Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment
Background 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...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Atmaca, Mustafa [verfasserIn] Gulhan, Bora [verfasserIn] Korkmaz, Emine [verfasserIn] Inozu, Mihriban [verfasserIn] Soylemezoglu, Oguz [verfasserIn] Candan, Cengiz [verfasserIn] Bayazıt, Aysun Karabay [verfasserIn] Elmacı, Ahmet Midhat [verfasserIn] Parmaksiz, Gonul [verfasserIn] Duzova, Ali [verfasserIn] Besbas, Nesrin [verfasserIn] Topaloglu, Rezan [verfasserIn] Ozaltin, Fatih [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2017 |
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| Schlagwörter: |
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| Übergeordnetes Werk: |
Enthalten in: Pediatric nephrology - Berlin : Springer, 1987, 32(2017), 8 vom: 24. März, Seite 1369-1375 |
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| Übergeordnetes Werk: |
volume:32 ; year:2017 ; number:8 ; day:24 ; month:03 ; pages:1369-1375 |
| Links: |
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| DOI / URN: |
10.1007/s00467-017-3634-3 |
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| Katalog-ID: |
SPR006868029 |
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| 100 | 1 | |a Atmaca, Mustafa |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment |
| 264 | 1 | |c 2017 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a Computermedien |b c |2 rdamedia | ||
| 338 | |a Online-Ressource |b cr |2 rdacarrier | ||
| 520 | |a Background 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. Methods 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. Results 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). Conclusions 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. | ||
| 650 | 4 | |a Adolescent |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Nephrotic syndrome |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Chronic kidney disease |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a ADCK4 mutation |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a CoQ10 supplementation |7 (dpeaa)DE-He213 | |
| 700 | 1 | |a Gulhan, Bora |e verfasserin |4 aut | |
| 700 | 1 | |a Korkmaz, Emine |e verfasserin |4 aut | |
| 700 | 1 | |a Inozu, Mihriban |e verfasserin |4 aut | |
| 700 | 1 | |a Soylemezoglu, Oguz |e verfasserin |4 aut | |
| 700 | 1 | |a Candan, Cengiz |e verfasserin |4 aut | |
| 700 | 1 | |a Bayazıt, Aysun Karabay |e verfasserin |4 aut | |
| 700 | 1 | |a Elmacı, Ahmet Midhat |e verfasserin |4 aut | |
| 700 | 1 | |a Parmaksiz, Gonul |e verfasserin |4 aut | |
| 700 | 1 | |a Duzova, Ali |e verfasserin |4 aut | |
| 700 | 1 | |a Besbas, Nesrin |e verfasserin |4 aut | |
| 700 | 1 | |a Topaloglu, Rezan |e verfasserin |4 aut | |
| 700 | 1 | |a Ozaltin, Fatih |e verfasserin |4 aut | |
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10.1007/s00467-017-3634-3 doi (DE-627)SPR006868029 (SPR)s00467-017-3634-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.88 bkl 44.67 bkl Atmaca, Mustafa verfasserin aut Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background 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. Methods 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. Results 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). Conclusions 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. Adolescent (dpeaa)DE-He213 Nephrotic syndrome (dpeaa)DE-He213 Chronic kidney disease (dpeaa)DE-He213 ADCK4 mutation (dpeaa)DE-He213 CoQ10 supplementation (dpeaa)DE-He213 Gulhan, Bora verfasserin aut Korkmaz, Emine verfasserin aut Inozu, Mihriban verfasserin aut Soylemezoglu, Oguz verfasserin aut Candan, Cengiz verfasserin aut Bayazıt, Aysun Karabay verfasserin aut Elmacı, Ahmet Midhat verfasserin aut Parmaksiz, Gonul verfasserin aut Duzova, Ali verfasserin aut Besbas, Nesrin verfasserin aut Topaloglu, Rezan verfasserin aut Ozaltin, Fatih verfasserin aut Enthalten in Pediatric nephrology Berlin : Springer, 1987 32(2017), 8 vom: 24. März, Seite 1369-1375 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:32 year:2017 number:8 day:24 month:03 pages:1369-1375 https://dx.doi.org/10.1007/s00467-017-3634-3 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 ASE 44.67 ASE AR 32 2017 8 24 03 1369-1375 |
| spelling |
10.1007/s00467-017-3634-3 doi (DE-627)SPR006868029 (SPR)s00467-017-3634-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.88 bkl 44.67 bkl Atmaca, Mustafa verfasserin aut Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background 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. Methods 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. Results 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). Conclusions 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. Adolescent (dpeaa)DE-He213 Nephrotic syndrome (dpeaa)DE-He213 Chronic kidney disease (dpeaa)DE-He213 ADCK4 mutation (dpeaa)DE-He213 CoQ10 supplementation (dpeaa)DE-He213 Gulhan, Bora verfasserin aut Korkmaz, Emine verfasserin aut Inozu, Mihriban verfasserin aut Soylemezoglu, Oguz verfasserin aut Candan, Cengiz verfasserin aut Bayazıt, Aysun Karabay verfasserin aut Elmacı, Ahmet Midhat verfasserin aut Parmaksiz, Gonul verfasserin aut Duzova, Ali verfasserin aut Besbas, Nesrin verfasserin aut Topaloglu, Rezan verfasserin aut Ozaltin, Fatih verfasserin aut Enthalten in Pediatric nephrology Berlin : Springer, 1987 32(2017), 8 vom: 24. März, Seite 1369-1375 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:32 year:2017 number:8 day:24 month:03 pages:1369-1375 https://dx.doi.org/10.1007/s00467-017-3634-3 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 ASE 44.67 ASE AR 32 2017 8 24 03 1369-1375 |
| allfields_unstemmed |
10.1007/s00467-017-3634-3 doi (DE-627)SPR006868029 (SPR)s00467-017-3634-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.88 bkl 44.67 bkl Atmaca, Mustafa verfasserin aut Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background 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. Methods 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. Results 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). Conclusions 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. Adolescent (dpeaa)DE-He213 Nephrotic syndrome (dpeaa)DE-He213 Chronic kidney disease (dpeaa)DE-He213 ADCK4 mutation (dpeaa)DE-He213 CoQ10 supplementation (dpeaa)DE-He213 Gulhan, Bora verfasserin aut Korkmaz, Emine verfasserin aut Inozu, Mihriban verfasserin aut Soylemezoglu, Oguz verfasserin aut Candan, Cengiz verfasserin aut Bayazıt, Aysun Karabay verfasserin aut Elmacı, Ahmet Midhat verfasserin aut Parmaksiz, Gonul verfasserin aut Duzova, Ali verfasserin aut Besbas, Nesrin verfasserin aut Topaloglu, Rezan verfasserin aut Ozaltin, Fatih verfasserin aut Enthalten in Pediatric nephrology Berlin : Springer, 1987 32(2017), 8 vom: 24. März, Seite 1369-1375 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:32 year:2017 number:8 day:24 month:03 pages:1369-1375 https://dx.doi.org/10.1007/s00467-017-3634-3 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 ASE 44.67 ASE AR 32 2017 8 24 03 1369-1375 |
| allfieldsGer |
10.1007/s00467-017-3634-3 doi (DE-627)SPR006868029 (SPR)s00467-017-3634-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.88 bkl 44.67 bkl Atmaca, Mustafa verfasserin aut Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background 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. Methods 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. Results 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). Conclusions 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. Adolescent (dpeaa)DE-He213 Nephrotic syndrome (dpeaa)DE-He213 Chronic kidney disease (dpeaa)DE-He213 ADCK4 mutation (dpeaa)DE-He213 CoQ10 supplementation (dpeaa)DE-He213 Gulhan, Bora verfasserin aut Korkmaz, Emine verfasserin aut Inozu, Mihriban verfasserin aut Soylemezoglu, Oguz verfasserin aut Candan, Cengiz verfasserin aut Bayazıt, Aysun Karabay verfasserin aut Elmacı, Ahmet Midhat verfasserin aut Parmaksiz, Gonul verfasserin aut Duzova, Ali verfasserin aut Besbas, Nesrin verfasserin aut Topaloglu, Rezan verfasserin aut Ozaltin, Fatih verfasserin aut Enthalten in Pediatric nephrology Berlin : Springer, 1987 32(2017), 8 vom: 24. März, Seite 1369-1375 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:32 year:2017 number:8 day:24 month:03 pages:1369-1375 https://dx.doi.org/10.1007/s00467-017-3634-3 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 ASE 44.67 ASE AR 32 2017 8 24 03 1369-1375 |
| allfieldsSound |
10.1007/s00467-017-3634-3 doi (DE-627)SPR006868029 (SPR)s00467-017-3634-3-e DE-627 ger DE-627 rakwb eng 610 ASE 44.88 bkl 44.67 bkl Atmaca, Mustafa verfasserin aut Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment 2017 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background 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. Methods 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. Results 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). Conclusions 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. Adolescent (dpeaa)DE-He213 Nephrotic syndrome (dpeaa)DE-He213 Chronic kidney disease (dpeaa)DE-He213 ADCK4 mutation (dpeaa)DE-He213 CoQ10 supplementation (dpeaa)DE-He213 Gulhan, Bora verfasserin aut Korkmaz, Emine verfasserin aut Inozu, Mihriban verfasserin aut Soylemezoglu, Oguz verfasserin aut Candan, Cengiz verfasserin aut Bayazıt, Aysun Karabay verfasserin aut Elmacı, Ahmet Midhat verfasserin aut Parmaksiz, Gonul verfasserin aut Duzova, Ali verfasserin aut Besbas, Nesrin verfasserin aut Topaloglu, Rezan verfasserin aut Ozaltin, Fatih verfasserin aut Enthalten in Pediatric nephrology Berlin : Springer, 1987 32(2017), 8 vom: 24. März, Seite 1369-1375 (DE-627)254638872 (DE-600)1463004-7 1432-198X nnns volume:32 year:2017 number:8 day:24 month:03 pages:1369-1375 https://dx.doi.org/10.1007/s00467-017-3634-3 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.88 ASE 44.67 ASE AR 32 2017 8 24 03 1369-1375 |
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Enthalten in Pediatric nephrology 32(2017), 8 vom: 24. März, Seite 1369-1375 volume:32 year:2017 number:8 day:24 month:03 pages:1369-1375 |
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Adolescent Nephrotic syndrome Chronic kidney disease ADCK4 mutation CoQ10 supplementation |
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Pediatric nephrology |
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Atmaca, Mustafa @@aut@@ Gulhan, Bora @@aut@@ Korkmaz, Emine @@aut@@ Inozu, Mihriban @@aut@@ Soylemezoglu, Oguz @@aut@@ Candan, Cengiz @@aut@@ Bayazıt, Aysun Karabay @@aut@@ Elmacı, Ahmet Midhat @@aut@@ Parmaksiz, Gonul @@aut@@ Duzova, Ali @@aut@@ Besbas, Nesrin @@aut@@ Topaloglu, Rezan @@aut@@ Ozaltin, Fatih @@aut@@ |
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2017-03-24T00:00:00Z |
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We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. Methods 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. Results 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). Conclusions ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. 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|
| author |
Atmaca, Mustafa |
| spellingShingle |
Atmaca, Mustafa ddc 610 bkl 44.88 bkl 44.67 misc Adolescent misc Nephrotic syndrome misc Chronic kidney disease misc ADCK4 mutation misc CoQ10 supplementation Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment |
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Atmaca, Mustafa |
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electronic Article |
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610 - Medicine & health |
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keep |
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springer |
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Not Illustrated |
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1432-198X |
| topic_title |
610 ASE 44.88 bkl 44.67 bkl Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment Adolescent (dpeaa)DE-He213 Nephrotic syndrome (dpeaa)DE-He213 Chronic kidney disease (dpeaa)DE-He213 ADCK4 mutation (dpeaa)DE-He213 CoQ10 supplementation (dpeaa)DE-He213 |
| topic |
ddc 610 bkl 44.88 bkl 44.67 misc Adolescent misc Nephrotic syndrome misc Chronic kidney disease misc ADCK4 mutation misc CoQ10 supplementation |
| topic_unstemmed |
ddc 610 bkl 44.88 bkl 44.67 misc Adolescent misc Nephrotic syndrome misc Chronic kidney disease misc ADCK4 mutation misc CoQ10 supplementation |
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ddc 610 bkl 44.88 bkl 44.67 misc Adolescent misc Nephrotic syndrome misc Chronic kidney disease misc ADCK4 mutation misc CoQ10 supplementation |
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Pediatric nephrology |
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254638872 |
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610 - Medicine & health |
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Pediatric nephrology |
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(DE-627)254638872 (DE-600)1463004-7 |
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Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment |
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(DE-627)SPR006868029 (SPR)s00467-017-3634-3-e |
| title_full |
Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment |
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Atmaca, Mustafa |
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Pediatric nephrology |
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Pediatric nephrology |
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eng |
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600 - Technology |
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2017 |
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Atmaca, Mustafa Gulhan, Bora Korkmaz, Emine Inozu, Mihriban Soylemezoglu, Oguz Candan, Cengiz Bayazıt, Aysun Karabay Elmacı, Ahmet Midhat Parmaksiz, Gonul Duzova, Ali Besbas, Nesrin Topaloglu, Rezan Ozaltin, Fatih |
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32 |
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610 ASE 44.88 bkl 44.67 bkl |
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Elektronische Aufsätze |
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Atmaca, Mustafa |
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10.1007/s00467-017-3634-3 |
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610 |
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verfasserin |
| title_sort |
follow-up results of patients with adck4 mutations and the efficacy of coq10 treatment |
| title_auth |
Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment |
| abstract |
Background 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. Methods 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. Results 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} $, P=0.61). Conclusions ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria, and may thereby be renoprotective. |
| abstractGer |
Background ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. Methods A total of 146 index patients aged 10–18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation. Results Twenty-eight individuals with bi-allelic mutation from 11 families were identified. Median age at diagnosis was 12.4 (interquartile range [IQR] 8.04–19.7) years. Upon first admission, all patients had albuminuria and 18 had CKD (6 ESKD). Eight were diagnosed either through the screening of family members following index case identification or during genetic investigation of proteinuria in an individual with a history of a transplanted sibling. Median age of these 8 patients was 21.5 (range 4.4–39) years. CoQ10 supplementation was administered following genetic diagnosis. Median estimated glomerular filtration rate (eGFR) just before CoQ10 administration was 140 (IQR 117–155) ml/min/1.$ 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} $, P=0.61). Conclusions ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria, and may thereby be renoprotective. |
| abstract_unstemmed |
Background ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. Methods A total of 146 index patients aged 10–18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation. Results Twenty-eight individuals with bi-allelic mutation from 11 families were identified. Median age at diagnosis was 12.4 (interquartile range [IQR] 8.04–19.7) years. Upon first admission, all patients had albuminuria and 18 had CKD (6 ESKD). Eight were diagnosed either through the screening of family members following index case identification or during genetic investigation of proteinuria in an individual with a history of a transplanted sibling. Median age of these 8 patients was 21.5 (range 4.4–39) years. CoQ10 supplementation was administered following genetic diagnosis. Median estimated glomerular filtration rate (eGFR) just before CoQ10 administration was 140 (IQR 117–155) ml/min/1.$ 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). Conclusions 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. |
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Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment |
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https://dx.doi.org/10.1007/s00467-017-3634-3 |
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Gulhan, Bora Korkmaz, Emine Inozu, Mihriban Soylemezoglu, Oguz Candan, Cengiz Bayazıt, Aysun Karabay Elmacı, Ahmet Midhat Parmaksiz, Gonul Duzova, Ali Besbas, Nesrin Topaloglu, Rezan Ozaltin, Fatih |
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Gulhan, Bora Korkmaz, Emine Inozu, Mihriban Soylemezoglu, Oguz Candan, Cengiz Bayazıt, Aysun Karabay Elmacı, Ahmet Midhat Parmaksiz, Gonul Duzova, Ali Besbas, Nesrin Topaloglu, Rezan Ozaltin, Fatih |
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10.1007/s00467-017-3634-3 |
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2024-07-04T01:02:14.369Z |
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We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. Methods 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. Results 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. 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| score |
7.4007597 |