A child with Polyglandular autoimmune syndrome Type-I and immune thrombocytopenic purpura Mohamad Pedram1, Korush Riahi2, Kaveh Jaseb3, Mohammad Hasan Alemzadeh Ansari4, Mohammad Javad Alemzadeh Ansari5 ABSTRACT
Polyglandular autoimmune syndrome type I (PGA I) is a rare disease. Its hallmarks are chronic
mucocutaneous candidasis, hypoparathyroidism and adrenal insufficiency. Immune
thrombocytopenic purpura (ITP) is one of the most common autoimmune disease in children.
Association of PGA I with ITP was not found in some previous studies, but, we report a child
with PGA I and ITP.
KEY WORDS: ITP, Polyglandular autoimmune syndrome.
Pak J Med Sci January - March 2011 Vol. 27 No. 1 223-225
How to cite this article:
Pedram M, Riahi K, Jaseb K, Alemzadeh-Ansari MH, Alemzadeh-Ansari MJ. A child with
Polyglandular autoimmune syndrome Type-I and immune thrombocytopenic purpura. Pak J Med
Sci 2011;27(1):223-225
Mohamad Pedram,Professor Pediatric Oncologist, Polyglandular autoimmune syndrome type I (PGA Research Center of Thalassemia & Hemoglobinopathy, I) is a monogenic autosomal disease with a recessive inheritance pattern. It is characterized by a widely Jundishapour University of Medical Sciences,Ahvaz, Iran.
variable combination of autoimmune destruction of tissues (predominantly endocrine glands), chronic superficial candidiasis, and ectodermal dystrophy complex including hypoplasia of the dental enamel, Pediatric Hematology/Oncology Fellowship, alopecia and nail dystrophy.1,2 Although, immune thrombocytopenic purpura (ITP) had been reported Mohammad Hasan Alemzadeh Ansari,Medical Student, in PGA II and III,3 but in previous studies we did not find any article reporting PGA I with ITP. Here, we report a 5 years old boy who presented with a Resident Cardiology, Tehran Heart Center Hospital,Tehran University of Medical Sciences, Tehran - Iran.
1-4: Jundishapour University of Medical Sciences, CASE REPORT
A five years old boy was admitted to our hospital with a four months history of multiple ecchymosis on the lower limbs. This ecchymosis occurred after Research Center of Thalassemia & Hemoglobinopathy, mild injuries. On physical examination he had alope- Shafa Hospital,Jundishapour University of Medical Sciences, cia of head and neck and chronic mucocutaneous candidiasis. (Figure-1,2) Other physical examination Routin laboratory examination revealed platelets 7 4000 /mm3 (normal range 150 000-450 000 /mm3), Partial thromboplastin time 41 sec (normal range 30-45 sec), Prothrombin time 13.7 sec (normal range Pak J Med Sci 2011 Vol. 27 No. 1 www.pjms.com.pk 223
11-13 sec), and international normalized ratio 1.4.
Other examination indicated serum calcium 5 mg/ dl (normal range 8.5-10.5 mg/dl), phosphate 12 mg/dl (normal range 3-6 mg/dl), serum albumin 5 develops or Addison’s disease is recognized in a (normal range 4-6 g/dl). Serum cratinin, protein and young child. The syndrome is rare but has an electrolytes was normal. Parathormone hormone was increased prevalence in certain populations (e.g., 3.1 pg/dl (normal range 15-65 pg/dl). T3, TSH, T4, inhabitants of Finland5 and Sardinia and Iranian FTI and T3RU were in normal range but anti-TPO Jews).6 Mutations in an autoimmune-suppressor was 167.8 Iu/dl (normal range <34 Iu/dl). Serology gene (AIRE, for autoimmune regulator), which en- examinations was negative for anti-nuclear codes a transcription factor, cause the syndrome.
Persons with any two of several specific conditions - Electrocardiogram indicated high corrected QT mucocutaneous candidiasis, hypoparathyroidism, interval (0.52 sec). He was diagnosed to have PGA I and Addison’s disease- almost always have AIRE based on presence of hypoparathyroidism and chronic muccocutaneous candidiasis. For approach After diagnosis, patients with autoimmune to thrombocytopenia, bone marrow aspiration was polyendocrine syndrome type I require close moni- done and its finding was compatible with ITP. After toring. Monitoring can help prevent illness associ- diagnosis of ITP, he was treated by Methylpredniso- ated with delayed diagnosis of additional autoim- lone 350 mg for five days. After five days the mune diseases such as Addison’s disease and hypo- platelets reached to normal range (375 000 /mm3).
parathyroidism, as well as oral cancer, which may DISCUSSION
develop if candidiasis is not treated aggressively, and infection due to asplenism, which is present in a sub- PGA I has been described under other names, such group of patients.9 Our patient was diagnosed PGA as Whitaker’s syndrome, polyglandular autoimmune I because this manifestations: hypoparathyroid, disease type 1, or autoimmune polyendocrinopathy, Chronic mucocutaneous candidiasis generally PGA I is an autoimmune syndrome with charac- presents earliest in life and is the most frequent of teristic disease associations that often appear early the three main diseases of PGA I. It can appear as in life, typically in infants with persistent chronic early as at the first month after birth up to 21 years mucocutaneous candidiasis without the systemic in- of age, with a peak of occurrence in early childhood.
fection generally associated with severe immunode- Chronic mucocutaneous candidiasis is present in polyendocrine syndrome type I is usually made later, Chronic hypoparathyroidism is the first endocrine when hypocalcemia due to hypoparathyroidism disease to occur during the time course of PGA I, 224 Pak J Med Sci 2011 Vol. 27 No. 1 www.pjms.com.pk
usually after chronic mucocutaneous candidiasis and 2. Neufeld M, Maclaren NK, Blizzard RM. Two types of before Addison’s disease, and can present between autoimmune Addison’s disease associated with different polyglandular autoimmune (PGA) syndromes. Medicine three months to 44 years of age (mean, 7.5 yr).
Chronic hypoparathyroidism has been reported in 3. Noriko O, Junko T, Yuko Y, Toshiaki K, Masteru N, Tooru Y, et al. Autoimmune Polyglandular Syndrome Type III Associated with Slowly Progressive Type 1 Diabetes Melli- tus, Chronic Thyroiditis, Pernicious Anemia and Idiopathic disease in children. Acute ITP occurs in 90% of the Thrombocytopenic Purpura: A Case Report. J Japan Diabetic children. It is generally seen in children from the ages of one to nine years, though the peak manifestation 4. Betterle C, Greggio NA, Volpato M. Clinical review 93: Autoimmune Polyglandular Syndrome Type 1. JCE M is between two to five years of age. The diagnosis of ITP was demonstrated by bone marrow aspiration.
5. Perheentupa J. APS-I/APECED: The clinical disease A treatment option is corticosteroidsý. Because our and therapy. Endocrinol Metab Clin North Am patient had multiple echemosis and thrombocytope- 6. Zlotogora J, Shapiro M.S. Polyglandular autoimmune syn- nia, bone marrow aspiration was done. The result of drome type I among Iranian Jews. J Med Genet bone marrow aspiration confirmed the ITP. After 7. Eisenbarth GS, Gottlieb PA. Autoimmune Polyendocrine treatment with Methylprednisolone 350 mg, the Syndromes. N Engl J Med 2004;350:2068-2079.
platelets increased to normal range.
8. Barker JM, Eisenbarth GS. Autoimmune polyendocrine syn- dromes. In: Eisenbarth GS, ed. Type 1 diabetes: Molecular, REFERENCES
cellular, and clinical immunology. Denver: Barbara Davis Center for Childhood Diabetes, 2003 (Web only). (Accessed 1. Ahonen P, Myllarniemi S, Sipila I, Perheentupa J. Clinical April 20, 2004, at http://www.barbaradaviscenter.org.) variation of autoimmune polyendocrinopathy-candidiasis- 9. Nugent DJ. Childhood immune thrombocytopenic purpura.
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