Pathogenic variants of the gene introduce an abnormal amino acid sequence in the β6 strand/α5 helix of Gsα, causing pseudohypoparathyroidism type 1A and pseudopseudohypoparathyroidism in two unrelated Japanese families.

Pseudohypoparathyroidism 1A (PHP1A) and pseudopseudohypoparathyroidism (PPHP) are caused by loss-of-function variants of , which encodes Gsα. We present two unrelated Japanese families with PHP1A and PPHP harboring unreported pathogenic variants of (c.1141delG, p.

Pseudo-malignant paranasal invasive aspergillosis showing bone destruction and FDG uptake on PET/CT: A case report.

Rationale: Aspergillosis is a rare disease that often occurs in patients with diabetes mellitus or compromised hosts. This study describes a 60-years-old woman with unusual pseudo-malignant paranasal invasive aspergillosis with 18F-fluorodeoxyglucose (18F-FDG) uptake treated by endoscopic sinus surgery. To the best of our knowledge, there are few reported cases of paranasal fungal infection with 18F-FDG uptake.

Thyroid-Stimulating Hormone, Age, and Tumor Size are Risk Factors for Progression During Active Surveillance of Low-Risk Papillary Thyroid Microcarcinoma in Adults.

Background: Active surveillance (AS) of low-risk papillary thyroid microcarcinoma (PTMC) was initiated at Kuma Hospital in 1993 and is gradually spreading worldwide. We assessed the effect of thyroid-stimulating hormone (TSH) levels on PTMC enlargement in patients on AS.

Methods: We enrolled 2705 patients with cytologically diagnosed PTMC who had undergone AS between January 2005 and July 2019.

Optimal target blood pressure in elderly with septic shock (OPTPRESS) trial: study protocol for a randomized controlled trial.

Background: Hemodynamic stabilization is a core component in the resuscitation of septic shock. However, the optimal target blood pressure remains debatable. Previous randomized controlled trials suggested that uniformly adopting a target mean arterial pressure (MAP) higher than 65 mmHg for all adult septic shock patients would not be beneficial; however, it has also been proposed that higher target MAP may be beneficial for elderly patients, especially those with arteriosclerosis.

Survey of Areas Underserved by Plastic Surgery in Japan.

 In Japan, there is a large regional disparity in plastic surgery availability. In order for plastic surgery to be widely available for all citizens, it is essential for at least one plastic surgery facility to be located in each secondary medical zone.  Using the Japan Society of Plastic and Reconstructive Surgery homepage and some databases, we extracted data on secondary medical zones that do not have a plastic surgery facility.

Co-Treatment with Steroid and Dupilumab for Eosinophilic Chronic Rhinosinusitis after Eosinophilic Pneumonitis Caused by Dupilumab.

Eosinophilic chronic rhinosinusitis (ECRS) is a Type 2 inflammatory disease that manifests as chronic inflammation of the paranasal sinus. IL-4/IL-13 receptor monoclonal antibodies (dupilumab) to suppress Type 2 inflammation have become a good treatment option for patients who are refractory to surgery. Most patients respond normally, although significant side effects such as eosinophilic pneumonia may occur, requiring discontinuation of dupilumab.

Dynamic change of fecal microbiota and metabolomics in a polymicrobial murine sepsis model.

Aim: Sepsis causes a systemic inflammatory reaction by destroying intestinal flora, which leads to a poor prognosis. In this study, we sought to clarify the characteristics of fecal flora and metabolites in a mouse model of sepsis by comprehensive metagenomic and metabolomic analysis.

Methods: We performed a cecal ligation and puncture model procedure to create a mild sepsis model.

Treatment of Descemet's membrane detachment after primary Descemet's stripping automated endothelial keratoplasty during surgery using intraoperative optical coherence tomography.

Purpose: To present a novel microscope-integrated optical coherence tomography (iOCT)-guided surgical technique wherein Descemet's membrane detachment (DMD), occurring during vitrectomy, was treated intraoperatively in a patient who had previously undergone Descemet's stripping automated endothelial keratoplasty (DSAEK).

Observations: The surgical technique was performed on a 75-year-old man with a history of DSAEK to intraoperatively treat DMD, which occurred during vitrectomy in the left eye. A fine needle mounted on a syringe was inserted into the supra-Descemet's space under iOCT guidance.

Elderly onset age is associated with low efficacy of first anti-tumor necrosis factor treatment in patients with inflammatory bowel disease.

The outcomes of patients with elderly onset (EO) inflammatory bowel disease (IBD) treated with anti-tumor necrosis factor (TNF) remains uncertain. The present study evaluated the efficacy and safety of anti-TNF treatment for bio-naïve EO-IBD. Elderly patients were defined as those 60 years and older, and further divided into those with EO (Elderly-EO) and those with non-elderly onset (Elderly-NEO).

Biopsy-proven CKD etiology and outcomes: the Chronic Kidney Disease Japan Cohort (CKD-JAC) study.

Background: The Kidney Disease: Improving Global Outcomes guidelines advocate the cause-glomerular filtration rate (GFR)-albuminuria (CGA) classification for predicting outcomes. However, there is a dearth of data supporting the use of the cause of chronic kidney disease. This study aimed to address how to incorporate a prior biopsy-proven diagnosis in outcome prediction.

Effect of Preserving the Percutaneous Gallbladder Drainage Tube Before Laparoscopic Cholecystectomy on Surgical Outcome: Post Hoc Analysis of the CSGO-HBP-017.

Background: When percutaneous transhepatic gallbladder drainage (PTGBD) is followed by laparoscopic cholecystectomy (LC), there is no consensus regarding whether the drainage tube should be preserved or removed before LC. We hypothesized that the surgical results of LC might differ between cases with PTGBD tube preservation versus removal. Here, we investigated how drainage tube preservation or removal affected the surgical outcome of LC.

Preoperative risk score to predict subtotal cholecystectomy after gallbladder drainage for acute cholecystitis: Secondary analysis of data from a multi-institutional retrospective study (CSGO-HBP-017B).

Introduction: Subtotal cholecystectomy (STC) has become recognized as a "bailout procedure" to prevent bile duct injury in patients undergoing laparoscopic cholecystectomy (LC). Predictors of conversion to STC have not been identified because LC difficulty varies based on pericholecystic inflammation. We analyzed data from patients enrolled in a previously performed multi-institutional retrospective study of the optimal timing of LC after gallbladder drainage for acute cholecystitis (AC).

Congenital Palmar Duplication of the Index Finger: Ventral Polydactyly With Ventral Dimelia.

This report describes the case of a 10-month-old boy who presented with a duplicated index finger enveloped by palmar skin on the palmar side of the first web of the left hand. He was healthy without any other abnormalities except the hand anomaly. Surgical resection of the extra finger was performed with triangular flap at 15 months of age.

Parathyroidectomy vs Cinacalcet Among Patients Undergoing Hemodialysis.

Context: Parathyroidectomy (PTx) and cinacalcet are both effective treatments for secondary hyperparathyroidism in hemodialysis patients, but limited data exist comparing the long-term outcomes of these interventions.

Objective: We aimed to compare the risk of mortality among hemodialysis patients who underwent PTx and those who started treatment with cinacalcet.

Methods: In this prospective cohort study, comprising patients from the Japanese Society for Dialysis Therapy Renal Data Registry, patients who had intact parathyroid hormone (PTH) levels ≥ 300 pg/mL in late 2007 and underwent PTx or started treatment with cinacalcet in 2008 to 2009 were matched by propensity score at 1:3.

A Comparative Study of Serum Phosphate and Related Parameters in Chronic Kidney Disease between the USA and Japan.

Introduction: Recent studies have suggested a higher incidence of cardiovascular disease (CVD) among patients with chronic kidney disease (CKD) in the USA than in Japan. Hyperphosphatemia, a possible risk for CVD, may explain this difference; however, international differences in phosphate parameters in CKD have not been well elaborated.

Methods: By using the baseline data from the USA and the Japanese nation-wide, multicenter, CKD cohort studies; the Chronic Renal Insufficiency Cohort Study (CRIC, N = 3,870) and the Chronic Kidney Disease-Japan Cohort Study (CKD-JAC, N = 2,632), we harmonized the measures and compared clinical parameters regarding phosphate metabolism or serum phosphate, fibroblast growth factor-23 (FGF23), and parathyroid hormone (PTH), in the cross-sectional model.