Author(s): Muneera Fadhil Ridha, Munib Ahmed Al zubaidi

Email(s): muneerafadhil@comed.uobaghdad.edu.iq

DOI: 10.52711/0974-360X.2022.00977   

Address: Muneera Fadhil Ridha1, Munib Ahmed Al zubaidi2
1Pediatric Endocrinology, Department of Pediatrics, College of Medicine, University of Baghdad, Baghdad, Iraq.
2Assistant Professor, Pediatric Endocrinology, Department of Pediatrics, College of Medicine, University of Baghdad, Baghdad, Iraq.
*Corresponding Author

Published In:   Volume - 15,      Issue - 12,     Year - 2022


ABSTRACT:
Background: Autoimmune polyendocrinopathy-candidiasisectodermal dystrophy is a rare disease with heterogenous presentation, its diagnosis and management make a challenge in our medical practice. Aim: To describe the variability of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) presentation and management in a group of pediatric Iraqi patients and define some measures for promoting recognition and adequate management of patients. Patients and Methods: We included 27 patients (24 families) with APECED (median age at diagnosis was 7 years; range 2 years – 12 years) 11 males and 16 Females, their medical records were reviewed and then followed up for a period from 2001 and 2021. The diagnosis of the disease was made by the presence of at least two major clinical features, prospectively Patients were scheduled for followed up visits every 6–12 months in our hospital. Full evaluation including monitoring for the presence of the new disease manifestations and treatment of every component was conducted semiannually. Results: The percentages of females more than the male. More patients have a negative family history and positive consanguinity. Patients with hypocalcemic fits and Addison crisis are the largest percentage of patients (59.3%, 18.5% consequently). The difference in median age and interquartile range between the three main features of disease was compared and were significant, p-value = 0.02. The mortality rate was 11.1% and 57% of patients reported difficulty in compliance with the follow-up program. Conclusion: The disease although it is rare and had very important major and minor autoimmune manifestations that can be life-threatening if it is not well promptly diagnosed and treated. Evidently many measures can be taken in order to improve its diagnosis and management.


Cite this article:
Muneera Fadhil Ridha, Munib Ahmed Al zubaidi.Challenges with Management of Autoimmune Polyendocrinopathy-Candidiasis-ectodermal dystrophy in Group of Iraqi patients. Research Journal of Pharmacy and Technology 2022; 15(12):5789-2. doi: 10.52711/0974-360X.2022.00977

Cite(Electronic):
Muneera Fadhil Ridha, Munib Ahmed Al zubaidi.Challenges with Management of Autoimmune Polyendocrinopathy-Candidiasis-ectodermal dystrophy in Group of Iraqi patients. Research Journal of Pharmacy and Technology 2022; 15(12):5789-2. doi: 10.52711/0974-360X.2022.00977   Available on: https://rjptonline.org/AbstractView.aspx?PID=2022-15-12-71


REFERENCES:
1.    Kahaly GJ. Polyglandular autoimmune syndromes. European Journal of Endocrinology. 2009 Jul 1; 161(1):11-20. https://doi.org/10.1530/EJE-09-0044
2.    Perheentupa J. Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy. The Journal of Clinical Endocrinology and Metabolism. 2006 Aug 1; 91(8):2843-50. https://doi.org/10.1210/jc.2005-2611
3.    Meloni A, et al. Autoimmune polyendocrine syndrome type 1: an extensive longitudinal study in Sardinian patients. The Journal of Clinical Endocrinology and Metabolism. 2012 Apr 1; 97(4):1114-24. https://doi.org/10.1210/jc.2011-2461
4.    Ahonen P, et al. Clinical variation of autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED) in a series of 68 patients. New England Journal of Medicine. 1990 Jun 28; 322(26):1829-36. DOI: 10.1056/NEJM199006283222601
5.    V. Sangamithra, Ranbir Verma, S. Sengottuvelu, R. Sumathi. Candida Infections of the Genitourinary Tract. Research J. Pharm. and Tech. 6(10): October 2013; Page 1111-1115.
6.    Better C, Greggio NA, Volpato M. Autoimmune polyglandular syndrome type 1. The Journal of Clinical Endocrinology and Metabolism. 1998 Apr 1; 83(4):1049-55.https://doi.org/10.1210/jcem.83.4.4682
7.    A. Porselvi. A Review on the Need of Advanced Clinical Pharmacy Education Services for Diabetes Prevention and Management in India in comparison with International Standards. Research J. Pharm. and Tech. 2021; 14(1):493-500. doi: 10.5958/0974-360X.2021.00090.1
8.    Aseem Kumar, Anil Kumar Sharma, Rohit Dutt. A Review of Gastro-retentive Drug Delivery Systems for Antidiabetics and its present status. Research J. Pharm. and Tech. 2021; 14(1):538-546. doi: 10.5958/0974-360X.2021.00098.6
9.    Buzi F, etal. Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome: time to review diagnostic criteria?. The Journal of Clinical Endocrinology & Metabolism. 2003 Jul 1; 88(7):3146-8.  https://doi.org/10.1210/jc.2002-021495
10.    Boe, etal. Mutational analysis of the autoimmune regulator (AIRE) gene in sporadic autoimmune Addison's disease can reveal patients with unidentified autoimmune polyendocrine syndrome type I. European journal of endocrinology, 146(4), pp.519-522.
11.    JaakkoPerheentupa; EXTENSIVE CLINICAL EXPERIENCE Autoimmune Polyendocrinopathy-Candidiasis- Ectodermal Dystrophy, The Journal of Clinical Endocrinology and Metabolism 91(8):2843–2850 Printed in the U.S.A. Copyright © 2006 by The Endocrine Societydoi: 10.1210/jc.2005-2611
12.    Samrithi Yuvaraj, Gheena. S, Gopinath. P. Comparison of Oral Candidal Flora of Diabetics and Non Diabetics. Research J. Pharm. and Tech 2016; 9(10):1645-1646.
13.    Donatella Capalbo, Lucia De Martino, GiulianaGiardino, Raffaella Di Mase, Iolanda Di Donato, Giancarlo Parenti, PietroVajro, Claudio Pignata, Mariacarolina Salerno, "Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy: Insights into Genotype-Phenotype Correlation", International Journal of Endocrinology, vol. 2012, Article ID 353250, 9 pages, 2012. https://doi.org/10.1155/2012/353250
14.    Halonen M, et al. Partanen J 2002 AIRE mutations and human leukocyte antigen genotypes as determinants of the autoimmune polyendocrinopathy- candidiasis-ectodermal dystrophy phenotype. J ClinEndocrinolMetab 87:2568–2574.  https://doi.org/10.1210/jcem.87.6.8564
15.    Sonal C, et al. Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy. The Journal of clinical and aesthetic dermatology. 2012 Dec; 5(12):18. PMC3533323
16.    Perheentupa J. APS-I/APECED: the clinical disease and therapy. Endocrinology and Metabolism Clinics. 2002 Jun 1;31(2):295-320. https://doi.org/10.1016/S0889-8529(01)00013-5.
17.    Anandkumar, Jessly Chacko, Theertha C. K, M. Usha. Thyroid Disorder: An Overview. Res. J. Pharmacology and Pharmacodynamics.2020; 12(1):01-04.
18.    Perheentupa J. APS-I/APECED: the clinical disease and therapy. Endocrinology and Metabolism Clinics. 2002 Jun 1; 31(2):295-320.
19.    Pramod Shinde, V. R. Salunkhe, C. S. Magdum. Formulation and evaluation of novel bucco-adhesive formulation containing Clotrimazole. Research J. Pharm. and Tech. 5(7): July 2012; Page 921-926.
20.    Rautemaa R, et al. Oral and oesophageal squamous cell carcinoma–a complication or component of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED, APS-I). Oral oncology. 2007 Jul 1;43(6):607-13. https://doi.org/10.1016/j.oraloncology.2006.07.005.
21.    Wolff, et al. Autoimmune polyendocrine syndrome type 1 in Norway: phenotypic variation, autoantibodies, and novel mutations in the autoimmune regulator gene. The Journal of Clinical Endocrinology and Metabolism, 92(2), pp.595-603. (2007).  https://doi.org/10.1210/jc.2006-1873.
22.    Rana I. Ahmad, Haider A. Al-Barry. Neonatal Screening for Congenital Hypothyroidism at Obstetrics and Pediatrics Hospital in Lattakia, Syria. Research J. Pharm. and Tech 2020; 13(6): 2749-2751.
23.    Hazaratali Panari, Vegunarani.M. Study on Complications of Diabetes Mellitus among the Diabetic Patients. Asian J. Nur. Edu. and Research. 2016; 6(2): 171-182.
24.    Borchers J, Mäkitie O, Laakso S. Infections and demanding endocrine care contribute to increased mortality in patients with APECED. European journal of endocrinology. 2021 Nov 1; 185(5):K15-9. https://doi.org/10.1530/EJE-21-0241

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