• Title/Summary/Keyword: Skin abnormalities

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Giant Neurofibroma on Both Buttocks (양측 엉덩이의 거대 신경섬유종)

  • Kim, Ji Hoon;Burm, Jin Sik;Kim, Yang Woo;Kang, So Ra;Kim, Hyoung Kyoung
    • Archives of Plastic Surgery
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    • v.36 no.4
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    • pp.512-515
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    • 2009
  • Purpose: Neurofibromatosis(NF) is an autosomal - dominant systemic disease. Up to fifty percent of patients with NF are reported to have concomitant vascular abnormalities. In the resection of a larger NF, the risk of uncontrolled hemorrhage is much higher due to the difficulty of hemostasis of large vessels within the tumor. We ligated the base of the giant NF with a simple loop - shaped ligation before removal of the giant NF in both buttocks. And then we could successfully reduce the amount of hemorrhage during the operation. Methods: A 46 - year - old female patient presented for giant masses of both gluteal area, which has been growing slowly for the last ten years. Each mass was about $30{\times}20cm$ in size. After designing the elliptical resection margin, we tightened the tumor base by using continuous loop - shaped suture ligation(weaving the thread up and down in a loop - shaped pattern, leaving a space of 2 cm between each loop) with a straight needle and prolene 2 - 0. After skin incision, we proceeded the dissection toward the central and inferior side of the mass obliquely while we avoided breaking large vascular sinuses. We resected the tumor in a wedged - shape. Subcutaneous tissue was sutured layer by layer and skin was closed by vertical mattress and interrupted suture. The loop - shaped ligation of the base was removed and compressive dressing was done with gauzes and elastic bandages. Results: Postoperative complications such as infection, hemorrhage, hematoma, and dehiscense did not occur. Perioperatively the patient was sufficiently transfused with five units of blood and two units of fresh frozen plasma. During the subsequent 1 year follow - up, the functional and cosmetic result was excellent. Conclusion: A continuous loop - shaped suture ligation procedure along the base of the giant NF effectively reduced the amount of hemorrhage during the operation, made dissection and ligation of vessels easily and quickly, and shorten the operating time and postoperative recovery time.

Outcome of pregnant mothers with systemic lupus erythematosus (focusing on congenital heart block) (전신성 홍반성 루푸스 산모의 출산아의 임상적 고찰 (완전 방실 블록을 중심으로))

  • Baek, Hey Sung;Choi, Jae Hyung;Kim, Nam Su;Kim, Chang Ryul;Moon, Su Ji
    • Clinical and Experimental Pediatrics
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    • v.49 no.4
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    • pp.381-387
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    • 2006
  • Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

Loss of EMP2 Inhibits Melanogenesis of MNT1 Melanoma Cells via Regulation of TRP-2

  • Enkhtaivan, Enkhmend;Kim, Hyun Ji;Kim, Boram;Byun, Hyung Jung;Yu, Lu;Nguyen, Tuan Minh;Nguyen, Thi Ha;Do, Phuong Anh;Kim, Eun Ji;Kim, Kyung Sung;Huy, Hieu Phung;Rahman, Mostafizur;Jang, Ji Yun;Rho, Seung Bae;Lee, Ho;Kang, Gyeoung Jin;Park, Mi Kyung;Kim, Nan-Hyung;Choi, Chang Ick;Lee, Kyeong;Han, Hyo Kyung;Cho, Jungsook;Lee, Ai Young;Lee, Chang Hoon
    • Biomolecules & Therapeutics
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    • v.30 no.2
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    • pp.203-211
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    • 2022
  • Melanogenesis is the production of melanin from tyrosine by a series of enzyme-catalyzed reactions, in which tyrosinase and DOPA oxidase play key roles. The melanin content in the skin determines skin pigmentation. Abnormalities in skin pigmentation lead to various skin pigmentation disorders. Recent research has shown that the expression of EMP2 is much lower in melanoma than in normal melanocytes, but its role in melanogenesis has not yet been elucidated. Therefore, we investigated the role of EMP2 in the melanogenesis of MNT1 human melanoma cells. We examined TRP-1, TRP-2, and TYR expression levels during melanogenesis in MNT1 melanoma cells by gene silencing of EMP2. Western blot and RT-PCR results confirmed that the expression levels of TYR and TRP-2 were decreased when EMP2 expression was knocked down by EMP2 siRNA in MNT1 cells, and these changes were reversed when EMP2 was overexpressed. We verified the EMP2 gene was knocked out of the cell line (EMP2 CRISPR/Cas9) by using a CRISPR/Cas9 system and found that the expression levels of TRP-2 and TYR were significantly lower in the EMP2 CRISPR/Cas9 cell lines. Loss of EMP2 also reduced migration and invasion of MNT1 melanoma cells. In addition, the melanosome transfer from the melanocytes to keratinocytes in the EMP2 KO cells cocultured with keratinocytes was reduced compared to the cells in the control coculture group. In conclusion, these results suggest that EMP2 is involved in melanogenesis via the regulation of TRP-2 expression.

Scapular Free Flap (유리 견갑 피판 이식술)

  • Chung, Duke-Whan;Han, Chung-Soo;Yim, Chang-Moo
    • Archives of Reconstructive Microsurgery
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    • v.5 no.1
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    • pp.24-34
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    • 1996
  • There are many kinds of free flaps for management of extensive soft tissue defect of extremities in orthopaedic field. Free vascularized scapular flap is one of the most useful and relatively easy to application. This flap has been utilize clinically from early eighties by many microsurgical pioneers. Authors performed 102 cases of this flap from 1984 to 1995. We have to consider about the surgical anatomy of the flap, technique of the donor harvesting procedures, vascular varieties and anatomical abnormalities and success rate and the weak points of the procedure. This flap nourished by cutaneous branches from circumflex scapular vessels emerges from the lateral aspect of the subscapular artery 2.5-5cm from its lateral origin passing through the triangular space(bounded by subscapularis, teres minor, teres major, long head of triceps). The terminal cutaneous branch runs posteriorly around the lateral border of the scapular and divided into two major branches, those transeverse horizontally and obliquely to the fascial plane of overlying skin of the scapular body. We can utilize these arteries for scapular and parascapular flap. The vascular pedicle ranged from 5 to 10 cm long depends on the dissection, usually two venae comitantes accompanied circumflex scapular artery and its major branches. The diameter of the circumflex scapular artery is more than 1mm in adult, rare vascular variation. Surgical techniques : The scapular flap can be dissected conveniently with prone or lateral decubitus position, prone position is more easier in my experience. There are two kinds of surgical approaches, most of the surgeon prefer elevation of the flap from its outer border towards its base which known easier and quicker, but I prefer elevation of the flap from its outer border because of the lowering the possibilities of damage to vasculature in the flap itself which runs just underneath the subcutaneous tissue of the flap and provide more quicker elevation of the flap with blunt finger dissection after secure pedicle dissection and confirmed the course from the base of the pedicle. There are minimal donor site morbidity with direct skin closure if the flap size is not so larger than 10cm width. This flap has versatility in the design of the flap shape and size, if we need more longer and larger one, we can use parascapular flap or both. Even more, the flap can be used with latissimus dorsi musculocutaneous flap and serratus anterior flap which have common vascular pedicle from subscapular artery, some instance can combined with osteocutaneous flap if we include the lateral border of the scapular bone or parts of the ribs with serratus anterior. The most important shortcoming of the scapular free flap is non sensating, there are no reasonable sensory nerves to the flap to anastomose with recipient site nerve. Results : Among our 102 cases, overall success rate was 89%, most of the causes of the failure was recipient site vascular problems such as damaged recipient arterial conditions, and there were two cases of vascular anomalies in our series. Patients ages from 3 years old to 62 years old. Six cases of combined flap with latissimus dorsi, 4 cases of osteocutaneous flap for bone reconstruction, 62 parascapular flap was performed - we prefer parascapular flap to scapular. Statistical analysis of the size of the flap has less meaningful because of the flap has great versatility in size. In the length of the pedicle depends on the recipient site condition, we can adjust the pedicle length. The longest vascular pedicle was 14 cm in length from the axillary artery to the enter point cutaneous tissue. In conclusion, scapular free flap is one of the most useful modalities to manage the large intractable soft tissue defect. It has almost constant vascular pedicle with rare anatomical variation, easy to dissect great versatility in size and shape, low donor morbidity, thin and hairless skin.

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A Case of the Shoulder-Hand Syndrome Caused by a Crush Injury of the Shoulder (견관절부 외상후 발생된 Shoulder-Hand Syndrome)

  • Jeon, Jae-Soo;Lee, Sung-Keun;Song, Hoo-Bin;Kim, Sun-Jong;Park, Wook;Kim, Sung-Yell
    • The Korean Journal of Pain
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    • v.2 no.2
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    • pp.155-166
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    • 1989
  • Bonica defined, that reflex sympathetic dystrophy (RSD) may develop pain, vasomotor abnoramalities, delayed functional recovery, and dystrophic changes on an affected area without major neurologic injury following trauma, surgery or one of several diseased states. This 45 year old male patient had been crushed on his left shoulder by a heavily laden rear car, during his job street cleaning about 10 years ago (1978). At first the pain was localizea only to the site of injury, but with time, it spreaded from the shoulder to the elbow and hand, with swelling. X-ray studies in the local clinic, showed no bone abnormalities of the affected site. During about 10 years following the injury, the had recieved several types of treatments such as nonsteroidal analgesics, steroid injections into the glenoidal cavity (10 times), physical therapy, some oriental herb medicines, and acupuncture over a period of 1~3 months annually. His shoulder pain and it's joint dysfunction persisted with recurrent paroxysmal aggrevation because of being mismanaged or neglected for a sufficiently long period these fore permiting progression of the sympathetic imbalance. On July 14 1988 when he visited our clinic. He complained of burning, aching and had a hyperpathic response or hyperesthesia in touch from the shoulder girdle to the elbow and the hand. Also the skin of the affected area was pale, cold, and there was much sweating of the axilla and palm, but no edema. The shoulder girdle was unable to move due to joint pain with marked weakness. We confirmed skin temperatures $5^{\circ}C$ lower than those of the unaffected axilla, elbow and palm of his hand, and his nails were slightly ridged with lateral arching and some were brittle. On X-ray findings of both the shoulder AP & lateral view, the left humerus and joint area showed diffuse post-traumatic osteoporosis and fibrous ankylozing with an osteoarthritis-like appearance. For evaluating the RSD and it's relief of pain, the left cervical sympathetic ganglion was blocked by injecting 0.5% bupivacaine 5 ml with normal saline 5 ml (=SGB). After 15 minutes following the SGB, the clinical efficacy of the block by the patients subjective score of pain intensity (=PSSPI), showed a 50% reduction of his shoulder and arm pain, which was burning in quality, and a hyperpathic response against palpation by the examiner. The skin temperatures of the axilla and palm rose to $4{\sim}5^{\circ}C$ more than those before the SGB. He felt that his left face and upper extremity became warmer than before the SGB, and that he had reduced sweating on his axilla and his palm. Horner's sign was also observed on his face and eyes. But his deep shoulder joint pain was not improved. For the control of the remaining shoulder joint pain, after 45 minutes following the SGB, a somatic sensory block was performed by injecting 0.5% bupivacaine 6 ml mixed with salmon calcitonin, $Tridol^{(R)}$, $Polydyn^{(R)}$ and triamcinolone into the fossa of the acromioclavicular joint region. The clinical effect of the somatic block showed an 80% releif of the deep joint pain by the PSSPI of the joint motion. Both blocks, as the above mentioned, were repeated a total of 28 times respectively, during 6 months, except the steroid was used just 3 times from the start. For maintaining the relieved pain level whilst using both blocks, we prescribed a low dose of clonazepam, prazocin, $Etravil^{(R)}$, codeine, etodolac micronized and antacids over 6 months. The result of the treatments were as follows; 1) The burning, aching and hyperpathic condition which accompanied with vaosmotor and pseudomotor dysfunction, disappeared gradually to almost nothing, within 3 weeks from the starting of the blocks every other day. 2) The joint disability of the affected area was improved little by little within 6 months. 3) The post-traumatic osteoporosis, fibrous ankylosis and marginal sclerosis with a narrowed joint, showed not much improvement on the X-ray findings (on April 25, 1989) 10 months later in the follow-up. 4) Now he has returned to his job as a street cleaner.

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Stevens-Johnson Syndrome : A Case Report (스티븐 존슨 증후군 : 증례보고)

  • Song, Yongho;Lee, Nanyoung;Lee, Sangho;Jih, Myeongkwan;Lim, Yujin;Yoon, Youngmi
    • Journal of the korean academy of Pediatric Dentistry
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    • v.44 no.4
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    • pp.455-460
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    • 2017
  • Stevens-Johnson syndrome (SJS), an extremely severe acute hypersensitivity reaction, causes extensive necrosis on the skin and the mucous membrane. SJS is a disease of unknown cause that can occur in all age groups. It is thought to be caused by drug allergy or induced by bacterial infection. Epidermal surface invasion of less than 10 percent is called SJS, and invasion of more than 30 percent is called toxic epidermal necrolysis. Although it is rare with an incidence of 1 - 2 cases per million people per year, it has effects on tooth development and therefore on children who are in a growth phase. The purpose of this case report is to examine the effect of SJS on tooth development in children. In general, eruption of the upper and lower 1st molars and lower central incisors starts at 6 - 7 years of age. Root development also occurs at this time. In the case reported here, SJS occurred in a 6-year-old patient. Although the patient's SJS was completely cured, he still suffers from aftereffects. Developmental abnormalities in the patient's teeth were observed only in teeth for which root development had been completed at the time. The purpose of this case report is to illustrate how to diagnose such systemic diseases by intra-oral features and to recognize and resolve tooth development problems associated with the disease.

Treatment of Multiple Pulmonary Arteriovenous Fistulas with Therapeutic Embolization in Osler-Rendu-Weber Syndrome (Osler-Rendu-Weber 증후군 환자에서 색전요법을 이용한 다발성 폐동정맥루 치험 1예)

  • Kim, Jae-Hag;Choi, Taek-Hee;Nam, Seung-Mo;Chang, Jae-Jin;Park, Yeon-Hee;Hur, Nam-Hyun;Choe, Du-Hwan;Lee, Byung-Hee;Kim, You-Cheoul;Lee, Choon-Taek
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.4
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    • pp.914-921
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    • 1997
  • Hereditary hemorrhagic telangiectasia(Osler-Rendu-Weber Syndrome) is characterized by telangiectasia of the skin and mucous membranes and intermittent bleeding from vascular abnormalities. About 20% of patients with this syndrome have pulmonary arteriovenous fistulas. Pulmonary arteriovenous fistula is uncommon malformation which has an abnormal connection between the pulmonary capillary bed, in which venous blood in the pulmonary artery is shunted through the fistula into the pulmonary vein without exposure to alveolar oxygen and result in unoxygenated, desaturated systemic arterial blood, polycythemia, cyanosis and clubbing. Death often results from cerebral abscess and rupture of the malformation with massive hemorrhage. Therapeutic intervention is recommended for all symptomatic patients because of the risk of those serious complications. Treatment options include surgery and transcatheter obliteration with steel coils or detachable balloons. Therapeutic embolization has the advantages that multiple bilateral pulmonary arteriovenous fistulas can be occluded and also that the procedure can be repeated if necessary. Recently we experienced a case of the multiple bilateral pulmonary arteriovenous fistulas associated with telangiectatic change of hepatic artery and multiple angiodysplasia on the gastric mucosa in 41 years old female patient who had mild dyspnea of exertion(NYHA class II). clubbing finger, severe iron deficiency anemia. She was treated with embolization technique using steel coils and iron replacement. After the therapeutic embolization. significant improvement of dyspnea of exertion with disappearance of multiple pulmonary nodule on follow-up simple chest x-ray was noted. During the subsequent six months follow-up period, she had the improvement of symptoms and iron deficiency anemia.

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The Clinical Characteristics and Pharmacologic Treatment of Neuralgic Pain in Maxillofacial Region (악안면 신경통성 동통의 임상적 특징과 약물 치료)

  • Sung-Chang Chung;Sung-Woo Lee;Young-Ku Kim
    • Journal of Oral Medicine and Pain
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    • v.21 no.1
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    • pp.173-182
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    • 1996
  • This study was performed to provide the information on the clinical characteristics of the most common paroxysmal pain disorder in maxillofacial region, trigeminal neuralgia, and the effects and side effects of carbamazepine. The patients who visited Orofacial Pain Clinic, Dept. of Oral Diagnosis, Seoul National University Dental Hospital for treating paroxysmal pain were studied by history taking, clinical examination, and radiography. Sixty-two patients(male 20, female42) without any clinical and radiological abnormalities were Included. The change of pain, blood tests, and side effects were investigated periodically after administration of carbamazepine. The obtained results were as follows : 1. Almost all patient with trigeminal neuralgia were over the age of forties and it was more common in women. 2. Trigeminal neuralgia was more right sided and the involved nerve was in the order of maxillary n., mandibular n., and ophthalmic n. 3. The mean duration of suffering was 20.7 months. Eighty percent of patients had apparent trigger area. 4. The duration of pain attack was in the older of several seconds, 1 min. to 5 min., more than 10 min., and 5 min. to 10 min. The frequency of pain attack was in the order of more than 10 per day, 6-10 per day, and 1-5 per day. 5. The clinic the patients had visited for reducing neuralgic pain was in the order of dental clinic, neurology, oriental medicine, otolaryngology, and pharmacy. 6. Unnecessary dental treatments for reducing neuralgic pain were performed in 41.9% of the patients. Almosit all treatments were irreversible ones such as endodontic treatment and tooth extraction. 7. The initial mean VAS was 8.6, but it was decreased to 3.8 after 1 month, to 2.7 after 2 months. Almost all patients showed decreased pain with 200-600mg/day of carbamazepine to 6 months. 8. WBC counts, especially neutrophil counts, was decreased in 1 week after administration of carbamazepine but reached initial level after 1 month. SGOT, SGPT, and creatinine did not show any significant change. 9. Blood pressure was not changed significantly after administration of carbamazepine. 10. Almost patients did not show any apparent side effects, but drowsiness, dizziness, skin itching, constipation, and gastric irritation were occurred in some patients.

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A Case of Pulmonary Sarcoidosis Combined with Neurosarcoidosis (신경유육종증이 병발한 폐유육종증 1예)

  • Park, Byung Hoon;Park, Seon Cheol;Shin, Sang Yun;Jeon, Han Ho;Jung, Kyung Soo;Chung, Woo Young;Byun, Min Kwang;Moon, Ji Ae;Kim, Young Sam;Kim, Se Kyu;Chang, Joon;Kim, Sung Kyu;Park, Moo Suk
    • Tuberculosis and Respiratory Diseases
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    • v.62 no.6
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    • pp.549-553
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    • 2007
  • Sarcoidosis is a multi-systemic syndrome of an unknown etiology, and it is characterized by the formation of multiple noncaseating granulomas that disrupt the architecture and function of the tissues in which they reside. The most commonly affected organs are lung, skin and lymph nodes. Overt clinical involvement of the nervous system is uncommon and this occurs in about 5% of all patients during the course of their disease. The most common manifestations are granulomatous leptomeningitis, cranial nerve palsy, electrolyte or other endocrinologic abnormalities, but isolated memory impairment is a rare manifestation. This is a case of 59 years-old male with recent memory impairment, and he was previously diagnosed with pulmonary sarcoidosis by transbronchial lung biopsy. The brain MRI imaging revealed the leptomeningeal and parenchymal involvement of sarcoidosis. He was treated with high dose corticosteroid and his memory function was improved to nearly a normal level. We report here on a case of successful treatment of pulmonary sarcoidosis combined with neurosarcoidosis with using high dose corticosteroid, and the patient presented with recent memory impairment.

A Study on the Health Status of Korean Vietnam Veterans' Children - A Result of Questionnaire Survey on Vietnam Veterans of Pusan Area Who Diagnosed as Cases by Korean Veteran's Hospital Diagnostic Criteria - (베트남전 참전자 2세의 건강상태에 관한 조사 - 부산지역 고엽제 위해증 환자를 대상으로 한 설문조사 결과 -)

  • Kim, Hak-Joon;Sohn, Hae-Sook;Urm, Sang-Hwa;Park, Soo-Kyung;Yu, Byung-Chul;Lee, Jong-Tae;Chun, Jin-Ho
    • Journal of Preventive Medicine and Public Health
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    • v.33 no.1
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    • pp.17-24
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    • 2000
  • Objectives : To propose the referential data to evaluate the health impacts of Vietnam veterans' children whose father were exposed to herbicides in Vietnam War. Methods : Vietnam veterans who visited to Pusan Veteran Hospital for medical care were recruited from April to October, 1998. They were 71 and asked about their own combat history, symptoms and illness, and health status of their 182 children. The informations were collected by direct and phone interview. Exposure estimation was also performed as exposure score depending on year and unit of participation, and personal episodes related to exposure to herbicide in the war. It classified into three groups; lower(<18.0), moderate(18-53), high$(\geq53)$ exposure group. Results : The mean age and the period into the combat of the veterans were 52.8 years and 15.0 months. The mean exposure score was $18.1{\pm}9.9$, and mainly distributed in lower (46.5%) and moderate(52.1%) exposure group. Most(90.1%) of them were diagnosed as sequelae(21 cases) and suspected sequelae(43 cases) of the herbicides by Korean veteran's hospital diagnostic criteria. The major sequelae was peripheral neuropathy 13 cases, chloracne 5 cases, and the major suspected sequelae was hypertension 20 cases, diabetes mellitus 18 cases, liver disease 12 cases, central neuropathy 11 cases, etc. About birth, 42.2% and 16.9% experienced spontaneous abortion and stillbirth, respectively. The mean exposure score was higher in stillbirth experience group(p<0.05). About half of the children(90 cases, 49.5%) hold the abnormal health status: those were skin pigmentation 38 cases, rash 23 cases, congenital anomaly 15 cases, general weakness 12 cases, purpura 8 cases, visual disturbance 8 cases, etc. These health problems had no association with father's exposure level(p>0.05). Conclusions : These results were depend on their own answers, and expectation for compensation did not excluded, therefore, this study may have limitations: inaccuracy of informations due to recall bias and response bias. Nevertheless, through this study, we could image the fundamental aspect for health impacts of Vietnam veterans' children for preparing the national control program and policy. A large scale epidemiologic study with valid exposure assessment on the health impacts of Vietnam veterans' children is recommneded.

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