 years fulfilling the Systemic Lupus International Collaborating Clinics criteria for SLE were included. A detailed history, clinical examination, and laboratory investigations were performed. Data on complications, treatment, and outcomes were collected. Statistical analysis was done using SPSS Statistics version 21 (IBM Corp. Released 2012. IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp.). RESULTS:Out of 114,009 patients (outdoor and indoor), 40 were diagnosed with pSLE, resulting in an incidence of 0.35 per 1000 children. The female-to-male ratio was 7:1. The mean age of presentation was 11.67 ± 2.37 years. Among the predominant symptoms observed, mucocutaneous manifestations were seen in 39 (97.5%), followed by pallor in 36 (90%), and fever in 33 (82.5%). The most common organ system involved was mucocutaneous, observed in 39 (97.5%) patients, followed by hematological in 36 (90%) and renal in 19 (47.5%). Lupus nephritis was observed in 19 (47.5%) patients, with class IV being the most common. Anti-nuclear antibody and anti-double-stranded DNA were positive in 39 (97.5%) and 27 (68%) of children, respectively. Complete remission was achieved in 14 (35%), improvement in 16 (40%), and flare-ups in 3 (7.5%) patients. CONCLUSION:pSLE is an uncommon but severe autoimmune disease with significant multi-system involvement. Early identification and prompt treatment are crucial to minimizing adverse outcomes. This study provides detailed insights into the clinical and immunological profile of pSLE in Eastern India, underscoring the need for larger multicentric studies with long-term follow-ups.

Background Empyema thoracis is a complication of bacterial pneumonia, which can cause significant morbidity and mortality in children. Timely diagnosis and adequate treatment of pneumonia is essential. Objective To determine clinico-bacteriological profile of children admitted with empyema thoracis and to study their outcome and short-term follow-up. Materials and Methods This study was a prospective observational study, performed in age group of 1 month to 14 years admitted in a Tertiary Care Hospital of Odisha over a period of 3 years (March 2020 to February 2023). Results Of 95 patients, most (45) were of age from 1 to 5 years with a mean age of 68.46 ± 39.33 months and male to female ratio of 1.3:1. The peak of cases was seen during the month of March to July. Most common presentation was fever (99%) followed by cough (97%). On pleural fluid analysis organisms were isolated in 45 cases, staphylococcus aureus being commonest (21). Eighty children (84.5%) were treated with antibiotics and intercostal drainage tube, whereas 8 cases (8.5%) needed aspiration and antibiotics. Seven cases (7%) required surgical treatment in the form of decortication or thoracotomy and Video Assisted Thoracoscopic Surgery (VATS). Most common complication was pyopneumothorax, found in 34 cases (36%). Out of 95, 81 (85.3%) were cured, 3(3.2%) died and 4 (4.2%) left against medical advice. Four cases at 15 days follow-up and 2 cases at 2 months follow-up showed restrictive pattern of Pulmonary Function Test. Conclusion Empyema thoracis in children is a dreaded complication of pneumonia. Early diagnosis and effective treatment of pneumonia can decrease morbidity and mortality.

The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.

An 11-year-old boy with marfanoid habitus and high myopia presented with multiple episodes of seizures. He was found to have arachnodactyly, hypermobile joints, ectopia lentis, cerebral venous sinus thrombosis (CVST) with very high serum methionine and homocysteine. Genetic evaluation unveiled homocystinuria due to cystathionine beta-synthase deficiency. The patient was treated with high-dose pyridoxine, methionine restricted diet, anticonvulsants, warfarin, and correction of ectopia lentis. Homocystinuria should be suspected in patients with tall stature and pathological myopia. Early treatment can prevent thromboembolic complications.

Initial presentation of childhood systemic lupus erythematosus (SLE) as antiphospholipid syndrome (APS) is uncommon; moreover, APS presenting with both hemorrhage and thrombosis is very rare.We report a case of a previously healthy eight-year-old boy, without any significant past or family history, who presented with ecchymotic patches, epistaxis, and right-side hemiparesis. Investigation showed severe thrombocytopenia and isolated high activated partial thromboplastin time (aPTT) not corrected by mixing study. During his hospital stay, the child developed left-sided focal seizure and digital gangrene as thrombotic events.Neuroimaging revealed initially hemorrhagic stroke and subsequently bilateral infarct of middle cerebral artery (MCA) territory. The child was diagnosed as a case of SLE with APS based on Systemic Lupus International Collaboration Clinics (SLICC) criteria, revised APS classification, clinicoimmunological profile and neuroimaging. As the child was progressing towards catastrophic APS, he was treated aggressively with intravenous pulse methylprednisolone, intravenous cyclophosphamide and plasmapheresis with successful recovery.A simple bleeding manifestation may mask a serious disorder. A simple test like mixing study is helpful in diagnosis and in avoiding unnecessary investigations. A combination of both hemorrhage and thrombosis is an unusual presentation of APS and should always be suspected in case of autoimmune disorder, especially in SLE.

Background: Atopic dermatitis (AD), also known as eczema, is one of the most common skin disorders among children and adults, with a steep rise in diagnoses among children. Many studies have investigated the relationship between Vitamin D3 (Vit D3) and AD. Methods: A randomized controlled trial was conducted among 60 children at SCB Medical College, Cuttack from August 2014 to November 2016. Children were randomly assigned to an intervention group that received 60,000 IU of Vit. D3 every week for 6 weeks in addition to regular treatment for AD and a control group that received same treatment of AD except for Vit. D3. Both the groups were followed up at 4 weeks and 8 weeks. Results: In 60 cases of moderate to severe AD, 70% of the patients were male. About 81.7% of patients were from urban areas and 56.7% belonged to a middle socioeconomic class. In 76.6% of cases, family history of atopy was present. At baseline, mean SCORAD was 47.8±7.5 in the intervention group, and 49.2±10.3 in the control group. At baseline, serum Vitamin. D3 level (ng/ml) was 17.6±1.8 in intervention and 17.3±3.5 in control group. After Vitamin. D3 supplementation, the SCORAD improved to 12.8±5.1 (75% reduction) at 4 weeks, and 3.6±2.1 (92% reduction) at 8 weeks in the intervention group. In the control group, the mean SCORAD was 18.8±9.1 (61% reduction) at 4 weeks, and 7.3±4 (85% reduction) at 8 weeks. Discussion: The improvement of serum 25-hydroxy Vit. D3 was 134% and 366% in the intervention group compared to 78% and 121% in the observation group after 4 weeks and 8 weeks respectively. The p-values at both the time points were significant (<0.05) for the intervention group as compared to the control group. Conclusion: Short-term therapeutic supplementation of Vitamin. D3 in children with moderate to severe AD improves the clinical score. There is an inverse relationship between serum Vitamin. D3 level and severity of AD.

Background: Kawasaki disease (KD) is a medium-vessel vasculitis having coronary predilection, usually affecting under-5 children presenting as acute febrile illness. Despite being a disease with long-term grievous outcome, only few published literature are available from India, even lesser from its eastern region. Methods: From January 2016 to December 2020, 30 case records of children with a discharge diagnosis of KD were enrolled in this study. Demographic profile, clinical manifestations, laboratory data, echocardiographic findings, and treatment done were extracted from the case records. Laboratory investigations were done at admission and repeated after 24 h of intravenous immunoglobulin administration as per the American Heart Association Guidelines 2004. Echocardiography was carried out at diagnosis, at 2 weeks, and at 6 weeks. Results: Out of 30 children diagnosed with KD, majority belong to 1–5 years of age group (72%) with male predominance. Complete KD was seen in 77% of children. The most common presentation was fever >5 days (100%) followed by oral changes in 26 (87%), conjunctivitis in 25 (83%), extremity changes in 23 (77%), and rash in 21 (70%) children. Desquamation of perineum and reactivation of bacillus Calmette–Guérin scar were seen in 10%. No children with complete KD and three children with incomplete KD developed coronary artery Abnormalities (CAA). Conclusion: Infants with incomplete KD have a higher incidence of CAA. Aggressive management results in better outcome.

Biotinidase deficiency (BTD) is a rare inherited metabolic disorder with predominant dermatogical and neurological manifestations, which if untreated leads to severe neurological sequelae. Early diagnosis and prompt treatment with biotin prevents further progression of neurological symptoms and resolution of cutaneous features. We report an interesting case of four and half year male child presenting with seizures, developmental delay with non resolving extensive skin lesions and alopecia, diagnosed as BTD and successfully treated.

Background: Enteric fever is a major health problem in developing countries.Varied clinical presentation leads to diagnostic dilemmas resulting in fatal complications.Objective: To determine the socio-demographic, clinical manifestations, complications, antibiotic sensitivity pattern, treatment, and outcome in hospitalized enteric fever patients.Methods: A retrospective case record analysis of hospitalized patients in the age group one to 14 years with a discharge diagnosis of enteric fever was done in a tertiary care centre of Odisha over a period of three years (January 2017 to December 2019).Results: Of 112 patients, 75% of children belonged to the six to 14 years age group with a mean age of 7.6 +/-3.6 years and a male to female ratio of 1.66:1.The peak of cases was seen during the month of January to June with 94% of cases occurring in low and middle socioeconomic status.The commonest presentation was fever in 98.21%; other features were vomiting (39.29%), pain in abdomen (21.43%), diarrhoea (26.79%), and anorexia (14.29%).Eosinopenia was found in 58.93%, transaminitis in 30.36%, and raised CRP in 73.21%.In 30 children blood culture was positive with sensitivity to third-generation cephalosporin.All isolates were nalidixic acid-resistant Salmonella Typhi (NARST).Complications were seen in 21.42%.All recovered and two left against medical advice.Conclusion: Enteric fever is a major threat in the paediatric age group.Early clinical diagnosis with rational use of antibiotics according to sensitivity pattern is important.Improved hygiene, vaccination, and awareness among people is necessary for prevention.

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.

Kikuchi–Fujimoto disease is a self-limiting disease which frequently appears with fever and lymphadenopathy, thus creating the need for differential diagnosis of persistent febrile lymphadenopathy. Although the cause of Kikuchi’s disease is unknown, some viral or bacterial infections or immunological conditions are attributed to it. It has occasionally been misdiagnosed as lymphoma or tubercular lymphadenitis; hence, clinicians should be made aware of this disease. We report a case of a 14-year-old female child who was presented with fever, rashes, multiple oral ulcers, and lymphadenopathy. The child was diagnosed as Kikuchi disease, on the basis of lymph node histopathology. There was a significant improvement of the clinical picture with oral prednisolone therapy for 2 weeks and tapering over the next 2 weeks.