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Zebra bodies

MedGen UID:
569370
•Concept ID:
C0333776
•
Cell or Molecular Dysfunction
Synonym: 'Zebra' bodies
SNOMED CT: Zebra bodies (51529008)
 
HPO: HP:0031361

Definition

Intralysosomal, osmiophilic, lamellated and sometimes concentric cytoplasmic inclusions comprised of broad transversely-stacked myelinoid membranes and said to resemble a zebra in electron microscopic images. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVZebra bodies

Professional guidelines

PubMed

Fabry's disease--a comprehensive review on pathogenesis, diagnosis and treatment.
Mahmud HM
J Pak Med Assoc 2014 Feb;64(2):189-94. PMID: 24640811

Recent clinical studies

Etiology

Additional diagnostic value of electron microscopic examination in endomyocardial biopsy in patients with suspected non-ischemic cardiomyopathy.
Yamamoto M, Sato K, Murakoshi N, Yamada Y, Nakagawa D, Nakatsukasa T, Ishizu T, Ieda M
J Cardiol 2023 Feb;81(2):236-243. Epub 2022 Sep 28 doi: 10.1016/j.jjcc.2022.09.012. PMID: 36182004
Myeloid bodies is not an uncommon ultrastructural finding.
Choung HYG, Jean-Gilles J, Goldman B
Ultrastruct Pathol 2022 Jan 2;46(1):130-138. Epub 2022 Feb 1 doi: 10.1080/01913123.2021.2022054. PMID: 35100945
Myelin bodies in LMX1B-associated nephropathy: potential for misdiagnosis.
Lei L, Oh G, Sutherland S, Abra G, Higgins J, Sibley R, Troxell M, Kambham N
Pediatr Nephrol 2020 Sep;35(9):1647-1657. Epub 2020 Apr 30 doi: 10.1007/s00467-020-04564-w. PMID: 32356190
Clinical and pathological characteristics of Fabry disease combined with IgA nephropathy in Chinese patients
.
Yang N, Wang X, Xu F, Zeng C, Wang J, Liu Z
Clin Nephrol 2017 Apr;87 (2017)(4):188-195. doi: 10.5414/CN108986. PMID: 28157068
Ultrastructural pathology of skeletal muscle in systemic lupus erythematosus.
Finol HJ, Montagnani S, Márquez A, Montes de Oca I, Müller B
J Rheumatol 1990 Feb;17(2):210-9. PMID: 2319520

Diagnosis

Myeloid bodies is not an uncommon ultrastructural finding.
Choung HYG, Jean-Gilles J, Goldman B
Ultrastruct Pathol 2022 Jan 2;46(1):130-138. Epub 2022 Feb 1 doi: 10.1080/01913123.2021.2022054. PMID: 35100945
Zebra Bodies in the Kidney.
Huang X, Zhang R
N Engl J Med 2020 Jul 2;383(1):e2. doi: 10.1056/NEJMicm1912889. PMID: 32609984
Myelin bodies in LMX1B-associated nephropathy: potential for misdiagnosis.
Lei L, Oh G, Sutherland S, Abra G, Higgins J, Sibley R, Troxell M, Kambham N
Pediatr Nephrol 2020 Sep;35(9):1647-1657. Epub 2020 Apr 30 doi: 10.1007/s00467-020-04564-w. PMID: 32356190
Hydroxychloroquine-induced podocytopathy mimicking Fabry disease.
Serre J, Buob D, Boffa JJ
BMJ Case Rep 2019 May 13;12(5) doi: 10.1136/bcr-2018-228876. PMID: 31088818Free PMC Article
Fabry's disease--a comprehensive review on pathogenesis, diagnosis and treatment.
Mahmud HM
J Pak Med Assoc 2014 Feb;64(2):189-94. PMID: 24640811

Therapy

Kidney Podocyte Zebra Bodies after Lung Transplantation for Lymphangioleiomyomatosis.
Ishide T, Nishi H, Miyano S, Hirakawa Y, Honda K, Abe H, Sato M, Nangaku M
Intern Med 2023 Jul 1;62(13):1965-1970. Epub 2022 Nov 9 doi: 10.2169/internalmedicine.0882-22. PMID: 36351586Free PMC Article
Myeloid bodies is not an uncommon ultrastructural finding.
Choung HYG, Jean-Gilles J, Goldman B
Ultrastruct Pathol 2022 Jan 2;46(1):130-138. Epub 2022 Feb 1 doi: 10.1080/01913123.2021.2022054. PMID: 35100945
Hydroxychloroquine-induced podocytopathy mimicking Fabry disease.
Serre J, Buob D, Boffa JJ
BMJ Case Rep 2019 May 13;12(5) doi: 10.1136/bcr-2018-228876. PMID: 31088818Free PMC Article
Clinical and pathological characteristics of Fabry disease combined with IgA nephropathy in Chinese patients
.
Yang N, Wang X, Xu F, Zeng C, Wang J, Liu Z
Clin Nephrol 2017 Apr;87 (2017)(4):188-195. doi: 10.5414/CN108986. PMID: 28157068
Fabry's disease--a comprehensive review on pathogenesis, diagnosis and treatment.
Mahmud HM
J Pak Med Assoc 2014 Feb;64(2):189-94. PMID: 24640811

Prognosis

Zebra-like bodies in COVID-19: is phospholipidosis evidence of hydroxychloroquine induced acute kidney injury?
Obeidat M, Isaacson AL, Chen SJ, Ivanovic M, Holanda D
Ultrastruct Pathol 2020 Nov 20;44(4-6):519-523. Epub 2020 Dec 4 doi: 10.1080/01913123.2020.1850966. PMID: 33274661
Familial hypertrophic obstructive cardiomyopathy with the GLA E66Q mutation and zebra body.
Oikawa M, Sakamoto N, Kobayashi A, Suzuki S, Yoshihisa A, Yamaki T, Nakazato K, Suzuki H, Saitoh S, Kiko Y, Nakano H, Hayashi T, Kimura A, Takeishi Y
BMC Cardiovasc Disord 2016 May 10;16:83. doi: 10.1186/s12872-016-0262-y. PMID: 27160240Free PMC Article
Zebra body myopathy is caused by a mutation in the skeletal muscle actin gene (ACTA1).
Sewry CA, Holton JL, Dick DJ, Muntoni F, Hanna MG
Neuromuscul Disord 2015 May;25(5):388-91. Epub 2015 Feb 14 doi: 10.1016/j.nmd.2015.02.003. PMID: 25747004
Disruption of PPT2 in mice causes an unusual lysosomal storage disorder with neurovisceral features.
Gupta P, Soyombo AA, Shelton JM, Wilkofsky IG, Wisniewski KE, Richardson JA, Hofmann SL
Proc Natl Acad Sci U S A 2003 Oct 14;100(21):12325-30. Epub 2003 Oct 3 doi: 10.1073/pnas.2033229100. PMID: 14528005Free PMC Article
Polyneuropathy in progressive external ophthalmoplegia.
Gemignani F, Juvarra G, Marbini A, Calzetti S, Bragaglia MM, Govoni E
Eur Neurol 1982;21(3):181-8. doi: 10.1159/000115478. PMID: 6889507

Clinical prediction guides

Additional diagnostic value of electron microscopic examination in endomyocardial biopsy in patients with suspected non-ischemic cardiomyopathy.
Yamamoto M, Sato K, Murakoshi N, Yamada Y, Nakagawa D, Nakatsukasa T, Ishizu T, Ieda M
J Cardiol 2023 Feb;81(2):236-243. Epub 2022 Sep 28 doi: 10.1016/j.jjcc.2022.09.012. PMID: 36182004
A possible case of carbamazepine-induced renal phospholipidosis mimicking Fabry disease.
Chen J, Bai L, He Y
Clin Exp Nephrol 2022 Mar;26(3):303-304. Epub 2022 Jan 21 doi: 10.1007/s10157-021-02172-y. PMID: 35059891
A case of latent heterozygous Fabry disease in a female living kidney donor candidate.
Minami M, Mizuma E, Nakahara M, Oda Y, Yoshimine H, Tokunaga K, Mitsuke A, Yamada Y, Enokida H, Masutani K, Goto N, Ido A
CEN Case Rep 2021 Feb;10(1):30-34. Epub 2020 Jul 25 doi: 10.1007/s13730-020-00510-9. PMID: 32712909Free PMC Article
Clinical and pathological characteristics of Fabry disease combined with IgA nephropathy in Chinese patients
.
Yang N, Wang X, Xu F, Zeng C, Wang J, Liu Z
Clin Nephrol 2017 Apr;87 (2017)(4):188-195. doi: 10.5414/CN108986. PMID: 28157068
Extraocular muscles: light microscopy and ultrastructural features.
Martinez AJ, Hay S, McNeer KW
Acta Neuropathol 1976 Mar 30;34(3):237-53. doi: 10.1007/BF00688678. PMID: 178143

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