1.Clinical Features of Breast Cancer in South Korean Patients with Germline TP53 Gene Mutations
Hassan ALYAMI ; Tae-Kyung YOO ; Jong-Ho CHEUN ; Han-Byoel LEE ; Sung Mi JUNG ; Jai Min RYU ; Soong June BAE ; Joon JEONG ; Chang Ik YOON ; Juneyoung AHN ; Pill Sun PAIK ; Min Kyung CHO ; Woo-Chan PARK
Journal of Breast Cancer 2021;24(2):175-182
Purpose:
Li-Fraumeni syndrome (LFS) is a rare autosomal cancer syndrome caused by a germline mutation in the TP53 gene. Breast cancer in LFS patients is of various subtypes;however, limited data are available on the clinicopathological features of these subtypes and their appropriate treatments. This study aimed to review the clinical features and treatments for breast cancer in South Korean patients with germline TP53 mutations.
Methods:
Data on the clinicopathological features and treatment of all breast cancer patients with LFS were collected retrospectively from the available database of 4 tertiary hospitals in the Republic of Korea.
Results:
Twenty-one breast cancer cases in 12 unrelated women with confirmed germline TP53 mutations were included in the study. The median age at diagnosis was 33.5 years. The histopathological diagnosis included invasive ductal carcinoma (n = 16), ductal carcinoma in situ (n = 3), and malignant phyllodes tumor (n = 2). While 42% and 31% of the cases were positive for estrogen and progesterone receptors, respectively, 52.6% were human epidermal growth factor receptor 2 (HER2) positive, and 21% were triple-negative. The treatments included mastectomy (52%) and breast-conserving surgery (38%). Five patients underwent radiotherapy (RT). The median follow-up period was 87.5 (8–222) months. There were 3 ipsilateral and 4 contralateral breast recurrences during the follow-up, and 8 patients developed new primary cancers. In the post-RT subgroup, there were 2 ipsilateral and 2 contralateral breast recurrences in 1 patient, and 4 patients had a new primary cancer.
Conclusion
As reported in other countries, breast cancer in LFS patients in South Korea had an early onset and were predominantly but not exclusively positive for HER2.A multidisciplinary approach with adherence to the treatment guidelines, considering mastectomy, and avoiding RT is encouraged to prevent RT-associated sequelae in LFS patients.
2.Clinical Features of Breast Cancer in South Korean Patients with Germline TP53 Gene Mutations
Hassan ALYAMI ; Tae-Kyung YOO ; Jong-Ho CHEUN ; Han-Byoel LEE ; Sung Mi JUNG ; Jai Min RYU ; Soong June BAE ; Joon JEONG ; Chang Ik YOON ; Juneyoung AHN ; Pill Sun PAIK ; Min Kyung CHO ; Woo-Chan PARK
Journal of Breast Cancer 2021;24(2):175-182
Purpose:
Li-Fraumeni syndrome (LFS) is a rare autosomal cancer syndrome caused by a germline mutation in the TP53 gene. Breast cancer in LFS patients is of various subtypes;however, limited data are available on the clinicopathological features of these subtypes and their appropriate treatments. This study aimed to review the clinical features and treatments for breast cancer in South Korean patients with germline TP53 mutations.
Methods:
Data on the clinicopathological features and treatment of all breast cancer patients with LFS were collected retrospectively from the available database of 4 tertiary hospitals in the Republic of Korea.
Results:
Twenty-one breast cancer cases in 12 unrelated women with confirmed germline TP53 mutations were included in the study. The median age at diagnosis was 33.5 years. The histopathological diagnosis included invasive ductal carcinoma (n = 16), ductal carcinoma in situ (n = 3), and malignant phyllodes tumor (n = 2). While 42% and 31% of the cases were positive for estrogen and progesterone receptors, respectively, 52.6% were human epidermal growth factor receptor 2 (HER2) positive, and 21% were triple-negative. The treatments included mastectomy (52%) and breast-conserving surgery (38%). Five patients underwent radiotherapy (RT). The median follow-up period was 87.5 (8–222) months. There were 3 ipsilateral and 4 contralateral breast recurrences during the follow-up, and 8 patients developed new primary cancers. In the post-RT subgroup, there were 2 ipsilateral and 2 contralateral breast recurrences in 1 patient, and 4 patients had a new primary cancer.
Conclusion
As reported in other countries, breast cancer in LFS patients in South Korea had an early onset and were predominantly but not exclusively positive for HER2.A multidisciplinary approach with adherence to the treatment guidelines, considering mastectomy, and avoiding RT is encouraged to prevent RT-associated sequelae in LFS patients.
3.An Easy Method of Masseter muscle sling for lower lip paralysis in senile Hansen Patients
Sung Yul AHN ; Jong-Pill KIM ; Hyang Joon PARK
Korean Leprosy Bulletin 2020;53(1):15-21
Background:
One of the Sequelae of facial nerve paralysis in leprosy patients is severe eye and lip deformity. Eye deformities are most common, followed by unilateral lip deviation. When there is bilateral failal nerve paralysis, the lower lip drooping may occur. The lower lip drooping makes mouth closure incomplete, so the fluid or saliva may flow out when eating. And the teeth may be exposed or even the gum may be visible, which causes serious cosmetic and functional impairments.
Objective:
It is to evaluate the masseter muscle sling (MMS) method in the treatment of the recurrent lower lip drooping in patients with Hansen's disease.
Methods:
In the case of masseter muscle sling, there is a traditional method of detaching, separating and raising anterior masseter muscle in half from the mandibular jaw line and fixing it to the lip muscle. However, the authors observed a 3cm gap between the anterior border of masseter muscle and the lateral edge of orbicularis oris muscle. Under the local anesthesia the nasolabial line was incised and 3-4 places were pulled up between both musle edges and plicated with a 2/0 PDS??threads the gap-space.
Results:
During 5 years (2016-2020), there were 17 recurrent lower lip palsy patients. The mean age was 71.3 years and the ratio of male and female was 9:8. Of 17 patents, 12 received temporal muscle transfer (TMT) attached with tensor fascia lata (TFL), 2 received temporal muscle transfer attached with Alloderm, and 3 received masseter muscle sling alone. The postoperative results of corrective masseter muscle sling were satisfactory in all 17 patients and there were no serious complication and recurrence during follow-up.
Conclusion
Masseter muscle sling is effective method in correction of recurrent lower lip drooping in patient with leprosy who treated previously with temporal muscle transfer attached with tensor fascia lata or temporal muscle transfer attached with Alloderm. In addition, masseter muscle sling may be considered as the first-line treatment in the lower lip paralysis.
4.Quick Sequential Organ Failure Assessment (qSOFA) to predict clinical outcome in tsutsugamushi disease patients in emergency department
Jong Min PARK ; Dong Wook LEE ; Hyung Jun MOON ; Hyun Jung LEE ; Do Eui KIM ; Dong Kil JUNG ; Sung Pill JO ; Hyun Joon KIM ; Jung Won LEE ; Jae Hyung CHOI
Journal of the Korean Society of Emergency Medicine 2020;31(1):99-104
Objective:
Tsutsugamushi disease is a febrile illness caused by tick bites. Delay in making the diagnosis and treatment cause an increase of the frequency of complications and mortality. The aim of this study was to determine quick sequential organ failure assessment (qSOFA) to predict the clinical outcome of scrub typhus patients in emergency departments.
Methods:
This was a retrospective, observational study of patients with tsutsugamushi disease and who presented to the emergency department of an urban hospital and a rural tertiary hospital between January 2013 and December 2018. The demographic and laboratory data was collected through a chart review. Statistical analysis was performed by dividing the patients into the general ward admission group (general ward) and the intensive care unit admission group (ICU).
Results:
Age, Acute Physiology and Chronic Health Evaluation II (APACHE) II score and laboratory tests such as pH, leukocyte count, C-reactive protein, and procalcitonin also showed significant differences between the general ward and ICU groups on the univariable logistic regression analysis, but only the qSOFA score among the variables showed a significant difference on the multivariate logistic regression analysis (P=0.014).
Conclusion
The qSOFA score will be a prompt and useful tool for predicting the prognosis of patients with tsutsugamushi disease in the emergency department.
5.An Easy Method of Masseter muscle sling for lower lip paralysis in senile Hansen Patients
Sung Yul AHN ; Jong-Pill KIM ; Hyang Joon PARK
Korean Leprosy Bulletin 2020;53(1):15-21
Background:
One of the Sequelae of facial nerve paralysis in leprosy patients is severe eye and lip deformity. Eye deformities are most common, followed by unilateral lip deviation. When there is bilateral failal nerve paralysis, the lower lip drooping may occur. The lower lip drooping makes mouth closure incomplete, so the fluid or saliva may flow out when eating. And the teeth may be exposed or even the gum may be visible, which causes serious cosmetic and functional impairments.
Objective:
It is to evaluate the masseter muscle sling (MMS) method in the treatment of the recurrent lower lip drooping in patients with Hansen's disease.
Methods:
In the case of masseter muscle sling, there is a traditional method of detaching, separating and raising anterior masseter muscle in half from the mandibular jaw line and fixing it to the lip muscle. However, the authors observed a 3cm gap between the anterior border of masseter muscle and the lateral edge of orbicularis oris muscle. Under the local anesthesia the nasolabial line was incised and 3-4 places were pulled up between both musle edges and plicated with a 2/0 PDS??threads the gap-space.
Results:
During 5 years (2016-2020), there were 17 recurrent lower lip palsy patients. The mean age was 71.3 years and the ratio of male and female was 9:8. Of 17 patents, 12 received temporal muscle transfer (TMT) attached with tensor fascia lata (TFL), 2 received temporal muscle transfer attached with Alloderm, and 3 received masseter muscle sling alone. The postoperative results of corrective masseter muscle sling were satisfactory in all 17 patients and there were no serious complication and recurrence during follow-up.
Conclusion
Masseter muscle sling is effective method in correction of recurrent lower lip drooping in patient with leprosy who treated previously with temporal muscle transfer attached with tensor fascia lata or temporal muscle transfer attached with Alloderm. In addition, masseter muscle sling may be considered as the first-line treatment in the lower lip paralysis.
6.Outcome and status of postcardiac arrest care in Korea: results from the Korean Hypothermia Network prospective registry
Soo Hyun KIM ; Kyu Nam PARK ; Chun Song YOUN ; Minjung Kathy CHAE ; Won Young KIM ; Byung Kook LEE ; Dong Hoon LEE ; Tae Chang JANG ; Jae Hoon LEE ; Yoon Hee CHOI ; Je Sung YOU ; In Soo CHO ; Su Jin KIM ; Jong-Seok LEE ; Yong Hwan KIM ; Min Seob SIM ; Jonghwan SHIN ; Yoo Seok PARK ; Young Hwan LEE ; HyungJun MOON ; Won Jung JEONG ; Joo Suk OH ; Seung Pill CHOI ; Kyoung-Chul CHA ;
Clinical and Experimental Emergency Medicine 2020;7(4):250-258
Objective:
High-quality intensive care, including targeted temperature management (TTM) for patients with postcardiac arrest syndrome, is a key element for improving outcomes after out-of-hospital cardiac arrest (OHCA). We aimed to assess the status of postcardiac arrest syndrome care, including TTM and 6-month survival with neurologically favorable outcomes, after adult OHCA patients were treated with TTM, using data from the Korean Hypothermia Network prospective registry.
Methods:
We used the Korean Hypothermia Network prospective registry, a web-based multicenter registry that includes data from 22 participating hospitals throughout the Republic of Korea. Adult comatose OHCA survivors treated with TTM between October 2015 and December 2018 were included. The primary outcome was neurological outcome at 6 months.
Results:
Of the 1,354 registered OHCA survivors treated with TTM, 550 (40.6%) survived 6 months, and 413 (30.5%) had good neurological outcomes. We identified 839 (62.0%) patients with preClinsumed cardiac etiology. A total of 937 (69.2%) collapses were witnessed, shockable rhythms were demonstrated in 482 (35.6%) patients, and 421 (31.1%) patients arrived at the emergency department with prehospital return of spontaneous circulation. The most common target temperature was 33°C, and the most common target duration was 24 hours.
Conclusion
The survival and good neurologic outcome rates of this prospective registry show great improvements compared with those of an earlier registry. While the optimal target temperature and duration are still unknown, the most common target temperature was 33°C, and the most common target duration was 24 hours.
7.Usefulness of the neutrophil-to-lmphocyte ratio as a prognostic predictor in elderly patients admitted to the intensive care unit through emergency department
Sang Kyu KIM ; Jeoung Ho PARK ; Sanghee OH ; Sang Hyun PARK ; Han Joon KIM ; Soo Hyun KIM ; Sung Wook KIM ; Jongho ZHU ; Seung Pill CHOI ; Jae Hun OH
Journal of the Korean Society of Emergency Medicine 2019;30(6):555-562
OBJECTIVE:
The purpose of this study was to test the hypothesis that an increase in the neutrophil-to-lymphocyte ratio (NLR) increases the risk of in-hospital density in patients aged 65 and older, who are hospitalized in intensive care through the emergency rooms.
METHODS:
A retrospective medical record study was conducted on elderly patients who were admitted to intensive care units via the emergency room. The exclusion criteria were data loss, intensive care unit reentry, cerebrovascular accidents, hematologic disease, and trauma cases.
RESULTS:
The study included 526 patients; the mean age was 79, and 261 (49.6%) were male. The in-hospital mortality was 18.4% (97 patients). The initial NLR was higher in the non-survivor group than the survivor group, but the difference was not statistically significant (9.82±11.02 vs. 11.48±6.11, P=0.080). In multivariate logistic analysis, the initial NLR had no statistical significance, and the odd ratio was increased from one day later. Comparing the receiver operating characteristic curve of the NLR and Acute Physiology and Chronic Health Evaluation II (APACHE II) and Simplified Acute Physiology Score II (SAPS II) scores, the NLR showed an increase in the area of under curve (AUC) value over time as well as the highest AUC with the SAPS II scores.
CONCLUSION
In elderly adults, early NLR was found to have weak power to predict in-hospital mortality. Over time, the NLR values more than two days after intensive care unit admission may be useful in predicting the in-hospital mortality for older patients. This may be due to the delay in the immune response and the complex medical history.
8.Easy method for Upper lid blepharoplasty in elderly Hansen affected persons
Sung Yul AHN ; Hyang Joon PARK ; Jong Pill KIM
Korean Leprosy Bulletin 2019;52(1):23-27
BACKGROUND:
The average age of Korean Hansen affected persons who have become less than 10,000 is 76 years old.The upper eyelid lesions of these patients coexist with senile lesions and paralytic lesions due to facial paralysis.This senile change includes skin laxity, eyebrow drooping, eyelid drooping, medial and lateral canthal relaxation and paralytic changes include wrinkle disappearance in the forehead, eyebrows asymmetry, eyelash drooping in the upper eyelid, and simultaneously symptoms of lower eyelid such as ectropion and lapophthalmos.
OBJECTIVE:
Authors want to present a prescribed method of treatment that makes it easier to fix changes in senile and paralytic eyelids in elderly Hanson affected persons.
METHODS:
For senile blepharochalasis, if the distance between eye blow and eyelash (brow-lash distance) is to the 2.5 cm or more the sub-brow resection should be done.However, if brow-lash distance is less than 2.5 cm, the supra-brow resection should be done. For paralytic cases if there is a difference in height on the eyebrow. the skin is removed to create the same level as the eyebrow height on the healthy side.if both eyes are paralyzed the supra blow skin excision are done with prudence not to make the lagophthalmos.RESULT: In recent 2 years of 2018–2019we treated 32 cases of blepharochalasis, Blephnaroptosis, paralylic eyebrow droping and lash-laden eyelid margin excision for trichiasis correction. Satisfactory results were obtained in all patients who underwent surgery.
CONCLUSION
The authors report our experience of treating various upper eyelid lesions in the past 2 years in 32 cases of Hansen affected persons a history of Hansen's disease, together with literature review.
9.Two cases of calcinosis cutis treated with surgical excision.
Hyang Joon PARK ; Jong Pill KIM ; Sung Yul AHN
Korean Leprosy Bulletin 2018;51(1):29-34
Calcinosis cutis is an uncommon disorder and defined as the deposit of insoluble calcium salts in the skin. It is classified as dystrophic, metastatic, idiopathic, and iatrogenic according to etiology. The dystrophic type is the most common and occurs in the previously damaged tissue, including connective tissue diseases, panniculitis, inherited disorders, benign and malignant tumors, a variety of scarring caused by burn, radiation, trauma, surgery, and keloid. Treatment options are limited. Whereas medical therapy is usually not very effective, surgical intervention has shown to be beneficial and is indicated when painful masses, recurrent infection, ulcerations, functional impairment, and cosmetic concerns exist. Herein, we report two cases of dystrophic calcinosis cutis associated with longstanding, painful, ulcerated cutaneous lesions in the extremities successfully treated with surgical excision.
Burns
;
Calcinosis*
;
Calcium
;
Cicatrix
;
Connective Tissue Diseases
;
Extremities
;
Keloid
;
Panniculitis
;
Salts
;
Skin
;
Ulcer
10.Aesthetic Surgical Treatment of the Peri-orbital area in Hansen's Patients
Sung Yul AHN ; Hyang Joon PARK ; Jong Pill KIM
Korean Leprosy Bulletin 2017;50(1):3-14
BACKGROUND: Upper eyelid blepharoplasty has become one of the commonly practiced aesthetic operation. This upper eyelid blepharoplasty primarily conducted in aged person, while also addressing aesthetic concerns, include methods to rejuvenate the peri-orbital area and provide unobstructed eyesight. OBJECTIVE: Methods to affect rejuvenation of the peri-orbital area are variable and depend heavily on the patients. This makes evaluation of the patient a critically important component of the treatment process. Upper eyelid blepharoplasty requires treatment of the anterior and posterior lamella structures. Anterior lamella undergone a resection of the skin and orbicularis muscle to reduce the sagging appearance on the anterior surface of the upper eyelid. The retractor structures of the posterior lamella, especially the levator aponeurosis and the Müller's muscle, are advanced, tucked and plicated to the tarsus to add tension as needed to correct the blepharoptosis. This procedure, while also addressing aesthetic concerns, primarily adds tension to reduce or eliminate eyesight obstruction. Methods & Conclusion: These operation methods include : The plication procedures of posterior membranous septum and turn-over orbital septum to 2mm below the tarsus and traditional levartor complex tucking including levator aponeurosis and Müller muscle added. In addition to blepharoplasty and blepharoptosis in aged persons the brow-ptosis may also experience. This drooping of the eyebrow also contribute to eyesight obstruction and should be corrected. In general, treatment consists of a subbrow resection when the brow-lash distance is 25mm or more. When the brow-lash distance is less that 25mm, a supra-brow excision is required to prevent an angry or harsh appearance. However, when using the supra-brow method the possibility of an unwanted visible scar is more likely, a short incision direct brow-lift could be another alternative. But short incision brow-lift procedure can only use under the normal forehead muscle function.
Ankle
;
Blepharoplasty
;
Blepharoptosis
;
Cicatrix
;
Eyebrows
;
Eyelids
;
Forehead
;
Humans
;
Methods
;
Orbit
;
Rejuvenation
;
Skin

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