Alloimmune thrombocytopenia: in utero treatment by high doses of intravenous gamma globulins.

The authors report successful in utero treatment by high doses of intravenous gamma globulins in a case of neonatal alloimmune thrombocytopenia. Early diagnosis allows an appropriate management: fetal blood sampling as early as 20 weeks of gestation; in case of fetal thrombocytopenia, treatment by intravenous gamma globulin (1.0 g/kg/b.w.) each week during 8 weeks or more; ultrasound screening of in utero hemorrhage, particularly intracranial hemorrhage; fetal blood sampling before delivery at term and in utero transfusion of platelet antigen negative in case of persistence of fetal thrombocytopenia.     

Neonatal alloimmune thrombocytopenia in Norway: poor detection rate with nonscreening versus a general screening programme

The implementation of an antenatal screening programme for neonatal alloimmune thrombocytopenia (NAIT) is currently under debate. We evaluated the detection rate for NAIT in a nonscreened population of 661 200 births where NAIT was diagnosed on clinical indication. We did a cross-sectional comparison with a population of 100 448 human platelet antigen 1a (HPA1a)-screened pregnancies from three of the five health regions in Norway. In a nonscreening situation, 7.5 cases of NAIT were detected per year compared with 53 cases when screening was applied. The detection rate of NAIT in Norway was therefore 14% of the expected rate.     

The immunologic profile of infants born after maternal immunoglobulin treatment and intrauterine platelet transfusions for fetal/neonatal alloimmune thrombocytopenia

OBJECTIVE: The purpose of this study was to evaluate whether maternally administered intravenous immunoglobulins (IVIG) and intrauterine platelet transfusions (IUPT) for fetal/neonatal alloimmune thrombocytopenia (FNAIT) affect the development of the fetal immune system. STUDY DESIGN: The lymphocyte subset distribution of mononuclear cells of cord blood of 20 FNAIT newborns was analyzed by flow cytometry and compared with a control group of healthy newborns and a reference group treated with intrauterine erythrocyte transfusions (IUET) for hemolytic disease. RESULTS: The percentage of monocytes, natural killer (NK) cells, ratios of mature and immature T cells and B cells, and resting or activated cells were not significantly different compared to the control group. In addition, the B-cell and T-cell populations showed a normal in vitro antibody production and T-cell proliferation when compared with the control group. CONCLUSION: Antenatal treatment for FNAIT with maternal IVIG with or without IUPT is not associated with lymphocyte activation or premature maturation of the neonatal immune system.     

丙种球蛋白不同剂量治疗小儿免疫性血小板减少症疗效观察

目的观察丙种球蛋白不同剂量治疗小儿免疫性血小板减少症的临床疗效。方法 2015年1月至2016年12月湖南省儿童医院血液内科收治住院的免疫性血小板减少症患儿94例,随机分为小剂量组与常规剂量组各47例。两组患儿均给予甲泼尼龙治疗,小剂量组给予200mg/(kg·d)丙种球蛋白治疗,常规剂量组给予400mg/(kg·d)丙种球蛋白治疗,每周1次,治疗1个月。观察两组临床疗效、血小板上升时间、血小板上升至正常时间、血小板达峰值、血小板止血时间、住院时间、治疗费用、白细胞计数、血红蛋白、血小板计数以及不良反应发生情况。结果小剂量组患儿治疗总有效率与常规剂量组比较差异无统计学意义(P0.05);两组患儿血小板上升时间、血小板上升至正常时间、血小板止血时间、住院时间比较差异无统计学意义(P0.05);两组患儿治疗后的白细胞计数、血红蛋白对比差异无统计学意义(P0.05);小剂量组患儿治疗后血小板计数显著高于常规剂量组,血小板达峰值时间显著短于常规剂量组,治疗费用显著少于常规剂量组,差异有统计学意义(P0.05);两组患儿不良反应发生率比较差异无统计学意义(P0.05)。结论不同剂量丙种球蛋白治疗免疫性血小板减少症患儿的临床疗效及安全性相当,但小剂量丙种球蛋白治疗血小板达峰值时间更短,且治疗费用更低,具有临床推广使用价值。     

Neonatal alloimmune thrombocytopenia due to anti-P1A1 (anti-HPA-1a): importance of paternal and fetal platelet typing for assessment of fetal risk.

Neonatal alloimmune thrombocytopenia (NAIT), which usually involves sensitization to P1A1 (HPA-1a), may have devastating complications for the fetus. These may be prevented by antenatal treatment of severe cases with either maternally administered high-dose gamma-globulin and/or repeated intrauterine platelet transfusions. Determination of the paternal platelet phenotype is useful for counseling parents who have had one or more affected pregnancies. This report of an unaffected pregnancy in a woman with a history of previous pregnancies complicated by NAIT illustrates the role of paternal and fetal platelet phenotyping in managing existing pregnancies at risk of NAIT.     

Neonatal thrombocytopenia induced by maternal anti-HLA antibodies: a potential side effect of allogenic leukocyte immunization for unexplained recurrent aborters

Allogenic leukocyte immunization is one of several treatments tried for unexplained recurrent aborters, and is reported to have few maternal and neonatal side effects after the immunotherapy having been reported to date. In the present study, we report a rare case of neonatal thrombocytopenia (41000 cells/microl) observed in a female infant delivered by an unexplained habitual aborter. The mother was immunized with her husband's leukocytes once before pregnancy and twice at the 5th and 6th week of her successful pregnancy. Serological studies using mixed passive hemagglutination assays (MPHA) showed that maternal serum did not contain any antibodies which were reactive to 11 platelet-specific antigens, or to granulocyte antigens extracted from 9 persons. Lymphocyte cytotoxicity tests, however, showed that maternal serum but not infant serum had anti-HLA antibodies against both paternal and infant lymphocytes. Moreover, the maternal serum was found to have anti-HLA IgGs against platelet antigens extracted from the father and the infant. It is highly likely that this case of neonatal thrombocytopenia was caused by transplacental perfusion of maternal anti-HLA antibodies whose production was induced or enhanced by the allogenic leukocytes immunizations.     

川崎病患儿退热后应用丙种球蛋白治疗剂量与预后关系探讨

目的探讨10d内已退热的川崎病(KD)患儿应用丙种球蛋白(IVGG)治疗的必要性以及不同剂量IVGG治疗对KD预后的影响。 方法研究对象为1999-10—2005-10山东省菏泽市立医院收治的56例KD患儿，所有患儿均为10d内退热后确诊且无冠脉病变。按IVGG治疗剂量分成3组，A组(11例)用1g/kg，B组(26例)用2g/kg，C组(19例)未使用，余治疗相同。对其冠状动脉损害(CAL)情况进行对比。 结果病程14~21d时发生CAL例数：A组2例(1818%),B组4例(1538%),C组16例(8421%),A、B组比较差异无显著性意义(P>005);A、B组与C组之间差异有非常显著性意义(P<001)。随访05年CAL例数：A组1例(909%)，B组1例(385%)，C组11例(5789%)，A、B组比较差异无显著性意义(P>005)，而A、B组与C组之间差异有非常显著性意义(P<001)。 结论10d内一经确诊的KD无论是否已退热均应给予IVGG治疗，对已退热且无冠脉损害的患儿应用总量1g/kg IVGG治疗可以达到满意的效果。     

Resolution of severe thrombocytopenia in a pregnant patient with rhesus-negative blood with autoimmune thrombocytopenic purpura after intravenous rhesus immune globulin

Treatment with 120 micrograms of intravenous rhesus immune globulin at 29 1/2 weeks' gestation resulted in an elevation of the platelet count from 38,000/mm3 to 73,000/mm3 in a patient with rhesus-negative blood with autoimmune thrombocytopenic purpura. Red blood cell immune blockade of the reticuloendothelial system is probably not the primary mechanism by which rhesus immune globulin elevates the platelet count.     

High-dose intravenous gamma globulin: does it have a role in the treatment of severe erythroblastosis fetalis?

The role of high-dose intravenous (IV) gamma globulin in the treatment of erythroblastosis fetalis was assessed in five pregnancies with severe Rh (four) or Kell (one) isoimmunization. These women were treated with IV gamma globulin (1.0 g/kg body weight) once a week. In addition, fetal blood transfusions were performed when indicated. In four patients with Rh sensitization, high-dose IV gamma globulin treatment had no apparent effect on the total number of intrauterine transfusions required, the interval between transfusions, or the volume of blood required at each transfusion. The treatment did not prevent fetal hydrops and had no effect on maternal antibody titers. In one patient with Kell sensitization, however, the course of the disease was less severe than anticipated, suggesting that IV gamma globulin treatment may have modified the severity of the disease. We conclude that high-dose IV gamma globulin does not appear to be useful in the treatment of severe Rh disease. Its role in Kell and other types of red-cell isoimmunization deserves further evaluation.     

Neonatal outcome after treatment with ritodrine: a controlled study

Neonatal condition was assessed in a group of 82 infants born at term after maternal treatment with ritodrine for an average of 28.5 days, and compared with that in a matched control group of infants. No statistically significant differences were found in umbilical pH, Apgar scores, head circumference, and neurological condition. Although the difference was not statistically significant, doubt remains as to a possible icterogenic effect of ritodrine.     

Immunological studies in recurrent spontaneous abortion: effects of immunization of women with paternal mononuclear cells on lymphocytotoxic and mixed lymphocyte reaction blocking antibodies and correlation with sharing of HLA and pregnancy outcome

The occurrence of maternal antipaternal lymphocytotoxic antibody (LCTA), mixed lymphocyte reaction blocking factors (MLRBF) and human leukocyte antigen (HLA) antigen sharing was studied in 115 couples with unexplained repeated spontaneous abortions (RSA). Comparisons were made to the same studies done on 41 couples with explained repeated miscarriages. We found no significant difference between the patient and control group with respect to the percent of couples sharing none, one, or two or more HLA-A,-B, or -DR antigens. Examination of the occurrence of LCTA and MLRBF likewise did not reveal differences between the groups, nor did the occurrence of these antibodies on initial testing correlate with HLA disparity between couples. Women with three or more spontaneous abortions were immunized with paternal mononuclear cells (MNC) if they met at least two of the following three criteria: they shared two or more HLA antigens; their serum was negative for paternal MNC-directed LCTA; their serum did not contain maternal versus paternal MLR blocking factors. Complete HLA, LCTA and MLRBF data pre- and post-treatment are available on 60 women. Sixty-three percent of women converted to LCTA positive 6 +/- 1 weeks after immunization, and 35% of women converted from negative to positive for MLR blocking after immunization. Fifty-eight women who had all three tests done prior to immunization became pregnant after immunization. Only 50% of this selected group have experienced successful pregnancy as judged by delivery of a live-born infant. In the patients presented, successful pregnancy outcome did not correlate with HLA antigen disparity, but successful patients were more likely than aborters to have either LCTA or MLRBF prior to pregnancy (28 vs. 7%). Post-immunization conversion to LCTA positive was more prevalent in the women who aborted after immunization (74%) compared to those who had successful pregnancy (48%) while MLR blocking antibody conversion from negative to positive was the same in both groups. The data indicate that neither HLA antigen sharing nor conversion to LCTA or MLR blocking positive after paternal WBC immunization are predictors for successful pregnancy outcome. Results so far suggest that conversion to LCTA positive after immunization may have a negative influence on pregnancy outcome.     

Retrograde ejaculation: successful treatment with artificial insemination.

Retrograde ejaculation is characterized by aspermia or oligospermia and results from an incompetent bladder neck, often due to a dysfunction of the internal sphincter. In almost 3 years, eight couples who suffered from infertility due to retrograde ejaculation were treated with inseminations with spermatozoa gained from the urine. Ovulation was predicted on the basis of blood LH levels. The urine-semen sample was collected in 100 mL of Hepes medium and 5 mL 1% human albumin (pH 7.4). After centrifuging, the remaining sperm pellet was dispersed on a Percoll gradient. After centrifuging and resuspending, followed by two washing procedures with Ham's F-10 and human albumin 1%, the remaining sample was used for intrauterine insemination. Twelve pregnancies were thus achieved; two women became pregnant twice and one three times. The pregnancy rate per cycle was 44.4%. In seven couples, pregnancy was achieved within three cycles. Four pregnancies ended in spontaneous abortion and five ended in the birth of a healthy child; three pregnancies were continuing at the time of writing. Retrograde ejaculation can be treated successfully with inseminations using spermatozoa obtained from urine. It seems important to collect the urine-semen sample in a buffered medium and to time the insemination on the basis of the LH surge.     

Normal pregnancy after successful treatment of choriocarcinoma with cerebral metastases. A case report

Pregnancy after treatment of choriocarcinoma with cerebral metastases is uncommon. We treated a patient successfully with less-toxic chemotherapeutic agents than those advocated by others together with whole brain irradiation. She subsequently had two uneventful pregnancies.     

Stuck twin with cotwin nonimmune hydrops: successful treatment by amniocentesis.

Until the recent introduction of therapeutic amniocentesis, pregnancies complicated by the stuck twin phenomenon usually suffered poor outcomes. We report the first case in which a single amniocentesis of the cotwin was followed by equalization of amniotic fluid volume in both sacs, resolution of nonimmune hydrops in the cotwin, and delivery of 2 healthy infants. The cause of the phenomenon in this was most likely severe twin-twin transfusion, based on a negative hematologic and serologic immune and nonimmune hydrops evaluation, and placental histology revealing a superficial large-caliber arteriovenous anastomosis joining the twins' circulations without an intervening capillary bed. The mechanism proposed for the physiologic response in this case to therapeutic amniocentesis is based on the correction of abnormal amniotic fluid volumes and pressures. This contention is supported by histologic evaluation of the placenta and the intrapartum fetal heart rate tracing.     

地塞米松联合丙种球蛋白治疗小儿过敏性紫癜性肾炎的疗效及对尿蛋白、尿红细胞的影响

目的观察地塞米松联合丙种球蛋白治疗小儿过敏性紫癜(HSP)性肾炎的疗效及对尿蛋白、尿红细胞的影响。方法选择2014年5月至2016年5月惠州市第一妇幼保健院儿科收治的90例HSP患儿为研究对象,随机分为观察组和对照组各45例。对照组采用地塞米松进行治疗,观察组采用地塞米松联合丙种球蛋白进行治疗。4周为1个疗程。观察两组临床疗效、治疗前后凝血指标、24h尿蛋白和尿红细胞水平及患儿不良反应情况。结果观察组治疗后总有效率为95.6%(43/45),显著高于对照组77.8%(35/45),差异有统计学意义(P0.05)。治疗4周后,两组凝血酶原时间较治疗前均无明显改变,差异无统计学意义(P0.05);两组部分凝血活酶时间显著高于治疗前,纤维蛋白原含量显著低于治疗前,差异有统计学意义(P0.05);观察组治疗后部分凝血活酶时间值显著高于对照组,纤维蛋白原含量显著低于对照组,差异有统计学意义(P0.05)。治疗后,两组患儿24h尿蛋白、尿红细胞水平与治疗前比较均降低,差异有统计学意义(P0.05);观察组24h尿蛋白、尿红细胞水平显著低于对照组,差异有统计学意义(P0.05)。观察组不良反应发生率为4.4%(2/45),对照组不良反应发生率为6.7%(3/45),两组患儿不良反应发生率比较差异无统计学意义(P0.05)。治疗前和治疗4周时两组患儿肾损害发生率比较差异无统计学意义(P0.05)。治疗3个月后随访观察组失访1例,对照组肾损害发生率为28.9%(13/45),显著高于观察组11.4%(5/44),差异有统计学意义(P0.05)。结论地塞米松联合丙种球蛋白治疗小儿过敏性紫癜性肾炎效果确切,能够显著降低患者尿蛋白和尿红细胞水平,改善患儿肾功能,不良反应较少,值得临床推广。     

Congenital syphilis after treatment of maternal syphilis with a penicillin regimen exceeding CDC guidelines.

BACKGROUND: Although congenital syphilis usually occurs as a result of a failure to detect and treat syphilis in pregnant women, failures of the currently recommended regimen to prevent congenital syphilis have been reported. CASE: This report describes an infant with congenital syphilis despite maternal treatment with a regimen exceeding current CDC guidelines. CONCLUSION: Regardless of the regimen used to treat syphilis during pregnancy, clinicians should recognize the possibility of occasional treatment failures and the importance of adequate follow-up of infants at risk for congenital syphilis.