Search

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a low to intermediate-grade, poorly differentiated myofibroblastic tumor and is characterized by its tendency to recur locally. It commonly occurs in the 1st year of life and is predominantly seen in the axial skeleton and rarely in the retroperitoneum. We report one such case of PMMTI, which is the second case reported in English literature.

Medical Thoracoscopy (MT) is commonly performed by respiratory physicians for diagnostic as well as therapeutic purposes. The aim of the study was to provide evidence-based information regarding all aspects of MT, both as a diagnostic tool and therapeutic aid for pulmonologists across India. The consensus-based guidelines were formulated based on a multistep process using a set of 31 questions. A systematic search of published randomized controlled clinical trials, open labelled studies, case reports and guidelines from electronic databases, like PubMed, EmBase and Cochrane, was performed. The modified grade system was used (1, 2, 3 or usual practice point) to classify the quality of available evidence. Then, a multitude of factors were taken into account, such as volume of evidence, applicability and practicality for implementation to the target population and then strength of recommendation was finalized. MT helps to improve diagnosis and patient management, with reduced risk of post procedure complications. Trainees should perform at least 20 medical thoracoscopy procedures. The diagnostic yield of both rigid and semirigid techniques is comparable. Sterile-graded talc is the ideal agent for chemical pleurodesis. The consensus statement will help pulmonologists to adopt best evidence-based practices during MT for diagnostic and therapeutic purposes.

Background: Kasai portoenterostomy (KPE) is the initial treatment for biliary atresia (BA). Even after initial jaundice clearance, a significant number of children presented with the reappearance of symptoms due to ongoing fibrosis involving porta and intrahepatic ducts. Mitomycin-C (MMC) is an antifibrotic agent, and the study hypothesized that local application of MMC at porta can decrease fibrosis, which can improve jaundice clearance and lead to better native liver survival (NLS). Materials and Methods: This prospective randomized control trial included children with BA, who were allocated to groups A or B. The patients in both groups underwent standard KPE; in addition, a 5 French infant feeding tube (IFT) was placed near the porta through the Roux limb in Group B children. During the postoperative period, MMC was locally instilled over the porta in Group B children through IFT. Postoperative jaundice clearance and NLS were assessed and compared. Results: A total of 27 children were enrolled in the study, 16 in Group A and 11 in Group B. Both groups were comparable preoperatively. Although the NLS was not statistically significant in Group B, the survival was quite higher, that was 91%, 81%, and 73% at 6 months, 1 year, and 2 years, respectively, compared to 63%, 50%, and 38% in Group A. Conclusion: Children in Group B clinically showed an early jaundice clearance and a better trend of serial bilirubin levels as well as longer NLS than Group A, but it was not statistically significant. The procedure was technically easy, and no complication was encountered related to surgical technique or MMC instillation.

Colonic atresia (CA) is an uncommon type of intestinal atresia commonly associated with other anomalies, while biliary atresia (BA) is also rare but usually an isolated anomaly. The pathogenesis for either of the anomalies is unclear. The co-occurrence of both pathologies has not been mentioned in the literature. We here discuss the management of CA with BA and the review of pertinent literature.

Aims: To assess the role of transcutaneous electrical nerve stimulation (TENS), alone or in combination with anticholinergic drugs in the management of neurogenic bladder (NB) in spina bifida (SB). Materials and Methods: All the consecutive patients, visiting outpatient clinic between July 2017 and December 2018, who were toilet trained and at least 1 year post-SB surgery with clinical and/or urodynamic evidence of NB, were included in the study. Out of 65 patients, 40 fulfilled the inclusion criteria and were randomised into: group A (ten patients, placebo TENS with anticholinergic agents), Group B (14 patients, TENS therapy with placebo medications) and Group C (16 patients, TENS therapy with anticholinergic medications). All the patients maintained a voiding diary and underwent assessment before and after the intervention. The study was approved by the Institutional Ethics Committee. Results: The presenting symptoms were urinary incontinence (100%), increased frequency (45%), straining during micturition (22.5%), urgency (22.5%), and hesitancy (30%). The demographic parameters were comparable in all the groups. After group specific intervention, the wet episodes/day significantly improved in Group C (P = 0.001). Similarly, the mean wet days/week also improved significantly in Group C (6.5–4.37 days/week, P = 0.01). Out of 40 patients, 29 had abnormal findings on ultrasonography before the start of the therapy. Following intervention, only two patients in Group C showed normalization of findings. On Urodynamic studies, detrusor pressure (Pdet max) decreased in all the groups; however, the patients in Group C, showed the maximum reduction (56.6 ± 11–30 ± 6.7 cm H2O). Similarly compliance (9.4 to 14.5 cm H2O, P = 0.02) and bladder capacity (68%–88% of EBC, P = 0.001) also improved significantly in Group C as compared to other 2 groups Overall, nine patients (Group A, B, and C = 1, 3, and five patients, respectively) showed detrusor instability, while post therapy, only one patient (Group B) had unstable bladder. Maximum decrease in postvoid residue (mean) was also observed in Group C (77–41 ml, P = 0.01). Conclusions: The application of TENS in NB secondary to SB is effective and its application led to improvement in symptoms, decrease in the wet episodes/day, maximum detrusor pressure, instability, bladder compliance, and capacity. TENS therapy in combination with anticholinergic agents had a better outcome as compared to monotherapy with either of the two modalities.

Malignant rhabdoid tumors (MRT) are uncommon, highly aggressive tumors arising usually from the central nervous system and kidneys. Nonrenal and noncentral nervous systems MRT are rare in neonates. To the best of our knowledge, only five cases of congenital MRT of neck have been described till date. We present a rare case of congenital MRT of the neck in a neonate along with review of literature.

Background: Cardiovascular disease is one of the major causes of mortality in chronic obstructive pulmonary disease (COPD) patients. Both microalbuminuria (MAB) and raised serum C-reactive protein (CRP) levels have strong association with cardiovascular events as they reflect generalized endothelial vascular dysfunction. The objectives of the study are (i) to assess the prevalence of MAB and serum CRP levels in stable COPD patients and (ii) to find out the relationship of MAB and serum CRP with clinical and physiological parameters in COPD patients. Methods: This comparative cross-sectional study was carried out on COPD patients attending OPD at Institute of Respiratory Diseases, Jaipur during the year from 2019 to 2020. Forty stable COPD patients and 40 healthy controls were enrolled in the study. Spot urinary albumin/creatinine ratio, serum CRP levels, smoking history, spirometry, blood gases, body mass index, and BMI, Obstruction (FEV1% predicted), Dyspnea (mMRC grading), Exercise Capacity (6 MWD) (BODE) index were assessed. Results: Out of 40 cases, 23(56%) had MAB and 38 (95%) had serum CRP levels >3 mg/L. There was a negative correlation between forced expiratory volume in one second (FEV1), partial pressure of oxygen in arterial blood (PaO2) levels and 6 MWD with both MAB levels and S.CRP levels respectively. There was a positive correlation between BODE Index and modified British Medical Research Council grading with both MAB levels and serum C reactive protein (S.CRP) levels respectively. There was a positive correlation between BODE index and modified British Medical Research Council grading with both MAB and S.CRP levels. Conclusion: COPD patients of varying severity should be screened regularly with MAB and serum CRP levels to determine the risk and progression of cardiovascular consequences so that adequate decision of interventional strategies can be taken out to prolong survival in COPD patients.

Background: Interstitial lung disease (ILD) is a complex and heterogeneous group of acute and chronic lung diseases of several known and unknown causes. While clinical practice guidelines (CPG) for idiopathic pulmonary fibrosis (IPF) have been recently updated, CPG for ILD other than IPF are needed. Methods: A working group of multidisciplinary clinicians familiar with clinical management of ILD (pulmonologists, radiologist, pathologist, and rheumatologist) and three epidemiologists selected by the leaderships of Indian Chest Society and National College of Chest Physicians, India, posed questions to address the clinically relevant situation. A systematic search was performed on PubMed, Embase, and Cochrane databases. A modified GRADE approach was used to grade the evidence. The working group discussed the evidence and reached a consensus of opinions for each question following face-to-face discussions. Results: Statements have been made for each specific question and the grade of evidence has been provided after performing a systematic review of literature. For most of the questions addressed, the available evidence was insufficient and of low to very low quality. The consensus of the opinions of the working group has been presented as statements for the questions and not as an evidence-based CPG for the management of ILD. Conclusion: This document provides the guidelines made by consensus of opinions among experts following discussion of systematic review of evidence pertaining to the specific questions for management of ILD other than IPF. It is hoped that this document will help the clinician understand the accumulated evidence and help better management of idiopathic and nonidiopathic interstitial pneumonias.

Background: High and intermediate types of anorectal malformations (ARMs) in male neonates may be managed either by primary neonatal reconstruction without colostomy cover or by traditional policy of staged reconstruction after neonatal colostomy. Posterior sagittal anorectoplasty (PSARP) is the current widely practiced reconstructive technique with varied results. Aim: To assess our functional results of PSARP without colostomy in male neonates with high and intermediate ARMs compared to 3-stage (neonatal colostomy – PSARP – colostomy closure) methodology in a high volume tertiary care institution of a developing country. Patients and Methods: The number of colostomies performed for male high/intermediate anomalies and the number of babies who completed 3-stage reconstruction during a 10-year period is analyzed. The outcome of primary neonatal PSARPs during the same period was analyzed. Eighty primary PSARPs were compared to 81 staged reconstructions for outcome analysis, using Kelly score. Results: A total of 453 colostomies were performed, but only 253 of them completed all stages of reconstruction (52%). Good continence was achieved in 45% of cases of primary PSARP versus 26% in 3-staged surgery. Conclusions: Primary PSARP in neonatal period without colostomy is a good option for high and intermediate ARMs in males if the treating surgeon is reasonably skilled in neonatal surgery and PSARP procedures.

Aim: To evaluate the health related quality of life (HRQOL) after endoscopic injection treatment for vesico ureteral reflux (VUR) in children. Materials and Methods : Fifty four children received treatment and were prospectively evaluated for their quality of life scores, according to resolution of reflux on cystograms and status of renal scars. Results: Of the 81 refluxing units, 72 (89%) had resolution of reflux whereas 9 (11%) did not resolve. The total average QOL was higher for the patients in the resolved group as compared to the non resolved group. Comparison of pre and post procedure renal DMSA scans in 44 patients showed status quo in 26, regression of scars in six, progression in two and formation of new scars in 6.The total increase in HRQOL was highest in regression group (67.91), and lowest in progression group (36.45). Conclusions: Successful endoscopic treatment of VUR is associated with improved quality of life, as indicated by higher HRQOL scores in the resolved group.

The usual accepted mode of treatment of neonates with necrotizing enterocolitis (NEC) and pneumoperitoneum is surgical. However, in a select group of patients conservative treatment is feasible. Pneumoperitoneum without peritonitis is a rare phenomenon which creates a management problem for the treating surgeon. We present two cases of pneumoperitoneum to discuss the non-surgical management of pneumoperitoneum and describe their outcomes.

Objective/background: Molecular epidemiology methods are very useful for differentiating between strains, assessing their diversity, and measuring the prevalence of the most circulating strain in an area. Various molecular typing methods using different molecular markers have been utilized worldwide, such as restriction fragment length polymorphism (RFLP), spoligotyping, Mycobacterial Interspersed Repetitive Unit – Variable Number of Tandem Repeat (MIRU-VNTR), and Double repetitive element-PCR (DRE-PCR) typing, for simultaneous detection and epidemiologic typing of Mycobacterium tuberculosis. The present study is conducted to assess the genetic diversity of M. tuberculosis by IS6110-RFLP and spoligotyping in patients attending a tertiary care hospital in eastern Uttar Pradesh, North India. Methods: A total of 83 representative isolates of M. tuberculosis were included in this study. These isolates were subjected to spoligotyping and IS6110-RFLP DNA fingerprinting techniques as described previously. Results: The spoligotype patterns were compared with SpolDB4.0; patterns of 64 out of 83 M. tuberculosis isolates were matched with the available data, while 19 isolates were found to be orphan, that is, absent in the SpolDB4.0 database. The majority of the M. tuberculosis strains (56.5%) belong to central Asian (32.5%), ill defined T (13.2%), and Beijing (10.8%) families. On IS6110-RFLP analysis, in 19.2% (16/83) of these isolates, IS6110 element was not found (0 copy number strains). Further, 15.6% (13/83) isolates were found to be low-copy-number strains having less than six copies of IS6110 element, and the remaining 65.0% (54/83) were multiple-copy-number strains with six or more copies of the element. On comparing the results of spoligotyping and IS-6110-RFLP, a total of 47 isolates were clustered by spoligotyping; out of these isolates, 40 were found to be unique by IS6110-RFLP. Conclusion: Spoligotype analysis resulted in the grouping of a much larger number of isolates within apparently identical clusters compared with IS6110-RFLP typing, while IS6110-RFLP was not found to effectively distinguish between zero- and low-copy-number isolates. Therefore, we concluded that, in India, the use of both the techniques simultaneously for DNA fingerprinting of M. tuberculosis could be a better approach.