Nursing Care of an Extremely Preterm Infant with Sodium Taurocholate Cotransporting Polypeptide Deficiency: A Case Report
American Journal of Nursing Science
Volume 9, Issue 4, August 2020, Pages: 257-262
Received: Jun. 17, 2020;
Accepted: Jul. 7, 2020;
Published: Jul. 17, 2020
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Ni Zhang, Department of Neonatal, The First Affiliated Hospital of Jinan University, Guangzhou, China
Yuanzong Song, Department of Neonatal, The First Affiliated Hospital of Jinan University, Guangzhou, China
Weiju Chen, Department of Neonatal, The First Affiliated Hospital of Jinan University, Guangzhou, China
Meixue Chen, Department of Neonatal, The First Affiliated Hospital of Jinan University, Guangzhou, China
Lingli Cai, Department of Neonatal, The First Affiliated Hospital of Jinan University, Guangzhou, China
Lijing Deng, Department of Neonatal, The First Affiliated Hospital of Jinan University, Guangzhou, China
Lilan He, Department of Neonatal, The First Affiliated Hospital of Jinan University, Guangzhou, China
Qingran Lin, Department of Neonatal, The First Affiliated Hospital of Jinan University, Guangzhou, China; Department of Nursing, The First Affiliated Hospital of Jinan University, Guangzhou, China
Sodium taurocholate cotransporting polypeptide (NTCP) is a protein encoded by the solute carrier family 10 member 1 gene and expressed in the basolateral membrane of the hepatocyte to uptake conjugated bile acids from the plasma. This study reported an extremely premature infant (EPI) with NTCP deficiency (NTCPD). The patient presented with jaundice, persistently elevated total bile acids, 25-(OH)-VitD deficiency, and cleft palate. Nursing care mainly focused on disease surveillance, jaundice care, nutrition, kangaroo mother care, and psychological care. In response to 5 months of the nursing care, the infant’s weight reached 3.324 kg, the jaundice was alleviated, and the infant gained the ability to suck milk. However, his TBA levels were abnormally elevated, and 25-(OH)-VitD was still deficient when the patient was discharged from hospital. The nurses taught the patient’s parents the importance of monitoring liver function and trace elements during a routine outpatient visit. Telephone follow-up 1 year later showed that the patient was in good health with no obvious clinical manifestations. This article reports our experiences in caring for an EPI with NTCPD. To the best of our knowledge, this is the first case report worldwide in NTCPD nursing care.
Nursing Care of an Extremely Preterm Infant with Sodium Taurocholate Cotransporting Polypeptide Deficiency: A Case Report, American Journal of Nursing Science.
Vol. 9, No. 4,
2020, pp. 257-262.
Pan W, Song IS, Shin HJ, et al. Genetic polymorphisms in Na+-taurocholate co-transporting polypeptide (NTCP) and ileal apical sodium-dependent bile acid transporter (ASBT) and ethnic comparisons of functional variants of NTCP among Asian populations. Xenobiotica. 2011; 41 (6): 501-510.
Anwer MS, Stieger B. Sodium-dependent bile salt transporters of the SLC10A transporter family: more than solute transporters. Pflugers Arch. 2014; 466 (1): 77-89.
Tan HJ, Deng M, Qiu JW, Wu JF, Song YZ. Monozygotic Twins Suffering From Sodium Taurocholate Cotransporting Polypeptide Deficiency: A Case Report. Front Pediatr. 2018; 6: 354.
Vaz FM, Paulusma CC, Huidekoper H, et al. Sodium taurocholate cotransporting polypeptide (SLC10A1) deficiency: conjugated hypercholanemia without a clear clinical phenotype. Hepatology. 2015; 61 (1): 260-267.
Lin H, Qiu JW, Rauf YM, et al. Sodium Taurocholate Cotransporting Polypeptide (NTCP) Deficiency Hidden Behind Citrin Deficiency in Early Infancy: A Report of Three Cases. Front Genet. 2019; 10: 1108. Published 2019 Nov 7.
Dong C, Zhang BP, Wang H, et al. Clinical and histopathologic features of sodium taurocholate cotransporting polypeptide deficiency in pediatric patients. Medicine (Baltimore). 2019; 98 (39): e17305.
Chen R, Deng M, Rauf YM, et al. Intrahepatic Cholestasis of Pregnancy as a Clinical Manifestation of Sodium-Taurocholate Cotransporting Polypeptide Deficiency. Tohoku J Exp Med. 2019; 248 (1): 57-61.
Li H, Deng M, Guo L, et al. Clinical and molecular characterization of four patients with NTCP deficiency from two unrelated families harboring the novel SLC10A1 variant c. 595A>C (p. Ser199Arg). Mol Med Rep. 2019; 20 (6): 4915-4924.
Li, H., et al., [Clinical and genetic analysis of a pediatric patient with sodium taurocholate cotransporting polypeptide deficiency]. Zhongguo Dang Dai Er Ke Za Zhi, 2018. 20 (4): p. 279-284.
Qiu JW, Deng M, Cheng Y, et al. Sodium taurocholate cotransporting polypeptide (NTCP) deficiency: Identification of a novel SLC10A1 mutation in two unrelated infants presenting with neonatal indirect hyperbilirubinemia and remarkable hypercholanemia. Oncotarget. 2017; 8 (63): 106598-106607.
Liu R, Chen C, Xia X, et al. Homozygous p. Ser267Phe in SLC10A1 is associated with a new type of hypercholanemia and implications for personalized medicine. Sci Rep. 2017; 7 (1): 9214. Published 2017 Aug 23.
Song, Y. Z. and M. Deng, [Sodium taurocholate cotransporting polypeptide deficiency manifesting as cholestatic jaundice in early infancy: a complicated case study]. Zhongguo Dang Dai Er Ke Za Zhi, 2017. 19 (3): p. 350-354.
Van Herpe F, Waterham HR, Adams CJ, et al. NTCP deficiency and persistently raised bile salts: an adult case. J Inherit Metab Dis. 2017; 40 (3): 313-315.
Deng M, Mao M, Guo L, Chen FP, Wen WR, Song YZ. Clinical and molecular study of a pediatric patient with sodium taurocholate cotransporting polypeptide deficiency. Exp Ther Med. 2016; 12 (5): 3294-3300.
Glass HC, Costarino AT, Stayer SA, Brett CM, Cladis F, Davis PJ. Outcomes for extremely premature infants. Anesth Analg. 2015; 120 (6): 1337-1351.
Vincent JL, Einav S, Pearse R, et al. Improving detection of patient deterioration in the general hospital ward environment. Eur J Anaesthesiol. 2018; 35 (5): 325-333.
Slijepcevic D, Kaufman C, Wichers CG, et al. Impaired uptake of conjugated bile acids and hepatitis b virus pres1-binding in na (+) -taurocholate cotransporting polypeptide knockout mice. Hepatology. 2015; 62 (1): 207-219.
Donnellan D, Moore Z, Patton D, O'Connor T, Nugent L. The effect of thermoregulation quality improvement initiatives on the admission temperature of premature/very low birth-weight infants in neonatal intensive care units: A systematic review. J Spec Pediatr Nurs. 2020; 25 (2): e12286.
Wyckoff MH, Aziz K, Escobedo MB, et al. Part 13: Neonatal Resuscitation: 2015 American Heart Association Guidelines Update for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation. 2015; 132 (18 Suppl 2): S543-S560.
Pols TW, Noriega LG, Nomura M, Auwerx J, Schoonjans K. The bile acid membrane receptor TGR5 as an emerging target in metabolism and inflammation. J Hepatol. 2011; 54 (6): 1263-1272.
Amstalden-Mendes LG, Magna LA, Gil-da-Silva-Lopes VL. Neonatal care of infants with cleft lip and/or palate: feeding orientation and evolution of weight gain in a nonspecialized Brazilian hospital. Cleft Palate Craniofac J. 2007; 44 (3): 329-334.
Lewis CW, Jacob LS, Lehmann CU; SECTION ON ORAL HEALTH. The Primary Care Pediatrician and the Care of Children With Cleft Lip and/or Cleft Palate [published correction appears in Pediatrics. 2017 Sep; 140 (3): null]. Pediatrics. 2017; 139 (5): e20170628.
Bisanalli S, Nesargi S, Govindu RM, Rao SP. Kangaroo Mother Care in Hospitalized Low Birth-Weight Infants on Respiratory Support: A Feasibility and Safety Study. Adv Neonatal Care. 2019; 19 (6): E21-E25.
Boundy EO, Dastjerdi R, Spiegelman D, et al. Kangaroo Mother Care and Neonatal Outcomes: A Meta-analysis. Pediatrics. 2016; 137 (1): e20152238.
Andres V, Garcia P, Rimet Y, Nicaise C, Simeoni U. Apparent life-threatening events in presumably healthy newborns during early skin-to-skin contact. Pediatrics. 2011; 127 (4): e1073-e1076.