Plasmapheresis in newborns with hyperbilirubinemia

With a miniaturized plasmapheresis system consisting of a double head pump and balancing pump, extracorporeal detoxification treatments such as plasmapheresis, hemofiltration, and hemodialysis were carried out. In total 15 premature infants and newborns with Rherythroblastosis and hyperbilirubinemia were treated using this miniaturized system. The average birth weight of the 15 patients was 2,386 g. With a main blood flow of 5.3 ml/min and a filtrate flow of 1.3 ml/min, an average of 192.4 ml of plasma per treatment was exchanged. In all patients 1 to 10 plasmapheresis treatments were necessary to reduce the mean serum bilirubin from 15.6 +/- 4.8 to 7.3 +/- 3.2 mg/dl, and after several days the serum bilirubin subsequently normalized in 12 patients, who improved. Three patients died 5 to 18 days after birth as a result of their primary disease and immaturity. For vascular access small catheters were inserted in the umbilical or the femoral vein. All treatments were well tolerated by the patients.     

Influence of Gender on the Ratio of Serum Aspartate Aminotransferase (AST) to Alanine Aminotransferase (ALT) in Patients With and Without Hyperbilirubinemia

The serum asparate aminotransferase (AST)/alanine aminotransferase (ALT) ratio is widely used in the differential diagnosis of icteric and non-icteric hepatic disorders. Our objective was to determine whether there are gender related-differences in the serum AST/ALT ratio. We used sera from 3,618 unselected patients sent to our laboratory for an automated chemistry panel, which included measurements of AST, ALT, alkaline phosphatase, and total bilirubin. Effects of gender on serum AST, ALT, and AST/ALT were examined in different age groups. Among patients with normal total serum bilirubin concentrations, serum AST and ALT concentrations were significantly lower in the females than in the males (P < 0.0001). However, the serum AST/ALT ratio was higher in the females than the males (median values of 0.90 and 0.81, respectively; P < 0.0001). AST and ALT were also lower in the 54 hyperbilirubinemic females than in the 102 hyperbilirubinemic males. Serum AST/ALT ratios were considerably higher in these 156 hyperbilirubinemic patients than in the normobilirubinemic group, with median ratios of 1.09 in females and 0.92 in males (P = 0.02). Significantly higher serum AST/ALT ratios in females were first evident in the 3rd age decade and remained significantly higher than ratios in males through the 8th decade. We conclude that serum AST/ALT ratios are higher in women than men. When clinicians utilize serum AST/ALT ratios to assess the etiology or chronicity of liver disease, the patient’s gender also should be taken into consideration.     

茵栀颗粒黄和贝飞达辅助治疗新生儿黄胆疗效观察

目的探讨茵栀黄颗粒联合贝飞达(双歧杆菌三联活菌肠溶胶囊)辅助治疗新生儿高胆红素血症的临床疗效。方法选择新生儿高胆红素血症120例,随机分为观察组和对照组,每组各60例,对照组给予常规治疗,观察组在对照组基础上加用茵栀黄颗粒和贝飞达(双歧杆菌三联活菌肠溶胶囊),比较两组疗效。结果观察组总有效率为93.4%,较对照组的66.7%明显增高,差异有统计学意义(P0.05)。结论茵栀黄颗粒联合贝飞达(双歧杆菌三联活菌肠溶胶囊)辅助治疗新生儿高胆红素血症疗效显著,具有安全、经济、方便等优点,值得临床推广应用。     

Effect of clofibrate in jaundiced term newborns

Objective: Clofibrate is a glucuronosyl transferase inducer that has been proposed to increase the elimination of bilirubin in neonates with hyperbilirubinemia. The aim of this study was to characterize the therapeutic effect of clofibrate in neonates born at full term and present with non-hemolytic jaundice.Methods: A clinical controlled study was performed in two groups of healthy full term neonates. Thirty neonates were treated with a single oral dose of clofibrate (100 mg/kg) plus phototherapy (clofibrate-treated group) while another 30 neonates (control group) received only phototherapy.Result: The mean plasma total bilirubin levels of 12^th, 24^th and 48^th hours were significantly lower in the clofibrate-treated group as compared with the control group (P<0.0001, P<0.0001 and P=0.004, respectively). Treatment with clofibrate also resulted in a shorter duration of jaundice and a decreased use of phototherapy (P<0.0001). No side effects were observed.Conclusion: Although other pharmacological agents such as metalloporphyrins and Sn-mesoporphyrin also seem to be effective in decreasing bilirubin production, these products are not available for routine use and cannot be used because the safety of these drugs has to be confirmed prior to their widespread use. Therefore, clofibrate is now the only available pharmacological treatment of neonatal jaundice.     

严重高胆红素血症新生儿换血治疗后血小板减少症的危险因素

目的探讨新生儿严重高胆红素血症换血治疗后,引起血小板减少症的危险因素。方法采用回顾性研究方法,经换血治疗的新生儿严重高胆红素血症患儿528例,换血后出现血小板减少症127例,换血后血小板计数正常401例,随机选取127例为对照组,利用Logistic回归分析血小板减少症临床资料的危险因素。结果换血后新生儿血小板减少症发生率高达24.1%(127/528例),血小板计数≥50×10~9/L占70.1%,血小板计数20~50×10~9/L占24.4%,血小板计数20×10~9/L占5.5%。换血时日龄[OR=1.213,95%CI(1.028,1.417)]、换血前总胆红素值[OR=1.172,95%CI(1.051,1.309)],颅内出血或头颅血肿[OR=5.594,95%CI(3.257,11.121)]、换血后酸中毒OR=7.246,95%CI(4.797,12.188),4项因素为换血后血小板减少症的危险因素。结论患儿换血时日龄、换血前总胆红素值、合并颅内出血或头颅血肿、换血后酸中毒是换血治疗新生儿严重高胆红素血症后引起血小板减少症的危险因素。     

Deafness,a Social Stigma: Physician Perspective

Hearing is an essential sensory sense of an individual for development of speech which is crucial for verbal communication and personality development. It is the second most common form of disability after loco motor disability in India. Disabling hearing loss is more than 40 dB hearing loss in better ear in a person more than 15 years of age and greater than 30 dB hearing loss in better hearing ear below 14 years of age. WHO estimated 360 million individuals in the world with disabling hearing loss, out of which 91 % are adults and only 9 % are children. Early and accurate identification of birth asphyxia, hyperbilirubinemia, auditory neuropathy Presbyacusis and avoiding noise pollution and discouraging use of mobile phone, tobacco chewing/smoking, in those who are prone to deafness, an intervention is a must to decrease deafness from our society. Deafness prevention can only be possible with mutual cooperation with dedication of different medical and non-medical personnel and also by helping the persons with deafness. We have to focus not only on the children but also on senior citizens as most alarming, up to 40 %, incidence of deafness is in senior citizens above the age of 75 years. Timely cure and preventive measures are essential for better socio-economic state of the country. By helping the persons with deafness, we will not only be doing a great service to the Nation but also to the society at large.     

SLCO1 B1基因多态性与新生儿高胆红素血症的关系

目的：探讨有机阴离子转运体1B1（SLCO1B1）基因多态性与新生儿高胆红素血症的关系。方法收集121例病因不明高胆红素血症新生儿（病例组）和87例正常新生儿（对照组）外周血，用聚合酶链式反应－限制性片段长度多态性（ PCR-RFLP）技术测定 SLCO1B1 c．388A／G、c．521T／C、c．571T／C 及 c．597C／T 基因型。结果（1）病例组与对照组SLCO1B1 c．388A／G、c．521T／C、c．571T／C、c．597C／T 各突变位点基因型分布及等位基因频率比较，差异均无统计学意义（P＞0．05）；（2）SLCO1B1 c．388A／G、c．521T／C、c．571T／C、c．597C／T突变位点与新生儿高胆红素血症发生无相关性（P＞0．05）；（3）高胆红素血症新生儿SLCO1B1同一位点的不同基因型的总胆红素、未结合胆红素、结合胆红素水平比较，差异均无统计学意义（ P＞0．05）。结论单一存在的SLCO1B1 c．388A／G、c．521T／C、c．571T／C及c．597 C／T可能与本地区新生儿高胆的发病无关。 SLCO1B1同一突变位点的不同基因型可能对患儿血清胆红素水平影响不大。     

广西南宁地区G6PD基因突变与新生儿黄疸的关系

目的：分析本地区最常见的三种基因突变型G1388A、G1376T和A95G与葡萄糖-6-磷酸脱氢酶（G-6-PD）活性之间的相关性,并探讨G-6-PD基因突变对新生儿黄疸的影响。方法：124例广西南宁的高胆红素血症新生儿为研究对象。应用突变特异性扩增系统法检测G-6-PD基因突变,应用硝基四氮唑蓝（NBT）定量法检测G-6-PD活性。比较G-6-PD不同基因突变型之间以及与正常组之间胆红素脑病发生率、出生72 h后血清胆红素峰值组间的差异。采用非条件logistic回归分析血清胆红素值>340 μmol/L的危险度。结果：124例中有37例G-6-PD 基因突变（G1388A 20例,G1376T 14例,A95G 4例,1例同时存在G1388A与A95G突变）。20例G1388A突变者中5例（25%）G-6-PD酶活性正常,14例G1376T突变者中4例（29%）G-6-PD酶活性正常,4例A95G突变者G-6-PD 酶活性均缺乏。G1388A与G1376T组胆红素脑病发生率及出生72 h后血清胆红素峰值差异无显著性。G-6-PD 突变组出生72 h后血清胆红素峰值、胆红素脑病发生率及血清胆红素>340 μmol/L的危险度与G-6-PD正常组相比,差异无显著性。结论：广西南宁地区G-6-PD突变仍常见G1388A、G1376T和A95G基因型。NBT法诊断G-6-PD缺乏存在假阴性。不同基因型对出生72 h后血清胆红素峰值、胆红素脑病发生率的影响无差异。单独的G-6-PD基因突变对生后72 h血清胆红素峰值、急性胆红素脑病发生率及血清胆红素大于340 μmol/L危险性均无影响。［中国当代儿科杂志,2009,11（12）：970-972］     

Management of hyperbilirubinemia and prevention of kernicterus in 20 patients with Crigler-Najjar disease

We summarize the treatment of 20 patients with Crigler-Najjar disease (CND) managed at one center from 1989 to 2005 (200 patient-years). Diagnosis was confirmed by sequencing the UGTA1A gene. Nineteen patients had a severe (type 1) phenotype. Major treatment goals were to maintain the bilirubin to albumin concentration ratio at <0.5 in neonates and <0.7 in older children and adults, to avoid drugs known to displace bilirubin from albumin, and to manage temporary exacerbations of hyperbilirubinemia caused by illness or gallstones. A variety of phototherapy systems provided high irradiance over a large body surface. Mean total bilirubin for the group was 16+/-5 mg/dl and increased with age by approximately 0.8 mg/dl per year. The molar ratio of bilirubin to albumin ranged from 0.17 to 0.75 (mean: 0.44). The overall non-surgical hospitalization rate was 0.12 hospitalizations per patient per year; one-half of these were for neonatal hyperbilirubinemia and the remainder were for infectious illnesses. Ten patients (50%) underwent elective laproscopic cholecystectomy for cholelithiasis. No patient required invasive bilirubin removal or developed bilirubin-induced neurological damage under our care. Visual acuity and color discrimination did not differ between CND patients and age-matched sibling controls. Four patients treated with orthotopic liver transplantation were effectively cured of CND, although one suffered significant transplant-related complications.Conclusions. While patients await liver transplantation for CND, hyperbilirubinemia can be managed safely and effectively to prevent kernicterus. Lessons learned from CND can be applied to screening and therapy of non-hemolytic jaundice in otherwise healthy newborns.