Feasibility Study for the Long-Term Management of Refractory Hyperkeratotic Eczema with Calcipotriol and Betamethasone Dipropionate (Daivobet®), Viaminate and Concomitant Conventional Therapies: A Retrospective Study.

BACKGROUND: The available treatments for refractory hyperkeratotic eczema are inadequate with frustrating results. We, therefore, incorporated Calcipotriol and Betamethasone Dipropionate (Daivobet®), and Viaminate into the mainstay treatment to improve the clinical symptoms. The study aimed to evaluate the efficacy of Daivobet ® and Viaminate as a potential treatment alternative for refractory hyperkeratotic eczema. PATIENTS AND METHODS: Between 2013 and 2015, 61 patients diagnosed with refractory hyperkeratotic eczema (RHE) who had shown inadequate response to conventional therapies were pooled from a single center. Besides, they were all treated with Daivobet ® , Viaminate, and an occlusive dressing mixture containing 5% salicylic acid ointment and 25% zinc oxide paste following inadequate response to conventional therapies (corticosteroids plus 25% zinc oxide paste and 5% salicylic acid ointment). Investigators Global Assessment (IGA) and Patient-Oriented Eczema Measure (POEM) assessed baseline and outcome measures for the degree of hyperkeratinization (0-clear; 3-moderate; 4-severe). RESULTS: Of the 61 patients, 49 (80.3%) patients presented with moderate RHE and 12 (19.7%) with severe RHE. After 24 weeks of treatment, the period for loss of keratinization was significantly lower in patients with moderate RHE (3.9±1.9 weeks) than those with severe RHE (10.8±1.0 weeks) with a P-value <0.01. Furthermore, they required a significantly shorter total treatment duration (10.6 ± 4.3 weeks) than those with severe RHE (20.3±3.6 weeks) with a P-value of <0.01. However, there were no significant differences in post hoc analysis at week 36 with P-values of 0.46 and 1.00 for IGA and POEM, respectively. CONCLUSION: Our results showed that the incorporation of Viaminate and Daivobet® into mainstay treatment was effective and safe for the long-term management of RHE.

Efficacy and Safety of NTrap® Stone Entrapment and Extraction Device for Ureteroscopic Lithotripsy.

PURPOSE: NTrap® stone entrapment and extraction device (NTrap®) is a device used to extract and remove stones from the urinary tract and to minimize retrograde stone migration during ureterolithotripsy (URS). This study aimed to evaluate the efficacy and safety of NTrap® in URS. METHODS: From Jan 2014 to June 2017, 148 patients underwent URS with the aid of NTrap® (Group A), and 209 patients underwent standard URS without any anti-retropulsion device (Group B). Their demographics, operation time, complications, stone migration rate, and stone-free rate (SFR) were recorded for comparison. RESULTS: Compared with group B, Group A had a significantly shorter operative and lasering time (P = 0.003, P<0.001, respectively). There was no significant difference between the 2 groups in overall complications, a decrease in mean hemoglobin, and length of stay (LOS) (P = 0.426, P = 0.097, P = 0.058, respectively). The incidence of stone migration was significantly lower in Group A than Group B (P = 0.035). The postoperative auxiliary procedure rate (in patients with stones retropulsion during the operation) was significantly lower in Group A compared to Group B (P = 0.024). The SFR was considerably higher in Group A than Group B (P = 0.009). CONCLUSION: URS, with the aid of NTrap®, is an effective and safe method for treating ureteric stones. It may prevent stones from retropulsion and shorten the operative time.

Type II acquired cutis laxa associated with recurrent urticarial vasculitis: brief report.

BACKGROUND: Cutis laxa is a connective tissue disease characterized by loose, wrinkled, and redundant skin. It is either inherited or acquired. In most cases, acquired cutis laxa is associated with neoplasms, drugs, and autoimmune diseases. We present a rare case of acquired cutis laxa following a recurrent urticaria-like eruption in the absence of an autoimmune disease, neoplasm, drugs and or syndrome. CASE PRESENTATION: We report a case of a 45-year-old Chinese lady with a 1-year history of widespread pruritic urticarial eruption and a 6-month history of progressive skin wrinkling. On examination, the patient appeared older than her actual age, with apparent wrinkling on the mid-torso with generalized smooth, erythematous macules and wheals. A family history of similar conditions was absent. Biopsy revealed hypersensitivity and atrophy. Following the Food and Drug Administration (FDA) guidelines, we administered antihistamines, which relieved the itching, but her hyperpigmentation and cutis laxa never improved. CONCLUSION: Our case shows that the decrease of elastic fibers may be associated with the infiltration of inflammatory cells in the dermis. This supports the hypothesis that chemical mediators may play a major role in the destruction of elastic fibers, thus causing cutis laxa. In addition, we advise practitioners to take a complete clinical and family history to determine if the condition is inherited or acquired.

Metatropic Dysplasia of Nonlethal Variant in a Chinese Child - A Case Report.

Metatropic dysplasia (MD), is a rare skeletal dysplasia occurring predominantly in infants characterized by a distinctive long torso and short limbs; it is as a result of mutations in the TRPV4 gene. However, a clear distinction between various forms of skeletal dysplasias caused by the transient receptor potential vanilloid 4 (TRPV4) gene is difficult but could be achieved by a combination of gene sequencing, medical and radiological criteria. We hereby report a case of a 14-month old girl who presented with an abnormal stature. The diagnosis of nonlethal MD was confirmed by X-ray with dumbbell-shaped long bones, platyspondyly, and delayed carpal ossification, as well as broadened pelvis with marginally widened ilia, epiphyseal plates, and slightly flattened acetabula. Furthermore, gene sequencing confirmed gene mutation on exon 15 of the TRPV4 gene with a heterozygous missense mutation (c.2396C > T), but no mutation was present in her parents. Our findings recorded metatropic dysplasia with the c.2396C > T mutation in the TRPV4 gene in China. This mutation caused changes in amino acid of TRPV4, which can induce growth retardation in children.

Differentiating Nonfunctional Paraganglioma of the Bladder from Urothelial Carcinoma of the Bladder: Pitfalls and Breakthroughs.

BACKGROUND: Although both nonfunctional paraganglioma of the bladder (NPB) and urothelial carcinoma of the bladder (UCB) are subtypes of bladder tumors, they are entirely different entities with distinct tissue origins and anatomical locations. However, NPB is frequently misdiagnosed as UCB chiefly due to the similarities in their clinical characteristics and cystoscopic features. This study aimed to compare the differences in their clinical characteristics and cystoscopic features. PATIENTS AND METHODS: Between April 2007 and September 2017, 14 patients with NPB (NPB group) were retrieved from 2 centers, and 42 patients with new-onset UCB (UCB group) were randomly retrieved. Demographic, symptomatic, imaging, and cystoscopic data of patients in both groups were collected and compared. RESULTS: NPB group comprised 7 males and 7 females, with a mean age of 43.1 ± 13.6 years. Compared with the UCB group, patients in the NPB group were significantly younger (p < 0.001), less likely to be male (p < 0.05), and to present with hematuria (p < 0.01). However, no significant difference in maximum tumor diameter was observed between the 2 groups (p=0.609). Compared with the UCB group, cystoscopically, patients in the NPB group were significantly more likely to present with hypervascularization but less likely to present with hemorrhage, necrosis, calcification, pedunculation, and multilesion (p < 0.05). No patients with NPB were clinically diagnosed correctly before cystoscopy. Of the 5 patients who underwent both cystoscopy and biopsy, 4 were diagnosed with NPB, while 1 remained undiagnosed. Of the remaining 9 patients who underwent cystoscopy only, 5 were diagnosed with nonepithelial tumor, and 4 were misdiagnosed with UCB. CONCLUSIONS: Age, sex, and hematuria may provide clues to differentiating NPB from UCB. Differences in cystoscopic features between NPB and UCB are of high diagnostic value. Cystoscopic biopsy should be considered in the preoperative diagnosis of NPB.

Early Intratracheal Administration of Corticosteroid and Pulmonary Surfactant for Preventing Bronchopulmonary Dysplasia in Preterm Infants with Neonatal Respiratory Distress Syndrome: A Meta-analysis.

There is uncertain result with regard to the use of inhalation or instillation steroids to prevent bronchopulmonary dysplasia in preterm infants. This meta-analysis was designed to evaluate the efficacy and safety of early airway administration (within 2 days after birth) of corticosteroids and pulmonary surfactant (PS) for preventing bronchopulmonary dysplasia (BPD) in premature infants with neonatal respiratory distress syndrome (NRDS). The related studies were retrieved in PubMed, EMBASE, the Cochrane Library, Clinical Trial, CNKI, Wanfang and VIP Database from inception to August 2018. Two reviewers independently screened the studies to ensure that all patients with diagnosis of NRDS were enrolled to studies within 1 day after birth, assessed the quality of included studies by GRADEpro system and extracted the data for review. The meta-analysis was performed by RevMan 5.2 software. A subgroup analysis about inhaled corticosteroid (ICS) delivery method was made between ICS inhalation subgroup [inhalation of ICS by nebulizer or metered dose inhaler (MDI)] and ICS intratracheal instillation subgroup (PS used as a vehicle). Eight randomized controlled trials were enrolled in the meta-analysis, 5 trials of which stated the randomized method, grouping and blinded method, and the follow-up procedures were reported. GRADEpro system showed high quality of 4 trials (5 articles), and the rest 4 trials had moderate quality. Meta-analysis showed that the incidence of BPD was decreased in ICS group, the relative risk (RR) was 0.56 (95% CI: 0.42-0.76), and similar trends were found in ICS inhalation subgroup and ICS intratracheal instillation subgroup, with the corresponding RR being 0.58 (95% CI: 0.41-0.82) and 0.47 (95% CI: 0.24-0.95) respectively. ICS could also significantly reduce the mortality risk as compared with placebo control group (RR: 0.67; 95% CI: 0.45-0.99), with RR of ICS inhalation subgroup and ICS intratracheal instillation subgroup being 0.81 (95% CI: 0.34-1.94) and 0.64 (95% CI: 0.41-0.99) respectively. Moreover, the percentage of infants using PS more than one time was lower in ICS group than in the placebo control group, with the RR and 95% CI being 0.55 (95% CI: 0.45-0.67), and that in ICS intratracheal instillation subgroup lower than in ICS inhalation subgroup (RR: 0.56; 95% CI: 0.45-0.69, and RR: 0.35; 95% CI: 0.08-1.52 respectively). There was no significant difference in the incidence of infection or retinopathy of prematurity and neuro-motor system impairment between ICS group and placebo control group, with the corresponding RR being 0.95 (95% CI: 0.59-1.52), 0.92 (95% CI: 0.62-1.38) and 1.13 (95% CI: 0.92-1.39), respectively. It was concluded that early administration of ICS and PS is an effective and safe option for preterm infants with NRDS in preventing BPD and reducing mortality, decreasing the additional PS usage, especially for the ICS intratracheal instillation subgroup. Furthermore, the appropriate dose and duration of ICS, combined use of inhalation or instillation of ICS with PS and the long-term safety of airway administration of corticosteroids need to be assessed in large trials.

Aneurysmal Fibrous Histiocytoma: Clinicopathology Analysis of 30 Cases of a Rare Variant of Cutaneous Fibrohistiocytoma.

Aneurysmal fibrous histiocytoma is often clinically misdiagnosed. In this study, we put forward an insight on how to help diagnose this disease clinically. A retrospective chart review was performed on all patients diagnosed with aneurysmal fibrous histiocytoma from 2007 to 2017 in the Department of Dermatology, Union Hospital, China, and all clinical data were collected from the hospital archives. From a total of 418 patients diagnosed with cutaneous fibrous histiocytoma, only 30 patients were confirmed to have aneurysmal fibrous histiocytoma out of which only 2 patients were clinically diagnosed with aneurysmal fibrous histiocytoma. The remaining 28 patients were diagnosed with various types of vascular tumors although pathology classified them as having aneurysmal fibrous histiocytoma. Among the 30 patients, 9 were male and 21 were female. There were following age groups: 13-19 (mean 16, n=4), 20-29 (mean 26.25, n=8), 30-39 (mean 33, n=7), 40-49 (mean 44, n=4), 50-59 (mean 56.75, n=4), 60 and above (mean 61, n=3). Tumors were present on the head, neck, back, waist, hips and upper and lower extremities. After complete excision, there was no recurrence and no complications. Histologically, lesions showed the typical pseudoangiomatoid spaces without endothelial lining and infiltration of fibrohistiocytes in hemosiderotic pigmentation. It was suggested that although the prognosis of aneurysmal fibrous histiocytoma is good, accurate diagnosis is paramount to avoid clinical misdiagnosis and subsequent complications.

Clinical significance of functional and anatomical classifications in paraganglioma of the urinary bladder.

OBJECTIVES: Paragangliomas of the urinary bladder (PUBs) are challenging catecholamine-producing neuroendocrine tumors. We aimed to facilitate their diagnosis and treatment by functional and anatomical classifications. MATERIALS AND METHODS: Between April 2007 and September 2017, 31 cases from 2 centers were retrieved, in which the patients were pathologically diagnosed with PUB. Besides classifying them into functional and nonfunctional PUBs, functional PUBs were further subclassified into typical functional PUB (with typical symptoms and elevated catecholamines/metabolites levels) and atypical functional PUB. Anatomically, they were classified into submucosal, intramural, and subserosal PUBs. RESULTS: Functionally, these cases comprised 17 (54.8%) functional and 14 (45.2%) nonfunctional PUBs. Functional PUBs had significantly larger diameters than nonfunctional PUBs (P < 0.01). Of the 17 functional PUB cases, 8 were further subclassified into typical functional PUB, of which 4 were diagnosed without cystoscopy. Anatomically, these cases comprised 14 (45.2%) submucosal, 13 (41.9%) intramural, and 4 (12.9%) subserosal PUBs. Intramural and subserosal PUBs had significantly larger diameters and were more likely to be functional than submucosal PUBs (P < 0.05). Cystoscopy failed to detect the tumor in all patients with subserosal PUB. Besides all patients with intramural or subserosal PUB, 1 patient with submucosal PUB underwent partial cystectomy. The remaining 13 patients with submucosal PUB underwent transurethral resection of bladder tumor, 5 of whom required extra surgical intervention. CONCLUSIONS: By functional classification, omitting cystoscopy is feasible in the diagnosis of typical functional PUBs. By anatomical classification, intramural, and subserosal PUBs tend to be large and functional. Moreover, negative cystoscopic findings are not sufficient to exclude subserosal PUBs. Finally, not all submucosal PUBs are amenable to transurethral resection of bladder tumor.

Efficacy of Hydroxy-L-proline (HYP) analogs in the treatment of primary hyperoxaluria in Drosophila Melanogaster.

BACKGROUND: Substrate reduction therapy with analogs reduces the accumulation of substrates by inhibiting the metabolic pathways involved in their biosynthesis, providing new treatment options for patients with primary hyperoxalurias (PHs) that often progress to end-stage renal disease (ESRD). This research aims to evaluate the inhibition efficacy of Hydroxy-L-proline (HYP) analogs against calcium oxalate (CaOx) crystal formation in the Drosophila Melanogaster (D. Melanogaster) by comparing them with Pyridoxine (Vitamin B6). METHODS: Three stocks of Drosophila Melanogaster (W118, CG3926 RNAi, and Act5C-GAL4/CyO) were utilized. Two stocks (CG3926 RNAi and Act5C-GAL4 /CyO) were crossed to generate the Act5C > dAGXT RNAi recombinant line (F1 generation) of D. Melanogaster which was used to compare the efficacy of Hydroxy-L-proline (HYP) analogs inhibiting CaOx crystal formation with Vitamin B6 as the traditional therapy for primary hyperoxaluria. RESULTS: Nephrolithiasis model was successfully constructed by downregulating the function of the dAGXT gene in D. Melanogaster (P-Value = 0.0045). Furthermore, the efficacy of Hydroxy-L-proline (HYP) analogs against CaOx crystal formation was demonstrated in vivo using D. Melanogaster model; the results showed that these L-Proline analogs were better in inhibiting stone formation at very low concentrations than Vitamin B6 (IC50 = 0.6 and 1.8% for standard and dietary salt growth medium respectively) compared to N-acetyl-L-Hydroxyproline (IC50 = 0.1% for both standard and dietary salt growth medium) and Baclofen (IC50 = 0.06 and 0.1% for standard and dietary salt growth medium respectively). Analysis of variance (ANOVA) also showed that Hydroxy-L-proline (HYP) analogs were better alternatives for CaOx inhibition at very low concentration especially when both genetics and environmental factors are intertwined (p < 0.0008) for the dietary salt growth medium and (P < 0.063) for standard growth medium. CONCLUSION: Addition of Hydroxy-L-Proline analogs to growth medium resulted in the reduction of CaOx crystals formation. These analogs show promise as potential inhibitors for oxalate reduction in Primary Hyperoxaluria.

Comparison of the efficacy and feasibility of laser enucleation of bladder tumor versus transurethral resection of bladder tumor: a meta-analysis.

The transurethral resection of bladder tumor (TURBT) remains the most widely used method in the surgical treatment of the non-muscle invasive bladder tumor (NMIBT). Despite its popularity, the laser technique has been widely used in urology as an alternative, via the application of transurethral laser enucleation of bladder tumor. The aim of the present study was to compare the efficacy and feasibility between transurethral laser enucleation and transurethral resection of bladder tumor. A systematic search of the following databases was conducted: PubMed, Wed of Science, Cochrane Library, EMBASE, Google scholar, and Medline. The search included studies up to the 1st of January 2017. The outcomes of interest that were used in order to assess the two techniques included operation time, catheterization time, hospitalization time, obturator nerve reflex, bladder perforation, bladder irritation, 24-month-recurrence rate, and the postoperative adjuvant intravesical chemotherapy. A total of 13 trials with 2012 participants were included, of which 975 and 1037 underwent transurethral laser enucleation and transurethral resection of bladder tumor, respectively. No significant difference was noted in the operation time between the two groups, although significant differences were reported for the variables catheterization time, hospitalization time, obturator nerve reflex, bladder perforation, bladder irritation, and 24-month-recurrence rate. In the mitomycin and epirubicin subgroups, no significant differences were observed in the laser enucleation and TURBT methods with regard to the 24-month-recurrence rate. The laser enucleation was superior to TURBT with regard to the parameters obturator nerve reflex, bladder perforation, catheterization time, hospitalization time, and 24-month-recurrence rate. Moreover, laser enucleation can offer a more accurate result of the tumor's pathological stage and grade.