Wouda Eva Maria Nadine, Thielemans Laurence, Darakamon Mue Chae, Nge Aye Aye, Say Wah, Khing Sanda, Hanboonkunupakarn Borimas, Ngerseng Thatsanun, Landier Jordi, van Rheenen Patrick Ferry, Turner Claudia, Nosten Francois, McGready Rose, Carrara Verena Ilona
Shoklo Malaria Research Unit, Mahidol-Oxford Tropical Medicine Research Unit, Faculty of Tropical Medicine, Mahidol University, Mae Sot, Thailand.
University Medical Center, University of Groningen, Groningen, Netherlands.
BMJ Paediatr Open. 2020 May 28;4(1):e000641. doi: 10.1136/bmjpo-2020-000641. eCollection 2020.
To describe neonatal survival and long-term neurological outcome in neonatal hyperbilirubinaemia (NH) with extreme serum bilirubin (SBR) values.
Retrospective chart review, a one-off neurodevelopmental evaluation.
Special care baby unit in a refugee camp and clinics for migrant populations at the Thailand-Myanmar border with phototherapy facilities but limited access to exchange transfusion (ET).
Neonates ≥28 weeks of gestational age with extreme SBR values and/or acute neurological symptoms, neurodevelopment evaluation conducted at 23-97 months of age.
Neonatal mortality rate, prevalence of acute bilirubin encephalopathy (ABE) signs, prevalence of delayed development scores based on the Griffiths Mental Development Scale (GMDS).
From 2009 to 2014, 1946 neonates were diagnosed with jaundice; 129 (6.6%) had extreme SBR values during NH (extreme NH). In this group, the median peak SBR was 430 (IQR 371-487) µmol/L and the prevalence of ABE was 28.2%. Extreme NH-related mortality was 10.9% (14/129). Median percentile GMDS general score of 37 survivors of extreme NH was poor: 11 (2-42). 'Performance', 'practical reasoning' and 'hearing and language' domains were most affected. Four (10.8%) extreme NH survivors had normal development scores (≥50th centile). Two (5.4%) developed the most severe form of kernicterus spectrum disorders.
In this limited-resource setting, poor neonatal survival and neurodevelopmental outcomes, after extreme NH, were high. Early identification and adequate treatment of NH where ET is not readily available are key to minimising the risk of extreme SBR values or neurological symptoms.
描述血清胆红素（SBR）值极高的新生儿高胆红素血症（NH）的新生儿存活率和长期神经学转归。
回顾性病历审查，一次性神经发育评估。
泰国-缅甸边境一个难民营中的特殊护理婴儿病房以及为流动人口设立的诊所，具备光疗设施，但换血疗法（ET）的可及性有限。
胎龄≥28周、SBR值极高和/或有急性神经症状的新生儿，在23至97月龄时进行神经发育评估。
新生儿死亡率、急性胆红素脑病（ABE）体征的患病率、基于格里菲斯心理发育量表（GMDS）的发育迟缓评分的患病率。
2009年至2014年，1946例新生儿被诊断为黄疸；129例（6.6%）在NH期间SBR值极高（极高NH）。该组中，SBR峰值中位数为430（四分位间距371 - 487）µmol/L，ABE患病率为28.2%。极高NH相关死亡率为10.9%（14/129）。极高NH的37例幸存者GMDS总体评分中位数百分位数较差：11（2 - 42）。“操作”、“实际推理”和“听力与语言”领域受影响最大。4例（10.8%）极高NH幸存者发育评分正常（≥第50百分位数）。2例（5.4%）发生了最严重形式的核黄疸谱障碍。
在这种资源有限的环境中，极高NH后的新生儿存活率低和神经发育转归不良的情况很常见。在无法轻易获得ET的情况下，早期识别和充分治疗NH是将SBR值极高或神经症状风险降至最低的关键。
