| Title |
Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment
|
|---|---|
| Published in |
Pediatric Nephrology, March 2017
|
| DOI | 10.1007/s00467-017-3634-3 |
| Pubmed ID | |
| Authors |
Mustafa Atmaca, Bora Gulhan, Emine Korkmaz, Mihriban Inozu, Oguz Soylemezoglu, Cengiz Candan, Aysun Karabay Bayazıt, Ahmet Midhat Elmacı, Gonul Parmaksiz, Ali Duzova, Nesrin Besbas, Rezan Topaloglu, Fatih Ozaltin |
| Abstract |
ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. A total of 146 index patients aged 10-18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation. Twenty-eight individuals with bi-allelic mutation from 11 families were identified. Median age at diagnosis was 12.4 (interquartile range [IQR] 8.04-19.7) years. Upon first admission, all patients had albuminuria and 18 had CKD (6 ESKD). Eight were diagnosed either through the screening of family members following index case identification or during genetic investigation of proteinuria in an individual with a history of a transplanted sibling. Median age of these 8 patients was 21.5 (range 4.4-39) years. CoQ10 supplementation was administered following genetic diagnosis. Median estimated glomerular filtration rate (eGFR) just before CoQ10 administration was 140 (IQR 117-155) ml/min/1.73m(2), proteinuria was 1,008 (IQR 281-1,567) mg/m(2)/day. After a median follow-up of 11.5 (range 4-21) months following CoQ10 administration, proteinuria was significantly decreased (median 363 [IQR 175-561] mg/m(2)/day, P=0.025), whereas eGFR was preserved (median 137 [IQR 113-158] ml/min/1.73m(2), P=0.61). ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria, and may thereby be renoprotective. |
X Demographics
The data shown below were collected from the profiles of 19 X users who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Netherlands | 2 | 11% |
| Venezuela, Bolivarian Republic of | 2 | 11% |
| United Kingdom | 1 | 5% |
| Spain | 1 | 5% |
| Canada | 1 | 5% |
| United States | 1 | 5% |
| Australia | 1 | 5% |
| Brazil | 1 | 5% |
| Unknown | 9 | 47% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 15 | 79% |
| Scientists | 2 | 11% |
| Practitioners (doctors, other healthcare professionals) | 1 | 5% |
| Science communicators (journalists, bloggers, editors) | 1 | 5% |
Mendeley readers
The data shown below were compiled from readership statistics for 44 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 44 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 7 | 16% |
| Other | 5 | 11% |
| Researcher | 4 | 9% |
| Student > Bachelor | 4 | 9% |
| Student > Master | 4 | 9% |
| Other | 9 | 20% |
| Unknown | 11 | 25% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 15 | 34% |
| Biochemistry, Genetics and Molecular Biology | 7 | 16% |
| Nursing and Health Professions | 3 | 7% |
| Agricultural and Biological Sciences | 3 | 7% |
| Pharmacology, Toxicology and Pharmaceutical Science | 1 | 2% |
| Other | 4 | 9% |
| Unknown | 11 | 25% |
Attention Score in Context
This research output has an Altmetric Attention Score of 11. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 27 September 2017.
All research outputs
#2,790,346
of 22,961,203 outputs
Outputs from Pediatric Nephrology
#337
of 3,572 outputs
Outputs of similar age
#53,619
of 309,211 outputs
Outputs of similar age from Pediatric Nephrology
#10
of 100 outputs
Altmetric has tracked 22,961,203 research outputs across all sources so far. Compared to these this one has done well and is in the 87th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 3,572 research outputs from this source. They receive a mean Attention Score of 4.9. This one has done particularly well, scoring higher than 90% of its peers.
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