Commentary: Statewide Variation and Changes in Practices for Primary Spontaneous Pneumothorax in Maryland, United States: A Retrospective Study.

See Article page 34.

An elderly woman with Birt-Hogg-Dubé syndrome having multiple pulmonary cysts mimicking lymphangioleiomyomatosis.

The characteristics of the pulmonary cysts on the high-resolution computed tomography (HRCT) chest images are an important diagnostic clue to distinguish among cystic lung diseases. The diagnostic accuracy of HRCT was reported to be as high as 90% by experienced pulmonologists and radiologists. Herein, we report the case of an elderly woman with Birt-Hogg-Dubé syndrome (BHDS) whose HRCT images displayed lymphangioleiomyomatosis-like features of the pulmonary cysts, rendering it difficult for us to diagnose BHDS.

Current situation of management of spontaneous pneumothorax in Japan: A cross-sectional cohort study.

Background: Limited epidemiological information is available on spontaneous pneumothorax. To address this gap, the Japan Society for Pneumothorax and Cystic Lung Disease (JSPCLD) conducted a nationwide retrospective survey to investigate the current epidemiology of spontaneous pneumothorax in Japan.

Methods: In this study, we conducted a retrospective cross-sectional cohort study to demonstrate the clinical features of spontaneous pneumothorax in one year from April 2019 to March 2020, compare patient characteristics and treatment outcomes between primary (PSP) and secondary spontaneous pneumothorax (SSP), and investigate the risk factors associated with in-hospital mortality among patients with SSP.

[Epstein-Barr virus-associated gastritis with perigastric lymphadenopathy accompanied by infectious mononucleosis:a case report].

A 28-year-old female patient with no particular medical history had a sore throat seven days before admission. Subsequently, she developed malaise, right abdominal pain, and a fever of 38°C and visited our hospital. A blood test revealed a mild inflammatory response and elevated liver enzymes, and she was admitted to the hospital for detailed examination and acute liver injury treatment.

Examine the optimal multidisciplinary care teams for patients with chronic kidney disease from a nationwide cohort study.

Background: Multidisciplinary team-based integrated care (MDC) has been recommended for patients with chronic kidney disease (CKD). However, team-based specific structured care systems are not yet established. Therefore, we investigated the efficacy of MDC system and the optimal number of professionals that make up the team for maintaining kidney function and improving prognosis.

Postoperative hormonal therapies reduce the recurrence of thoracic endometriosis-related pneumothorax.

Objectives: Thoracic endometriosis-related pneumothorax (TERP) frequently recurs even after surgery. Meanwhile, postoperative hormonal therapies (HTx) are believed to be effective for pelvic endometriosis. Therefore, we evaluated the relationship between postoperative TERP recurrence and postoperative HTx in a retrospective observational study.

Clinicopathological Impacts of Expression of Neuronal Markers in Lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a tuberous sclerosis complex (TSC)-associated tumor, characterized by the expression of neural crest lineages including neuronal markers. Neural crest cells can differentiate into multiple cell types that contribute to tissues associated with TSC-related tumors, and TSC-related tumors could be specifically associated with distinct neural crest subtypes. This study aimed to clarify the clinicopathological effects of expression of neuronal markers in LAM.

Clinical and genetic features of 334 Asian patients with Birt-Hogg-Dubé syndrome (BHDS) who presented with pulmonary cysts with or without a history of pneumothorax, with special reference to BHDS-associated pneumothorax.

Background: The clinical pulmonary manifestations and genetic features of Birt-Hogg-Dubé syndrome (BHDS) in Asian patients remained unclear. We aimed to clarify the clinical features of BHDS-associated pneumothorax (PTX) and retrospectively investigate potential contributing factors in the largest Asian cohort to date.

Methods: We reviewed the clinical and genetic data collected in 2006-2017, from the BHDS patients who were Asian and presented with pulmonary cysts with or without a history of PTX.

Inpatient multidisciplinary care can prevent deterioration of renal function in patients with chronic kidney disease: a nationwide cohort study.

Background: Multidisciplinary care is necessary to prevent worsening renal function and all-cause mortality in patients with chronic kidney disease (CKD) but has mostly been investigated in the outpatient setting. In this study, we evaluated the outcome of multidisciplinary care for CKD according to whether it was provided in an outpatient or inpatient setting.

Methods: This nationwide, multicenter, retrospective, observational study included 2954 Japanese patients with CKD stage 3-5 who received multidisciplinary care in 2015-2019.

Diabetic Polyneuropathy: New Strategies to Target Sensory Neurons in Dorsal Root Ganglia.

Diabetic polyneuropathy (DPN) is the most common type of diabetic neuropathy, rendering a slowly progressive, symmetrical, and length-dependent dying-back axonopathy with preferential sensory involvement. Although the pathogenesis of DPN is complex, this review emphasizes the concept that hyperglycemia and metabolic stressors directly target sensory neurons in the dorsal root ganglia (DRG), leading to distal axonal degeneration. In this context, we discuss the role for DRG-targeting gene delivery, specifically oligonucleotide therapeutics for DPN.

Patient-Specific Femoral Guide with 3-Dimensional Support Software in Anterolateral Supine Approach of Total Hip Arthroplasty: A Single-Arm Trial.

Currently, there is no surgical assistance system that can perform a three-dimensional (3D) planned total hip arthroplasty (THA) by methods other than surgical assistance navigation or robots. However, they are expensive, cumbersome, and subject to additional invasiveness, so there is a need for a simpler and less expensive 3D surgical support system. In this study, THA was performed using the anterolateral approach (Watson-Jones) in the supine position in 23 subjects to examine the efficacy and safety of a patient-specific femoral guide linked to 3D surgery support software.

A cross-sectional study evaluating patients' preferences for Salter innominate osteotomy.

Background: Residual acetabular dysplasia in children after reduction of hip dislocation is often treated using Salter innominate osteotomy to prevent future osteoarthritis. Preventive surgery for asymptomatic patients, which could result in overtreatment, should be carefully applied with consideration of patients' opinions. In this study, we aimed to describe opinions on Salter innominate osteotomy as preventive surgery for children among adult patients who had undergone periacetabular osteotomy for hip pain due to hip dysplasia.

CLINICOPATHOLOGICAL ANALYSIS OF SECONDARY RETINAL VASOPROLIFERATIVE TUMOR/REACTIVE RETINAL ASTROCYTIC TUMOR SUCCESSFULLY TREATED BY ENDORESECTION.

Purpose: To report the clinicopathological findings of retinal vasoproliferative tumor/reactive retinal astrocytic tumor (VPT/RRAT) with retinal vasculitis, treated by tumor resection.

Methods: A retrospective single case report.

Patient: A 29-year-old Japanese woman was referred with cystoid macular edema and retinal vasculitis in the right eye.

Effects of Calcium Polystyrene Sulfonate Formulation Change from Dry Syrup to Oral Solution in Patients with Chronic Kidney Disease.

Calcium polystyrene sulfonate, a cation exchange resin preparation, is used to treat hyperkalaemia. The effects of switching from dry syrup to oral solution forms have been rarely evaluated. We investigated changes in serum potassium levels, incidence of adverse events, and patients' perception and satisfaction associated with the change in calcium polystyrene sulfonate dosage forms from dry syrup to oral solution in chronic kidney disease patients.

A propensity score-matched comparison between Mako robotic arm-assisted system and conventional technique in total hip arthroplasty for patients with osteoarthritis secondary to developmental dysplasia of the hip.

Background: The clinical effectiveness of robotic arm-assisted systems remains unclear for total hip arthroplasty (THA) in patients suffering from osteoarthritis secondary to developmental dysplasia of the hip (DDH).

Methods: Patients with DDH who underwent primary THA were included in this study. We conducted a propensity score-matched comparison between THAs using a robotic arm-assisted system (Mako group) versus those using the manual procedure (manual group) to compare the absolute differences in cup placement angles measured using postoperative computed tomography and those planned preoperatively.

Safety of linezolid, rifampicin, and clindamycin combination therapy in patients with prosthetic joint infection.

We investigated adverse events in patients with prosthetic joint infections receiving combination therapy with linezolid, rifampicin, and clindamycin for ≥ 7 days. Twenty-two patients were evaluated. The combination therapy was administered for 15.

Three-dimensional foot trajectory in female patients with end-stage hip osteoarthritis during walking.

Osteoarthritis (OA) is a risk factor for falls. To decrease the fall risk, it is important to evaluate the detailed features of the gait of patients with OA. This study aimed to investigate the spatio-temporal parameters of gait in patients with end-stage hip OA, especially foot trajectory.

Dynamics of thoracic endometriosis in the pleural cavity.

Background: Thoracic endometriosis-related pneumothorax is a secondary spontaneous pneumothorax caused by thoracic endometriosis. Diaphragmatic endometriosis is well-studied, but visceral and/or parietal pleural lesions are not. Although surgery is an effective treatment, postoperative recurrence rates are unsatisfactory probably due to inadequate understanding of underlying pathophysiology.

The serological diversity of serum IgG/IgA/IgM against SARS-CoV-2 nucleoprotein, spike, and receptor-binding domain and neutralizing antibodies in patients with COVID-19 in Japan.

Background: We compared the temporal changes of immunoglobulin M (IgM), IgG, and IgA antibodies against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) nucleoprotein (N), spike 1 subunit (S1), and receptor-binding domain (RBD), and neutralizing antibodies (NAbs) against SARS-CoV-2 in patients with coronavirus disease 2019 (COVID-19) to understand the humoral immunity in COVID-19 patients for developing drugs and vaccines for COVID-19.

Methods: A total of five confirmed COVID-19 cases in Nissan Tamagawa Hospital in early August 2020 were recruited in this study. Using a fully automated chemiluminescence immunoassay analyzer, we measured the levels of IgG, IgA, and IgM against SARS-CoV-2 N, S1, and RBD and NAbs against SARS-CoV-2 in COVID-19 patients' sera acquired multiple times in individuals from 0 to 76 days after symptom onset.

Relationship between compliance with management target values and renal prognosis in multidisciplinary care for outpatients with chronic kidney disease.

Background: Although multidisciplinary care (MDC) is necessary for controlling chronic kidney disease (CKD), its impact on compliance with management target values in the CKD guidelines remains unclear. This study was designed to clarify the relationship between compliance with management target values and renal prognosis in CKD outpatients who received MDC.

Methods: There were 255 outpatients with pre-dialysis CKD who received MDC.

The Role of Long Noncoding RNA MALAT1 in Diabetic Polyneuropathy and the Impact of Its Silencing in the Dorsal Root Ganglion by a DNA/RNA Heteroduplex Oligonucleotide.

Diabetic polyneuropathy (DPN) is the most common complication of diabetes, yet its pathophysiology has not been established. Accumulating evidence suggests that long noncoding RNA metastasis-associated lung adenocarcinoma transcript 1 (MALAT1) plays pivotal roles in the regulation of cell growth and survival during diabetic complications. This study aimed to investigate the impact of MALAT1 silencing in dorsal root ganglion (DRG) sensory neurons, using an α-tocopherol-conjugated DNA/RNA heteroduplex oligonucleotide (Toc-HDO), on the peripheral nervous system of diabetic mice.

Seventh BHD international symposium: recent scientific and clinical advancement.

The 7th Birt-Hogg-Dubé (BHD) International Symposium convened virtually in October 2021. The meeting attracted more than 200 participants internationally and highlighted recent findings in a variety of areas, including genetic insight and molecular understanding of BHD syndrome, structure and function of the tumor suppressor Folliculin (FLCN), therapeutic and clinical advances as well as patients' experiences living with this malady.