Ultrasonography Measurement of Renal Dimension and Its Correlation with Age, Body Indices, and eGFR in Type 1 Diabetes Mellitus Patients: Real World Data in Taiwan.

BACKGROUND: Assessment of renal size is clinically significant for the screening, diagnosis, and follow-up of renal diseases as the basis of clinical decisions. However, the relationship of renal dimension with age, body indices, and the estimated glomerular filtration rate (eGFR) has rarely been reported in the Chinese type 1 diabetes mellitus (T1DM) population. METHODS: A total of 220 T1DM patients were retrospectively analyzed from the Chang Gung Research Database in Taiwan. Demographic data, laboratory data, and ultrasonographic images from January 2001 to November 2018 were extracted. RESULTS: Eighty-five participants (38.6%) were male. The mean age was 34.2 years. The median eGFR was 60.0 mL/min/1.73 m2. The mean ultrasonographic left and right renal lengths (LL and RL) with S.D. were 10.9 ± 1.5 cm and 11.0 ± 1.1 cm, respectively. Renal lengths were longer with increasing body height and body weight but shorter with increasing age in patients with T1DM. In trajectory analysis, a linear mixed model revealed no significant trend in the changes in eGFR during the follow-up period. Moreover, renal length did not play a significant role in predicting KDIGO CKD stage 5 in the cohort. CONCLUSIONS: Renal length and its comparison to the reference ranges demonstrated very limited advantages in predicting renal function decline in T1DM patients.

An observational study on prevalence of latent tuberculosis infection and outcome of 3HP treatment in patients under hemodialysis in Taiwan.

BACKGROUND: Identification and treatment for latent tuberculosis infection (LTBI) are of great epidemiological importance of controlling tuberculosis (TB) worldwide. Identification in high-risk population on dialysis and treatment with 12-week weekly rifapentine plus isoniazid (3HP) help improve prevention outcomes effectively. METHODS: We conducted a single-center, nonrandomized follow-up study on end-stage renal disease patients on hemodialysis. The interferon-gamma release assay (IGRA) was used for the diagnosis of LTBI. Participants were treated with 3HP, and treatment responses were recorded and analyzed. RESULTS: A total of 123 of the 641 patients showed positive IGRA results. The male sex, age >60 years, low serum albumin level (<4.0 g/dL), and hypercalcemia (serum calcium level > 10.2 mg/dL) were associated with IGRA positivity. Seventy-five patients were treated with 3HP, with a completion rate of 66.67%. The male sex, albumin level >4.0 g/dL, and absence of adverse drug reaction were associated with increased completion rates. Adverse drug reactions included dizziness, fatigue, nausea and vomiting, fever, and hypertension. CONCLUSION: Risk factors for LTBI in dialysis patients were identified to prioritize LTBI screening and initiate early treatment. The completion rate in dialysis patients were approximately 2 of 3 patients with mild adverse drug reaction, leading to discontinuation of the treatment.

Diagnosis and management of jejunoileal diverticular haemorrhage: An update on the experience in a single centre.

INTRODUCTION: Jejunoileal diverticular haemorrhage is a rare disease that is difficult to diagnose and treat. Despite advances in endoscopic technology, recommendations on diagnosis and management for jejunoileal diverticular haemorrhage have remained unchanged and these new options have not been compared against traditional surgical management. MATERIALS AND METHODS: We retrospectively reviewed the diagnosis, management, and outcome for jejunoileal diverticular haemorrhage cases at our institution over the past 20 years. Data were organized and analysed by chi-square test, student t-test and Kaplan-Meier survival analysis. RESULTS: The most utilised diagnostic procedure was computed tomography, followed by enteroscopy, angiography, small bowel flow-through and surgery. Primary treatments included, in a decreasing order, medical therapy, surgery, endoscopy and radiology. Surgical treatment was not associated with rebleeding, but it did result in longer hospital stays and larger blood transfusions than non-surgical treatments. The bleeding-related mortality rate was very low. Notably, there was also little change in the diagnosis and treatment between decades. CONCLUSION: We presented our experience with the diagnosis and management of jejunoileal diverticular haemorrhage, as well as long-term follow-up after treatments that have not been reported previously. Surgical treatment continues to dominate management for jejunoileal diverticular haemorrhage, but we support increasing the role of endoscopy for select patient groups.

The Functional Role of CONNEXIN 26 Mutation in Nonsyndromic Hearing Loss, Demonstrated by Zebrafish Connexin 30.3 Homologue Model.

Nonsyndromic hearing loss (NSHL) is of great clinical importance, and mutations in the GJB2 gene and the encoded human CONNEXIN 26 (CX26) protein play important roles in the genetic pathogenesis. The CX26 p.R184Q mutation was shown to be a dominant-negative effect in our previous study. Previously, we also demonstrated that zebrafish Cx30.3 is orthologous to human CX26. In the present study, we established transgenic zebrafish models with mutated Cx30.3 specifically expressed in the supporting cells of zebrafish inner ears driven by the agr2 promoter, to demonstrate and understand the mechanism by which the human CX26 R.184 mutation causes NSHL. Our results indicated that significant structural changes in the inner ears of transgenic lines with mutations were measured and compared to wild-type zebrafish. Simultaneously, significant alterations of transgenic lines with mutations in swimming behavior were analyzed with the zebrafish behavioral assay. This is the first study to investigate the functional results of the CX26 p.R184Q mutation with in vivo disease models. Our work supports and confirms the pathogenic role of the CX26 p.R184Q mutation in NSHL, with a hypothesized mechanism of altered interaction among amino acids in the connexins.

Superiority of NBI endoscopy to PET/CT scan in detecting esophageal cancer among head and neck cancer patients: a retrospective cohort analysis.

BACKGROUND: Second primary cancer of the esophagus is frequent in head and neck patients, especially in high-risk populations, and has a great impact on the prognosis. Although Positron emission tomography (PET)/computed tomography (CT) scan is commonly conducted in head and neck patients, its ability to detect early esophageal cancer is limited. Narrow-band imaging endoscopy is an accurate and convenient technique for esophageal examination. We aimed to compare PET/CT scan and narrow-band imaging endoscopy for the detection of esophageal cancer in head and neck cancer patients. METHODS: From November 2015 to November 2018, all head and neck cancer patients who underwent both PET/CT scan and narrow-band imaging endoscopy at Changhua Christian Hospital were retrospectively enrolled. Descriptive statistics, receiver operating characteristic curve analysis, logistic regression analysis, independent Student's t-test, and Kaplan-Meier survival analysis were conducted with MedCalc Statistical Software. RESULTS: A total of 147 subjects were included in the analysis; suspicious esophageal lesions were identified by PET/CT scan in 8 (5.44%) and by narrow-band imaging in 35 (23.81%). The final pathologic diagnoses were esophageal squamous cell carcinoma in 10 and high-grade dysplasia in 5. The respective sensitivity, specificity, and area under the curve for detecting suspicious esophageal lesions were 33.33, 97.73%, and 0.655 for PET/CT scan, and 100.0, 84.85%, and 0.924 for narrow-band imaging endoscopy. Hypopharyngeal or laryngeal location of the primary head and neck cancer was the only risk factor for developing second primary esophageal cancer. CONCLUSIONS: PET/CT scan was inferior to narrow-band imaging endoscopy in detecting second primary esophageal cancer in head and neck cancer patients. In addition to PET/CT scan, narrow-band imaging endoscopy should be considered in head and neck patients at high risk for developing second primary esophageal cancer.

Reference ranges for ultrasonographic renal dimensions as functions of age and body indices: A retrospective observational study in Taiwan.

An ethnicity-based reference range for normal renal size is fundamental for ultrasonographic assessment. Herein, we aimed to establish a Chinese renal reference by a large sample size, as well as to elucidate the relationship of renal dimension to age and body indices, with the aid of a comprehensive literature review. Records of 3707 healthy cases were obtained from health evaluation centers of Kaohsiung and Linkuo Chang Gung Memorial Hospitals. As a result, the mean right renal length was 10.62±0.69 cm, left renal length 10.76±0.70 cm, right renal width 4.78±0.75 cm, and left renal width 5.10±0.64 cm. Renal size was well-correlated curvilinearly to age, while linearly to body height, body weight, and body mass index. Renal size increases and then decreases with aging, and significant variations of renal size exist among different ethnicities. Our work provides a reliable reference range for renal size in the Chinese population, and valid relationships between renal dimensions and other parameters.

An Update on Clinicopathological and Molecular Features of Plexiform Fibromyxoma.

Plexiform fibromyxoma is a rare and newly described gastric mesenchymal tumor with only 121 reported cases in the literature. Our understanding of plexiform fibromyxoma requires updating since the first case has been reported by Takahashi et al. 12 years ago. The present review summarized reported cases in the literature, and both clinical and pathological aspects of plexiform fibromyxoma were comprehensively discussed. Plexiform fibromyxoma usually causes nonspecific or bleeding signs or symptoms, and therefore clinical recognition of the disease is challenging. Plexiform fibromyxoma is of benign nature without any metastasis or recurrence reported, and more conservative surgical treatment should be considered.

An Application of NGS for WDR36 Gene in Taiwanese Patients with Juvenile-Onset Open-Angle Glaucoma.

Primary open-angle glaucoma (POAG) is one of the most important disease in ophthalmology with high prevalence and risk of irreversible blindness. If diagnosed before the age of 35, it is usually categorized as juvenile open-angle glaucoma (JOAG). The WDR36 gene is reckoned as one of the major causative genes of POAG, and had been studied to be related to the pathogenesis of POAG in the literature. We have selected 61 JOAG patients and 61 JOAG-free individuals, and by next-generation sequencing method, the WDR36 gene of the subjects were analyzed. We identified 26 variations exclusively in JOAG group. Among these 26 variations, there were 3 noteworthy variations. First, a novel variation c.460-650A>G was found in our study which might cause premature termination of splicing of the conserved domain in WDR36; second, c.1494+1111G>T (rs13178997) had significantly different frequency in our JOAG patients compared to the reference frequency on NCBI; third, a variation c.710+30C>T (rs10038177) was found in our study, which had already been reported to be related to high-pressure glaucoma. We offer the profile of WDR36 in JOAG in Taiwan population, and we suggest that WDR36 gene is involved in the pathogenesis of JOAG as a subordinate modifier gene.

Deep Dermatophytosis Caused by Zoophilic Strain of Trichophyton interdigitale with Successful Treatment of Itraconazole.

We report a 66-year-old female patient with deep dermatophytosis caused by zoophilic strain of Trichophyton interdigitale, a rare granulomatous presentation of Trichophyton species infection in patients with underlying systemic diseases, and she was successfully cured by itraconazole. Since the identification of Trichophyton mentagrophytes complex had been misused for years, a brief discussion of molecular diagnosis and taxonomy of T. mentagrophytes complex is given. The pathogenesis and comparison with cases reported in the literature are also discussed.

Xeroderma pigmentosum complementation group C protein (XPC) expression in basal cell carcinoma.

BACKGROUND: The Xeroderma pigmentosum complementation group C protein (XPC) is a general sensor of damaged DNA. Individuals carrying a mutation in XPC genes exhibit marked photosensitivity and increased occurrence of skin cancers. Little is known about the distribution of XPC protein in basal cell carcinoma (BCC). AIM: To determine whether the XPC protein is associated with basal cell carcinoma. MATERIALS AND METHODS: In the present study, we investigated the protein expression of XPC by immunohistochemistry in 86 cases of BCC and paired-adjacent normal epidermis. RESULTS: The intensity of nuclear XPC expression was significantly higher in BCC compared to adjacent normal epidermis (p<0.001). Attenuated XPC expression was associated with high-risk BCC (p=0.045) but was not significantly associated with age, gender and body area. CONCLUSION: Our results indicate that XPC is associated with BCC and further studies are warranted to determine if the XPC-BCC interaction is specific to just one cancer cell type and to investigate potential mechanisms.