Case Report: IgG4-related kidney disease complicated by interstitial pneumonia [version 1; peer review: 1 approved, 2 approved with reservations]

Immunoglobulin G4 (IgG4)-related disease is a systemic inflammatory disorder characterized by tubulointerstitial nephritis with IgG4-positive plasma cell infiltration. We report the case of an 84-year-old male who presented with a history of dyspnea on exertion and cough. The lymph nodes were palpated in the axilla. Urinalysis revealed mild proteinuria and increased levels of NAG and β2-microglobulin. Blood tests showed hyperglobulinemia with a marked elevation of serum IgG4 levels. Chest computed tomography showed bilateral ground-glass and reticular opacities in the lower and peripheral portions of the lungs. Ga-67 scintigraphy showed kidney uptake. The patient was diagnosed with IgG4-related kidney disease based on the renal pathology indicative of typical tubulointerstitial nephritis with extensive IgG4-positive plasma cell infiltration. The patient was treated with prednisolone and showed a prompt response in his clinical condition. The patient achieved normalization of serum IgG4 levels 6 months after the initiation of treatment. Although IgG4-related disease is thought to be potentially associated with organ fibrosis, there are few reports on combination of interstitial pneumonia and IgG4-related kidney disease. Our case report presents a possible pattern of IgG4-related disease

糖尿病性末梢神経障害における関節可動域制限とバランス制御が歩容変化に及ぼす影響

Aims This study analyzed the gait patterns of diabetic peripheral neuropathy (DPN) patients and changes in the center of mass sway to prevent the formation and recurrence of foot ulcers. Methods Forty-two subjects were divided into the diabetes mellitus (DM), DPN, and diabetic foot ulcer (DFU) groups. We measured the range of motion (ROM) of the lower limb joints in the resting position and the center of mass sway in the standing position. Joint angles, ROM during walking, and distance factors were evaluated. Results In the DFU group, ROM limitation during walking was detected at the knee joint, and functional and ROM limitations were found at the ankle joint. The step length ratio and step width in the DFU group were significantly lower and higher than those in the DM group, respectively. The sway distances in the DFU group were greater than those in the DM and DPN groups. Conclusions Functional joint limitations and gait changes due to the decreased ability to maintain the center of gravity were observed in the DFU group. As DPN progressed, the patients’ gait became small, wide, and shuffled. Thus, supporting joint movement during walking may help reduce the incidence and recurrence of foot ulcers

Effective cardiac resynchronization therapy for an adolescent patient with dilated cardiomyopathy seven years after mitral valve replacement and septal anterior ventricular exclusion

Cardiac resynchronization therapy (CRT) is a new treatment for refractory heart failure. However, most heart failure patients treated with CRT are middle-aged or old patients with idiopathic or ischemic dilated cardiomyopathy. We treated a 17 year 11 month old girl with dilated cardiomyopathy after mitral valve replacement (MVR) and septal anterior ventricular exclusion (SAVE). Seven years after the SAVE procedure, she presented complaining of palpitations and general fatigue with normal activity. Her echocardiogram showed reduced left ventricular function. Despite of optimal medical therapy, her left ventricular function continued to decline and she experienced regular arrhythmias such as premature ventricular contractions. We thus elected to perform cardiac resynchronization therapy with defibrillator (CRT-D). After CRT-D, her clinical symptoms improved dramatically and left ventricular ejection fraction (LVEF) improved from 31.2% to 51.3% as assessed by echocardiogram. Serum BNP levels decreased from 448.2 to 213.6 pg/ml. On ECG, arrhythmias were remarkably reduced and QRS duration was shortened from 174 to 152 msec. In conclusion, CRT-D is an effective therapeutic option for adolescent patients with refractory heart failure after left ventricular volume reduction surgery

Physical performance disorder（Locomotive syndrome）closely relates to both hypertension and diabetes mellitus which are components of metabolic syndrome

Locomotive syndrome is physical performance disorder in the elderly person. The physical performance disorder can become cause of metabolic syndrome. This study focused on the locomotive syndrome’s relationship to the hypertension, diabetes mellitus, and hyperlipidemia which are components of the metabolic syndrome. To evaluate the locomotive syndrome, questionnaire score system : brand-new loco-check-score and established locomo-５-score were adopted. In the questionnaire, current medications were also listed especially about hypertension, diabetes mellitus, and hyperlipidemia. A correlation between loco-check-score and locomo-５-score was statistically evaluated by Spearman rank correlation coefficient. Moreover correlations between body mass index（BMI）and loco-check-score, BMI and locomo-５-score were statistically evaluated by Spearman rank correlation coefficient. In a health consultation event, １４６ persons checked the questionnaire （mean age ７２．５yo）. Differences of the loco-check-score and the locomo-５-score in the hypertension-group, diabetes mellitus group, and the hyperlipidemia group were statistically evaluated by the Mann-Whitney U test respectively. Age matched persons with no medications comprised control groups. Significant differences were observed in the hypertension group and diabetes mellitus group both in the loco-check-score and locomo-５-score systems. These two groups showed significantly high loco-check-score and locomo-５-score than the control groups. The loco-check-score closely related to the locomo-５-score. On the other hand, no relations were observed between BMI and the loco-check-score, and between BMI and the locomo-５-score

Physical performance disorder（Locomotive syndrome）closely relates to both hypertension and diabetes mellitus which are components of metabolic syndrome

Locomotive syndrome is physical performance disorder in the elderly person. The physical performance disorder can become cause of metabolic syndrome. This study focused on the locomotive syndrome’s relationship to the hypertension, diabetes mellitus, and hyperlipidemia which are components of the metabolic syndrome. To evaluate the locomotive syndrome, questionnaire score system : brand-new loco-check-score and established locomo-５-score were adopted. In the questionnaire, current medications were also listed especially about hypertension, diabetes mellitus, and hyperlipidemia. A correlation between loco-check-score and locomo-５-score was statistically evaluated by Spearman rank correlation coefficient. Moreover correlations between body mass index（BMI）and loco-check-score, BMI and locomo-５-score were statistically evaluated by Spearman rank correlation coefficient. In a health consultation event, １４６ persons checked the questionnaire （mean age ７２．５yo）. Differences of the loco-check-score and the locomo-５-score in the hypertension-group, diabetes mellitus group, and the hyperlipidemia group were statistically evaluated by the Mann-Whitney U test respectively. Age matched persons with no medications comprised control groups. Significant differences were observed in the hypertension group and diabetes mellitus group both in the loco-check-score and locomo-５-score systems. These two groups showed significantly high loco-check-score and locomo-５-score than the control groups. The loco-check-score closely related to the locomo-５-score. On the other hand, no relations were observed between BMI and the loco-check-score, and between BMI and the locomo-５-score

IgA nephropathy after COVID-19 vaccination and analysis of reported cases

Background: Immunoglobin A nephropathy (IgAN) is one of the most common forms of chronic glomerulonephritis and has been shown to occur in association with vaccinations. While various vaccines against COVID-19 have become widely used, their side effects, especially on IgAN following COVID-19 vaccines are still unclear. In this report, we describe the clinical courses and histopathologic findings of a newly diagnosed case of IgAN after receiving the mRNA-1273 (Moderna) COVID-19 vaccination. Methods: This study presents a case of new-onset IgAN following mRNA-1273 COVID-19 (Moderna) vaccination. In addition, we review and analyze similar cases previously reported in the literature up to October 2022. Results: Of the 52 cases reviewed, including our own, the majority of patients were female, and 64% of patients had initial onset IgAN. The most common manifestation was gross hematuria (87%), other associated symptoms were fever (44%), myalgia (8%), arthralgia (4%), and edema (4%). Many of these cases occurred after receiving Pfizer products as the second vaccination. Oral corticosteroids were used to 16 cases, and steroid pulse therapy was used to treat 7 cases. Conclusion: While this is not a controlled study, it is important for physicians to consider the possibility that COVID-19 vaccines may provoke a flare of IgAN. Several therapeutic agents may be useful for treating COVID-19 vaccine-induced IgAN, although a specific mechanism or pathophysiological association cannot be confirmed without further research

Predictive significance of glomerular insulin receptor substrate-1 in patients with diabetic kidney disease

Background: In rodents, glomerular expression of insulin receptor substrate 1 (IRS1) is decreased in diabetic kidney disease (DKD) and reduced associated functioning is involved in the development and progression of DKD. This study aimed to evaluate the significance of glomerular IRS1 expression in DKD patients, and investigated whether glomerular IRS1 expression can reflect renal pathology and predict renal outcomes. Methods: This study included 10 patients who underwent renal biopsy and were diagnosed with DKD or minor glomerular abnormality (MGA). IRS1-positive cells were determined based on renal biopsy and immunostaining, and the associations of the number of these cells with baseline and prognostic parameters were analyzed. Results: IRS1-positive cells were significantly decreased in DKD than in MGA. IRS1 positivity tended to be negatively correlated with global glomerulosclerosis and tubulointerstitial fibrosis. The rate of change in estimated glomerular filtration rate before and 12 months after renal biopsy was positively correlated to the number of IRS1-positive cells. Furthermore, a tendency towards negative correlation was observed between the number of glomerular IRS1-positive cells and the proteinuria. Conclusions: This study shows the glomerular IRS1-positive cell count was significantly decreased in DKD, and that the degree IRS1 positivity was partially correlated with renal pathology and function

Conserved hydrophobic amino acid residues in the N-terminal region of DnaA protein are involved in DnaA-DnaA interaction.

We previously reported that a leucine-zipper-like structure (I26, L33 and L40) located in the N-terminal region of DnaA is essential for the duplex opening at oriC by DnaA. In this study, we focused on three other conserved hydrophobic amino acid residues, L3, L10 and L17, and examined the function of DnaA proteins mutated in these amino acid residues. DnaA427 (L17S) and DnaA413 (L3S, L10S and L17S) were inactive for oriC DNA replication both in vitro and in vivo. Although these mutant DnaA proteins maintained their binding activities for both ATP and oriC, they were unable to induce the opening of duplex DNA at oriC. Glutathione-S-transferase (GST)-fused wild-type DnaA interacted with wild-type DnaA but not with DnaA427 and DnaA413. Based on these results, we propose that conserved hydrophobic amino acid residues in the N-terminal region of DnaA are involved in DnaA oligomerization, in which DnaA-DnaA interaction is required