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.

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.

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.

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.

Infection-associated haemophagocytic lymphohistiocytosis: a case series using steroids only protocol for management.

Haemophagocytic lymphohistiocytosis (HLH) is a heterogeneous group of clinical syndromes characterised by activation and subsequent uncontrolled non-malignant proliferation of T lymphocytes and macrophages, leading to a cytokine storm that accounts for most of its clinical features such as acute febrile illness, hepatosplenomegaly, multi-organ dysfunction and fulminant pancytopenia-resembling severe sepsis. Here, we present a series of 23 cases of infection-associated HLH diagnosed in our hospital within a time period of last three and half years. Though the presentation and progression of disease was variable, the patients shared some common features like prolonged fever unresponsive to broad spectrum antibiotics, organomegaly and cytopenias. In most of the cases, however, the triggering infectious agent could not be identified. They were treated using a steroid only protocol along with supportive measures and showed an excellent response.

Orange-brown chromonychia, a novel finding in Kawasaki disease.

Kawasaki disease (KD) is one of the commonest vasculitis of childhood, where diagnosis is clinical based on a plethora of signs and symptoms. One of the typical findings is the changes in the extremities including the nail changes. Orange-brown chromonychia is a colour change in the nails which has been observed in some cases of KD. Here, we report a series of 40 patients of KD, where a typical transverse orange-brown discolouration of nails or chromonychia was noted in 29 patients. Though chromonychia is noted in many other rheumatic and nonrheumatic diseases, the typical transverse orange-brown chromonychia observed in KD patients can be included as an additional clinical feature in diagnosis of KD.

Infantile systemic hyalinosis.

Infantile systemic hyalinosis is a rare disorder characterized by widespread deposition of hyaline. They usually present with skin lesions, joint contractures, and intractable diarrhea. We report a 2 year 4 month old boy with growth retardation, typical facial appearance, gingival enlargement, generalized stiff skin, joint contractures, and intermittent diarrhea. Skin biopsy revealed deposition of hyaline.

Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum.

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis) after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS) and spontaneous air leak syndrome (pnemothorax and pneumomediastinum). She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.