• Archives of Plastic Surgery
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• v.50 no.6
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• pp.573-577
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• 2023

Pachyonychia congenita is a rare genetic disorder characterized by hypertrophic nail plates, hyperkeratotic nail beds, and thickened hyponychium of the fingers and toes, impairing manual dexterity and resulting in poor aesthetics. The current body of literature describes various treatment modalities, but no singular approach has been defined as the gold standard. In this case, the authors employed different surgical techniques for treating pachyonychia congenita to evaluate the most effective approach. A 3-year-old boy presented with hypertrophic nail growth involving all digits of both hands and feet. Three surgical procedures were performed on the patient's fingers and toes using germinal matrix excision (GME) alone, GME plus partial sterile matrix excision (pSME), or GME plus complete sterile matrix excision (cSME). The digits treated with GME + cSME exhibited no recurrence of nail growth. Those treated with GME alone exhibited recurrence of hypertrophic nail growth, although their growth slowed. Excision of GME + cSME prevented recurrence of hypertrophic nails, while GME alone or with pSME led to slower-growing hypertrophic nails. Complete excision of the germinal and sterile matrices with skin graft closure may be a definitive treatment for pachyonychia congenita, but further studies are needed to validate these findings.

• Dementia and Neurocognitive Disorders
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• v.23 no.1
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• pp.1-10
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• 2024

Background and Purpose: Voice, reflecting cerebral functions, holds potential for analyzing and understanding brain function, especially in the context of cognitive impairment (CI) and Alzheimer's disease (AD). This study used voice data to distinguish between normal cognition and CI or Alzheimer's disease dementia (ADD). Methods: This study enrolled 3 groups of subjects: 1) 52 subjects with subjective cognitive decline; 2) 110 subjects with mild CI; and 3) 59 subjects with ADD. Voice features were extracted using Mel-frequency cepstral coefficients and Chroma. Results: A deep neural network (DNN) model showed promising performance, with an accuracy of roughly 81% in 10 trials in predicting ADD, which increased to an average value of about 82.0%±1.6% when evaluated against unseen test dataset. Conclusions: Although results did not demonstrate the level of accuracy necessary for a definitive clinical tool, they provided a compelling proof-of-concept for the potential use of voice data in cognitive status assessment. DNN algorithms using voice offer a promising approach to early detection of AD. They could improve the accuracy and accessibility of diagnosis, ultimately leading to better outcomes for patients.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.28 no.1
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• pp.92-98
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• 2024

The solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas is an uncommon tumor that accounts for approximately 1%-2% of exocrine pancreatic neoplasms. It predominantly affects female in their second and third decades of life. In this case report, we present a clinical scenario of a 21-year-old pregnant woman who incidentally discovered a solid cystic lesion in her pancreas, exhibiting features suggestive of SPEN. The patient underwent surgery during the second trimester. Management of pregnant females with SPEN poses challenges due to the absence of definitive treatment guidelines, particularly in determining the ideal timing for surgical intervention. Notably, during pregnancy, the presence of a small SPEN does not necessarily require immediate resection. However, if the tumor is of significant size, it can give rise to complications such as tumor rupture, multivisceral resection, recurrence, spontaneous abortion, intrauterine growth restriction, or premature delivery if not addressed. In the existing literature, a common finding is that approximately two-thirds of pregnant females with SPEN underwent surgery in the second trimester, often without complications for the mother or fetus. All these tumors were larger than 8 cm. The decision to operate before or after birth can be individualized based on team discussion. However, delay in surgery may lead to larger tumors and higher risks like bleeding, rupture, multivisceral resection, and recurrence. Therefore, second-trimester surgery seems safer, and lessens dangers, emergency surgery, and tumor recurrence.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.28 no.1
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• pp.48-52
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• 2024

Backgrounds/Aims: Splenectomy is the most frequently performed procedure as definitive management or as part of shunt surgery or devascularization in portal hypertension. Splenectomy is technically challenging because of the frequent coexistence of multiple collateral varices, splenomegaly, poor liver function, and thrombocytopenia. Early arterial ligation and late mobilization (EALDEM) is the traditional method for splenectomy in portal hypertension. Early spleen mobilization offers good control of the hilum. We aim to compare the effect of the early mobilization and delayed arterial ligation (EMDAL) technique with that of the conventional splenectomy technique in patients with portal hypertension. Methods: During the study period from September 2011 to September 2022, 173 patients underwent surgical intervention for portal hypertension at our institution. Among these patients, 114 underwent the conventional method of splenectomy (early arterial ligation and late splenic mobilization) while 59 underwent splenectomy with the EMDAL technique. Demographics were compared between the two groups. Intraoperative and postoperative outcomes were analyzed using the Mann-Whitney test in each group. A minimum follow-up of 12 months was performed in each group. Results: Demographics and type of surgical procedure were comparable in the two surgical method groups. Median blood loss was higher in the conventional group than in the EMDAL method. The median duration of surgery was comparable in the two surgical procedures. Clavien-Dindo grade III/IV complications were reported more frequently in the conventional group. Conclusions: The splenic hilum can be controlled well and bleeding can be minimised with early mobilization and delayed arterial ligation.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.26 no.4
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• pp.325-332
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• 2022

Backgrounds/Aims: To analyze relationships of hepatic histopathological findings and bile microbiological profiles with perioperative outcomes and risk of late biliary stricture in individuals undergoing surgical bile duct injury (BDI) repair. Methods: A historical cohort study was carried out at a tertiary university hospital. Fifty-six individuals who underwent surgical BDI repair from 2014-2018 with a minimal follow-up of 24 months were enrolled. Liver biopsies were performed to analyze histopathology. Bile samples were collected during repair procedures. Hepatic histopathological findings and bile microbiological profiles were then correlated with perioperative and late outcomes through uni- and multi-variate analyses. Results: Forty-three individuals (76.8%) were females and average age was 47.2 ± 13.2 years; mean follow-up was 38.1 ± 18.6 months. The commonest histopathological finding was hepatic fibrosis (87.5%). Bile cultures were positive in 53.5%. The main surgical technique was Roux-en-Y hepaticojejunostomy (96.4%). Overall morbidity was 35.7%. In univariate analysis, liver fibrosis correlated with the duration of the operation (R = 0.3; p = 0.02). In multivariate analysis, fibrosis (R = 0.36; p = 0.02) and cholestasis (R = 0.34; p = 0.02) independently correlated with operative time. Strasberg classification independently correlated with estimated bleeding (R = 0.31; p = 0.049). The time elapsed between primary cholecystectomy and BDI repair correlated with hepatic fibrosis (R = 0.4; p = 0.01). Conclusions: Bacterial contamination of bile was observed in most cases. The degree of fibrosis and cholestasis correlated with operative time. The waiting time for definitive repair correlated with the severity of liver fibrosis.

• Korean Circulation Journal
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• v.52 no.4
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• pp.288-300
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• 2022

Background and Objectives: We compared real-world clinical outcomes of patients receiving intravascular lithotripsy (IVL) versus rotational atherectomy (RA) for heavily calcified coronary lesions. Methods: Fifty-three patients who received IVL from January 2017 to July 2020 were retrospectively compared to 271 patients who received RA from January 2017 to December 2018. Primary endpoints were in-hospital and 30-day major adverse cardiovascular events (MACE). Results: IVL patients had a higher prevalence of acute coronary syndrome (56.6% vs 24.4, p<0.001), multivessel disease (96.2% vs 73.3%, p<0.001) and emergency procedures (17.0% vs 2.2%, p<0.001) compared to RA. In-hospital MACE (11.3% vs 5.9%, p=0.152), MI (7.5% vs 3.3%, p=0.152), and mortality (5.7% vs 3.0%, p=0.319) were not statistically significant. 30-day MACE was higher in the IVL cohort vs RA (17.0% vs 7.4%, p=0.035). Propensity score adjusted regression using IVL was also performed on in-hospital MACE (odds ratio [OR], 1.677; 95% confidence interval [CI], 0.588-4.779) and 30-day MACE (OR, 1.910; 95% CI, 0.774-4.718). Conclusions: These findings represent our initial IVL experience in a high-risk, real-world cohort. Although the event rate in the IVL arm was numerically higher compared to RA, the small numbers and retrospective nature of this study preclude definitive conclusions. These clinical outcomes are likely to improve with greater experience and better case selection, allowing IVL to effectively treat complex calcified coronary lesions.

• The Korean Journal of Medicine
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• v.99 no.2
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• pp.116-121
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• 2024

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly involving medium- or small-sized arteries, typically of the kidneys and other internal organs. Given the rarity of PAN and the variable clinical presentation, diagnosis is challenging and, to date, no definitive diagnostic marker has been identified. A patient diagnosed with immunoglobulin A nephropathy was observed to exhibit deterioration in renal function. To determine whether new structural abnormalities had developed, computed tomography scans of the kidneys, ureters, and bladder were obtained. Both kidneys exhibited multiple cortical defects, and a renal angiogram was performed to determine the cause. Angiography revealed partial obliteration of the left distal renal artery branches and multifocal extensive infarctions in both kidneys, and the patient was diagnosed with renal-limited PAN. Following steroid monotherapy, an improvement in renal function was observed. We believe that this case report may be helpful to physicians who assess and treat patients with suspected renal-limited PAN.

• The Journal of Advanced Prosthodontics
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• v.16 no.4
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• pp.231-243
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• 2024

PURPOSE. The objective of the study was to analyze the impact of cement, bonding pretreatment, and ceramic abutment material on the overall color results of CAD-CAM ceramic crowns bonded to titanium-based hybrid abutments. MATERIALS AND METHODS. For single implant restoration of a maxillary lateral incisor a total of 51 CAD-CAM-fabricated monolithic lithium disilicate crowns were fabricated and subsequently bonded onto 24 lithium disilicate Ti-base abutments, 24 zirconia Ti-base abutments and 3 resin abutment replicas as a control group. The 48 copings were cemented with three definitive and one provisional cement on both grit-blasted and non-blasted Ti-bases. The color of each restoration and surrounding artificial gingiva was measured spectrophotometrically at predefined measuring points and the CIELAB (ΔE_ab) color scale values were recorded. RESULTS. The color outcome of ceramic crowns bonded to hybrid abutments and soft tissues was affected differently by cements of different brands. Grit-blasting of Ti-bases prior to cementing CAD-CAM copings affected the color results of allceramic crowns. There was a significant difference (P = .038) for the median ΔE value between blasted and non-blasted reconstructions at the cervical aspect of the crown. Full-ceramic crowns on zirconia Ti-base abutments exhibited significantly lower ΔE values below the threshold of visibility (ΔE 1.8). In all subcategories tested, the use of a highly opaque temporary cement demonstrated the lowest median ΔE for both the crown and the artificial gingiva. CONCLUSION. Various cements, core ceramic materials and airborne particle abrasion prior to bonding can adversely affect the color of Ti-base supported ceramic crowns and peri-implant soft tissue. However, zirconia CAD-CAM copings and an opaque cement can effectively mask this darkening.

• Journal of Korean Neurosurgical Society
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• v.30 no.5
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• pp.611-621
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• 2001

Objective : We analyzed the clinical and endocrinological results of the transsphenoidal microsurgery for ACTH secreting pituitary adenomas. Marerials and Methods : From October 1995 to August 2000, 18 patients underwent transsphenoidal microsurgery for Cushing's disease. We analyzed the surgical results of 17 patients, one patient who was previously operated from other hospital was excluded. Age of the patients were 18 to 61 years old(mean 37.7), male to female ratio was 1 : 3.3, and follow-up period was 3 to 50 months(mean 20.3). The selection of candidates for transsphenoidal exploration was based on endocrinologic criteria. Magnetic resonance imaging was the preferred radiologic test. Selective inferior petrosal sinus sampling of adrenocorticotropic hormone futher refined the diagnosis when endocrinologic and radiologic procedures were not definitive. Results : Results of the preoperative endocrinological test were : level of serum ACTH 29.4 to $225{\mu}g/dL$(mean $93.88{\mu}g/dL$) ; serum cortisol 11.9 to $47.5{\mu}g/dL$(mean $27.49{\mu}g/dL$) ; 24-hour urine free cortisol 235 to $1019{\mu}g/day$(mean $571.0{\mu}g/day$). Inferior petrosal sinus sampling for ACTH was performed in 11 patients and all were confirmed by Cushing's disease and we could predict the laterality of the tumor in 9 of 11 patients. We performed transsphenoidal selective adenomectomy in 5 patients, adenomectomy and subtotal hypophysectomy in 2 patients, adenomectomy and partial hypophysectomy in 9 patients, and in the remaining one patient, hemihypophysectomy followed by total hypophysectomy due to remission failure. Fifteen of 17 patients(88.2%) showed endocrinological remission. Glucocorticoid replacement therapy was performed in all the patients who showed remission for 1 to 24 months(mean 5.9 months), and 6 patients received steroid over 6 months. Conclusion : We conclude that the direct demonstration of a tumor in the pituitary gland by MRI is the most important and definitive diagnostic tool and the location of a mass should be confirmed with increased level of ACTH by the inferior petrosal sinus sampling. Transsphenoidal microsurgery is effective treatment modality for Cushing's disease and the immediate postoperative evaluation of the surgical resection of the tumor is very important. The patients should show hypocortisolism, decreased, subnormal serum ACTH and cortisol levels and 24-hours urine free cortisol. We performed 18 transsphenoidal microsurgery for Cushing's disease in 17 patients and 15 patients(88.2%) showed endocrinological remission.

• Radiation Oncology Journal
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• v.25 no.4
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• pp.193-200
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• 2007

Purpose: This study evaluated the pretreatment expression patterns of MDM2, p53, and pRb proteins to determine if the expression patterns could predict the outcome of concurrent chemoradiotherapy (CCRT) for esophageal squamous cell carcinoma and aid in the decisions for the selection of treatment modalities. Materials and Methods: Fifty-one patients that were treated with definitive chemoradiotherapy for stage $I{\sim}IVa$ esophageal squamous cell carcinoma were selected for this study. Radiotherapy was administered with daily $1.8{\sim}2\;Gy$ fractions up to a median dose of 54 Gy for primary tumors, and with four cycles of cisplatin/5-fluorouracil chemotherapy that was administered every 4 weeks, the first two cycles of which were administered concurrently with radiotherapy. Expression of MDM2, p53, and pRb was investigated by immunohistochemical analysis using pretreatment biopsy specimens. Results: MDM2, p53, and pRb were detected with high immunoreactivity in 19.6%, 27.5%, and 66.7% of the patients, respectively. However, there was no significant correlation between expression of these factors and clinical outcome. By the use of multivariate analysis with nine covariates-age, tumor location, tumor length, stage, pathological response, clinical response, MDM2 expression, p53 expression, and pRb expression, only pathological response and stage were significant factors for cause-specific survival. Conclusion: Expression of MDM2, p53, and pRb was not found to be clinically significant for predicting outcomes after CCRT in this study. Further studies with a larger patient population and longer follow-up periods are needed to re-evaluate the expression pattern and to identify new predictors for CCRT response.