Cystic fibrosis in asia

Shakil Ahmed; Gary Cheok; Anne E N Goh; Aye Han; SJ Hong; Wahyuni Indawati; A R M Lutful Kabir; SK Kabra; Harutai Kamalaporn; Hyung Young Kim; Shen Kunling; Sorasak Lochindarat; Mohammad Ashkan Moslehi; Anna Marie Nathan; Daniel Ng; Nguyen Ng The Phung; V Singh; Masato Takase; Rina Triasih; Zen-Kong Dai

doi:10.4103/prcm.prcm_5_20

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ORIGINAL ARTICLE

Year : 2020 | Volume : 4 | Issue : 1 | Page : 8-12

Cystic fibrosis in asia

Shakil Ahmed¹, Gary Cheok², Anne E N Goh³, Aye Han⁴, SJ Hong⁵, Wahyuni Indawati⁶, A R M Lutful Kabir⁷, SK Kabra⁸, Harutai Kamalaporn⁹, Hyung Young Kim¹⁰, Shen Kunling¹¹, Sorasak Lochindarat¹², Mohammad Ashkan Moslehi¹³, Anna Marie Nathan¹⁴, Daniel Ng¹⁵, Nguyen Ng The Phung¹⁶, V Singh¹⁷, Masato Takase¹⁸, Rina Triasih¹⁹, Zen-Kong Dai²⁰
¹ Department of Pediatrics, Shaheed Suhrawardy Medical College, Dhaka, Bangladesh, India
² Yee Wui Medical Centre, Macau SAR, China
³ Department of Paediatrics, KK Women's and Children's Hospital, Singapore
⁴ Department of Paediatrics, University of Medicine, Yangon, Myanmar
⁵ Department of Pediatrics, Childhood Asthma Atopy Center, Humidifier disinfectant Health Center, Asan Medical Center, University of Ulsan College of Medicine Seoul, Republic of Korea
⁶ Respirology Division, Child Health Department, Jakarta, Indonesia
⁷ Department of Pediatrics, Ad-din Women's Medical College, Dhaka, Bangladesh, India
⁸ Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110029, India
⁹ Department of Pediatrics, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
¹⁰ Department of Pediatrics, Pusan National University Yangsan Hospital, Pusan, Republic of Korea
¹¹ Department of Respiratory, Beijing Children's Hospital, Capital Medical University, Beijing, China
¹² Department of Pediatric Intensive Care and Respiratory Medicine, Queen Sirikit National Institute of Child Health Bangkok, Thailand
¹³ Pediatric Interventional Pulmonology Division, Department of Pediatric, Shiraz University of Medical Sciences, Shiraz, Iran
¹⁴ Department of Pediatrics, University Malaya, Kuala Lumpur, Malaysia
¹⁵ Department of Pediatrics, Hong Kong Sanatorium and Hospital, Hong Kong SAR, China
¹⁶ Department of Pediatrics, University of Medicine and Pharmacy at Ho Chi Minh City, Vietnam
¹⁷ Kalawati Saran Children Hospital, Lady Hardinge Medical College, New Delhi 110029, India
¹⁸ Department of Pediatrics, Nippon Medical School Tama-Nagayama Hospital, Tokyo, Japan
¹⁹ Department of Pediatrics, Dr. Sardjito Hospital, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia
²⁰ Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Taiwan

Correspondence Address:
S K Kabra
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi - 110 029
India

Source of Support: None, Conflict of Interest: None

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Read associated Erratum: Erratum: Cystic fibrosis in Asia with this article

DOI: 10.4103/prcm.prcm_5_20

Background: Cystic fibrosis (CF) is the most common inherited life-limiting illness in the Caucasian population. It is considered to be rare in Asia, but recent reports suggest that CF does occur in Asia. Methods: We carried out a questionnaire-based survey to determine the prevalence of CF across Asian countries and the diagnostic and therapeutic capabilities available in member countries. A questionnaire was sent to member countries of the Asian Pediatric Pulmonology Society. The questionnaire included the total number of children diagnosed with CF seen in the country and the available resources. available resources for diagnosis and management. Results: Fifteen countries responded to the questionnaire. Three countries/regions (Myanmar, Vietnam, and Macau) have not recorded any case of CF. The remaining 12 countries have recorded a variable number of cases which have also been reported in the literature. Sweat chloride testing facilities were available in all the countries except Taiwan that reported cases of CF. Some countries such as India and Bangladesh have developed their own indigenous method for sweat testing. Some countries have facilities for mutation testing. Basic medications such as pancreatic enzyme supplementation and antibiotics were available in all the countries where children with CF have been diagnosed. Inhaled antibiotics and dornase alfa were available only in a few countries. Some other countries reported using the injectable preparation of gentamicin and amikacin for inhalation therapy. Testing for genetic mutation wherever available showed a low frequency of the Delta F 508 mutation which is the most common mutation found in the Caucasian population. Only two countries (India and Japan) have formal CF associations for the affected community. Two countries Japan and China maintain a CF registry, whereas India already started the process of developing it. Conclusion: CF is increasingly being diagnosed over the past two decades in Asian countries. There is a need to create awareness among pediatricians and to develop regional or country-specific protocols and tools for the diagnosis and treatment of children with CF.

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