Long-Term Outcomes of Survivors of COVID-19 with Moderate to Severe Infection and Children with Multisystem Inflammatory Syndrome or MIS-C.

The study aims to evaluate the long-term outcomes - functional, pulmonary and non-pulmonary (other organs) - in children hospitalized with COVID-19 infection or with Multisystem inflammatory syndrome (MIS-C) after 1-2 y of discharge. All children with moderate or severe COVID-19 or MIS-C were enrolled. Out of 45 enrolled subjects, 19.8% had COVID-19 infection and 82% had MIS-C. Four children (8.9%) had abnormal baseline echocardiography; two each with cardiac dysfunction and coronary dilatation. At baseline, 44% had moderate disability and 24% had mild disability as per Pediatric Cerebral Performance Category (PCPC). On follow-up, only 8.9% (n = 4) had mild and 2.2% (n = 1) had moderate disability as per the PCPC score. One child developed new onset tuberculosis of the bone. None had any pulmonary morbidities. Follow-up echocardiogram was also within normal limits for children with abnormal findings. Further studies in different populations (settings) are required to draw meaningful conclusions about long-term effects of COVID-19 on children.

Monogenic lupus with homozygous C4A deficiency presenting as bronchiectasis and immune-mediated thrombocytopenia.

Monogenic lupus is a subset of lupus caused by single-gene disorders, integrating the paradoxical combination of autoimmunity and immunodeficiency. Pulmonary manifestations with recurrent pneumonia and bronchiectasis have rarely been described as the predominant presentation of juvenile lupus and may suggest an alternate differential like primary immunodeficiency, especially in early childhood. We describe a case of 10-year girl who presented with a history of recurrent pneumonia, arthritis, alopecia, and poor weight gain for the past 2 years. On examination, she had respiratory distress, bilateral diffuse crackles and arthritis of the small joints of hands. Lab investigations showed pancytopenia, low complement levels and high titers of ANA and anti-dsDNA antibodies. The patient was diagnosed with juvenile lupus. Imaging studies revealed evidence of multiple lobar collapse and consolidation with bronchiectasis. She was started on steroids, HCQ and supportive measures for bronchiectasis. The child reported relief in initial symptoms of lupus on follow-up but developed recurrent thrombocytopenia requiring IVIG and escalating the doses of oral steroids. The young age and atypical presentation prompted a screening for monogenic lupus, and clinical exome sequencing revealed a novel homozygous missense variation in exon 20 of the C4Agene with clinically reduced C4 levels, consistent with the diagnosis of C4A deficiency.

Efficacy of pulse dexamethasone in non-systemic juvenile idiopathic arthritis: a double-blind randomized controlled trial.

OBJECTIVE: Early aggressive therapy using biologicals is increasingly being used in JIA for early disease remission. Pulse steroids are used in induction regimes for rheumatic disorders such as SLE and systemic JIA; however, no controlled studies have demonstrated their use in non-systemic JIA. The objective of the present study was to evaluate the efficacy and safety of pulse dexamethasone therapy in children with treatment-naïve non-systemic JIA as early aggressive therapy in resource-limited settings. METHODS: Sixty treatment-naïve children with non-systemic JIA with an active joint count of ≥5 and/or involvement of hip or cervical joints were randomized to receive either pulse dexamethasone (3 mg/kg/day, max 100 mg/day) or placebo (normal saline) for three consecutive days during each visit at 0, 6 (±2) and 12 (±2) weeks; along with standard therapy (MTX and NSAIDs). The use of oral bridge steroids was permissible for persistent severe disease as per predefined criteria. The primary outcome was ACR-Pedi 70 response at 16 (±2) weeks after enrolment in the two groups. RESULTS: The proportion of children achieving ACR-Pedi 70 in the two groups at last follow-up was 11/30 (36.7%) in pulse dexamethasone arm vs 11/28 (39.3%) in the placebo arm (P-value 0.837, relative risk 0.93, 95% CI 0.48, 1.80). We did not observe any significant difference in the proportion of children requiring bridge steroids. Adverse events were comparable in the two groups. CONCLUSION: The addition of pulse dexamethasone to standard treatment may not add any advantage in improving ACR-Pedi 70 scores at medium-term follow-up. TRIAL REGISTRATION: Clinical Trial Registry-India; www.ctri.nic.in CTRI/2018/08/015151.

Entomophthoromycosis in a child: Delayed diagnosis and extensive involvement.

Entomophthoromycosis is a rare fungal infection of the skin and subcutaneous tissue occurring predominantly in tropical and subtropical regions. In children, it mostly affects the lower half of the body. With this, we report a case of Entomophthoromycosis in a 6-year-old girl who presented late with extensive involvement of the upper half of the body. She responded well to treatment with potassium iodide and itraconazole. We also reviewed cases of Entomophthoromycosis reported in children.

Isotonic versus Hypotonic Intravenous Maintenance Fluids in Children: A Randomized Controlled Trial.

OBJECTIVE: To compare the incidence of hyponatremia during the first 48 h in hospitalized children receiving normal saline vs. N/2 saline as maintenance intravenous fluid. METHODS: This open label, randomized controlled trial to compare the incidence of hyponatremia in hospitalized children receiving normal saline (0.9% sodium chloride in 5% dextrose) vs. N/2 saline (0.45% sodium chloride in 5% dextrose) as maintenance fluid was conducted from December 2014 through November 2015 in a tertiary care teaching hospital. Children between 1 mo and 18 y requiring maintenance intravenous fluids were randomized to receive normal saline with 5% dextrose (n = 75) or N/2 saline with 5% dextrose (n = 75). RESULTS: Both groups were comparable for demographic variables and illness severity at baseline. Incidence of hyponatremia at 24 h of hospitalization was comparable between normal saline and N/2 saline group, 3(4%) vs. 6(8%) cases, respectively; p value 0.494. Mean serum sodium levels were marginally higher in normal saline group (138.3 ± 6.0 mEq/L) as compared with N/2 saline group (135.1 ± 4.4 mEq/L) (p value <0.01) at 24 h of hospitalization. Incidence of hyponatremia at 48 h and hypernatremia at 24 and 48 h was comparable in two groups. CONCLUSIONS: The use of either N/2 saline or normal saline in sick children at standard maintenance fluid rates is associated with low but comparable incidence of hypo or hypernatremia in first 24 h of hospitalization. Both types of fluids appear acceptable in hospitalized sick children.

Impact of Malnutrition on the Outcome of Critically Ill Children.

OBJECTIVE: To assess the impact of nutritional status on outcomes like mortality rate, length of mechanical ventilation and length of Pediatric Intensive Care Unit (PICU) stay, in critically ill children. METHODS: In this retrospective study conducted at a tertiary care center, records of 332 critically ill children between 1 mo to 15 y of age for whom anthropometric parameters were available were included. Anthropometric parameters for the study subjects were used to assess the nutritional status using the WHO growth charts as the reference. The study subjects were categorized as non-malnourished, moderately, and severely malnourished, defined by Body mass index (BMI) for age 0 to -2 SD, -2 to -3 SD and less than -3 SD of WHO growth charts, respectively. Various outcomes like mortality, duration of PICU stay and duration of mechanical ventilation were assessed in the 3 groups based on the nutritional status. RESULTS: The prevalence of malnutrition in the index study was 51.2 % with an overall mortality of 38.8 %. No difference was found between mortality rates and proportion of ventilated children in the three study groups. However, more children who were severely malnourished had significantly prolonged ICU stay (>7 d) as well as duration of mechanical ventilation (>7 d). When the outcome variables were compared after adjusting for PIM2 scores, there were increasing odds of mortality, ventilation, prolonged PICU stay and duration of mechanical ventilation with increasing severity of malnutrition. CONCLUSIONS: After stabilization of the initial critical phase, PICU outcome is influenced by the nutritional status of the children.