Headache as the sole manifestation of Wilson’s disease

Wilson’s disease (WD) is an inborn error of copper metabolism caused by a mutation to the copper-transporting gene ATP7B. In the first decade of life, hepatic involvement predominates but neurological manifestations occur in the third or fourth decades. Studies showed Indian children with neuro WD present with behavior abnormality, speech and cognitive impairment, sub-clinical affection of visual pathway, and autonomic function. As a treatable disease, WD should be detected early in the course of the disease by any health professional at any care level, but the rare prevalence of the disease explains the lack of awareness of this disease. Even a high index of suspicion about this entity gets more difficult when the rare and atypical symptom is the only presentation of the disease. Here, we present the case of a 15-year-old girl with worsening headache for the past 3 months as the only neurological manifestation of WD, and that also without any evidence of hepatic involvement.

Applicability of near-miss tools to address maternal mortality

In India, there is a mandate to increase institutional delivery, which has led to an overwhelming response in health-care delivery. The maternal mortality rate has been an essential yardstick, used by health-care planners to assess the quality of obstetric services. As of late, there has been a shift in focus to maternal near-misses (MNMs) as a more valuable indicator of maternal health than the maternal mortality rate. The aim of this research article was to compare various standardized tools to address MNM, namely, the World Health Organization MNM tool, The Government of India's facility-based MNM review tool, etc.

Variability in the Epithelial Expression of Ki67 and Polarization Characteristics of Collagen in Odontogenic Cysts and Tumours

ABSTRACT Objective: To assess the proliferation of epithelium (using the Ki67 index) and the polarization pattern of collagen in selected odontogenic cysts and tumours. In addition, an exploratory analysis of the effect of inflammation on the proliferation rate was done. Material and Methods: Following immunohistochemical staining, the labelling/proliferation index of Ki67 was calculated. The thickness and corresponding polarization colour of 100 juxta-epithelial picrosirius red-stained collagen fibers were assessed using linear micrometry with an eyepiece reticule under × 1000 magnification. Inflammation was graded subjectively as mild, moderate, and severe. Results: Overall Ki-67 expression was higher in the radicular cyst, Odontogenic Keratocyst, Ameloblastoma, while suprabasal Ki-67 positivity was maximum in Odontogenic Keratocyst. The stromal collagen fibers in Ameloblastoma showed predominantly green birefringence, whereas Odontogenic Keratocyst had orange birefringence. There was no significant association of inflammation with Ki-67 expression or birefringence patterns. Conclusion: The highest Ki67 expression in the radicular cyst, followed by Odontogenic Keratocyst and Ameloblastoma. Differences in the collagen maturation pattern were noted innately in five lesions studied and were further influenced by inflammatory changes. Epithelial proliferation and concomitant expression of thickness and maturity of the stromal collagen are innate features of the lesion further influenced by inflammation in various odontogenic cysts and tumours and may, in turn, guide the clinical behavior.

Third Molar Maturity Index as an Efficient Tool for Prediction of Age in a Sample Indian Population - A Retrospective Study

Introduction: The age of 18 years is an important milestone for legal matters, and developmental parameters of teeth are often used for estimation of this age. The aim of this study is to assess the efficiency of third molar maturity index (I3M) in establishing age of 18 years in Dakshina Kannada population. Methods: A total of 700 orthopantomograms were retrieved from the archives of the radiology department of Manipal College of Dental Sciences, Mangalore. The length and the width of the open apex of the mandibular left third molar was assessed using Image J software and the Third molar maturity Index (I3M) was calculated and correlated with the age of the individual. Results: Receiver operating characteristic (ROC) curve analysis showed the Area under curve of 0.94 and 0.96 in females and males, respectively, for prediction of age of 18 years. The cut?off of 0.08 predicted the 18?year cut?off with 97% specificity and 90.2% negative predictive value. The percentage of the accuracy was 80.23% if the I3M was <0.08. Conclusion: The efficiency of the cut?off of 0.08 of I3M has been tested in various population including Kosovar, Peruvian, South Indian, Libyan, Montenegro, Croatian, African (Botswana), Albanian, and Serbian. Our study also shows the efficiency of the same in South Indian Dakshina Kannada population.

An Exploration of Pulmonary Fitness of Construction Workers in Delhi NCR in Light of the Building and Other Construction Workers Act, 1996

Workers in the construction sector are exposed to high concentrations of particulate matter at their workplace. This increases their susceptibility to various respiratory diseases, particularly chronic obstructive pulmonary disease (COPD) and acute respiratory distress syndrome (ARDS). The study reports comparative pulmonary fitness and hematological parameters of the migrant workers in the construction sector versus other sectors in Delhi. Parameters such as forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1 ), the ratio of FEV1 to FVC, and peak expiratory flow were measured in both groups using a spirometer. We observed significant differences (P < 0.05) in FEV1 and FVC between both groups. The study thus confirms that workers exposed to poor air quality at the construction site are susceptible to respiratory diseases, particularly ARDS. All of this reflects the poor enforcement of the adequate safety measures well enlisted in social legislations such as the Building and Other Construction Workers Act.

Diagnosis and Management of Global Development Delay: Consensus Guidelines of Growth, Development and Behavioral Pediatrics Chapter, Neurology Chapter and Neurodevelopment Pediatrics Chapter of the Indian Academy of Pediatrics

Justification: Global developmental delay (GDD) is a relatively common neurodevelopmental disorder; however, paucity of published literature and absence of uniform guidelines increases the complexity of clinical management of this condition. Hence, there is a need of practical guidelines for the pediatrician on the diagnosis and management of GDD, summarizing the available evidence, and filling in the gaps in existing knowledge and practices. Process: Seven subcommittees of subject experts comprising of writing and expert group from among members of Indian Academy of Pediatrics (IAP) and its chapters of Neurology, Neurodevelopment Pediatrics and Growth Development and Behavioral Pediatrics were constituted, who reviewed literature, developed key questions and prepared the first draft on guidelines after multiple rounds of discussion. The guidelines were then discussed by the whole group in an online meeting. The points of contention were discussed and a general consensus was arrived at, after which final guidelines were drafted by the writing group and approved by all contributors. The guidelines were then approved by the Executive Board of IAP. Guidelines: GDD is defined as significant delay (at least 2 standard deviations below the mean with standardized developmental tests) in at least two developmental domains in children under 5 years of age; however, children whose delay can be explained primarily by motor issues or severe uncorrected visual/ hearing impairment are excluded. Severity of GDD can be classified as mild, moderate, severe and profound on adaptive functioning. For all children, in addition to routine surveillance, developmental screening using standardized tools should be done at 9-12 months,18-24 months, and at school entry; whereas, for high risk infants, it should be done 6-monthly till 24 months and yearly till 5 years of age; in addition to once at school entry. All children, especially those diagnosed with GDD, should be screened for ASD at 18-24 months, and if screen negative, again at 3 years of age. It is recommended that investigations should always follow a careful history and examination to plan targeted testing and, vision and hearing screening should be done in all cases prior to standardized tests of development. Neuroimaging, preferably magnetic resonance imaging of the brain, should be obtained when specific clinical indicators are present. Biochemical and metabolic investigations should be targeted towards identifying treatable conditions and genetic tests are recommended in presence of clinical suspicion of a genetic syndrome and/or in the absence of a clear etiology. Multidisciplinary intervention should be initiated soon after the delay is recognized even before a formal diagnosis is made, and early intervention for high risk infants should start in the nursery with developmentally supportive care. Detailed structured counselling of family regarding the diagnosis, etiology, comorbidities, investigations, management, prognosis and follow-up is recommended. Regular targeted follow-up should be done, preferably in consultation with a team of experts led by a developmental pediatrician/ pediatric neurologist.

Closure of meningomyelocele defects using various types of keystone-design perforator island flaps

Background@#Various methods have been described to close large meningomyelocele defects, but no technique has been proven superior to others. This study presents cases of meningomyelocele defect closure with a keystone-design perforator island flap. @*Methods@#A retrospective study was performed on 14 patients with meningomyelocele defects closed using various types of keystone flaps. @*Results@#The median age of the patients at surgery was 10.5 days (range, 1–369 days) and the average defect size was 22.5 cm2 (range, 7.1–55.0 cm2). The average operative time for defect closure was 89.6 minutes (range, 45–120 minutes). Type IV bilateral keystone flaps were used for four defects, type IV unilateral flaps for six defects, type IIA flaps for two defects, and type III flaps for two defects. @*Conclusions@#All the defects healed completely with no major complications. The keystone-design perforator island flap is a reliable, easy, and fast technique to close large meningomyelocele defects.

Closure of meningomyelocele defects using various types of keystone-design perforator island flaps

Background@#Various methods have been described to close large meningomyelocele defects, but no technique has been proven superior to others. This study presents cases of meningomyelocele defect closure with a keystone-design perforator island flap. @*Methods@#A retrospective study was performed on 14 patients with meningomyelocele defects closed using various types of keystone flaps. @*Results@#The median age of the patients at surgery was 10.5 days (range, 1–369 days) and the average defect size was 22.5 cm2 (range, 7.1–55.0 cm2). The average operative time for defect closure was 89.6 minutes (range, 45–120 minutes). Type IV bilateral keystone flaps were used for four defects, type IV unilateral flaps for six defects, type IIA flaps for two defects, and type III flaps for two defects. @*Conclusions@#All the defects healed completely with no major complications. The keystone-design perforator island flap is a reliable, easy, and fast technique to close large meningomyelocele defects.

Angiogenesis-Targeted 68Ga-DOTA-RGD 2 PET/CT Imaging: a Potential Theranostic Application in the Case of Chondrosarcoma / 대한핵의학회잡지

Chondrosarcoma is a cartilaginous tumor of mesenchymal origin. The histology and grade of the tumor determine the chances of relapse and survival. These tumors usually respond poorly to chemo-radiotherapy in cases of non-resectable and recurrent disease. 18F-FDG PET/CT has been used in evaluation of recurrence. However, these tumors show only mild to moderate FDG avidity due to their lower mitotic activity and large acellular matrix. These tumors are known to have a high degree of angiogenesis, especially in those of higher grade. We present a case of a 53-year-old man with grade II chondrosarcoma of the left femur showing only mild avidity on 18F-FDG PET/CT but showing moderate to intense tracer avidity on 68Ga-DOTA-RGD2PET/CT. This may enable the use of angiogenesis-targeted positron and beta-emitting radiopharmaceuticals as a potentially new theranostic alternative treatment in cases of refractory metastatic chondrosarcoma.

Ovarian maldescent: a case report and discussion

Undescended ovary or ovarian maldescent is a rare condition usually associated with mullerian malformation and typically found in case of infertility during their workup. Embryologically, the ovarian development from the genital ridge is totally different from the paramesonephric duct which forms the uterus and fallopian tubes. Still the ovarian maldescent has strong association with mullerian abnormalities, especially with unicornuate uterus. Therefore, supporting the hypothesis that abnormal migration could affect their normal fusion. In strong correlation of ovarian maldescent with mullerian abnormality here we report case of a patient presented to infertility clinic with unicornuate uterus with unilateral renal agenesis diagnosed to have unilateral undescended ovary with conclusion that the accurate diagnosis is important and radiological effort should be made to locate the undescended ovary if the ovary could not be found in normal location especially when associated with mullerian abnormality.

King Vision Videolaryngoscopy Versus Direct Laryngoscopy in Patients Requiring Endotracheal Intubation for General Anaesthesia: A Comparative Study

With new advances in technology, intubation using video laryngoscopy has been gaining popularity, particularlyin patients with difficult airways or as rescue devices in failed intubation attempts. This study was done tocompare the effectiveness of King Vision video laryngoscope (KVL) and Macintosh laryngoscope whenperforming tracheal intubation under general anesthesia Eighty patients requiring endotracheal intubationduring general anaesthesia were randomly assigned into two groups to undergo tracheal intubation usingeither a King Vision video laryngoscope (n=40) or Macintosh laryngoscope (n=40). The primary outcomeswere the time of intubation and Cormack-Lehane grading and secondary outcomes were number of attemptsand optimisation manoeuvres required and complications related to laryngoscopy and intubation. King Visionvideo laryngoscope was found to be significantly better than the Macintosh laryngoscope in terms of Cormackand Lehane grading, requirement of optimisation manoeuvres and need of second attempt for intubationexcept time of intubation which was comparable between the two groups.

Oral antifungal: the safety and efficacy of oral itraconazole in dermatophytosis

Background: The systemic antifungals like Griseofulvin, Itraconazole, Terbinafine, Ketoconazole and Fluconazole are widely used for superficial fungal infection. Hepatotoxicity with oral antifungals is well established fact. The rate of transient asymptomatic changes in liver function tests accounts for about 0.5 - 10% of all patients treated with systemic antifungals. Clinical hepatic toxicity is seen less frequently. The aim of this study is to evaluate the effect of oral Itraconazole on hepatic function and it抯 efficacy in patients with extensive dermatophytosis.Methods: The total of 524 patients with extensive dermatophytosis were included in our study which was conducted in a tertiary care hospital in Navi Mumbai.Results: Itraconazole, a systemic antifungal agent is efficiently used in treatment of superficial and deep mycoses. It inhibits fungal cytochrome P450 dependent enzyme and thus impaires conversion of lanosterol to ergosterol. Adverse reactions to itraconazole includes drug reactions, gastrointestinal upset, headache, dizziness, thrombocytopenia, gynecomastia, reversible edema of extremities and metabolic side effects like hypokalemia, and hypertriglyceridemia. The level of hepatic transaminases increases in about 1%-5% of patients who have received continuous therapy with systemic itraconazole. Clinical hepatitis rarely occurs in patients and, recovery generally ensues with the cessation of medication.Conclusions: The baseline and post treatment liver function test is important to monitor if patient is on higher dose and longer duration of itraconazole therapy. The screening for high risk patients like poor liver function test, history of alcoholism, history of liver disease should be taken before stating the therapy.

The role of pregnancy-associated plasma protein-a level in the first trimester of pregnancy and clinical outcome in an urban referral centre

Background: Estimation of serum PAPP-A levels studied predictability for adverse perinatal outcome. This case control study tries to establish the association between low PAPP-A levels among the pregnant woman and adverse maternal foetal outcome.Methods: This is an case-control study during 2017-2018 in the women delivered at Department of OBG at Mehta Hospitals. Women delivered in the labour room  had a first trimester screening of PAPP-A level were explained, taken informed consent, questionnaire which include detailed antenatal history, mode of delivery and baby data.  Depending upon outcome, the subjects are classified as case group or control group, out of the study sample of 264 subjects, 88 patients who had complications were taken as cases and 176 patients with no complications taken as control were undertaken.Results: Low PAPP-A level (<0.5 MoM) showed high incidence of PIH and preeclampsia, followed by IUGR and Preterm. PAPP-A level >0.5 MoM, normal outcome is more than the adverse outcome. The difference in the PAPP-A levels is statistically significant. In women with low PAPP A level, low birth weight found statistically significant when compared with <0.5 PAPP A level. The sensitivity of PAPP A levels in identifying the complicated outcomes was 17.04%.The specificity was 98.85%. The positive predictive value of predicting the complications was 88.23% and negative predictive value of 70.44%.Conclusions: The low PAPP-A levels confirmed during first trimester of pregnancy is associated with adverse maternal and foetal outcome such as PIH, preeclampsia, preterm, IUGR and LBW.

Assessing the new ICD-MM classification for assigning the cause of maternal mortality at a tertiary centre in Western India: a retrospective study

Background: The World Health Organisation (WHO) in 2012 introduced the 10th revision of International Classification of Disease (ICD 10) to deaths in pregnancy, labour and puerperium (ICD-MM) for consistent collection, analysis and interpretation of information on maternal deaths. The proper use of this classification requires training to avoid heterogeneity and error in the classification of maternal deaths.Methods: We analysed the Maternal Death Review (MDR) forms of 295 deaths over a period of 5 years (January 2014 to December 2018 inclusive) occurring at a tertiary health centre in Western India. The ICD-MM classification was used to reassign the cause of death.Results: There were 295 deaths in women during pregnancy, childbirth and puerperium during the 5 year period. Of these there were 294 maternal deaths and one coincidental death. There were 173 deaths of the direct type (58.84%), 105 deaths of the indirect type (35.71%) and 16 deaths (5.44%) of the unspecified type. Obstetric haemorrhage was  the highest contributor to direct deaths (23.8%) and anaemia contributed to the maximum deaths from indirect causes (13.6%) followed by liver diseases in pregnancy (10.54%).Unanticipated complications of management accounted for 2% of the total deaths. There was considerable inaccuracy in assigning cause of death by consultants who were untrained in the use of the ICD-MM classification.Conclusions: ICD-MM classification promotes an accurate assignment of the cause of death. Training of healthcare providers performing maternal death reviews in the use of this classification is essential to identify accurate underlying cause of death and contributory conditions.

Task sharing for family planning services, Burkina Faso

Problem: In Burkina Faso, the coverage of services for family planning is low due to shortage of qualified health staff and limited access to services.Approach:Following the launch of the Ouagadougou Partnership, an alliance to catalyse the expansion of family planning services, the health ministry created a consortium of family planning stakeholders in 2011. The consortium adopted a collaborative framework to implement a pilot project for task sharing in family planning at community and primary health-care centre levels in two rural districts. Stakeholders were responsible for their areas of expertise. These areas included advocacy; monitoring and evaluation; and capacity development of community health workers (CHWs) to offer oral and injectable contraceptives to new users and of auxiliary nurses and auxiliary midwives to provide implants and intrauterine devices. The health ministry implemented supportive supervision cascades involving relevant planning and service levels.Local setting In Burkina Faso, only 15% (2563/17 087) of married women used modern contraceptives in 2010.Relevant changes Adoption of new policies and clinical care standards expanded task sharing roles in family planning. The consortium trained a total of 79 CHWs and 124 auxiliary nurses and midwives. Between January 2017 and December 2018, CHWs provided injectables to 3698 new users, and auxiliary nurses or midwives provided 726 intrauterine devices and 2574 implants to new users. No safety issues were reported.Lessons learnt The pilot project was feasible and safe, however, financial constraints are hindering scale-up efforts. Supportive supervision cascades were critical in ensuring success

Reference range of random urinary calcium creatinine ratio in North Indian children and adolescents

PURPOSE: Urinary calcium creatinine ratio (UCaCrR) is a reliable indicator for monitoring hypercalciuria following vitamin D supplementation. However, the reference range varies from region to region. Previous studies did not take vitamin D and parathyroid hormone status into account while evaluating UCaCrR. Hence, we undertook this study to establish the 95th percentile of UCaCrR as an indicator of hypercalciuria in North Indian children and adolescents. METHODS: Four hundred seventy-three participants (boys 62.2%, girls 37.8%) with adequate dietary calcium intake, normal serum levels of 25-hydroxy-vitamin D (>20 ng/mL), and without secondary hyperparathyroidism following supplementation were selected for evaluation of UCaCrR. RESULTS: The mean age and body mass index of subjects were 11.2±2.6 years and 18.0±3.6 kg/m2, respectively. The 95th percentile of UCaCrR in the study population was 0.126. The mean, median, and 95th percentile of UCaCrR was significantly higher in prepubertal children (age ≤10 years) (0.0586±0.0374, median=0.0548, 95th percentile=0.136) compared to those >10 years old (0.0503±0.0363, median=0.0407, 95th percentile=0.123, P=0.02). No significant difference in UCaCrR was observed between genders and different weight categories. CONCLUSIONS: UCaCrR of 0.13 defines the cutoff value for hypercalciuria in North Indian children and adolescents with adequate dietary intake of calcium and sufficient serum vitamin D levels.

Supplementation with Three Different Daily Doses of Vitamin D3 in Healthy Pre-pubertal School Girls: A Cluster Randomized Trial

Objective: To compare the adequacy and efficacy of differentdoses of vitamin D3 in pre-pubertal girls.Design: Cluster Randomized controlled trial.Setting: Public school in Delhi, India, between August 2015 andFebruary 2016.Participants: 216 healthy pre-pubertal girls, aged 6.1-11.8 years.Intervention: Daily supplementation with 600 IU (n=74), 1000 IU(n=67) or 2000 IU (n=75) of vitamin D3 under supervision for 6months.Outcome measures: Primary: Rise in serum 25 hydroxy VitaminD (25(OH)D); Secondary: Change in bone formation andresorption markers.Results: Following 6 months of supplementation, the mean (SD)rise in serum 25(OH)D was maximum with 2000 IU (24.09 (8.28)ng/mL), followed by with 1000 IU (17.96 (6.55) ng/mL) and 600 IU(15.48 (7.00) ng/mL). Serum 25(OH)D levels of ?20 ng/mL wereseen in 91% in 600 IU group , 97% in 1000 IU group and 100% in2000 IU group. The overall mean (SD) rise in urinary calciumcreatinine ratio (0.05 (0.28) to 0.13 (0.12) mg/mg), and serumprocollagen type I N-terminal propeptide (538.9 (199.78) to 655.5(218.24) ng/mL), and reduction in serum carboxy-terminaltelopeptide (0.745 (0.23) to 0.382 (0.23) ng/mL) was significant(P<0.01). The change in the above parameters was comparableamong the three groups after adjustment for age.Conclusion: Daily vitamin D supplementation with 600 IU to 2000IU for 6 months results in Vitamin D sufficiency in >90% of pre-pubertal girls

Comparative Study Of High Dose Versus Incremental Doses Of Sevoflurane For Induction In Paediatric Patients.

ackground: We compared high doses of sevoflurane with incremental doses of sevoflurane during induction in paediatric patients. Methods: The present prospective, randomized single blind study conducted in the Dept. of Anaesthesiology PGIMS, Rohtak. . A total of 80 patients of either sex and age ( 1-6 yrs) years presenting for elective surgery under general anesthesia were included in the study.Ethical clearance and written informed consent taken for study. . Patients were randomly divided in 2 groups (I and II) of 40 each.Group I patients ) were induced with high dose (8%) sevoflurane whereas group II patients were induced with incremental dose of sevoflurane (1% to 8%). Study parameters (HR, BP, SpO2) were recorded just before starting induction (T0) and at 30 seconds (T1), 60 seconds (T2), loss of eyelash reflex (T3) and after insertion of PLMA (T4). Results: Our primary outcome, time required for induction of anaesthesia in Group I was found to be 60.225 ±4.932 secs and for the Group II it was found to be 84.9± 6.953secs.The difference was highly significant between the two groups (p value = 0.0001). Conclusion: This randomised,blind controlled study suggests that the time for induction of anesthesia could be significantly shortened using sevoflurane with a high concentration primed circuit as compared with incremental induction technique. The effect of both these techniques on haemodynamic parameters was statistically insignificant. Also both the techniques were safe and well tolerated in paediatric patients.

Use of scale for the growth study of Indian major carp (Cirrhinus mrigala Ham., 1822) in tropical freshwater

Age and growth study provide detail information on the life history, ecology of fish and habitat which is important to manage the water body for fish production and optimization of harvestable size. Scale based age and growth of Indian major carp (Cirrhinus mrigala, Ham. 1822) from Vallabhsagar reservoir was studied and minimum (25.0 cm and 145.0 g) and maximum (82.0 cm and 6500.00 g) length and weight of fish were observed. In length-weight relationship, constant (a) -2.212, exponent of slope; (b) 3.141 and correlation coefficient (r) 0.937 were determined. The back calculations of scale study evident that the fish attains the length and weight (27.07 cm, 243.42 g) at 1st, (38.18 cm, 644.26 g) at 2nd, (46.78 cm, 1222.73 g) at 3rd, (53.30 cm, 1813.97 g) at 4th, (62.82 cm, 2811.34 g) at 5th and (71.39 cm, 4248.94 g) at 6th year of age. The growth parameters, such as index of species average size (Øh) 11.86 and index of weight growth (ØCw) 81.78 were observed. Similarly, growth constant average (Cltav) were (0.410 and 0.211) indicating for two growth phases existed in fish, first is fast growing phase up to 2 years shows sexual immaturity and second is slow growing phase 2 years onwards shows sexual maturity. The results of this studies concluded that the growth of studied fish was satisfactory and environment of Vallabhsagar reservoir is conducive for survival of Indian major carp (Cirrhinus mrigala).