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Background: Metacarpal fractures are among the most common fractures majority of which are treated conservatively. There are numerous indications and techniques for operative xation. The purpose of this research is to study the management of metacarpal fracture treated with intramedullary pre-bent K-wires using Bouquet technique. 100 patients aged between 18-60yea Materials and Method: rs, with 100 closed metacarpal fractures involving shaft and neck region, which were ≤2weeks old, were included in this study. Radiograph of the involved hand anteroposterior (AP) and oblique views were obtained. Postoperative immobilization and rehabilitation regimen were followed for all the patients. Outcome assessment was done using Visual analogue score (VAS), Disabilities of the arm, shoulder and hand (DASH) score, Total active movement (TAM) and grip strength. Radiographs were taken at regular intervals. Descriptive statistical analysis was done using MS Excel 2019. Result and Observations: Out of 100 patients, 73 were male, with overall mean age of 31.76±10.46 years. Right side was affected in 63 patients, however the dominant side was involved in 73 cases. Metacarpal shaft was involved more often (77/100 cases). Most common cause was sports injury. At nal follow-up, mean post-op VAS was 0.33±0.54, mean post-op DASH score was 2.44±3.91, mean TAM score was 232.33±22.38º and the mean grip strength was 94.37±6.53%. Pin tract infection was seen in 13 patients; pin loosening in 10 patients and partial stiffness in 7 patients. Conclusion: The proposed technique is simple, minimally invasive, less time consuming and provides satisfactory stabilization for early union. This xation technique also showed good functional and radiographic outcome with few complications

Industrial growth with increased automation has made electricity as the key resource for the modern society. Electricpower in today’s context has assumed importance as necessity of life next to food, shelter and clothes. This also impliesthat all elements of entire chain of power generation, transmission and distribution needs to offer fail safe performanceat all instance of time, and in case of any unforeseen faults, are required not only to provide effective protection to powersystem elements and isolate the fault but also ensure continuity of electric supply for rest of network, by ensuring veryminimal region/loads over faulted section remains without power. Power system protection hence remains one of the mostcomplex discipline in electrical engineering. Protective relays are the decision-making devices in the protection scheme.These relays have undergone, through more than a century, important changes in their architecture, functionalities andtechnologies. In this paper, after giving insight on the evolution of protective relays from onset of electrical energy tocurrent deployment, emerging trends are also touched upon. Specific emphasis on very recent deployment of CentralizedProtection and Control solution is then described. Finally, future trends are also highlighted at the end.

Scaphoid bone fracture is one of the commonest carpal bone fractures. It is predominantly seen in young population. Unstable or displaced scaphoid fractures can be treated either by primary use of cast immobilization alone, by use of Open Reduction Internal Fixation (ORIF) technique or by percutaneous fixation with Herbert screw under direct C arm fluoroscopic guidance. This study aims to review clinical, radiological and function outcome following ORIF technique Vs Percutaneous Fixation Technique in scaphoid fractures.Cases of acute scaphoid fractures who came to our department and met inclusion and exclusion criterias were identified and included in this study. Clinical details of these patients were obtained and scaphoid radiographs were taken. Injuries were graded as per Herbert and Fisher Classification. All our cases underwent grip-strengthening exercises and relevant physiotherapy Clinico-radiological details of our cases were obtained at each follow-up. Final follow-up of our cases was performed using Modified Mayo Wrist Score (MMWS). Grip strength was graded as per Medical Research Council (MRC) scale and range of motion was assessed using goniometer.A total of 40 cases were enrolled in our study. 16 patients (40% cases) underwent percutaneous herbert screw fixation and rest of our cases underwent open reduction and internal fixation. Radiological union in cases treated with percutaneous fixation was faster as compared to cases treated by ORIF. Also patients treated with percutaneous method demonstrated higher mean range of motion score, mean grip strength score and activity score. In our study excellent results have been obtained in cases treated with percutaneous approach in terms of post-operative morbidity and this approach also allows earlier return to sports and work thereby necessitating importance of this approach over ORIF approach in a developing resource poor country like India.

Background: Several studies have documented that BCG vaccine which is a part of Primary Immunization in certain countries not only prevent occurrence of Tuberculosis in children but also have numbers of heterologus protective effects which subsequently decreases the chances of occurrence of sepsis and various respiratory infections during childhood. In the present pandemic of SARS-Cov2 it is observed worldwide that Pediatric patients neither show severity nor adverse outcome of COVID 19 as compare to Adult population. Objectives: 1. To nd out the proportion of BCG vaccination among COVID 19 positive paediatric patients admitted in a tertiary care centre. 2. To document the progress of the COVID 19 disease between BCG vaccinated COVID 19 patients and BCG non-vaccinated COVID 19 patients admitted in the tertiary care centre. It is a cross Methods: sectional study conducted in All COVID 19 positive patients admitted in the department of Paediatrics of the tertiary care centre during the study period shall be included. All 57 COVID 19 positive Results: children were BCG vaccinated. Majority of children (98.2%) recovered from COVID 19 and discharge from hospital, only 1.8% death occurred due to COVID 19 in children. we conclude that BCG vaccination has a protective Conclusion: effect against severity of COVID-19 in childhood. The main limitation of study, there were not any unvaccinated covid-19 positive children admitted in hospital during study period.

Background: Hip fractures in the elderly are commonly associated with osteoporosis, and the surgical outcome is therefore inuenced by its concurrent management. Understanding the role of the osteoporosis drug and its healing mechanism with the fracture is crucial for managing the fracture and osteoporosis properly. The purpose of our study is to determine whether bisphosphonate administration in stable intertrochanteric (IT) fractures helps in early fracture healing. Aim: The aim of the study is to assess the effect of bisphosphonates on intertrochanteric fracture among elderly people aged >50 years in preoperatively conrmed osteoporotic patients. Methodology: This is a non-randomized study done in the Department of Orthopaedics at Assam Medical College, Dibrugarh for a period of 1 year, from May 2021 to April 2022. The total sample size attained was 50, consisting of 25 patients in each group. Demographic details like age, sex, mean bone mineral density of hip and spine, and mean body mass index were obtained. The collected data was entered into MS Excel and statistics were done with SPSS 16. Results: Among the study participants, the majority were female: 15(60%) in the bisphosphonate group and 19(76%) in the control group; the rest were males. The mean age of the study participants was 56±2.1 years. In both groups, xation of the fracture was done by intramedullary nailing. The mean time difference in fracture healing between the two groups was not found to be signicant. Conclusion:The mean healing time for the fracture healing was not found to be statistically signicant when compared with the placebo group

We present the synthesis, characterization and radical ring-opening polymerization (rROP) capabilities of thionoisochromanone (TIC), a fungi-derivable thionolactone. TIC is the first reported thionolactone to readily homopolymerize under free radical conditions without the presence of a dormant comonomer or repeated initiation. Even more, the resulting polymer is fully degradable under mild, basic conditions. Computations providing molecular-level insights into the mechanistic and energetic details of polymerization identified a unique S,S,O-orthoester intermediate that leads to a sustained chain-end. This sustained chain-end allowed for the synthesis of a block copolymer of TIC and styrene under entirely free radical conditions without explicit radical control methods such as reversible addition-fragmentation chain transfer polymerization (RAFT). We also report the statistical copolymerization of ring-retained TIC and styrene, confirmed by elemental analysis and energy-dispersive X-ray spectroscopy (EDX). Computations into the energetic details of copolymerization indicate kinetic drivers for ring-retaining behavior. This work provides the first example of a sustainable feedstock for rROP and provides the field with the first six-membered monomer susceptible to rROP, expanding the monomer scope to aid our fundamental understanding of thionolactone rROP behavior.

We described workflows and methodologies for fully automated parametric imaging using clinical positron emission tomography (PET) systems with continuous bed motion (CBM) which aim to enable physicians to practice parametric PET routinely. The key components of our implemented methods include automatic generation of image-derived blood input functions, accurate tracking of slice imaging time during CBM acquisitions, and parametric image formation with advanced algorithms. Locations of the left ventricle and the descending aorta were automatically detected from high resolution anatomical images and registered to dynamic PET images to generate blood input functions. A method to accurately track dynamic scans and calculate time information for whole body CBM parametric PET was implemented based on bed position tags. This approach of calculating time information based on finely sampled bed position tags can be applied to flexible scan modes with various scan speeds over different body regions. We applied the calculated slice time information for whole body parametric imaging using a linear Patlak model. Parametric images were reconstructed with a nested expectation maximization (EM) algorithm. We demonstrated that parametric images generated by our automated workflow can provide quantitative parameters in addition to standardized uptake value images. We presented results for a uni-direction dynamic scan from a Biograph mCT system and a bi-direction dynamic scan from a Biograph Vision system.

© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ., Objective: Alcohol-related liver disease (ALD) ranges from never-decompensated ALD (ndALD) to the life-threatening decompensated phenotype, known as alcohol-related hepatitis (AH). A multidimensional study of the clinical, histological and molecular features of these subtypes is lacking. Design: Two large cohorts of patients were recruited in an international, observational multicentre study: a retrospective cohort of patients with ndALD (n=110) and a prospective cohort of patients with AH (n=225). Clinical, analytical, immunohistochemistry and hepatic RNA microarray analysis of both disease phenotypes were performed. Results: Age and mean alcohol intake were similar in both groups. AH patients had greater aspartate amino transferase/alanine amino transferase ratio and lower gamma-glutamyl transferase levels than in ndALD patients. Patients with AH demonstrated profound liver failure and increased mortality. One-year mortality was 10% in ndALD and 50% in AH. Histologically, steatosis grade, ballooning and pericellular fibrosis were similar in both groups, while advanced fibrosis, Mallory-Denk bodies, bilirubinostasis, severe neutrophil infiltration and ductular reaction were more frequent among AH patients. Transcriptome analysis revealed a profound gene dysregulation within both phenotypes when compare to controls. While ndALD was characterised by deregulated expression of genes involved in matrisome and immune response, the development of AH resulted in a marked deregulation of genes involved in hepatocyte reprogramming and bile acid metabolism. Conclusions: Despite comparable alcohol intake, AH patients presented with worse liver function compared with ndALD patients. Bilirubinostasis, severe fibrosis and ductular reaction were prominent features of AH. AH patients exhibited a more profound deregulation of gene expression compared with ndALD patients., This work was supported by the National Institute on Alcohol Abuse and Alcoholism (NIAAA 1U01AA021908-01, 1U01AA020821 and P50AA011999) and the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK P30DK120531 and P30DK120515). PSwas supported by Fondo de Investigación Sanitaria Carlos III, cofinanced by Fondo Europeo de Desarrollo Regional (FEDER), Unión Europea, 'Una manera de hacer Europa' PI17/00673; PI20/00765 and Miguel Servet, CPII16/0004. MV-C is a recipient of the Juan Rodés JR19/00015. JA is a recipient of a grant from Agencia Estatal de Salud (PI20/01663).

To investigate the kinetics ofTwenty-four oncological patients underwent dynamicWe report estimates of kinetic parameters and metabolic rate of glucose consumption (MRWe performed pharmacokinetic modelling of multiple organs using linear and non-linear models using dynamic total-body

Introduction: Sickle Cell Disease (SCD) is an inherited chronic haemolytic anaemia. The diseased person suffers from various complications such as anaemia, frequent infection, fever, hand-foot syndrome, stroke, etc. Puberty changes includes the appearance of the secondary sexual characteristics, increase in height, change in body composition and development of reproductive capacity. Aim: To study the sexual maturity and effect of multiple blood transfusions in adolescents suffering from SCD. Materials and Methods: It was a cross-sectional study conducted on 35 adolescents of age group 11 to 15 years, suffering from SCD. Study was conducted over a period of six months from March 2018 to September 2018 at Department of Paediatrics. SCD was diagnosed by Haemoglobin (Hb) electrophoresis. Weight and height were measured of all the participants. For assessing the sexual maturity, Tanners staging was used. Unpaired t-test was done for data analysis. Results: The mean age of the patients was 13.03±1.7 years. There were 25 males and 10 females. The mean age of male patients between Tanner stage 2(14.63±0.52 years) and Tanner stage 3 (14.75±0.5 years) was significantly higher than the Indian data for males (11.3 and 12.8 years, respectively). The mean age of female patients between Tanner stages 2 (13.5±2.12 years) and Tanner stage 3 (14.33±1.16 years) was higher than the Indian reference data for girls (10.2 and 11.6 years respectively). Conclusion: This study concluded that adolescents with SCD were significantly shorter in height and weight than the standard reference population. Sexual maturity is delayed in adolescents with sickle cell anaemia.

Purpose In PSMA-ligand PET/CT imaging, standardized evaluation frameworks and image-derived parameters are increasingly used to support prostate cancer staging. Clinical applicability remains challenging wherever manual measurements of numerous suspected lesions are required. Deep learning methods are promising for automated image analysis, typically requiring extensive expert-annotated image datasets to reach sufficient accuracy. We developed a deep learning method to support image-based staging, investigating the use of training information from two radiotracers. Methods In 173 subjects imaged with 68Ga-PSMA-11 PET/CT, divided into development (121) and test (52) sets, we trained and evaluated a convolutional neural network to both classify sites of elevated tracer uptake as nonsuspicious or suspicious for cancer and assign them an anatomical location. We evaluated training strategies to leverage information from a larger dataset of 18F-FDG PET/CT images and expert annotations, including transfer learning and combined training encoding the tracer type as input to the network. We assessed the agreement between the N and M stage assigned based on the network annotations and expert annotations, according to the PROMISE miTNM framework. Results In the development set, including 18F-FDG training data improved classification performance in four-fold cross validation. In the test set, compared to expert assessment, training with 18F-FDG data and the development set yielded 80.4% average precision [confidence interval (CI): 71.1–87.8] for identification of suspicious uptake sites, 77% (CI: 70.0–83.4) accuracy for anatomical location classification of suspicious findings, 81% agreement for identification of regional lymph node involvement, and 77% agreement for identification of metastatic stage. Conclusion The evaluated algorithm showed good agreement with expert assessment for identification and anatomical location classification of suspicious uptake sites in whole-body 68Ga-PSMA-11 PET/CT. With restricted PSMA-ligand data available, the use of training examples from a different radiotracer improved performance. The investigated methods are promising for enabling efficient assessment of cancer stage and tumor burden.

BackgroundArterial blood sampling is the gold standard method to obtain the arterial input function (AIF) for quantification of whole body (WB) dynamic18F-FDG PET imaging. However, this procedure is invasive and not typically available in clinical environments. As an alternative, we compared AIFs to population-based input functions (PBIFs) using two normalization methods: area under the curve (AUC) and extrapolated initial plasma concentration (CP*(0)). To scale the PBIFs, we tested two methods: (1) the AUC of the image-derived input function (IDIF) and (2) the estimatedCP*(0). The aim of this study was to validate IDIF and PBIF for FDG oncological WB PET studies by comparing to the gold standard arterial blood sampling.MethodsThe Feng18F-FDG plasma concentration model was applied to estimate AIF parameters (n= 23). AIF normalization used either AUC(0–60 min) orCP*(0), estimated from an exponential fit.CP*(0) is also described as the ratio of the injected dose (ID) to initial distribution volume (iDV).iDVwas modeled using the subject height and weight, with coefficients that were estimated in 23 subjects. In 12 oncological patients, we computed IDIF (from the aorta) and PBIFs with scaling by the AUC of the IDIF from 4 time windows (15–45, 30–60, 45–75, 60–90 min) (PBIFAUC) and estimatedCP*(0) (PBIFiDV). The IDIF and PBIFs were compared with the gold standard AIF, using AUC values and PatlakKivalues.ResultsThe IDIF underestimated the AIF at early times and overestimated it at later times. Thus, based on the AUC andKicomparison, 30–60 min was the most accurate time window for PBIFAUC; later time windows for scaling underestimatedKi(− 6 ± 8 to − 13 ± 9%). Correlations of AUC between AIF and IDIF, PBIFAUC(30–60), and PBIFiDVwere 0.91, 0.94, and 0.90, respectively. The bias ofKiwas − 9 ± 10%, − 1 ± 8%, and 3 ± 9%, respectively.ConclusionsBoth PBIF scaling methods provided good mean performance with moderate variation. Improved performance can be obtained by refining IDIF methods and by evaluating PBIFs with test-retest data.

BACKGROUND Computed tomographic coronary angiogram (CTCA) is a non-invasive test with a negative predictive value of nearly 100% for the detection of coronary artery study. While diagnostic yield of a dedicated CTCA with bubble contrast is not yet evaluated OBJECTIVE To assess the diagnostic performance of injected bubble contrast and ability to measure difference in hounsfield units and use it as a "negative contrast" in computed tomographic METHODS This is a single center, single patient study. Baseline acquisition of a non-contrast CT scan was acquired to get hounsfield unit count in the aorta and pulmonary artery- (Calcium scan protocol) 1.4 mGy (19.5 mGy/cm). Secondly, Echo contrasts (Definity) - 5mls was injected and an echocardiogram confirmed filling in the aortic region. Finally, bubble contrast (1ml air, 8mls water and 1mls blood was drawn up and agitated through a 3 way tap) - was injected, a timing run was initiated to calculate for the bubbles to opacity the pulmonary artery. The same scan protocol was used– 1.4 mGy (19.5 mGy/cm). RESULTS Hounsfield units’ difference in the aorta and pulmonary artery from baseline compared to echo contrast and bubble contrast were not significant. CONCLUSIONS We believe this is the first ever recorded case to use bubbles as CT contrast. While results were not significant, secondary to small volume of bubbles injected. Further research needs to be implemented to assess clinical difference with amount of bubbles and volume required. CLINICALTRIAL Single centre study

Background Arterial blood sampling is the gold standard method to obtain the arterial input function (AIF) for quantification of whole body (WB) dynamic 18F-FDG PET imaging. However, this procedure is invasive and not typically available in clinical environments. As an alternative, we compared AIFs to population-based input functions (PBIFs) using two normalization methods: area under the curve (AUC) and extrapolated initial plasma concentration (CP*(0)). To scale the PBIFs, we tested two methods: (1) the AUC of the image-derived input function (IDIF) and (2) the estimated CP*(0). The aim of this study was to validate IDIF and PBIF for FDG oncological WB PET studies by comparing to the gold standard arterial blood sampling. Methods The Feng 18F-FDG plasma concentration model was applied to estimate AIF parameters (n = 23). AIF normalization used either AUC(0–60 min) or CP*(0), estimated from an exponential fit. CP*(0) is also described as the ratio of the injected dose (ID) to initial distribution volume (iDV). iDV was modeled using the subject height and weight, with coefficients that were estimated in 23 subjects. In 12 oncological patients, we computed IDIF (from the aorta) and PBIFs with scaling by the AUC of the IDIF from 4 time windows (15–45, 30–60, 45–75, 60–90 min) (PBIFAUC) and estimated CP*(0) (PBIFiDV). The IDIF and PBIFs were compared with the gold standard AIF, using AUC values and Patlak Ki values. Results The IDIF underestimated the AIF at early times and overestimated it at later times. Thus, based on the AUC and Ki comparison, 30–60 min was the most accurate time window for PBIFAUC; later time windows for scaling underestimated Ki (− 6 ± 8 to − 13 ± 9%). Correlations of AUC between AIF and IDIF, PBIFAUC(30–60), and PBIFiDV were 0.91, 0.94, and 0.90, respectively. The bias of Ki was − 9 ± 10%, − 1 ± 8%, and 3 ± 9%, respectively. Conclusions Both PBIF scaling methods provided good mean performance with moderate variation. Improved performance can be obtained by refining IDIF methods and by evaluating PBIFs with test-retest data.

Prostate specific membrane antigen (PSMA) targeting Positron Emission Tomography (PET) imaging is becoming the reference standard for prostate cancer (PC) staging, especially in advanced disease. Yet, the implications of PSMA-PET derived whole-body tumor volume for overall survival are poorly elucidated to date. This might be due to the fact that (semi-) automated quantification of whole-body tumor volume as PSMA-PET biomarker is an unmet clinical challenge. Therefore, a novel semi-automated software is proposed and evaluated by the present study, which enables the semi-automated quantification of PSMA-PET biomarkers such as whole-body tumor volume. Methods: The proposed quantification is implemented as a research prototype (MI Whole Body Analysis Suite, v1.0, Siemens Medical Solutions USA, Inc., Knoxville, TN). PSMA accumulating foci were automatically segmented by a percental threshold (50% of local SUVmax). Neural networks were trained to segment organs in PET-CT acquisitions (training CTs: 8,632, validation CTs: 53). Thereby, PSMA foci within organs of physiologic PSMA uptake were semi-automatically excluded from the analysis. Pretherapeutic PSMA-PET-CTs of 40 consecutive patients treated with 177Lu-PSMA-617 therapy were evaluated in this analysis. The volumetric whole-body tumor volume (PSMATV50), SUVmax, SUVmean and other whole-body imaging biomarkers were calculated for each patient. Semi-automatically derived results were compared with manual readings in a sub-cohort (by one nuclear medicine physician using syngo.MM Oncology software, Siemens Healthineers, Knoxville, TN). Additionally, an inter-observer evaluation of the semi-automated approach was performed in a sub-cohort (by two nuclear medicine physicians). Results: Manually and semi automatically derived PSMA metrics were highly correlated (PSMATV50: R2=1.000; p

Introduction: Malnutrition is a pathological state of deficiency or excess of nutrients. Under-nutrition indicates a state wherein the weight for age, height for age, and weight for height indices are below-2 Z-score of the National Centre for Health Statistics (NCHS) norms. In children aged

Venous malformations (VM) of the pelvis are rare and difficult to treat due to extensive lesional feeding vessels. Little exists in the published literature for management of such conditions in children, but lessons learned from congenital causes can help. Herein we discuss the preoperative evaluation and management of an 11-year-old child with a congenital pelvic and perirectal VM that presented as hematochezia. MRI showed extensive malformation with involvement of the calf, leg, perirectal region, and pelvis. The patient had continued hematochezia despite several sclerotherapy and embolization attempts. She eventually underwent transanal and open Swenson type pull-through and resection of colonic and rectal VM with derotation of the transverse colon, and has been doing extremely well since her operation.

Eukaryotic transfer RNAs (tRNAs) are exported from the nucleus, their site of synthesis, to the cytoplasm, their site of function for protein synthesis. The evolutionarily conserved β-importin family member Los1 (Exportin-t) has been the only exporter known to execute nuclear export of newly transcribed intron-containing pre-tRNAs. Interestingly, LOS1 is unessential in all tested organisms. As tRNA nuclear export is essential, we previously interrogated the budding yeast proteome to identify candidates that function in tRNA nuclear export. Here, we provide molecular, genetic, cytological, and biochemical evidence that the Mex67–Mtr2 (TAP–p15) heterodimer, best characterized for its essential role in mRNA nuclear export, cofunctions with Los1 in tRNA nuclear export. Inactivation of Mex67 or Mtr2 leads to rapid accumulation of end-matured unspliced tRNAs in the nucleus. Remarkably, merely fivefold overexpression of Mex67–Mtr2 can substitute for Los1 in los1Δ cells. Moreover, in vivo coimmunoprecipitation assays with tagged Mex67 document that the Mex67 binds tRNAs. Our data also show that tRNA exporters surprisingly exhibit differential tRNA substrate preferences. The existence of multiple tRNA exporters, each with different tRNA preferences, may indicate that the proteome can be regulated by tRNA nuclear export. Thus, our data show that Mex67–Mtr2 functions in primary nuclear export for a subset of yeast tRNAs.

Introduction: Worldwide Haemophilia A (HA) is the most common coagulation factor deficiency. It affects all populations and its prevalence varies among different countries, but roughly prevalence is estimated to be of 3-20 cases per 100,000 population. The classification of HA provides a guidance to possible types of bleeding and the rate of occurrence of hemorrhagic episodes. The hemophiliac patients with severe form, can be presented with spontaneous bleeding and hemorrhage after minor trauma about 1-6 times in a month, including hemarthrosis and intramuscular hemorrhage. In moderate form, the patients usually experience bleeding after mild to moderate injuries, while HA patients with mild form may not be diagnosed for years and bleed after surgery or major trauma. About 70% of children who have a positive history of HA in the family are diagnosed at birth or after the first bleeding episode. Material and Methods: A complete medical history of the patient was taken with reference to the name, age, sex, address, occupation, history of present illness, duration illness, education of patient and parent. Previous history of treatment, past and personal history and socio-economic status were noted. Parents were explained the purpose of the study and the procedures involved.The Hemophilia Joint Health Score (HJHS) was used to assess joint health in children included in the study. Results: Age of diagnosis >=12 months was observed in 28 (54.90%) of cases while in 23 (45.10%) cases age of diagnosis was < 12 months. Out of 51 cases Severe disease was observed in 45(88.23%) of cases, moderate in 4(7.84%) and mild form was observed in 2(3.95%).Out of total 51 cases 42 (82.4%) were from the nuclear family while 9(17.6%) were from the joint family. Mean HJHS was higher in patients of nuclear family as compared to joint family. Conclusion: HJHS shows construct validity and is more sensitive for mild arthropathy and can be used for studies in children with hemophilia. No correlation of different demographic parameters in children suffering from hemophilia A was seen. Keywords: HJHS, HA, Hemophilia A and B, CDC, HTCs, UDC,FA.