ABSTRACT
Plasma exchange is an effective treatment for Kawasaki disease (KD), suggesting that plasma from patients with KD bears its causative agents. The aim of this study was to use mass spectrometry to identify candidate agents in patient sera. Serum samples were obtained from 17 KD patients. In six patients, samples were collected in each of three phases: the acute phase prior to acetylsalicylic acid (ASA) and intravenous immunoglobulin administration (Phase A1), the remission phase with ASA (Phase A2), and the remission phase without any medication (Phase A3). Sera from the remaining 11 patients were collected during Phases A1 and A2. The study also included two age- and gender-matched control groups, one with eight afebrile children and one with eight febrile children diagnosed with infectious disease. Patients in Phase A1 and febrile controls did not differ in body temperature, white blood cell counts, or C-reactive protein levels. Mass spectrometry analysis revealed that the intensity levels of m/z 9416, identified as apolipoprotein CIII (Apo CIII), were lower in Phase A1 samples compared with samples from patients in Phases A2 and A3, and from febrile controls (all comparisons, p < 0.01). Serum Apo CIII levels were also lower in Phase A1 samples compared with samples from Phase A2 patients and afebrile controls (both p < 0.01), but samples from patients in Phase A2 did not differ significantly from those of the afebrile controls (p = 0.55). This study demonstrated that serum Apo CIII level was decreased in the acute phase of KD.
ABSTRACT
In kidney transplant recipients (KTRs), viral infection can lead to antibody and/or T-cell mediated rejection, resulting in kidney transplant dysfunction. Therefore, it is critical to prevent infections. However, KTRs exhibit suboptimal responses to SARS-CoV-2 and/or influenza vaccines, partly due to immunosuppressant therapy. Inter- and intra-individual differences in the biological responses to vaccines may also affect patients' antibody production ability. This study included KTRs who received an messenger RNA SARS-CoV-2 vaccine (3 doses), and an inactivated quadrivalent influenza vaccine (1 or 2 doses). We measured the patients' total antibody titers against SARS-CoV-2 spike antigen, and hemagglutination inhibition (HI) titers against influenza A/H1N1, A/H3N2, B/Yamagata, and B/Victoria. Five patients were eligible for this study. Of these 5 KTRs, two produced anti-SARS-CoV-2 spike antibody titers to a seroprotective level, and also produced HI titers against A/H1N1 to a seroprotective level. Another 2 KTRs did not produce seroprotective anti-SARS-CoV-2 antibody titers, but produced seroprotective HI titers against A/H1N1. The remaining KTR produced a seroprotective anti-SARS-CoV-2 antibody titer, but did not produce a seroprotective HI titer against A/H1N1. The 2 KTRs who did not produce seroprotective anti-SARS-CoV-2 antibody titers following vaccination, later developed COVID-19, and this infection increased their titers over the seroprotective level. This study demonstrated that inter- and intra-individual differences in biological responses to vaccines should be considered in pediatric KTRs, in addition to immunosuppressant effects. Personalized regimens, such as augmented or booster doses of vaccines, could potentially improve the vaccination efficacy against SARS-CoV-2 and influenza.
Subject(s)
Antibodies, Viral , COVID-19 Vaccines , COVID-19 , Influenza Vaccines , Influenza, Human , Kidney Transplantation , SARS-CoV-2 , Humans , Influenza Vaccines/administration & dosage , Influenza Vaccines/immunology , Male , Female , COVID-19/prevention & control , COVID-19/immunology , COVID-19 Vaccines/administration & dosage , COVID-19 Vaccines/immunology , Influenza, Human/prevention & control , Influenza, Human/immunology , SARS-CoV-2/immunology , Antibodies, Viral/blood , Child , Adolescent , Transplant Recipients , Influenza A Virus, H1N1 Subtype/immunology , Vaccination/methodsABSTRACT
AIMS: Patients with atrophic gastritis unrelated to autoimmune gastritis (AIG) and without active Helicobacter pylori (H.pylori) infection or previous eradication therapy are considered to have previous Helicobacter pylori infection-induced atrophic gastritis (PHIG). This study aimed to clarify the clinical characteristics of patients with PHIG. METHODS: Consecutive patients who underwent upper gastrointestinal endoscopy during the study period were enrolled in the study. Pepsinogen and gastrin levels, H. pylori serology, and endoscopic atrophic grade were assessed. Patients were divided into five groups based on their H. pylori status and disease history (PHIG, without H. pylori infection, with active H. pylori infection, with successful H. pylori eradication, and AIG). Their gastric cancer risk status was classified according to the ABC method of serological gastric cancer screening. RESULTS: Of 536 consecutive patients who underwent upper gastrointestinal endoscopy during the study period, 318 were included (31 with PHIG, 77 without H. pylori infection, 101 with active H. pylori infection, 80 with successful H. pylori eradication, and 29 with AIG). Of the 31 patients with PHIG, 21 (68%) were H. pylori-seronegative, and 20 (65%) were classified as group A (normal pepsinogen, H. pylori-seronegative). Patients with PHIG accounted for 90.1% of the patients at high risk for gastric cancer misclassified as group A. The pepsinogen and H. pylori serological profiles of patients with PHIG were similar to those of patients with successful H. pylori eradication more than six years previously. A receiver-operating characteristic curve (ROC) analysis that included 13 patients with AIG and without active H. pylori infection and no previous eradication therapy and 31 patients with PHIG revealed that an endoscopic atrophy grade of O-III or greater according to the Kimura-Takemoto classification can predict AIG. CONCLUSIONS: Two-thirds of the patients with PHIG were misclassified as being at low risk (group A) according to the ABC method, suggesting that endoscopy is necessary for group A patients. The results of the serological evaluation of PHIG indicated that patients with PHIG may have experienced spontaneous H. pylori eradication, possibly because of the use of antibiotics for other conditions. Autoimmune gastritis should be considered in the presence of grade 0-III or greater gastric mucosal atrophy in patients with suspected PHIG, even if the autoantibody and histological findings are not available.
ABSTRACT
A 60-year-old woman with a history of neurofibromatosis type 1, who was admitted with pulmonary hypertension, developed buttock pain and anemia, and contrast-enhanced computed tomography showed a large subcutaneous hematoma with minimal active extravasation. Angiography of the bilateral internal iliac arteries revealed diffuse, irregular blood vessels without extravasation. As the exact bleeding site could not be identified, the patient was managed conservatively. However, the patient's symptoms and anemia worsened the following day. Repeat angiography revealed two pseudoaneurysms in the right inferior gluteal artery, which were embolized using n-butyl-2-cyanoacrylate. Nonetheless, the patient's anemia further worsened the following day. Repeat contrast-enhanced CT revealed another site of extravasation in the enlarging hematoma, but no extravasation was observed on the subsequent angiography. Owing to the worsening anemia and enlarging hematoma, proximal embolization of the irregular bilateral inferior gluteal arteries was performed using gelatin sponge particles. The patient's anemia and symptoms improved. Vasculopathy associated with neurofibromatosis type 1 is rare, with an incidence of approximately 3%. In patients with neurofibromatosis type 1, the blood vessels become fragile because of tunica media thinning and elastic-lamina rupture. Histopathologically, neurofibromatosis type 1-associated vasculopathy is characterized by a mixture of normal and abnormal vessels. Abnormally fragile blood vessels may repeatedly rupture followed by physiological hemostasis, which may explain the diagnostic and therapeutic challenges during angiography in this case. In patients with neurofibromatosis type 1 with acute bleeding, irregular vessels without active extravasation on angiography may be indicated for embolization.
ABSTRACT
Heat stroke may cause multi-organ dysfunction and death. Some patients with neurological abnormalities in the acute phase have neurological sequelae, particularly cerebellar ataxia, in the recovery phase. However, there is no method to predict the neurological prognosis, and the usefulness of imaging has not yet been established. We report the case of an 86-year-old woman with dementia brought to our emergency department in a coma and hyperthermia. The patient was diagnosed with heat stroke and promptly treated in the ICU but remained unconscious. The patient gained consciousness on day 19, but difficulty with stillness associated with cerebellar ataxia in her right upper extremity became apparent. On day 1, head magnetic resonance imaging (MRI) showed no obvious abnormality. However, on day 6, high-signal areas, suggestive of edema, were seen in the bilateral cerebellar hemispheres. Single-photon emission computed tomography (SPECT) on day 9 revealed significant hypoperfusion in the right cerebellum. These changes improved at the time of hospital discharge. This was a case of persistent cerebellar ataxia due to heat stroke, in which imaging findings improved over time. In most cases, MRI findings do not match clinical symptoms. However, the low cerebral blood flow in the early SPECT images was consistent with the clinical symptoms. MRI may not be a prognostic indicator; however, SPECT images may be useful for predicting sequelae.
ABSTRACT
"Welcome to OBGYN World!" A novel recruitment event for medical students organized by the Japan Society of Obstetrics and Gynecology. Since 2012, the number of doctors in Japan who specialize in obstetrics and gynecology has shown a decreasing trend. To increase the number of doctors majoring in obstetrics and gynecology, the Japanese Trainees in Obstetrics and Gynecology subcommittee developed a new recruitment event called Welcome to OBGYN World! (WOW!); the aim of this event was to focus on lower grades of medical students. The present report describes the content of WOW! and the results of a post-event questionnaire administered to participating students and tutors. WOW! was held online in order to avoid the risk of Coronavirus Disease 2019 infection for participants. Sixty of the 82 medical schools nationwide (73.2%) participated in this event. Overall, there were 285 participating students, ranging from first to fourth grade in medical school, and 106 tutors were involved to teach material at the event. In the post-event questionnaire survey, 97.6% (248/254) and 100% of the participants stated they now had a high degree of interest in obstetrics and gynecology and found the specialty attractive, respectively. Furthermore, 93.6% (90/94) of the tutors stated that WOW! had helped recruitment activities in their universities. Based on this outcome, members of the Japanese Trainees of Obstetrics and Gynecology subcommittee will now try to increase the number of doctors specializing in obstetrics and gynecology by holding WOW! annually.
Subject(s)
COVID-19 , Gynecology , Obstetrics , Students, Medical , Female , Pregnancy , Humans , Gynecology/education , Obstetrics/education , JapanABSTRACT
RATIONALE: Vacuolated podocytes are the most common form of renal damage in Fabry disease, but other types of renal damage have been reported, such as membranous nephropathy (MN) or IgM nephropathy. Enzyme replacement therapy (ERT) is effective at preventing renal damage, but the nephropathies require appropriate treatment to prevent renal damage. PATIENT CONCERNS: A 22-year-old male with Fabry disease presented with proteinuria during ERT with agalsidase-ß and carbamazepine. He had received the treatment for 10âyears and maintained normal plasma globotryaosylceramide levels. DIAGNOSIS: Renal biopsy revealed MN without vacuolated podocytes. Immunofluorescent staining of the IgG subclass revealed granular patterns of IgG1, G2, G4, and C3 deposition in the glomerular basement membrane. INTERVENTIONS: The carbamazepine dose was reduced from 600âmg/day to 200âmg/day (serum concentration 10.0-11.0-4.0-5.0âµg/mL). OUTCOMES: After reducing the carbamazepine dose, proteinuria was negative, and the patient has had a normal urinalysis for 17âmonths. Plasma globotryaosylceramide levels have also remained normal. LESSONS: This report is a reminder of the co-existence of MN without vacuolated podocytes in Fabry disease during ERT with agalsidase-ß and carbamazepine.Physicians should be aware of this form of renal damage in Fabry disease, even during treatment.
Subject(s)
Fabry Disease , Glomerulonephritis, Membranous , Podocytes , Adult , Carbamazepine/therapeutic use , Enzyme Replacement Therapy , Fabry Disease/complications , Fabry Disease/drug therapy , Glomerulonephritis, Membranous/complications , Glomerulonephritis, Membranous/drug therapy , Glomerulonephritis, Membranous/pathology , Humans , Male , Podocytes/pathology , Young AdultABSTRACT
AIM: Glucocorticoids (GC) are essential medicines for idiopathic steroid-sensitive nephrotic syndrome (ISSNS) and IgA nephropathy (IgAN), with good clinical results. However, they cause bone fragility. The aim of this study was to elucidate GC effects on bone strength assessed as bone mineral density (BMD) and bone quality, using bone turnover markers (BTM), in children with ISSNS or IgAN. METHODS: Eleven children with ISSNS and 13 with IgAN were included. All the patients received GC treatment according to each protocol. The BMD and BTM-serum alkaline phosphatase (S-ALP), tartrate-resistant acid phosphatase 5b (S-TRACP-5b), and undercarboxylated osteocalcin (S-ucOC)-were measured from the initiation of steroid treatment (STx) to the end of STx in both groups. RESULTS: In ISSNS, S-ALP and S-ucOC levels were decreased significantly at 1 month. BMD and S-TRACP-5b levels showed no significant change through this observation period. In IgAN, BMD and S-ALP levels were decreased significantly at 1 and 3 months, respectively, and recovered to baseline at 10 months after the initiation of GC dosage reduction. S-TRACP-5b levels were decreased significantly at 3 months and remained lower than at baseline through the observation period. In both groups, S-ucOC levels did not directly reflect bone strength. CONCLUSION: This study clarified the following three points regarding GC effects on bone strength in children with ISSNS or IgAN: first, S-ALP is a more sensitive bone quality marker than S-TRACP-5b; second, BMD loss was observed only when both S-ALP and S-TRACP-5b levels decreased, and third, S-ucOC levels do not directly reflect bone strength.
Subject(s)
Bone Density/drug effects , Bone Remodeling/drug effects , Glomerulonephritis, IGA/drug therapy , Glucocorticoids/adverse effects , Nephrotic Syndrome/drug therapy , Adolescent , Alkaline Phosphatase/blood , Biomarkers/blood , Child , Child, Preschool , Female , Glomerulonephritis, IGA/diagnosis , Humans , Male , Nephrotic Syndrome/diagnosis , Osteocalcin/blood , Tartrate-Resistant Acid Phosphatase/blood , Treatment OutcomeABSTRACT
In individuals treated with immunosuppressive therapies, the varicella-zoster virus (VZV) infection can become disseminated and lead to a life-threatening condition. There is currently no established treatment strategy for this life-threatening condition. Here, we describe a case where plasma exchange (PE) with a high dose of acyclovir (ACV) ameliorated the severe effects, including VZV-hemophagocytic lymphohistiocytosis (VZV-HLH) and disseminated intravascular coagulation (DIC), in a 9-year-old girl with steroid-dependent nephrotic syndrome. This 9-year-old girl experienced frequent relapse steroid-dependent nephrotic syndrome. She had been treated with steroids, tacrolimus, mizoribine, and rituximab. She had not previously received a varicella vaccine. She was admitted with only one vesicular rash. At admission, a serum test revealed 1.6 × 106 copies/mL of VZV DNA. The patient rapidly developed VZV-HLH and DIC. A combination of a high dose of ACV, immunoglobulin, and steroid pulse therapy could not improve these severe complications. Therefore, PE was applied. PE with a high dose of ACV successfully reduced serum VZV DNA from 7.5 × 106 to 2.8 × 104 copies/mL. This reduction in the VZV DNA copy number suggested that the combination of PE and a high dose of ACV was effective in treating a disseminated VZV infection. To the best of our knowledge, this is the first report showing that PE with a high dose of ACV ameliorated the severe complications of disseminated VZV by reducing the VZV DNA copy number.
Subject(s)
Acyclovir/therapeutic use , Chickenpox/therapy , Nephrotic Syndrome/complications , Plasma Exchange/methods , Acyclovir/administration & dosage , Antiviral Agents/administration & dosage , Antiviral Agents/therapeutic use , Chickenpox/complications , Chickenpox/immunology , Child , Combined Modality Therapy , Disseminated Intravascular Coagulation/complications , Disseminated Intravascular Coagulation/immunology , Disseminated Intravascular Coagulation/therapy , Female , Herpesvirus 3, Human/drug effects , Herpesvirus 3, Human/genetics , Herpesvirus 3, Human/isolation & purification , Humans , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/immunology , Lymphohistiocytosis, Hemophagocytic/therapy , Nephrotic Syndrome/drug therapy , Nephrotic Syndrome/immunology , Nephrotic Syndrome/virology , Treatment OutcomeABSTRACT
BACKGROUND: We previously reported that the top-down approach (TDA) for infants with febrile urinary tract infections (fUTI) could prevent recurrent fUTI (r-fUTI) but produced a high number of false-positives on acute-phase 99m Tc dimercaptosuccinic acid (DMSA) renal scintigraphy. Therefore we compared the ultrasonography-oriented approach (USOA) with TDA from the viewpoint of prevention of r-fUTI. METHODS: The TDA was applied between July 2010 and February 2014 and the USOA was applied between March 2014 and April 2017 in infants with first fUTI. In the USOA group, voiding cystourethrography (VCUG) was performed in the case of abnormality on acute-phase renal bladder ultrasonography (RBUS) or on chronic- phase DMSA, which were performed in all cases. The frequency of r-fUTI was compared between the TDA group and USOA group retrospectively. RESULTS: Seventy-four infants (52 male) and 79 infants (60 male) received TDA or USOA, respectively. No significant differences were found between the TDA and USOA groups in male : female ratio, age in months at initial onset of fUTI, observation period, or number of cases of r-fUTI (TDA group, n = 4; USOA group, n = 5). Seventy-four DMSA scintigraphy and 25 VCUG were carried out in the USOA group, and 111 DMSA scintigraphy and 34 VCUG in the TDA group. CONCLUSIONS: Both USOA and TDA were valid for prevention of r-fUTI, but USOA was superior to TDA with regard to the reduced number of patients undergoing VCUG and DMSA.
Subject(s)
Fever/etiology , Secondary Prevention/methods , Urinary Tract Infections/diagnostic imaging , Urinary Tract Infections/prevention & control , False Positive Reactions , Female , Follow-Up Studies , Humans , Infant , Male , Radionuclide Imaging , Radiopharmaceuticals , Recurrence , Retrospective Studies , Technetium Tc 99m Dimercaptosuccinic Acid , Ultrasonography , Urinary Tract Infections/complicationsABSTRACT
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder. ADPKD is characterized clinically by the presence of multiple bilateral renal cysts that lead to chronic renal failure. The cysts evolve from renal tubular epithelial cells that express the Klotho gene. Notably, Klotho acts as a co-receptor for fibroblast growth factor 23 (FGF23); in this context, it induces phosphaturia and maintains serum phosphate at a normal level. Many reports have shown that decreases in the soluble Klotho level and increases in the FGF23 level are associated with glomerular filtration rate (GFR) decline, but a recent study observed these changes in patient with normal eGFR. It remains unclear whether the decrease in the Klotho level precedes the increase in FGF23. Here, we present an ADPKD patient with enlarged kidneys due to multiple cysts who had a decreased soluble Klotho level but a normal eGFR and a normal FGF23 level. The patient's serum phosphate level was normal, as was the fractional excretion of phosphate (FEP). This appears to be the first reported case to show a decreased soluble Klotho level plus normal eGFR, FGF23, and FEP. These results suggest that Klotho decreases before FGF23 increases and further suggest that Klotho is not required to maintain normal serum phosphate levels in ADPKD if the FEP and serum phosphate levels are normal.
Subject(s)
Glucuronidase/metabolism , Phosphates/blood , Polycystic Kidney, Autosomal Dominant/metabolism , Polycystic Kidney, Autosomal Dominant/pathology , Angiotensin II Type 1 Receptor Blockers/therapeutic use , Benzimidazoles/administration & dosage , Benzimidazoles/therapeutic use , Biphenyl Compounds/administration & dosage , Biphenyl Compounds/therapeutic use , Calcium Channel Blockers/therapeutic use , Child, Preschool , Cysts/diagnostic imaging , Cysts/pathology , Dihydropyridines/administration & dosage , Dihydropyridines/therapeutic use , ErbB Receptors/metabolism , Fibroblast Growth Factor-23 , Fibroblast Growth Factors/metabolism , Glomerular Filtration Rate/physiology , Humans , Kidney/diagnostic imaging , Kidney/metabolism , Kidney/pathology , Klotho Proteins , Magnetic Resonance Imaging , Male , Phosphates/urine , Polycystic Kidney, Autosomal Dominant/diagnosis , Polycystic Kidney, Autosomal Dominant/drug therapy , Tetrazoles/administration & dosage , Tetrazoles/therapeutic useABSTRACT
BACKGROUND: C1q nephropathy (C1qN) was first described as glomerular disease characterized by predominant meangial C1q deposits in patients with proteinuria and no evidence of systemic lupus erythematosus. Several studies, however, revealed the clinical heterogeneity of C1qN, showing some cases with normal urinalysis. To confirm the existence of cases with predominant mesangial C1q deposits and negative or mild proteinuria and/or hematuria, we investigated renal graft biopsy specimens showing negative to mild proteinuria (less than or equal to 1+ by dip stick test) and/or hematuria. METHODS: Eligible participants were kidney transplant cases who corresponded to the criteria for C1qN and were followed more than 10 years. Their medical records were reviewed to determine the age at detection of predominant mesangial C1q deposits, gender, original renal disease and reason for renal graft biopsy, blood pressure, degree of proteinuria and hematuria, and serum creatinine levels. RESULTS: From 414 cases in adults and children, five pediatric patients (the male to female ratio, 1:1.5) were eligible. At the time when predominant mesangial C1q deposits were detected, 2 cases presented with mild proteinuria without hematuria, but the other 3 cases showed normal urinalysis. Light microscopy revealed minor glomerular abnormality in all the cases. Immunofluorescent study showed predominant mesangial C1q deposits with IgG, IgM and C3 in all cases. All selected specimens presented electron dense-depos in the mesangium. Ten years later from the detection, 2 cases continued to be normal urinalysis and 3 cases had mild proteinuria without hematuria. During this follow-up period, no cases presented with persistent proteinuria and/or hematuria greater than or equal to 2+ by dip stick test. And no cases developed systemic lupus erythematosus. Follow-up renal graft biopsies were performed once in 2 cases 8 years later from the detection. They showed minor glomerular abnormalities. C1q deposit disappeared in one case. In another case, immunofluorescent study was not examined. CONCLUSIONS: This long-term observational study on transplanted kidneys confirms the existence of cases with predominant but silent C1q deposits in the mesangium who have negative or mild proteinuria.
Subject(s)
Complement C1q/analysis , Glomerular Mesangium/immunology , Glomerular Mesangium/pathology , Kidney Diseases/immunology , Kidney Diseases/pathology , Kidney Transplantation/adverse effects , Adolescent , Adult , Biopsy , Child , Child, Preschool , Female , Follow-Up Studies , Hematuria/pathology , Humans , Longitudinal Studies , Male , Postoperative Complications/immunology , Postoperative Complications/pathology , Proteinuria/pathology , Urinalysis , Young AdultABSTRACT
The utility of non-enhanced magnetic resonance imaging (MRI) has not been examined extensively for diagnosing acute pyelonephritis (APN) in children. The aims of this study were to compare non-enhanced MRI with technetium-99 m dimercaptosuccinic acid (99m Tc-DMSA) renal scintigraphy in detecting APN. Six boys and one girl with temperature ≥38°C and positive urine culture received both non-enhanced MRI with whole body diffusion-weighted imaging (DWI) and 99m Tc-DMSA scintigraphy ≤7 days from the fever onset. The sensitivity and specificity of MRI in detecting APN lesions diagnosed on 99m Tc-DMSA scintigraphy were 80% and 100%, respectively. Non-enhanced MRI in children with suspected APN ≤7 days from fever onset might be a suitable replacement for 99m Tc-DMSA scintigraphy for the detection of APN.
Subject(s)
Diffusion Magnetic Resonance Imaging/methods , Kidney/diagnostic imaging , Pyelonephritis/diagnostic imaging , Radionuclide Imaging/methods , Female , Humans , Infant , Infant, Newborn , Kidney/pathology , Male , Prospective Studies , Radiopharmaceuticals , Sensitivity and Specificity , Technetium Tc 99m Dimercaptosuccinic AcidABSTRACT
Self-assembled monolayers (SAMs) have been used to elucidate interactions between cells and material surface chemistry. Gold surfaces modified with oligopeptide SAMs exhibit several unique characteristics, such as cell-repulsive surfaces, micropatterns of cell adhesion and non-adhesion regions for control over cell microenvironments, and dynamic release of cells upon external stimuli under culture conditions. However, basic procedures for the preparation of oligopeptide SAMs, including appropriate cleaning methods of the gold surface before modification, have not been fully established. Because gold surfaces are readily contaminated with organic compounds in the air, cleaning methods may be critical for SAM formation. In this study, we examined the effects of four gold cleaning methods: dilute aqua regia, an ozone water, atmospheric plasma, and UV irradiation. Among the methods, UV irradiation most significantly improved the formation of oligopeptide SAMs in terms of repulsion of cells on the surfaces. We fabricated an apparatus with a UV light source, a rotation table, and HEPA filter, to treat a number of gold substrates simultaneously. Furthermore, UV-cleaned gold substrates were capable of detaching cell sheets without serious cell injury. This may potentially provide a stable and robust approach to oligopeptide SAM-based experiments for biomedical studies.
Subject(s)
Cell Adhesion , Cell Culture Techniques , Detergents , Gold/chemistry , Oligopeptides/chemistry , Tissue Scaffolds/chemistry , Cell Adhesion/radiation effects , Cell Culture Techniques/instrumentation , Cell Culture Techniques/methods , Coated Materials, Biocompatible/chemistry , Coated Materials, Biocompatible/radiation effects , Color , Equipment Reuse , Protein Multimerization , Surface Properties/radiation effects , Ultraviolet RaysABSTRACT
BACKGROUND: Acute-phase technetium-99 m dimercaptosuccinic acid (DMSA) scintigraphy is recommended for initial imaging in children with febrile urinary tract infection (fUTI). Recently, the importance of identifying patients at risk of recurrent fUTI (r-fUTI) has been emphasized. To clarify the effectiveness of DMSA scintigraphy for predicting r-fUTI in infants, we investigated the relationship between defects on DMSA scintigraphy and r-fUTI. METHODS: Seventy-nine consecutive infants (male: female, 60:19) with fUTI were enrolled in this study. DMSA scintigraphy was performed in the acute phase, and patients with defect underwent voiding cystourethrography and chronic-phase (6 months later) DMSA scintigraphy. Patients were followed on continuous antibiotic prophylaxis (CAP). RESULTS: Defects on acute-phase DMSA scintigraphy were observed in 32 children (40.5%) of 79. The mean follow-up observation period was 17.0 ± 10.1 months. Four patients had r-fUTI (5%). Two of them had defects on DMSA scintigraphy in both the acute phase and chronic phase, and had bilateral vesicoureteral reflux (VUR) grade IV. Two others had r-fUTI without defects on DMSA and did not have VUR. Twelve patients had defect on chronic-phase DMSA scintigraphy and four of them had no VUR. CONCLUSIONS: The top-down approach is a possible method for predicting r-fUTI in infants and does not miss clinically significant VUR. Also, given that the prevalence of r-fUTI was 5% regardless of the presence of defects on acute-phase DMSA, then, in conjunction with genital hygiene and CAP, acute-phase DMSA might be unnecessary if chronic-phase DMSA is performed for all patients to detect renal scar.
Subject(s)
Cicatrix/diagnostic imaging , Cicatrix/etiology , Kidney/diagnostic imaging , Radiopharmaceuticals , Technetium Tc 99m Dimercaptosuccinic Acid , Urinary Tract Infections/diagnostic imaging , Cicatrix/epidemiology , Female , Fever/etiology , Follow-Up Studies , Humans , Incidence , Infant , Kidney/pathology , Male , Radionuclide Imaging , Recurrence , Risk Assessment , Urinary Tract Infections/complications , Urinary Tract Infections/epidemiology , Urinary Tract Infections/pathologyABSTRACT
Humoral factors may cause idiopathic steroid-sensitive nephrotic syndrome (ISSNS). In the present study, we analyzed serum proteins using mass spectrometry (MS) to identify proteins associated with the pathophysiology of pediatric ISSNS. We collected serial serum samples from 33 children during each ISSNS phase; Phase A1 is the acute phase prior to steroid treatment (STx), Phase A2 represents the remission period with STx, and Phase A3 represents the remission period after completion of STx. Children with normal urinalyses (Group B) and children with a nephrotic syndrome other than ISSNS (Group C) served as controls. No significant differences in urinary protein/urinary creatinine (UP/UCr) ratios were observed between the children with phase A1 ISSNS and Group C. We used surface-enhanced laser desorption/ionization time of flight MS for sample analysis. Four ion peaks with a mass-to-charge ratio (m/z) of 6,444, 6,626, 8,695, and 8,915 were significantly elevated during ISSNS Phase A1 compared to Phase A2, Phase A3, and Group C. The intensity of an m/z of 6,626 significantly correlated with the UP/UCr ratio and an m/z of 6,626 was identified as apolipoprotein C-I (Apo C-I). Apo C-I levels correlate with the UP/UCr ratio in pediatric ISSNS. Our findings provide new insights into the pathophysiology of ISSNS.
ABSTRACT
UNLABELLED: Fabry disease is an X-linked glycosphingolipidosis caused by deficient synthesis of the enzyme α-galactosidase A, which results in accumulations of globotriaosylceramide (GL-3) in systemic tissues. Nephropathy is a dominant feature of Fabry disease. It still remains unclear how the nephropathy progresses. Recombinant agalsidase replacement therapy is currently the only approved, specific therapy for Fabry disease. The optimal dose of replacement enzyme also still remains unclear. The worldwide shortage of agalsidase-ß in 2009 forced dose reduction of administration. It showed that the proteinuria emerged like surges, followed by temporary plasma GL-3 elevations in the early stages of classic Fabry disease. Additionally, it also showed that 1 mg/kg of agalsidase-ß every other week could clear the GL-3 accumulations from podocytes and was required to maintain negative proteinuria and normal plasma GL-3 levels. CONCLUSION: This observation of a young patient with classic Fabry disease about 5 years reveals that the long-term, low-dose agalsidase-ß caused proteinuria surges, but not persistent proteinuria, followed by temporary plasma GL-3 elevations, and agalsidase-ß at 1 mg/kg every other week could clear accumulated GL-3 from podocytes and was required to maintain normal urinalysis and plasma GL-3 levels.
Subject(s)
Fabry Disease/blood , Isoenzymes/administration & dosage , Podocytes/pathology , Proteinuria/blood , Trihexosylceramides/blood , alpha-Galactosidase/administration & dosage , Adolescent , Enzyme Replacement Therapy/methods , Fabry Disease/drug therapy , Humans , Kidney Diseases/complications , MaleABSTRACT
A 10-year-old girl presented with mild proteinuria and hypertension. Laboratory data indicated slightly elevated serum creatinine (0.67 mg/dL) and elevated serum IgG (2111 mg/dL). On renal arteriography mild stenosis over the entire length of the right renal artery and irregular stenosis of the interlobar arteries in the right kidney were seen. She was diagnosed with renovascular hypertension, and received conventional anti-hypertensive therapy, but did not respond to them. The right kidney had atrophy and dysfunction on technetium-99m-labeled dimercaptosuccinic acid renal scintigraphy, and was therefore resected. Histopathology of the kidney indicated severe necrotizing granulomatous vasculitis affecting the arteries from the renal hilus to the interlobar area. After nephrectomy plus steroid pulse therapy, blood pressure and urinary protein returned to normal. To our knowledge, this is the first report of necrotizing granulomatous vasculitis limited to the medium-sized renal arteries.