Overexpression of ARM repeat/U-box containing E3 ligase, PUB2 positively regulates growth and oxidative stress response in Arabidopsis.

Plant growth and development are governed by selective protein synthesis and degradation. Ubiquitination mediated protein degradation is governed by activating enzyme E1 followed by conjugating enzyme E2 and E3 ligase. Plant Armadillo (ARM) repeat/U-box (PUB) protein family is one of the important classes of E3 ligase. We studied the function of AtPUB2 by loss-of-function (knockout and knock down mutants) and gain-of-function (CaMV 35S promoter driven overexpression lines) approach in Arabidopsis. Under normal growth condition, we observed that loss-of-function mutant plants did not show any significant difference in growth when compared with wild-type possibly due to functional redundancy between PUB2 and PUB4. However, AtPUB2-OE lines exhibit early flowering and improved vegetative growth. Also, AtPUB2-OE seedlings showed sensitive phenotype in the presence of exogenous cytokinin. We found that AtPUB2 expression is induced under oxidative stress. Subcellular localization analysis shows that AtPUB2 is predominantly localized in the nucleus. We performed the phenotypic analysis under oxidative stress condition induced by methyl viologen (MV) and observed that overexpression lines display tolerance to oxidative stress in light and dark conditions. Furthermore, we found less amount of ROS accumulation, enhanced proline accumulation and decreased levels of MDA after MV treatment in AtPUB2-OE lines. PUB2-OE lines showed enhanced oxidative stress marker genes expression. By in vitro auto-ubiquitination assay, we also show that it possesses the E3 ligase activity. Overall, our findings suggest the possible role of AtPUB2 in plants ability to tolerate oxidative stress by enhancing the activity of antioxidant enzymes, which in turn improves ROS scavenging activity and homeostasis.

Magnetic resonance imaging patterns and perfusion changes of posterior reversible encephalopathy syndrome in children with clinical outcome correlation.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) in children has a propensity towards atypical features on magnetic resonance (MR) imaging, with limited literature on perfusion changes and clinicoradiological correlation. OBJECTIVE: We aimed to comprehensively study MR imaging patterns of pediatric PRES, including cerebral blood flow variations on arterial spin labeling, and looked for any MR biomarkers of poor clinical outcome. MATERIALS AND METHODS: In this retrospective observational study conducted in a tertiary hospital setting, MR records over a 4-year period (May 2019 to May 2023) were systematically searched along with their clinical details. Patients with an age less than 18 years and a clinicoradiological constellation consistent with PRES were included. MR scans were analyzed by two neuroradiologists with 8 years' and 10 years' experience. Association was sought with poor clinical outcome (defined as modified Rankin Scale score at discharge of > 2). RESULTS: A total of 45 patients (29 boys) were included in the study, with a mean age (± standard deviation) of 11.19 (± 4.53) years. On MR imaging, 95.6% of patients (n = 43) showed atypical features and/or atypical areas of involvement. The superior frontal sulcus (n = 18) was the most predominant MR pattern, and cerebellar involvement was not uncommon (n = 15). Unilateral involvement (n = 3), isolated central pattern (n = 1), and spinal cord involvement (PRES-SCI: n = 1) were also encountered. Brainstem involvement (n = 4) showed a characteristic "V-sign" of anterior medullary hyperintensity. Patchy restricted diffusion (46.6%), punctate hemorrhages (37.7%), and leptomeningeal contrast enhancement (36%) were not uncommon. Arterial spin labeling sequence (available in 24 patients) showed increased cerebral blood flow in the involved areas in 79.2% of patients. Univariate analysis showed a significant association of the presence of hemorrhage (P = 0.003), involvement of brainstem (P = 0.007), deep white matter (P = 0.008), and thalamus (P = 0.026) with poor clinical outcome. Multivariate regression analysis found that hemorrhage on MRI (P = 0.011, odds ratio 8) was an independent factor associated with poor clinical outcome. CONCLUSIONS: The conventionally described atypical features in PRES are common in children and therefore may no longer be considered exceptions. Raised perfusion on arterial spin labeling sequence was seen in the majority of cases. Hemorrhage on MRI was an independent predictor of poor clinical outcome in pediatric PRES.

Familial hemiplegic migraine in Indian children-a tertiary center experience.

Familial hemiplegic migraine (FHM), an autosomal dominant subtype of hemiplegic migraine, is a channelopathy presenting with severe headache, visual field defect, paresthesia, unilateral motor deficit, encephalopathy, seizures and aphasia. This cross-sectional study was conducted over 10 months in children aged 1-18 years suspected of hemiplegic migraine at a tertiary care pediatric hospital. Fourteen children were screened and five children with genetically confirmed FHM were included. The symptoms in the study population were paroxysmal hemiparesis (5/5), headache (5/5) and focal seizures (1/5). The hemiplegia episodes lasted from 4 h to 7 days. The mean age at the onset of neurological symptoms was 6.8 ± 0.7 years and the mean age at diagnosis was 12.8 ± 1.7 years, with a mean delay of 6.1 ± 1.9 years for the diagnosis. Neuroimaging during acute episodes revealed accentuated gray, white differentiation in the contralateral cerebral hemisphere with mild effacement of sulcal spaces in T2/fluid-attenuated inversion recovery (FLAIR) images. Genetic testing revealed ATP1A2 mutations (FHM2) in 4/5 and SCN1A (FHM3) in 1/5 patients. All of them (5/5) were initiated on oral topiramate and had favorable treatment responses with a mean follow-up duration of 7 ± 1.4 months. Diagnosis of FHM is mainly clinical and can be confirmed by genetic analysis. Perfusion and diffusion-weighted MRI should be considered during acute headache episodes, as it is mostly normal in symptom-free periods. Routine MRI sequences like T1 weighted, T2 weighted, FLAIR and contrast remain normal even during acute attacks.

Neurodevelopmental Outcomes of a Cohort of Children with Tuberous Sclerosis Complex with Epileptic Spasms.

The neurodevelopmental outcomes in children with tuberous sclerosis complex (TSC) with epileptic spasms remain underdiagnosed and might be responsible for significant morbidity and mortality burdens, even after spasms abate. The study was a cross-sectional study over 18 months at a tertiary care pediatric hospital, involving 30 children with TSC who had epileptic spasms. They were assessed with Diagnostic and Statistical Manual of Mental Disorders-5 criteria for autism spectrum disorder (ASD), attention deficit hyperactivity disorder (ADHD), and intellectual disability (ID), and childhood psychopathology measurement schedule (CPMS) for behavioral disorders. The median age at onset of epileptic spasms was 6.5 (1-12) months, and the age at enrolment was 5 (1-15) years. Of 30 children, 2 (6.7%) had only ADHD, 15 (50%) had only ID/GDD (global developmental delay), 4 (13.3%) had ASD and ID/GDD, 3 (10%) had ADHD and ID/GDD, and 6 (20%) had none. The median intelligence quotient/development quotient (IQ/DQ) score was 60.5 (20-105). CPMS assessment revealed significant behavioral abnormalities in almost half the children. Eight (26.7%) patients were completely seizure-free for at least 2 years, 8 (26.7%) had generalized tonic-clonic seizures, 11 (36.6%) had focal epilepsy, and 3 (10%) had evolved into Lennox-Gastaut syndrome. A high proportion of neurodevelopment disorders, including ASD, ADHD, ID/GDD, and behavioral disorders were seen in this pilot study with a small cohort of children with TSC with epileptic spasms.

Late Onset Pompe's Disease: Clinical, Pathological & Molecular Analysis of Two Adolescent Patients.

INTRODUCTION: The estimated global prevalence of late onset Pompe's disease is 1/57000 live birth(1). We present the case of two patients diagnosed to have Pompe's disease with a rare association of cardiomyopathy. MATERIALS: Two siblings born out of non consanguineous marriage presented with proximal myopathy of 5 years duration. Patient 1 - 19 year female, there was atrophy and weakness of face, neck, girdle and limbs. Her Echocardiogram showed LV dilation with low ejection fraction, ECG showed LV hypertrophy with incomplete LBBB. Her CK-NAC values came to be 918 U/L. Patient 2 - 16 year male; progression, distribution and severity slightly different to his sister but had exertional dyspnea since last one year. His echocardiogram showed LV diastolic dysfunction, ECG showed short PR interval partial LBBB and his CK-NAC came to be 2347 U/L. His skeletal muscle biopsy showed deposition of glycogen. Genetic analysis revealed pathogenic mutation in GAA gene (c.2040G>A) in both patients. RESULT: Late onset Pompe disease is of less severity but progressive. The involvement of heart is less likely compared to infantile onset(2). It is also interesting that the same variant presents differently in both patients. CONCLUSION: Genetic diseases manifest as rare phenotype which in itself is a clinical puzzle. When rare disease present with a rare manifestation of itself, this pose a great diagnostic challenge. Pompe's disease is one of the very few inherited disorder which has definitive treatment- enzyme replacement therapy. Molecular characterization of the variant is absolutely necessary before initiating therapy. References Ausems MG, Verbiest J, Hermans MM, et al. Frequency of glycogen storage disease type II in The Netherlands: implications for diagnosis and genetic counselling. Eur J Hum Genet 1999;7(6):713-716. Van der Beek NA, Soliman OI, Van Capelle CI, et al. Cardiac evaluation in children and adults with Pompe disease sharing the common c.-32-13T>G genotype rarely reveals abnormalities. J Neurol Sci 2008;275(1-2):46-50.

Predictors of Poor Outcome in Patients with COVID-19 associated Respiratory Failure: A Retrospective Observational Study.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is majorly known to cause mild to moderate disease, but a small fraction of patients may develop respiratory failure due to diffuse lung injury, requiring management in the intensive care unit (ICU). This study attempts to identify factors that can predict unfavorable outcomes in moderate to severe COVID-19 patients. METHODS: Hospital records of 120 COVID-19 patients admitted to the ICU were retrospectively analyzed and data pertaining to demographic, clinical, and laboratory parameters were obtained. These data were then compared with outcome parameters like survival, duration of hospital stay, and various adverse events. RESULTS: Out of 120 patients, 70% were male, with a mean age of 54.44 years [standard deviation (SD) ± 14.24 years]. Presenting symptoms included breathlessness (100%), cough (94.17%), fever (82.5%), and sore throat (10.83%). Diabetes, hypertension, and chronic obstructive pulmonary disease (COPD) were the common comorbidities associated. Increased serum D-dimer, ferritin, interleukin-6 (IL-6) levels, and unvaccinated status were associated with higher mortality. Overall, 25.83% of patients survived, 24.41% of patients developed septic shock, and 10.6% of patients were discharged on oxygen. World Health Organization (WHO) clinical progression scale score ≥ 6 had 57 and 82% sensitivity and 83 and 77% specificity on days 7 and 14 after admission, respectively, for predicting mortality. A baseline National Early Warning Score 2 (NEWS 2) ≥ 9 had 48% sensitivity and 88% specificity for predicting mortality. CONCLUSION: Advanced age and associated comorbidities are linked to adverse outcomes in moderate to severe COVID-19. Persistently high D-dimer levels, despite standard treatment, may also contribute to increased mortality. WHO clinical progression scale and NEWS 2 have high specificity for predicting mortality.

Predictors of Risk for Cerebral Palsy: A Review.

PURPOSE: To identify the earliest predictors of risk for diagnosis of cerebral palsy (CP). METHODS: A comprehensive literature search was conducted using various databases. The publications were reviewed to identify risk factors for CP from conception to early infancy. Studies were critically appraised with Joanna Briggs Institute guidelines for quality appraisal and evaluated for risk of bias using the Agency for Health Care Research and Quality guidelines. RESULTS: The initial search yielded 129 studies and 20 studies were included. Forty-seven risk factors for CP were extracted of which several were duplicate terms. The significant risk factors found to be indicative of CP were low birth weight (<1500 g), birth at less than 28 weeks of gestational age, periventricular leukomalacia, grade 3 or 4 intraventricular hemorrhage, preeclampsia, prematurity, an Apgar score of less than 4 at the first minute, birth asphyxia, preterm premature rupture of membrane, and absent fidgety movements. CONCLUSION: Twenty-three factors were consistently reported as predictors of CP.

PPP2R5D-Related Neurodevelopmental Disorder or Developmental and Epileptic Encephalopathy?: A Novel Phenotypic Description and Review of Published Cases.

BACKGROUND: Protein phosphatase 2 regulatory subunit B' delta (PPP2R5D)-related neurodevelopmental disorder is caused by pathogenic variations in the PPP2R5D gene, product of which is involved in dephosphorylation. This is a rare disorder with description limited to case reports. Its phenotypic spectrum has expanded over the last decade. METHODS: We report a child with a developmental and epileptic encephalopathy phenotype with a pathogenic PPP2R5D variant. This phenotype has not been previously reported. We also reviewed the previously published reports of patients with this disorder. RESULTS: Including the index child, 28 cases (15 girls) were identified from nine relevant research items for analysis. All patients had developmental delay. History of seizures was observed in seven patients while macrocephaly was seen in nearly 80% of patients. Nonneurological manifestations were observed in 13 patients with the most common one being ophthalmological manifestations. The most common genetic variation was c.G592A (p.E198K). The common phenotypic associations of this variation were developmental delay, macrocephaly (11/15), and epilepsy (6/15). CONCLUSION: PPP2R5D gene variations should be suspected in children with developmental delay, autistic features, macrocephaly with or without epilepsy in the absence of any clear etiology. Dysmorphic features might provide a diagnostic clue. DEE phenotype may also be the presenting feature and might be an underreported entity.

Seroprevalence of MOG and AQP4 Antibodies and Outcomes in an Indian Cohort of Pediatric Acquired Demyelinating Syndromes.

This study aimed to determine the seropositivity of myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) and aquaporin-4 antibodies (AQP4-Ab) and outcomes in children with acquired demyelinating syndromes (ADSs). Children (6 months-15 years) with suspected ADS were enrolled and tested for MOG-Ab and AQP4-Ab prospectively over 18 months at a tertiary care hospital in North India. Children with proven nonimmune-mediated neurological disorders were enrolled as controls. Of 79 children with suspected ADS, 66 were enrolled. Among the enrolled children with ADS, acute demyelinating encephalomyelitis (ADEM) (25) was the most common first clinical event followed by optic neuritis (ON) (20) and transverse myelitis (TM) (19; one child had ON and TM simultaneously [neuromyelitis optica spectrum disorders [NMOSDs]]), while two children had clinically isolated syndrome (CIS) apart from ON and TM. Fourteen (21.2%, confidence interval [CI] 11.3-31.1) tested positive for one antibody (12 [18.1%; 95% CI 10.5-25.5%] for MOG-Ab and 2 [3%; 95% CI 0-7.2%] for AQP4-Ab). None of the 62 controls tested positive for any antibody. The final diagnosis in those with the monophasic ADS was ADEM (21), ON (13), TM (16), and other CIS (1) while that in children with recurrent events was multiphasic disseminated encephalomyelitis (MDEM) (2), NMOSD (3), ADEM-ON (4), recurrent ON (4), and MS (2). Among those with the first event, 4/51 (7.8%; 95% CI 0.5-15.2%) were MOG-Ab positive and 2 AQP4-Ab positive, whereas 8/15 (53.3% [95% CI 28.1-78.6%]) with recurrent events (MDEM [2], ADEM-ON [4], recurrent ON [1], and recurrent TM [1]) were MOG-Ab positive. Hence, MOG-Abs are the most common antibodies detected in one in five children with pediatric ADS, especially in relapsing disease. AQP4-Abs are rare in children with ADS.

Lyme Neuroborreliosis with Intracranial Hypertension and Erythema Multiforme: A Rare Presentation.

A 9-year-old previously healthy boy presented with high-grade intermittent fever, severe headache associated with neck stiffness for 5 days, rash over trunk and extremities for 4 days, vomiting for 3 days and diplopia for 2 days. There was no history of seizures, abnormal body movements, altered sensorium or focal deficits. On examination, he had maculopapular erythematous rashes over the trunk and extremities and erythema multiforme. He had bilateral abducens nerve palsy and the rest of the cranial nerve, sensory and motor examination was normal. He had neck stiffness and positive Kernig's sign. Fundus examination showed grade 4 papilledema. Cerebrospinal fluid workup revealed elevated opening pressure, lymphocytic pleocytosis, normal protein and glucose levels. Neuroimaging showed features suggestive of intracranial hypertension. Borrelia IgM and IgG antibodies came positive. The uniqueness of our case lies with two rare presenting manifestations of Lyme neuroborreliosis in the same child.

Parenting a child with autism spectrum disorder: A qualitative study.

Background: Research in India has seldom studied caregivers' perceptions, experiences, and needs for information and personal support after an autism spectrum disorder (ASD) diagnosis. Objectives: The objectives of the study were to understand the perceived barriers for obtaining a diagnosis and the perspectives and experiences of parents of children with autism. Materials and Methods: Parents with a diagnosed ASD child (within a year of diagnosis) in the 3-8 years range were recruited from the Pediatric Psychology and Neurodevelopmental Clinic from a tertiary care teaching hospital in North India. An interview guide elicited information about experiences regarding obtaining an ASD diagnosis, perceived barriers and facilitators, reactions to diagnosis, postdiagnostic family and community experiences, and stress experienced by parents. Qualitative responses were analyzed using thematic analysis. Participants were recruited till there was a saturation of themes. The ethics clearance was provided by the institutional review board. Results: Twenty-eight caregivers of children with ASD were recruited for the study. Overall, nine themes were identified from the qualitative analysis of the interviews: two before diagnosis (delayed help-seeking and experiences with healthcare), one at the time of diagnosis disclosure (heightened emotional response to diagnosis), and six themes after the diagnosis (increased stress, behavioral challenges, deterioration in family relationships, negative attitudes of the family, seeking support, and moving forward with hope for the future). Conclusions: There are several barriers and gaps in the autism-related available services in the country, and there is a need to provide inclusive, supportive, culturally sensitive, and family-centered model of care for parents raising children with ASD.

Horizontal Gaze Palsy, Scoliosis, and Split Pons Sign in a 6-Year-Old Girl.

ABSTRACT: A 6-year-old girl presented with complaints of absent horizontal eye movements since birth. There was also associated progressive scoliosis for past 1 year. Neuroimaging revealed split pons sign, butterfly-shaped medulla, and prominent inferior olivary nuclei. The presence of congenital horizontal gaze palsy, childhood onset progressive scoliosis, and abnormal neuroimaging findings confirmed the diagnosis of horizontal gaze palsy with progressive scoliosis. This case highlights the importance of neuroimaging in a child presenting with horizontal gaze palsy and scoliosis that helped for starting early rehabilitation of the child, prevention of permanent vision loss, and parental counseling for future pregnancies.

Efficacy and Safety of Phenobarbitone as First-Line Treatment for Neonatal Seizure: A Systematic Review and Meta-Analysis.

BACKGROUND AND OBJECTIVE: Phenobarbitone is used as a first-line drug for neonatal seizures. However, its poor short- and long-term safety profile is concerning. We aim to systematically synthesize the data on the efficacy and safety of phenobarbitone as a first-line agent and compare it against other anti-epileptic drugs (AEDs) in neonates. METHODS: Using keywords related to the study population (neonatal seizure) and intervention (phenobarbitone), we searched CENTRAL, Embase, PubMed and Web of Science until 15 December 2020. Randomized controlled trials (RCTs) comparing phenobarbitone with any other AED as first-line therapy for seizure control in the neonates were considered eligible. The random-effect meta-analysis was done using RevMan 5.3 software. RESULTS: We screened through 443 records and identified nine eligible studies (719 participants). Five RCTs comparing phenobarbitone with levetiracetam did not find any difference in seizure control with the first dose [risk ratio (RR) 1.43, 95% CI 0.79-2.57] or adverse effects (RR 4.66; 95% CI 0.33-65.83). Two trials comparing phenobarbitone and phenytoin also did not find any difference in seizure control with the first dose (RR 2.09; 95% CI 0.31-14.03) and other outcomes. Only one RCT compared phenobarbitone and lorazepam and found lorazepam to be more efficacious in seizure control with the first dose (RR 0.71; 95% CI 0.53-0.94). Three trials compared neurodevelopmental outcomes, in which levetiracetam was better in two, whereas one did not find any difference. CONCLUSION: Phenobarbitone is at least as efficacious and safe as other drugs like phenytoin and levetiracetam. The data over the long-term neurodevelopmental outcome are lacking. The existing evidence is insufficient to recommend other drugs over phenobarbitone.

CNS autoimmunity in children: An unwanted wrinkle in a smooth narrative.

The emerging paradigm of childhood autoimmune neurological disorders has exploded in recent times due to reliable diagnostic methods and their ease of availability, well-defined diagnostic criteria, and universal awareness about these disorders. The most important aspect of these disorders is a considerable recovery in response to early targeted immunotherapy. If left untreated and/or ill-treated, these can lead to mortality or lifelong morbidity. Autoantibodies can target any part of the central nervous system (CNS), ranging from superficial structures like myelin to deep intracellular ion channels like voltage-gated potassium channels, resulting in contrasting and at times overlapping symptomatology. Though neuroimaging characteristics and serological tests confirm these disorders' diagnosis, it is essential to suspect them clinically and start management before the reports are available for minimizing morbidity and mortality. In the pediatric age group, several metabolic conditions, like mitochondrial disorders and enzyme deficiencies like HMG-CoA-lyase deficiency, can develop neuroimaging patterns similar to those seen in childhood CNS autoimmune disorders and may also show a favorable response to steroids in acute phases. Hence, the clinician must suspect and work up the index patient appropriately. Here, we briefly discuss the pathophysiology, clinical clues, and potential therapeutic targets related to pediatric CNS autoimmune disorders.

Disseminated Intravascular Coagulation Score and Sepsis-induced Coagulopathy Score in Prediction of COVID-19 Severity: A Retrospective Analysis.

BACKGROUND: The novel disseminated intravascular coagulation (DIC) score (platelet count, prolonged prothrombin time, D-dimer, and fibrinogen) and sepsis-induced coagulopathy (SIC) score (platelet count, international normalized ratio, and sequential organ failure assessment score) are markers of coagulopathy, which, for the first time, are explored in line with the coronavirus disease-2019 (COVID-19) disease outcomes. The correlation of D-dimer with these findings is also studied. MATERIALS AND METHODS: A retrospective analysis of hospital-based records of 168 COVID-19 patients was done. Data including D-dimer, routine investigations, DIC, and SIC scorings (all within 3 days of admission) were collected and correlated with the outcomes. The study was conducted in a tertiary care center catering to North India's population. RESULTS: Higher DIC score (1.59 ± 1.18 vs 0.96 ± 1.18), SIC score (1.60 ± 0.89 vs 0.63 ± 0.99), and D-dimer titers (1321.33 ± 1627.89 vs 583·66 ± 777.71 ng/mL) were significantly associated with severe COVID-19 disease (p <0.05). DIC score and SIC score ≥1, and D-dimer ≥1315 ng/mL for severe disease; DIC score ≥1, SIC score ≥2, and D-dimer ≥600 ng/mL for pulmonary embolism (PE); and DIC score and SIC score ≥1, and D-dimer level ≥990 ng/mL for mortality were the respective cutoff values we found from our study. CONCLUSION: Higher DIC scores, SIC scores, and D-dimer values are associated with severe COVID-19 disease, inhospital mortality, and PE risk. They can serve as easily accessible early markers of severe disease and prioritize hospital admissions in the presently overburdened scenario and may be used to develop prognostic prediction models. HOW TO CITE THIS ARTICLE: Kapoor M, Panda PK, Saini LK, Bahurupi Y. Disseminated Intravascular Coagulation Score and Sepsis-induced Coagulopathy Score in Prediction of COVID-19 Severity: A Retrospective Analysis. Indian J Crit Care Med 2021;25(12):1357-1363.

Epidermal necrolysis as the presenting manifestation of pediatric lupus.

Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) represents the spectrum of skin lesions characterized by rashes, exfoliation, and sloughing usually following drug intake. Occasionally, TEN-like cutaneous manifestations have also been described with systemic lupus erythematosus. Recognition of lupus in a child presenting with TEN-like skin changes is clinically challenging and requires a high degree of suspicion. We describe the case of a child who had epidermal necrolysis as the presenting feature of lupus and had severe neurological complications. TEN-like skin changes in association with severe neurological complications in pediatric lupus are uncommon. Lupus must be considered in the differential diagnosis of a child presenting with epidermal necrolysis with no provocative risk factors such as a history of exposure to medications.

An Uncommon Cause of Hypertensive Urgency in Young Adolescent: Case Report.

Mid aortic syndrome is rare cause of hypertensive urgency in children with poor outcome if left untreated, high index of suspicion with prompt management is the key to survival with good outcome. A 12-year-old boy was presented with fever, puffiness of face, and breathing difficulty. Clinically, he had hypertension with differential pulsation and BP in upper and lower limbs. He had peak systolic gradient of 80 mm Hg between upper and lower limb. His echocardiography and CT angiography was suggestive of significant isolated 80% narrowing of abdominal aorta without involvement any other large vessels. Percutaneous balloon dilatation of aorta was done considering multiple parameters. Post procedure, there was significant improvement in BP and we could wean his multiple anti-hypertensive drugs to keep his blood pressures below 95th centile. His BP remained control with minimum upper and lower limb gradient on follow up of almost 1 year. HOW TO CITE THIS ARTICLE: Dekate PS, Reddy S, Prasad VSV, Boda S, Saini L, Patil P. An Uncommon Cause of Hypertensive Urgency in Young Adolescent: Case Report. Indian J Crit Care Med 2019;23(7):339-341. KEY MESSAGE: Mid aortic syndrome is most uncommon amongst them. With prompt diagnosis and proper selection of therapeutic options like balloon dilatation or surgical correction, it has good prognosis. Aortic narrowing because of different diseases is an uncommon cause of HT urgency in children.