Hemophagocytic syndrome associated with hypercytokinemia in a patients with rheumatoid arthritis]

A 65-year old female, who had been suffered from rheumatoid arthritis, was admitted to our hospital because of fever, oral ulcers, perianal skin ulcers, petechiae in the both legs, hepatosplenomegaly and cervical lymphadenopathy. Her laboratory data showed severe anemia, leukocytopenia, and thrombocytopenia as well as low PT activity, prolonged APTT, decreased fibrinegen and elevated FDP. In addition to raised values of liver enzymes and triglyceride, marked elevation of several cytokines were found. IgM and IgG class antibodies to cytomegalovirus were demonstrated positive and their titers were 2.60 and 938.0, respectively. The study for the aspiration of bone marrow revealed hemophagocytosis of erythrocytes, leukocytes and thrombocytes. Based upon these findings, she was diagnosed as having hemophagocytic syndrome associated with cytomegalovirus infection. Steroid treatment inducing mini-pulse therapy was introduced to her and bought full recovery from the illness. The association of hemophagocytic syndrome to rheumatoid arthritis was reviewed in the literature and five cases were documented to have good prognosis with steroid treatment.     

Concurrent occurrence of allergic granulomatous angiitis and temporal arteritis: a case report and review of the literature

Allergic granulomatous angiitis (AGA) is a disease entity that was first distinguished from classical polyarteritis nodosa by Churg and Strauss in 1951, and is characterized by the clinical features of allergic rhinitis or bronchial asthma, eosinophilia, and vasculitis. Allergic granulomatous angiitis has been described to mainly involve small vessels. We herein describe a case of Churg–Strauss syndrome which demonstrated the clinical and laboratory findings supporting a diagnosis of AGA and was also associated with the clinical and pathological findings for temporal arteritis, which was characterized by eosinophil infiltration and granuloma formation of the temporal artery (middle-sized vessel).     

Perinatal diagnosis of a fetus with an unbalanced translocation 46,XY,der(10)t(6;10)(p22;q26.1) with multiple malformations: a case report and literature review

The phenotype of an unbalanced translocation is characterized by the dosage effects of the affected genes in the translocated chromosome. We present the case of a fetus with a paternally derived unbalanced 46,XY,der(10)t(6;10)(p22;q26.1) translocation, detected following growth retardation and cardiac malformation. In trisomy 6p and 10q26 monosomy, external surface malformations, including characteristic facial abnormalities, and neurological or higher effects have been reported. Developmental delay and hypotonia are reported in ≤ 80% of cases of 10q monosomy. Herein, low birth weight, cephalic abnormalities including microcephaly, low-set ears and a high arched palate, ambiguous genitalia including scrotal hypoplasia and cryptorchidism, and congenital heart defects, including ventricular septal defect and pulmonary atresia, were observed. Neurological impact was not evaluated due to neonatal death. The mortality rate and frequency of low birth weight in such translocations has been seldom reported. In this case, severe cardiac malformation and low birth weight may have caused early neonatal death. Whilst Trisomy 6 is associated with low birth weight and perinatal death, few studies have reported these outcomes in 10q26 deletion syndrome. Our findings therefore contribute to the evidence base regarding unbalanced translocations and may improve the clinical management of such patients.     

Effect of proinflammatory diet before pregnancy on gestational age and birthweight: The Japan Environment and Children's Study

The daily diet plays a role in systematic inflammation and may be one of the causes of preterm birth. We aimed to examine the effect of a daily proinflammatory diet before pregnancy on gestational age and birthweight using a large birth cohort in Japan. We used data of singleton pregnancies in the Japan Environment and Children's Study involving live birth from 2011 to 2014 to calculate the dietary inflammatory index. We used individual meals with 30 food parameters from a semiquantitative food frequency questionnaire, which assessed diet intake before pregnancy. Participants were categorized according to the quartile of dietary inflammatory index. A multiple logistic regression model was used to estimate the risk of a proinflammatory diet on preterm birth (PTB) before 37 or 34 weeks and low birthweight (LBW) less than 2,500 or 1,500 g, accounting for maternal age, body mass index before pregnancy, smoking status, education, and household income. After applying our inclusion criteria, 89,329 participants were eligible for the present study. Multiple regression analysis showed that the proinflammatory diet had an increased risk of PTB < 34 weeks (adjusted odds ratio: 1.29, 95% confidence interval [1.07, 1.55]) and <2,500‐g LBW (adjusted odds ratio: 1.08, 95% confidence interval [1.01, 1.16]) compared with the control. In conclusion, a proinflammatory diet before pregnancy was a risk factor for PTB < 34 weeks and LBW < 2,500 g. Therefore, proinflammatory diet needs to be controlled to improve perinatal prognosis.     

Expression of matrix metalloproteinases 2 and 9 and tissue inhibitors of matrix metalloproteinases 2 and 1 in the glomeruli of human glomerular diseases: the results of studies using immunofluorescence, in situ hybridization, and immunoelectron microscopy

Background

It has been reported matrix metalloproteinases (MMPs) and their inhibitors, tissue inhibitors of matrix metalloproteinases (TIMPs), play important roles in the decomposition of the extracellular matrices of the glomerulus during the pathological processes in various glomerular diseases. Although the activity of these enzymes in cases of experimental glomerulonephritis has been described, the expression sites in the glomeruli of human renal diseases have been identified in only a few articles and remain controversial.

Methods

The expression of the gelatinase group of MMPs (MMP-2 and MMP-9) and their inhibitors (TIMP-2 and TIMP-1) were evaluated in 19 renal biopsies of several types of glomerular diseases by immunofluorescence (IF) labeling. In addition, several samples of immunoglobulin A nephropathy (IgAN) were also investigated by in situ hybridization (ISH) and immunoelectron microscopy (IEM).

Results

The expression of MMP-2 was observed in all the cases examined by IF and ISH. TIMP-2 expression varied from negative to positive among 11 cases of IgAN, but was negative in the cases with lupus nephritis (LN) (n?=?3), membranoproliferative glomerulonephritis (MPGN) (n?=?2), and post-streptococcal glomerulonephritis (n?=?1). However, it was weakly positive in the cases of diabetic nephropathy (DMN) (n?=?2). MMP-2 was mainly observed along glomerular capillary loops (GCLs) and Bowman??s capsules, whereas TIMP-2 was found in the mesangial area. The expression of MMP-9 in cases of IgAN varied, and was local, not diffuse, if it was present. MMP-9 expression in cases of LN, MPGN, and DMN was diffuse, but the intensity of staining varied. MMP-9 was primarily expressed in the mesangium. TIMP-1 expression was negative in all cases except for those with IgAN. The localization of MMP-2 in patients with IgAN, which was investigated by IEM, was revealed to be mainly on the endothelial cell membranes of GCLs, podocyte membranes, the parietal cell membranes of Bowman??s capsules, and some on the membranes of mesangial cells.

Conclusion

The study results suggest that the expression levels and patterns of MMPs and TIMPs are generally similar in several types of glomerular diseases, even though each case has a somewhat different distribution and intensity of expression. When these enzymes were present, their main sites were as follows: MMP-2 was found along glomerular basement membrane, TIMP-2 was located in the acellular mesangial area, MMP-9 was seen in the mesangium, and TIMP-1 was hardly detected. MMP-2 expression is clearly demonstrated to exist at the above-described sites by IEM in patients with IgAN.     

General corrosion during metal-assisted etching of n-type silicon using different metal catalysts of silver,gold, and platinum

Metal-assisted etching is a promising technique for microfabrication of semiconductors. In this method, porous silicon (Si) can be produced with a very simple procedure, and various nanostructures can be designed by changing the catalyst patterns. The kind of metal catalysts is one of the key factors to control the porous structure. In this work, we performed the etching of n-type Si (100) in a hydrofluoric acid solution containing hydrogen peroxide in the dark using silver, gold, and platinum particles electrolessly deposited at a constant coverage, and demonstrated the difference in the porous structures obtained for the different kind of metal catalysts. By comparing the mass loss of substrates with the depth of pores formed under the metal particles, we found that general corrosion occurred on the top-surface of the Si substrate around the metal particles even under the dark condition. The general corrosion depended on the metal species and it was explained by the formation and dissolution of a mesoporous layer. The kind of metal catalysts influences the dissolution of the Si surface not only under the metal catalysts but also between them.

The first report on general corrosion during metal-assisted etching of silicon.     

Matrix metalloproteinase-2, matrix metalloproteinase-9, and tissue inhibitor of metalloproteinase-1 in the peripheral blood of patients with various glomerular diseases and their implication in pathogenetic lesions: study based on an enzyme-linked assay and immunohistochemical staining

Background Various glomerular diseases progress to end-stage renal failure due to an accumulation of the mesangial matrix (MM) and a thickening of the glomerular basement membrane (GBM). Both the MM and GBM are consistently metabolized through the synthesis and destruction of the matrix. Such synthesis is influenced by transforming growth factor-β (TGF-β) and other factors, whereas the destruction is presumed to be mediated by both matrix metalloproteinases (MMPs) and inhibitors of matrix metalloproteinases (TIMPs). Based on such evidence, we tried to detect MMP-2, MMP-9, and TIMP-1 in the peripheral blood of patients with various glomerular diseases. Methods Serum was used to detect MMP-2 and TIMP-1, while plasma was used to detect MMP-9. These enzymes were detected using an enzyme-linked assay. Results The findings showed an increased level of MMP-2 in patients with a alteration of GBM, typically membranous nephropathy (MN), regardless of the differences in their etiological processes. In contrast, MMP-9 did not show a strong association with any specific glomerular abnormalities. However, it mainly tended to increase in patients with MM accumulation. In addition, the localization of MMP-2, MMP-9, and TGF-β1 was studied using immunohistochemical staining. MMP-2 was demonstrated to exist in the glomerular capillary loop (GCL) as well as in the mesangial cells and the mesangial matrix. MMP-9 was found to exist in mesangial cells and the matrix, GCL, infiltrated neutrophils, and some tubular epithelial cells. Positive staining for TGF-β1 in GCL was found to be associated with an increased level of MMP-2 in patients with MN, whereas in MM such positive staining was not necessarily associated with an increased level of MMP-9. Conclusions These results therefore suggest that MMP-2 plays an important role in the degradation of GBM, while MMP-9 only moderately affects the degradation of MM.     

Pregnancy complicated by central diabetes insipidus and oligohydramnios

This is the fourth reported case of central diabetes insipidus with oligohydramnios. Central diabetes insipidus does not adversely affect pregnancy; it can present with oligohydramnios, which can be improved by treating central diabetes insipidus.