Comparison of high-dose and low-dose corticosteroid therapy for refractory Mycoplasma pneumoniae pneumonia in children

Background

Mycoplasma pneumoniae pneumonia (MPP) is generally a self-limiting disease, but it may become refractory. It is thought that refractory MPP is linked to the excessive immunologic responses of the host. Consequently, the use of adjunctive systemic corticosteroids may have beneficial effects. In this study, we compared the effects of high- and low-dose corticosteroid therapy in a pediatric population with refractory MPP.

Clinical manifestations and epilepsy treatment in Japanese patients with pathogenic CDKL5 variants

ObjectivePatients with pathogenic cyclin-dependent kinase-like-5 gene (CDKL5) variants are designated CDKL5 deficiency disorder (CDD). This study aimed to delineate the clinical characteristics of Japanese patients with CDD and elucidate possible appropriate treatments.MethodsWe recruited patients with pathogenic or likely pathogenic CDKL5 variants from a cohort of approximately 1,100 Japanese patients with developmental and epileptic encephalopathies, who underwent genetic analysis. We retrospectively reviewed clinical, electroencephalogram, neuroimaging, and genetic information.ResultsWe identified 29 patients (21 females, eight males). All patients showed severe developmental delay, especially in males. Involuntary movements were observed in 15 patients. No antiepileptic drugs (AEDs) achieved seizure freedom by monotherapy. AEDs achieving ≥ 50% reduction in seizure frequency were sodium valproate in two patients, vigabatrin in one, and lamotrigine in one. Seizure aggravation was observed during the use of lamotrigine, potassium bromide, and levetiracetam. Adrenocorticotrophic hormone (ACTH) was the most effective treatment. The ketogenic diet (KD), corpus callosotomy and vagus nerve stimulation did not improve seizure frequency in most patients, but KD was remarkably effective in one. The degree of brain atrophy on magnetic resonance imaging (MRI) reflected disease severity. Compared with females, males had lower levels of attained motor development and more severe cerebral atrophy on MRI.ConclusionOur patients showed more severe global developmental delay than those in previous studies and had intractable epilepsy, likely because previous studies had lower numbers of males. Further studies are needed to investigate appropriate therapy for CDD, such as AED polytherapy or combination treatment involving ACTH, KD, and AEDs.     

Surface phenotypic changes of spleen cells on acquisition of encephalitogenicity

The relationship between surface marker expression and encephalitogenicity of spleen cells was studied in Lewis rats. Donors were sensitized with either BP/CFA or BP/IFA, or given BP-cultured naive spleen cells. The adoptive transfer of EAE was successfully achieved in every case after culture with BP, although only spleen cells from BP/CFA-sensitized rats proliferated significantly in response to BP. The observed encephalitogenicities were BP/CFA-sensitized cells greater than BP/IFA-sensitized cells much greater than cells from recipients of BP-cultured naive cells in descending order. In BP/CFA-sensitized cells, the expression of both W3/25 and OX-3 antigens on T cells increased markedly after culture with BP, but the expression of neither OX-19 nor OX-8 antigen increased significantly. Cells from neither BP/IFA-sensitized rats nor recipients of BP-cultured naive cells showed a significant change in the surface marker expression after culture with BP. Therefore, the generation of T cells coexpressing large amounts of both W3/25 and OX-3 antigens after culture with BP seems to correspond to the acquisition of the strong encephalitogenicity in vivo.     

Soluble and Cell-Associated Forms of Some Yet to be Identified Factor in Transfused Blood Which Promotes Solid Tumor Growth in Mice

Purpose The mechanism underlying the immunomodulation caused by blood transfusion has yet to be elucidated. The aim of the present study was to determine whether the transfusion of a soluble or insoluble factor present in stored blood can induce immunomodulation, which would thereby promote solid tumor growth.Methods C57Bl/6J mice were subcutaneously inoculated with B16-CG melanoma cells, which secrete -human chorionic gonadotropin (-hCG). Following inoculation, each of three different products of allogeneic and syngeneic blood were transfused on days 0 and 1: fresh whole blood, stored whole blood, and supernatants from the stored blood. Tumor growth was then monitored by measuring urinary -hCG. All mice were killed on day 15, and the tumor weight and volume were measured.Results Transfusion of all allogeneic blood products enhanced tumor growth, as did the stored syngeneic whole blood. Neither fresh syngeneic blood nor the supernatant from stored syngeneic blood promoted tumor growth. Although the tumors were not visually detectable until day 10 after inoculation, by day 7 the levels of urinary -hCG were significantly higher in the mice that received allogeneic blood supernatant than in the mice that received saline.Conclusions A soluble alloantigen enhances solid tumor growth, as does an insoluble factor present in stored syngeneic whole blood. The immunomodulation associated with this factor begins to enhance tumor growth within 7 days after transfusion.     

Phyllodes tumor in epileptics: A report of two cases

Phyllodes tumor is an uncommon breast neoplasm characterized by a proliferation of both stromal and epithelial elements. In 1989, two young patients with phyllodes tumors were referred to our surgical department because of the detection of breast lumps. Interestingly, both patients also had epilepsy and had been taking anticonvulsants. An analytical case control study revealed that no significant difference between the control group and phyllodes group was found for various categories. In addition, no anticonvulsant medication had been prescribed in either the control group or the phyllodes group except for these two cases. We herein report two cases of phyllodes tumors occurring in two young epileptic patients.     

The importance of postoperative choledochoscopy for management of retained biliarytract stones

From 1974-8, 808 postoperative choledochoscopy procedures, conducted by insertion of choledochofiberscope into the biliary tract through the sinus tract after the T-tube had been removed, were carried out in 292 patients at Teikyo University Hospital, Tokyo, Japan. In this series, 104 with retained biliary tract stones were encountered, and complete removal of stones was successfully carried out in 101, using postoperative choledochoscopy. Any failures of removal of retained biliary tract stones were attributed to improper insertion of the T-tube. The T-tube, of at least 18 French calibers should be inserted into the common bile duct at a right angle so as to obviate a tortuous sinus tract. The follow-up study in cases of complete extraction of the retained biliary tract stones showed that this approach is most effective. Recurrent stone with a silk nidus was found in one patient in whom postoperative choledochoscopy had been performed one year previously. Reoperation was carried out in this particular case. All other patients have remained asymptomatic. Finally, we advise routine use of postoperative choledochoscopy as an adjunct to the T-tube cholangiography, in order to prevent the possibility of retained biliary tract stones. Contents of this paper were read before the Annual Meeting of the American Society for Gastrointestinal Endoscopy, Digestive Disease Week '79 on May 22nd, 1979 in New Orleans, U.S.A.     

De novo CD5+ diffuse large B-cell lymphoma: a clinicopathologic study of 109 patients.

De novo CD5+ diffuse large B-cell lymphoma (CD5+ DLBCL) is known to have phenotypically and genotypically different characteristics than CD5- DLBCL and mantle cell lymphoma (MCL). To further characterize CD5+ DLBCL, 109 patients with CD5+ DLBCL were reviewed, and the results were compared with those of 384 CD5- DLBCL and 128 cyclin D1+ MCL patients. Patients with CD5+ DLBCL showed a higher age distribution (median, 66 years; P =.0083) and a female predominance (male-female ratio, 49:60, P =.011) compared with those with CD5- DLBCL. CD5+ DLBCL was more closely associated with many aggressive clinical features or parameters than CD5- DLBCL: 69% older than 60 years (P =.039), 34% with performance status greater than 1 (P =.0016), 69% with serum lactate dehydrogenase level higher than normal (P <.0001), 62% with stage III/IV disease at diagnosis (P =.0023), 35% with more than one extranodal site (P =.023), and 40% with B symptoms (P =.0031). The overall International Prognostic Index score was thus significantly higher for the patients with CD5+ DLBCL than for those with CD5- DLBCL (P =.00005). The most frequent site of extranodal involvement was bone marrow (28%), a higher frequency than that for CD5- DLBCL (P <.0001) but lower than that for cyclin D1+ MCL (P =.0015). Histopathologically, CD5+ DLBCL showed centroblastic morphology except for 3 patients with immunoblastic disease, and interfollicular growth pattern (7%) and intravascular or intrasinusoidal infiltration (19%) were observed. Immunophenotypically, CD5+ DLBCL was characterized by a CD5+CD10-CD19+CD20+CD21-CD23- cyclin D1- phenotype and a predominance of surface IgMkappa. Of particular interest is that CD5+ DLBCL was characterized by a survival curve significantly inferior to that for patients with CD5- DLBCL (P =.0026). These findings suggest that CD5+ DLBCL may constitute a unique subgroup of DLBCL.     

MK615 decreases RAGE expression and inhibits TAGE-induced proliferation in hepatocellular carcinoma cells

AIM:To investigate the proliferative effect of advanced glycation end-products(AGEs) and the role of their cellular receptor(RAGE) on hepatocellular carcinoma(HCC) cells,and the inhibitory effects of MK615,an extract from Japanese apricot,against AGEs were also evaluated.METHODS:Two HCC cell lines,HuH7 and HepG2,were used.Expression of RAGE was investigated by poly-merase chain reaction,Western blotting,and flow cytemetry(FACS).The effect of MK615 on RAGE expression was also evaluated by FACS.The proliferat...