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  ~ Table of Contents - Current issue
September-October 2019
Volume 29 | Issue 5
Page Nos. 307-377

Online since Wednesday, September 11, 2019

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Composite pheochromocytoma: A rare form of tumor p. 307
GR Lakshminarayana
The pheochromocytomas are one of the rare and curable causes of secondary hypertension arising from adrenal medulla, commonly presenting withhypertension; either paroxysmal or persistent. Very rarely they may show cells belonging to more than one line of differentiation and are called as mixed or composite pheochromocytoma.
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Oxidative stress in peritoneal dialysis patients: Association with the dialysis adequacy and technique survival p. 309
Natalia Stepanova, Lesya Korol, Olena Burdeyna
Introduction: Increased oxidative stress is suggested as one of the possible mechanisms of structural and functional damage to the peritoneal membrane in peritoneal dialysis patients. But there are few available data on the association of oxidative stress with peritoneal dialysis adequacy and technique survival. The present study was undertaken to investigate the association of oxidative stress biomarkers with the peritoneal dialysis adequacy and technique survival. Methods: This prospective single-center observational study was conducted between January 2010 and May 2015. Adequacy of dialysis, malondialdehyde levels in the serum and erythrocytes (as an indicator of lipid peroxidation), the concentration of ceruloplasmin, transferrin, and sulfhydryl groups in the blood, and total peroxidase activity in erythrocyte (as indicators of antioxidant system) were determined in 44 stable ambulatory non-diabetic peritoneal dialysis patients. Results: The follow-up period was 3 years. We identified a negative correlation between the serum level of malondialdehyde in the patients and total weekly Kt/V. Peritoneal weekly CrCl was positively correlated with the levels of transferrin, total peroxidase activity, and SH– groups. Daily peritoneal ultrafiltration had a positive correlation with the total peroxidase activity and the serum transferrin levels. The results of the Kaplan–Meier analysis and the log-rank test also demonstrated a significant difference in the cumulative technique survival rate between the patients with ceruloplasmin level ≤0.19 g/l and ≥0.2 g/l. Conclusions: The results mentioned above could be considered as one of the ways to explain better technique survival in PD patients.
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Effect of isothermic dialysis on intradialytic hypertension Highly accessed article p. 317
I Veerappan, G Thiruvenkadam, G Abraham, BR Dasari, A Rajagopal
The primary outcome was incidence of intradialytic hypertension (IDH) during standard and cooler isothermic dialysate temperatures. Two pair of haemodialysis sessions were done at 37°C (SHD) and at isothermic temperature (IHD). All the four dialysis were done on the same time of the day to negate the changes due to circadian variation in body temperature. Axillary and tympanic temperatures were measured before start of the dialysis and dialysis temperature was adjusted as per axillary temperature. Sixty patients were enrolled and completed the study. The mean delivered dialysate temperature in the intervention group was 36.5 ± 0.2 achieving a 0.5 ± 0.2°C between-group separation. The incidence of IDH and intradialytic hypotension while on SHD and IHD were 79/120 (66%) vs 44/120 (37%), odds ratio (OR) 3.3, 95% confidence interval (CI) (1.96–5.65) and 45/120 (38%) vs 14/120 (12%), OR 4.5, 95% CI (2.3–8.7), respectively. The 4 h time averaged mean systolic blood pressure (SBP) at IHD and SHD were 154 ± 1.7 and 157.2 ± 1.1 mmHg, respectively, the mean difference in SBP being −3.4 mmHg to −3.1 mmHg, 95% CI, P < 0.001. The standard deviation, a measure of BP variability was lower at IHD than at SHD (P < 0.001). In a subgroup analysis during IHD there was a significant reduction of both SBP and diastolic BP during the entire duration of dialysis in 35 out of 60 patients (systolic 4 h mean 154.96 ± 2.22 vs 164.32 ± 1.99 mmHg), (diastolic 4 h mean 79.24 ± 0.82 vs 82.54 ± 0.68 mmHg) – (rANOVA for systolic and diastolic <0.001). This phenomenon of cooler dialysis causing reduction of BP was reproduced in the same group of patients when the IHD was repeated another time (systolic 4 h mean 157.95 ± 1.88 vs 160.65 ± 1.47), (diastolic 4 h mean 79.27 ± 0.74 vs 82.03 ± 1.07) rANOVA for systolic and diastolic <0.001. The incidence of IDH can be reduced significantly by reducing the dialysate temperature to patients' body temperature. Hypertension during dialysis is related to heat gain during dialysis.
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Thrombotic microangiopathy: An under-recognised cause of snake-bite-related acute kidney injury p. 324
Indu Ramachandra Rao, Attur Ravindra Prabhu, Shankar Prasad Nagaraju, Dharshan Rangaswamy
Introduction: Thrombotic microangiopathy (TMA) as a cause of snake-bite-induced acute kidney injury (AKI) is rarely reported. Very little is known about the clinical course, optimal management, and prognosis of this entity. We describe a series of snake-bite-induced TMA and compare their outcomes with those without TMA. Methods: This was a single-center retrospective study of patients with AKI following snake envenomation admitted between January 2012 and December 2017. Demographic profile, clinical parameters, and outcomes were studied. TMA was diagnosed based on presence of triad of microangiopathic hemolytic anemia, thrombocytopenia, and AKI, and groups with and without TMA were compared. Results: Of 103 patients with AKI following snake bite, 19 (18.5%) had clinical evidence of TMA. All patients with TMA had advanced azotemia (mean peak serum creatinine 9.5 ± 3.0 mg/dL), with 18 (95%) requiring renal replacement therapy (RRT). Thirteen (68%) had either complete or partial recovery of renal functions, two (10%) progressed to end-stage renal disease, and one died (three patients were lost to follow-up). Age ≥50 years, presence of oliguria/anuria, anti-snake venom dose ≥10 vials, and urea ≥80 mg/dL at presentation were independently associated with TMA (P < 0.05). RRT requirement (95% vs. 57%), mean number of RRT sessions (18 vs. 4.5 sessions), and hospital stay ≥7 days (84% vs. 58%) were higher in patients with TMA (P < 0.05), but patient outcomes did not differ. Conclusions: In conclusion, TMA was seen in 18.5% of patients with snake-bite-related AKI in our study and was associated with almost universal need for RRT, longer duration on RRT, and hospital stay compared with patients without TMA.
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Impact of Angiotensin-converting Enzyme and Matrix Metalloproteinase-3 Gene Polymorphisms on Risk for Developing Vascular Access Failure in Hemodialysis Patients — A Pilot Study p. 329
Aleksandar Jankovic, Jelena Tosic, Ivana Buzadzic, Petar Djuric, Ana Bulatovic, Dragana Marković, Jovan Popovic, Nada Dimkovic
For adequate hemodialysis, functional vascular access is obligatory. Neointimal hyperplasia (NIH) has a central role in stenosis and thrombosis development, which represent the most frequent causes of vascular access failure. Polymorphism of different genes that have a significant role in endothelial function may have an impact on NIH development. Therefore, the aim of our study is to determine the effect of angiotensin-converting enzyme (ACE) I/D and matrix metalloproteinase-3 (MMP3) 5A/6A polymorphism on risk for developing vascular access failure in hemodialysis patients. The study included 200 patients on regular hemodialysis at Nephrology Department, University Medical Center Zvezdara. Retrospective analysis included a collection of general and vascular access data from medical records. Genetic analysis was performed by using polymerase chain reaction-restriction fragment length polymorphism method (PCR-RFLP). Patients were divided into two groups: Group 1—patients who have never experienced vascular access failure and Group 2—patients who have at least one spontaneous vascular access failure. There was no difference in age, gender, hemodialysis vintage, main diagnosis, presence of hypertension, and diabetes mellitus between the two groups. There were no statistically significant differences in the frequencies of ACE and MMP3 genotypes between the two groups. Without statistical significance, it was found that homozygotes for I allele had two times higher risk for developing vascular access failure than homozygotes for D allele (OR 2.00; 95%CI: 0.727–5.503; P = 0.180). In addition, patients with 5A allele have 1.7 times higher risk for developing vascular access failure compared with patients without this allele (OR 1.745; 95% CI: 0.868–3.507; P = 0.118). Patients with vascular access failure do not have different genotype distribution regarding ACE gene and MMP3 gene polymorphism as compared with patients without vascular access failure. Still, homozygotes for I allele and homozygotes for 5A allele have higher risk for developing vascular access failure compared with other patients.
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Clinical Characteristics, Patient and Technique Survival in Elderly Patients on Peritoneal Dialysis p. 334
Balasubramanian Karthikeyan, Raj K Sharma, Anupama Kaul, Amit Gupta, Narayan Prasad, Dharmendra S Bhadauria
The outcomes of the elderly population on peritoneal dialysis (PD) in developing countries are less known. In this study, we intended to study the clinical characteristics and patient and technique survival of elderly patients on PD. In this study, data of 148 elderly patients with end-stage renal disease who initiated PD between January 2001 and December 2015 were collected. Baseline clinical characteristics and events during the study period were recorded. Overall patient and technique survival rates of diabetic and non-diabetic elderly patients on PD were analyzed. Around 128 patients who were initiated PD during the study period were included for final analysis. The mean age of the study group was 70.3 ± 5.1 years, and 94 (80%) were males. Among these, 79 (65.8%) had diabetes. At the end of the study period, only 20 (16.6%) patients were remained on PD. Eighty-four (70%) patients died during PD and 15 (12.5%) patients were transferred to hemodialysis during the study period. The main reasons for death were cardiovascular (56.6%) and sepsis due to peritonitis (18.8%). The mean patient survival time was 38.2 ± 2.6 months. The patient survival rates were 91.2%, 45.3%, and 22.8% at 1, 3, and 5 years, respectively. Predictors of mortality were increased serum phosphorus, peritonitis episodes, urine output <400 mL, and ultrafiltration <1000 mL/day at beginning of PD. The mean technique survival time was 92.0 ± 5.1 months. Technique survival rates at 1, 3, and 5 years were 94.8%, 85.3%, and 71.7%, respectively. None of the factors was found to be predictive of technique survival. We found no significant difference between diabetic and non-diabetic patients in terms of technique and patient survival. Mortality was higher in elderly patients on PD. Factors affecting mortality in elderly patients on PD are low urine output, low ultrafiltration at beginning of PD, high serum phosphorus, and presence of peritonitis episodes. Patient and technique survival rates were comparable between diabetic and non-diabetic elderly patients on PD.
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Urinary neutrophil gelatinase associated lipocalin – A sensitive marker for urinary tract infection in children p. 340
Iswarya Jagadesan, Indira Agarwal, Swasti Chaturvedi, Arun Jose, Rani D Sahni, Jude J Fleming
Early antibiotic treatment for urinary tract infection (UTI) in young children can prevent renal scarring. Sensitivity of pyuria and positive urine nitrite test as indicators of UTI are low, whereas results of urine culture, the gold standard for diagnosing UTI, may not be available for 48--72 h. Novel markers for rapid and accurate diagnosis of UTI would help in the early initiation of treatment in children with suspected UTI. We studied the utility of urinary neutrophil gelatinase-associated lipocalin (NGAL) as an early marker of UTI. This study included 100 children between 3 months and 5 years with suspected UTI. After parental consent, a midstream clean catch or suprapubic aspirate urine specimen was sent for culture and NGAL analysis. Sensitivity and specificity of urine NGAL as a marker of UTI were estimated. Of the 100 children evaluated, urine culture was positive in 34%. Median urine NGAL values were higher in culture-positive children than in culture-negative children (223.20 vs 13.65, P = 0.0001). Receiver operating curve analysis showed an optimal cutoff level of 27 ng/ml for urine NGAL (odds ratio, 8.2, 95% confidence interval, 3.1--22.1) correlating best with culture positivity. Sensitivity and specificity of urine NGAL estimation were significantly better (79.4% and 68.2%) when compared with urine white blood cell estimation (70.6% and 53%). Urine NGAL is a sensitive and specific marker to predict UTI in children with a cutoff level of 27 ng/ml. It may serve as a screening test for detecting simple, uncomplicated UTI in young children.
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Medical comorbidity of nocturnal enuresis in children p. 345
Pietro Ferrara, Roberta Autuori, Flavia Dosa, Alessandro Di Lucia, Antonio Gatto, Antonio Chiaretti
Introduction: The purpose of this study was to evaluate the characteristics of patients with nocturnal enuresis (NE). Methods: We enrolled 403 children with NE referred to the Services of Pediatrics, Campus Bio-Medico University Hospital of Rome between June 2013 and July 2018. We excluded 2 children, respectively, with renal agenesis and chromosomopathy. Results: Of the 401 patients, 101 girls (25.2%) and 300 boys (74.8%), aged 5–16 years; mean age at first visit 8.8 ± 2.44 years. During the physical examination, we asked the patients and their parents specific questions to identify signs and symptoms of voiding disorders and comorbid conditions. In addition, we evaluated family history and behavioral characteristics of patients. In this study, NE was heredity in the 31.2% of cases. We found urogenital abnormalities in 15.7% of cases, constipation in 14.5% of cases, innocence heart murmur in 21.4% of cases and parasomnias in a good percentage of cases, especially snoring (13.7%), restless sleep (5.7%), somniloquy (23.7%) and bruxism (14.7%). Conclusions: Our experience demonstrates that there are a lot of comorbidities that are associated with NE and can influence the prognosis and the response to the therapy in these children.
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Composite pheochromocytoma presenting as severe lactic acidosis and back pain: A case report p. 353
R Mathi Manoj Kumar, NK Narayanan, KJ Raghunath, S Rajagopalan
Pheochromocytoma is a rare, catecholamine secreting tumor arising from chromaffin cells. Presentation of this tumor is highly variable, the most common being hypertension, tachycardia, sweating, and headache. Lactic acidosis and back pain are rare complications of this tumor. We report a 51-year-old gentleman with composite pheochromocytoma, which is rarer than pheochromocytoma, presenting as severe back pain and lactic acidosis.
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Burning feet, dilated heart and failed kidneys p. 357
SP Yadav, A Abraham, R Nadig, A Vasudevan
Fabry's disease, X-linked lysosomal storage disease, results from deficient activity of alpha galactosidaseA (α-GalA). Renal manifestation usually begins at third decade of life. We report a 16 year male with initial presentation with end stage renal disease (ESRD) andthe diagnosis confirmed by presence of myeloid bodies on electron microscopy of kidney biopsy and low serum α-GalA level.
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Antenatal bartter syndrome caused by a novel homozygous mutation in SLC12A1 Gene p. 360
Filiz Aktürk Acar, Güneş Işik, Mehmet Mutlu, Şebnem Kader, Yakup Aslan, Mukaddes Kalyoncu
Antenatal Bartter syndrome (BS) is an autosomal recessive hereditary renal tubular disorder caused by mutation in the solute carrier family 12 member 1 (SLC12A1) gene on chromosome 15q21.1. This syndrome is characterized by polyuria, hyponatremia, hypokalemic hypochloremic metabolic alkalosis, and hypercalciuria associated with increased urinary loss of electrolytes. Herein, we report a very low-birth-weight premature newborn with antenatal BS caused by a novel homozygous mutation in the SLC12A1 gene, c.596G>A (p.R199H).
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Isolated Vascular Lesions (IVL) in early allograft biopsies: A case series p. 364
Vennela Bangaru, Megha Uppin, Manjusha Yadla, Swarnalatha Gudithi, Gangadhar Taduri, SreeBhushan Raju
This case series includes five patients diagnosed as isolated vascular lesion (IVL) on allograft biopsy in an early post-transplant period. These patients presented with graft dysfunction. The biopsies satisfied the criteria for IVL as laid down by Banff 2009. Four of thesepatients were treated with corticosteroids and other anti rejection measures. C4d and DSA were negative in all. The patients showed good response to treatment with stable graft function at the longest follow-up of one year. We have also reviewed the literature about IVL as a specific entity. There are differences between the molecular and clinical data of IVL. It is difficult to differentiate whether IVL is a rejection or non-rejection process. This study aims to highlight the importance of a rare entity.
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Association of membranoproliferative glomerulonephritis with papillary carcinoma thyroid p. 368
Navin Pattanashetti, Gargi Kapatia, Ritambhra Nada, KL Gupta, Raja Ramachandran
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Bilateral renal cortical necrosis in a case of granulomatosis with polyangitis p. 369
Jasmine Sethi, Ritambhra Nada, Harbir Singh Kohli, Krishan Lal Gupta
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Takayasu aortoarteritis in children: A diagnostic and management challenge p. 371
Punam Barjacharya, Jyoti Sharma, Jyoti Singhal, Suprita Kalra
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Comparison of two steroid regimens in induction therapy of proliferative lupus nephritis: A randomized controlled trial p. 373
Joyita Bharati, Manish Rathi, Raja Ramachandran, Aman Sharma, Vivek Kumar, Harbir Singh Kohli, Krishan Lal Gupta
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Antiglomerular basement membrane disease combined with IgA nephropathy p. 375
Yashita Gupta, Meenakshi Swain, Swarnalata Gowrishankar
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Indian Journal of Nephrology
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Online since 20th Sept '07