{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T19:45:50Z","timestamp":1790279150394,"version":"4.1.0"},"reference-count":25,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2020,10,28]],"date-time":"2020-10-28T00:00:00Z","timestamp":1603843200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["5 R01 HD072929 09 (SL)"],"award-info":[{"award-number":["5 R01 HD072929 09 (SL)"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of high pulmonary vascular resistance (PVR) commonly seen all over the world in the immediate newborn period. Several case reports from India have recently described severe pulmonary hypertension among infants in the postneonatal period. These cases typically present with respiratory distress in 1\u20136-month-old infants, breastfed by mothers on a polished rice-based diet. Predisposing factors include respiratory tract infection such as acute laryngotracheobronchitis with change in voice, leading to pulmonary hypertension, right atrial and ventricular dilation, pulmonary edema and hepatomegaly. Mortality is high without specific therapy. Respiratory support, pulmonary vasodilator therapy, inotropes, diuretics and thiamine infusion have improved the outcome of these infants. This review outlines four typical patients with thiamine-responsive acute pulmonary hypertension of early infancy (TRAPHEI) due to thiamine deficiency and discusses pathophysiology, clinical features, diagnostic criteria and therapeutic options.<\/jats:p>","DOI":"10.3390\/children7110199","type":"journal-article","created":{"date-parts":[[2020,10,28]],"date-time":"2020-10-28T11:43:06Z","timestamp":1603885386000},"page":"199","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Thiamine-Responsive Acute Pulmonary Hypertension of Early Infancy (TRAPHEI)\u2014A Case Series and Clinical Review"],"prefix":"10.3390","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4316-0517","authenticated-orcid":false,"given":"Nalinikanta","family":"Panigrahy","sequence":"first","affiliation":[{"name":"Rainbow Children\u2019s Hospitals, Hyderabad 500034, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinesh Kumar","family":"Chirla","sequence":"additional","affiliation":[{"name":"Rainbow Children\u2019s Hospitals, Hyderabad 500034, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rakshay","family":"Shetty","sequence":"additional","affiliation":[{"name":"Rainbow Children\u2019s Hospital, Bengaluru 560037, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Farhan A. R.","family":"Shaikh","sequence":"additional","affiliation":[{"name":"Rainbow Children\u2019s Hospitals, Hyderabad 500034, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Poddutoor Preetham","family":"Kumar","sequence":"additional","affiliation":[{"name":"Rainbow Children\u2019s Hospital, Vikrampuri, Hyderabad 500009, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajeshwari","family":"Madappa","sequence":"additional","affiliation":[{"name":"SIGMA Hospital, Mysore 570009, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anand","family":"Lingan","sequence":"additional","affiliation":[{"name":"Narayana Multispecialty Hospital, Mysore 570019, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6098-2155","authenticated-orcid":false,"given":"Satyan","family":"Lakshminrusimha","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, UC Davis Children\u2019s Hospital, Sacramento, CA 95817, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,10,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2037","DOI":"10.1161\/CIR.0000000000000329","article-title":"Pediatric pulmonary hypertension: Guidelines from the American Heart Association and the American Thoracic Society","volume":"132","author":"Abman","year":"2015","journal-title":"Circulation"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"154","DOI":"10.14712\/23362936.2014.17","article-title":"The Relationship between Vitamin D Deficiency and Pulmonary Hypertension","volume":"114","author":"Demir","year":"2013","journal-title":"Prague Med. Rep."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.nut.2015.08.007","article-title":"Thiamine responsive acute life threatening metabolic acidosis in exclusively breast-fed infants","volume":"32","author":"Qureshi","year":"2016","journal-title":"Nutrition"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"e1699","DOI":"10.1542\/peds.2012-3054","article-title":"Pulmonary Hypertension Associated with Scurvy and Vitamin Deficiencies in an Autistic Child","volume":"132","author":"Duvall","year":"2013","journal-title":"Pediatrics"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1111\/j.1651-2227.1992.tb12346.x","article-title":"Shoshin beriberi in an infant of a thiamine-deficient mother","volume":"81","author":"Debuse","year":"1992","journal-title":"Acta Paediatr."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1527","DOI":"10.1016\/j.jacc.2008.01.024","article-title":"Pulmonary arterial hypertension","volume":"51","author":"Chin","year":"2008","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Sastry, U.M.K., Jayranganath, M., Kumar, R.K., Ghosh, S., Bharath, A.P., Subramanian, A., Managuli, A., Gangadhara, M., and Manjunath, C.N. (2020). Thiamine-responsive acute severe pulmonary hypertension in exclusively breastfeeding infants: A prospective observational study. Arch. Dis. Child.","DOI":"10.1136\/archdischild-2019-318777"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1007\/s13312-019-1592-5","article-title":"Infantile Thiamine Deficiency: New Insights into an Old Disease","volume":"56","author":"Nazir","year":"2019","journal-title":"Indian Pediatr."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1016\/j.jpeds.2012.05.006","article-title":"Thiamine Deficiency in Cambodian Infants with and without Beriberi","volume":"161","author":"Coats","year":"2012","journal-title":"J. Pediatr."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"16","DOI":"10.3389\/fnut.2016.00016","article-title":"Thiamine Deficiency in Tropical Pediatrics: New Insights into a Neglected but Vital Metabolic Challenge","volume":"3","author":"Hiffler","year":"2016","journal-title":"Front. Nutr."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1038\/ejcn.2013.14","article-title":"Micronutrient deficiency and cognitive and physical performance in Indian children","volume":"67","author":"Swaminathan","year":"2013","journal-title":"Eur. J. Clin. Nutr."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Barennes, H., Sengkhamyong, K., Ren\u00e9, J.P., and Phimmasane, M. (2015). Beriberi (Thiamine Deficiency) and High Infant Mortality in Northern Laos. PLoS Negl. Trop. Dis., 9.","DOI":"10.1371\/journal.pntd.0003581"},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Soukaloun, D., Lee, S.J., Chamberlain, K., Taylor, A.M., Mayxay, M., Sisouk, K., Soumphonphakdy, B., Latsavong, K., Akkhavong, K., and Phommachanh, D. (2011). Erythrocyte Transketolase Activity, Markers of Cardiac Dysfunction and the Diagnosis of Infantile Beriberi. PLoS Negl. Trop. Dis., 5.","DOI":"10.1371\/journal.pntd.0000971"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1177\/0148607114565245","article-title":"Thiamin in Clinical Practice","volume":"39","author":"Frank","year":"2015","journal-title":"J. Parenter. Enter. Nutr."},{"key":"ref_15","first-page":"368","article-title":"Estimating dietary micronutrient supply and the prevalence of inadequate intakes from national Food Balance Sheets in the South Asia region","volume":"25","author":"Mark","year":"2016","journal-title":"Asia Pac. J. Clin. Nutr."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.ihj.2016.07.015","article-title":"Shoshin beriberi-thiamine responsive pulmonary hypertension in exclusively breastfed infants: A study from northern India","volume":"69","author":"Bhat","year":"2017","journal-title":"Indian Hear. J."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1093\/tropej\/fmp108","article-title":"Cardiac Beriberi: Often a Missed Diagnosis","volume":"56","author":"Rao","year":"2010","journal-title":"J. Trop. Pediatr."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"omaa041","DOI":"10.1093\/omcr\/omaa041","article-title":"Acute respiratory failure in an infant and thiamine deficiency in West Africa: A case report","volume":"2020","author":"Hiffler","year":"2020","journal-title":"Oxf. Med. Case Rep."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"H394","DOI":"10.1152\/ajpheart.00833.2006","article-title":"Abolition of reperfusion-induced arrhythmias in hearts from thiamine-deficient rats","volume":"293","author":"Oliveira","year":"2007","journal-title":"Am. J. Physiol. Circ. Physiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1136\/hrt.22.4.483","article-title":"Haemodynamic Studies in High Output Beri-Beri","volume":"22","author":"Blacket","year":"1960","journal-title":"Br. Heart J."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"S15","DOI":"10.1681\/ASN.V12suppl_1s15","article-title":"Lactic acidosis update for critical care clinicians","volume":"12","author":"Luft","year":"2001","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1007\/s12291-017-0690-1","article-title":"Thiamine Deficiency Induced Dietary Disparity Promotes Oxidative Stress and Neurodegeneration","volume":"33","author":"Chauhan","year":"2018","journal-title":"Indian J. Clin. Biochem."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2050","DOI":"10.18203\/2349-3291.ijcp20174729","article-title":"Idiopathic acute onset pulmonary artery hypertension in infancy need for research","volume":"4","author":"Sangeeta","year":"2017","journal-title":"Int. J. Contemp. Pediatr."},{"key":"ref_24","first-page":"187","article-title":"Mahendrappa and Nandakishore Reddy. Association of severe acute lower respiratory tract infections with pulmonary hypertension in infants aged 2 months to 1 year, a prospective study","volume":"11","author":"Kotyal","year":"2019","journal-title":"Int. J. Curr. Res."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"e0010","DOI":"10.1097\/MD.0000000000010010","article-title":"Wet beriberi with multiple organ failure remarkably reversed by thiamine administration","volume":"97","author":"Lei","year":"2018","journal-title":"Medicine"}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/7\/11\/199\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:29:37Z","timestamp":1760178577000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/7\/11\/199"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,28]]},"references-count":25,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2020,11]]}},"alternative-id":["children7110199"],"URL":"https:\/\/doi.org\/10.3390\/children7110199","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,28]]}}}