27 results on '"Tiewsoh K"'
Search Results
2. Pulmonary Thromboembolism: A Rare but Serious Complication of Nephrotic Syndrome.
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Sandal, S., Tiewsoh, K., Hansdak, N., and Parajuli, B.
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RESPIRATORY distress syndrome , *HEART ventricle diseases , *BLOOD vessels , *VASCULAR surgery , *COMPUTED tomography , *ECHOCARDIOGRAPHY , *EMBOLISMS , *RIGHT heart ventricle , *HEPARIN , *INTRAVENOUS therapy , *NEPHROTIC syndrome , *PULMONARY artery , *PULMONARY embolism , *DISEASE remission , *RIGHT heart atrium , *TACHYPNEA , *DISEASE complications , *DIAGNOSIS - Published
- 2018
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3. Factors determining the outcome of children hospitalized with severe pneumonia
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Broor Shobha, Pandey Ravindra M, Lodha Rakesh, Tiewsoh Karalanglin, Kalaivani M, and Kabra Sushil K
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Pediatrics ,RJ1-570 - Abstract
Abstract Background Pneumonia is one of the leading causes of morbidity and mortality in under fives. We carried out a comprehensive study to identify factors influencing both mortality and morbidity for children less than 5 years of age hospitalized with severe pneumonia. Methods 200 hospitalized children aged 2–60 months with World Health Organization (WHO) defined severe pneumonia were enrolled in the study. The children were managed using a standard protocol. They were closely followed up for need for change in antibiotics, prolonged hospital stay, need for mechanical ventilation and mortality. Data on the factors influencing the outcome were collected. Results Of 200 children enrolled in the study, 113 (56.5%) needed a change in antibiotics, 102 (51%) stayed for more than 5 days in the hospital, 41 (20.5%) needed mechanical ventilation and 21 (10.5%) died. On multivariate analysis, lack of exclusive breastfeeding [RR (95%CI) 2.63 (2.16–2.86)], overcrowding [RR (95%CI) 1.94 (1.35–2.38)] and an abnormal chest x-ray [RR (95%CI) 2.29 (1.22–3.44)] were associated with the need for change of antibiotics. Lack of exclusive breastfeeding [RR (95%CI) 2.56 (2.0–2.93)], overcrowding [RR (95%CI) 2.59 (1.78–3.23)] and an abnormal chest x-ray [RR (95%CI) 2.99 (1.65–4.38)] were identified as determinants for prolonged hospital stay. Head nodding [RR (95%CI) 8.34 (2.71–12.77)], altered sensorium [RR (95%CI) 5.44 (1.34–17.56)], abnormal leukocyte counts [RR (95%CI) 5.85(1.36–17.14)] and pallor [RR (95%C) 10.88 (2.95–20.40)] were associated with mortality. Head nodding (RR (95% CI) 4.73 (1.50–6.36)] and cyanosis (RR (95%CI) 5.06 (1.80–11.34)] were the determining factors for mechanical ventilation. In radiographically confirmed pneumonia, the determining factors for change of antibiotics were: lack of exclusive breast feeding [RR (95% CI) 2.05 (1.69–2.2)] and low birth weight [RR (95% CI) 1.59 (1.1–1.89)]. For prolonged hospital stay, the factors identified were mothers' education less than graduation [RR (95% CI) 1.5 (1.19–1.7)], lack of exclusive breast feeding [RR (95% CI) 1.77 (1.19–2.09)] and oxygen saturation of < 90% at time of presentation [RR (95% CI) 2.06 (1.42–2.42)]. Determinants for mechanical ventilation were mothers' education less than graduation [RR (95% CI) 3.6 (1.15–6.3)] and cyanosis at presentation [RR (95% CI) 10.9 (1.56–18.9)]. For mortality, the only determinant was pallor [RR (95% CI) 10.54 (1.8–21.79)]. Conclusion Children hospitalized with severe community acquired pneumonia [as defined by World Health Organization (WHO)] who had not received exclusive breast feeding, had stayed in an overcrowded homes and had an abnormal chest radiograph were more likely to fail to respond with primary antibiotic regimen and require change of antibiotics and prolonged hospital stay. In children with radiographically confirmed pneumonia, lack of breast feeding and low birth weight was associated with need for change in antibiotics.
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- 2009
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4. Sporadic Form of Glomerulocystic Kidney Disease in a Child: A Case Report.
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Kaur N, D P, Nada R, Bhatia A, Dawman L, and Tiewsoh K
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Glomerulocystic kidney disease (GCKD) is a rare form of cystic renal disease. We report a four-week-old baby girl born to non-consanguineous parents; their antenatal third-trimester ultrasound showed severe oligohydramnios that required amnioinfusion. Post-natal ultrasound examination showed few tiny cysts (2-3mm) involving the cortices in bilateral kidneys. Kidney biopsy showed dilatation of Bowman's space and cystically dilated glomeruli, suggestive of GCKD. Whole exome sequencing revealed no pathogenic or likely pathogenic variant., Competing Interests: There are no conflicts of interest., (© 2023 Indian Journal of Nephrology | Published by Scientific Scholar.)
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- 2024
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5. Renal Amyloidosis in a Child with Recessive Dystrophic Epidermolysis Bullosa Due to a Novel Variant in COL7A1 Gene.
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Daniel R, Dawman L, Nada R, Sekar A, Mahajan R, and Tiewsoh K
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Secondary amyloidosis may complicate inherited dermatoses, but recessive dystrophic epidermolysis bullosa (RDEB) complicated by renal amyloidosis is rare. We report a case of a 12-year-old male child with RDEB presenting with progressive generalized anasarca for 20 days. Kidney biopsy showed diffuse expansion of mesangial matrix by pale acellular Periodic Acid-Schiff (PAS)-negative amorphous material, which was congophilic on Congo red stain and gave apple green birefringence on polarization and extending along the glomerular basement membrane, suggestive of amyloidosis. Genetic analysis showed a compound heterozygous pathogenic variant in the COL7A1 gene with autosomal recessive inheritance., Competing Interests: There are no conflicts of interest., (© 2023 Indian Journal of Nephrology | Published by Scientific Scholar.)
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- 2024
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6. Copeptin as a potential biomarker of chronic kidney disease to predict the disease progression in children with chronic kidney disease.
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Dawman L, Rawat A, Meena J, and Tiewsoh K
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Background: Biomarkers to predict the onset and progression of chronic kidney disease (CKD) in children are lacking, and no such definite biomarkers have been implicated in the diagnosis of CKD. We conducted this study to evaluate copeptin as a CKD marker and predict the disease progression by estimating the copeptin levels at baseline and 12 months follow-up in children with CKD stage 2 and above., Materials and Methods: This prospective single-centre cohort study was conducted in children under 14 years with CKD stages 2-4. Blood and urine samples were collected at enrolment and 1-year follow-up for routine investigations and serum copeptin, cystatin C and urinary neutrophil gelatinase-associated lipocalcin (uNGAL) estimation., Results: A total of 110 children (60 cases and 50 controls) were enrolled in the study. The mean estimated glomerular filtration rate (eGFR) of cases was 58.3 ± 18.7 ml/min/1.73 m
2 . Among the cases, there was a significant rise in the serum copeptin levels from baseline 483.08 ± 319.2 pg/ml to follow-up at 1 year, that is, 1046.82 ± 823.53 pg/ml ( P < 0.0001). A significant difference was noted in the baseline values of serum cystatin C, that is, 1512.98 ± 643.77 ng/ml and 719.68 ± 106.96 ng/ml ( P < 0.0001), and uNGAL, that is, 13.53 ± 11.72 and 1.76 ± 2.37 ng/ml ( P < 0.0001) between the cases and controls. There was no significant correlation (correlation coefficient = 0.10) between change in eGFR and copeptin levels during 12 months of follow-up., Conclusion: No significant correlation was found between the change in eGFR and copeptin levels during 12 months of follow-up. This can be due to the slow deterioration of renal functions, as most of the cases had underlying congenital anomalies of the kidney and urinary tract (CAKUT), which is known to have a slow progression of CKD and a small sample size., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Journal of Family Medicine and Primary Care.)- Published
- 2024
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7. Diptheroids can cause nosocomial UTI and be multidrug resistant: A case report of Corynebacterium striatum, first from India.
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Kumar MB, Pahil S, Yadav S, Tiewsoh K, Singh T, Mohan B, and Taneja N
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- Child, Humans, Anti-Bacterial Agents therapeutic use, Corynebacterium, Cross Infection microbiology, Corynebacterium Infections diagnosis, Corynebacterium Infections drug therapy, Corynebacterium Infections epidemiology, Urinary Tract Infections diagnosis, Urinary Tract Infections drug therapy, Urinary Tract Infections microbiology
- Abstract
Gram positive bacilli in the urine are usually dismissed as contaminants in urine specimens as these are commensal flora of skin and mucous membranes. Corynebacterium species were misidentified in the past due to complex biochemicals but the advent of modern diagnostics has made their identification quicker and accurate. Corynebacterium species have recently emerged as pathogens of nosocomial outbreak potential. C. striatum has been identified as opportunistic nosocomial pathogen causing various infections. We report first case of C. striatum as nosocomial urinary tract infection (UTI) pathogen in a child with bilateral renal disease. C. striatum causing UTI is very rarely reported., 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 © 2024 Indian Association of Medical Microbiologists. Published by Elsevier B.V. All rights reserved.)
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- 2024
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8. Personal Experience and Individual Measures to Manage Physician Burn-Out.
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Tiewsoh K
- Abstract
Competing Interests: There are no conflicts of interest.
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- 2022
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9. Hypertensive urgency in a child with focal epilepsy: Is it sodium valproate induced?
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Vijayan A, Dawman L, Das G, Tiewsoh K, and Sharawat IK
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- Anticonvulsants adverse effects, Child, Humans, Epilepsies, Partial drug therapy, Valproic Acid adverse effects
- Abstract
Competing Interests: None
- Published
- 2022
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10. Peripheral Neuropathy in Children With Chronic Kidney Disease: Are We Looking Enough?
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Sonbhadra A, Reddy BVC, Saini AG, Tiewsoh K, Paria P, Kesavan S, Suthar R, Dawman L, and Attri S
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Background: Peripheral neuropathy in chronic kidney disease (CKD) is the most common neurological complication. We aimed to look at the prevalence and patterns of neuropathy in children with CKD., Methods: This cross-sectional study was conducted over 1 year in children with CKD, stage III and above. Nerve conduction studies (NCS) were performed as per standard protocols using surface electrodes on the muscles and by supramaximal stimulation of the corresponding nerves. Presence of electrophysiological abnormalities in the absence of clinical symptoms or signs was considered as subclinical neuropathy., Results: Nearly 45 children were evaluated. The majority were males ( n = 39, 86.7%). The mean age was 7.9 ± 3 years (range 2-14). The mean estimated glomerular filtration rate (GFR) at enrolment was 23.3 ± 14.6 mL/min/1.73 m
2 (range 5-67). The majority of children were in stage III ( n = 19, 42%), followed by stages V ( n = 15, 33%) and IV ( n = 11, 25%). There was no evidence of clinical neuropathy; 13 children (29%) showed subclinical neuropathy. All the nerves had an axonal pattern of involvement. Motor polyneuropathy was most common type of peripheral neuropathy. The commonest nerves involved were tibial and common peroneal nerves. There were no biochemical or clinical predictors of neuropathy in our cohort., Conclusion: The prevalence of subclinical neuropathy is high in children with CKD, stage III and above. Axonal motor polyneuropathy is the predominant pattern. Electrophysiological assessment of nerve function should be routinely done in children with advanced stages of CKD to prevent chronic complications., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Annals of Indian Academy of Neurology.)- Published
- 2022
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11. Anti-Compliment Factor H Antibody Associated Hemolytic Uremic Syndrome in Children with Abbreviated Plasma Exchanges: A 12-Month Follow-up Study.
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Tiewsoh K, Govindarajan S, Dawman L, Rawat A, Ramachandran R, and Hans R
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- Child, Child, Preschool, Follow-Up Studies, Humans, Plasma Exchange, Retrospective Studies, Complement Factor H, Hemolytic-Uremic Syndrome diagnosis, Hemolytic-Uremic Syndrome therapy
- Abstract
Introduction: Anti-Compliment Factor H antibody hemolytic uremic syndrome (AFH-HUS) is a common cause of paediatric atypical HUS in India. We wanted to study the outcome of patients receiving less than recommended plasma exchange (PLEX) but adequate immunosuppression, with respect to hypertension, preservation of renal function and proteinuria., Methods: A retrospective study was performed in 15 children admitted from 2016 to 2018 with AFH-HUS. Follow up details including physical examination, hematological parameters, renal function test and urine examination performed at 3, 6, and 12 months were noted. Risk stratification and staging for chronic kidney disease (CKD) were done according to the Kidney Disease Improving Global Outcomes (KDOQI) guidelines. Standard statistical tests which were appropriate were used., Results: Mean age of our study cohort was 7.8 ± 1.9 years. 14 children had hypertension. Mean nadir hemoglobin was 5.8 ± 1.0 g/dL and platelet = 58 ± 37.7 × 109 cells/L. Median anti factor H (AFH) level was 316 AU/mL (150 to 452). Hemodialysis was required in 7 children. Fourteen children received PLEX with a mean of 11 ± 6 cycles. Thirteen children received 6 cycles of intravenous cyclophosphamide. After six months, therapy was switched to mycophenolate mofetil in 4 children, steroids alone in 2 children and 9 children with azathioprine. On follow-up, risk of CKD reduced from 80% at 3 months to 26% at 12 months (P = .01). Only 40% patients had CKD stage 2 at the end of 12 months (mean eGFR = 95.0 ± 19.4 mL/min/1.73m2)., Conclusion: The adequate number of PLEX needed in AFH-HUS needs further studies. Till such reports come, PLEX in recommended strategies or lesser, if not available, with immunosuppression in AFH-HUS can decrease progression to CKD. DOI: 10.52547/ijkd.6507.
- Published
- 2021
12. Chronic peritoneal dialysis in children with chronic kidney disease: An experience from a North Indian teaching institute.
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Tiewsoh K, Soni A, Dawman L, Peters NJ, and Malik MA
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Introduction: Chronic peritoneal dialysis (CPD) is an important modality of renal replacement therapy (RRT) in children of all ages with end-stage renal disease (ESRD). We retrospectively assessed the clinical profile of children with chronic kidney disease (CKD) initiated on CPD at a tertiary care centre in Northern India., Materials and Methods: Retrospective data of 13 children with CKD and initiated on CPD between 2016 and 2019 were retrieved and analysed. The demographic and clinical profile, aetiology of CKD, method of catheter insertion, mode of dialysis, complications, and catheter survival rate were analysed., Results: The median age at the onset of the symptoms was 81 months interquartile range (IQR 11-90) and the median age at the diagnosis was 81 months (IQR 36-103). The median age at the initiation of CPD was 92.97 months (IQR 74.43-108.79). The median serum creatinine at the initiation of CPD was 6.3 mg/dL (IQR 4.25-8.4). During a total study period of 84 CPD months, we observed 16 catheter-related complications and a complication rate of 1 per 5.25 CPD months. The overall peritonitis rate was 1 episode per 13.66 patient-months (0.87 episodes per patient-year). The catheter displacement/migration was seen in 23% of the cases. The median duration of follow-up was 175 days (IQR 85-249) with the longest follow-up duration of 502 days., Conclusion: CPD is the modality of choice for smaller children with ESRD as venous access is difficult to achieve in smaller children. Complications especially related to infections are a major concern in addition to poor growth associated with ESRD., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Journal of Family Medicine and Primary Care.)
- Published
- 2021
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13. Pericardial effusion in anti-complement factor H antibody-associated atypical hemolytic uremic syndrome: two case reports.
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Govindarajan S, Ramachandran R, Rawat A, Naganur SH, Nada R, Dawman L, Kumar A, and Tiewsoh K
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- Atypical Hemolytic Uremic Syndrome immunology, Child, Humans, Male, Antibodies, Anti-Idiotypic blood, Atypical Hemolytic Uremic Syndrome diagnosis, Complement Factor H immunology, Pericardial Effusion complications
- Abstract
Hemolytic uremic syndrome (HUS), a cause of pediatric acute kidney injury (AKI), has a spectrum of extra-renal manifestations. While neurological and gastrointestinal system involvement is common, cardiac involvement is rare. This is more so with pericardial involvement, though it has been reported in a handful of HUS cases associated with shiga toxin-producing Escherichia coli (STEC HUS). However, this complication has scarcely been reported in atypical HUS (aHUS) where there is alternate complement abnormality or DKGE (diacylglycerol kinase epsilon) mutation. We describe two children diagnosed with anti-complement factor H (CFH) antibody-associated aHUS who had pericardial involvement. Two boys, one 10-year-old and another 8-year-old, presented with pallor, oliguria and hypertension. They both had microangiopathic haemolytic anemia, thrombocytopenia and AKI suggestive of HUS. Complement workup revealed elevated anti-CFH antibody titres. With a diagnosis of anti-CFH antibody aHUS, they were started on plasmapheresis, pulse methylprednisolone and cyclophosphamide. The first case developed cardiac tamponade during the second week of hospital stay for which he needed pigtail drainage and further immunosuppression with rituximab. He gradually improved and pigtail was removed. The second case presented with pericardial effusion which subsequently resolved during the course of treatment. Thus, our patients developed pericardial effusion, with one of them progressing to life-threatening cardiac tamponade. Therefore, it is prudent that we are aware of this complication while treating children with aHUS.
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- 2021
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14. Phenotype and Genotype Profile of Children with Primary Distal Renal Tubular Acidosis: A 10-Year Experience from a North Indian Teaching Institute.
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Dawman L, Tiewsoh K, Barman P, Pratyusha K, Chaakchhuak L, and Sharawat IK
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Primary distal renal tubular acidosis (dRTA) or Type 1 RTA in children is caused by a genetic defect (involved genes ATP6V0A4 , ATP6V1B1 , SLC4A1 , FOXI1 , or WDR72 ), which causes tubular transport defects characterized by an inability to appropriately acidify urine with resultant persistent hyperchloremic metabolic acidosis. Retrospective analysis of 28 children (14 males) under the age of 14 years with dRTA seen from 2010 to 2019 was reviewed, and detailed clinic records were analyzed. The clinical features, investigations, and response to treatment were recorded. The median age of the children at presentation was 30 months (range: 9.25-72 months), and the median age at onset of symptoms was 2 months. All the children had growth failure, polyuria, and polydipsia at presentation. Mean serum potassium, pH, bicarbonate, and anion gap at presentation was 2.3 ± 0.5 mmol/L, 7.22 ± 0.09, 13.28 ± 4.37 mmol/L, and 9.3 ± 2.18, respectively. Mean serum potassium, pH, bicarbonate at follow-up was 3.88 ± 0.6 mmol/L, 7.35 ± 0.06, and 20.13 ± 4.17 mmol/L, respectively. The median z-score for the weight for age and height for age at initial presentation was -4.77 (-7.68 to -3.74) and -4.21 (-5.42 to -2.37) and at follow-up was -3.35 (-5.29 to -1.55) and -3.84 (-5.36 to -1.63), respectively. Twenty-two (78.6%) children had medullary nephrocalcinosis. Four children had sensorineural hearing loss. Seven children had genetic testing done, and six had pathogenic or likely pathogenic variants in ATP6V1B1 and ATP6V0A4 gene. Children with dRTA have a guarded prognosis and ATP6V1B1 and ATP6V0A4 mutations are the most common implicated genetic defect in Indian children with distal RTA., Competing Interests: Conflict of Interest None declared., (Thieme. All rights reserved.)
- Published
- 2021
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15. Bladder Mass Masquerading as Eosinophilic Cystitis in a Child: When to Think Beyond Malignancy?
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Dawman L, Peters NJ, Tiewsoh K, Bal A, Sodhi K, and Samujh R
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Eosinophilic cystitis is a rare inflammatory disease in the pediatric population with varied presentations. Diagnosis requires a high index of suspicion and cystoscopy with biopsy of the bladder mass. There are no standard treatment guidelines, however, these patients usually respond with medical management, but recurrence is a possibility. We present a case of eosinophilic cystitis in a 6-year-old boy who presented with lower urinary tract symptoms, gross hematuria, and bladder mass., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Journal of Indian Association of Pediatric Surgeons.)
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- 2021
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16. Alarming rates of psychological problems among caregivers of pediatric kidney patients admitted during the COVID-19 pandemic lockdown.
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Sharma R, Kumar K, Pilania R, Dawman L, Kaur N, Sharma R, and Tiewsoh K
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Introduction: Caregivers of children with comorbidities suffer from various psychological problems. We envisage more such complications during this COVID-19 pandemic., Methodology: A cross-sectional study to assess psychological issues in caregivers of children with kidney diseases, admitted during lockdown period in India was done. Psychological tools including Peritraumatic Distress Inventory (PDI), Insomnia Severity Index, Depression Anxiety Stress Scale (DASS II), Positive and Negative Affect Schedule (PANAS) and a new "COVID Stress Survey Questionnaire" were used. Standard statistical analysis using SPSS Statistic 23 (IBM SPSS Statistics, New York, United States) was done., Results: Forty-seven caregivers (33 mothers; 14 fathers) were included. Of these, 33 (70.2%) experienced psychological distress. On PANAS, 45 (95.7%) scored below cut off on a positive affect and 42 (89.4%) scored above cut off on a negative effect. The DASS II score revealed that 38 (80.9%) reported mild stress, 23 (48.9%) severe anxiety, and 37 (78.7%) had moderate depression. Upper middle socioeconomic status caregivers reported more insomnia. Further, parents of children with acute kidney injury (AKI) or prolonged hospital stay scored higher on subjective distress and aversive feelings., Conclusion: We observed an alarming level of distress, insomnia, and anxiety among caregivers, more so in upper middle socioeconomic status, children with AKI and prolonged hospital stay. We suggest due counseling should be done., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Industrial Psychiatry Journal.)
- Published
- 2021
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17. Leclercia adecarboxylata Causing Spontaneous Bacterial Peritonitis in a Child with Nephrotic Syndrome: A Case Report and Review of Literature.
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Hassan I, Gupta P, Ray P, and Tiewsoh K
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Infection is an important complication of childhood nephrotic syndrome (NS) and spontaneous bacterial peritonitis (SBP) is a frequently encountered one. We present a 7-year-old boy with NS who had decreased urine output, generalized body swelling, and abdominal pain. Urine analysis showed proteinuria of 50 mg/m
2 /d. Ascitic tap showed total leukocyte count of 100 cells/mm3 , sugar of 67 mg/dL, and protein of 1.1 g/dL. Gram stain revealed gram-negative bacilli with pus cells and culture grown Leclercia adecarboxylata (LAD). LAD was identified using matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS) with an identification score of 2.0. The organism showed good susceptibility to common antibiotics. The boy had no direct contact with livestock and the source of infection remains speculative. Devitalized skin because of massive edema seems to be the most plausible site of entry for the organism. Our patient was started on ceftriaxone and improved. LAD is a rare opportunistic pathogen, which belongs to Enterobacteriaceae and usually causes soft tissue infections. As far as we know, this is the first case where it has caused peritonitis in a child with NS. We also reviewed other pediatric cases., Competing Interests: Ethical ClearanceConflict of Interest An ethical clearance from Departmental Board was taken (DRB-121–19). None., (The Indian Association of Laboratory Physicians. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.).)- Published
- 2020
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18. Autosomal Recessive Congenital Ichthyosis and Steroid-Resistant Nephrotic Syndrome due to Homozygous Mutation in the ALOX12B gene: A Novel Association with Review of Literature.
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Dawman L, Kaur A, Nada R, Chakraborty S, Handa S, Sharawat IK, and Tiewsoh K
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Nephrotic syndrome (NS) associated with autosomal recessive congenital ichthyosis (ARCI) is a rare association. In this article, we described a 4-year-old boy with steroid-resistant NS (SRNS) who had a history of ichthyotic skin lesions since birth. Renal biopsy revealed focal segmental glomerulosclerosis (tip variant). The skin biopsy was consistent with the findings of ichthyosis. Next-generation sequencing revealed a homozygous pathogenic variant (c.1625_1626del) in the exon 12 of the ALOX12B gene, confirming the diagnosis of ARCI2. The ALOX12B gene belongs to the lipoxygenase family and has a pivotal role in the formation of lipid layers in the epidermis. Leukotrienes have a counter-regulatory effect within the inflamed glomeruli, which influences the vascular tone and glomerular basement membrane permeability, that can be implicated in the pathogenesis of the NS. This child is currently in remission, on tacrolimus and low-dose prednisolone, with emollients and is on regular follow-up. SRNS associated with congenital ichthyosis secondary to a mutation in the ALOX12B gene has never been reported so far. The knowledge regarding this novel association will help the treating physicians in diagnosing this condition early, which will enable proper genetic counseling and prognostication of the disease to the family., Competing Interests: Conflict of Interest None declared., (Thieme. All rights reserved.)
- Published
- 2020
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19. Chylothorax in a Child with Nephrotic Syndrome.
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Rathore V, Bhattacharya D, Pandey J, Bhatia A, Dawman L, and Tiewsoh K
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Chylothorax is an uncommon presentation of venous thrombosis in nephrotic syndrome. We present a case of an 8-year-old boy with nephrotic syndrome who presented with prolonged respiratory difficulty and dry cough. A detailed evaluation revealed left chylothorax secondary to thrombosis of the left brachiocephalic vein. He improved with conservative management including anticoagulation therapy, intercostal chest tube drainage, and dietary modification. This case highlights the need to consider venous thrombosis as a cause of chylothorax in patients with nephrotic syndrome to institute appropriate treatment., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Indian Journal of Nephrology.)
- Published
- 2020
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20. Chylous ascites during peritoneal dialysisin a toddler: a rare complication.
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Bhattacharya D, Indla RT, Tiewsoh K, and Rathore V
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- Acute Kidney Injury therapy, Child, Preschool, Chylous Ascites etiology, Diagnosis, Differential, Humans, Male, Chylous Ascites diagnosis, Peritoneal Dialysis adverse effects
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Chylous ascites is a rare complication of peritoneal dialysis (PD) and is often mistaken for peritonitis. It usually resolves following conservative management and does not pose any risk to the dialysis procedure. We report the case of a 2-year-old boy, who developed chylous ascites at 36 hours of PD and spontaneously resolved within the next 48 hours., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)
- Published
- 2019
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21. Infantile form of Niemann-Pick disease type C with demyelination: An uncommon feature.
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Tiewsoh K, Gupta K, and Bhatia A
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- Brain diagnostic imaging, Demyelinating Diseases complications, Humans, Infant, Male, Niemann-Pick Disease, Type C complications, Brain pathology, Demyelinating Diseases diagnosis, Demyelinating Diseases pathology, Niemann-Pick Disease, Type C diagnosis, Niemann-Pick Disease, Type C pathology
- Abstract
Demyelination, neurofibrillary tangles, and axonal spheroids are neuropathological features rarely encountered in infantile form of Niemann-Pick disease type C compared to swollen neurons and neuronal loss which are more commonly seen. We describe clinico-pathological findings in an autopsy case of an infant who died of suspected inborn error of metabolism. At autopsy, storage cells of Niemann-Pick type were observed in plenty in spleen and lymph nodes, and sparsely in liver and brain. Preterminally, the child also developed fungal meningitis with minimal boderzone encephalitis. The neuropathological findings are unique and have been illustrated in detail.Congenital anomaly of the urogenital system was an incidental associated finding., Competing Interests: There are no conflicts of interest
- Published
- 2019
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22. Complement factor H gene polymorphisms and vivax malaria associated thrombotic microangiopathy.
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Agrawal P, Kumar A, Parwaiz A, Rawat A, Tiewsoh K, and Nada R
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- Child, Female, Genetic Predisposition to Disease, Humans, Kidney Diseases etiology, Kidney Diseases pathology, Polymorphism, Single Nucleotide, Complement Factor H genetics, Kidney Diseases diagnosis, Malaria, Vivax complications, Thrombotic Microangiopathies genetics, Thrombotic Microangiopathies virology
- Abstract
Acute kidney injury (AKI) occurs in about 1% of cases of malaria; however, in these cases, the mortality rate can be as high as 45%. Thrombotic microangiopathy (TMA) as a cause of AKI in malaria is rare with only a handful cases documented in literature so far. Alternate complement pathway (ACP) dysregulation as a major mechanism of injury in the development of thrombotic microangiopathies is well known. It is proposed that patients with preexisting defects in ACP are usually clinically silent, until stress condition such as infections help manifest them. Herein, we describe the presence of two complement factor H (CFH) variants in an 8-year-old female with vivax malaria associated TMA. The complement workup confirmed dysregulated ACP and revealed two single-nucleotide polymorphisms in CFH gene, i.e. exon-7 rs1061147 (p.Ala243Ala) and exon-9 rs1061170 (p.His402Tyr) which predisposed this patient to develop TMA precipitated by vivax malaria.
- Published
- 2019
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23. Snake bite-induced renal medullary angitiis in a child: A case report.
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Dawman L, Sekar A, Varma TH, Nada R, and Tiewsoh K
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- Child, Female, Humans, Acute Kidney Injury diagnosis, Acute Kidney Injury etiology, Acute Kidney Injury pathology, Kidney Diseases diagnosis, Kidney Diseases etiology, Kidney Diseases pathology, Kidney Medulla blood supply, Kidney Medulla pathology, Snake Bites complications, Vasculitis diagnosis, Vasculitis etiology, Vasculitis pathology
- Abstract
Snake bite envenomation is common in tropical countries during the summer. Snake bite-induced acute kidney injury (AKI) has varied histopathological manifestations such as acute cortical necrosis, acute tubular necrosis (ATN), and acute interstitial nephritis. However, snake bite-induced renal medullary angiitis has rarely been reported. We describe a nine-year-old child with AKI following viperine snake bite and renal biopsy revealed pigment cast nephropathy, ATN and medullary angiitis.
- Published
- 2019
24. Usefulness of mycophenolate mofetil in Indian patients with C3 glomerulopathy.
- Author
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Bharati J, Tiewsoh K, Kumar A, Nada R, Rathi M, Gupta KL, Kohli HS, Jha V, and Ramachandran R
- Abstract
Background: C3 glomerulopathy (C3G) is a heterogeneous disease caused by alternative complement pathway abnormalities without any standardized treatment. An immunosuppressive agent, mycophenolate mofetil (MMF), has been recently shown to be useful in treating C3G, mainly in studies from the west. We report the clinical outcome of 17 Indian C3G patients treated with MMF with or without steroids., Methods: The clinical and histology details of the C3G patients treated with MMF for at least 6 months with a follow-up of at least 12 months were retrieved from the medical records of our center., Results: The median serum creatinine and proteinuria at presentation were 0.8 mg/dL and 3.7 g/day, respectively, with the majority (88.2%) presenting as nephrotic syndrome. The mean dose of MMF was 1.65 (±0.56) g/day, and the median duration of MMF therapy was 18 months. Two-thirds (64%) of the patients responded to the treatment, with complete remission in 4 (23%) and partial remission in 7 (41%) (median time: 9 months). Three patients progressed to end-stage renal disease (ESRD) on follow-up. Of the three patients, one (33%) had an initial response in proteinuria to MMF but did not respond after a relapse and subsequently progressed to ESRD and two (67%) other patients were nonresponsive to MMF from the start of the therapy., Conclusion: Despite a small sample size and lack of a control arm, this study describes the effectiveness of MMF in treating C3G patients from Asia and forms a basis for future randomized trials.
- Published
- 2018
- Full Text
- View/download PDF
25. Furosemide-induced tubular dysfunction responding to prostaglandin synthesis inhibitor therapy in a child with nephrotic syndrome.
- Author
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Varma TH, Sharma A, Santhiya S, Dawman L, and Tiewsoh K
- Subjects
- Alkalosis chemically induced, Alkalosis etiology, Bartter Syndrome etiology, Child, Preschool, Cyclooxygenase Inhibitors therapeutic use, Diuretics adverse effects, Diuretics therapeutic use, Female, Furosemide therapeutic use, Humans, Hypokalemia complications, Hypotension etiology, Indomethacin administration & dosage, Indomethacin therapeutic use, Kidney drug effects, Kidney physiopathology, Kidney Tubules drug effects, Kidney Tubules physiopathology, Nephrotic Syndrome blood, Nephrotic Syndrome complications, Nephrotic Syndrome diagnosis, Polyuria chemically induced, Polyuria diagnosis, Polyuria etiology, Prostaglandin Antagonists therapeutic use, Treatment Outcome, Bartter Syndrome diagnosis, Furosemide adverse effects, Nephrotic Syndrome drug therapy
- Abstract
Furosemide is one of the most common drug used to treat anasarca in childhood nephrotic syndrome. It has minimal side effects on short-term usage, but prolonged use can result in polyuria, hypokalemia and metabolic alkalosis. This pseudo-bartter complication can be treated by discontinuation of the drug with adequate potassium replacement. We report a child who was given furosemide for 20 days elsewhere to treat the edema due to nephrotic syndrome and then presented to us with bartter-like syndrome. Furosemide was discontinued and potassium replacement was initiated. However, the child continued to have polyuria leading to repeated episodes of hypotensive shock. In view of severe symptoms, she was given a short course of oral indomethacin for 6 days, to which she responded. This case highlights the fact that indomethacin can provide symptomatic improvement in furosemide induced pseudo-bartter.
- Published
- 2018
- Full Text
- View/download PDF
26. HIV-infected children with hepatomegaly and ascites: is there something more than an infection?
- Author
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Jindal AK, Ahluwalia J, Sharma A, Dhawan S, Tiewsoh K, Suri D, Sinha A, Saxena A, and Singh S
- Subjects
- Blood Cells, Blood Chemical Analysis, Child, Humans, Male, Radiography, Abdominal, Thrombosis pathology, Tomography, X-Ray Computed, Ascites etiology, Ascites pathology, HIV Infections complications, Hepatomegaly etiology, Hepatomegaly pathology, Portal Vein pathology, Thrombosis diagnosis
- Published
- 2017
- Full Text
- View/download PDF
27. Melanonychia following cyclophosphamide therapy.
- Author
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Gupta A, Kumar A, Suri D, and Tiewsoh K
- Subjects
- Child, Humans, Hyperpigmentation diagnosis, Male, Nail Diseases diagnosis, Nephrotic Syndrome diagnosis, Cyclophosphamide adverse effects, Hyperpigmentation chemically induced, Immunosuppressive Agents adverse effects, Nail Diseases chemically induced, Nephrotic Syndrome drug therapy
- Published
- 2016
- Full Text
- View/download PDF
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