Saikia Bedangshu, Vashisht Neetu, Gupta Neeraj, Sharma Archna
Department of Pediatrics and Neonatology, St Stephens Hospital, Tis Hazari, New Delhi, 110054 India.
Department of Pathology, St Stephens Hospital, Tis Hazari, New Delhi, 110054 India.
Springerplus. 2016 Jan 20;5:52. doi: 10.1186/s40064-016-1667-x. eCollection 2016.
Haemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in children but it is uncommon in newborns. To our knowledge only five cases have been reported so far (probably underreported). The known modalities of treatment include transfusion of plasma and plasmapheresis. We report a case of neonatal HUS for whom we performed an exchange transfusion to good effect.
A term vaginally born baby, meconium stained and floppy at birth presented with severe anaemia in the first few hours of life. The baby later on developed renal failure and blood picture was suggestive of severe thrombocytopenia and microangiopathic haemolytic anaemia. No extra renal manifestations of birth asphyxia were noted. A double volume exchange transfusion was performed relatively early and subsequently platelet and haemoglobin stabilised and renal failure improved.
The clinical impression in this case was convincing of neonatal HUS, likely attributable to birth asphyxia but needs to be differentiated from disseminated intravascular coagulation (DIC) and thrombotic thrombocytopenic purpura (TTP). The coagulation profile is usually normal in HUS but it is abnormal in DIC, whereas in TTP one would find hyperbilirubinemia, increased creatinine, haemolysis etc. TTP is rare but not very uncommon in infancy. Congenital TTP is attributed to an inherent deficiency of ADAMTS-13, which is a vWF-cleaving metalloprotease. Irrespective of the etiology of HUS in our case, a dramatic response was observed with exchange transfusion. Transfusion of fresh frozen plasma (FFP) and plasmapheresis are known treatment modalities. FFP replaces the missing or altered complement factors and plasmapheresis removes antibodies, immune complexes and toxins. An exchange transfusion combines both these functions.
In the absence of facilities for plasmapheresis, exchange transfusion is a good alternative.
溶血尿毒综合征（HUS）是儿童急性肾衰竭最常见的病因之一，但在新生儿中并不常见。据我们所知，迄今为止仅报告了5例（可能存在报告不足的情况）。已知的治疗方法包括血浆输注和血浆置换。我们报告了1例新生儿HUS病例，对其进行了换血治疗，效果良好。
一名足月顺产的婴儿，出生时羊水粪染且肌张力低下，出生后数小时出现严重贫血。该婴儿随后发展为肾衰竭，血液检查结果提示严重血小板减少和微血管病性溶血性贫血。未发现出生窒息的肾外表现。相对早期进行了双倍量换血，随后血小板和血红蛋白稳定，肾衰竭得到改善。
该病例的临床印象高度提示新生儿HUS，可能归因于出生窒息，但需要与弥散性血管内凝血（DIC）和血栓性血小板减少性紫癜（TTP）相鉴别。HUS的凝血指标通常正常，而DIC异常，在TTP中会发现高胆红素血症、肌酐升高、溶血等情况。TTP在婴儿期罕见但并非极不常见。先天性TTP归因于一种vWF裂解金属蛋白酶ADAMTS - 13的固有缺陷。无论我们病例中HUS的病因如何，换血治疗均观察到显著效果。输注新鲜冰冻血浆（FFP）和血浆置换是已知的治疗方法。FFP可替代缺失或改变的补体因子，血浆置换可去除抗体、免疫复合物和毒素。换血治疗兼具这两种功能。
在缺乏血浆置换设备的情况下，换血治疗是一种良好的替代方法。
