Malla K, Malla T, Hanif Md
Kathmandu Medical College, Sinamangal.
Kathmandu Univ Med J (KUMJ). 2004 Oct-Dec;2(4):291-6.
This study aims to review the clinical presentations of Haemolytic Uraemic Syndrome (HUS) and to compare the poor prognostic indicators with mortality.
Prospective study carried out in Renal Dialysis ward of Dhaka Shishu Hospital, Bangladesh from September 2001 November 2003 for a period of 26 months. All children admitted to renal dialysis ward with oliguria or anuria with pallor was included in this study. HUS was confirmed after laboratory investigations showing features of hemolytic anaemia, thrombocytopenia and renal insufficiency. Various clinical presentations were reviewed. Then bad prognostic factors were compared with mortality.
There were total 25 cases of HUS in 26 months.17 (68%) were males and 8(32%) females.21 (84%) children were < 5 years. Only 4(16%) were > 5 years. Before onset of HUS 40% children had bloody diarrhoea, 36% had acute watery diarrhoea and 24% had others symptoms. The other presentations noted were fever 88%, respiratory distress and convulsion 52% and oliguria 40%, anuria 60%, reluctant to feed 40% and cough 28%. The main physical findings noted were irritability 40%, pallor 100%, dehydration 28%, puffy face with oedema 32%, high blood pressure 16%, hepatomegaly 28%, jaundice, sclerema and petechial rashes 8%, lethargic 16%, acidotic breathing 48% and rectal prolapse 12%. 44% children died after HUS and 56% recovered from the illness. Mortality was 66% when duration of illness before onset of HUS was > 14 days. With duration of anuria < 3 days there was no mortality but it was 91% and 100% with anuria > 3 to 8 days and >8 days respectively. Mortality was 78% when age was < 18 months and it was 75% when age was > 5 years. Diarrhoea associated HUS had 27% and non diarrhoea associated HUS had 85% mortality. Mortality was 77% and 100% respectively when HUS was associated with convulsion and hypertension. WBC > 30,000 had mortality 100% and decreased platelet count < 30,000 had mortality 80%. With creatinine level > 700 micromol/L mortality was 80% and with Serum potassium level 5.6 to 7.5 mmol/L mortality was 67%.
HUS comprised of varieties of presentations. Diarrhoea was the commonest preceding illness before onset of HUS. The bad prognostic indicators carrying high mortality was duration of illness before onset of HUS >14 days, anuria > 3 days, age < 18 months and >5 years, Non diarrhoea associated HUS, HUS associated with convulsion and hypertension, WBC > 30,000/cumm, platelets < 30,000/cumm, creatinine level > 700 micromol/L and serum potassium level 5.6 to 7.5 mmol/L. Since bad prognostic factors may progress rapidly to mortality, consultation with paediatrician and transfer to a tertiary care centre should be done when HUS is diagnosed so that it can be managed appropriately in time.
本研究旨在回顾溶血尿毒综合征（HUS）的临床表现，并比较不良预后指标与死亡率。
2001年9月至2003年11月在孟加拉国达卡儿童医院肾透析病房进行了为期26个月的前瞻性研究。所有因少尿或无尿伴面色苍白入住肾透析病房的儿童均纳入本研究。经实验室检查显示有溶血性贫血、血小板减少和肾功能不全特征后确诊为HUS。回顾了各种临床表现。然后将不良预后因素与死亡率进行比较。
26个月内共25例HUS。男性17例（68%），女性8例（32%）。21例（84%）儿童年龄<5岁。仅4例（16%）年龄>5岁。HUS发病前，40%的儿童有血性腹泻，36%有急性水样腹泻，24%有其他症状。其他观察到的表现为发热88%、呼吸窘迫和惊厥52%、少尿40%、无尿60%、不愿进食40%和咳嗽28%。主要体格检查发现为易激惹40%、面色苍白100%、脱水28%、面部浮肿32%、高血压16%、肝肿大28%、黄疸、硬肿和瘀点皮疹8%、嗜睡16%、酸中毒呼吸48%和直肠脱垂12%。44%的儿童在HUS后死亡，56%康复。HUS发病前病程>14天的死亡率为66%。无尿持续时间<3天无死亡，但无尿持续时间>3至8天和>8天的死亡率分别为91%和100%。年龄<18个月的死亡率为78%，年龄>5岁的死亡率为75%。腹泻相关性HUS的死亡率为27%，非腹泻相关性HUS的死亡率为85%。HUS合并惊厥和高血压时，死亡率分别为77%和100%。白细胞>30000/mm³的死亡率为100%，血小板计数<30000/mm³的死亡率为80%。肌酐水平>700微摩尔/升时死亡率为80%，血清钾水平5.6至7.5毫摩尔/升时死亡率为67%。
HUS有多种表现形式。腹泻是HUS发病前最常见的前驱疾病。携带高死亡率的不良预后指标是HUS发病前病程>14天、无尿>3天、年龄<18个月和>5岁、非腹泻相关性HUS、HUS合并惊厥和高血压、白细胞>30000/mm³、血小板<30000/mm³、肌酐水平>700微摩尔/升和血清钾水平5.6至7.5毫摩尔/升。由于不良预后因素可能迅速进展至死亡，因此诊断HUS时应咨询儿科医生并转至三级护理中心，以便及时进行适当管理。
