Objectives: The highest incidence of epilepsy in children coupled with the need of long-term antiepileptic treatment could lead to development of metabolic complications at an early age. Phenytoin and valproic acid are commonly used antiepileptic drugs in children. This study aimed to assess the serum lipid profile and liver function tests in children with epilepsy on phenytoin or valproic acid monotherapy for 6 months and beyond. Materials and Methods: This prospective descriptive study recruited children from the pediatric outpatient department of a tertiary care GB Pant Children Hospital, Srinagar, Kashmir, India from August 2009 to September 2011. All consecutive children diagnosed with epilepsy as per International League Against Epilepsy definition aged 3–18 years on phenytoin or valproic acid monotherapy for 6 months or beyond were enrolled. After baseline clinical and anthropometric evaluation (including body mass index [BMI]), the fasting blood samples were analyzed for serum lipid profile and liver enzyme changes. Results: Total of 75 children were enrolled. There were 25 patients each in phenytoin, valproic acid, and control group. We observed statistically significant high mean total cholesterol and alkaline phosphatase levels in group receiving phenytoin when compared with valproic acid or control group. Conclusion: The lipid and liver enzyme abnormalities may be observed in children on phenytoin or valproic acid therapy, which warrants careful screening and monitoring.

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Supernumerary teeth may be defined as any teeth or tooth substance in excess of the usual configuration of 20 deciduous and 32 permanent teeth. Their prevalence in primary dentition has been recorded variously between 0.3% and 0.8%, and between 0.1% and 3.8% in relation to permanent dentition. They may be a part of number of developmental disorders such as cleft lip and palate, cleidocranial dysostosis, Gardner's syndrome, Fabry Anderson's syndrome, Ehlers–Danlos syndrome, incontinentia pigmenti, and Trico Rhino-Phalangeal syndrome. If neglected, they can be the harbinger of a number of esthetic and pathologic complications. The management is dodged with several controversies.

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Introduction: Human cysteine rich secretory protein 3 (CRISP3), also known as specific granule protein (SGP28) is a small 28kDa protein. CRISP-3 is widely expressed in salivary glands, pancreas (particularly in pancreatic disease), lachrymal glands, prostate, myeloid precursors, human epididymis, ovary, thymus and colon. Human CRISP3 share similarity with snake venoms as well as Glioma Pathogenesis Related protein GLIPR1. Here we are reporting the extent of alignment of human CRISP3 protein with pancreatic enzymes and snake venom proteins and presence of GLIPR1 and CRISP3 genes containing conserved GHYTQVVW motifs in clinical samples of pancreatitis and pancreatic cancer. Material and Methods: Protein sequences with accession numbers for Human CRISP3, Snake Venom and enzymes present in pancreatic juices i.e. Trypsinogen, Cathepsin, Lipase, Amylase and Elastase were retrieved from swissprot, protein database. These sequences were aligned in silico for demonstrating the presence of 16 conserved cysteine residues and other motifs i.e. GENL and GHYTQVVW. The multiple sequence alignment (MSA) was performed. Presence of CRISP-3 conserved motifs in clinical samples of pancreatitis and pancreatic cancer was evaluated by PCR. DNA extracted from a small piece taken from inflamed pancreatic tail biopsy with confirmed diagnosis for pancreatitis and an excised pancreatic cancer tissue was subjected to PCR to conform presence of CRISP3 and GLIPR1 genes. Results: Our results show conservation of 9 Cysteine residues out of 16 Cysteine residues in majority of pancreatic enzymes and notable conservation of GENL and GHYTQVVW motifs across snake venom proteins. PCR yielded desired amplicon of expected size with the primers spanning conserved residues and core consensus motifs specifically designed to amplify CRISP3 gene in clinical samples of pancreatitis and pancreatic cancer. We also detected presence of GLIPR1 gene sequence in the DNA extracted from clinical samples of pancreatitis and pancreatic cancer thus confirming similarity between the pathogenesis related protein and CRISP3. Conclusion: Notable conservation of cysteine residues across pancreatic enzymes, snake venom and Human CRISP3 opens avenues of further research to evaluate the likely clinical potential of the conserved residues as putative biomarkers in exocrine pancreatic diseases. To the best of our knowledge this is the first report where primers designed around conserved GHYTQVVW motifs to amplify CRISP3 gene and its structural similarity with GLIPR1 gene sequence in clinical samples of pancreatitis and pancreatic cancer has been validated.

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Objective: The purpose of this study was to characterize the renal abnormalities with renal scintigraphy in patients with high anorectal malformation (HARM). Patients and Methods: We retrospectively reviewed 53 patients who had undergone corrective surgery for HARM from July 2011 to January 2017 and who were referred to this department for Technetium-99m dimercaptosuccinic acid (Tc99m-DMSA) scan for evaluation of renal cortical scarring. Scintigraphic evaluation of all 53 patients included both dynamic renography [Tc-99m diethylene triamine penta acetic acid (DTPA) and Tc-99m-L, L-ethylenedicysteine (L-EC) scan] and cortical scintigraphy. A total of 68 scans were done in these 53 patients in which there were 38 Tc-99m DMSA scans, 15 Tc-99m DTPA and 15 Tc-99m EC scans. Tc-99m DTPA scans were done in 15 patients (26 kidneys) to look for glomerular filtration rate and drainage pattern. Results: Mean age of presentation of these 53 patients (86 kidneys or renal units) with HARM was 28.9 months. Out of 53 patients, male comprised 62.26% of cases (n = 33) and female comprised 37.7% of cases (n = 20). Spectrum of urologic abnormalities noted in our patients with HARM included unilaterally non-visualized kidney in 37.7% cases, i.e., 20/53 patients; hydroureteronephrosis in 28.3% of cases, i.e., 15/53 patients; hydronephrosis in 16.98% cases, i.e., 9/53 patients. Scarring was noted in 28.3% cases, i.e., 15/53 patients. Small kidney was noted in 10 patients out of 53, i.e., 18.8% of cases. Ectopic kidney was seen in 5.6% cases, i.e., 3/53 patients, horseshoe kidney in 3.7% (2/53) and duplex kidney was found in 1 patient, i.e., 1.8% of cases. Conclusion: Children with HARM have a high incidence of renal anomalies and these are the most common cause of morbidity and mortality in these patients. It is important to document these renal anomalies early in life and initiate management as early as possible. Apart from routine investigations, such as ultrasound, renal scintigraphy performed with Tc-99m DTPA, Tc-99m EC and Tc-99m DMSA-III are non-invasive investigations that may provide important help in diagnosis, follow-up and management of renal anomalies in these patients.

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Hereditary hemorrhagic telangiectasia also known as Osler–Rendu–Weber disease is a rare autosomal dominant disorder affecting small vessels of multiple systems whose main pathological change is the presence of abnormal arteriovenous communications. It usually presents as skin and mucosal telangiectasia, epistaxis, gastrointestinal bleeding, and visceral arteriovenous malformations. Although epistaxis and gastrointestinal blood loss can result in anemia, patients with hereditary hemorrhagic telangiectasia rarely present with severe anemia and central nervous system (CNS) infections. Here, we report the case of a 57-year-old man who presented with severe anemia resulting in congestive cardiac failure with a history of recurrent blood transfusions and recurrent CNS infections who was ultimately diagnosed as hereditary hemorrhagic telangiectasia with recurrent epistaxis as a cause of his severe anemia.

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Coexistence of astrocytoma with cerebral vascular malformations is unusual, especially if both lesions occur separately. Preoperative angiogram will help us to identify any coexisting arteriovenous malformation (AVM). This will in turn change the treatment strategy for astrocytoma. Preoperative embolization for AVM may be done prior to the glioma excision. There is increased risk of bleeding if AVMs are not preoperatively diagnosed. AVMs coexisting with astrocytomas are rare. We report a case of anaplastic astrocytoma coexisting with an acquired AVM.

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Coarctation of aorta with aberrant right subclavian artery distal to coarctation is very rare. Detailed evaluation and planning is required for management of such case. Coarctation of the thoracic aorta (CoA) occurs at an incidence of approximately 1:1000 in the general population, ranging from 0.08 to 0.14% in large surveys. Anomalous origin of right subclavian artery (RSCA) is reported in up to 1% cases of CoA.Presence of aberrant RSCA makes treatment of such anomaly challenging. We report here successful placement of bare metal stent in a young lady with severe CoA and aberrant RSCA distal to CoA. Transcatheter stenting of CoA with aberrant RSCA is safe and effective procedure with good outcome. A detailed preprocedure evaluation and planning is the key to success.

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Introduction: This study compares two commonly practised methods of didactic teaching employed during theory classes i.e., power point presentation and blackboard teaching, and evaluates the impact of substituting these age-old techniques with case discussions based on real life clinical scenarios. Setting and Design: This cross sectional study was carried out in the Department of Biochemistry, Lady Hardinge Medical College, New Delhi, India. Subjects and Methods: A total of 198 first year MBBS students participated in the study during the period September 2015 to June 2016. The tool used was a pretested questionnaire which served as a feedback form. The obtained data was systemically tabulated. A SPSS software was used to analyze the data and appropriate pie charts and tables were drawn. Results: A large majority of students found case discussions to be far more interesting and useful from the perspective of learning and understanding. Conclusion: Didactic teaching methods do not contribute significantly to the knowledge base of the students. Case discussions, which highlight the clinical relevance of the subject through real life situations are a far more effective and robust method for developing a keener understanding of biochemistry among the students.

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Background: Exfoliative dermatitis (ED) is an inflammatory skin disorder in which generalized skin erythema and scaling affect at least 90% of the body. Aims: To determine the etiology, clinical presentations, laboratory findings, management, and outcomes of ED. Materials and Methods: This retrospective study was conducted at the Department of Pediatrics, Siriraj Hospital. Data of pediatric patients diagnosed with ED during January 1992 and June 2014 were reviewed. Results: Forty-seven patients were identified. The median age at diagnosis was 4 years and 8 months (range 1–164 months), and 10 cases (21.3%) were infants. Presenting symptoms included itching (76.6%), fever (38.3%), painful sensation (17.0%), and diarrhea (12.8%). Physical findings were dehydration (42.6%), fever (34.0%), failure to thrive (29.8%), tachycardia (29.8%), hepatomegaly (17.0%), edema (12.8%), lymphadenaopathy (8.5%), and splenomegaly (6.4%). Common laboratory abnormalities revealed thrombocytosis (51.4%), eosinophilia (48.6%), elevated erythrocyte sedimentation rate (42.9%), and elevated liver enzymes (30.3%). Hepatosplenomegaly and lymphadenopathy were found to be statistically significantly correlated with immunodeficiency syndrome (P < 0.05). No other clinical or laboratory findings were associated with any specific etiology. Preexisting skin diseases (53.2%), including atopic dermatitis (23.4%), psoriasis (17.0%), pityriasis rubra pilaris (10.6%), and seborrheic dermatitis (2.1%), were the most common causes of ED. Other causes were primary immunodeficiency (12.8%), congenital ichthyosis (10.6%), drugs (10.6%), metabolic disorders (4.3%), and unknown etiology (8.5%). Mortality rate was 8.5%. Limitation: Some data from this retrospective study may have been missing and some investigations may not have been performed in all patients. Conclusion: The most common etiology of ED in children was preexisting skin diseases. Prognosis was poor in immunodeficiency disorders.

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Background: Despite not finding favor with a number of apex global bodies including the American Heart Association, American College of Cardiology, United States Preventive Services Task Force, exercise stress test continues to be performed customarily among asymptomatic individuals to screen for potential presence of ischemic heart disease. Materials and Methods: This retrospective study comprises a successive total of 2042 individuals who were put through a screening stress test as a part of their health checkup package. A telephonic communication was made with all the persons who had been advised further assessment such as repeat stress test, drug therapy, stress thallium testing, or coronary angiography. The compliance of this group to the advice offered was noted along with their subjective sense of satisfaction with the overall process. Based on these inputs the practical utility of stress test was calculated as either good or poor. Results: Out of 2042 individuals who underwent stress test, 183 (9%) were reported as positive. Out of these 183 individuals only 132 with positive stress test could be contacted telephonically. After a detailed analysis of their compliance with the advice offered, it was noted that the screening had best practical utility when the test report was negative. Only one person with a positive report had followed the advice to the logical end by undergoing coronary artery bypass grafting and had a likely good practical utility. In rest of the scenarios, the practical utility was poor. Conclusion: The screening exercise stress tests are sought by most individuals as means of reassurance about health. Its practical significance in all other scenarios is poor. Therefore, unless definite indications exist, stress test should not be performed as a screening test.

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The most common clinical presentation of patients with ankle and foot pathology is pain and swelling followed by pain alone who attends the musculoskeletal clinic or orthopedic department. This leads to disability in performing regular normal activities. Although magnetic resonance imaging is gold standard for musculoskeletal pathologies, but the recent improvement of technology, wide availability, and radiation-free modality makes ultrasound the first line of investigation in musculoskeletal pathologies. This pictorial essay based on high-resolution ultrasonography (HRSG) study of 70 patients at our institute describes the role of HRSG in the evaluation of spectrum of various pathologies of ankle and foot with their positive sonographic findings.

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