Clinical Profile, Intensive Care Needs and Outcome of Children with Dilated Cardiomyopathy Associated with Vitamin D Deficiency: A 5-year PICU Experience.

Aim: To describe the clinical profile, treatment details, intensive care needs, and long-term outcome of children with dilated cardiomyopathy (DCM) associated with Vitamin D deficiency (VDD). Materials and methods: Case records of 14 children with DCM associated with VDD [25(OH)D3 levels <20 ng/mL] admitted to the pediatric intensive care unit (PICU) of a tertiary care teaching hospital between January 2017 and December 2021 were retrospectively analyzed for clinical features, echocardiographic findings, treatment details, intensive care needs, and outcomes. Results: The median (IQR) age was 6 (2-9) months and 71% (n=10) were males. The common modes of presentation included respiratory distress or failure (78.6%), congestive cardiac failure (71.4%), cardiogenic shock (37.5%), and seizures and encephalopathy (14.3% each). The median (IQR) serum calcium was 8.7 (7-9.5) mg%, ionized calcium 0.7 (0.7-1.1) mmol/L, alkaline phosphatase 343 (316-415) IU/L, phosphate 3.5 (2.6-4.5) mg%, PTH 115 (66-228) pg/mL, and 25(OH)D3 5 (3-7) ng/mL. The median (IQR) left ventricular ejection fraction (LVEF) at admission was 22 (17-25)%. The treatment included intravenous calcium infusion (35.7%), vitamin D supplementation in all (57.1% parenteral and 42.9% oral), mechanical ventilation (35.7%), and vasoactive drugs (57.1%). There was no mortality. The median (IQR) duration of PICU and hospital stay was 76 (31-98) hours and 6 (4.7-10) days, respectively. Out of 14 children, 10 (71.4%) were followed-up till median (IQR) of 10 (7-58) months. All were asymptomatic and had normal LEVF (except one had residual moderate mitral regurgitation). Conclusion: Vitamin D deficiency is a potentially treatable and reversible cause of DCM in children. How to cite this article: Kumar S, Randhawa MS, Angurana SK, Nallasamy K, Bansal A, Kumar MR, et al. Clinical Profile, Intensive Care Needs and Outcome of Children with Dilated Cardiomyopathy Associated with Vitamin D Deficiency: A 5-year PICU Experience. Indian J Crit Care Med 2023;27(7):510-514.

Clinical Features Associated With Need for Mechanical Ventilation in Children With Guillain-Barré Syndrome: Retrospective Cohort From India.

OBJECTIVES: To analyze the clinical features associated with the need for mechanical ventilation (MV) in children with Guillain-Barré syndrome (GBS). DESIGN: Retrospective cohort study, 2010-2019. SETTING: PICU. PATIENTS: All children, 1 month to 12 years old, diagnosed with GBS in our single-center PICU. INTERVENTION: Retrospective chart and data review. MEASUREMENTS AND MAIN RESULTS: Out of 189 children identified with a diagnosis of GBS, 130 were boys (69%). The median (interquartile range [IQR]) age was 6 years (3-9 yr). At admission, the Hughes disability score was 5 (4-5), and cranial nerve palsies were present in 81 children (42%). Autonomic instability subsequently occurred in a total of 97 children (51%). In the 159 children with nerve conduction studies, the axonal variant of GBS (102/159; 64%) predominated, followed by the demyelinating variant (38/189; 24%). All children received IV immunoglobulins as first-line therapy at the time of admission. The median (IQR) length of PICU stay was 12 days (3-30.5 d). Ninety-nine children (52%) underwent invasive MV, and median duration of MV was 25 days (19-37 d). At admission, upper limb power less than or equal to 3 (p = 0.037; odds ratio (OR), 3.5 [1.1-11.5]), lower limb power less than or equal to 2 (p = 0.008; OR, 3.5 [1.4-8.9]), and cranial nerve palsy (p = 0.001; OR, 3.2 [1.6-6.1]) were associated with subsequent need for MV. Prolonged (> 21 d) MV was associated with more severe examination findings at admission: upper limb power less than or equal to 2 (p < 0.0001; OR, 4.2 [2.5-6.9]) and lower limb power less than or equal to 1 (p < 0.0001; OR, 4.5 [2.6-7.9]). CONCLUSIONS: In children with GBS, referred to our center in North India, severe neuromuscular weakness at admission was associated with the need for MV. Furthermore, greater severity of this examination was associated with need for prolonged (> 21 d) MV. Identification of these signs may help in prioritizing critical care needs and early PICU transfer.

Severe dengue associated with Staphylococcus aureus sepsis in pediatric patients: a case series.

BACKGROUND: Staphylococcus aureus co-infection is seldom reported in children with severe dengue. METHODOLOGY: In this retrospective study, we reported five children with severe dengue and S. aureus co-infection admitted to pediatric intensive care unit (PICU) during July-December 2021. RESULTS: All children had prolonged fever, persistence of bilateral pleural effusion beyond the critical phase, thrombocytopenia and raised inflammatory markers [C-reactive protein (CRP) and procalcitonin]. S. aureus was isolated from pleural fluid (n = 2, 40%), blood (n = 2, 40%) and endotracheal aspirate (n = 1, 20%). Four children (80%) grew methicillin-sensitive S. aureus, while 1 (20%) had methicillin-resistant S. aureus. Two children (40%) had septic thromboemboli in skin, and 1 (20%) had limb cellulitis. One child required anterior thoracotomy, pericardiectomy and bilateral pleural decortication, while all other children required intercostal chest tube drainage. All children required prolonged targeted antibiotics, invasive mechanical ventilation and had prolong stay in PICU and all of them survived. CONCLUSION: In children with severe dengue, persistence of fever, persistence of pleural effusion beyond critical phase and raised CRP and procalcitonin should raise suspicion of bacterial/S. aureus co-infection.

Intensive Care Needs and Short-Term Outcome of Multisystem Inflammatory Syndrome in Children (MIS-C): Experience from North India.

OBJECTIVES: To describe the intensive care needs and outcome of multisystem inflammatory syndrome in children (MIS-C). METHODOLOGY: This retrospective study was conducted in the pediatric emergency, pediatric intensive care unit (PICUs) and the coronavirus disease 2019 (COVID 19) hospital of a tertiary teaching and referral hospital in North India over a period of 5 months (September 2020 to January 2021). Clinical details, laboratory investigations, intensive care needs, treatment and short-term outcome were recorded. RESULTS: Forty children with median interquartile range age of 7 (5-10) years were enrolled. The common clinical features were fever (97.5%), mucocutaneous involvement (80%), abdominal (72.5%) and respiratory (50%) symptoms. Shock was noted in 80% children. Most cases (85%) required PICU admission where they received nasal prong oxygen (40%), non-invasive (22.5%) and invasive (22.5%) ventilation and vasoactive drug support (72.5%). The confirmation of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) exposure was in the form of positive serology (66.7%), reverse transcriptase polymerase chain reaction (10%), and contact with SARS-CoV-2 positive case (12.5%). The common echocardiographic findings included myocardial dysfunction (ejection fraction <55%; 72.5%), and coronary artery dilatation or aneurysm (22.5%). The immunomodulatory treatment included intravenous immunoglobulin (2 g/kg) (100%) and steroids (methylprednisolone 10-30 mg/kg/day for 3-5 days) (85%). Aspirin was used in 80% and heparin (low molecular weight) in 7.5% cases. Two children died (5%) and median duration of PICU and hospital stay in survivors were 5 (2-8) and 7 (4-9) days, respectively. Children with shock showed higher total leucocyte count and higher rates of myocardial dysfunction. CONCLUSION: Cardiovascular involvement and shock are predominant features in severe disease. Early diagnosis can be challenging given the overlapping features with other diagnoses. A high index of suspicion is warranted in children with constellation of fever, mucocutaneous, gastrointestinal and cardiovascular involvement alongwith evidence of systemic inflammation and recent or concurrent SARS-CoV-2 infection. The short-term outcome is good with appropriate organ support therapies and immunomodulation.

Recurrent Guillain-Barré Syndrome: Clinical Profile and Outcome.

OBJECTIVES: To compare clinical, neurological, treatment and outcome of recurrent Guillian-Barré Syndrome (GBS) with non-recurrent GBS. Also, to compare different episodes of recurrent GBS. METHODS: The patient's case records were retrieved retrospectively from the electronic database and case record files. Clinical profile, nerve conduction study, treatment, and outcome details of children with a diagnosis of GBS admitted in Pediatric Intensive Care Unit (PICU) from 2010 to March 2022 were screened. Recurrent GBS cases, as defined by the National Institute of Neurological and Communicative Diseases and Strokes (NINCDS) criteria, were analysed separately. The comparison was made between the first episode of recurrent GBS with non-recurrent GBS, and predictors of recurrence were identified. A comparison of course and outcome was also done between different episodes of GBS in recurrent cases. RESULTS: Recurrent GBS was observed in 11 (4.7%) out of 234 cases during the study period. The presence of respiratory (p 0.015) and gastrointestinal illness (p 0.007) as preceding illnesses were associated with recurrence. No difference was noted between the first episode of recurrent GBS and non-recurrent GBS. The first and second episodes of GBS in 11 recurrent cases were similar in course and outcome. CONCLUSIONS: Recurrent GBS is underreported but a known entity. No difference was seen between the two episodes of recurrent GBS. However, more data is required to find the features of recurrent GBS so that limited PICU resources can be used judiciously and intervention planned accordingly.

Long-Term Neurological, Behavioral, Functional, Quality of Life, and School Performance Outcomes in Children With Guillain-Barré Syndrome Admitted to PICU.

BACKGROUND: Most children with Guillain-Barré syndrome (GBS) recover but may suffer from long-term sequelae, interfering with development and quality of life. Owing to the lack of published data, we aimed to assess the long-term neurological, behavioral, functional, quality of life, and school performance outcomes them. METHODS: Design: Cross-sectional observational. SETTING: Pediatric intensive care unit. PATIENTS: Children, aged one to 12 years, with GBS admitted over five years (July 2012 to June 2017) were enrolled during one year (July 2017 to June 2018). These children were assessed for the following outcomes: neurological (Hughes disability score, Pediatric Cerebral Performance Category [PCPC], Pediatric Overall Performance Category [POPC], and Glasgow Outcome Scale-Extended Pediatric version [GOS-E Peds] scales), behavioral (Childhood Psychopathology Measurement Schedule [CPMS]), functional (Vineland Social Maturity Scale [VSMS]-Indian Adaptation), quality of life (Pediatric Quality of Life [PedsQL]), and school performance (Parent-Directed Questionnaire). RESULTS: Eighty children were enrolled after a median of 3.0 (1.3-4.2) years from discharge. The majority (95%) had favorable neurological recovery (Hughes disability score 0 to 1). Favorable outcome was noted in 95% of children on PCPC, 87.5% on POPC, 60% on GOS-E Peds, 86.2% on CPMS, 92.5% on VSMS, and 98% on PedsQL. The majority (97.5%) of childre were attending schools, and 57.7% had satisfactory school performance. The presence of quadriparesis at admission, mechanical ventilation, tracheostomy requirement, poor ambulatory status at discharge, and longer pediatric intensive care unit and hospital stay predicted unfavorable neurological outcome on different tools. Absence of quadriparesis at admission and no requirement of mechanical ventilation predicted a favorable result on all outcome measures. CONCLUSIONS: On long-term follow-up, most children with severe GBS showed favorable neurological, behavioral, functional, and quality of life outcomes. Severe clinical presentation and prolonged intensive care unit stay predict poor long-term outcome.

Comparison of Multisystem Inflammatory Syndrome (MIS-C) and Dengue in Hospitalized Children.

OBJECTIVE: Multisystem inflammatory syndrome (MIS-C) in children is a febrile illness that has overlapping presentation with other locally prevalent illnesses. Clinicolaboratory profile of children admitted with MIS-C and dengue were compared to understand their presentation at the outset. METHODS: This was a retrospective study of children ≤ 12 y admitted with MIS-C (WHO definition) or laboratory-confirmed dengue between August 2020 and January 2021 at a tertiary center in North India. RESULTS: A total of 84 children (MIS-C - 40; dengue - 44) were included. The mean (SD) age [83.5 (39) vs. 91.6 (35) mo] was comparable. Rash (72.5% vs. 22.7%), conjunctival injection (60% vs. 2.3%), oral mucocutaneous changes (27.5% vs. 0) and gallop rhythm (15% vs. 0) were seen more frequently with MIS-C, while petechiae [29.5% vs. 7.5%], myalgia (38.6% vs. 10%), headache (22.7% vs. 2.5%), and hepatomegaly (68.2% vs. 27.5%) were more common with dengue. Children with MIS-C had significantly higher C-reactive protein (124 vs. 3.2 mg/L) and interleukin 6 (95.3 vs. 20.7 ng/mL), while those with dengue had higher hemoglobin (12 vs. 10.2 g/dL) lower mean platelet count (26 vs. 140 × 109/L), and greater elevation in aspartate (607 vs. 44 IU/L) and alanine (235.5 vs. 56 IU/L) aminotransferases. The hospital stay was longer with MIS-C; however, PICU stay and mortality were comparable. CONCLUSION: In hospitalized children with acute febrile illness, the presence of mucocutaneous features and highly elevated CRP could distinguish MIS-C from dengue. The presence of petechiae, hepatomegaly, and hemoconcentration may favor a diagnosis of dengue.

Acute encephalopathy with biphasic seizures and late restricted diffusion temporally associated with human bocavirus infection.

Acute encephalitis is a syndromic diagnosis. In the last two decades, a unique clinico-radiological entity, named acute encephalopathy with biphasic seizures and late restricted diffusion (AESD), has been reported in children from Asia. It is characterised by an acute febrile illness with seizures and encephalopathy, with some initial improvement followed by a second flurry of seizures and deep encephalopathy, 3-4 days later. MRI may show a pattern of 'bright tree appearance'. An aetiological agent may not always be identified but an infectious trigger is proposed. Immunomodulatory therapy has been tried with variable results. The prognosis is variable, and children are usually left with neurological sequelae including epilepsy and cognitive impairment. We describe a female infant who presented with the typical clinico-radiological syndrome of AESD and human bocavirus was identified in the stool. She received steroids and antiepileptic drugs. She has persistent cognitive impairment at follow-up but remained seizure free.

AHA Pediatric Advanced Life Support Update 2020 - "More Breaths, Less Fluids, and a Focus on Recovery".

Pediatric Advanced Life Support (PALS) guidelines are updated every five years and the new 2020 guidelines were issued recently. We briefly review the important changes in terms of rates of rescue breaths, timing of epinephrine, resuscitation in septic shock, use of extracorporeal therapies, and the new component in the chain of survival - recovery.

Impact of Recent SARS-CoV-2 Infection on the Course and Severity of Dengue in Children: A Prospective Observational Study from North India.

In 2020, a considerable overlap occurred between the COVID-19 pandemic and seasonal dengue transmission in India. This study aimed to evaluate the effects of acute or recent infection with SARS-CoV-2 on the course and outcomes of dengue fever in children. We prospectively enrolled 44 children with a clinical and laboratory diagnosis of dengue fever. Assessment of acute and recent SARS-CoV-2 infection was done using reverse transcription-polymerase chain reaction and IgG antibody through ELISA. Children were grouped based on evidence of SARS-CoV-2 exposure and clinical severity, and outcomes were compared. The median age of the study cohort was 96 months (interquartile range [IQR]: 69-129 months). Fever (98%), vomiting (78%), abdominal pain (68%), hepatomegaly (68%), and edema (32%) were the common features. About two-thirds (N = 30) had severe dengue; 20 (45%) had dengue shock. Liver dysfunction (58%) and acute kidney injury (25%) were other major organ dysfunctions. Nineteen (43%) children stayed in the pediatric intensive care unit for a median duration of 5 days (IQR: 2-11 days). None had acute SARS-CoV2 infection; however, IgG against SARS-CoV-2 was detected in 15 (34%) cases. Children with recent exposure to SARS-CoV-2 showed a trend toward a lower incidence of acute kidney injury, fewer organ dysfunctions, and a lower frequency of invasive ventilation. Four children (9%) died; none of the deaths were in the SARS-CoV-2-exposed group. The present study exposes preliminary evidence that dengue fever might follow a less severe course in children with recent exposure to SARS-CoV-2 infection. However, it is pertinent to understand the antigenic similarity and cross-protective antibody response between the two viruses and their clinical relevance.

Neurenteric Cyst Masquerading as Acute Flaccid Paralysis in a 2-Month-Old Infant.

BACKGROUND: Neurenteric cysts rarely present in infancy. Compressive myelopathy or meningitis are the usual presenting features of these cysts in infants. CASE DESCRIPTION: We discuss a case of intradural extramedullary neurenteric cyst at the cervicomedullary junction in a 2-month-old infant who presented with features of acute onset flaccid upper limb weakness. The cyst was excised completely and the child improved. CONCLUSIONS: Although rare, compressive lesions such as neurenteric cysts may present with acute flaccid paralysis in very young children. Differentiating from other causes and timely intervention bears an excellent outcome.

Comparison of Multisystem Inflammatory Syndrome (MIS-C) and Dengue in Hospitalized Children

Objective Multisystem infammatory syndrome (MIS-C) in children is a febrile illness that has overlapping presentation with other locally prevalent illnesses. Clinicolaboratory profle of children admitted with MIS-C and dengue were compared to understand their presentation at the outset. Methods This was a retrospective study of children?12 y admitted with MIS-C (WHO defnition) or laboratory-confrmed dengue between August 2020 and January 2021 at a tertiary center in North India. Results A total of 84 children (MIS-C - 40; dengue - 44) were included. The mean (SD) age [83.5 (39) vs. 91.6 (35) mo] was comparable. Rash (72.5% vs. 22.7%), conjunctival injection (60% vs. 2.3%), oral mucocutaneous changes (27.5% vs. 0) and gallop rhythm (15% vs. 0) were seen more frequently with MIS-C, while petechiae [29.5% vs. 7.5%], myalgia (38.6% vs. 10%), headache (22.7% vs. 2.5%), and hepatomegaly (68.2% vs. 27.5%) were more common with dengue. Children with MIS-C had signifcantly higher C-reactive protein (124 vs. 3.2 mg/L) and interleukin 6 (95.3 vs. 20.7 ng/mL), while those with dengue had higher hemoglobin (12 vs. 10.2 g/dL) lower mean platelet count (26 vs. 140× 109 /L), and greater elevation in aspartate (607 vs. 44 IU/L) and alanine (235.5 vs. 56 IU/L) aminotransferases. The hospital stay was longer with MIS-C; however, PICU stay and mortality were comparable. Conclusion In hospitalized children with acute febrile illness, the presence of mucocutaneous features and highly elevated CRP could distinguish MIS-C from dengue. The presence of petechiae, hepatomegaly, and hemoconcentration may favor a diagnosis of dengue.