Literature DB >> 34911613

Cystinosis induced by CTNS gene mutation: a rare disease study.

Xin Wang1, Bi-Li Zhang1, Xiao-Ying Chen1, Zhen Guo1.   

Abstract

A boy, aged 1 year and 6 months, was found to have persistent positive urine glucose at the age of 4 months, with polydipsia, polyuria, and growth retardation. Laboratory examinations suggested that the boy had low specific weight urine, anemia, hypokalemia, hyponatremia, hypomagnesemia, metabolic acidosis, glycosuria, acidaminuria, increased fractional excretion of potassium, and decreased tubular reabsorption of phosphate. X-ray examinations of the head, thorax, and right hand showed changes of renal rickets. The slit-lamp examination showed a large number of cystine crystals in the cornea. The genetic testing showed a suspected pathogenic homozygous mutation of the CTNS gene, C.922g>A(p.Gly308Arg). The boy was finally diagnosed with cystinosis. At the beginning of treatment, symptomatic supportive treatment was given to maintain the stability of the internal environment, and cysteamine tartaric acid capsules were used after diagnosis to remove cysteine. This article reported a case of cystinosis caused by CTNS gene mutation and summarized the etiology, clinical features, diagnosis, and treatment of this disease, which can provide a reference for the early diagnosis, treatment, and subsequent study of the disease.

Entities:  

Keywords:  CTNS gene; Child; Cystinosis

Mesh:

Substances:

Year:  2021        PMID: 34911613      PMCID: PMC8690714          DOI: 10.7499/j.issn.1008-8830.2109042

Source DB:  PubMed          Journal:  Zhongguo Dang Dai Er Ke Za Zhi        ISSN: 1008-8830


  17 in total

1.  The slow progressive nature of duodenal neuroendocrine tumor: a case report of long-term observation over 14 years.

Authors:  Kiichi Takahashi; Waku Hatta; Tomoyuki Koike; Takeshi Kanno; Nobuyuki Ara; Kiyotaka Asanuma; Naoki Asano; Akira Imatani; Fumiyoshi Fujishima; Hironobu Sasano; Tooru Shimosegawa
Journal:  Clin J Gastroenterol       Date:  2017-06-29

2.  Clinical utility gene card for: Dent disease (Dent-1 and Dent-2).

Authors:  Michael Ludwig; Elena Levtchenko; Arend Bökenkamp
Journal:  Eur J Hum Genet       Date:  2014-03-12       Impact factor: 4.246

3.  A mouse model suggests two mechanisms for thyroid alterations in infantile cystinosis: decreased thyroglobulin synthesis due to endoplasmic reticulum stress/unfolded protein response and impaired lysosomal processing.

Authors:  H P Gaide Chevronnay; V Janssens; P Van Der Smissen; X H Liao; Y Abid; N Nevo; C Antignac; S Refetoff; S Cherqui; C E Pierreux; P J Courtoy
Journal:  Endocrinology       Date:  2015-03-26       Impact factor: 4.736

Review 4.  Nephropathic cystinosis: an update.

Authors:  Koenraad R Veys; Mohamed A Elmonem; Fanny O Arcolino; Lambertus van den Heuvel; Elena Levtchenko
Journal:  Curr Opin Pediatr       Date:  2017-04       Impact factor: 2.856

Review 5.  Fanconi Syndrome.

Authors:  John W Foreman
Journal:  Pediatr Clin North Am       Date:  2019-02       Impact factor: 3.278

6.  CTNS mutations in an American-based population of cystinosis patients.

Authors:  V Shotelersuk; D Larson; Y Anikster; G McDowell; R Lemons; I Bernardini; J Guo; J Thoene; W A Gahl
Journal:  Am J Hum Genet       Date:  1998-11       Impact factor: 11.025

7.  Nephropathic cystinosis: an international consensus document.

Authors:  Francesco Emma; Galina Nesterova; Craig Langman; Antoine Labbé; Stephanie Cherqui; Paul Goodyer; Mirian C Janssen; Marcella Greco; Rezan Topaloglu; Ewa Elenberg; Ranjan Dohil; Doris Trauner; Corinne Antignac; Pierre Cochat; Frederick Kaskel; Aude Servais; Elke Wühl; Patrick Niaudet; William Van't Hoff; William Gahl; Elena Levtchenko
Journal:  Nephrol Dial Transplant       Date:  2014-09       Impact factor: 5.992

8.  Molecular pathogenesis of cystinosis: effect of CTNS mutations on the transport activity and subcellular localization of cystinosin.

Authors:  Vasiliki Kalatzis; Nathalie Nevo; Stéphanie Cherqui; Bruno Gasnier; Corinne Antignac
Journal:  Hum Mol Genet       Date:  2004-05-05       Impact factor: 6.150

9.  First report of CTNS mutations in a Chinese family with infantile cystinosis.

Authors:  Yong-jia Yang; Yuan Hu; Rui Zhao; Xinyu He; Liu Zhao; Ming Tu; Lijun Zhou; Jihong Guo; Linqian Wu; Tantai Zhao; Yi-min Zhu
Journal:  ScientificWorldJournal       Date:  2015-03-17

Review 10.  Cystinosis: a review.

Authors:  Mohamed A Elmonem; Koenraad R Veys; Neveen A Soliman; Maria van Dyck; Lambertus P van den Heuvel; Elena Levtchenko
Journal:  Orphanet J Rare Dis       Date:  2016-04-22       Impact factor: 4.123

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