Hemophagocytic syndrome and T-cell lymphoma after kidney transplantation: a case report

Lymphoma in immunocompromised transplant patients is a feared cause of morbidity and mortality. Superimposed on the lymphoma and the transplantation immunosuppression is a rare condition: hemophagocytic syndrome (HS). HS is characterized by fever, hepatosplenomegaly and lymphadenopathy, skin rashes, jaundice, coagulopathy, and phagocytosis of blood elements with pancytopenia. Here we describe a rare but fatal case of a kidney transplant patient who developed T-cell lymphoma and HS, without evidence of EBV replication. A short review of the diagnosis, treatment, and prognosis of HS is given. Received: 4 March 1997 Received after revision: 6 June 1997 Accepted: 30 June 1997     

小儿肾脏疾病血,尿内皮素的变化

目的研究小儿肾脏疾病血、尿内皮素（ＰＥＴ、ＵＥＴ）的水平及其相互关系。方法采用同位素放免方法检测了肾病综合征（ＮＳ），肾小球肾炎（ＧＮ），肾功能衰竭（ＲＦ）共７２例患儿血及尿中ＥＴ，血心钠素（ＡＮＰ）水平。结果ＮＳ，ＧＮ，ＲＦ三组的ＰＥＴ及ＵＥＴ值明显高于对照组，尤其ＲＦ组（Ｐ＜００５，Ｐ＜０．０１）。ＡＮＰ值在ＧＮ组和ＲＦ组明显高于对照组（Ｐ＜００１）。８例ＡＲＦ患儿恢复期血ＥＴ水平下降，６例ＣＲＦ患儿虽经治疗，但血ＥＴ水平不降或上升。结论ＥＴ在小儿肾脏疾病发病机理及病情进展中可能起重要作用，其值高低与病情严重程度及预后有关。     

老年人微小病变性肾病综合征的临床特征和长期随访

探讨老年人微小病变性(MC)特发性肾病综合征(PNS)的临床特征及预后。方法 观察成人MC性PNS118例,其中老年患者18例,占15.2％。结果 发现老年患者NS的临床表现与成年组相似;但血尿发病率较低,22％比38％;高血压和肾功能不全的发病率明显增高,38.9％比16％和33.3％比19％(P<0.01)。老年组对激素治疗的敏感性略差;但NS复发率明显减少,11.1％比43％。结论 尽管NS的复发和对激素依赖使治疗有一定困难,但激素、细胞毒性药物和环孢素A对于消除蛋白尿,延缓肾功能不全是有助的。     

成人原发性肾病综合征血容量与肾素,醛固酮,心钠素的关系

探讨成人原发性肾病综合征(NS)血容量与肾素、醛固酮及心钠素的关系。方法 应用~(113)mInCl标记转铁蛋白稀释法测定血容量,放免法测定血浆激素水平。对水肿期NS28例、正常26例及其中NS缓解期随访18例进行检测。结果 (1)水肿期NS血容量与正常组无差别,血浆肾素活性(PRA)、血管紧张素Ⅱ(ATⅡ)、醛固酮(Ald)、心钠素(ANP)水平均较正常组高,白蛋白、各激素水平与血容量无显著相关;(2)缓解期与水肿期比较,总的血容量无差异,Ald、ANP显著降低,PRA、ATⅡ则无明显差异;(3)Ald与24小时尿排钠(UNaV)显著负相关。结论 Ald和ANP是NS钠排泄的主要调节因子。     

Endocrinology: High incidence of embryo transfer cancellations in patients with polycystic ovarian syndrome

This study was aimed at assessing the outcome of in-vitro fertilization(IVF) and embryo transfer in patients with polycystic ovariansyndrome (PCOS). The results of IVF and embryo transfer in PCOSpatients (PCOS group, 78 cycles of 26 patients) were comparedwith those of a control group (423 cycles in 202 patients withoutmale factor; age and ovarian stimulation protocol were matched).Although the pregnancy rate per transfer was not different inthe two groups of patients (25 versus 34%, PCOS versus controlgroup), the PCOS group had a significantly lower pregnancy rateper follicle aspiration (19 versus 31%, P < 0.05). A notableresult was a significantly higher incidence of embryo transfercancellations in the PCOS group (22 versus 8%, P < 0.01),which resulted from unpredictable failure of either oocyte recoveryor fertilization. The incidence of unexplained complete failureof fertilization was significantly higher in the PCOS group(18 versus 5%, P < 0.01). These results may reflect a reducedquality of the oocytes in the PCOS group, and there was a subgroupof PCOS patients who repeatedly produced poor results of treatment.Although the ovarian stimulation regimen best suited to PCOSpatients remains to be determined, special care should be takenduring ovarian stimulation, especially when the PCOS patientshad experienced unexplained failure of oocyte recovery or fertilizationin the previous treatment cycle(s).     

Incidence of post-lumbar puncture syndrome reduced by reinserting the stylet: a randomized prospective study of 600 patients

The post-lumbar puncture syndrome (PLPS) can best be explained by prolonged spinal fluid leakage owing to delayed closure of a dural defect. Its incidence after spinal anaesthesia is much lower than after diagnostic lumbar puncture (LP). This difference could be caused by a strand of arachnoid, which might enter the needle with the outflowing cerebrospinal fluid (CSF) during diagnostic LP and upon removal of the needle be threaded back through the dura to produce prolonged CSF leakage. To find a technique that further reduces the incidence of PLPS, this hypothesis was tested by evaluating the effect that reinserting the stylet before removing the needle had on the incidence of PLPS. By reinserting the stylet to the tip of the needle, the hypothesized strand would be pushed out, thereby reducing the frequency of PLPS. Sprotte’s “atraumatic needle” (21 gauge) was used for LP. A total of 600 patients participated in the prospective study. They were randomized into two groups and questioned about their complaints every day for up to 7 days after the LP. All LPs were performed by two experienced neurologists (T.B., M.S.). In 300 patients, the stylet was reinserted to the tip of the needle; in the other 300 it was not reinserted. Whereas 49 of the 300 patients without reinsertion developed PLPS, only 15 of the 300 patients with reinsertion did. This significant difference (16.3 vs 5.0%, P < 0.005, chi square test) supports our hypothesis. On the basis of our results, we recommend reinserting the stylet before removing the needle in order to reduce the incidence of PLPS. Received: 30 September 1997 Received in revised form: 9 March 1998 Accepted: 20 March 1998     

Case Report: Gianotti-Crosti Syndrome Associated with Human Herpesvirus-6 Infection

We report a case of Gianotti-Crosti syndrome associated with human herpesvirus-6 (HHV-6) infection. An eight-month-old girl developed monomorphous papules on her cheeks, buttocks, and extremities after the subsidence of exanthema subitum. Viral antibody analysis confirmed primary HHV-6 infection. HHV-6 may be added to the list of causative agents of Gianotti-Crosti syndrome.     

Nephrotic syndrome in a mother and her infant: relationship with cytomegalovirus infection

This case report describes infantile nephrotic syndrome (NS) in a baby girl with a clinically severe cytomegalovirus (CMV) infection. Culture of the baby's urine was positive for CMV and IgM anti-CMV antibodies were detected. After an unsuccessful course of corticosteroids, gancyclovir treatment was started and a remission of cutaneous, pulmonary, and renal symptoms was achieved. As the mother also developed NS at the end of pregnancy, a common etiology could be postulated, although there were no signs of recent CMV infection in the mother, only anti-CMV IgG. The relationship between CMV infection and glomerular disease is still unclear: NS may represent another manifestation of CMV disease.     

Benign intracranial hypertension and recombinant growth hormone therapy in Australia and New Zealand

Benign intracranial hypertension (BIH) is reported in three children from Australia and one from New Zealand, who were being treated with recombinant human growth hormone (rhGH). Three males and one female, aged between 10.5 and 14.2 y, developed intracranial hypertension within 2 weeks to 3 months of starting treatment. A national database, OZGROW, has been prospectively collecting data on all 3332 children treated with rhGH in Australia and New Zealand from January 1986 to 1996. The incidence of BIH in children treated with growth hormone (GH) is small, 1.2 per 1000 cases overall, but appears to be greater with biochemical GHD (<10IUml ^-1), i.e. 6.5/1000 (3 in 465 cases), relative risk 18.4, 95% confidence interval 1.9-176.1, than in all other children on the database. The incidence in patients with Turner's syndrome was 2.3/1000 (1 in 428 cases). No cases in patients with partial GHD (10–20 IUml ^-1) or chronic renal failure were identified. Possible causative mechanisms are discussed. The authors'practice is now to start GH replacement at less than the usual recommended dose of 14IUm-² week^-1 in those children considered to be at high risk of developing BIH. Ophthalmological evaluation is recommended for children before and during the first few months following commencement of rhGH therapy and is mandatory in the event of peripheral or facial oedema, persistent headaches, vomiting or visual symptoms. The absence of papilledema does not exclude the diagnosis.     

Pathogenesis of neuroimmunologic diseases

Animal models of autoimmune diseases have greatly improved our current understanding of the pathogenesis of human autoimmunity and have provided the potential for therapies based on manipulation of the immune system. In our laboratory, we have investigated the immunopathogenesis of autoimmune diseases of the nervous system and muscle. We have developed immune-based approaches for the suppression of experimental autoimmune encephalomyelitis (EAE), a model for multiple sclerosis (MS), and experimental autoimmune neuritis (EAN), a model for the Guillain-Barré syndrome (GBS). These approaches included induction of peripheral tolerance, immunotoxin targeting of activated T cells, and cytokine manipulations. In addition, we identified the antigen and characterized immunopathologically an autoimmune inflammatory disease of skeletal muscle, experimental autoimmune myositis (EAM), a model for the human inflammatory muscle disease polymyositis.