Loeys-Dietz syndrome and Goldenhar syndrome unveiled together.

Haemifacial microsomia is an asymmetrical congenital tissue malformation developed from the first and second branchial arches with or without multi-system involvement. Alternatively recognised as Goldenhar syndrome or oculoauriculovertebral spectrum (OAVS), it is an aetiologically heterogeneous group of disorders showing dominant trends in inheritable form.We present a case of a boy in early childhood with concomitant craniofacial features of craniofacial microsomia with Loeys-Dietz syndrome. He had a unilateral hypoplastic face, asymmetrical ear malformations and multiple preauricular tags with epibulbar dermoid (features suggestive of Goldenhar syndrome). On detailed clinical evaluation, he met Beighton's criteria and was diagnosed with arterial tortuosity. Further molecular testing confirmed the diagnosis of Loeys-Dietz syndrome type II.Loeys-Dietz syndrome is characterised by aortic root enlargement or type A dissection with or without other vascular malformations and facial midline defects. Molecular testing is required to establish the diagnosis because of overlapping features with other connective tissue disorders.

Clinical characteristics, genetic profile and short-term outcomes of children with primary hyperoxaluria type 2: a nationwide experience.

BACKGROUND: Three types of primary hyperoxaluria (PH) are recognized. However, data on PH type 2 (PH2), caused by defects in the GRHPR gene, are limited. METHODS: We reviewed the medical records of patients < 18 years of age with genetically-proven PH2 from seven centres across India to identify the age of onset, patterns of clinical presentation, short-term outcomes and genetic profile, and to determine if genotype-phenotype correlation exists. RESULTS: We report 20 patients (all with nephrolithiasis or nephrocalcinosis) diagnosed to have PH2 at a median (IQR) age of 21.5 (7, 60) months. Consanguinity and family history of kidney stones were elicited in nine (45%) and eight (40%) patients, respectively. The median (IQR) serum creatinine at PH2 diagnosis was 0.45 (0.29, 0.56) mg/dL with the corresponding estimated glomerular filtration rate being 83 (60, 96) mL/1.73 m2/min. A mutational hotspot (c.494 G > A), rare in Caucasians, was identified in 12 (60%) patients. An intronic splice site variant (c.735-1G > A) was noted in five (25%) patients. Four (20%) patients required surgical intervention for stone removal. Major adverse kidney events (mortality or chronic kidney disease (CKD) stages 3-5) were noted in six (30%) patients at a median (IQR) follow-up of 12 (6, 27) months. Risk factors for CKD progression and genotype-phenotype correlation could not be established. CONCLUSIONS: PH2 should no longer be considered an innocuous disease, but rather a potentially aggressive disease with early age of presentation, and possible rapid progression to CKD stages 3-5 in childhood in some patients. A mutational hotspot (c.494 G > A variant) was identified in 60% of cases, but needs further exploration to decipher the genotype-phenotype correlation.

Thurston syndrome with thalassaemia: a rare case devising a novel molecular and phenotypic variation.

A male infant presented with progressive paleness of the body since 3 months of age. On examination, the child had pallor, microcephaly with dysmorphic facies (depressed nasal bridge, low set ears, retrognathia, high arched palate and tongue hamartoma). Postaxial polydactyly in bilateral hands and feet, broad great toes, with syndactyly of left fourth and fifth toes were present. The haemogram showed severe anaemia with a microcytic hypochromic picture. High-performance liquid chromatography (HPLC) was normal. However, the parents' HPLC was suggestive of beta thalassaemia trait. Whole-exome sequencing revealed Thurston syndrome with beta-thalassaemia in homozygous pattern with a novel mutation. It is a rare genetic syndrome exclusively found in the South Asian population. Due to the rarity, identification of this syndrome is often difficult and requires awareness among clinicians. However, it is important to diagnose the disorder accurately in order to provide appropriate genetic counselling and prognostication to the parents.

Acute liver failure due to hepatitis A virus presented with Guillain-Barré syndrome and ocular myasthenia gravis.

Hepatitis A is one of the most common causes of acute viral hepatitis in children. Immunological manifestations involving the nervous system are rare with hepatitis A infection. We report a case of a toddler who presented with acute liver failure secondary to hepatitis A infection. The child showed clinical and laboratory improvement initially with conservative management. However, after the initial improvement, she developed acute-onset ptosis along with areflexia. Serological and neurophysiological tests revealed the occurrence of ocular variant Guillain-Barré syndrome and ocular myasthenia gravis, which was only partially responsive to treatment (intravenous immunoglobulin and pyridostigmine). A sudden clinical deterioration was noted after the onset of ptosis. She succumbed on day 40 of hospitalisation due to hospital-acquired infection along with the primary hepatic pathology. This is a rare coincidental presentation of acute viral hepatitis A infection with autoimmune manifestations.

Functional Duration of Peripheral Intravenous Cannula in Neonates with or without Splint: A Randomized Controlled Trial.

OBJECTIVES: To determine the effect of splint on the functional duration of peripheral intravenous cannula (PIVC) in neonates. METHODS: The trial was prospectively registered with the Clinical Trial Registry of India (CTRI/2021/09/036337). One-hundred-fifty cannulations in 71 neonates were randomized to splint (n = 75) and no-splint (n = 75) groups, respectively. The median (interquartile range, IQR) functional duration of PIVC was calculated from the time of PIVC insertion till removal due to the development of signs of PIVC failure or treatment completion. Kaplan-Meier survival analysis was used to compute the time to complication of PIVC. Complications related to PIVC were noted and multivariate Cox-proportion hazard analysis was done to find the predictors associated with PIVC failure. RESULTS: Median (IQR) functional duration of PIVC in the splint and the no-splint group was 28 (23-48) and 30 (25-48) h, respectively (p = 0.477). PIVC duration was higher in the splint group in term neonates and the no-splint group in preterm neonates; however, the differences were not statistically significant. No difference was observed in continuous vs. intermittent infusion subgroups. Time to complication development was also comparable between the groups. CONCLUSIONS: Splint application did not affect functional PIVC duration and its related complications in neonates.

Point-of-Care Serum Amyloid A as a Diagnostic Marker for Neonatal Sepsis.

OBJECTIVES: To evaluate diagnostic accuracy of point-of-care Serum Amyloid A (POC-SAA) and its comparison with procalcitonin for diagnosis of neonatal sepsis. METHODS: The present diagnostic accuracy study consecutively recruited neonates with suspected sepsis. Blood samples for sepsis screen, culture, high sensitivity C-reactive protein (CRP) (hs-CRP, as a part of sepsis screen), procalcitonin and POC-SAA were collected before starting antibiotics. The optimum cut-off level of biomarkers (POC-SAA and procalcitonin) was determined by receiver-operating-characteristics curve (ROC) analysis. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of POC-SAA and procalcitonin were derived for 'clinical sepsis (neonates with suspected sepsis and either positive sepsis screen and/or blood culture)' and 'culture positive sepsis' (neonates with suspected sepsis and positive blood culture). RESULTS: Seventy-four neonates with mean±SD gestational age of 32.8±3.7 wk were evaluated for suspected sepsis, of which the proportion of 'clinical sepsis' and 'culture positive sepsis' was 37.8% had 16.2%, respectively. At a cut-off of 25.4 mg/L, POC-SAA had sensitivity, specificity, PPV and NPV of 53.6%, 80.4%, 62.5% and 74.0%, respectively for diagnosis of clinical sepsis. The sensitivity, specificity, PPV and NPV of POC-SAA for detection of culture positive sepsis were 83.3%, 61.3%, 29.4% and 95.0%, respectively at a cut-off of 10.3 mg/L. There was no significant difference in the diagnostic accuracy of biomarkers for detection of culture positive sepsis (area under the curve, AUC of POC-SAA vs. procalcitonin vs. hs-CRP: 0.72 vs. 0.85 vs. 0.85; p = 0.21). CONCLUSIONS: POC-SAA is comparable to procalcitonin and hs-CRP for diagnosis of neonatal sepsis.

Association between neutrophil lymphocyte ratio and status of symptom control in children and adolescents with bronchial asthma.

Neutrophil lymphocyte ratio (NLR), an easy and readily available biomarker of systemic inflammation, has been less studied so far as a putative marker of asthma control. Our study aimed to assess its feasibility. A total of 90 asthmatic children, aged 5-18 years, diagnosed according to Global Initiative for Asthma (GINA) guidelines, were. Control status of asthma was assessed using the asthma control test (ACT) or childhood ACT and categorized as controlled group-1 (ACT > 19) and uncontrolled group-2 (ACT ≤ 19). The difference between mean values in both groups was analysed, finding a significant difference between children with and without a family history (p = 0.004) and those with and without a need for admission (p = 0.045). Also, a significant association was established between NLR and the type of severity of asthma (p = 0.049), but none between NLR and age, gender, BMI, coexisting allergic rhinitis, or asthma exacerbation. Thus we found no significant association between NLR and symptom control status. However, NLR has the potential to be a putative marker of inflammation, although its relative status to CRP needs further studies.

Impact of Delivery Room Gastric Lavage on Exclusive Breastfeeding Rates Among Neonates Born Through Meconium-Stained Amniotic Fluid: A Randomized Controlled Trial.

BACKGROUND: Delivery-room gastric lavage reduces feeding intolerance and respiratory distress in neonates born through meconium-stained amniotic fluid (MSAF). OBJECTIVES: To evaluate the effects of gastric lavage on exclusive breastfeeding and skin-to-skin contact in neonates delivered through MSAF. DESIGN: Randomized controlled trial. PARTICIPANTS: 110 late preterm and term neonates delivered through MSAF not requiring resuscitation beyond initial steps. METHODS: Participants randomized into gastric lavage (GL) (n=55) and no-GL (n=55) groups. The primary outcome was the rate of exclusive breastfeeding at 72±12 hours of life. Secondary outcomes were time to initiate breastfeeding and establish exclusive breastfeeding, rate of exclusive breastfeeding at discharge, time to initiate skin-to-skin contact and its duration, rates of respiratory distress, feeding intolerance, and the procedure-related complications of gastric lavage monitored by pulse oximetry and videography. RESULTS: Both the groups were similar in baseline characteristics. 49 (89.1%) neonates in GL group could achieve exclusive breast-feeding at 72 hours compared to 48 (87.3%) in no-GL group [RR (95% CI) 1.02 (0.89-1.17); P=0.768]. Initiation of skin-to-skin contact was significantly delayed and the total duration was significantly less in GL group compared to no-GL group. No difference in respi-ratory distress and feeding intolerance was observed. Procedure-related complications included retching, vomiting, and mild desaturation. CONCLUSION: Gastric lavage did not help to establish exclusive breastfeeding, delayed the initiation of skin-to-skin contact in delivery room and reduced its total duration. Moreover, the procedure of gastric lavage was associated with neonatal discomfort.

Prevalence, severity, and predictors of malnutrition in Indian children with cerebral palsy and their impact on health-related quality of life.

Although several studies have shown that undernutrition is frequent in children with cerebral palsy (CP), studies determining predictors of undernutrition and its impact on health-related quality of life (HRQoL) are scarce. This study aimed to assess the prevalence, severity, and predictors of malnutrition in children with CP and its impact on quality of life. This prospective study was performed between August 2019 and December 2021 in children with a clinical diagnosis of CP aged 2-18 years. We also intended to determine the socio-demographic and clinical predictors of undernutrition in these children and its impact on HRQoL, measured by the cerebral palsy quality of life (CPQoL)-Primary Caregiver reported version. Out of 569 (5.4 ± 2.8 years of age, 74% boys) children with CP, 71%, 44%, and 72% children were underweight, wasted, and stunted respectively, whereas 22%, 11%, and 21% were severely underweight, wasted and stunted respectively. Lower socioeconomic status, higher Gross Motor Function Classification System, and Manual Ability Classification System level were found to be significantly associated with the severity of stunting and underweight (p < 0.05), but not with wasting. CPQoL score in children with CP aged > 4 years was lower in patients with severe wasting, stunting, and underweight, as compared to their rest of the counterparts when adjusted for socio-demographic and other clinical variables (p < 0.05).  Conclusion: Chronic undernutrition is more common than severe acute malnutrition in children with CP. The severity of undernutrition is an important predictor of impaired HRQoL in children with CP. What is Known: • Several studies have shown that undernutrition is frequent in children with cerebral palsy; however, studies determining predictors of undernutrition and its impact on health-related quality of life are scarce. What is New: • Our study identifies that lower socioeconomic status, higher Gross Motor Function Classification System, and Manual Ability Classification System level are significantly associated with the severity of stunting and being underweight. • Chronic undernutrition is more common than severe acute malnutrition in children with cerebral palsy. Its severity is an important predictor of impaired health-related quality of life in children with cerebral palsy.

Clinico-Microbiological Profile and Clinical Predictor of Urinary Tract Infection in Children: A Single-Center Study From Himalayan Foothills.

BACKGROUND:  Urinary tract infection (UTI) in children is one of the commonest bacterial infections seen in the pediatric population. Clinical presentation ranges from fever with or without focus and isolation of microbiological agents streamline the treatment. Moreover, local/regional microbial profiles are helpful in antibiotic selection, we conducted a study to assess the prevalence of urine culture positivity in a suspected case of UTI. In addition, antibiotic susceptibility patterns and ultrasonography (USG) finding in culture-positive patients were also studied. METHODS AND MATERIALS: It is a prospective observational study comprising symptomatic children aged one month to 18 years presenting to the outpatient department (OPD), inpatient department (IPD), and the emergency department of Pediatrics with UTI during the period of September 2019 to September 2020. The recorded variables were demographic, clinical presentation, anthropometry, physical examination, blood biochemistry, and outcome. Urine samples were collected and processed as per standard protocols. USG was done for all culture-positive children. Data were presented as frequency, mean (SD) and parametric and non-parametric data were analyzed by Wilcoxon-Mann-Whitney U Test, Chi-Squared Test, or Fisher's Exact Test.  Results: Of the total 354 children, 202 (57.1%) were male and the prevalence of UTI was 64 (18.1%). E. coli (70.3%) was the commonest isolated organism followed by Klebsiella spp (15.6%) and Pseudomonas spp (7%) respectively. The mean (SD) age (months) of presentation of symptoms was significantly lower in culture-positive children as compared to [ 83.49 (58.96) vs 110.10 (58.60); p=0.001] culture-negative children. Fever (96.6%) followed by dysuria (20.1%) were the most common symptoms presented for UTI however dysuria (p=0.003), pus cells (p<0.0001), and RBCs (p=0.002) were significantly present in culture positive children. This study shows increased resistance to third generation of cephalosporins. This study revealed significant differences among various groups (organism growth in positive culture) and the Antibiotic susceptibility test (AST) with a p-value of <0.001.  Conclusion: The prevalence of culture-positive UTI was similar to the reported literature and the presence of fever, dysuria, pus cells, and RBC in urine were commonly observed in the lower age group. Amikacin can be used in suspected UTIs with cephalosporin as empirical antibiotics in the Himalayan Foothills region.

Association of serum zinc levels and symptom control of asthma in children and adolescents- a prospective observational study.

Dysregulation of zinc (Zn) homeostasis causes a shift in the Th1/Th2 balance towards a Th2 response, which may lead to a heightened inflammatory response. Asthma is associated with an exaggerated Th2 response to antigens. This study attempts to find the association of serum Zn with the status of symptom control of asthma in children and adolescents with bronchial asthma. A total of 67 asthmatic children, diagnosed as per Global Initiative for Asthma (GINA) 2019 guidelines, were included in the study. Symptom control of asthma was assessed by Asthma Control Test (ACT) and Childhood Asthma Control Test (C-ACT) scores. Spirometry was performed on those participants who were able to perform satisfactorily. Serum Zn was analyzed using the photometric method. Participants were divided into two groups: controlled and uncontrolled groups according to ACT/C-ACT score. Mean age of the participants was 10.78 ± 3.67 years. The mean S. Zn (µg/dL) was 136.97 ± 48.37. This study found a higher mean S. Zn value in the controlled asthma group as compared to the uncontrolled group (158.06 vs 129.23, p = 0.006). At a cutoff of S. Zn (µg/dL) ≥ 126.84, it predicted controlled asthma with a sensitivity of 89% and a specificity of 55%. No significant difference was found between the mean serum Zn levels in terms of age, sex, severity, and CRP levels. CONCLUSION: A significant difference was observed between the mean value of Zn and symptom control of asthma (p = 0.006) with a weak positive correlation between the two which was statistically significant (rho = 0.26, p = 0.031). However, low levels of zinc were not significantly associated with symptom control of asthma. Thus, we conclude that maintaining an adequate zinc level could help in achieving better control of asthma in pediatric populations. WHAT IS KNOWN: • Zinc has a role in immunological response in the pathophysiology of immunological disorders such as bronchial asthma. WHAT IS NEW: • This study adds a significant association of serum zinc levels with symptom control of asthma in pediatric populations. • This study also gives a cut-off value of serum zinc level which predicts adequate symptom control of asthma.

Development and Validation of a Prognostic Model and Bedside Score for the Neurological Outcome in Children with Tuberculous Meningitis.

Only a few studies have explored prognostic factors for tuberculous meningitis (TBM) in children, and an easily applicable bedside prognostic score for TBM has not been developed yet. We compared the sociodemographic, clinical, radiological, and cerebrospinal fluid parameters in the cohort of 94 TBM cases aged 1 to 18 years, with at least 6 months of completed follow-up and determined the prognostic factors associated with poor functional outcome. We assessed our proposed prognostic model using both discrimination and calibration and subsequently used the bootstrap method to validate the model internally. We finally derived an easily applicable bedside prognostic score by rounding off the regression coefficients to the nearest integers. A total of 39 (41%) and 55 (59%) patients had poor and good functional outcomes, respectively, at the end of 6 months (12 died, 13%). In multivariate analysis, a high baseline Pediatric Cerebral Performance Category (PCPC) score, brain infarction in neuroimaging, tonic motor posturing, younger age, and stage III TBM were independent predictors of poor functional outcomes. The final model showed good discrimination (area under the curve = 88.2%, P < 0.001) and good calibration (Hosmer-Lemeshow test, P = 0.53). Bootstrapping also confirmed the internal validity of this model. The PITAS (PCPC score [P], brain infarction in neuroimaging [I], tonic motor posturing [T], age [A], and stage of TBM [S]) score developed from this model has a score ranging from 0 to 12, with a higher score predicting a higher risk of poor functional outcome. The PITAS score performed better than medical research council staging alone in predicting poor functional outcomes (area under the curve = 87.1% versus 82.3%). Our study's PITAS score, developed and internally validated, has good sensitivity and specificity in predicting poor functional outcomes in pediatric TBM cases at 6 months.

Performance of a pediatric adaptation of the RITE2 and APE2 scores in children with autoimmune epilepsy: P-RITE2 and P-APE2 scores.

INTRODUCTION: In adult patients with epilepsy, predictive models have been developed and validated for anticipating a favorable response to immunotherapy. However, no such model has been evaluated in children. METHODS: This retrospective cohort study intended to assess the performance of a pediatric adaptation of the Response to Immunotherapy in Epilepsy (RITE2) score: P-RITE2 score and Antibody Prevalence in Epilepsy (APE2) score: P-APE2 score in patients aged 1-18 years. We included data of those patients who had epilepsy duration of not more than 12 months, no other known etiology (e.g., genetic, metabolic, neoplastic, or structural causes), and tested for neural-specific antibody in cerebrospinal fluid or serum for P-APE2 score and only those who received immunotherapy for P-RITE2 score. We added cognitive dysfunction, speech dysfunction, sleep disturbance, and movement disorder to the original scores to increase specificity for pediatric autoimmune epilepsy. We assumed at least a 50% reduction in seizure frequency at 6 months as a favorable response to immunotherapy. Cut-offs were chosen for both scores to maximize true positives and minimize false negatives using ROC curves. RESULTS: We included data from a total of 237 patients with epilepsy (10.4 ± 2.5 years, 129 boys, 54%), out of which, 25 (10.5%, 13 girls, 52%) tested positive for autoantibodies. The median P-APE2 score in the subgroup with and without antibody positivity were 7 (IQR: 5-11) and 2 (IQR: 1-5), respectively (p<0.0001). ROC analysis of the P-APE2 score determined an AUC of 0.96. The sensitivity and specificity values of the P-APE2 score ≥6 were 94% and 92%, respectively. A total of 162 patients (10.3 ± 2.5 years, 88 boys, 54%) received immunotherapy, out of which, 101 had a favorable response at 6 months. The median P-RITE2 score in the subgroup with and without favorable response following a trial of immunotherapy were 10 (IQR: 6-17) and 3 (IQR: 1-6), respectively (p<0.0001). ROC analysis of the P-RITE2 score determined an AUC of 0.96. The sensitivity and specificity values of P-RITE2 score ≥8 were 95% and 93%, respectively. The AUC of both these ROCs was significantly higher than the AUC of ROCs for original scores in our cohort. CONCLUSION: The P-RITE2 and P-APE2 scores can be used to predict the response to immunotherapy and predict autoantibody positivity in children with epilepsy with/without encephalopathy or cognitive dysfunction.

Comparison of Seizure Characteristics and Recurrence Rates in Children with Viable Parenchymal Neurocysticercosis and Tuberculoma in India.

Inflammatory granulomas (neurocysticercosis [NCC] and tuberculomas) are important causes of seizures in children and adults in the developing world. Although several studies have explored seizure characteristics individually in patients with either NCC or tuberculoma, none has compared the seizure recurrence rate between them. This study included patients aged 5 to 18 years with viable parenchymal NCC or tuberculomas who had completed regular follow-up of at least 12 months at a tertiary institute in India. Their baseline seizure and electroencephalographic characteristics, antiseizure medications (ASMs), and breakthrough seizure rates at 12, 24, and 52 weeks were noted. A total of 103 and 54 patients with active NCC and tuberculomas respectively were included. The number of patients who had at least one breakthrough seizure at 12, 24, and 52 weeks in both groups was comparable (P = 0.32, 0.27, and 0.13), and the vast majority were controlled on monotherapy (98% cases in each group). The proportion of patients who required an increase in the dose or change of ASMs or polytherapy, the proportion of children who had status epilepticus at or before 12 months, abnormal electroencephalogram at 12 months, and calcified and active granuloma in neuroimaging at 12 months were also comparable between the two groups (P > 0.05 for all). The number of patients who had ASM-related adverse events and discontinued ASM due to serious adverse events was comparable between both groups, except for hepatotoxicity in the tuberculoma group. The predictors for breakthrough seizures that were found to be statistically significant in the NCC group were the presence of perilesional edema in the baseline magnetic resonance imaging (P = 0.02) and more than five active granulomas (P = 0.01); predictors in the tuberculoma group were the presence of severe perilesional edema causing midline shift in the baseline magnetic resonance imaging (P = 0.01) and more than five active granulomas (P = 0.04). The recurrence rates of breakthrough seizures over the next 12 months in newly detected cases of active NCC and tuberculomas were comparable.

Feasibility, Utility, and Interrater Reliability of the Cluster Headache Severity Scale, Cluster Headache Quality of Life, Cluster Headache Index, and 6-Item Headache Impact Test in Pediatric Cluster Headache.

Introduction: Cluster headache is rare in children and only a few clinical studies have systematically evaluated cluster headache in children. Methods: This study was conducted between August 2019 and December 2021 with the primary aim to evaluate the feasibility and utility of the Cluster Headache Severity Scale in determining the severity of cluster headache in patients aged less than 18 years and monitoring response to prescribed treatment. Secondary objectives were to evaluate the feasibility and utility of Cluster Headache Quality of Life, Cluster Headache Index, and 6-item Headache Impact Test in pediatric cluster headache patients to assess the quality of life, severity, and impact of cluster headache. Results: A total of 32 children (age of onset 11.9 ± 2.3 years, age of diagnosis 13.7 ± 2.4 years, 68% boys) were enrolled. Although 30 cases had their headache episodes occurring during nighttime, only 16 children had a Children's Sleep Habits Questionnaire (CSHQ) score >41 at baseline. All children responded to prednisolone as bridging therapy and 23 of 32 showed adequate pain relief after sumatriptan nasal spray for an acute attack. The average time taken for completion of Cluster Headache Index, Cluster Headache Severity Scale, Cluster Headache Quality of Life, and Headache Impact Test-6 scores were 5.2 ± 0.7, 5.1 ± 0.8, 27.4 ± 3.5, and 6.2 ± 0.8 minutes, respectively. The interrater reliability was good for Cluster Headache Severity Scale, Cluster Headache Quality of Life, and Headache Impact Test-6 (Cronbach α 0.93, 0.81, and 0.89, respectively). There was a strong positive correlation between the Cluster Headache Severity Scale score with Headache Impact Test-6 score and Cluster Headache Quality of Life score (correlation coefficient r = 0.90 and 0.98). Conclusion: Majority of pediatric cluster headache patients are likely to respond to prednisolone and sumatriptan. Cluster Headache Severity Scale, Cluster Headache Quality of Life, and Headache Impact Test-6 can be used for pediatric cluster headache patients for treatment monitoring.

A Prospective Observational Study of Hepatic Dysfunction in Children on Antitubercular Drugs.

The frequency, risk factors, and prognosis of antitubercular drug-induced liver injury (TB-DILI) was assessed in this prospective observational study. All consecutive children < 18 y put on antitubercular therapy (ATT) for pulmonary or extrapulmonary tuberculosis between July 2019 and December 2020 were included. Liver function tests (LFTs) were done at baseline and at 2, 4, 6 wk, and then 2 monthly after initiation of therapy till completion of ATT regimen. A total of 81 children [14.27 ± 3.38 y, 34 (42%) males] were included. Out of the patients enrolled, 10 (12.3%) developed TB-DILI at a median of 8.5 (3-18) d of starting ATT. All patients were symptomatic with the most common symptoms being anorexia and nausea (80%). A higher baseline ALT was independently associated with DILI with adjusted OR 2.1 (95% CI 1.3-3.4), p = 0.01. Eight patients tolerated reintroduction of ATT in a sequential manner, 9-24 d after discontinuation.

Adjuvant testosterone therapy in chronic heart failure (ATTIC): a randomised open-label trial.

INTRODUCTION: Heart failure is a major contributor to morbidity and mortality in the geriatric population, with no promising therapy currently available with considerable benefit. Testosterone therapy is an emerging viable treatment option given its beneficial effects, including improving cardiac functional capacity, alleviating symptoms and low cost, among others. METHODS: We have planned an open-label, parallel design, 1:1 randomised controlled trial, which aims to recruit 986 adult males above the age of 60 diagnosed with chronic stable heart failure fulfilling the eligibility criteria. The participants will be randomised into 2 groups of 493 each. Both groups will receive standard recommended treatment regimen of chronic stable heart failure and intervention arm participants will receive additional testosterone gel. All participants will be assessed at baseline, 4 weeks, 6 weeks and 12 weeks. The primary endpoints will assess the differences in functional capacity, frailty and quality of life at 3 months compared with baseline. The secondary endpoints will include the mean change from baseline at 3 months in cardiac remodelling using echocardiography, serum brain natriuretic peptide levels, the incidence of adverse drug reaction. STATISTICAL ANALYSIS: The data will be analysed with the help of SPSS 23 software. Primary objectives of change in 6-minute walk test, frailty index and quality of life will be analysed using the student's t-test. The statistical significance will be defined as p value<0.05 and taking confidence level as 95%. ETHICAL CLEARANCE: Institutional Ethics Committee clearance taken via letter no AIIMS/IEC/20/847, dated 21 November 2020. This study involves human participants and was approved by institutional ethical committee, DHR Reg: EC/NEW/Inst/2020/1046CDSCO, Reg No: ECR/736/Inst/UK/2015/RR-18. Participants gave informed consent to participate in the study before taking part. TRIAL REGISTRATION NUMBER: (CTRI)-REF/2020/12/030292.

Incidence, predictors and outcome of sepsis-associated liver injury in children: a prospective observational study.

Sepsis-associated liver injury (SALI) occurs as a result of the systemic and microcirculatory changes that happen because of sepsis. Its prognostic significance in the paediatric population is unclear. We enrolled all children < 19 years, admitted between July, 2020 and July, 2021 to the paediatric unit (ward or intensive care unit) with a diagnosis of sepsis for this study. Clinical and biochemical parameters of children with sepsis who developed SALI were compared with those without SALI to determine the risk factors of SALI and its impact on in-hospital mortality. A total of 127 children, median age 72 (1-204) months, 74 males were included. SALI developed in 45 (31.3%) at a median 1 (1-13) days after the diagnosis of sepsis. The SALI pattern was cholestatic in 18 (40%), hepatocellular in 17 (37.7%) and hypoxic hepatitis in 10 (22.3%). Paediatric sequential organ failure assessment (pSOFA) was an independent predictor of SALI - OR 1.17 (95% CI 1.067-1.302), p = 0.001. A pSOFA score of > 9.5 predicted the development of SALI with 66.7% sensitivity and 77.1% specificity. SALI was an independent predictor of mortality in children with sepsis - OR 1.9 (95% CI 1.3-3.4), p = 0.01.   Conclusions: SALI develops in 45 (31.3%) with sepsis. A higher pSOFA score is associated with SALI. Children who develop SALI have a ~ twofold higher risk of mortality than those without SALI. What is Known: • During the process of sepsis, the liver plays a role by scavenging bacteria and producing inflammatory mediators. However, at times the liver itself becomes a target of the dysregulated inflammatory response. This is known as sepsis-associated liver injury (SALI). • The incidence of sepsis-associated liver injury and its prognostic significance in children is not known.. What is New: • SALI develops in one-third children with sepsis and is associated with a higher pSOFA score. • Children who develop SALI have a higher risk of mortality.