Scrub typhus with secondary hemophagocytic lymphohistiocytosis in a 3-month-old child from a tertiary care hospital of Odisha.

Scrub typhus is one of the re-emerging infectious diseases in India, whereas hemophagocytic lymphohistiocytosis (HLH) results from an uncontrolled and ineffective hyperinflammatory response to a variety of triggers. HLH is categorized into primary and secondary type with infection being one of the leading causes of secondary HLH. Here, we report a case of 3-month-old girl diagnosed with scrub typhus associated with secondary HLH as both the age of presentation and the association are rarely reported in literature.

Study of Cardiac Dysfunction in Portal Hypertension: A Single-Center Experience From Eastern India.

INTRODUCTION: Cardiac functional abnormalities are common in patients with cirrhosis of the liver. Nonetheless, the effect of portal hypertension and liver disorder on cardiac abnormalities is yet to be investigated. The current study evaluated the contribution of cirrhotic and non-cirrhotic portal hypertension as the potential cause of cardiac abnormalities. METHODS: The present study was a cross-sectional observational study. After excluding known heart diseases, 128 patients with portal hypertension from different causes were enrolled in the study. Cardiac functional activity was assessed by electrocardiogram (ECG) and transthoracic echocardiography (TTE).  Results: This study included a total of 128 patients, out of which 24 had extrahepatic portal vein obstruction (EHPVO), four patients had Budd-Chiari syndrome and 100 had liver cirrhosis. Normal ventricular function was observed in patients with EHPVO and Budd-Chiari syndrome. Sixty-eight percent of cases had liver cirrhosis diastolic abnormalities. The mean QTc interval in patients with cirrhotic cardiomyopathy (CCM) was 0.49 ± 0.05 sec which was significantly increased when compared to patients without CCM with 0.432 ± 0.07 at p=0.0016. The Child Turcotte Pugh (CTP) score and MELD (Model for End-Stage Liver Disease) score in patients with CCM were significantly higher as compared to patients without CCM. All alcoholic cirrhotic and non-alcoholic cirrhotic patients had equal prevalence of diastolic dysfunction (p-value >0.05). CONCLUSION:  Patients with Child class C or a high MELD score are associated with a higher prevalence rate of CCM while normal cardiac function was observed among patients having portal hypertension due to extrahepatic causes. We recommend cardiac evaluation by echocardiography in all cirrhotic patients. Institution of specific medical therapy and early referral for liver transplantation should be considered to improve survival in patients with decompensated cirrhosis.

High Myopia: A Pointer of an Inborn Error of Metabolism.

An 11-year-old boy with marfanoid habitus and high myopia presented with multiple episodes of seizures. He was found to have arachnodactyly, hypermobile joints, ectopia lentis, cerebral venous sinus thrombosis (CVST) with very high serum methionine and homocysteine. Genetic evaluation unveiled homocystinuria due to cystathionine beta-synthase deficiency. The patient was treated with high-dose pyridoxine, methionine restricted diet, anticonvulsants, warfarin, and correction of ectopia lentis. Homocystinuria should be suspected in patients with tall stature and pathological myopia. Early treatment can prevent thromboembolic complications.

Antiphospholipid Antibody Syndrome in Childhood Systemic Lupus Erythematous With a Unique Presentation: A Case Report.

Initial presentation of childhood systemic lupus erythematosus (SLE) as antiphospholipid syndrome (APS) is uncommon; moreover, APS presenting with both hemorrhage and thrombosis is very rare. We report a case of a previously healthy eight-year-old boy, without any significant past or family history, who presented with ecchymotic patches, epistaxis, and right-side hemiparesis. Investigation showed severe thrombocytopenia and isolated high activated partial thromboplastin time (aPTT) not corrected by mixing study. During his hospital stay, the child developed left-sided focal seizure and digital gangrene as thrombotic events. Neuroimaging revealed initially hemorrhagic stroke and subsequently bilateral infarct of middle cerebral artery (MCA) territory. The child was diagnosed as a case of SLE with APS based on Systemic Lupus International Collaboration Clinics (SLICC) criteria, revised APS classification, clinicoimmunological profile and neuroimaging. As the child was progressing towards catastrophic APS, he was treated aggressively with intravenous pulse methylprednisolone, intravenous cyclophosphamide and plasmapheresis with successful recovery. A simple bleeding manifestation may mask a serious disorder. A simple test like mixing study is helpful in diagnosis and in avoiding unnecessary investigations. A combination of both hemorrhage and thrombosis is an unusual presentation of APS and should always be suspected in case of autoimmune disorder, especially in SLE.

Clinical Manifestations and Outcome of Scrub Typhus in Infants From Odisha.

OBJECTIVE: To study manifestations and outcome of scrub typhus in infants. METHODS: Case record analysis of infants with scrub typhus admitted to a tertiary care hospital, diagnosed by IgM ELISA from January 1, 2016 to December 31, 2019. RESULTS: Out of 374 children diagnosed with scrub typhus, 34 (9%) were infants. Chief presentation were fever 34 (100 %), feeding difficulty 24 (70.6%), lethargy 18 (52.9%) and irritability 15 (44.1%). Clinically, pallor 30 (88.2%), tachycardia 29 (85.3%), tachypnea 24 (70.6%), hepatosplenomegaly 30 (88.2%) and eschar 6 (17.6%) were detected. Significant laboratory parameters were anemia 33 (97.1%), leukocytosis 33 (97.1%), thrombocytopenia 17 (50%) and transaminitis 21 (63.6%). Pneumonia 18 (52.9%) was noticed as the major complication. Infants requiring intensive care 17(50%) had characteristic thrombocytopenia, hypoalbuminemia and transaminitis (P<0.05). They recovered well with doxycycline. CONCLUSIONS: Manifestation of scrub typhus in infants tends to be severe with combination of hematologic, pulmonary and hepatic involvement requiring intensive care. The response to doxycycline is good.

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.

Clinical and Laboratory Profile of Enteric Fever in Children From a Tertiary Care Centre in Odisha, Eastern India.

BACKGROUND: Enteric fever is a major health problem in developing countries. Varied clinical presentation leads to diagnostic dilemmas resulting in fatal complications. OBJECTIVE:  To determine the socio-demographic, clinical manifestations, complications, antibiotic sensitivity pattern, treatment, and outcome in hospitalized enteric fever patients. METHODS: A retrospective case record analysis of hospitalized patients in the age group one to 14 years with a discharge diagnosis of enteric fever was done in a tertiary care centre of Odisha over a period of three years (January 2017 to December 2019). RESULTS: Of 112 patients, 75% of children belonged to the six to 14 years age group with a mean age of 7.6 +/- 3.6 years and a male to female ratio of 1.66:1. The peak of cases was seen during the month of January to June with 94% of cases occurring in low and middle socioeconomic status. The commonest presentation was fever in 98.21%; other features were vomiting (39.29%), pain in abdomen (21.43%), diarrhoea (26.79%), and anorexia (14.29%). Eosinopenia was found in 58.93%, transaminitis in 30.36%, and raised CRP in 73.21%. In 30 children blood culture was positive with sensitivity to third-generation cephalosporin. All isolates were nalidixic acid-resistant Salmonella Typhi (NARST). Complications were seen in 21.42%. All recovered and two left against medical advice. CONCLUSION: Enteric fever is a major threat in the paediatric age group. Early clinical diagnosis with rational use of antibiotics according to sensitivity pattern is important. Improved hygiene, vaccination, and awareness among people is necessary for prevention.

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.