Sensory ataxic polyneuropathy unmasking late-onset urea cycle defect.

A 63-year-old man with type 2 diabetes mellitus, alcohol consumption in moderation, and three episodes of hepatic encephalopathy presented with symmetrical lower limb distal weakness, sensory ataxia, thickened palpable nerves, mood disturbances for seven years, and a family history of schizophreniform disorders. Nerve conduction studies showed demyelinating sensorimotor polyradiculoneuropathy. CSF analysis showed mild albumino-cytological dissociation. MRI brain and lumbosacral plexus showed thickened fifth cranial nerves and lumbosacral roots. He was treated with steroids for a provisional diagnosis of chronic inflammatory polyneuropathy and became encephalopathic. EEG showed triphasic waves. Serum ammonia was 201 micrograms/dL. Further evaluation suggested ornithine transcarbamylase (OTC) deficiency. The patient underwent hemodialysis with a low protein diet, rifaximin, and sodium benzoate, with subsequent recovery.

Retrospective Analysis of Spectrum of Infections and Antibiotic Resistance Pattern in Chronic Kidney Disease Patients on Maintenance Hemodialysis in a Tertiary Care Centre in North India.

Introduction: Hemodialysis patients are at risk of infections. This study examines the spectrum of infections and antibiotic resistance patterns. Methods: We retrospectively reviewed the records of 586 hemodialysis patients from May 2018 to April 2020 in a tertiary care hospital in North India. Results: The study identified 99 episodes of confirmed infections. Urinary tract infections were the most common type of infections (55.5%), followed by catheter-related bloodstream infections (CRBSI) (definitive 21.2%). Other infections were pneumonia (8.1%), tuberculosis (6.1%), skin and soft tissue infection (4.0%), dengue fever (3.03%), and empyema thoracis (1.0%). Overall, Escherichia coli (33.3%) was the most common organism isolated. The most frequent uropathogens recovered were Escherichia coli (54%). In confirmed CRBSI, P. aeruginosa (23.8%) and MSSA (23.8%) were the most common pathogen isolated. K. pneumonia (37.5%) was the most common pathogen in pneumonia. Uropathogens showed the highest resistance to fluoroquinolones (93.3%-100%). Pathogens isolated in CRBSI showed maximum resistance to ciprofloxacin (100%). In pneumonia, the highest resistance was seen to third-generation cephalosporins (75%-100%). Conclusion: Though the bacterial spectrum remains the same over time, antibiotic resistance is changing in uropathogens. There is a trend of predominance of Gram-negative bacterial infections in CRBSI. Tuberculosis incidence rate was much higher than the general population. There is a need for nationwide and worldwide continuous surveillance.

Relationship between biochemical parameters of mineral bone disease and static bone histomorphometry in chronic kidney disease patients on hemodialysis: An Indian cross-section study.

AIM: We estimated the relationship between routine biochemical laboratory parameters with static bone histomorphometric parameters and their high and low bone turnover capacity predictability in hemodialysis patients. METHOD: It was a single-center cross-sectional study, included 28 hemodialysis patients. The routine biochemical parameters measured including calcium, phosphorous, alkaline phosphatase, intact PTH, and 25-hydroxycholecalciferol. The histomorphometric parameters assessed were osteoblasts perimeter, osteoclast perimeter, eroded perimeter, osteoid perimeter, bone fibrosis and bone volume. RESULT: Total 28 hemodialysis patients underwent bone biopsy. Seventy percent were male, with a mean age was 33.07±10.42 yrs; serum alkaline phosphatase was 219.10±311.3IU/ml; vitamin D was 18.18±9.56ng/ml, and intact PTH was 650.7±466.0pg/ml. Intact PTH had a significant positive association with osteoblast, osteoclast, eroded surface, and osteoid perimeter. Serum alkaline phosphatase had a significant relationship with bone fibrosis (r=0.525, p-value=0.004). Intact PTH was significantly higher in females than males (1078.75±533.04 vs. 479.6±309.83; p-value=0.004). The osteoid surface was significantly high in females compared to males (p=0.038). Age had a significant impact on osteoblast and eroded surface (p=0.008 and p=0.031, respectively). Intact PTH is a reliable biomarkers for bone turnover compare to ALP (p<0.001 and p=0.554, respectively). CONCLUSION: Intact PTH strongly associated with bone formation, bone resorption parameters. Gender and age had significant impact on static histomorphometric parameters in our study.

Study of renal parenchymal changes by diffusion tensor imaging in diabetic nephropathy.

Purpose: To evaluate differences in diffusion imaging parameters, including fractional anisotropy (FA) and the apparent diffusion coefficient (ADC), in control and diabetic subjects, and to assess changes in these parameters to patient's urine albumin/protein levels, estimated glomerular filtration rate (eGFR), and glycated haemoglobin (HbA1c). Material and methods: This is a cross-sectional analytical study involving 100 patients who underwent diffusion imaging including diffusion tensor imaging (DTI) of the kidneys in our hospital from 2019 to 2020. Diffusion imaging parameters (ADC and FA) were obtained from the medulla and cortex of both kidneys using dedicated software. Statistical analysis was done. Results: Out of 100 subjects, 27 were controls and 73 were diabetics (19 normoalbuminuric, 23 microalbuminuric, and 31 proteinuric). The medullary FA (0.419 ± 0.024 vs. 0.346 ± 0.042), cortical FA (0.194 ± 0.035 vs. 0.303 ± 0.067), and cortical ADC (3.307 ± 0.341 vs. 2.309 ± 0.515) values showed significant differences between controls and diabetics. Medullary FA and cortical ADC values showed a decreasing trend with an increasing amount of albumin/protein in the urine, decreasing renal function (reducing eGFR), and increasing HbA1c, whereas the trend was opposite for cortical FA. In addition, on ROC curve analysis a cut-off value for medullary FA of 0.4 had a sensitivity of 64% and specificity of 80.95% to differentiate healthy volunteers and diabetics with normo-albuminuria. Conclusions: DTI has the potential to be a promising non-invasive test for the detection of early renal parenchymal changes in diabetic nephropathy.