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The current study aimed to characterize patients from a rheumatology referral center in north India, who satisfied the definition of interstitial pneumonia with autoimmune features (IPAF) as given by the American Thoracic Society and European Respiratory Society (ATS/ERS) consensus committee in 2015. Thirty-five adult patients aged 18 years and above, fulfilling the 2015 ATS/ERS criteria for IPAF were included in the study. The clinical and immunological profile, and radiologic findings on high-resolution computerized tomography thorax were noted. Antinuclear antibody (ANA) by indirect immunofluorescence at 1:320 titer and myositis-specific antibody (MSA) assays were performed. Non-parametric tests were used to compare variables between groups. The study cohort included predominantly female patients with a mean age of 50.6 ± 13 years and mean duration of disease of 38.8 ± 28.4 months. Majority of patients (49%) fulfilled the morphologic and serologic domains as per the IPAF consensus criteria and 31% patients had features in all three domains. Non-specific interstitial pneumonia was the most common pattern observed in 77% patients. Raynaud's phenomenon and inflammatory arthritis were the predominant autoimmune features. Pulmonary arterial hypertension was documented in 60% of patients on echocardiography. Positive ANA at 1:320 dilution was present in all 26 patients tested, whereas extractable nuclear antigen and MSA assays detected autoantibodies in 49% and 51% of patients respectively. IPAF predominantly affected females in the age group of 50 years and above, with varied autoimmune manifestations and autoantibody profile. [ABSTRACT FROM AUTHOR]

Aim: To evaluate the outcome of innervation preserving sphincteroplasty along with anatomical bladder neck reconstruction (IPS-ABNR) compared to classic Young-Dees-Leadbetter (YDL) bladder neck reconstruction in exstrophy with insufficient bladder capacity requiring detubularized-ileocystoplasty. Materials and Methods: Sixteen male patients of exstrophy bladder who required ileocystoplasty from 2004 to 2010 were randomized into group A (n = 7) and group B (n = 9). After detubularized-ileocystoplasty with Mitrofanoff stoma and ureteric reimplantation in all, group A received YDL bladder neck repair while group B received IPS-ABNR repair through a midline scrotoperineal approach. Outcome measurement included operative and postoperative problems, continence, and upper tract status. Results: In group A, two had incompetent bladder neck with gross incontinence, while four had a dry interval of more than 3 h without the ability of voiding per urethra. In group B, seven patients had dry interval of more than 3 h with an ability of urethral voiding and midstream holding in five. Conclusions: Exstrophy patients requiring augmentation cystoplasty and repaired with IPS-ABNR can achieve dynamic bladder outlet resistance with adequate leak point pressure and ability to void voluntarily with midstream holding capability. The children had the satisfaction of voiding per urethra with ability to stop in midstream similar to that in normal children. [ABSTRACT FROM AUTHOR]

Aims: To analyze whether outcome of neonates having esophageal atresia with or without tracheoesophageal fistula (EA±TEF) associated with anorectal malformation (ARM) can be improved by doing surgery in 2 stages. Materials and Methods: A prospective study of neonates having both EA±TEF and ARM from 2004 to 2011. The patients with favorable parameters were operated in a single stage, whereas others underwent first-stage decompression surgery for ARM. Thereafter, once septicemia was under control and ventilator care available, second-stage surgery for EA±TEF was performed. Results: Total 70 neonates (single stage = 20, 2 stages = 30, expired after colostomy = 9, only EA±TEF repair needed = 11) were enrolled. The admission rate for this association was 1 per 290. Forty-one percent (24/70) neonates had VACTERL association and 8.6% (6/70) neonates had multiple gastrointestinal atresias. Sepsis screen was positive in 71.4% (50/70). The survival was 45% (9/20) in neonates operated in a single stage and 53.3% (16/30) when operated in 2 stages (P = 0.04). Data analysis of 50 patients revealed that the survived neonates had significantly better birth weight, better gestational age, negative sepsis screen, no cardiac diseases, no pneumonia, and 2-stage surgery (P value 0.002, 0.003, 0.02, 0.02, 0.04, and 0.04, respectively). The day of presentation and abdominal distension had no significant effect (P value 0.06 and 0.06, respectively). This was further supported by stepwise logistic regression analysis. Conclusions: In a limited resources scenario, the survival rate of babies with this association can be improved by treating ARM first and then for EA±TEF in second stage, once mechanical ventilator care became available and sepsis was under control. [ABSTRACT FROM AUTHOR]

Background: Developing countries at tertiary referral centre. The aim of this study was to share our experience of paediatric achalasia in Indian scenario. Materials and Methods: This was a retrospective analysis of children <16 years, operated for achalasia at our centre, from December 1998 to December 2011. Results: Total 40 patients (mean age 39 ± 4.29 months), including 1 patient of megaesophagus were operated over 13 years of period; 17 patients (associated congenital H-type tracheoesophageal fistula in one patient, non- responders/ lost follow-up for minimum of 3 years in 16 patients) were excluded from the study. The response rate of parents in followup was 60.0%. Mean symptoms duration was 27.88 ± 2 months. Most common symptoms were regurgitation and failure to thrive (78.2%). Mean symptom scoring in follow-up after 3 year was 1 ± 0.7 compared to 5 ± 0.51 at the time of admission (P < 0.012). One infant expired (mediastenitis), one developed adhesive intestinal obstruction and one needed posterior re-myotomy (for megaesophagus). There were no treatment failures in mean follow-up of 40.2 ± 5.07 months. Conclusions: Cardiomyotomy with partial fundoplication is the best modality of treatment for paediatric achalasia cardia, even from parents' perspective. [ABSTRACT FROM AUTHOR]

Aim: To assess the usefulness of ultrasonography in the differentiation of causes of chronic cervical lymphadenopathy in children. Materials and Methods: Children with palpable cervical lymph nodes were included. An ultrasonographic examination was performed to delineate multiple lymph nodes, irregular margins, tendency towards fusion, internal echos, the presence of strong echoes and echogenic thin layer. Results: The total number of patients was 120. Echogenic thin layer and strong internal echoes were specific for tuberculosis. Long axis to short axis (L/S) ratio was more than 2 in most of the tubercular nodes (85.71%). Hilus was present in 50 (73.53%) tubercular lymphadenitis, 12 (40%) lymphoma and 10 (62.5%) cases with metastatic lymph nodes. Hypoechoic center was present in 60 (88.24%) tubercular lymphadenitis cases followed by 62.5% metastatic and 60% malignant lymphoma cases. Conclusions: Ultrasonography is a non-invasive tool for lymph nodal evaluation in children. It may be used to differentiate cervical lymphadenopathy with different etiologies in children. When correlated clinically, it may avoid biopsy in a patient. [ABSTRACT FROM AUTHOR]

Introduction: Congenital pouch colon (CPC) is a pouch-like dilatation of shortened colon associated with anorectal malformation (ARM). The disease is prevalent in northern India. Postoperatively, the continence results are not as good as in other ARMs and there is higher incidence of incontinence and perineal soiling in these patients. The present study aimed to evaluate the pelvic floor and sphincter muscle characteristics in patients of CPC with the help of 64-slice computerized tomography with three-dimensional (3D) volumetric reconstructions of images, thus, to know the overall quality of these muscles in the patients.Materials and Methods: The study was conducted in patients admitted over a period of July 2007 to November 2008 in our department. Totally, eight patients of CPC were subjected to 64-slice CT with three-dimensional reconstructions of images and different parameters such as quality of pelvic floor muscles, configuration of vertical and parasagittal fibres, shape and thickness of sphincter muscle complex, attenuation values of sphincters were studied.Results: The 3D reconstructed images of pelvis in patients of CPC showed a well-developed pelvic floor and sphincter muscle complex. The length of the parasagittal fibres, transverse width of the vertical fibres and CT attenuation values of these structures with overall muscle quality were found to be good in these patients.Conclusion: In cases of CPC, the pelvic floor muscles including striated muscle complex (vertical and parasagittal fibres) are well developed. Higher rates of incontinence and soiling in CPC are not because of poorly developed pelvic floor and sphincter muscles. Three-dimensional CT can also provide important anatomical information that can help the operating surgeon while performing surgery. [ABSTRACT FROM AUTHOR]

Purpose: Diaphragmatic injuries have been reported to be a predictor of serious associated injuries in trauma and a marker of severity. Because of its rarity in children, the diagnosis is often delayed for months and years, due to overshadowing injuries. Perhaps due to the elasticity of their tissues, traumatic diaphragmatic rupture is uncommon in children. The problem remains a challenging clinical entity and the description of such type of injuries in children remains scarce in the literature. Most of the cases are described along with associated injuries; presence of isolated diaphragmatic injuries in children is unusual. The present study highlights the presentation, diagnosis and management of all of the cases admitted with traumatic diaphragmatic injuries in a single pediatric surgical center.Methods: We retrospectively studied eight children admitted to our center with a diagnosis of diaphragmatic injury following trauma during a period of 5 years (2003-2008). Relevant information regarding the mode and pattern of injuries were noted in all cases. Type of injury and surgical intervention and outcome of patients were evaluated.Results: Mean age of presentation was 6.8 years (range 2-12 years). Seven patients were males, while one patient was female. Seven patients had a history of blunt trauma abdomen some time back. Only one patient had acute presentation with respiratory distress following road traffic accident, rest of the patients had no associated grievous injury at the time of presentation. One patient presented with features of acute obstruction. All patients could be diagnosed preoperatively and surgical intervention was performed in all cases. The patients recovered well and there was no mortality.Conclusion: Diaphragmatic injuries in children are rare. They are usually associated with other severe injuries; however, isolated diaphragmatic injuries occur more frequently in children than adults. A high index of clinical suspicion supported by prompt radiological tests is needed to diagnose these injuries in patients who otherwise have no associated grievous injuries. [ABSTRACT FROM AUTHOR]

The article analyzes the clinical features and mutations in the steroid 5α-reductase type 2 gene (SRD5A2) in Indian patients with 5α-reductase deficiency (5RD). The SRD5A2 gene was sequenced in two patients with elevated testosterone ratio and in another patient with classical clinical features and virilization at puberty. The study concluded that p.R246Q is a common SRD5A2 mutation in 5RD patients in India.

Objective: To study the clinical profile of the cases of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and various factors affecting the surgical and early postoperative management and their outcome. Materials and Methods: A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading. Results: EA with TEF was the commonest type in 117 cases (92%). Associated congenital anomalies were present in 52 (41%) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5%) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (P = < 0.001). Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%). Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases). Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C. Conclusion Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome. [ABSTRACT FROM AUTHOR]

Background: India introduced rotavirus vaccines (RVV, monovalent, Rotavac™ and pentavalent, Rotasiil™) in April 2016 with 6, 10 and 14 weeks schedule and expanded countrywide in phases. We describe the epidemiology of intussusception among children aged 2-23 months in India.Methods: The prospective surveillance at 19 nationally representative sentinel hospitals from four regions recruited children with intussusception from April 2016 to September 2017. Data on sociodemography, immunization, clinical, treatment and outcome were collected. Along with descriptive analysis, key parameters between four regions were compared using Chi-Square/Fisher's exact/Mann-Whitney U/Kruskal-Wallis tests. The pre- and post-RVV periods were compared to estimate the risk ratios.Results: Six hundred twenty-one children with intussusception from South (n = 262), East (n = 190), North (n = 136) and West (n = 33) regions were recruited. Majority (n = 465, 74.8%) were infants (40.0% aged 4-7 months) with median age 8 months (IQR 5, 13 months), predominantly males (n = 408, 65.7%) and half (n = 311, 50.0%) occurred during March-June months. A shorter interval between weaning and intussusception was observed for ragi based food (median 1 month, IQR 0-4.2 months) compared to rice (median 4 months, IQR 1-9 months) and wheat (median 3 months, IQR 1-7 months) based food (p < 0.01). Abdominal pain or excessive crying (82.8%), vomiting (72.6%), and bloody stool (58.1%) were the leading symptoms. Classical triad (abdominal pain, vomiting and bloody stool) was observed in 34.8% cases (24.4 to 45.8% across regions). 95.3% of the cases were diagnosed by ultrasound. 49.3% (10.5 to 82.4% across regions) cases were managed by reduction, 39.5% (11.5 to 71.1% across regions) cases underwent surgery and 11.1% spontaneously resolved. Eleven (1.8%) cases died. 89.1% cases met Brighton criteria level 1 and 7.6% met Level 2. RVV was received by 12 cases within 1-21 days prior to intussusception. No increase in case load (RR = 0.44; 95% CI 0.22-1.18) or case ratio (RR = 0.5; 95% CI 0.3-1.2) was observed after RVV introduction in select sites.Conclusions: Intussusception cases were observed across all sites, although there were variations in cases, presentation and mode of management. The high case load age coincided with age of the RVV third dose. The association with ragi based weaning food in intussusception needs further evaluation. [ABSTRACT FROM AUTHOR]

Aim: To assess impact of primary fulguration v/s diversion on renal outcome in patients with Posterior Urethral Valve (PUV). Material and Methods: Patients with PUV admitted in Department of Pediatric Surgery, KGMU, Lucknow between January 2000 - Jan 2007, were included. They were divided into three groups according to their renal profile. Group I (n=41) - normal serum creatinine at presentation; Group II (n=33) - elevated serum creatinine at presentation responding to catheter drainage; Group III (n=38) - elevated serum creatinine not responding to catheter drainage. The patients were further subdivided according to urological ultrasound (hydronephrosis, echogenic kidney) and/or MCU (VUR) findings. Renal outcome and improvement of radiological abnormalities were evaluated. Results: 112 patients of PUV were admitted in department from 1998-2006. Of the 41 patients in Group I 26 were treated by primary fulguration and 15 by vesicostomy followed by delayed fulguration and vesicostomy closure. All have normal renal profile after mean follow up of 40 months. The radiological abnormalities resolved in 55% of cases. In Group II 20 patients were treated by primary fulguration and 13 by vesicostomy followed by delayed fulguration and vesicostomy closure. After mean follow up of 31 months, 30 patients have normal renal profile although upper tract abnormalities resolved in only 38% of cases. In Group III patients (n=38), 21 were treated by diversion (vesicostomy=13, pyelostomy-3, ureterostomy=5), 9 by primary fulguration and 8 patients expired. After mean follow up of 36 months, only 15% patients had improvement in renal profile and upper tract abnormalities resolved only in 10% of patients. In all three groups, the improvements of renal and radiological abnormalities were not dependent upon treatment done. Conclusion- Primary fulguration is preferred option in all patients with PUV and proximal urinary diversion does not affect the renal outcome. The least improvement is expected in children who have an elevated serum creatinine that is not responsive to catheterizing. [ABSTRACT FROM AUTHOR]

Background and Objectives: Interstitial lung diseases (ILDs) are a varied group of disorders that have been clubbed together due to some common attributes. The data is abundant from the developed world. However, the developing countries have gradually amassed the knowledge of these disorders and epidemiological data is still missing. We aimed to profile the ILD patients at our center., Methods: All ILD patients above the age of 14 years were included in the study. A detailed history, examination, exercise capacity assessment, pulmonary function test and relevant serological investigations were done. Tissue was obtained wherever possible. Diagnosis was made after discussion from concerned specialists., Results: Among the total 884 subjects recruited, 54% were females with a mean age of 53 years. Most were residents of rural areas. Reticulations and traction bronchiectasis was seen in most subjects. Hypersensitivity pneumonitis was present in 35.9% and connective-tissue disease related ILD (CTD-ILD) in 30.9%., Conclusion: Most common ILDs at our center are hypersensitivity pneumonitis and CTD-ILD. Reticulations and traction bronchiectasis are the most common findings on HRCT thorax., Competing Interests: Conflicts of interest The authors have none to declare., (Copyright © 2021 Tuberculosis Association of India. Published by Elsevier B.V. All rights reserved.)

The study aims to estimate the prevalence of hydroxychloroquine (HCQ) retinopathy in a cohort of Indian patients and analyse the associated factors. Adult patients with rheumatological disorders aged ≥ 18 years using HCQ for more than 5 years and/or having received a cumulative dose > 400 g were included. Demographic and clinical data were collected and all underwent ophthalmological tests which included Humphrey automated visual fields (AVF) and spectral domain optical coherence tomography (SD-OCT). The various clinical characteristics of the patients were compared. The study included 110 patients with a mean age of 43.5 ± 10.1 years and predominantly females. Eleven patients (10%) were diagnosed with definite HCQ retinopathy. The mean daily dose of HCQ (mg/kg of real body weight) was significantly different in the groups with and without retinopathy (5.7 ± 0.9 vs 5.1 ± 0.8, p = 0.04). Patients with retinopathy had significantly more colour vision abnormalities (odds of 16.9; confidence interval 4.1-69.1, p = 0.0001) and higher prevalence of both parafoveal and perifoveal thinning (p < 0.0001). Age, gender, duration of HCQ use, cumulative HCQ dose and body mass index were not found to be associated with retinopathy. Four out of 11 patients had abnormalities only on 30-2 protocol for AVF testing, two had abnormalities only on 10-2 protocol, whereas five patients had abnormalities on both protocols. SD-OCT abnormalities were present in all patients with retinopathy. Hydroxychloroquine retinopathy was prevalent in the study cohort and significantly associated with a higher daily dose of HCQ (mg/kg real body weight).

Background: A three-dose, oral rotavirus vaccine (Rotavac) was introduced in the universal immunization program in India in 2016. A prelicensure trial involving 6799 infants was not large enough to detect a small increased risk of intussusception. Postmarketing surveillance data would be useful in assessing whether the risk of intussusception would be similar to the risk seen with different rotavirus vaccines used in other countries., Methods: We conducted a multicenter, hospital-based, active surveillance study at 27 hospitals in India. Infants meeting the Brighton level 1 criteria of radiologic or surgical confirmation of intussusception were enrolled, and rotavirus vaccination was ascertained by means of vaccination records. The relative incidence (incidence during the risk window vs. all other times) of intussusception among infants 28 to 365 days of age within risk windows of 1 to 7 days, 8 to 21 days, and 1 to 21 days after vaccination was evaluated by means of a self-controlled case-series analysis. For a subgroup of patients, a matched case-control analysis was performed, with matching for age, sex, and location., Results: From April 2016 through June 2019, a total of 970 infants with intussusception were enrolled, and 589 infants who were 28 to 365 days of age were included in the self-controlled case-series analysis. The relative incidence of intussusception after the first dose was 0.83 (95% confidence interval [CI], 0.00 to 3.00) in the 1-to-7-day risk window and 0.35 (95% CI, 0.00 to 1.09) in the 8-to-21-day risk window. Similar results were observed after the second dose (relative incidence, 0.86 [95% CI, 0.20 to 2.15] and 1.23 [95% CI, 0.60 to 2.10] in the respective risk windows) and after the third dose (relative incidence, 1.65 [95% CI, 0.82 to 2.64] and 1.08 [95% CI, 0.69 to 1.73], respectively). No increase in intussusception risk was found in the case-control analysis., Conclusions: The rotavirus vaccine produced in India that we evaluated was not associated with intussusception in Indian infants. (Funded by the Bill and Melinda Gates Foundation and others.)., (Copyright © 2020 Massachusetts Medical Society.)

Objective: Intussusception has been linked with rotavirus vaccine (RVV) as a rare adverse reaction. In view of limited background data on intussusception in India and in preparation for RVV introduction, a surveillance network was established to document the epidemiology of intussusception cases in Indian children., Methods: Intussusception in children 2-23 months were documented at 19 nationally representative sentinel hospitals through a retrospective surveillance for 69 months (July 2010 to March 2016). For each case clinical, hospital course, treatment and outcome data were collected., Results: Among the 1588 intussusception cases, 54.5% were from South India and 66.3% were boys. The median age was 8 months (IQR 6, 12) with 34.6% aged 2-6 months. Seasonal variation with higher cases were documented during March-June period. The most common symptoms and signs were vomiting (63.4%), bloody stool (49.1%), abdominal pain (46.9%) and excessive crying (42.8%). The classical triad (vomiting, abdominal pain, and blood in stools) was observed in 25.6% cases. 96.4% cases were diagnosed by ultrasound with ileocolic location as the commonest (85.3%). Management was done by reduction (50.8%) and surgery (41.1%) and only 1% of the patients' died. 91.1% cases met Brighton criteria level 1 and 3.3% Level 2. Between 2010 and 2015, the case load and case ratio increased across all regions., Conclusion: Intussusception cases have occurred in children across all parts of the country, with low case fatality in the settings studied. The progressive rise cases could indicate an increasing awareness and availability of diagnostic facilities., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Objective: To assess physicians' perception and their felt competence in dealing with patients with rheumatic complaints., Methods: We assessed the quantum of rheumatological disorders seen by physicians in India, their felt competency in dealing with such patients, and their perceived adequacy of undergraduate and postgraduate medical training in Rheumatology by means of an anonymized questionnaire conducted at the annual national conference of internal medicine specialists., Results: Our analysis of 333 respondents revealed that while they saw an average of 10 patients with rheumatic complaints every month, the felt competence in dealing with such cases was only a median of 6/10 (interquartile range 5-7). About 75% professed little or no exposure to Rheumatology as undergraduates, whereas only 20% perceived adequacy of training during internal medicine residency to treat such diseases confidently. 78.37% and 67.7% perceived an inadequacy of rheumatology training at undergraduate and postgraduate level respectively, and 83% felt the need for further training or sensitization in Rheumatology., Conclusion: There remains an unmet need to enhance existing undergraduate and postgraduate internal medicine curricula in India to impart greater skills in the diagnosis and management of rheumatic diseases. Initiatives and government funding to establish short-term training courses in Rheumatology for established internal medicine faculty, to enable them to provide basic Rheumatology services at their respective hospitals, are urgently needed., (© Journal of the Association of Physicians of India 2011.)

Betel quid (paan) chewing is common in India, especially in Uttar Pradesh. Betel quid has multifaceted relationship with health, including metabolic and psychosocial health. The current recommendations have been released keeping in view the public health and clinical importance of this addictive behavior. The objective of this document is to offer clinical guidance for screening, diagnosis and management of co-occurring betel quid chewing among persons with Diabetes Mellitus (DM). The document aims to provide education and guidance to clinicians engaged in care and management of persons with DM, and improve access to treatment for co-occurring betel quid chewing among persons with DM. The current recommendation grades are based on published evidence, and categorized as strong, intermediate, weak and no evidence. The strength of these recommendations is based on the level of evidence., (© Journal of the Association of Physicians of India 2011.)

Introduction: Ten to 15% of patients with sarcoidosis have associated arthritis. Chronic arthritis is fairly uncommon. There is a paucity of data on articular manifestations of the disease from India., Methods: Case records of adult patients with sarcoidosis presenting to 11 rheumatology centers from 2005 to 2017 were retrospectively reviewed. Joint involvement was assessed clinically, classified as acute or chronic depending on duration of symptoms less or greater than 6 months, respectively., Results: A total of 117 patients with sarcoid arthritis were reviewed. Forty-five patients were classified as having Lofgren's syndrome. The pattern of joint involvement revealed the ankle to be most commonly affected in both the groups. Shoulder, wrist, metacarpophalangeal, proximal interphalangeal joints of hands and knee joint involvement were significantly more common in chronic sarcoid arthritis. Peripheral lymphadenopathy and uveitis were significantly more frequent in chronic sarcoid arthritis. Forty out of 49 patients with acute arthritis followed up over a median of 1.8 years had achieved complete remission. Twelve out of 16 chronic sarcoid arthritis (median follow up 2.5 years) had achieved complete remission with 15, 12 and five patients on steroids, methotrexate and hydroxychloroquine, respectively. One patient with acute sarcoid arthritis with concomitant interstitial lung disease had died due to lung infection., Conclusion: Acute oligoarthritis was the commonest presentation with the ankle being the most commonly affected joint. Upper limb joint (predominantly distal) and knee involvement were more common as reported in our largest series worldwide of chronic sarcoid arthritis in adults. Hilar adenopathy and erythema nodosum were common extra-articular features in both acute and chronic sarcoid arthritis. A limitation of the study was the retrospective nature of the analysis., (© 2018 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Competing Interests: Disclosure: The authors have no potential conflicts of interest to disclose.

Tobacco use is one of the main preventable causes of mortality and morbidity worldwide. The global disease burden due to tobacco use is huge with projected mortality of eight million lives per year by 2030. Metabolic syndrome (MS) is defined as a constellation of cardiovascular and endocrine risk factors such as insulin resistance, obesity, raised blood pressure, and abnormal lipid profile. The relationship between tobacco use and MS has been well established. Also, the causal association between tobacco use and development of individual components of MS is well established. The Uttar Pradesh Association of Physicians of India (UP API) has drafted this position statement on managing tobacco use among persons with or at risk of developing Metabolic Syndrome (MS). This position statement presents evidence-based recommendations as described below. Scope and purpose The objective of this position statement is to offer clinical recommendations for screening, diagnosis and management of tobacco use among persons with or at risk of developing Metabolic Syndrome (MS). The purpose of this document is to aid in identification and treatment of maladaptive patterns of tobacco use i.e. tobacco use disorder (tobacco dependence, harmful use, abuse) in person with or at risk of developing MS. Intended Audience The position statement is targeted at the clinicians engaged in care and management of person with or at risk of developing Metabolic Syndrome (MS). This might also be of relevance to the policy makers considering the public health burden of both MS and tobacco use disorders., (© Journal of the Association of Physicians of India 2011.)

Competing Interests: The authors have no potential conflicts of interest to disclose.

Aim: To determine sexual dysfunctions and urinary symptoms in male ankylosing spondylitis (AS) patients and their association with various disease and patient factors., Methods: In this prospective case control study conducted at a tertiary care teaching institution, 100 males with AS were compared to 100 controls using International Index of Erectile Function-15 (IIEF), International Prostate Symptom Score (IPSS), Hospital Anxiety and Depression Scale (HADS) and a global question for overall relationship with their partners. Bath AS Functional Index (BASFI), visual analogue scale pain scores, patient global assessment scale and Bath AS Disease Activity Index were also assessed in the AS group. Chi-square test, unpaired t-test and univariate and multivariate binary logistic regression analyses were used to analyze the data., Results: Anxiety, depression, erectile dysfunction (ED), orgasmic dysfunction, intercourse dissatisfaction, overall sexual dissatisfaction, altered overall relationship with partner and lower urinary tract symptoms (LUTS) were significantly (P < 0.05) higher in the AS group as compared to controls. Sexual desire, severe LUTS and bothersome LUTS (quality of life score > 2) were not different (P = 0.76, 0.82 and 0.30 respectively) between the two groups. ED was associated with anxiety, depression, longer disease duration, higher BASFI and higher age in AS patients (P = 0.02, 0.001, 0.02, 0.003 and 0.001 respectively)., Conclusions: AS is associated with higher incidence of sexual dysfunction in male patients. ED is associated with anxiety, depression, longer duration of disease, higher BASFI score and higher age in AS patients., (© 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.)

Aim: Leprosy classically presents with cutaneous and neural involvement. Rheumatological manifestations are frequent, although often under-recognized. At times, these may present to a rheumatology clinic prior to the diagnosis of leprosy. Herein, we present our experience with patients referred with various rheumatological disorders who were subsequently diagnosed as having leprosy., Methods: This retrospective study (January 2001-September 2010) was carried out at the Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, in northern India. Patients who were confirmed as having leprosy were included. Details regarding demographic and clinical presentations were collected., Results: Forty-four cases (30 male, mean age 40 ± 13.6 years and mean disease duration 18.7 ± 24.3 months) were identified. Musculoskeletal manifestations included arthritis (n = 22), swollen hands and feet syndrome (SHFS) (n = 11), tenosynovitis (n = 9), painful swollen feet (n = 9), arthralgias (n = 7) and vasculitis (n = 1). Distribution of joints mimicked rheumatoid arthritis (n = 14) and spondyloarthropathy (n = 7). Arthritis and/or tenosynovitis were part of spontaneous onset lepra reaction in 28 cases. Other clinical manifestations were: paresthesias (n = 28), erythematous nodules (n = 25) and anesthetic patches (n = 7). Thirty-one patients had thickened nerves (ulnar n = 28, common peroneal n = 21). Eight patients did not have any cutaneous manifestations and had presented with SHFS and arthritis or tenosynovitis. These were labeled as pure neuritic leprosy. Most of the patients responded to multidrug anti-leprosy therapy and glucocorticoids., Conclusion: Rheumatological presentations of leprosy may mimic RA, spondyloarthropathy or vasculitis. Pure neuritic variety and spontaneous type 2 lepra reaction pose unique diagnostic challenges. Increased awareness may avoid delay in diagnosis., (© 2012 The Authors International Journal of Rheumatic Diseases © 2012 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.)

Aim: Congenital lumbar hernia is an uncommon anomaly with only few cases reported in the English literature. This study was done to study the incidence, associated conditions, management and complications of congenital lumbar hernia repair in paediatric patients., Methods: Retrospective study of all patients over a period of 20 years (January 1990 to January 2010) was analysed., Results: A total of 18 patients (two bilateral hernias) were encountered in this series. All were in the age group of 1 day to 6 years. All the patients were operated by open technique. Primary repair was done in 14 patients and prosthetic meshplasty in two. Two patients could not be operated. The average follow-up period was 3 years. There was no follow-up loss in our study. There was no evidence of recurrence in this series., Conclusion: Up until now, the total number of cases is 68. The large size of hernia defect (>10 cm) may determine the use of prosthetic mesh repair. Proper surgical treatment, either primary repair or hernioplasty, provides adequate management of this condition., (© 2012 The Authors. Journal of Paediatrics and Child Health © 2012 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Purpose: Vestibular fistula is the commonest anorectal malformation in the female child. This article reports the treatment and long-term follow-up of 1206 patients of vestibular fistula treated by anterior sagittal anorectoplasty (ASARP) in a single center for 38 years., Material and Methods: All patients of vestibular fistula admitted and operated on at the Department of Pediatric Surgery, King George Medical University (Lucknow, UP India), from 1970 were included in the study; the age ranged from 2 days to 40 years. The diagnosis was made by clinical examination. We differentiated between anovestibular fistula (AVF) and rectovestibular fistula (RVF) in that the latter is a longer narrow fistula closely applied to the posterior wall of the vagina. Preoperative investigations included hemogram and blood glucose. Echocardiography was done in those patients showing a physical sign of cardiac anomaly. All patients were operated on in the lithotomy position by ASARP; this was done without colostomy in 1169 patients. In 6 patients, preliminary colostomy was done because of excessive perineal excoriation, and 31 others had colostomy done elsewhere. The striated muscle complex was delineated by electrostimulation, and anoplasty was performed after anchoring the rectum within the muscle complex. Washing of the perineum after passage of stools with application of povidone-iodine ointment constituted the local care. Intravenous antibiotics were administered for 48 hours and oral antibiotics (including metronidazole) for 5 days. The patient was discharged home by the fifth day., Results: Follow-up ranges from 3 months to 19 years; uneventful postoperative recovery was seen in 1147 patients. They had normal growth and development, normal appearance of the perineum, and a normal quality of life. Complications were seen in 60 patients (5%) of which 42 had AVF and 18 had RVF. Eight patients had postoperative wound disruption that was minor in 4 and required colostomy in the other 4. Four patients had recurrence of vestibular fistula thus creating an iatrogenic perineal canal; this could be repaired by a second ASARP in 3 patients and required colostomy and PSARP in one child. Anal stenosis was seen in 11 patients; this was treated by dilatation alone in 6 and required posterior Y-V plasty in 5. The rate of complications in RVF was lower than AVF probably because of lesser number of patients; there was no difference in stooling pattern or continence between uncomplicated patient of RVF and AVF; however, fecal staining was seen in all patients undergoing revision surgery for complications., Conclusions: This experience with ASARP showed a good result in 95% patients in a single-stage procedure. The technical ease and minimal preoperative and postoperative measures make ASARP the procedure of choice for vestibular fistula in females at all ages. Until sufficient experience is gained, it may be safer to operate on patients with RVF under cover of a protective colostomy.

Aim: The purpose was to evaluate the diagnosis and efficacy of management of congenital diaphragmatic hernia (CDH) in a tertiary health center of a developing country., Methods: Forty-six children aged from 1 day to 7 years were studied. Parameters studied were age, sex, clinical features, and management., Results: Fifty-six percent of patients presented in the neonatal period; however, none of them presented on the first day of life. The majority (91.3%) of patients had left-sided CDH. Respiratory distress was the most common clinical feature observed (91.3%). Chest X-ray confirmed the diagnosis in 82.6% of patients, and contrast study was needed in the remaining 17.4%. The survival rate was 87%. It was better in patients presenting late than those presenting in the early neonatal period. Stabilization in the preoperative period improved survival. Not using a chest tube had no adverse effect on survival., Conclusion: The relatively increased survival rate of CDH in a tertiary health center of a developing country is attributed to delayed arrival to the center. Respiratory infections compound the survival. More studies are needed before it can be safely said that not using a chest tube has no adverse outcome. Late presentation has been associated with varied manifestations, hence proper clinical evaluation, a high index of suspicion and adequate management, which includes imaging and surgery after stabilization, gives excellent results.

Objective: Culture-specific tools to assess longterm psychosocial outcomes for patients with disorders of sexual differentiation are scant. We conducted a study to develop tools for evaluating gender role behavior and health related quality of life for Indian adolescent patients with intersex disorders. We also studied factors important to parents while deciding sex of rearing for their baby., Methods: A 29-item gender role behavior questionnaire and an 18-item health related quality-of-life questionnaire were administered to 82 healthy controls, 13 patients with intersex disorders and 18 patients with type 1 diabetes mellitus. Internal consistency was checked by Cronbach's alpha and test-retest reliability using intra-class correlation coefficient. Responses of 28 parents to a questionnaire on factors affecting the decision of sex of rearing were recorded on a 5-point Likert scale in order of importance., Results: Cronbach's alpha was 0.92 and 0.75, and intra-class correlation coefficient 0.76 and 0.75, for the gender role behavior and quality-of-life questionnaires respectively, indicating a high degree of internal consistency and stability. The mean composite scores for healthy girls on the gender role behavior questionnaire (82.5 +/- 8.7) differed significantly from that for healthy boys (53.2 +/- 7.1, p <0.001). Factors important to parents while making decisions for sex of rearing were appearance of the genitalia, medical advice, ability to bear children and economic independence., Conclusions: We have created valid tools to study gender role behavior and quality of life in adolescent patients with intersex disorders in India. We have also identified in a quantitative way the factors of greatest importance to parents while deciding sex of rearing. These results have direct utility in the management of patients with intersex disorders in India and other similar cultures.

Background & Objectives: Early onset pauciarticular disease with uveitis is distinctly uncommon in Indian children with juvenile rheumatoid arthritis (JRA). The occurrence of anti-histone antibodies (AHA) in serum is strongly associated with presence of uveitis. There is a paucity of information from India on the levels of AHA in patients of JRA. In this study, an attempt was made to evaluate the levels of IgG and IgM antibodies to histones in children with JRA in north India., Methods: Serum samples of 148 children with JRA (84 boys, 64 girls) were collected. Clinical details including onset, symptoms and course of the disease in each patient were recorded. Detailed eye examination including slit lamp examination was done in all patients at presentation and yearly thereafter to rule out uveitis. The presence of antihistone IgG and IgM antibodies was studied by ELISA. Antinuclear antibodies (ANA) were measured by indirect immunofluorescence using HEP-2 cells as substrate at a screening dilution of 1:40., Results: Of the 148 children, 54 had pauciarticular (12 early onset and 42 late onset), 64 polyarticular and 30 systemic onset disease respectively. ANA were present in two children. AHA were raised in 15 (10%) children, of whom 10 had IgM antibodies, 3 had IgG and 2 had both isotypes. None of the children with early onset pauciarticular disease had uveitis, ANA or AHA., Interpretation & Conclusion: The low occurrence of AHA and uveitis in our subset of patients with JRA is in contrast to that reported from Western countries. The low occurrence is unlikely due to technical reasons as the antigen that has been used consistently showed significant binding to serum from patients with systemic lupus erythematosus (SLE). This is in accordance with the rarity of early onset pauciarticular disease and chronic uveitis in these patients. More studies from other parts of the country are required to validate this observation.

Objective: To study the prevalence of secondary amyloidosis in Asian North Indian patients with rheumatoid arthritis (RA) and to determine its clinical significance., Methods: RA patients with disease duration > 5 years were included in this prospective study over a 2 year period. Abdominal subcutaneous fat pad aspiration (ASFA) was performed, and smears were stained with Congo red and observed for apple-green birefringence under polarized light microscopy. The amyloid deposits were graded from 1+ to 3+. Clinical, radiological, and laboratory variables of the patients were correlated with the presence or absence of amyloidosis., Results: Thirty out of 113 patients were positive for amyloid by ASFA (26.5%). Out of these, 8 patients had features suggestive of clinical amyloidosis in the form of proteinuria, organomegaly, or symptomatic gastrointestinal involvement. In another 22 patients amyloidosis was subclinical. The majority of patients with clinical amyloidosis had either 2+ or 3+ deposits., Conclusion: Abdominal fat amyloid deposits are not uncommon in adult Asian North Indian patients with RA. However, only one-fourth of patients had evidence of clinical amyloidosis. A longer followup and a larger multicentric collaborative study is needed to determine the significance of subclinical amyloid deposits.

A very rare case of Teratoma of the bladder in an eight year old girl is presented. The diagnosis was made on cyctoscopy and confirmed histopathologically. Complete excision was possible. No recurrence was seen at four years follow up. Only one such case has been reported in the literature and this is the first such case reported from India and Asian continent.

We reviewed our experience with 1369 inguinal herniotomies in 1340 children performed over the last one decade. Different grades of surgeons were assigned work according to the complexity of cases. Except for the minor scrotal hematoma, other complications were hardly seen. Recurrences were seen in only 2 cases. Careful training and supervision of junior staff in the technique of inguinal herniotomy has led to results that compare favorably to those of specialized units in developed countries.