Dengue Associated Demyelinating Disorders - A Report of 2 Cases.

Dengue is a common viral infection worldwide, though its neurological manifestations are infrequent (2%-11% in recent years) and can be varied as the Dengue virus per se is a non-neurotropic virus. Neurological manifestations of Dengue usually result from multisystem dysfunction which may be secondary to vascular leak or it can be due to direct virus invasion (dengue encephalitis) or an immunological phenomenon which is triggered by dengue infection (demyelinating disorders). Here we present two cases of dengue fever associated demyelinating disorders in two pediatric patients aptly depicting the two spectra of the disease. One of them is a case of fatal acute hemorrhagic leukoencephalopathy (AHLE) in a 3-year-old girl, that developed severe neurological sequelae while the other one is a case of an Acute disseminated Encephalomyelitis (ADEM) in a 3-year-old boy who had recovered with timely immunomodulatory therapy and appropriate management.

A Case Study of Dual Infection of Dengue and COVID-19: Presenting as Multiorgan Dysfunction in an Infant.

Dengue is a major health concern in South Asian countries transmitted by bite of day breeder mosquitoes Aedes aegypti and Aedes albopictus. Severity of plasma leak, shock, bleeding tendency and other organ dysfunction can be more pronounced in infants. The management becomes further complicated in the presence of a co-existing COVID-19 infection. Although COVID-19 infection is usually asymptomatic or has mild manifestations in children, however in presence of serious co-infection like dengue it can modify the course of the illness and lead to drastic consequences. Here, we present one such case of a 9-month-old female child who tested positive for dengue as well as COVID-19 during the ongoing corona pandemic and went on to develop shock, encephalopathy with deranged liver enzymes but managed to overcome all odds and recover from the disease by day 14 of illness.

Are we ignoring coexisting rhabdomyolysis as an important aggravating factor for acute kidney injury among childhood diabetic ketoacidosis?

OBJECTIVES: Although Acute Kidney Injury (AKI) has been described among childhood diabetes ketocidosis (cDKA) there is scarcity of literature on the role of concomitant rhabdomyolysis. METHOD: A retrospective chart review was undertaken (2014-2018) to identify cDKA who developed AKI and had evidence of rhabdomyolysis defined by serum creatine phosphokinase (CPK) > 5 times upper limit of normal. RESULT: 46 cDKA were identified. Ten (22%) developed AKI with 6/10 reaching peak AKI Stage 3 and 8/10 had co-current rhabdomyolysis. In comparison to non rhabdomyolysis group, cDKA with rhabdomyolysis were at presentation significantly more likely to be hypotensive and have higher corrected sodium and calculated osmolality. Subsequently they were more likely to develop lower trough potassium levels during treatment. Five patients, all with rhabdomyolysis, needed dialysis: median duration 9 days (range 4-35). Three children in our cohort died, all from infection complications during treatment, one in AKI only group who did not receive dialysis and two in AKI with rhabdomyolysis on dialysis. CONCLUSION: Rhabdomyolysis was common among our cohort of cDKA with AKI and was associated with high morbidity and mortality. Rapid flux in electrolytes and osmolality may be important precipitating factors. We recommend larger prospective studies exploring the importance of rhabdomyolysis among cDKA with AKI.

A case of post adenoviral bronchiectasis being managed at home with humidified high flow nasal cannula (HHFNC).

Human adenovirus is an important cause of febrile illness affecting mainly respiratory system ranging from pharyngitis, coryza to fatal pneumonia. Though most of the infections are trivial and results in complete recovery but it may result in considerable morbidities and mortalities in selected patients who developed severe adenoviral infections especially Pneumonia(ADVP)/Lower respiratory tract infection(LRTI). Severe adenoviral pneumonia is notorious to produce long term sequelae in the form of post infectious bronchiolitis obliterans (PIBO) or even bronchiectasis. Here we present a case of a ten months old boy developed bronchiectasis as a sequela of severe adenoviral LRTI and needed prolonged and recurrent respiratory support in the pediatric intensive care unit(PICU) and ultimately discharged on home Humidified high flow nasal cannula(HHFNC).

Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis.

OBJECTIVE: To develop a composite disease activity score for systemic JIA (sJIA) and to provide preliminary evidence of its validity. METHODS: The systemic Juvenile Arthritis Disease Activity Score (sJADAS) was constructed by adding to the four items of the original JADAS a fifth item that aimed to quantify the activity of systemic features. Validation analyses were conducted on patients with definite or probable/possible sJIA enrolled at first visit or at the time of a flare, who had active systemic manifestations, which should include fever. Patients were reassessed 2 weeks to 3 months after baseline. Three versions were examined, including ESR, CRP or no acute-phase reactant. RESULTS: A total of 163 patients were included at 30 centres in 10 countries. The sJADAS was found to be feasible and to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha 0.64-0.65), fair ability to discriminate between patients with different disease activity states and between those whose parents were satisfied or not satisfied with illness outcome (P < 0.0001 for both), and strong responsiveness to change over time (standardized response mean 2.04-2.58). Overall, these properties were found to be better than those of the original JADAS and of DAS for RA and of Puchot score for adult-onset Still's disease. CONCLUSION: The sJADAS showed good measurement properties and is therefore a valid instrument for the assessment of disease activity in children with sJIA. The performance of the new tool should be further examined in other patient cohorts that are evaluated prospectively.

Macrophage activation syndrome in pediatrics: 10 years data from an Indian center.

AIMS: Macrophage activation syndrome (MAS) is a dreaded complication of systemic inflammatory diseases and is most commonly seen in systemic juvenile idiopathic arthritis (sJIA). We evaluate the clinical features, laboratory findings and outcomes in pediatric MAS, assess the response to different pharmacological therapies, and finally identify possible factors associated with an unfavorable outcome. METHODS: This is a retrospective analysis of data from patients diagnosed as having MAS, admitted between July 2008 and April 2018 into the Department of Pediatric Rheumatology, Institute Of Child Health Kolkata. The data noted were the clinical and laboratory features, treatment details, responses to therapy and outcomes. RESULTS: Thirty-one patients were diagnosed as having MAS. Primary illness was sJIA in 26 (84%), systemic lupus erythematosus in 4 (13%) and Kawasaki disease (KD) in 1 (3%). All had fever with varying degrees of multisystemic involvement. Hyperferritinemia was universally present. Pulse methylprednisolone with cyclosporine was used for treating the majority. Ten patients (32%) expired. CONCLUSION: Macrophage activation syndrome is a near fatal complication with protean manifestations and multiorgan dysfunction. Hyperferritinemia is characteristic, higher values being associated with increased mortality. Cases resistant to steroids and cyclosporine had a poor prognosis. Late presentations with multiorgan dysfunction were associated with the poorest outcomes.

Life-threatening Episodes of Malignant Hyperthermia Following Halothane Anesthesia in Three Children: A Case Series and Review of Literature.

Malignant hyperthermia (MH) is an inherited, pharmacogenetic disorder of the skeletal muscle, characterized by dangerous hypermetabolic state after anesthesia with succinylcholine and/ or volatile halogenated anesthetic agents, clinically manifested as hyperpyrexia and related complications like tachycardia, tachypnea, increased carbon dioxide production, increased oxygen consumption, acidosis, rigid muscles, rhabdomyolysis and disseminated intravascular coagulation (DIC). Here we present a series of three cases of MH, admitted in our hospital in a span of 8 months for three different operative procedures to be done under general anesthesia (cleft lip repair, Duhamel's operation for Hirschsprung's disease and surgical repair of development dysplasia of hip), who developed probable hyperthermia owing to Halothane being used as an anesthetic agent. HOW TO CITE THIS ARTICLE: Laha S, Giri PP, Saha A, Gupta PP, De A. Life-threatening Episodes of Malignant Hyperthermia Following Halothane Anesthesia in Three Children: A Case Series and Review of Literature. Indian Journal of Critical Care Medicine, January 2019;23(1):47-50.

Cyclosporine in Resistant Systemic Arthritis - A Cheaper Alternative to Biologics.

OBJECTIVES: To assess the efficacy of cyclosporine (CsA) in patients of oral steroid unresponsive or steroid dependent systemic juvenile idiopathic arthritis (sJIA); to evaluate the optimum dosage and blood level of CsA to achieve and maintain remission and to observe for side-effects on prolonged usage. METHODS: This prospective observational study was conducted on children with steroid dependent /refractory sJIA admitted at the Institute of Child Health, Kolkata from July 2009 through November 2014. A total of 82 sJIA was diagnosed; 15 were steroid dependent /refractory and were included as candidates for cyclosporine therapy. RESULTS: CsA was used in 15 patients; 13 showed a favourable response with significant steroid sparing effect and minimal toxicity. CONCLUSION: CsA was found to be effective in almost 75% of frequently relapsing steroid dependent sJIA to achieve and maintain remission. The average cost of therapy for a 20 kg patient on CsA was found to be 10,000 INR (132 EURO)/ patient over a 6 mo period; which would amount to 100,000 INR (1318 EURO)/patient with Tocilizumab for the same duration.

Early Initiation of Steroid-sparing Drugs in Idiopathic Pulmonary Hemosiderosis.

Idiopathic pulmonary hemosiderosis is conventionally treated with steroids, prolonged usage of which maybe deleterious and disease often recurs on tapering. We initiated hydroxy-chloroquine and azathioprine early in treatment along with steroids in seven children with idiopathic pulmonary hemosiderosis, and observed that early introduction of second line immunosuppressants helped in reducing disease flare and steroid toxicity without serious adverse effects.

Cervical Myelopathy in a Child: A Rare Cause of Hypoventilation Syndrome Presenting with Type 2 Respiratory Failure.

Hypoventilation syndrome leading to Type 2 respiratory failure is not a rare cause of Pediatric Intensive Care Unit admission and mechanical ventilation. Common causes in pediatric population are Guillain-Barre syndrome and various central nervous system disorders such as encephalitis, traumatic brain injury, and drugs. Any injury or disease in the cervical cord can also produce respiratory paralysis causing respiratory failure. Here, we present two cases of mixed cerebral palsy with cervical myelopathy due to compression effect of fractured segments of first and second cervical vertebrae. Both of them presented with Type 2 respiratory failure.

A Young Child with Eosinophilia, Rash, and Multisystem Illness: Drug Rash, Eosinophilia, and Systemic Symptoms Syndrome After Receipt of Fluoxetine.

Drug rash, eosinophilia, and systemic symptoms (DRESS) syndrome is a severe systemic hypersensitivity reaction that usually occurs within 6 weeks of exposure to the offending drug. Diagnosis is usually straightforward in patients with pyrexia, skin rash, hepatitis, and eosinophilia with a preceding history of exposure to agents often associated with DRESS syndrome, such as aromatic anticonvulsants and sulfa drugs, but diagnosis of DRESS may still be a challenge. We report a 4-year-old child with probable DRESS syndrome complicated by multiple hematologic complications that developed 1 month after exposure to fluoxetine, a drug not known to be associated with such severe reactions.

Two cases of non-cystic fibrosis (CF) bronchiectasis with allergic bronchopulmonary aspergillosis.

Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction in patients with asthma or cystic fibrosis (CF), which is associated with bronchi colonized by the fungus Aspergillus species, most often Aspergillus fumigatus. ABPA is an important consideration for asthmatics that do not respond to asthma management or with recurrent chest infections and deteriorating lung function in children with cystic fibrosis. We present two cases of non CF bronchiectasis associated with ABPA who presented to our hospital with recurrent hospitalisations of undiagnosed aetiology.

Acute carbolic acid poisoning: A report of four cases.

Phenol (carbolic acid) is one of the oldest antiseptic agents. Apart from being used in many commercially available products, in rural India, it is often used in the household to prevent snake infestation. We hereby present four cases of acute carbolic acid poisoning which we saw over the last monsoon. The cases highlight the multiple routes of absorption of carbolic acid as well as its multi-organ complications which often necessitate intensive care of local therapy decreases systemic phenol absorption from cutaneous exposure and may help in reducing severity.

Acute disseminated encephalomyelitis: A clinical and neuroradiological profile of pediatric patients.

OBJECTIVES: To discuss the clinical and neuroradiological profile of pediatric patients with acute disseminated encephalomyelitis (ADEM). DESIGN: Prospective observational study Settings: Children with clinico-radiological diagnosis of ADEM admitted in the pediatric ward of the Institute of Child Health between July 2009 to Sepetember 2014 were included. MATERIALS AND METHODS: Clinical and neuroradiological parameters of children suffering from ADEM were noted. RESULTS: A total of 36 children were included in the study, among whom approximately 50% had a history of either a preceding viral infection or the administration of a vaccination; and, 92% of them presented with some form of neurological deficits. Encephalopathy was present in 92% children at the time of admission. Seizures were present in 42% of children. The cerebrospinal fluid (CSF) study was non-contributory and magnetic resonance imaging (MRI) was diagnostic in most of the cases. The lesions were usually asymmetrically placed. All of them were treated with steroids and had a good prognosis, with complete neurological recovery in 75% of the children. CONCLUSION: Although it is perceived as a rare disease, ADEM is fairly common in the pediatric population, Most often, it is misdiagnosed as a meningoencephalitis. An early neuroradiological identification of the demyelinating lesions and treatment with steroid therapy leads to an excellent outcome.

Clinical Presentation of Inadvertent Intrathecal Vincristine Masquerading Guillain-Barre Syndrome.

Vincristine, a potent chemotherapeutic agent, is highly neurotoxic. If given intrathecally by accident it is almost always fatal. We are reporting a 6 year old girl with acute lymphoblastic leukaemia in complete remission, who was given inadvertent intrathecal Vincristine instead of Methotrexate. She developed gradually progressive quadriplegia and respiratory paralysis requiring prolonged mechanical ventilation, initially mimicking Guillain-Barre Syndrome, both clinically and electro-physiologically. She also developed progressive encephalopathy. The clinical deterioration subsequently plateaued without any significant improvement and after more than 5 months, she finally expired.

Familial Hemophagocytic Lymphohistiocytosis due to Mutation of UNC13D Gene.

Hemophagocytic lymphohistiocytosis (HLH) are basically a heterogenous group of clinical syndromes, characterised by activation and non-malignant proliferation of benign histiocytes i.e. lymphocytes and macrophages, leading to a cytokine storm that accounts for the fever, organomegaly and multi-organ dysfunction. Two types of HLH are described, either due to known genetic defect (familial HLH/FHL) or due to some acquired cause either infection or rheumatological diseases. Here we present a case of a 3 months old baby, admitted with fever, hepatosplenomegaly and cytopenia and ultimately was diagnosed to be a case of Familial HLH type 3 due to defect in UNC13D gene as a result of compound heterozygous for two nonsense mutation resulting in the Munc13-4 protein defect.

Dengue associated hemophagocytic lymphohistiocytosis: a case series.

Hemophagocytic lymphohistiocytosis is a rare complication of dengue. We present 8 cases of dengue associated hemophagocytic lymphohistiocytosis diagnosed in our hospital during the dengue outbreak of 2012. All the cases were treated with a short (4 weeks) course of steroids along with supportive measures, and showed an excellent response.