scholarly journals Long-term renal outcome in methylmalonic acidemia in adolescents and adults

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Myriam Dao ◽  
Jean-Baptiste Arnoux ◽  
Frank Bienaimé ◽  
Anaïs Brassier ◽  
François Brazier ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Filtration Rate ◽  
Methylmalonic Acidemia ◽  
Renal Outcome ◽  
Schwartz Formula ◽  
Glomerular Proteinuria ◽  
Therapeutic Decisions ◽  
Proximal Tubular

Abstract Background Chronic kidney disease (CKD) is one of the main long-term prognosis factors in methylmalonic acidemia (MMA), a rare disease of propionate catabolism. Our objective was to precisely address the clinical and biological characteristics of long-term CKD in MMA adolescent and adult patients. Patients and methods In this retrospective study, we included MMA patients older than 13 years who had not received kidney and/or liver transplantation. We explored tubular functions, with special attention to proximal tubular function. We measured glomerular filtration rate (mGFR) by iohexol clearance and compared it to estimated glomerular filtration rate (eGFR) by Schwartz formula and CKD-EPI. Results Thirteen patients were included (M/F = 5/8). Median age was 24 years (13 to 32). Median mGFR was 57 mL/min/1.73 m2 (23.3 to 105 mL/min/1.73 m2). Ten out of 13 patients had mGFR below 90 mL/min/1.73 m2. No patient had significant glomerular proteinuria. No patient had complete Fanconi syndrome. Only one patient had biological signs suggestive of incomplete proximal tubulopathy. Four out of 13 patients had isolated potassium loss, related to a non-reabsorbable anion effect of urinary methylmalonate. Both Schwartz formula and CKD-EPI significantly overestimated GFR. Bias were respectively 16 ± 15 mL/min/1.73 m2 and 37 ± 22 mL/min/1.73 m2. Conclusion CKD is a common complication of the MMA. Usual equations overestimate GFR. Therefore, mGFR should be performed to inform therapeutic decisions such as dialysis and/or transplantation. Mild evidence of proximal tubular dysfunction was found in only one patient, suggesting that other mechanisms are involved.

scholarly journals Long-term renal function and continence status in patients with

2013 ◽  
Vol 1 (4) ◽  
pp. 371 ◽  
Author(s):  
Luis H.P. Braga ◽  
Armando J. Lorenzo ◽  
Sumit Dave ◽  
Maria H. Del-Valle ◽  
Antoine E. Khoury ◽  
...  
Keyword(s):  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Filtration Rate ◽  
Urogenital Sinus ◽  
Renal Outcome ◽  
Urinary Continence ◽  
Renal Ultrasound ◽  
Common Channel

Introduction: Urinary continence after cloacal repair is difficult to achieveand renal outcome in patients with cloacal malformations has been scarcelyreported. As a result, we reviewed our experience with cloacal malformationsto determine the status of continence and the long-term renal function in thesechildren.Methods: A retrospective chart review from 1990 to 2003 identified 12 patientswith cloacal malformation (1 posterior, 4 complex and 7 classical) who underwentsurgical reconstruction. The confluence was defined as high (commonchannel ≥ 3 cm) and low (< 3 cm) by cystovaginoscopy. Renal ultrasound,voiding cystouretrogram, renal scan and sacral radiograph were performedin all children. Most patients underwent 1-stage abdominoperineal pull-through,applying the principle of total urogenital sinus mobilization. We collected dataregarding hydronephrosis, vesicoureteral reflux and split differential renal function.Renal outcome was evaluated based on glomerular filtration rate and ageadjustedserum creatinine values (μmol/L). Urinary continence was definedas a dry interval > 4 hours.Results: Patients’ mean age at surgery was 20 months (range 7–29 mo). Ofthe 12 children who underwent cloacal repair, 7 (58.3%) had a common channel≥ 3cm. Renal anomalies were identified in 3 of 12 (25%) girls: there were 2 solitary kidneys and 1 pelvic kidney. Lumbar–sacral radiography demonstrated bony abnormalities in 11 of the 12 (91.6%) cases: hemivertebra in 3 cases, sacral agenesis in 4 cases, hypoplastic sacrum in 3 cases and bifid sacrum in 1 case. Total urogenital sinus mobilization through an abdominoperineal approach in a single stage was performed in 8 girls. Follow-up ranged from 4 to 14 years (mean 8.5 yr). Eight (66.6%) children had dry intervals > 4 hours, 5 (62.5%) of them were on clean intermittent catheterization through aMitrofanoff channel and 1 (12.5%) was through the urethra. The remaining 2 (25%) patients were voiding spontaneously. Three (33.3%) patients were totally incontinent, and 1 (8.3%) patient was awaiting reconstruction. The mean measured glomerular filtration rate was 93.5 mL/min/1.73m2 (range 34–152 mL/min/1.73m2). Four (57.1%) of 7 patients who had a common channel ≥ 3 cm ended up needing augmentation cystoplasty, compared with none of the patients with a common channel < 3 cm (57.1% v. 0%, p = 0.038).Conclusion: Urinary continence can be achieved in most patients with cloacalmalformation at the expense of major reconstructive surgery and despite thepresence of associated urological abnormalities. However, these childrenharbour an important risk for renal impairment later in life and should be closelymonitored.

scholarly journals Profile of estimated glomerular filtration rate of children with Human Immunodeficiency Virus/ Acquired Immune Deficiency Syndrome

2019 ◽  
Vol 6 (5) ◽  
pp. 1931
Author(s):  
Gabriel O. Ezeh ◽  
Oluseyi Oniyangi ◽  
Iretiola B. Babaniyi ◽  
Vincent E. Nwatah ◽  
Felicia U. Eke
Keyword(s):  
Glomerular Filtration Rate ◽  
Hiv Infection ◽  
Glomerular Filtration ◽  
Filtration Rate ◽  
Developed Countries ◽  
People Living With Hiv ◽  
Schwartz Formula ◽  
Cross Sectional ◽  
Female Ratio ◽  
The Mean

Background: Kidney disease is more common in people of African descent in developed countries. Studies reporting estimate Glomerular Filtration Rate (eGFR) in African populations and people living with HIV have been carried more frequently on adults than children. The study aimed to assess eGFR by use of the SCHWARTZ formula in HIV infected children seen at tertiary hospital.Methods: A descriptive, prospective and cross sectional study of 221 children with HIV infection. Schwartz formula was used to determine eGFR. The main outcome measures were eGFR. The study population comprised HIV infected children attending Paediatric out-patients’ clinic and those admitted into the Paediatric wards, aged between 6 months and 15 years. Data was analysed using SPSS version 20 and results presented in tables and figures. Results: The age range of the study subjects was 12 months to 15 years with the mean age and SD of 8.21±3.61 years. There were 129 (58.4%) male and 92 (41.6%) female children with male to female ratio 1: 0.7.  The mean age for males was 7.87±3.49 years while that for females was 8.70±3.71 years. The eGFR for the study as determined by Schwartz formula had a range of 49.21 to 463.67 ml/ min/ 1.73m2 with the mean of 159.56±59.04 ml/min. The mean eGFR for the males and females were 166.39±63.54ml/ min and 149.99±45.01 ml/ min respectively. Conclusion: The study, in comparison with other studies, observed a lower prevalence of CKD in HIV infected children. Detection of CKD in HIV infection children may be more optimal if combined methods are employed.

Long-Term Follow-Up of Separate Glomerular Filtration Rate in Partially Obstructed Kidneys

1988 ◽  
Vol 22 (4) ◽  
pp. 327-333 ◽  
Author(s):  
A. Piepsz ◽  
H. R. Ham ◽  
M. Hall ◽  
Y. Thoua ◽  
J. L. Froideville ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Filtration Rate ◽  
Long Term Follow Up

Protein Intake and Long-term Change in Glomerular Filtration Rate in the Jackson Heart Study

2018 ◽  
Vol 28 (4) ◽  
pp. 245-250 ◽  
Author(s):  
Rakesh Malhotra ◽  
Loren Lipworth ◽  
Kerri L. Cavanaugh ◽  
Bessie A. Young ◽  
Katherine L. Tucker ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Protein Intake ◽  
Filtration Rate ◽  
Jackson Heart Study ◽  
Term Change ◽  
Heart Study

Glomerular Filtration Rate and Calcium Metabolism in Long-Term Lithium Treatment

1981 ◽  
Vol 7 (2) ◽  
pp. 105-111 ◽  
Author(s):  
H. Thysell ◽  
G. Brante ◽  
L. Sjöstedt ◽  
B. Lindergård ◽  
T. Lindholm ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Calcium Metabolism ◽  
Filtration Rate ◽  
Lithium Treatment

scholarly journals Matching Human Unilateral AKI, a Reverse Translational Approach to Investigate Kidney Recovery after Ischemia

2019 ◽  
Vol 30 (6) ◽  
pp. 990-1005 ◽  
Author(s):  
Danielle E. Soranno ◽  
Hyo-Wook Gil ◽  
Lara Kirkbride-Romeo ◽  
Christopher Altmann ◽  
John R. Montford ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Renal Injury ◽  
Filtration Rate ◽  
Human Study ◽  
Renal Ischemia ◽  
Research Approach ◽  
Urine Biomarkers ◽  
Injured Kidney

BackgroundThe duration of renal ischemia that is associated with (or leads to) renal injury in patients is uncertain, and a reverse translational research approach has been proposed to improve animal models of AKI to facilitate clinical translatability. We developed a two murine models of unilateral renal ischemia to match a recently published human study that investigated renal injury after unilateral renal ischemia during partial nephrectomy.MethodsEight 10-week-old C57BL/6 male mice underwent left UiAKI or sham procedure, with or without intra-operative ice packs. Functional, histological, and biomarker outcomes were followed at 2, 6 and 24 hours, or 14 or 28 days later. The 14 and 28 day cohorts were duplicated such that contralateral nephrectomy could be performed 3 days prior to sacrifice with functional measurements obtained to isolate the glomerular filtration rate of the injured kidney.ResultsThe short-term outcomes correlated with the human study findings with urine and serum biomarkers of injury peaking around 24 hours and then normalizing, and reassuring immediate histological outcomes. Functional and histological outcomes at the later time-points (14 and 28 days) demonstrate an increase in fibrosis markers, and a reduction in glomerular filtration rate in the injured kidney, corresponding to the duration of ischemia, while serum and urine biomarkers remained reassuring.ConclusionsOur findings suggest that clinically available biomarkers of renal function are falsely reassuring against long-term injury following UiAKI, and that the duration of ischemia correlates with impaired function and increased fibrosis.

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