scholarly journals BARDET — BIEDL SYNDROME WITH CONGENITAL KIDNEY MALFORMATION IN A 14-YEAR-OLD GIRL

2019 ◽  
Vol 26 (3) ◽  
pp. 129-134
Author(s):  
Anna V. Burlutskaya ◽  
Natalia V. Savelyeva

Aim. To show the variety and severity of the clinical symptoms of a rare hereditary disease — Вardet — Biedle syndrome — in a14-year-old girl. Materials and methods. We carried out a retrospective analysis of anamnestic data, the course of the disease, laboratory and instrumental data, as well as the therapy of a 14-year-old patient with Вardet-Вiedlе syndrome. Results. A 14-year-old patient with hereditary Вardet — Вiedlе syndrome (polydactyly, mental retardation, progressive obesity, pigment retinopathy) was found to have a congenital abnormality of kidney development — hypoplasia and dysplasia of the only right kidney complicated by chronic secondary pyelonephritis with the development of chronic kidney disease. Urine tests revealed leukocyturia, bacteriuria and proteinuria. The echo-signs of diffuse changes in the parenchyma of the right kidney were found during the ultrasound kidney examination. The left kidney was not determined. The conclusion of dynamic renal scintigraphy: the image of the left kidney is not reliably visualized (a sharp decrease in the function or absence of the kidney), the preserved filtration function and a moderate decrease in the excretory function of the right kidney. Intoxication syndrome, leukocyturia, bacteriuria and proteinuria were relieved against the background of antibacterial (Ceftriaxone) and symptomatic therapy. Conclusion. We have described a clinical case of the Вardet — Вiedlе syndrome with congenital kidney malformation (impaired function), but with the normal structure of the internal reproductive organs and sexual development. In the future, due to the development of chronic kidney disease, the patient should receive specialized nephrological care, as well as be observed by endocrinologist due to a high risk of developing type 2 diabetes mellitus.

Author(s):  
Christine U. Lee ◽  
James F. Glockner

53-year-old woman with chronic kidney disease Coronal SSFSE image (Figure 17.15.1) demonstrates massive enlargement of the right kidney, which contains multiple fatty lesions as well as several round nodules. The left kidney is surgically absent. Coronal respiratory-triggered FSE T2-weighted image (Figure ...


2019 ◽  
Vol 61 (1) ◽  
pp. 117-127 ◽  
Author(s):  
Philippe Braconnier ◽  
Maciej Piskunowicz ◽  
Nima Vakilzadeh ◽  
Marie-Eve Müller ◽  
Emilie Zürcher ◽  
...  

Background Renal length, volume, and parenchymal thickness are important clinical parameters, yet data concerning the accuracy and reproducibility of ultrasound (US)-based renal length and volume assessment in patients with chronic kidney disease (CKD) are scarce. Purpose To establish whether renal length, volume, and parenchymal thickness can be reliably measured with renal US in patients with CKD. Material and Methods All participants underwent renal US, immediately followed by 3-T magnetic resonance imaging (MRI). Renal length, width, transverse diameter, and parenchyma thickness were measured with both methods; renal volume was calculated using the ellipsoid formula. A total of 45 patients with CKD (eGFR [mean ± SD] 57.4 ± 4.4 mL/min/1.73 m2) and 46 participants without CKD (eGFR 97.0 ± 2.4 mL/min/1.73 m2) were included. Results US-measured renal length correlated strongly with MRI-measured renal length in no-CKD patients (Spearman’s r = 0.83 and 0.85 for the right and left kidney, respectively; P < 0.005) and CKD patients (r = 0.89 and 0.92 for the right and left kidney, respectively; P < 0.005). There was a significant but weaker correlation between MRI- and US-measured right and left renal volume (r = 0.72, P < 0.005) in no-CKD (r = 0.74 and r = 0.72, respectively; for both: P < 0.005) and CKD patients (r = 0.83 and 0.85, P < 0.005). Weak to moderate correlations were found for parenchyma thickness for the right (CKD group: r = 0.29, no-CKD: r = 0.23; for both: P < 0.05) and left kidney (CKD: r = 0.52, no-CKD group: r = 0.37, P < 0.05). Both intra-observer (Pearson’s correlations of 0.82 for the right and 0.89 for the left kidney) and inter-observer (Lin’s correlation coefficient of 0.90 for the right and 0.82 for the left kidney) reproducibility of US-assessed renal length was high. Conclusions US-based assessment of renal length in CKD patients is comparable to MRI measures. Both intra- and inter-observer reproducibility of US-assessed renal length in CKD patients are high. Measurements of US renal volume and parenchymal thickness should, however, be interpreted with caution.


2017 ◽  
Vol 59 (7) ◽  
pp. 884-890 ◽  
Author(s):  
Yutaka Sasaki ◽  
Yoshiki Hirooka ◽  
Hiroki Kawashima ◽  
Takuya Ishikawa ◽  
Kyosuke Takeshita ◽  
...  

Background Chronic kidney disease (CKD) patients have advanced glomerulosclerosis and renal interstitial fibrosis. Shear wave elastography (SWE) is useful to diagnose liver fibrosis. However, there are few data available regarding evaluation of kidney function on the use of SWE. Purpose To assess the utility of SWE by evaluating the correlation between renal function and renal elasticity using SWE. Material and Methods A total of 187 participants who had available serum creatinine levels and also underwent SWE of the kidney using a transabdominal ultrasonography were recruited at Nagoya University Hospital. We measured the depth of the shear wave (SW) in the right and left kidneys and calculated the measurement success rates. The glomerular filtration rate (GFR) classification and shear wave value (SWV) were compared. Results The success rates of the right and left kidneys were 93.6% and 71.6%, respectively. Based on these results, the correlation between GFR classification and SWV were analyzed in only the right kidneys because the success rates and the number of enrolled patients were low for the left kidney. There were significant differences found between G1 and G3a, G2 and G3a, G3a and G3b, G3a and G4, and G3a and G5. SWV significantly negatively and positively correlated with the G2–G3a and G3a–G3b classifications. Conclusion There is no correlation between renal function and SW. However, we can diagnose the progression to the CKD stages G3a and G3b by observing the changes over time using the SWV.


2022 ◽  
Vol 2022 ◽  
pp. 1-9
Author(s):  
Jie Zhang ◽  
Cuirong Duan ◽  
Xingxing Duan ◽  
Yuan Hu ◽  
Jinqiao Liu ◽  
...  

Objective. This research was to study the application value of real-time shear wave elastography (SWE) quantitative evaluation based on deep learning (DL) in the diagnosis of chronic kidney disease (CKD) in children. Methods. 60 children with pathological diagnoses of CKD were selected as a CKD group. During the same period, 45 healthy children for physical examination were selected as the control group. The application value of real-time shear-wave elastography based on DL in the evaluation of CKD in children was explored by comparing the differences between the two groups. Results. It was found that the elastic modulus values of the middle and lower parenchyma of the left kidney and right kidney in the case group were (22.02 ± 10.98) kPa and (21.99 ± 11.87) kPa, respectively, which were substantially higher compared with (4.61 ± 0.47) kPa and (4.50 ± 0.59) kPa in the control group. Young’s modulus (YM) of the middle and lower parenchyma of the left kidney in patients with CKD stages 3 to 5 was 13.27 ± 0.83, 24.21 ± 5.69, and 31.67 ± 3.82, respectively, and that of the right kidney was 17.26 ± 0.98, 26.76 ± 7.22, and 32.37 ± 4.27, respectively, and the difference was significant ( P  < 0.05). In patients with moderate and severe CKD, the YM values of the middle and lower parenchyma of the left kidney were 17.27 ± 0.83, 27.93 ± 6.49, and those of the right kidney were 17.26 ± 0.98, 29.56 ± 6.49, respectively, and the difference was statistically significant ( P  < 0.05). The serum creatinine (Scr) of the CKD group was substantially higher than that of the control group, and the estimated glomerular filtration rate (eGFR) level of the former was lower than that of the latter. However, there was no statistical difference between the YM values of the middle and lower parts of the left and right kidneys of the CKD group and the control group. Conclusion. The DL-based SWE is a new noninvasive, real-time, and quantitative detection method, which can effectively evaluate the stiffness of the kidney and help to better detect the progress of CKD as a clinical reference.


The Lancet ◽  
2011 ◽  
Vol 377 (9784) ◽  
pp. 2153-2154 ◽  
Author(s):  
Kathryn K Stevens ◽  
Alan G Jardine

2021 ◽  
Vol 19 (6) ◽  
pp. 98-106
Author(s):  
Monawar Muhsin Jabr ◽  
Hussain S. Hasan ◽  
Hind Ahmed Mahdi

Background: Chronic kidney disease (CKD) is a public health problem over all the world. CKD may also be defined by the presence of kidney damage or a reduced glomerular filtration rate (GFR), which is the best overall indicator or index of kidney function. CKD patients are usually treated using kidney dialysis (hemodialysis) that uses a blood filtration mechanism (HD). Several metabolic parameters, such as blood urea, sodium, potassium, and glucose levels, can alter during HD. Osmotic alterations in blood, aqueous and vitreous humor, and other extracellular fluids arise from these fluctuations. That also can affect visual acuity, intraocular pressure (IOP), and retinal thickness. Aim of the Study: To evaluate some of the ocular findings undergoing HD to keep prevent the loss of patient vision such as visual acuity (VA), intraocular pressure (IOP), central corneal thickness (CCT), central Foveal Thickness (CFT), retinal nerve fiber layer (RNFL). Patient& Methods: This is a cohort (prospective) design study. This study including Seventy nine patients divided into two groups the first group from one week to six month (9 femal & 18 males) another group over than six month (36 female & 16 male) the average age between (12 to 70 years). This research performed in the three places department of the eye in Al-Hussein hospital in Samawah city, Al-Haboby hospital, Al-Hussein hospital in Dhi Qar city finally in Al-Shaheed Gazy hospital and Baghdad teaching hospital in Baghdad. Examining Visual Acuity by Snellen chart & auto refractometer, IOP& CCT by (CT.1 Computerized Tonometer TOPCON), RNLF and Central Foveal Thickness by OCT (Carl ZEISS, TOPCON). The inclusion criteria were as follows: all the patients undergoing dialysis from one week to over six months. Exclusion criteria were as follows: the patients have diabetic, any patients have a hereditary disease or glaucoma history or laser therapy, or intraocular injection in the eye before dialysis, the patients have a problem in the eye before dialysis such as cataracts or opacity leads to does surgery, the patients who have a refractive error or wear glass had been also excluded. Result: Includes the results of seventy-nine patients (45 females and 34 males) with chronic kidney disease examined ocular findings before a session of dialysis divided into two groups based on their duration of dialysis. Group one with twenty-seven patients (9 female & 18 male) under dialysis from one week to six months with mean & standard deviation (3.2037, ± 1.89259), group tow with fifty tow patients (36 female & 16 male) under dialysis from the duration over than six months with mean & standard deviation (44.2308, ± 26.24367) respectively. Patients aged (12 to 70 years) had mean age & ± standard deviation (35.1481, ± 12.88918), (44.4038, ± 15.42249) for two groups respectively. Patients in two groups had IOP (Right eye), its mean & standard deviation (15, ± 2.34), (15.69, ± 2.56) for group one & group tow respectively. Also, patients had CCT (Right eye) with mean & standard deviation (5.3467E2, ± 39.00296), (5.2312E2, ± 30.44162) for group one & group tow respectively. Patients had CCT (Left) with mean & standard deviation (5.2878E2, ± 37.55748), (5.2179E2, ± 29.58957) for group one & group tow respectively. Patients in two groups had average thickness RNFL (Right eye) with mean & standard deviation (1.0604E2, ± 25.17551), (95.6154, ± 21.27150) for group one & group tow respectively. Also, patients had average thickness RNFL (left eye) with mean & standard deviation (1.0930E2, ±23.80177), (98.7500, ± 23.77334) for group one & group tow respectively. Conclusions: This study found CCT effective with dialysis tend to be thin (53 patient,18 patient in group one &35 in group two) and that will be had a threefold higher risk of developing glaucoma when compared with thick average because of the IOP value affected by it. Refractive error effective with dialysis & become was more prominent that can be shown in the group two have (40 patient from 52) while (15 patient from27) in the group one although a lot of them corrected to the BCVA. In conclusion high value of the C/D ratio formed about (45.57%, 53.16%) to the right &left eye respectively this value will be form important sign of risk factor to progressive of glaucomatous need to be alert in the future. Also our research reveals CFT effective undergoing dialysis the thick value was (56 in the right eye, 55 in the left eye) high compared with the thin (9 in the right&9 in the left eye) & normal (14 in the right eye, 15 in the left eye). All the two groups of patients will be effected by the duration of dialysis with a time.


Author(s):  
Natalie Ebert ◽  
Elke Schaeffner

Both acute and chronic states of kidney disease have considerable healthcare impact as they can produce enormous disease burden and costs. To classify chronic kidney disease into the CKD staging system, glomerular filtration rate as an index of kidney function, as well as albuminuria as a marker of kidney damage have to be assessed as correctly as possible. Misclassification is a serious concern due to the difficulties in precise GFR assessment and correct interpretation of results. Differentiating between pure senescence and true disease among older adults can be a delicate issue. To find the right renal replacement option for individuals that progress to end-stage renal disease can be challenging, and some older patients may even benefit from conservative care without dialysis. To prevent acute kidney injury as a frequent and potentially life-threatening complication, clinicians need to develop an understanding of the common vulnerability to kidney damage among older adults.


2019 ◽  
Vol 34 (Supplement_1) ◽  
Author(s):  
Mabel Aoun ◽  
Antoine Dfouni ◽  
Ghassan Sleilaty ◽  
Rayane Salloum ◽  
Dania Chelala

2020 ◽  
Vol 19 (1) ◽  
Author(s):  
Nam Ju Heo ◽  
Sang Youl Rhee ◽  
Jill Waalen ◽  
Steven Steinhubl

Abstract Background Diabetes is an independent risk factor for atrial fibrillation (AF), which is associated with increases in mortality and morbidity, as well as a diminished quality of life. Renal involvement in diabetes is common, and since chronic kidney disease (CKD) shares several of the same putative mechanisms as AF, it may contribute to its increased risk in individuals with diabetes. The objective of this study is to identify the relationship between CKD and the rates of newly-diagnosed AF in individuals with diabetes taking part in a screening program using a self-applied wearable electrocardiogram (ECG) patch. Materials and methods The study included 608 individuals with a diagnosis of diabetes among 1738 total actively monitored participants in the prospective mHealth Screening to Prevent Strokes (mSToPS) trial. Participants, without a prior diagnosis of AF, wore an ECG patch for 2 weeks, twice, over a 4-months period and followed clinically through claims data for 1 year. Definitions of CKD included ICD-9 or ICD-10 chronic renal failure diagnostic codes, and the Health Profile Database algorithm. Individuals requiring dialysis were excluded from trial enrollment. Results Ninety-six (15.8%) of study participants with diabetes also had a diagnosis of CKD. Over 12 months of follow-up, 19 new cases of AF were detected among the 608 participants. AF was newly diagnosed in 7.3% of participants with CKD and 2.3% in those without (P < 0.05) over 12 months of follow-up. In a univariate Cox proportional hazard regression analysis, the risk of incident AF was 3 times higher in individuals with CKD relative to those without CKD: hazard ratios (HR) 3.106 (95% CI 1.2–7.9). After adjusting for the effect of age, sex, and hypertension, the risk of incident AF was still significantly higher in those with CKD: HR 2.886 (95% CI 1.1–7.5). Conclusion Among individuals with diabetes, CKD significantly increases the risk of incident AF. Identification of AF prior to clinical symptoms through active ECG screening could help to improve the clinical outcomes in individuals with CKD and diabetes.


2020 ◽  
Vol 11 ◽  
pp. 204062232092201
Author(s):  
Yang Zhao ◽  
Yanyan Li ◽  
Junwei Li ◽  
Weijuan Song ◽  
Jun Zhao ◽  
...  

Aim: The aim of this study was to evaluate the reporting quality of chronic kidney disease (CKD) guidelines. Methods: PubMed, EMBASE, and guideline-related websites were searched from 2008 to 2019. The CKD guidelines were included. Two reviewers used the RIGHT (Reporting Items for Practice Guidelines in Healthcare) checklist to assess the quality of guidelines and calculate the reporting proportion of each guideline. Results: We included 13 guidelines, of which 30.8% (4/13) were developed in Europe and about two-thirds (8/13) were published on their own website. The average quality of the 13 guidelines was 68.57%. The reporting proportion of the seven domains (i.e. basic information; background; evidence; recommendations; review and quality assurance; funding and declaration and management of interests; other information) were 65.39%, 81.73%, 63.08%, 69.23%, 53.85%, 63.46%, and 61.54%, respectively. Conclusion: CKD guidelines had moderate reporting quality in some domains, but guideline developers should increase the reporting items in basic information, guideline evidence, and recommendations. The RIGHT checklist would be a useful tool to improve the reporting quality of guidelines.


Sign in / Sign up

Export Citation Format

Share Document