Clinical and Laboratory Profile of Macrophage Activation Syndrome in Kawasaki Disease: A Single Centre Cross-Sectional Study.

OBJECTIVE: To study the prevalence of Macrophage Activation Syndrome (MAS) in children with Kawasaki disease (KD) and to devise a classification tree for predicting MAS in early KD based on easily available clinical and laboratory information using artificial intelligence (AI) technology. METHODS: A prospective cross-sectional observational study was conducted (March 2020 - October 2021) during which hospitalized children aged 1-18 years with KD were consecutively enrolled. Those with positive RTPCR test or IgM/IgG serology for COVID-19 were excluded. The clinical and laboratory profiles of children with and without MAS were studied. A multivariable logistic regression (LR) model was developed utilizing backward elimination method to determine the relationship between select candidate predictor variables and MAS in patients with KD. A classification tree was created based on these using artificial intelligence algorithms. RESULTS: Sixty-two children were diagnosed with KD during the study period, of these, 42 children with KD were included; 14 (33.3 %) were diagnosed with MAS. The median (IQR) duration of fever (days) was significantly more in MAS than those without MAS [7 (5, 15) vs 5 (5, 9), P < 0.05]. Serum albumin (g/dL) was significantly lower in those with MAS [2.3 (2.2, 2.7) vs 2.8 (2.3, 3.1), P = 0.03]. The classification tree constructed by the AI-based algorithm predicted that in children with KD who had myocardial dysfunction, serum albumin ≤ 2.8 g/dL and fever > 6 days duration at admission had increased likelihood of developing MAS. In children without myocardial dysfunction, alanine transaminase (ALT) levels > 70 U/L and fever > 5 days were equally predictive of MAS. CONCLUSION: Nearly one-third of the children with KD had MAS. Clinicians should consider screening all children with KD for MAS at admission. A classification tree based on the presence of myocardial dysfunction, duration of fever > 6 days, ALT levels and hypoalbuminemia can identify MAS in the course of KD.

Mucolipidosis: A mimicker of juvenile idiopathic arthritis.

Juvenile idiopathic arthritis is the most common form of chronic arthritis in children and at times misdiagnosed in those presenting with arthropathy secondary to non-inflammatory causes. The overlap of symptoms often pose a diagnostic challenge for clinicians. This mostly results in a delayed diagnosis subjecting children to unnecessary use of long-term immunosuppressants and disease-modifying drugs. We present the case of a 9-year-old boy who was previously misdiagnosed as a case of juvenile idiopathic arthritis. Detailed evaluation later led to the diagnosis of mucolipidosis (type III) which was confirmed on genetic testing. Emphasis on detailed history and clinical examination including the subtle hints like lack of signs of inflammation, family history, no morning stiffness and normal inflammatory markers should be picked up to make a timely diagnosis. In today's era of genetic testing and diagnosis, it is prudent to offer these tests for such patients to make an accurate diagnosis and prognosticate them for the long-term outcome.

Serum Calprotectin Levels in Different Subtypes of Juvenile Idiopathic Arthritis (JIA) and Its Correlation with Quantitative CRP and JADAS-27.

OBJECTIVE: To study the serum calprotectin levels in children with juvenile idiopathic arthritis (JIA) and correlate it with C-reactive protein (CRP) and the Juvenile Arthritis Disease Activity Score-27 (JADAS-27). METHODS: This was a cross-sectional observational study done between November 2017 and March 2019. Fifty treatment-naive children, aged 1 to 18 y with the diagnosis of JIA as per the International League of Associations of Rheumatology (ILAR) criteria were enrolled. Assessment of disease activity was done according to the Juvenile Arthritis Disease Activity Score (JADAS-27). Determination of serum calprotectin and CRP levels was done by immunoassay. The correlation between calprotectin levels with CRP and JADAS-27 was calculated. RESULTS: Of the 50 patients with JIA included in the study, there were 18 female and 32 male children. The median age of presentation to the hospital was 9 y (IQR 5.82-13). The median JADAS-27 was 14 (IQR 6, 20.25). The median serum calprotectin level was 45,375 ng/mL (IQR 30,725, 52,270; range 8,560-63,160 ng/mL). The median CRP was 35.4 mg/L (IQR 3.48, 80.3; range 0.02 and 107.4 mg/L). The levels of calprotectin in different JIA subtypes were not statistically different using Kruskal-Wallis test. The study also demonstrated a positive correlation between serum calprotectin with CRP and the JADAS-27 (r = 0.418). CONCLUSION: The calprotectin levels in JIA were significantly higher than those reported in the literature irrespective of the subtype. Serum calprotectin positively correlated with CRP and JADAS-27 in children with JIA.

Kawasaki Disease with Peripheral and Facial Gangrene: A Case report and review of literature.

With the onset of the SARS-CoV-2 pandemic, Kawasaki Disease (KD) has come to the fore with its many atypical manifestations. Atypical clinical neurological, ophthalmological, musculoskeletal, gastrointestinal and pulmonary manifestations in a febrile child with raised markers should prompt the clinician to think of Kawasaki disease. Peripheral gangrene is a rare atypical manifestation of KD reported in infancy. We present a three-and-a-half-year-old boy with extensive gangrene all four limbs and face along with purpura fulminans. He was successfully treated with two doses of intravenous immunoglobulin (IVIG) and infliximab, with no residual gangrene. This case highlights that very severe forms of Kawasaki disease require IVIG, pulse steroids as well as infliximab for adequate control and complete resolution of the disease.

Magnitude of SARS-CoV-2 Infection and Outcome in Paediatric Surgical Inpatients during the First Wave of Pandemic at a Tertiary Care Children's Hospital in India.

This study was conducted at a tertiary care centre of Delhi, to assess the magnitude of SARS-CoV-2 disease and outcome in paediatric surgical inpatients. All the admitted patients were evaluated excluding outpatients and minor procedures. Out of 312 operated patients 2.88% patients were SARS-CoV-2 positive and out of 167 non-operative patients 6.58% were SARS-CoV-2 positive. These patients received standard care as per guidelines using standard protective measures and were discharged home. Only 1 death occurred due to perforation peritonitis with sepsis. The overall prevalence of SARS-CoV-2 in paediatric surgical patients was 4.17% and SARS-CoV-2 positive patients had similar outcomes as compared to non-SARS-CoV-2 patients.

Comparison of clinical and laboratory profile of survivors and non-survivors of SARS-CoV-2-related multisystem inflammatory syndrome of childhood in India: An observational study.

AIM: The SARS-CoV-2 pandemic is characterised by multiple reports of paediatric multisystem inflammatory disease or multisystem inflammatory syndrome in children (MIS-C) with Kawasaki disease-like features often complicated by myocarditis, shock and macrophage activation syndrome. Certain clinical and laboratory markers may be used to identify high risk cases. METHODS: All sequentially admitted patients hospitalised between April 2020 and October 2020, who met the WHO case definition for MIS-C were included. Data included patient demographic information, presenting symptoms, organ dysfunction and laboratory parameters. SARS-CoV-2 infection was diagnosed by nasopharyngeal swab real-time reverse transcription-polymerase chain reaction and/or rapid antibody test for SARS-CoV-2 as recommended. The clinical and laboratory criteria were compared in the survival and non-survival groups. RESULTS: A total of 29 patients with MIS-C were treated during the study period. There were 21 survivors and 8 non-survivors. The non-survivors had more neurocognitive and respiratory symptoms along with increased incidence of myocarditis compared with survivors. The serum levels of CPK-MB, D-dimer, ferritin and triglyceride were significantly raised in non-survivors as compared to survivors. CONCLUSION: The non-survivor group had higher CPK and greater proportion of children with troponin-T elevation indicating higher incidence of myocardial injury and necrosis. The D-dimer, ferritin and triglyceride were also higher in the mortality group, indicating the greater extent of inflammatory damage in this group.

Profile of Children with Kawasaki Disease Associated with Tropical Infections.

OBJECTIVE: To describe various infectious triggers for Kawasaki disease (KD) in India. METHODS: A series of 10 children with diagnosed infections who developed KD during their course of illness has been presented. They were diagnosed by the American Heart Association (AHA) 2017 guidelines. Echocardiography was done to check for coronary artery dilation. Treatment was instituted as per standard protocol. RESULTS: Kawasaki disease was diagnosed in 8 boys and 2 girls, aged 1 mo to 11 y. These children were being treated for dengue, chikungunya, SARS-CoV-2, hepatitis A, tuberculosis, brucellosis, disseminated staphylococcal sepsis, scrub typhus, and enteric fever. CONCLUSIONS: Kawasaki disease has been associated with infectious triggers. It should be considered in febrile patients with mucocutaneous involvement or in nonresponsive sepsis, despite adequate therapy.

Acute Disseminated Encephalomyelitis After Plasmodium Vivax Infection: Case Report and Review of Literature.

Acute demyelinating encephalomyelitis (ADEM) usually occurs after viral infections or vaccination. Its occurrence after Plasmodium vivax infection is extremely uncommon. We report the case of an 8-year-old girl who had choreo-athetoid movements and ataxia after recovery from P.vivax infection. Diagnosis of ADEM was made on the basis of magnetic resonance imaging findings. The child responded to corticosteroids with complete neurological recovery.

An unusual case of neonatal stridor.

A25-day baby neonate presented with fever and stridor. He had severe respiratory distress at admission. The systemic examination was unremarkable. The roentgenogram of soft tissues of neck revealed widening of superior mediastinum. Computed tomography of neck and upper chest revealed multiple abscesses in the retropharyngeal space, parapharyngeal space, and superior mediastinum. The child improved on aggressive antibiotic treatment protocol. It raises awareness among paediatricians to consider this diagnosis when confronting neonate with fever and stridor. An early diagnosis and aggressive appropriate management will reduce mortality and morbidity associated with this life-threatening condition. A thorough search for a primary source of infection should be done. Neonate should be screened for primary and secondary immunodeficiency disorders before discharge.

Adherence to antiretroviral therapy and its determinants in children with HIV infection - Experience from Paediatric Centre of Excellence in HIV Care in North India.

The objective of this study is to assess adherence to antiretroviral therapy (ART) in HIV-infected children using the pill count method, and determine factors leading to adherence failure. Records of 106 children living with HIV (CLHIV) age <15 years and on ART for >6 months were reviewed. Average adherence to ART by pill count method over preceding six months was calculated and re-assessed by 3-day recall method. The caregivers of 105 children and one child himself were interviewed about the problems encountered while giving ART. Median age of enrolled children was 104 (inter-quartile range [IQR] 77.3-133.8) months. Median duration of ART was 25 (IQR 16-35) months. The desired adherence level of >95% during six months of review assessed by pill count was achieved in 95.3% children. The 3-day recall method yielded >95% adherence in 99% children (p ≤ .001). Caregivers of 59 children (56.2%) reported multiple problems while administering drugs. In most instances, problems encountered were related to family/caregivers, the commonest being multiple caregivers, job constraints and death/illness in the family. In conclusion, we found a very high level of adherence to ART in CLHIV. Poor adherence was mainly associated with issues related to the family/caregivers.

Common endocrine problems in children (hypothyroidism and type 1 diabetes mellitus).

Hypothyroidism and type 1 diabetes mellitus (T1DM) constitute two common endocrine disorders of children. Both these are chronic conditions which require long term follow-up for optimum control, can be missed on initial presentation unless a high index of suspicion is maintained and may be associated with adverse outcome if not properly managed. This article deals with basic pathology, recognition, initial investigations, and principles of treatment of these conditions from the perspective of a general pediatrician. It also describes the role of a practitioner in management of these disorders.