Nephrotic syndrome due to minimal-change disease superimposed on anti-glomerular basement membrane antibody positive glomerulonephritis; a case report.

BACKGROUND: The prognosis for renal function in anti-GBM glomerulonephritis (anti-GBM GN) is extremely poor, and when renal impairment progresses severely, it is difficult to expect improvement. In addition, it is also known that once the disease activity can be controlled by aggressive treatment, its recurrence is rare. We experienced an anti-GBM GN that improved from severe renal dysfunction and relapsed. A possible cause was the superimpose of nephrotic syndrome due to minimal change disease (MCD). CASE PRESENTATION: A 30-year-old man was admitted to our hospital because of general malaise, fever, oliguria and renal dysfunction. The patient's laboratory data showed serum creatinine as high as 6.6 mg/dl, and severe inflammation (C-reactive protein 20.6 mg/dl). Anti-glomerular basement membrane antibody (anti-GBM Ab) was detected in his serum, which led to the diagnosis of anti-GBM GN. Treatment was initiated with high-dose glucocorticoid (GC) and plasma exchange therapy (PE), and the patient's renal function and oliguria improved rapidly and he was discharged 40 days after admission. Renal biopsy findings showed cellular crescents associated with linear IgG depositions along the glomerular tufts compatible with anti-GBM GN, but only about one-third of the glomeruli was involved, suggesting that it still remains an early stage of the disease. However, 2 months after discharge, he had a relapse and was readmitted due to severe proteinuria with positive anti-GBM Ab. On the second admission, after high-dose GC and PE combined with intravenous cyclophosphamide, and remission was achieved. Despite the relatively minor renal biopsy findings, the patient showed rapid renal dysfunction and relatively rapid improvement with our treatment. Electron microscopy of the renal biopsy tissue showed significant foot process effacement on podocytes in the apparently normal glomeruli, without electron dense deposits. CONCLUSION: On the basis of clinical course and renal pathology, it is suggested that the present case was a rare complication of an early stage of anti-GBM GN and minimal change nephrotic syndrome. Although the simultaneous development of anti-GBM GN and MCD with anti-GBM antibody is unclear, it might have been precipitated by influenza infection or some unknown factor.

Characterization of a bacterial laminaribiose phosphorylase.

Bacterial laminaribiose phosphorylase (LBP(bac)) was first identified and purified from cell-free extract of Paenibacillus sp. YM-1. It phosphorolyzed laminaribiose into α-glucose 1-phosphate and glucose, but did not phosphorolyze other glucobioses. It slightly phosphorolyzed laminaritriose and higher laminarioligosaccharides. The specificity of the degree of polymerization of the substrate was clearly different from that of the enzyme of Euglena gracilis (LBP(Eug)): LBP(bac) was more specific to laminaribiose than LBP(Eug). It showed acceptor specificity in reverse phosphorolysis similar to LBP(Eug). Cloning of the gene encoding LBP(bac) (lbpA) has revealed that LBP(bac) is a member of the glucoside hydrolase family 94, which includes cellobiose phosphorylase, cellodextrin phosphorylase, and N,N'-diacetylchitobiose phosphorylase. The genes that encode the components of an ATP-binding cassette sugar transporter specific to laminarioligosaccharides were identified upstream of lbpA, suggesting that the role of LBP(bac) is to utilize laminaribiose generated outside the cell. This role is different from that of LBP(Eug), which participates in the utilization of paramylon, the intracellular storage 1,3-ß-glucan.

A Case of a Stafne Bone Defect Associated with Sublingual Glands in the Lingual Side of the Mandible.

A Stafne bone defect from the mandibular anterior to the premolar region is an extremely rare case. A case of a Stafne bone defect extending from the mandibular anterior to the premolar region was presented. Computed tomography (CT) and magnetic resonance imaging (MRI) suggested that salivary gland tissue connected to the sublingual glands was involved in the formation of the cavity. The patient was a 68-year-old man who was examined at our hospital's emergency outpatient department after a traffic accident. He was referred to our department for the treatment of contusions of the lips and oral cavity. A bone defect in the lingual side of the mandible from the right anterior to the right premolar region was incidentally detected on CT. CT showed a rounded cavity in the lingual side of the mandible that had a lingual opening, was monocystic, and had a cortical margin. The margin of the cavity was relatively dull and regular. MRI showed that the tissue filling the cavity in the lingual side of the mandible had similar signal intensity as the sublingual glands and was contiguous with the normal sublingual glands. Based on these findings, the bone defect was diagnosed as a Stafne bone defect filled with salivary gland tissue connected to the sublingual gland tissue.

[MRI-intracranial compliance analysis in patients with NPH].

We devised a method for non-invasively assessing intracranial compliance with retrospective ECG-triggered phase contrast cine MRI. This method was examined in patients with normal pressure hydrocephalus (NPH) group and those with asymptomatic ventricular dilation or brain atrophy (VD group), and in healthy volunteers (control group). Intracranial volume change (DeltaV(max)) was calculated from arterial inflow, venous outflow, cerebrospinal fluid (CSF) flow, and spinal cord motion at the C2 level during a cardiac cycle. Next, craniospinal CSF pressure gradient change (DeltaPG(max)) was calculated from measured CSF flow velocity using a simplified Navier-Stokes equation. Finally, Ci was obtained by dividing Delta V(max) into DeltaPG(max). Ci in the NPH group was significantly smaller and could be differentiated from other groups. This method makes it possible to non-invasively obtain a more detailed determination of the intracranial state and dynamics in NPH and to assist in its diagnosis.

High Performance, Low Temperature Solution-Processed Barium and Strontium Doped Oxide Thin Film Transistors.

Amorphous mixed metal oxides are emerging as high performance semiconductors for thin film transistor (TFT) applications, with indium gallium zinc oxide, InGaZnO (IGZO), being one of the most widely studied and best performing systems. Here, we investigate alkaline earth (barium or strontium) doped InBa(Sr)ZnO as alternative, semiconducting channel layers and compare their performance of the electrical stress stability with IGZO. In films fabricated by solution-processing from metal alkoxide precursors and annealed to 450 °C we achieve high field-effect electron mobility up to 26 cm2 V-1 s-1. We show that it is possible to solution-process these materials at low process temperature (225-200 °C yielding mobilities up to 4.4 cm2 V-1 s-1) and demonstrate a facile "ink-on-demand" process for these materials which utilizes the alcoholysis reaction of alkyl metal precursors to negate the need for complex synthesis and purification protocols. Electrical bias stress measurements which can serve as a figure of merit for performance stability for a TFT device reveal Sr- and Ba-doped semiconductors to exhibit enhanced electrical stability and reduced threshold voltage shift compared to IGZO irrespective of the process temperature and preparation method. This enhancement in stability can be attributed to the higher Gibbs energy of oxidation of barium and strontium compared to gallium.