Effect of early weight bearing program with conventional physiotherapy on functional outcomes in surgically treated proximal tibia fracture: a pilot randomized controlled trial.

INTRODUCTION: Patients are often instructed to avoid weight bearing on the proximal tibia for 6 - 12 weeks post-surgery to avoid fracture fixation failure. However, delayed weight bearing leads to delayed mobility, causing difficulties in daily activities; problems such as pain, limited knee ROM, reduced quadriceps muscle strength, and impaired functional outcomes are reported in long-term follow-up. This study primarily aimed to evaluate the feasibility and explore the effect size. The secondary aim was to determine the effectiveness of early weight bearing along with conventional physiotherapy on functional outcomes. MATERIALS AND METHODS: A single-blinded pilot randomized controlled trial with 30 participants with proximal tibia type I, II, and III fractures were included; they were randomized using computer-based software. Seven patients were lost to follow-up. Group I received early weight bearing along with conventional physiotherapy whereas Group II received restricted weight bearing along with conventional physiotherapy. Assessments were made on post-operative day 3 (POD-03), at discharge, at 6 weeks, and at 12 weeks. Radiographs were recorded immediately after the operation, at discharge, and at 12 weeks. RESULTS: A full-scale RCT is feasible with an effect size between 0.3 - 0.7. A statistically significant difference (p < 0.05) was found within both groups 12 weeks post-surgery in all outcome measures; the difference was more prominent in the intervention group. A statistically significant difference (p < 0.05) was found between both the groups post-12 weeks in all outcome measures except quadriceps isometric muscle strength. CONCLUSION: A full-scale RCT for early weight bearing along with conventional physiotherapy for patients operated for Type I, II, and III proximal tibia fracture is feasible. Early weight bearing along with conventional physiotherapy could have a positive effect on reducing patient's pain perception and increasing knee joint mobility, quadriceps isometric muscle strength, knee function, and quality of life without any adverse effects. TRIAL REGISTRATION: ClinicalTrialsRegistry.gov (CTRI/2022/10/046797).

Cost-effectiveness analysis of low-dose prophylaxis versus on-demand treatment for moderate-to-severe hemophilia A in India.

AIM: Hemophilia A (HA) is an inherited bleeding disorder caused by a deficiency of clotting factor VIII in the blood. In resource-limited settings like India, affordability is a significant challenge in managing patients with severe HA. This study aims to assess the cost-effectiveness of intermediate-dose prophylaxis versus on-demand factor therapy in adult and pediatric populations with moderate-to-severe congenital HA without inhibitors in India. METHOD: We conducted a prospective cost-effectiveness analysis from a societal perspective, categorizing patients into a base state and a joint disease state (patients with Hemophilia suffering extensive bleeds leading to chronic joint disease). Using targeted literature search and primary market research, we developed a Markov model measuring the total cost of Hemophilia treatment and health outcomes, including life-years (LYs), quality-adjusted life-years (QALYs), incremental cost-utility ratio (ICUR), and incremental cost-effectiveness ratio (ICER). The model extended over a lifetime horizon of 70 years with a one-year cycle length. Sensitivity analyses assessed study robustness. RESULTS: Low-dose prophylactic therapy was cost-effective for adults (>18 years) and pediatric populations (<18 years), yielding better health outcomes (adults: 0.15 LYs and 2.43 QALYs gained; pediatric: 0.40 LYs and 3.12 QALYs gained). Intermediate-dose prophylaxis showed positive net monetary benefits in terms of Quality-Adjusted Life Years (QALYs) for both adult and pediatric populations, with dominant ICER and ICUR values in both cases. CONCLUSION: Using intermediate-dose prophylactic factor VIII therapy is a cost-effective approach that improves clinical outcomes compared to on-demand therapy in the Indian adult and pediatric HA populations without inhibitors.

Effect of Pain Neuroscience Education with Conventional Physiotherapy via Telerehabilitation on Pain Catastrophizing and Function in Patients with Osteoarthritis Knee: A Randomized Controlled Trial.

Aim: By explaining to the patient the biological processes underneath their pain condition, pain neuroscience education (PNE) is a form of educational intervention that aims to relieve pain and impairment. Materials and Methods: Patients with knee osteoarthritis (OA) referred to outpatient physiotherapy clinic in India during August 2021 to June 2022 were asked to participate. Out of the eligible patients, 35 were randomly assigned to PNE group and 35 to the control group. Self-reports of Pain Catastrophizing Scale (PCS), Patient Specific Function Scale (PSFS), and Numerical Pain Rating Scale (NPRS) were recorded at baseline (T1) and at 2 weeks (T2). Results: After 2 weeks of follow-up, all the three outcome measures were found to be significant in the PNE group. The results of the unpaired t-test revealed statistically significant result posttest for PCS (mean difference 11.4) and NPRS (mean difference 1.20). There was no mean difference found in the patient function (PSFS) between groups. Conclusion: The results suggest that adding a program of PNE to conventional physiotherapy exercises led to a greater reduction in pain catastrophization, patient-specific function, and pain intensity rather than conventional physiotherapy alone in patients with knee OA at 2 weeks' follow-up.

Immunogenicity and safety of the first indigenously developed Indian tetravalent influenza vaccine (split virion) in healthy children (6 months to 17 years of age): a randomized, multicenter, phase III clinical trial.

This phase III clinical trial was conducted to evaluate the immunogenicity and safety of the Tetravalent Influenza Vaccine (Split virion) I.P. (TetIV), containing two strains each of influenza A and B, developed indigenously in the country for the first time by M/s Cadila Healthcare Limited, India for use in the pediatric population (6 months -17 years of age), and compare it to that of a licensed seasonal Trivalent Influenza Vaccine (TriIV) of Sanofi Pasteur India Private Limited, containing two influenza A and one influenza B strains. Three hundred six subjects of either sex, 6 months to 17 years of age, were randomized in a 1:1 ratio to receive either TetIV or TriIV. Immunogenicity assessments (antibodies against A/H1N1, A/H3N2, B/Phuket, and B/Brisbane) were performed using the hemagglutination inhibition assay at baseline and 28 days after the last vaccination. TetIV was found to fulfill the criteria set by the United States Food and Drug Administration on the requirements of clinical data for licensure of seasonal inactivated influenza vaccines for the pediatric population. The seroconversion rates with TetIV were 94.6% for A/H1N1, 93.9% for A/H3N2, 91.2% for B/Brisbane, and 87.2% for B/Phuket strains. TetIV showed non-inferiority and superiority in immune response, as compared to TriIV, against the shared strains and an additional B strain, respectively. Both the vaccines were tolerated well by all the study participants, and an addition of the fourth strain in TetIV did not compromise the safety as compared to that of TriIV. The most common adverse event reported in both groups was fever.

Comparison of Diagnostic Accuracy of Different Sites for Transcutaneous Bilirubin Measurement in Early Preterm Infants.

OBJECTIVE: To evaluate correlation of transcutaneous bilirubin (TcB) measured at different body sites with serum total bilirubin (TSB) in early preterm infants. METHODS: This hospital based prospective comparative study was carried out in the Department of Pediatrics, SMS Medical College, Jaipur between April 2015 to March 2016. Early preterm infants with gestational age ≤ 34 weeks in whom clinical jaundice was significant mandating TSB measurement were included in the study. Study subjects who met the inclusion criteria were chosen consecutively. Neonates in whom phototherapy was already initiated and those with poor perfusion (capillary refill time > 3 s) were excluded from the study. All the measurements were carried out within first postnatal week. Transcutaneous bilirubin was measured from three sites: forehead, sternum and interscapular region and within 15 min of TcB measurement, blood samples were taken and serum bilirubin level was calculated. RESULTS: Correlation coefficients of transcutaneous bilirubin measured from forehead, sternum and interscapular sites were 0.82, 0.84 and 0.86 respectively. TcB measured from all the three sites correlated significantly with serum bilirubin (p < 0.001), but the correlation was best at interscapular site. Sensitivity, specificity and false negative rates were calculated with respect to starting phototherapy. Interscapular site had the highest sensitivity and lowest false negative rate (87.6 and 12.4% respectively) as compared to forehead (79.2 and 20.8% respectively) and sternum (87.1 and 12.9% respectively). CONCLUSIONS: Transcutaneous bilirubin measurement is an acceptable method for identification of hyperbilirubinemia requiring treatment in early preterm newborns. The authors recommend interscapular region as a reliable site in infants of gestational age ≤34 wk for measuring transcutaneous bilirubin.

Consensus Statement of the Indian Academy of Pediatrics in Diagnosis and Management of Hemophilia.

JUSTIFICATION: Despite having standard principles of management of hemophilia, treatment differs in various countries depending on available resources. Guideline for management of hemophilia in Indian setting is essential. PROCESS: Indian Academy of Pediatrics conducted a consultative meeting on Hemophilia on 18th September, 2016 in New Delhi, which was attended by experts in the field working across India. Scientific literature was reviewed, and guidelines were drafted. All expert committee members reviewed the final manuscript. OBJECTIVE: To bring out consensus guidelines in diagnosis and management of Hemophilia in India. RECOMMENDATIONS: Specific factor assays confirm diagnosis and classify hemophilia according to residual factor activity (mild 5-40%, moderate 1-5%, severe <1%). Genetic testing helps in identifying carriers, and providing genetic counseling and prenatal diagnosis. Patients with hemophilia should be managed by multi-specialty team approach. Continuous primary prophylaxis (at least low-dose regimen of 10-20 IU/kg twice or thrice per week) is recommended in severe hemophilia with dose tailored as per response. Factor replacement remains the mainstay of treating acute bleeds (dose and duration depends on body weight, site and severity of bleed). Factor concentrates (plasma derived or recombinant), if available, are preferred over blood components. Other supportive measures (rest, ice, compression, and elevation) should be instantly initiated. Long-term complications include musculoskeletal problems, development of inhibitors and transfusion-transmitted infections, which need monitoring. Adequate vaccination of children with hemophilia (with precautions) is emphasized.

Efficacy and Safety of Direct Acting Antiviral Therapy for Chronic Hepatitis C in Thalassemic Children.

OBJECTIVES: There is limited data on the efficacy and safety of directly acting antiviral therapy (DAA) for chronic hepatitis C in pediatric population. The aim was to assess the efficacy and safety of DAA in chronic hepatitis C ß-thalassemic major pediatric patients. METHODS: Prospective study was conducted from September 2015 to January 2017. All ß-thalassemic major chronic hepatitis C pediatric patients with age between 5 and 14 years were included in this study. Data related to demography, laboratory parameters, hepatitis C viral load, genotype and outcome of antiviral therapy was analyzed. DAA was planned according to EASL guidelines 2015 for chronic hepatitis C therapy in adults. OBSERVATIONS: Fourteen ß-thalassemic major patients (median age was 9.5 y, 12 male) were studied. All patients were of genotype 3, received DAA (sofosbuvir 400 mg+daclatasvir 80 mg) for 12 weeks. The median viral load was 2.5×10 IU/mL. End of treatment response and sustained virological response at 12 weeks was achieved in all the patients. Serum alanine aminotransferase, aspartate aminotransferase, ferritin, and albumin significantly reduced after DAA. CONCLUSIONS: DAA in adult dosage are safe and effective for treatment of chronic hepatitis C (genotype 3) in pediatric ß-thalassemic major population.

Comparison of Endoscopic and Histological Findings between Typical and Atypical Celiac Disease in Children.

PURPOSE: Celiac disease is a common non-communicable disease with varied presentations. Purpose of this study was to find the duodeno-endoscopic features in celiac disease and to compare duodeno-endoscopic and histological findings between typical and atypical celiac disease in children. METHODS: Hospital based observational study was conducted at Sir Padampat Mother and Child Health Institute, Jaipur from June 2015 to May 2016. Patients were selected and divided in two groups- typical and atypical celiac disease based upon the presenting symptoms. Upper gastrointestinal endoscopy and duodenal biopsy was performed for serology positive patients. Results were analysed using appropriate statistical test of significance. RESULTS: Out of 101 enrolled patients, 47.5% were male. Age ranged from 1 to 18 years. Study showed that 54.5% were typical and 45.5% were atypical. Patients presenting with atypical symptoms were predominantly of older age group. On endoscopy, scalloping, mosaic pattern, reduced fold height and absent fold height; and in histology, advanced Marsh stage were significantly higher in the typical group. CONCLUSION: Awareness of atypical presentations as well as duodeno-endoscopic features may have considerable practical importance for the diagnosis of celiac disease in children. Scalloping, mosaic pattern, reduced fold height and nodularity are main endoscopic markers of celiac disease in children. Endoscopic markers of duodenal mucosa may be important in early diagnosis of celiac disease, in children subjected to endoscopy for atypical presentations or indication other than suspected celiac disease.

Joint Involvement in Children with Celiac Disease.

OBJECTIVE: To determine early joint involvement as detected by ultrasonography in children with newly diagnosed celiac disease, and in children with celiac disease on gluten-free diet for more than 6 months. METHODS: Cross-sectional comparative study evaluating joint abnormalities by ultrasonography. RESULTS: Ultrasonography showed abnormalities in 19 out of 60 (31.7%) children with newly diagnosed celiac disease as compared to 2 (3.3%) out of 60 in those on a gluten-free diet for more than 6 months. CONCLUSION: Subclinical synovitis as detected by ultrasound is a frequent finding in newly diagnosed children with celiac disease.

Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease) and Anaplastic Large Cell Lymphoma.

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare and benign cause of lymphadenopathy and was first described in 1969. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy mainly at cervical nodal sites, composed of a population of histiocytes. Since its description, over 400 cases have been described, but there are few reports in the literature on its association with lymphomas. This case report describes a case with co-existing SHML and anaplastic large cell lymphoma (ALCL). The diagnosis of SHML in our patient did not alter the clinical outcome and the patient responded well to treatment for ALCL. Clinicians should maintain a high index of suspicion in cases of infra-diaphragmatic SHML for the presence of occult lymphoma. LEARNING POINTS: A rare presentation of sinus histiocytosis with massive lymphadenopathy (SHML) with anaplastic large cell lymphoma in the same lymph node at the same time is described.SHML has no proven clinical significance.A diagnosis of SHML in the presence of infra-diaphragmatic lymphadenopathy should raise a high index of suspicion for lymphoma.

A Case of Congenital Erythropoietic Porphyria without Haemolysis.

Porphyrias are a group of disorders caused by enzymatic defects in the biosynthesis of haem. Congenital erythropoietic porphyria (CEP) or Günther's disease is an extremely rare autosomal recessive disorder. Clinical manifestations include onset in infancy of blistering of sun-exposed areas, atrophic scars, mutilated fingers, and bright red fluorescence of the urine and teeth without neurological involvement. The only available prophylactic treatment for CEP is total avoidance of sunlight. A high degree of suspicion is required for the diagnosis. Early diagnosis is very important to prevent subsequent damage. LEARNING POINTS: A rare case of congenital erythropoietic porphyria (CEP) without evidence of haemolysis is described.A high degree of suspicion is required for diagnosing CEP.Early diagnosis prevents further damage.

Assessment of Musculoskeletal Function and its Correlation with Radiological Joint Score in Children with Hemophilia A.

OBJECTIVE: To evaluate the functional independence of children with hemophilia A and its correlation to radiological joint score. METHODS: The present cross sectional study was conducted at SPMCHI, SMS Medical College, Jaipur, India. Children in the age group of 4-18 y affected with severe, moderate and mild hemophilia A and with a history of hemarthrosis who attended the OPD, emergency or got admitted in wards of SPMCHI, SMS Medical College were examined. Musculoskeletal function was measured in 98 patients using Functional Independence Score in Hemophilia (FISH) and index joints (joints most commonly affected with repeated bleeding) were assessed radiologically with plain X rays using Pettersson score. RESULTS: The mean FISH score was 28.07 ± 3.90 (range 17-32) with squatting, running and step climbing as most affected tasks. The mean Pettersson score was 3.8 ± 3.2. A significant correlation was found between mean Pettersson score and FISH (r = -0.875, P < 0.001) with knee and elbow having r = -0.810 and -0.861 respectively, but not in case of ankle with r = -0.420 (P 0.174). CONCLUSIONS: The FISH and radiological joint (Pettersson's) scores may be extremely useful in the clinical practice in the absence of magnetic resonance imaging (MRI), which is considered very sensitive to detect early joint damage, but at a cost that makes it relatively inaccessible. FISH seems to be a reliable tool for assessment of functional independence in patients with hemophilia A.

What a practitioner needs to know about celiac disease?

Celiac disease (CD) is an immune-mediated systemic disorder elicited by gluten and related prolamines in genetically susceptible individuals and is characterized by the presence of a variable combination of gluten-dependent clinical manifestations, CD-specific antibodies, HLA-DQ2 or HLA-DQ8 haplotypes and enteropathy. CD is triggered by wheat gluten and related prolamines in barley and rye. Worldwide, the disease affects approximately 1 % of the general population. Clinical features of CD vary considerably. Intestinal symptoms are more common in young children. In older children extra intestinal manifestations affecting almost all organs are seen. IgA tTG antibody, upper GI endoscopy with histological analysis of multiple biopsies of the duodenum and in selected cases HLA DQ2 and DQ8 positivity and endomysial antibodies (EMA) are needed for diagnosis. Currently, the only treatment for CD is a life-long gluten-free diet (GFD). Strict avoidance of wheat, rye, barley and their derivatives will result in intestinal healing and relief of symptoms for the majority of individuals with CD. The GFD is simple in principle, however, completely eliminating all foods and ingredients containing wheat, rye, barley, and most commercial oats can be very challenging. Newly diagnosed CD children should undergo testing and treatment for micronutrient deficiencies specially iron, folic acid, vitamin D, and vitamin B12. Long-term monitoring and follow up of patients with CD is necessary.