Dr Bijoy Patra

RESEARCH, TEACHING, or OTHER INTERESTS

Pediatrics, Perinatology and Child Health, Neurology (clinical), Infectious Diseases, Endocrinology

Scopus Publications

Missed Diagnosis of Cystic Fibrosis in Developing Countries—Need to Raise the Awareness!
Komal Attri, Kiran Kumar Banothu, Arun K, Suchismita Nanda, Manju Nimesh, Bijoy Patra, and Sheetal Agarwal
Wiley



The Great Masquerade: Varying Manifestations of Lysinuric Protein Intolerance
Soumalya Chakraborty, Ravneet Kaur, Bijoy Patra, JP Meena, SK Kabra, Madhulika Kabra, and Neerja Gupta
Springer Science and Business Media LLC



Purpura Fulminans and Transient Nephrotic Range Proteinuria: Rare Manifestation of Leptospirosis
Sumiti Banga, Sudha Chandelia, and Bijoy Patra
Ovid Technologies (Wolters Kluwer Health)



Fulminant Parvovirus B-19 Infection Manifesting as Acute Encephalitis Syndrome and Myocarditis in a 10-year-old Boy
Iqra Khan, Nimisha Sharma, Kiran Kumar Banothu, Sakshi Sachdeva, Ira Agarwal, Kavita Vani, Bijoy Patra, and Sheetal Agarwal
Ovid Technologies (Wolters Kluwer Health)



Accuracy of Advanced Pediatric Life Support Intubation Depth Formula in Indian Children Aged 1 to 12 Years
Sagar Agrawal, Vishal Kumar, Maansi Gangwal, Komal, Bijoy Patra, and Shahina Bano
Springer Science and Business Media LLC



Interesting Etiology in An Adolescent Girl with Pleural Effusion
H G Mittal, P Jain, R Shamitha, S Biradar, Arvind Ahuja, and B Patra
Springer Science and Business Media LLC



Tubercular Bronchoesophageal Fistula in an Adolescent Girl
Hema Gupta Mittal, Parasdeep Kaur, Shamitha Rangarajan, Kavita Srivastava, and Bijoy Patra
Springer Science and Business Media LLC



Intravenous Methylprednisolone Versus Oral Prednisolone for West Syndrome: A Randomized Open-Label Trial
Dipti Kapoor, Suvasini Sharma, Divyani Garg, Sukla Samaddar, Isha Panda, Bijoy Patra, Sharmila B Mukherjee, and Harish K. Pemde
Springer Science and Business Media LLC



Evaluation of the Modified Atkins Diet for the Treatment of Epileptic Spasms Refractory to Hormonal Therapy: A Randomized Controlled Trial
Suvasini Sharma, Shaiphali Goel, Dipti Kapoor, Divyani Garg, Isha Panda, Aman Elwadhi, Bijoy Patra, Sharmila B. Mukherjee, and Harish Pemde
SAGE Publications
Objectives: We aimed to evaluate the efficacy of the modified Atkins diet in children with epileptic spasms who had failed hormonal therapy. Methods: Children aged 9 months to 3 years having daily epileptic spasms despite a trial of ACTH or oral prednisolone and 1 additional anticonvulsant medication were enrolled. Children were randomly assigned to receive the modified Atkins diet either immediately or after a delay of 4 weeks. The ongoing anticonvulsant medications were continued unchanged. The primary outcome variable was the proportion of children who achieved spasm freedom as per parental reports at 4 weeks. Secondary outcomes included time to spasm cessation, proportion of children with electroclinical remission, the proportion of children with >50% reduction of spasms at 4 weeks, and adverse effects of the diet. ( ClinicalTrials.gov Identifier: NCT03807141). Results: A total of 91 children were enrolled in the study; 46 in the diet group and 45 in the control group. At the end of 4 weeks, 11 children in the diet group were spasm free compared with none in the control group ( P ≤ .001). The median time to achieve spasm cessation was 10 days (interquartile range 9-20). Nine of these had resolution of hypsarrhythmia on electroencephalography (EEG). Thirty (65.2%) in the diet group had >50% reduction in spasms, compared with none in the control group ( P < .001). The most common side effect was constipation, noted in 34.8% of the children. Conclusions: The modified Atkins diet was found to be effective and well tolerated in children with epileptic spasms refractory to hormonal therapy.


A Young Infant with Joint Swellings and Subcutaneous Nodules
Aakanksha Choudhary, Sunita Bijarnia-Mahay, Divyani Garg, Bijoy Patra, Alex Solyom, and Suvasini Sharma
Springer Science and Business Media LLC



Use of the International League Against Epilepsy (ILAE) 1989, 2010, and 2017 Classification of Epilepsy in children in a low-resource setting: A hospital-based cross-sectional study
Suvasini Sharma, Aakanksha Anand, Divyani Garg, Sakshi Batra, Sharmila B. Mukherjee, Bijoy Patra, and Satinder Aneja
Wiley
Abstract Objectives This cross‐sectional study was designed to test the applicability of the 1989, 2010, and 2017 International League Against Epilepsy (ILAE) classification of epilepsy in children from a resource‐limited setting in India. Methods Classification of seizure types and syndromes was done through parental interviews and review of medical records in children with epilepsy aged one month to 18 years. Available investigations including EEG, MRI, and metabolic/genetic tests were used in classifying patients as per the 1989, 2010, and 2017 ILAE (level II‐epilepsy type) classification. We compared the proportion of children remaining unclassified by each scheme. Results Seven hundred and twenty‐six children (436 males, mean age 6.4 ± 4.6 years) were enrolled. Using the 1989 ILAE classification, we were able to classify 95.7%, and 82.6% children by the 2010 scheme. The 2017 ILAE classification could classify all 726 children at level I (seizure type), 664 (91.0%) children at level II (epilepsy type), and an electroclinical syndrome could be identified in 409 (56.1%) of the children. An etiology could be identified in 75%, perinatal brain injury being the most frequent. West syndrome was the most common electroclinical syndrome, identified in 22.7% patients. The 1989 ILAE classification system was superior to the 2010 system (P = .01) in epilepsy classification. There was no difference between the 1989 and 2017 schemes (P = .31) or the 2010 and 2017 schemes (P = .10). Significance The 2017 ILAE classification, being multidimensional, allowed classification of children who could not undergo extensive evaluation due to economic constraints and also provided room for overlapping etiologies.


Electroclinical spectrum of childhood epilepsy secondary to neonatal hypoglycemic brain injury in a low resource setting: A 10-year experience
Dipti Kapoor, Sidharth, Suvasini Sharma, Bijoy Patra, Sharmila B Mukherjee, and Harish K Pemde
Elsevier BV



An unusual presentation of Menkes disease masquerading as a leukodystrophy with macrocephaly
A. Tayal, Aman Elwadhi, Suvasini Sharma and Bijoy Patra

Background: Menkes disease is an X-linked neurodegenerative disease caused by mutation in ATP7A gene, which codes for copper-transporting ATPase. It usually presents in early infancy with neuro-regression, hypotonia, seizures, and kinky hair. Magnetic resonance imaging (MRI) of the brain shows cerebral atrophy, subdural effusions, and tortuous cerebral blood vessels. Case Characteristics: We report the case of a 7-month-old boy who presented with global developmental delay, seizures, and increasing head size since 2 months of age and history of sibling death. He had macrocephaly, sparse, hypopigmented hair, seborrheic dermatitis of scalp, hypotonia, and brisk reflexes. Brain MRI was suggestive of megalencephalic leukodystrophy. Careful reexamination of films revealed tortuous blood vessels. Serum copper and ceruloplasmin levels were significantly reduced, leading to diagnosis of Menkes disease. Conclusion: This case exemplifies a rare presentation of Menkes disease, simulating a leukodystrophy with macrocephaly. Tortuosity of cerebral blood vessels is an important finding, which can help in differentiating Menkes disease from white matter disorders.


Association of Child Neurology-Indian Epilepsy Society Consensus Document on Parental Counseling of Children with Epilepsy
Kavita Srivastava, , Rachna Sehgal, Ramesh Konanki, Ridhimaa Jain, Suvasini Sharma, Rekha Mittal, Anaita Hedge, Anju Aggarwal, Arijit Chattopadhyay,et al.
Springer Science and Business Media LLC



Leukoencephalopathy due to variants in GFPT1- associated congenital myasthenic syndrome
Guy Helman, Suvasini Sharma, Joanna Crawford, Bijoy Patra, Puneet Jain, Stephen J. Bent, J. Andoni Urtizberea, Ravindra K. Saran, Ryan J. Taft, Marjo S. van der Knaap,et al.
Ovid Technologies (Wolters Kluwer Health)
ObjectiveTo determine the molecular etiology of disease in 4 individuals from 2 unrelated families who presented with proximal muscle weakness and features suggestive of mitochondrial disease.MethodsClinical information and neuroimaging were reviewed. Genome sequencing was performed on affected individuals and biological parents.ResultsAll affected individuals presented with muscle weakness and difficulty walking. In one family, both children had neonatal respiratory distress while the other family had 2 children with episodic deteriorations. In each family, muscle biopsy demonstrated ragged red fibers. MRI was suggestive of a mitochondrial leukoencephalopathy, with extensive deep cerebral white matter T2 hyperintense signal and selective involvement of the middle blade of the corpus callosum. Through genome sequencing, homozygous GFPT1 missense variants were identified in the affected individuals of each family. The variants detected (p.Arg14Leu and p.Thr151Lys) are absent from population databases and predicted to be damaging by in silico prediction tools. Following the genetic diagnosis, nerve conduction studies were performed and demonstrated a decremental response to repetitive nerve stimulation, confirming the diagnosis of myasthenia. Treatment with pyridostigmine was started in one family with favorable response.ConclusionsGFPT1 encodes a widely expressed protein that controls the flux of glucose into the hexosamine-biosynthesis pathway that produces precursors for glycosylation of proteins. GFPT1 variants and defects in other enzymes of this pathway have previously been associated with congenital myasthenia. These findings identify leukoencephalopathy as a previously unrecognized phenotype in GFPT1-related disease and suggest that mitochondrial dysfunction could contribute to this disorder.


Aetiopathogenesis of Myelofibrosis and its management in a child with Beta thalassemia major
Bijoy Patra, Mohemmed Ajij, Jagdish Chandra, Nupur Agarwal, and Anita Nangia
Elsevier BV



Neonatal exchange transfusions at a tertiary care centre in north India: An investigation of historical trends using change-point analysis and statistical process control
Viswas Chhapola, Ankita Goel Sharma, Sandeep Kumar Kanwal, Virendra Kumar, and Bijoy Patra
Oxford University Press (OUP)
Background The need for exchange transfusion (ET) as a treatment modality for neonatal hyperbilirubinaemia declined in developed countries with the advent of effective phototherapy. The trends of ET from India are unknown. Our objective was to investigate the trends of ET in India. Methods Retrospective data (January 2006-December 2016) was collected on total outborn neonatal admissions and ET procedures from a centre in north India. A combination of change-point analysis (CPA) and statistical process control (SPC) was used to investigate the trends of ET. Results During the study period, a total of 39 217 outborn neonates were admitted and 1575 (4%) underwent 1816 ET procedures. The CPA unravels four critical change points (October 2009, May 2011, September 2011 and November 2014) in ET rates. An SPC chart showed a decline in mean ET rate from 89.3 (upper control limit [UCL] 176.9, lower control limit [LCL] 1.7)/1000 neonatal admissions at the start of the study to 7.7 (UCL 34.6, LCL 0)/1000 at the end of the study. The greatest decline in ET rate was witnessed in October 2009, from 89.3 (UCL 176.9, LCL 1.7)/1000 neonatal admissions to 34.8 (UCL 87.1, LCL 0)/1000 neonatal admissions. Conclusions Our study demonstrated a progressive decline in the number of neonatal ET procedures over 11 y.


Pendred syndrome in a newborn with neck swelling: A case report
Mohemmed Ajij, Shambhavi, Bijoy Patra, Ankur Singh, and Seema Kapoor
Oxford University Press (OUP)
BACKGROUND Pendred syndrome is a rare autosomal recessive condition, characterized by functional impairment of thyroid gland and sensorineural hearing loss. The syndrome presents in patients with homozygous or compound heterozygous mutation. The presentation in the form of neck mass in a newborn is rare. CASE CHARACTERISTICS A 1 month old baby presented to us with neck mass, which was found to be an enlarged thyroid gland. Thyroid function tests were consistent with hypothyroidism. Further evaluation revealed moderate sensorineural hearing loss; genetic analysis showed that baby was homozygous for the known mutations causing the disease. INTERVENTION Thyroid hormone replacement and hearing habilitation were done. Follow up showed regression of the neck mass and normalization of thyroid function tests. Genetic counseling of the family was done. MESSAGE Identification of the exact cause of congenital hypothyroidism can prevent grave consequences later on for the patient as well as for the family.


Fixation-off sensitivity in idiopathic childhood occipital epilepsy of Gastaut
Puneet Jain, Suvasini Sharma, Bijoy Patra, and Satinder Aneja
SAGE Publications
A 7-year-old boy presented with episodic blindness for the last 2 months with occipital paroxysms and fixation-off sensitivity on electroencephalography (EEG). The clinico-EEG features were suggestive of idiopathic childhood occipital epilepsy of Gastaut. The interesting phenomenon of fixation-off sensitivity is discussed.


Unusual Neuroimaging Findings in Two Families with Giant Axonal Neuropathy
Puneet Jain, Suvasini Sharma, Mahesh Kamate, Virupaxi Hattiholi, Bijoy Patra, Anita Mahadevan, and Satinder Aneja
Cambridge University Press (CUP)
An 8-year-old boy, born to a third-degree consanguineous couple with no adverse perinatal events, presented with complaints of delayed development and gait abnormalities. He had global developmental delay with independent ambulation achieved at two years of age and monosyllables at 2.5 years of age. There was no regression. His hearing and vision were normal. He had had two episodes of generalized tonic-clonic seizures in the previous year. His examination revealed normal fundus and absence of musculoskeletal deformities. He had frizzy hair, mild global hypotonia, no motor weakness, absent muscle-stretch reflexes, and positive cerebellar signs. He had an older male sibling (not investigated) with a similar illness who was now bedridden. Magnetic resonance imaging of the brain showed T2 hyperintensities in the bilateral cerebellar dentate nuclei, posterior limb of the internal capsule, and the globus pallidi (Figure 1). Nerveconduction studies were suggestive of sensorimotor axonal polyneuropathy. Nerve biopsy showed giant axons. Echocardiography was unremarkable.


Clinical spectrum and treatment outcome of West Syndrome in children from Northern India
Jaya Shankar Kaushik, Bijoy Patra, Suvasini Sharma, Dinesh Yadav, and Satinder Aneja
Elsevier BV



Report of a novel Indian case of congenital erythropoietic porphyria and overview of therapeutic options
Meenu Pandey, Sharmila B. Mukherjee, Bijoy Patra, Seema Kapoor, Cecile Ged, Satinder Aneja, and Anju Seth
Ovid Technologies (Wolters Kluwer Health)
Congenital erythropoietic porphyria is a rare disorder of heme biosynthesis, resulting from decreased enzymatic activity of uroporphyrinogen III synthase. Clinical manifestations are heterogenous, of variable severity, and with occasional phenotypic-genotypic correlation. A 14-month-old boy developed fever, extensive dermatitis, and reddish colored urine. Anemia, erythrodontia, hepatosplenomegaly, and massive urinary elimination of predominantly type I porphyrins was suggestive of congenital erythropoietic porphyria. Although hemolysis remained mild and compensated, facial and digital mutilation developed indicative of moderate clinical phenotype. Mutational analysis revealed compound heterozygosity of mutant alleles, including a novel mutation (p.Pro190Leu). The child received supportive management and underwent facial reconstruction successfully.


Spinal congenital dermal sinus presenting as a diagnostic conundrum
Abhisek Chopra, Bijoy Patra, Satinder Aneja, Sharmilla Mukherjee, Anu Maheswari, and Anju Seth
S. Karger AG
Spinal congenital dermal sinus is a rare entity. Still rarer is its location over the thoracic and cervical spine. Secondary to congenital dermal sinus, intramedullary abscesses of the spinal cord are uncommon. Only few cases of such an association have been reported in the literature. We report such an interesting case of thoracic spinal congenital dermal sinus associated with intramedullary abscess in an 18-month-old boy who presented with a diagnostic conundrum. The pathogenesis, clinical presentation, neuroimaging and management of such cases are discussed. Awareness, detection, timely referral and definitive operative intervention for a better neurological outcome are emphasized.


Holocord syrinx, tethered cord and diastematomyelia: Case report and review of literature
A Maheshwari, D Yadav, S Aneja, S Kaur, B Patra, and A Seth

Syringomyelia refers to the presence of cavities within the spinal cord or a dilatation of the central spinal cord canal. In 90% of cases, syringomyelia is associated with a Chiari I malformation. The association of syringomyelia with tethered cord is well known but syrinxes associated with these defects are usually below vertebral level T6. Holocord syrinx associated with tethered cord is rare and is almost always associated with Chiari 1 malformation. To the best of our knowledge, only a single case report of holocord syrinx with tethered cord has been reported, but this patient had multiple overt lumbosacral defects (tethered cord, meningocele and diastematomyelia). We are reporting a three year old child with holocord syrinx with tethered cord and diastematomyelia and no evidence of Chiari malformation, meningocele or any overt spinal malformation and minimal neurological abnormalities. J Nepal Paediatr Soc 2012;32(2):169-171 doi: http://dx.doi.org/10.3126/jnps.v32i2.6098


Chronic idiopathic myelofibrosis with myeloid metaplasia presenting as refractory ascites
Bijoy Patra, Anu Maheshwari, Jagdish Chandra, Satinder Aneja, Shilpi Agarwal, Anita Nangia, and Anju Seth
Wiley
Chronic idiopathic myelofibrosis (CIM) with myeloid metaplasia is a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extra‐medullary hematopoesis (EMH), and varying degree of myelofibrosis. CIM, presenting as refractory ascites secondary to peritoneal hematopoesis, is extremely rare with only six adult cases reported in literature. This is a report of a child with CIM presenting as refractory ascites as a consequence of EMH in the peritoneum. The patient was treated with intermittent hydroxyurea with favorable response over 3 weeks. The patient was thereafter lost to follow up. Pediatr Blood Cancer 2010; 54:151–153. © 2009 Wiley‐Liss, Inc.