Atypical Presentation of Cystic Fibrosis in An Infant.

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.

Short-term outcomes in children recovered from multisystem inflammatory syndrome associated with SARS-CoV-2 infection.

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.

Etiology, Clinical Profile, Evaluation, and Management of Stridor in Children.

OBJECTIVE: To evaluate various causes of pediatric stridor and their management among admitted patients in last 2 y. METHODS: Retrospective study of 67 stridor cases in pediatric age group (from birth to 18 y), admitted to the Department of Pediatrics and ENT (Ear, Nose and Throat) from May 2018 to April 2020 were included in the study. Data were obtained from medical records regarding age, gender, clinical presentation, and management. RESULTS: Out of 67 cases of pediatric stridor, 28.3% were infants, 50.7% were between 1 to 5 y, while 20.9% were between 5 to 18 y. Foreign body trachea (FB) was the most common (38.8%) cause of stridor. The commonest cause of stridor among infants was laryngomalacia (47.4%) while FB trachea (55.9%) was the commonest cause among 1 to 5 y age group. In age group between 5 to 18 y, peritonsillar abscess and bacterial tracheitis (21.4% each) were found to be the most common. Primary management with securing of airways were done in all cases. Curative treatment was provided according to the underlying pathology. Eight patients (11.9%) required tracheostomy to bypass airway obstruction. There was no mortality in the present study population. CONCLUSION: Pediatric stridor management is a teamwork between ENT surgeons, pediatricians, and anaesthetists. Management starts with suspicion from history followed by clinical and radiological evaluation. Securing airway is of utmost importance and precise management of cause is carried out later.

Tracheostomy in Pediatric Intensive Care Unit: Experience from Eastern India.

OBJECTIVE: Tracheostomy is one of the most commonly used surgical intervention in sick children in the intensive care unit. The literature in the pediatric population is limited, therefore, we conducted this study to evaluate the indications, timing, complications, and outcomes of tracheostomy among the children at our center. METHODS: This retrospective study was conducted from January 2016 through December 2019. Data was collected from the patients' records and analyzed. RESULTS: During this study period, 283 children were ventilated, of which 26 (9.1%) required tracheostomy. Among this 73% were boys. The median age of the children who underwent tracheostomy was 6.32 y. The most common indication for tracheostomy was prolonged mechanical ventilation [24 cases (92%)] followed by upper airway obstruction [2 cases (8%)]. The average time of tracheostomy was 11.65 d, range (1-21 d). Complications were seen in 14 patients (55%). The most common complications were accidental decannulation, occlusion, pneumothorax, and granulation tissue. Twenty one (80%) patients were successfully discharged, out of which 16 (61%) patients were discharged after decannulation and 5 (21%) were sent home with a tracheostomy tube in situ. Overall mortality in present study was 11.5%; none was directly related to tracheostomy. CONCLUSIONS: The indication for tracheostomy has been changed from emergency to more elective one. Prolonged mechanical ventilation is the most common indication for tracheostomy. Although the timing of tracheostomy is not fixed, two weeks time is reasonable and it can be done safely at the bedside in pediatric intensive care.

Clinical Profile and Immediate Outcome of Multisystem Inflammatory Syndrome in Children Associated with COVID-19: A Multicentric Study.

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.

An Infant with Milky Serum and a Rare Mutation.

A 40-day-infant having milky serum, eruptive xanthomas, hepatosplenomegaly, lipemia retinalis, high cholesterol and triglyceride, was found to have lipoprotein lipase (LPL) deficiency on genetic workup. Triglyceride decreased with dietary fat restriction, medium chain triglyceride and fibrates. LPL deficiency in early infancy can be treated with pharmacological and dietary interventions.

Coordination of cardiac rhythmic output and circadian metabolic regulation in the heart.

Over the course of a 24-h day, demand on the heart rises and falls with the sleep/wake cycles of the organism. Cardiac metabolism oscillates appropriately, with the relative contributions of major energy sources changing in a circadian fashion. The cardiac peripheral clock is hypothesized to drive many of these changes, yet the precise mechanisms linking the cardiac clock to metabolism remain a source of intense investigation. Here we summarize the current understanding of circadian alterations in cardiac metabolism and physiology, with an emphasis on novel findings from unbiased transcriptomic studies. Additionally, we describe progress in elucidating the links between the cardiac peripheral clock outputs and cardiac metabolism, as well as their implications for cardiac physiology.

Dilemmas and Challenges in Treating Seronegative Autoimmune Encephalitis in Indian Children.

OBJECTIVE: The main objective is to assess the challenges in diagnosis and treatment while managing seronegative cases of autoimmune encephalitis (AIE) in Indian children. METHODS: A cohort study of patients with AIE was done where clinical presentations, investigations, management were analyzed and these patients were followed up to assess the evolution of the disease. RESULTS: Nine patients were included in the study. Four patients presented with super-refractory status epilepticus (SRSE). Other presentations were behavioral change, hemiplegia, and autonomic dysfunction. Initial magnetic resonance imaging brain was suggestive of AIE in two patients. Only two were seropositive for cerebrospinal fluid (CSF) autoimmune panel. Five patients responded to the first-line immunotherapy and four required the second-line immunotherapy. CONCLUSION: The possibility of autoimmune encephalitis should be considered in patients with super-refractory status epilepticus. A large proportion of children with suspected AE may be "seronegative." A trial of immunotherapy should be given to these children when there is a strong clinical suspicion of autoimmune encephalitis even in the absence of cerebrospinal fluid autoantibodies.