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1.
PLoS One ; 19(6): e0305869, 2024.
Article in English | MEDLINE | ID: mdl-38913676

ABSTRACT

BACKGROUND: This study aimed to measure the preferences for mental health support among health professionals, their willingness to support the mental health of colleagues and associated factors. METHOD: A descriptive cross-sectional study was performed from August to October 2022 within five hospitals located in Hanoi, Vietnam. A total of 244 health professionals participated in the study. Data on socio-economic status, health and COVID-19-related characteristics, Depression Anxiety Stress Scale (DASS-21); and preferences for mental health support services were collected by using a structured self-reported questionnaire. Multivariate logistic regression models were utilized to identify associated factors with the demand for mental support services. RESULTS: 13.9%, 17.1% and 8.6% reported having at least mild depression, anxiety and stress, respectively. There 13.9% did not seek any mental health support during the COVID-19 pandemic. The most common support included talking with friends (52.9%), family (50.8%), colleagues (47.6%) and using social networks/Internet (43.5%). There 31.1% had been aware of mental health services, but only 18.0% used this service at least once. Regarding preferences, 47.3% had a demand for mental support services, and the most preferred service was providing coping skills (25.9%), followed by skills to support others against mental problems (22.2%). Major sources of support included psychiatrists (34.4%), colleagues (29.1%) and family (27.9%). The main preferred channels for support included telephone/mobile phone (35.7%) and Internet (20.9%). Only 12.3% were willing to provide mental support for colleagues during the pandemic. Age, education, perceived mental health status, ever seeking any mental service, and DASS-21 depression score were associated with demand for mental support services. CONCLUSION: This study found a lack of awareness of mental health services for health professionals, as well as moderate levels of demand for this service in this population. Raising awareness and developing tailored mental health support services are important to enhancing the mental well-being of health professionals in Vietnam to prepare for the next pandemic.


Subject(s)
COVID-19 , Depression , Health Personnel , Mental Health Services , Mental Health , Humans , COVID-19/epidemiology , COVID-19/psychology , Vietnam/epidemiology , Male , Female , Adult , Cross-Sectional Studies , Health Personnel/psychology , Middle Aged , Depression/epidemiology , Anxiety/epidemiology , Pandemics , Surveys and Questionnaires , SARS-CoV-2 , Stress, Psychological/epidemiology , Social Support
3.
Cureus ; 16(3): e57164, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38681391

ABSTRACT

Purpose We investigated the impact of intravascular ultrasound guidance on reducing the incidence of contrast-induced acute kidney injury (CI-AKI) in patients undergoing percutaneous coronary intervention (PCI). Methods Ninety-nine patients were enrolled in this prospective cohort who were not randomly assigned to angiography-guided percutaneous coronary intervention or intravascular ultrasound-guided percutaneous coronary intervention. The patients were hospitalized at the Vietnam National Heart Institute - Bach Mai Hospital between 2019 and 2020. Acute kidney injury incidence during hospitalization was the primary endpoint. Results A total of 99 patients were divided into two groups: the intravascular ultrasound-guided group (33 participants) and the angiography-guided group (66 participants). The mean ± SD contrast volume of each group was 95.2 ± 37.1 mL and 133.0 ± 36.0 mL for the ultrasound-guided and angiography-guided groups, with P < 0.0001. Intravascular imaging-guided percutaneous coronary intervention (IVUS-guided PCI) was associated with reduced acute kidney injury incidence during hospitalization: 0.0% vs. 12.12% and P = 0.049. Conclusions Intravascular ultrasound is a safe imaging tool that guides percutaneous coronary intervention and significantly reduces the rate of acute kidney injury compared to angiography alone. Patients who have a high chance of experiencing acute kidney injury benefit from using intravascular ultrasound.

4.
Ann Med Surg (Lond) ; 86(3): 1261-1267, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38463078

ABSTRACT

Introduction: Acute-on-chronic liver failure (ACLF) has a high mortality rate, and liver transplantation is considered a definite treatment for patients with this condition. This study aims to evaluate the outcomes of living donor liver transplantation (LDLT) in ACLF patients in a single centre in a lower middle-income country, Vietnam. Materials and methods: This was a retrospective study at the 108 Military Central Hospital (Hanoi, Vietnam), enroling 51 patients diagnosed with ACLF based on Asian Pacific Association for the Study of the Liver (APASL) criteria who underwent LDLT with a right lobe graft from December 2019 to December 2022. The authors utilize the model for end-stage liver disease (MELD) and APASL ACLF Research Consortium (AARC) scores to evaluate and stratify the severity of ACLF. Results: The average age of all patients was 47.27±13.61, with 88.24% being male. The average BMI was 22.78±2.61. The most common underlying liver disease was chronic viral hepatitis B (88.2%). The average MELD score of the patients was 34.90±5.61, with 33.3% having MELD score greater than or equal to 40. In terms of ACLF severity, five patients (9.8%) had grade I ACLF, 35 patients (68.6%) had grade II ACLF, and 11 patients (21.6%) had grade III ACLF. The average AARC score was 9.43±1.68. The duration of treatment in the ICU was 8.59±7.27 days, and the length of hospital stay was 28.02±13.45 days. The most common post-transplant complication was biliary complication (19.61%). Death occurred in 7 patients (13.7%). The survival rates at 6 months, 1 year, and 3 years were 84%, 81.7%, and 81.7%, respectively. Conclusion: Living donor liver transplantation for ACLF patients is safe and has a high post-transplant survival rate. Multidisciplinary care before, during, and after surgery, and the decision to do a liver transplant early, is essential in saving the lives of ACLF patients.

5.
Polymers (Basel) ; 16(3)2024 Jan 31.
Article in English | MEDLINE | ID: mdl-38337283

ABSTRACT

This study surveys the impacts of injection parameters on the deformation rate of the injected flexure hinge made from ABS, PP, and HDPE. The flexure hinges are generated with different filling time, filling pressure, filling speed, packing time, packing pressure, cooling time, and melt temperature. The amplification ratio of the samples between different injection parameters and different plastic types is measured and compared to figure out the optimal one with a high amplification ratio. The results show that the relationship between the input and output data of the ABS, PP, and HDPE flexure hinges at different injection molding parameters is a linear relation. Changing the material or many injection molding parameters of the hinge could lead to a great impact on the hinge's performance. However, changing each parameter does not lead to a sudden change in the input and output values. Each plastic material has different optimal injection parameters and displacement behaviors. With the ABS flexure hinge, the filling pressure case has the greatest amplification ratio of 8.81, while the filling speed case has the lowest value of 4.81. With the optimal injection parameter and the input value of 105 µm, the ABS flexure hinge could create a maximum average output value of 736.6 µm. With the PP flexure hinge, the melt temperature case achieves the greatest amplification ratio of 6.73, while the filling speed case has the lowest value of 4.1. With the optimal injection parameter and the input value of 128 µm, the PP flexure hinge could create a maximum average output value of 964.8 µm. The average amplification ratio values of all injection molding parameters are 6.85, 5.41, and 4.01, corresponding to ABS, PP, and HDPE flexure hinges. Generally, the ABS flexure hinge has the highest amplification ratios, followed by the PP flexure hinge. The HDPE flexure hinge has the lowest amplification ratios among these plastic types. With the optimal injection parameter and the input value of 218 µm, the HDPE flexure hinge could create a maximum average output value of 699.8 µm. The results provide more insight into plastic flexure hinges and broaden their applications by finding the optimal injection parameters and plastic types.

6.
FEBS J ; 291(5): 927-944, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38009294

ABSTRACT

There has been a great deal of research on cell division and its mechanisms; however, its processes still have many unknowns. To find novel proteins that regulate cell division, we performed the screening using siRNAs and/or the expression plasmid of the target genes and identified leucine zipper protein 1 (LUZP1). Recent studies have shown that LUZP1 interacts with various proteins and stabilizes the actin cytoskeleton; however, the function of LUZP1 in mitosis is not known. In this study, we found that LUZP1 colocalized with the chromosomal passenger complex (CPC) at the centromere in metaphase and at the central spindle in anaphase and that these LUZP1 localizations were regulated by CPC activity and kinesin family member 20A (KIF20A). Mass spectrometry analysis identified that LUZP1 interacted with death-associated protein kinase 3 (DAPK3), one regulator of the cleavage furrow ingression in cytokinesis. In addition, we found that LUZP1 also interacted with myosin light chain 9 (MYL9), a substrate of DAPK3, and comprehensively inhibited MYL9 phosphorylation by DAPK3. In line with a known role for MYL9 in the actin-myosin contraction, LUZP1 suppression accelerated the constriction velocity at the division plane in our time-lapse analysis. Our study indicates that LUZP1 is a novel regulator for cytokinesis that regulates the constriction velocity of the contractile ring.


Subject(s)
Cytokinesis , Leucine Zippers , Cytokinesis/genetics , Constriction , Actin Cytoskeleton , Mitosis
7.
Ann Hepatobiliary Pancreat Surg ; 28(1): 42-47, 2024 Feb 29.
Article in English | MEDLINE | ID: mdl-38114078

ABSTRACT

Backgrounds/Aims: Hepatolithiasis and choledocholithiasis are frequent pathologies and unfortunately, with the current treatment strategies, the recurrence incidence is still high. This study aimed to assess the outcomes of laparoscopic choledochotomy using cholangioscopy via the percutaneous-choledochal tube for the treatment of hepatolithiasis and choledocholithiasis in Vietnamese patients. Methods: A cross-sectional study of patients with hepatolithiasis and/or choledocholithiasis who underwent laparoscopic choledochotomy using intraoperative cholangioscopy via percutaneous-choledochal tube at the Department of Hepatopancreatobiliary Surgery, 108 Military Central Hospital, from June 2017 to March 2020. Results: A total of 84 patients were analyzed. Most patients were females (56.0%) with a median age of 55.56 years. Among them, 41.8% of patients had previous abdominal operations, with 33.4% having choledochotomy. All patients underwent successful laparoscopic common bile duct exploration followed by T-tube drainage without needing to convert to open surgery. Most patients (64.3%) had both intrahepatic and extrahepatic stones. The rate of stones ≥ 10 mm in diameter was 64.3%. Biliary strictures were observed in 19.1% of patients during cholangioscopy. Complete removal of stones was achieved in 54.8% of patients. Intraoperative complications were encountered in two patients, but there was no need to change the strategy. The mean operating time was 121.85 ± 30.47 minutes. The early postoperative complication rate was 9.6%, and all patients were managed conservatively. The residual stones were removed through the T-tube tract by subsequent choledochoscopy in 34/38 patients, so the total success rate was 95.2%. Conclusions: Laparoscopic choledochotomy combined with cholangioscopy through the percutaneous-choledochal tube is a safe and effective strategy for hepatolithiasis and/or choledocholithiasis, even in patients with a previous choledochotomy.

8.
Glob Pediatr Health ; 10: 2333794X231216556, 2023.
Article in English | MEDLINE | ID: mdl-38073663

ABSTRACT

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome and Stevens-Johnson Syndrome (SJS) are severe cutaneous adverse reactions to drugs. Those reactions which are rare in children can be especially severe and challenging to diagnose and manage. Herein we present a 59-month-old male who presented with a rash, fever, and multiple organ dysfunction initiation of Phenobarbital for epilepsy. Diagnosis of ovelaping SJS and DRESS syndrome had been made based on clinical manifestations accompanied with skin biopsy according to RegisSCAR diagnostic criteria. A therapy with intravenous immune globulin (IVIG), corticosteroids and supportive care was given successfully for the patient. This case underscored the significance of promptly and effectively recognizing and managing these intricate reactions.

9.
Arch Craniofac Surg ; 2023 Dec 08.
Article in English | MEDLINE | ID: mdl-38072001

ABSTRACT

Ethmoid myoepithelial carcinoma is a rare tumor, with only 14 cases reported to date. This report discusses the largest tumor of this type ever recorded in the ethmoid region. The tumor caused extensive damage to facial structures, complicating treatment. The patient's age and comorbidities increased the risk of intraoperative bleeding, presenting challenges to the complete removal of the tumor and the reconstruction of the damaged structures. To reduce the risk of intraoperative hemorrhage, shorten the surgery time, and manage potential heartrelated complications, arterial embolization was performed using gelatin sponges and coils. Definitive surgery was then carried out using a skin flap and mucosal flap to successfully reconstruct the defect. Postoperative radiotherapy was deemed unnecessary. The patient recovered well, with a satisfactory aesthetic outcome. No recurrence was observed during a 3-year follow-up period.

11.
Pract Lab Med ; 37: e00339, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37886110

ABSTRACT

Backgrounds and aims: The aim of this study is to provide a good approach for a quantitative EQA scheme assigned value with limited resources. Materials and methods: Twelve lyophilized EQA items were distributed to participants in 2021 from North to Southeast Vietnam to measure the concentration of nine parameters, including glucose, urea, creatinine, cholesterol, triglyceride, uric acid, AST, ALT, and GGT. The consensus value of the expert group and all participants were calculated and statistically compared to choose the most appropriate consensus value. Results: Fifty-nine laboratories attended the EQA scheme, including an expert group using automatic biochemistry analyzers (AAs) and all participants with auto and semi-auto biochemistry (SAA) analyzers. Consensus values of six per nine parameters were different between the two groups for at least two EQA items, including glucose, creatinine, cholesterol, uric acid, AST, and ALT. The coefficients of variation of glucose, urea, creatinine, triglycerides, uric acid, and GGT in the expert group were significantly lower than those in all the participants. Conclusion: Using the consensus values of expert groups as the assigned values of the EQA program is a relevant strategy to increase testing quality in developing countries with limited resources, such as Vietnam.

12.
J Clin Lab Anal ; 37(15-16): e24958, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37621139

ABSTRACT

OBJECTIVE: To develop and validate an LC-M/SMS method for the determination of tacrolimus in human whole blood. METHOD: The LC-MS/MS method for the determination of tacrolimus in whole blood was developed and validated according to the guidelines. Concentrations of TAC in 100 kidney transplant patients measured by LC-MS/MS were compared with CMIA using correlation analysis and Bland-Altman plots. RESULTS: The method had a total chromatographic run time of 5 min. The calibration curves were linear over the range of 0.5-100.0 ng/mL with a lower limit of quantification of 1 ng/mL. The intra- and interday accuracy was within the range of 93.3%-109.2% and 96.0%-108.4%, respectively, with precision ranging from 0.8 to 9.4%. The mean extraction recoveries of TAC ranged from 102.6 to 107.8%. The mean concentrations of TAC in whole blood of kidney transplant patients measured by the two assays were different at 1, 3 months and all time points (p < 0.001), but no significant difference was observed at 6 months (p = 0.094). The correlation of data was good with the correlation coefficients (r2 ) of 0.7581, 0.8811, 0.8777, and 0.8077, respectively. Passing-Bablok regression analysis demonstrated good correlations with r2 values higher than 0.88 between TAC levels measured by LC-MS/MS and CMIA. Using Bland-Altman plots yielded average biases of 1.29, 0.79, 0.11, and 0.65 ng/mL at 1, 3, and 6 months and all time points. CONCLUSION: The LC-MS/MS method was validated for the accurate determination of TAC in human whole blood. The comparison of tacrolimus concentrations measured by the LC-MS/MS with CMIA showed a good correlation and agreement of two methods, suggesting LC-MS/MS should be used routinely to monitor TAC concentrations in kidney transplant patients.


Subject(s)
Kidney Transplantation , Tacrolimus , Humans , Chromatography, Liquid/methods , Tandem Mass Spectrometry/methods , Drug Monitoring/methods , Immunosuppressive Agents
13.
Beilstein J Nanotechnol ; 14: 781-792, 2023.
Article in English | MEDLINE | ID: mdl-37441001

ABSTRACT

We present the in situ synthesis of silver nanoparticles (AgNPs) through ionotropic gelation utilizing the biodegradable saccharides lactose (Lac) and alginate (Alg). The lactose reduced silver ions to form AgNPs. The crystallite structure of the nanocomposite AgNPs@Lac/Alg, with a mean size of 4-6 nm, was confirmed by analytical techniques. The nanocomposite exhibited high catalytic performance in degrading the pollutants methyl orange and rhodamine B. The antibacterial activity of the nanocomposite is pH-dependent, related to the alterations in surface properties of the nanocomposite at different pH values. At pH 6, the nanocomposite demonstrated the highest antibacterial activity. These findings suggest that this nanocomposite has the potential to be tailored for specific applications in environmental and medicinal treatments, making it a highly promising material.

14.
Light Sci Appl ; 12(1): 146, 2023 Jun 15.
Article in English | MEDLINE | ID: mdl-37322023

ABSTRACT

The realization of hybrid optics could be one of the best ways to fulfill the technological requirements of compact, light-weight, and multi-functional optical systems for modern industries. Planar diffractive lens (PDL) such as diffractive lenses, photonsieves, and metasurfaces can be patterned on ultra-thin flexible and stretchable substrates and be conformally attached on top of arbitrarily shaped surfaces. In this review, we introduce recent research works addressed to the design and manufacturing of ultra-thin graphene optics, which will open new markets in compact and light-weight optics for next-generation endoscopic brain imaging, space internet, real-time surface profilometry, and multi-functional mobile phones. To provide higher design flexibility, lower process complexity, and chemical-free process with reasonable investment cost, direct laser writing (DLW) of laser-induced-graphene (LIG) is actively being applied to the patterning of PDL. For realizing the best optical performances in DLW, photon-material interactions have been studied in detail with respect to different laser parameters; the resulting optical characteristics have been evaluated in terms of amplitude and phase. A series of exemplary laser-written 1D and 2D PDL structures have been actively demonstrated with different base materials, and then, the cases are being expanded to plasmonic and holographic structures. The combination of these ultra-thin and light-weight PDL with conventional bulk refractive or reflective optical elements could bring together the advantages of each optical element. By integrating these suggestions, we suggest a way to realize the hybrid PDL to be used in the future micro-electronics surface inspection, biomedical, outer space, and extended reality (XR) industries.

15.
Int Rev Financ Anal ; 88: 102675, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37144179

ABSTRACT

This study explores the association between the Covid-19 outbreak, corporate financial distress and earnings management practices in China. We investigate whether firms took advantage of the downturn in economic conditions during the pandemic to adjust their earnings using different earnings management techniques. Utilising a sample of 1832 listed firms and underlying theoretical frameworks (i.e., positive accounting and signalling theory), we find that firms were more inclined to manage earnings during the pandemic period. They favoured using the accrual-based rather than the real activity-based earnings management technique. We also find that firms engaged more in income-increasing practices in the shadow of the outbreak. In addition, our results further demonstrate that financially distressed firms were involved in earnings management, particularly accrual-based earnings management. However, compared to privately-owned firms, state-owned enterprises seem to be involved less in earnings management during the Covid-19 pandemic. Findings from this study raise some concerns for policymakers about the credibility of financial reporting information during Covid-19.

16.
World J Surg Oncol ; 21(1): 140, 2023 May 05.
Article in English | MEDLINE | ID: mdl-37147674

ABSTRACT

PURPOSE: Total laparoscopic right hemicolectomy with complete mesocolic excision (CME), central vascular ligation (CVL), and D3 lymphadenectomy is still the most challenging colon procedures for gastrointestinal surgeons. We herein report the technical details and our preliminary experience of Bach Mai Procedure - a novel-combining (cranial, medial to lateral, and caudal) approach with early resection of the terminal ileum. METHODS: The dissection stage was central vascular isolation and ligation by a combined multiple approaches in the following four steps: cranial approach, dissecting along the inferior aspect of pancreatic isthmus to reveal the middle colic vessels and the anterior aspect of the superior mesentery vein and then exposed the right gastroepiploic vein and the trunk of Henle; medial-to-lateral approach, exposing the surgical axis - the superior mesenteric vascular axis and then early resection of the terminal ileum to open the dissection from the bottom up; and caudal approach, radical ligation of the ileocecal artery and right colic artery (central vascular ligation), lymph node dissection (D3 lymphadenectomy), and resecting the Toldt fascia of the colon to release the entire right colon from the abdominal wall. RESULTS: In 12 months, there were 32 cases of primary right-sided colon malignancies that have undergone tLRHD3, CME/CVL based on the Bach Mai Procedure. In 3 cases (9.4%), the tumor site was hepatic flexure. The median of lymph node number (LNN) was 38, with the maximum number which was 101. No serious postoperative complications (grade 3 or higher) neither inhospital mortality was detected. CONCLUSION: This Bach Mai procedure, a novel-combining approach with early resection of the terminal ileum, is technically feasible and safe for tLRHD3, CME/CVL. Further investigations and follow-up must be proceeded to evaluate the long-term outcomes of our technique.


Subject(s)
Colonic Neoplasms , Laparoscopy , Mesocolon , Humans , Prospective Studies , Colonic Neoplasms/surgery , Colonic Neoplasms/pathology , Lymph Node Excision/methods , Ligation , Mesocolon/surgery , Mesocolon/pathology , Colectomy/methods , Laparoscopy/methods
17.
Radiol Case Rep ; 18(4): 1549-1551, 2023 Apr.
Article in English | MEDLINE | ID: mdl-36815144

ABSTRACT

Posterior reversible encephalopathy syndrome is a clinicoradiologic entity characterized by seizure, headaches, visual symptoms, impaired consciousness, and vasogenic cerebral edema of occipital and parietal lobes of the brain. Magnetic resonance imaging (MRI) is the diagnostic gold standard. The pathophysiology of posterior reversible encephalopathy syndrome is still unknown, but it is thought to be closely related to several medical conditions including hypertension, preeclampsia, eclampsia, immunosuppressive agents, transplantation, and sepsis. We report a rare case of posterior reversible encephalopathy syndrome in patient with myasthenia gravis and sepsis. A 22-year-old male was diagnosed with myasthenia gravis combined with sepsis due to pneumonia. During his recovery, the patient suffered multiple generalized convulsions and subsequent loss of consciousness. On cranial MRI, the abnormalities were observed with hyperintense within the subcortical white matter of the temporal, parietal, and bilateral occipital lobes on T2-weighted and T2 FLAIR. Reversibility of the symptoms and characteristic imaging findings led us to a diagnosis of posterior reversible encephalopathy syndrome. Early recognition and management of posterior reversible encephalopathy syndrome as a cause of encephalopathy in patients with septicemia and myasthenia gravis is necessary to prevent secondary complications in this condition.

18.
Wounds ; 35(1): E7-E13, 2023 01.
Article in English | MEDLINE | ID: mdl-36749991

ABSTRACT

INTRODUCTION: Radiation-induced ulceration is a late-stage skin reaction after RT for cancer treatment. OBJECTIVE: The present study examined the use of a single-stage reconstructive procedure to manage radiation-related wounds. MATERIALS AND METHODS: Nine patients with radiation-induced chronic ulcer with accompanying severe complications were admitted to the Plastic, Reconstructive, and Regenerative Center of Viet Nam National Burn Hospital between October 2015 and September 2019. The patients ranged in age from 49 to 77 years. Complications included exposed cheekbone (n = 1), exposed trachea (n = 1), exposed carotid artery (n = 2), exposed axillary artery (n = 2), exposed pleura (n = 1), and exposed pericardium (n = 2). Flap type used to achieve defect coverage after debridement was individualized to each patient and included ALT, LD, SCA, and DIEP flaps. Additionally, a high-density polyethylene was used to reconstruct the trachea to recover breathing function in the patient with exposed trachea. RESULTS: Complete survival of all flaps was achieved. Most vital organs (the trachea, axillary artery, and carotid artery) were covered. Hospital length of stay ranged from 15 to 120 days. CONCLUSIONS: The successful management of patients with severe complications suggests that immediate single-stage reconstruction may be a valuable option for managing radiation-induced ulcers.


Subject(s)
Plastic Surgery Procedures , Radiodermatitis , Skin Ulcer , Surgery, Plastic , Humans , Middle Aged , Aged , Ulcer , Debridement , Surgical Flaps/blood supply , Treatment Outcome
19.
Pediatr Neonatol ; 64(4): 405-410, 2023 07.
Article in English | MEDLINE | ID: mdl-36658016

ABSTRACT

BACKGROUND: Pachyonychia congenita (PC) is a group of autosomal dominant disorders caused by mutations in one of five keratin genes (KRT6A, KRT6B, KRT6C, KRT16, or KRT17). PC is an extremely rare condition. To our knowledge, this is the largest genotype-phenotype study of PC in a Vietnamese population to date. MATERIALS AND METHODS: We investigated keratin gene mutations and clinical features of seven Vietnamese children with PC. RESULTS: The seven Vietnamese patients were from six different families (two patients in the same family) from across Northern, Central, and Southern Vietnam. All children displayed PC symptoms before 1 year of age, but diagnosis was delayed in 4/7 patients. Thick fingernails, thick toenails, oral leukokeratosis, and follicular hyperkeratosis were the most common features recorded by all seven patients. Plantar keratoderma and thick fingernails were the clinical features associated with the most significant effect on daily function. All patients had mutations in KRT6A (PC-K6a) focused on the 1A and 2B domains. We found three distinct types of mutations (K6a R466P, K6a N171K, and K6a N172del). One mutation (N172del) was common to 5/7 (71.4%) of the patients. CONCLUSIONS: Individuals displaying nail dystrophy, oral leukokeratosis, follicular hyperkeratosis, and plantar keratoderma should be referred for genetic testing given the high likelihood of a PC-K6a-related mutation in patients with this constellation of clinical signs.


Subject(s)
Exanthema , Pachyonychia Congenita , Humans , Child , Pachyonychia Congenita/genetics , Pachyonychia Congenita/complications , Pachyonychia Congenita/diagnosis , Keratin-6/genetics , Southeast Asian People , Vietnam , Genotype , Phenotype , Mutation , Keratins/genetics , Leukoplakia, Oral/complications
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