Prognosis and pathological characteristics of five children with non-Shiga toxin-mediated hemolytic uremic syndrome

BACKGROUND: The three major signs of hemolytic uremic syndrome (HUS) are hemolytic anemia, thrombopenia and acute renal failure. HUS is classified into Shiga toxin-mediated HUS (Stx-HUS) and non-Shiga toxin-mediated HUS (nStx-HUS). The prognosis of nStx-HUS is reported to be less favorable than that of Stx-HUS. Although the association between the prognosis and pathological characteristics of HUS have been reported such that the prognosis was considered to be poor for thrombotic microangiopathy (TMA) with predominant arterial involvement (arterial TMA), good for TMA with predominant glomerular involvement (glomerular TMA) and dependent on the extent of necrosis in cases of renal cortical necrosis, it is not yet clear whether pathological findings are also related to the renal prognosis of nStx-HUS cases. Therefore the purpose of the present paper was to analyze renal biopsy findings and prognosis for five children with nStx-HUS. METHODS: Clinical records of five cases of nStx-HUS among 74 cases of diagnosed HUS were reviewed, and information and data were summarized. RESULTS: Histological examination of the kidney led to the diagnosis of arterial TMA in three cases, and glomerular TMA and severe renal cortical necrosis in one case each. Analysis of the relationship between renal histological findings and the prognosis found that three patients with arterial TMA and one patient with severe renal cortical necrosis later developed end-stage renal failure while one patient with glomerular TMA has continued to show normal renal function. CONCLUSIONS: These findings indicate that pathological findings are closely related to the prognosis in cases of nStx-HUS.     

Unilateral or bilateral nerve-sparing radical hysterectomy: a surgical technique to preserve the pelvic autonomic nerves while increasing radicality

We performed unilateral or bilateral nerve-sparing (UNS or BNS) radical hysterectomies combined with a parametrial excision in patients with locally advanced cervical cancer. The parametrial excision technique is characterized by a meticulous sharp dissection of the avascular plane outside the visceral fascia of the uterus and vagina under direct vision, providing an en bloc parametria and ensuring that all regional spread of the disease is contained within negative surgical margins. The aim of this study was to describe this surgical technique and to retrospectively evaluate the feasibility and the impact on early bladder function. From February 2005 to November 2006, 32 patients with FIGO stage IB-IIB cervical cancer, who had the tumor of more than 20 mm in diameter, underwent the UNS surgery or BNS surgery. A parametrial excision was performed in all the patients. The surgical procedure was safely completed in all the patients. Though 14 patients had tumor invasion to the parametria, none of the patients had a positive surgical margin in the parametrium. The bladder function of patients in the UNS group immediately after surgery was more damaged than that in the BNS group. However, all the patients in both groups recovered spontaneous voiding with no need of self-catheterization during the perioperative periods. This preliminary study showed that the surgical technique is feasible and safe. For confirmation of the efficacy of this technique, further large prospective studies are needed.     

Physiological changes in the treatment of acrophobia (fear of height)

In order to investigate the physiological changes produced by the treatment of acrophobic patients body movement and Microvibration were measured before and after treatment. Eighteen acrophobic patients were assigned at random to 1 of the 2 groups: a treatment group (n= 8) and a non-treatment group (n= 10). The control group consisted of 16 healthy volunteers. Body movement area while viewing a slide of a high place or imagining a high place in the treatment group decreased significantly after treatment. Body movement of the control group showed almost no changes, and that of the non-treatment group was situated between the above-mentioned 2 groups. Simple body movements of the acrophobic patients without any stimulation of height were bigger than those of the control subjects. MV pattern of the treatment group had a tendency to improve under psychotherapy. Acrophobic patients had more abnormal MV patterns than the normal subjects.     

VACCINE-ASSOCIATED POLIOMYELITIS IN AN INFANT WITH AGAMMAGLO BULINEMIA

Abstract. Sakano, T., Kittaka, E., Tanaka, Y., Yamaoka, H., Kobayashi, Y. and Usui, T. (Department of Paediatrics, Hiroshima University Hospital and Hiroshima City Hospital, Hiroshima, Japan). Vaccine-associated poliomyelitis in an infant with agammagiobulinemia. Acta Paediatr Scand, 69:549, 1980.—We describe a female infant with agammaglobulinemia who contracted vaccine-associated poliomyelitis. Poliovirus type 2 was isolated from the initial stool specimen. In our patient, temporary changes in the cerebrospinal fluid resembled those in patients without immunodeficiencies, although gammaglobulin therapy had not yet been started. Pleocytosis was observed for a short time after viremia, but soon there was a return to normal without antibody production.     

A case of linear IgA buUous dermatosis with IgA anti-type VII collagen autoantibodies

Summary In this study we present a patient with the sublamina densa type of linear IgA bullous dermatosis (LABD). with IgA autoantibodies reactive with the 290-kDa type VII collagen (the epidermolysis bullosa acquisita (EBA) antigen) and with immunoblotting of normal human dermal extracts. The clinical and histological features of the present case were compatible with those of LABI) but quite different from those of RBA. Although EBA sera reacted with the bacterial fusion protein of the N-terminal globular (NC1) domain of type VII collagen, this patient's serum did not show reactivity. Furthermore, ultrastructural localization of target epitopes on the anchoring fibrils in this patient was considerably different from EBA. These results indicate that, whereas EBA antibodies react with the NC1 domain of type VII collagen, the epitope in this case is different from that of EBA (and is most likely on the central triple helical domain). This difference may be responsible for the clinical presentation in this patient being distinct from that of EBA.     

Prognosis of the middle-aged and aged patients with obstructive sleep apnea syndrome

Abstract The long-term natural course of obstructive sleep apnea syndrome (OSAS) is studied in order to determine whether severity of nocturnal oxygen desaturation associated with apnea/hypopnea, body mass index (BMI), and hypertension influence survival in young (<40 years), middle-aged (40–64 years), and aged (65 years) using a questionnaire survey. One hundred and forty-eight patients with OSAS aged 17–78 years (136 men, 12 women, mean 52.0±12.3 yean), who were diagnosed by standard polysomnography (PSG) between 1983 and 1993, participated in the study. The survey revealed 15 deaths and 101 survivors; 32 subjects could not be located. The survival rate was 71.4% (95% confidence interval:55.6–87.2%). The survival rate in patients with OSAS was significantly lower than that in the age and sex-adjusted control Japanese population (87.6%). In the young group, only one death (a 19–year-old woman) occurred unexpectedly during sleep. The patient showed an apnea/hypopnea index (AHI) of 33 per h and the length of time that the nocturnal oxygen saturation (SaO₂) fell below 90% (time of SaO₂ < 90%) of 205 min. The survival rate in middle-aged patients with OSAS was significantly lower than that in the age and sex-adjusted control Japanese population (79.2 vs 91.0%), but this pattern was not seen among the aged. Time of SaO₂ <90% was significantly longer in the middle-aged patients than in the aged patients, but AHI did not differ between the two groups. Moreover, it was significantly correlated with AHI in middle-aged patients, but not in the aged patients. The survival rate was significantly lower in patients with hypertension than in the patients without such complications in the middle-aged group (57.9 vs 90.4%). Cox proportional-hazard regression model including age, hypertension, BMI, AHI, lowest SaO₂, and time of SaO₂ <90% showed that hypertension was only significantly associated with lower survival rate in total group and middle-aged patients, but not in the young or aged patients. The prognosis in patients with OSAS may differ among the generations. The prognosis in the middle-aged population may depend on the role of OSAS on the complications of hypertension or severity of oxygen desaturation, but not on AHI only.     

大豆异黄酮抗血小板聚集作用的实验研究

[目的 ]研究大豆异黄酮对血小板聚集性的影响 .[方法 ]普通饲料中添加 2 0 g/kg大豆异黄酮混合物 ,饲喂GK/Jcl糖尿病大鼠 2 0周 ,测定其血小板聚集率 .利用ODS柱层析和高效液相色谱技术分离大豆异黄酮 ,体外观察大豆异黄酮单体对人血小板聚集率的影响 .[结果 ]长期食用大豆异黄酮的GK/Jcl糖尿病大鼠血小板聚集率显著降低 .在人体外血小板聚集实验中 ,大豆异黄酮均抑制腺嘌呤核苷二磷酸钠、胶原蛋白和肾上腺素诱导的血小板聚集 ,大豆异黄酮类型中金雀异黄素的最强 ,其 5 0 %抑制浓度值分别为 0 38,0 30 ,0 2 3mmol/L .[结论 ]大豆异黄酮具有血小板聚集抑制效应 ,在大豆抗血小板聚集中起着重要作用 .     

The real status of sudden infant death (SIDS) in forensic medicine

To comprehend the real status of sudden infant death syndrome (SIDS) in forensic medicine, a statistical study was conducted on 156 cases subjected to medico-legal autopsies at university medical schools throughout Japan from 1987 to 1991 and diagnosed as SIDS. Furthermore, 138 cases that had been subjected to autopsies at the Tokyo Medical Examiner's Office and given the diagnosis of SIDS were evaluated for comparison. It was noted that the incidence of SIDS diagnosed at each facility has increased.     

A Case of Pneumocytoma (So-Called Sclerosing Hemangioma) With Lymph Node Metastasis

A case of "sclerosing hemangionia" (pneumocytoma) of the lungwith lymph node metastasis is reported. A 22-year-old Japaneseman was found to have a well-defined round lesion in the rightlung (S⁷), which increased in size slightly during a 2-yearfollow-up period. He underwent right lower lobectomy with a preoperative diagnosisof a benign lung tumor. The pulmonary tumor revealed histologicalfeatures characteristic of "sclerosing hemangioma" of the lung,in addition to which there were many large polygonal foamy cells,forming tubular or papillary structures. These cells were foundby electron microscopy to contain numerous cytoplasmic lamellarbodies and showed a positive reaction with anti-surfactant apoproteinantibody immunohistochemically. Therefore, they were consideredto be cells differentiating toward type II pneumocytes. Reviewof 21 typical "sclerosing hemangionia" disclosed a few or somesuch foamy cells in 10 cases. A single hilar lymph node wasthe site of microscopic metastases, which consisted of "largeclear foamy cells" and smaller polygonal or round cells withslightly eosinophilic cytoplasm, both of which were componentsof the pulmonary "sclerosing hemangioma" This case supportsthe theory that "sclerosing hemangioma" is a neoplasm of typeII pneumocyte lineage. Although it is said to be benign, rarecases apparently show metastatic potential.     

Neuropathological Effects of Triphenyl Phosphite on the Central Nervous System of the Hen (Gallus domesticus)

The neurotoxic effects of single subcutaneous injections of1000 mg triphenyl phosphite (TPP)/kg body weight were investigatedin White Leghorn hens. At 7 days postexposure, birds began toshow signs of mild to moderate ataxia that progressed to severeataxia and paralysis at 21 days. Inhibition of whole brain neuropathytarget esterase was 85% at 48 hr and 73% by 21 days postexposure.After postexposure periods of 7, 14, and 21 days, hens werekilled and their brains and spinal cords were examined for degeneratingaxons and terminals using the Fink-Heimer silver impregnationmethod. A small amount of degeneration was noted at 7 days.By 21 days, dense degeneration was noted in the spinal graymatter and funiculi. Degeneration was also present in the granularcell layer of cerebellar folia I-VI and in nuclei and fibertracts of the medulla. Moderate to dense degeneration was alsoseen in several forebrain and midbrain areas including the paleostriatum,ansa lenticularis, the dorsointermediate thalamic nucleus, lateralspiriform, pedunculopontine tegmental, and lateral mesencephalicnuclei and in the deeper layers of the optic tectum. These resultsindicate that, in addition to affecting the spinal cord andbrainstem, exposure to TPP also damages higher order centersresponsible for processing and integrating sensorimotor, visual,and auditory information.