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严重新生儿高胆红素血症继发于 RhC 溶血病、先天性甲状腺功能减退症和巨大肾上腺血肿:病例报告。

Severe neonatal hyperbilirubinemia secondary to combined RhC hemolytic disease, congenital hypothyroidism and large adrenal hematoma: a case report.

机构信息

Department of Pediatrics, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, Zhejiang Province, China.

出版信息

BMC Pediatr. 2022 Sep 11;22(1):539. doi: 10.1186/s12887-022-03594-7.

Abstract

BACKGROUND

ABO blood group incompatibility, neonatal sepsis, G-6-PD deficiency, thyroid dysfunction, and hereditary spherocytosis are all probable causes of neonatal hyperbilirubinemia. However, the etiology of some hyperbilirubinemia is extremely complicated, which may be caused by multiple factors, resulting in severe jaundice. We report a case of severe jaundice due to three causes, showing the significance for the investigation of the etiology of neonatal hyperbilirubinemia.

CASE PRESENTATION

At 96 h of life, a full-term and vaginal delivery male infant with yellowish discoloration of body was transferred to our hospital. When he entered neonatal intensive care unit on the fourth day after birth, he developed jaundice and the transcutaneous bilirubin was 28 mg/dl. Total bilirubin was 540.2 μmol/L, while the indirect bilirubin was 516.7 μmol/L. Both parents and the baby's blood types were O Rh(D +), and direct coomb's test was negative. But mother's indirect coomb's test was positive. Investigating for minor blood group revealed that the father's blood type of Rh was CCDee, the mather's was ccDEE, and CcDEe for the baby. After intensive phototherapy and double volume exchange transfusion, the total bilirubin remained at 303 μmol/L. At day 10, the bilirubin level was 303.5 μmol/L, intensive phototherapy was continued, and intravenous immunoglobulin was used again. The test for thyroid hormones at day 10, the TSH was 13.334mIU/L. And the screening for congenital hypothyroidism showed the TSH was 33mIU/L. Because of the palpable abdominal mass, ultrasound and MRI was done, showed a huge mass in the right adrenal gland. Brainstem auditory evoked potential was performed at day 7, which indicated hearing impairment (65db for left ear and 70db for the right). Euthyrox and intermittent phototherapy were given as following treatment. The jaundice did not subside until the 12th day.

CONCLUSION

Even if their parents' ABO blood group and Rh (d) are consistent, a Coomb test is required for newborns with hyperbilirubinemia since they may have minor blood group incompatibilities. When bilirubin rises rapidly or the clinical treatment effect is inadequate, additional causes should be aggressively screened. Adrenal ultrasound should be performed on newborns with palpable abdominal mass, anemia and jaundice to determine whether there is adrenal hemorrhage.

摘要

背景

ABO 血型不合、新生儿败血症、G-6-PD 缺乏、甲状腺功能障碍和遗传性球形红细胞增多症都是新生儿高胆红素血症的可能原因。然而,一些高胆红素血症的病因极其复杂,可能由多种因素引起,导致严重的黄疸。我们报告了一例由三种原因引起的严重黄疸病例,表明对新生儿高胆红素血症病因调查的重要性。

病例介绍

患儿,男,胎龄 39 周,自然顺产,生后 96 小时因皮肤黄染转入我院。生后第 4 天入新生儿重症监护病房时出现黄疸,经皮胆红素 28mg/dl,总胆红素 540.2μmol/L,间接胆红素 516.7μmol/L。患儿父母及患儿血型均为 O Rh(D)+,直接 Coomb's 试验阴性,母亲间接 Coomb's 试验阳性。进一步行微柱凝集法血型鉴定,发现患儿父亲 Rh 血型为 CCDee,母亲为 ccDEE,患儿为 CcDEe。给予积极光疗及双倍换血治疗后,总胆红素仍维持在 303μmol/L。生后第 10 天,患儿胆红素水平为 303.5μmol/L,继续光疗,再次给予丙种球蛋白静注。生后第 10 天行甲状腺功能检查,TSH 为 13.334mIU/L,先天性甲状腺功能减退症筛查 TSH 为 33mIU/L。因触及腹部包块,行腹部超声及 MRI 检查,提示右肾上腺巨大占位。生后第 7 天行脑干听觉诱发电位检查,示双耳听力损失(左耳 65dB,右耳 70dB)。给予左甲状腺素钠片口服及间断光疗。第 12 天黄疸才逐渐消退。

结论

即使新生儿父母 ABO 血型和 Rh(D)相合,对于高胆红素血症患儿仍建议行 Coomb 试验,因其可能存在微小血型不合。当胆红素快速升高或临床治疗效果不佳时,应积极筛查其他病因。对于有腹部包块、贫血、黄疸的新生儿,应行肾上腺超声检查,以确定是否存在肾上腺出血。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f29a/9465864/e9d490286682/12887_2022_3594_Fig1_HTML.jpg

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