The molecular pathophysiology of vascular anomalies: Genomic research

Vascular anomalies are congenital localized abnormalities that result from improper development and maintenance of the vasculature. The lesions of vascular anomalies vary in location, type, and clinical severity of the phenotype, and the current treatment options are often unsatisfactory. Most vascular anomalies are sporadic, but patterns of inheritance have been noted in some cases, making genetic analysis relevant. Developments in the field of genomics, including next-generation sequencing, have provided novel insights into the genetic and molecular pathophysiological mechanisms underlying vascular anomalies. These insights may pave the way for new approaches to molecular diagnosis and potential disease-specific therapies. This article provides an introduction to genetic testing for vascular anomalies and presents a brief summary of the etiology and genetics of vascular anomalies.

Correction of microtia with constriction features using a superficial temporal fascial flap combined with a rib cartilage graft

Background@#Microtia with constricted features is characterized by a short helical length of variable severity, upper antihelical or scaphal deficiency, and a downfolded upper ear. No consensus has been reached regarding the most appropriate surgical method for this condition. In this study, we aimed to introduce a simple and safe surgical method for the correction or reconstruction of upper helix ear deformities. @*Methods@#Between February 2011 and June 2014, eight patients with microtia with constricted upper helix ear deformity underwent reconstruction of the ear deformity. The upper ear helical framework was constructed by carving and curving the eighth rib cartilage harvested from the ipsilateral chest wall, covering this cartilage with a superficial temporal fascial flap, and adjusting the skin graft to align with the ear contour. To evaluate their satisfaction, patients were asked to complete a questionnaire regarding ear shape, symmetry, position, color, and overall outcome scored on a 5-point scale at 12 months postoperatively. @*Results@#None of the patients experienced severe complications in the reconstructed ear. The preoperative and postoperative vertical ear length ratios were 0.88 and 1.02, respectively. And the mean patient satisfaction scores for shape, symmetry, position, color, and overall outcome were 4.2, 4.5, 4.7, 4.4, and 4.6 out of 5 points, respectively. All patients expressed a high level of satisfaction at 12 months postoperatively. @*Conclusions@#Our technique provides a good alternative method for the reconstruction of moderate constricted upper helix ear deformities in patients who meet the surgical indications with satisfactory outcomes and few complications.

Use of Abnobaviscum to treat refractory seroma after breast reconstruction with a latissimus dorsi flap: A case report

In recent years, there has been a notable increase in the rate of refractory donor site seroma, defined as seroma that persists for at least 3 months postoperatively, as the number of breast reconstructions using a latissimus dorsi (LD) musculocutaneous flap has increased. Various factors have been proposed to be related, including smoking, obesity, flap mass, and body weight, and several studies have been conducted to explore treatment methods. Typically, surgical treatment, such as capsulectomy, has been considered for refractory seroma, but in this case report, we describe positive outcomes achieved by using Abnobaviscum to treat three female patients who developed a donor site seroma at least 3 months after breast reconstruction using an LD flap.

Feasibility of Transradial Coronary Angiography Using a Single Judkins Left Catheter

BACKGROUND AND OBJECTIVES: Transradial coronary angiography and intervention are getting more popularity throughout the world because of lower incidence of puncture site complications and the patients have displayed early ambulation. However, radial arterial spasms and possible endothelial injury due to the small size of radial artery are troublesome. To overcome this problem, we investigated the usefulness of performing transradial coronary angiography using a single Judkins left catheter (JL). SUBJECTS AND METHODS: A total of 268 patients who underwent right transradial coronary angiography from June 2003 to December 2003 were enrolled for this prospective study. The Judkins Left 3.5(JL3.5), the Multipurpose (MP) and Amplatz Left (AL2 for males, AL1 for females) were assigned to be used in patients with a randomized protocol. The technical/angiographic success rate, procedural/fluoroscopic time and the frequency of stiffwire usage were assessed. RESULTS: Technical success was accomplished in 87 (96.7%) of the patients with a single JL3.5, in 90 (97.8%) of the patients with the MP and in 83 (96.5%) of the patients with the AL catheter. Successful angiography success was achieved in 77 (85.6%) of the patients using the JL3.5, in 71 (77.2%) of the patients using the MP and in 66 patients (76.7%) using the AL. The technical success rate (p=0.270) and the angiographic success rate (p= 0.162) showed no significant difference among the three catheters. The procedural time was 8.1+/-4.0 min in the JL3.5 group, 8.1+/-3.7 min in the MP group and 7.3+/-3.4 min in the AL group, respectively (p=0.431). Also, the fluoroscopic time was not different among the three groups. CONCLUSION: The JL3.5 catheter is an excellent tool for both coronary imaging and it is equally successful as compared with the MP and AL catheters. Therefore, the JL3.5 may be considered as the initial catheter that can be used, including for those difficult cases having tortuous vasculature of the subclavian artery and the ascending aorta, during transradial coronary angiography.

The Echocardiographic Study on the 13 Patients with the Hypertrophic Cardiomyopathy

The echocardiographic study was performed to 13 cases with Hypertrophic Cardiomyopathy and 105 normal persons between Nov. 1980 and Feb. 1982 in Heart center, Paik Hospital, In-Je Medical College, Busan, Korea. The left ventricular functions in the Hypertrophic cardiomyopathy were compared with those in the normal. The results were followings: 1. There were 10 male and 3 female of 13 cases with Hypertrophic Cardiomyopathy, whose ages were above 20 years old. 2. LVDeD 4.29+/-0.60cm, LVSeD 2.85+/-0.66cm, LVDeV 82.06+/-24.66ml and LVSeV 26.91+/-9.21ml in the Hypertrophic Cardiomyopathy were significantly changed with those in the normal. 3. E.F. 68.63+/-10.46% and F.S. 34.32+/-6.43% in the Hypertrophic Cardiomopathy were significantly increased with those in the normal. 4. VSTh 1.66+/-0.37cm, LVPWTh 1.04+/-0.29cm in the Hypertrophic Cardiomyopathy were significantly increased with those in the normal.

Blood Flow Pattern of Left and Right Coronary Arteries in Patients with Coronary Arterial Disease Measured by Intracoronary Doppler-tipped Guidewire

BACKGROUND: Measurement of coronary flow velocity in clinical caes contributes to understanding the pathophysiology of coronary circulation. To evaluate the coronary hemodynamics, we analyzed the pattern of coronary flow velocity with a new device consisting of 15Mhz piezoelectric transducer integrated into the tipped 0.018 inch or 0.014 inch flexible, steerable angioplasty guidewire. METHOD: A low profile(0.018 in. or 0.014 in.) Doppler angioplasty guidewire was used to measure the basal blood flow velocity in proximal coronary artery after intracoronary infusion of 200µg nitroglycerine, hyperemic blood flow velocity after intracoronary infusion of adenosine(12µg for LCA, 6µg for RCA). We measured several parameters such as APV (average peak velocity, cm/sec), DSVR(diastolic systolic velocity ratio), MPV(maximal peak velosity, cm/sec), PVI(peak velocity integral, cm), SPVI(systolic peak velocity integral, cm), DSIR(diastolic systolic integral ratio), ASPV(average systolic peak velocity, cm/sec) in basal and hyperemic states. This measurements were made in 17 patients undergoing coronary angiography. RESULTS: 1) APV, ADPV, MPV, in the basal state were higher in LCA than in RCA(32.1±16.6, 40.0±23.6, 57.1±29.0/15.8±9.1, 17.1±10.2, 24.5±20.9cm/sec) and DSVR was also higher in LCA(2.7±2.1/1.4±0.6). But ASPV and SPVI was not different(p>0.05). Significant increases in APV were noted in LCA(32.1±16.6→60.6±17.6cm/sec) and in RCA (15.8±9.1→42.1±15.5cm/sec) after adenosine infusion compared with basal state. DSVR measured in basal state were not statistically different from values in hyperemic state in LCA and RCA(2.7±2.1→2.3±1.7, 1.4±0.6→1.4±0.5, p>0.05). CONCLUSION: The blood flow patterns in both coronary arteries showed different biphasic flow patterns and this finding might be due to the pressure gradient during diastolic phase of both ventricles.

Oxygen Therapy after Correction of Funnel Chest-Ravitch`s Operation-in Postanesthetic Room / 대한마취과학회지

With low-flow oxygen administration, one of the methods of oxygen therapy, the concentration of oxygen depends on the flow rate and on the tidal volume of the patient. It is often casually referred to as 100% oxygen, though in fact it is usually 30-60%, and rarely exceeds 70%. A patient who receiving 6l/min of oxygen flow by nasal cannula is receiving an FiO2 of 44%, nasal cannula with more than 6l flow does little to increase inspired oxygen concentrations. Patients in control group received 0.11/min/kg of oxygen flow by modified T-piece in PAR,in low flow group received 0.11/min/kg of oxygen flow and in high group received 0.2Vmin/kg of oxygen flow. Blood pressure,heart rate, ABGA, SaO2, PAR score were measured in PAR with 5 minutes interval. PaO2 and SaO2 of low flow group were significantly (p<0.05) lower than control group with 5 of PAR score, PaO2 of low flow group was significantly (p<0.05) lower than control group with 10 of PAR score and PaO2 and SaO2 of high flow group were significantly (p<0.05) higher than low flow group with 10 of PAR score. These result suggest that patient who were taken Ravitch's operation receive a higher oxygen flow than others who were taken operation except Ravitch's operation for the same effect of oxygen therapy.

Transradial Interventions in Coronary Artery Disease: Comparison with Transfemoral Interventions

BACKGROUND: Transradial coronary intervention was introduced recently. It has less bleeding and vascular complications and advantage of early ambulation. METHODS: We compared 142 transradial coronary interventions (101 stents, 56 balloon angioplasty and 18 rotablation) with 120 transfemoral interventions in 230 patients from January to August 1998. RESULTS: Overall success rate was not different between two approaches (92% vs 89%), but smaller sized sheath and less amount of contrast agent were required in transradial interventions compared to transfemoral interventions. Conventional guiding catheters which are used in transfemoral approach were used in most cases (94%) of transradial interventions. Judkins left 3.5 (in stead of JL4.0 in femoral approach) and Judkins right 4.0 were the most frequently used guiding catheters in transradial approach. Stent implantation was successfully done in 99 out of 101 lesions (98%) in transradial intervention and 76 out of 78 lesions (97%) in transfemoral intervention. Rotational atherectomy and primary balloon angioplasty or stenting were done successfully in 10 - 20% of the patients in both groups. All procedures were done successfully without any major procedure-related complications (myocardial infarction, death, bypass surgery) or major vascular complications in both groups. During the clinical follow-up of transradial group, punctured arteries showed 10% incidence of radial artery pulse weakness with 3% of pulse loss. CONCLUSION: Transradial approach is useful another feasible route for coronary interventions. The feasibility of primary balloon angioplasty or stenting and rotational atherectomy by transradial approach should be evaluated in the future.

Echocardiographic Analysis of Left Ventricular Function in Hypertensive Patients

The echocardiographic results obtained from normal group(910 cases), borderline hypertention group(78 cases), define hypertension group(182 cases) and hypertensive heart disease group(55 cases) in Heart Center, Paik Hospital, Inje Medical College, Pusan, Korea between September 1985, were as followings; 1) LVDeD, 5.94+/-1.07cm in hypertensive heart disease group was significantly increased compared with 4.93+/-0.64cm in normal group, 4.92+/-0.73cm in borderline hypertension group and 4.90+/-0.64cm in define hypertension group. LVSeD, 4.86+/-0.98cm in hypertensive heart disease group was significantly increased compared with 3.24+/-0.58cm in normal group, 3.21+/-0.52cm in definite hypertension group. 2) LVDeV/s, 180.26+/-84.76ml/m2 in hypertensive heart disease group was significantly increased compared with 86.13+/-37.34ml/m2 in normal group, 86.26+/-41.02ml/m2 in borderline hypertension group and 83.78+/-30.86ml/m2 in definite hypertension group, and LVSeV/S, 97.51+/-55.67ml/m2 in hypertensive heart disease group was significantly increased compared with 25.28+/-13.25ml/m2 in normal group, 23.68+/-14.62ml/m2 in borderline hypertension group and 24.02+/-14.36ml/m2 in definite hypertension group. 3) There was no significant differance of SV/S, between 61.50+/-26.02ml/m2 in normal, 63.98+/-30.86ml/m2 in borderline, 63.06+/-22.96ml/m2 in definite hypertension group and 61.82+/-30.67ml/m2 in hypertesive heart disease group. E.F. 44.06+/-9.94% in hypertensive heart disease group was significantly reduced comparing with 71.08+/-8.89% in normal group, while 74.35+/-8.26% in borderline and 73.28+/-8.49% in definite hypertensive was significantly increased. F.S., 17.02+/-5.18% in hypertensive heart disease group was significantly reduced comparing with 34.44+/-6.81% in normal, while 35.64+/-7.62% in borderline and 36.87+/-7.26% in definite hypertension group was significantly increased. 4) VSTh, 1.24+/-0.32cm in hypertensive heart disease group, 1.17+/-0.12cm in definite hypertension group and 1.16+/-0.41cm in borderline hypertension group are significantly increased comparing with 0.99+/-0.21cm in normal group. LVPWTh, 1.01+/-0.27cm in hypertensive heart disease group and 0.90+/-0.21cm in definite hypertension group was significantly increased comparing with 0.79+/-0.16cm in normal group. 5) VSTh was increased in 13.3% of borderline hypertension group, 32.8% of definite hypertension group and 31.6% of hypertensive heart disease group. LVPWTh was increased in 4.4% of borderline hypertensive group, 26.9% of definite hypertension group and 30.2% of hypertensive heart disease group. Symmetrical septal hypertrophy was noted in 5.1% of borderline hypertension group, 20.1% of definite hypertension group and 32.1% of hypertensive heart disease group. Total septal or left ventricular posteior wall hypertrophy was noted in 17(21.8%) of 78 borderline hypertension group, 154(89.8%) of definite hypertension group and 51(93.9%) of 55 hypertensive heart disease group.

The Electrocardiographic Changes in the Anemia

The electrocardiography was performed to the 95 patients(58 male, 37 female) with chronic anemia in Department of Internal Medicine, Busan City Hospital, Busan University Hospital, Busan paik Hospital In-Je Medical College, Busan korea between April 1978 and March 1981. The hemoglobin levels were 4 Gm%-10Gm%, and the symptoms such as dizziness, palpitation, weakness, and appetite loss were continued for 6 months or more. The results were as following: 1. The abnormal electrocardiographic changes were observed in 49 of the 95 chronic anemic patients. 2. Prolonged QTc interval was observed in 12(12.6%) of 95 cases, depressed ST segment in 48(50.5%), inverted T wave in 32(33.7%), LVH in 31(32.6%), low voltage in 5(5.3%), and Rt. B.B.B. in 5(5.3%). 3. In the Hb level of 4.0~5.9Gm% 20% of the total 95 patients revealed prolonged QTc interval, 86.7% depressed ST segment, 40% inverted T wave, 20% LVH, 6.7% Rt. B.B.B., in the Hb level of 6.0~7.9Gm 10.6% prolonged QTc interval, 39.3% depressed St segment, 42.9% inverted T wave, 39.3% LVH, 3.6% low voltage and in the Hb level of 8.0-9.9Gm% 11.5% prolonged QTc interval, 46.1% depressed ST segment, 27% inverted T wave, 32.7% LVH and 7.7% low voltage.

The Effects of Blood and Normal Saline on Laser-resistant Endotracheal Tube Combustion / 대한마취과학회지

The protection afforded against CO2 and KTP(potassium titanium phosphate) laser-induced combustion by six different types of tracheal tubes was evaluated. Some of them were wrapped with aluminum foil. They were compared before and after the application of human blood to their external surfaces. The difference of protective efficacy against laser between normal saline filled and air filled cuff was evaluated, too. The tracheal tubes tested were laser-resistant tubes such as Bivona Fome-Cuf (Group 1), Xomed Laser-shield 11 (Group 2) and Mallincrodt Laser Flex (Group 3) tubes. Aluminum foil wrapped Rusch red rubber (RR) (Group 4) and Mallincrodt reinforced Polyvinylchloride (PVC) (Group 5) tubes and unwrapped Baxter plain PVC (Group 6) tubes were also tested. CO2 loser set to 38W in continuous mode and KTP laser set to 15W were directed at the shaft of the tracheal tube under study, which had 5L/min. of oxygen flowing through it. The laser was actuated for 90 seconds or until combustion or melting occurred. Bivona tubes (Group 1) resulted in combustion in 5 tubes before and after blood application with CO2 laser, but unaffected before blood and resulted in combustion in 4 of 5 after blood application with KTP laser. Laser-shield 11 tubes (Group 2) offered good protection form both laser before and after application of blood except 1 case. At one case in Group 2, combustion occurred after blood application with CO2 laser. A combustion occured in 1 of 5 prior to application of blood and 5 of 5 after blood in Laser Flex tubes (Group 3) with CO2 laser, but occurred no tube with KTP laser. Wrapped RR (Group 4) and reinforced PVC tubes (Group 5) were unaffected by both laser, but application of blood to the foil wrapped tube shaft resulted in melting in 4 of 5 tubes. Unwrapped PVC tube (Group 6) resulted in immediate combustion in all tubes tested with both laser. Saline inflated cuffs were not perforated only in Group 2 and 3 in KTP laser and perforated immediately in other groups, but there was no combustion with saline in all groups. Air filled cuffs were all perforated in both laser, and combustion occurred. The presence of blood on the surface of metallic foil wrapped or special laser-resistant tracheal tubes may make laser-induced combustion more likely during airway surgery. However, the Laser-shield 11 and aluminum foil wrapped tracheal tube provided good protection from even when covered with blood. In addition, Mallincrodt stainless steel Laser-Flex tube provided good protection from KTP laser only. The saline filling cuffs served as a heat sink that will absorb the lasers energy, thus preventing combustion of cuffs.

The Effect of Minizide(R) in the Hypertensive Patients

The effect of Minizide(R) (prazosine 1mg and polythiazide 0.5mg) was studied in 34 hypertensive patients (female 16 cases, male 18 cases) at the Department of Internal Medicine, Paik Hospital, In-Je Medical College between July, 1979 and August 1980 for 14 months. The patients didn't take the any drugs for one week before the clinical trial with Minizide(R), and thereafter they took minizide 1 tablet 3 times daily for 2 weeks. The blood pressure was checked before and after the trial in the supine and standing position. The results were as followings: 1) The systolic blood pressure before the treatment with Minizide in the supine position was 196+/-24mmHg and in the standing position 188+/-22mmHg. The systolic blood pressure after the treatment with minizide in the supine position was 148+/-16mmHg and in the standing position 139+/-9mmHg. 2) The diastolic blood pressure before the treatment with minizide in the supine position was 118+/-11mmHg, and in the standing position 113+/-10mmHg. The diastolic blood pressure after the treatment with minizide in the supine position was 87+/-6mmHg and in the standing position 84+/-5mmHg. 3) There was no significant orthostatic hypotension after the 34 patients. 4) There was no changes of laboratory findings after the treatment. 5) 3 of 34 hypertensive patients after the treatment complained of headache, 3 palpitation, 2 edema, 2 drowsiness, and 1 fatigability and 1 patient complained of weakness.

The Reliability and Safety of Selective Carotid Angiography from the Right Transradial Approach

BACKGROUND: Carotid artery stenosis is not infrequently associated with coronary artery disease. However, the technique performing selective carotid angiography is not established during right transradial coronary angiography. We investigated the reliability and safety of selective carotid angiography from the right transradial approach. MATERIALS AND METHODS: Following right transradial coronary angiography, selective carotid angio-graphy was performed using a 5 Fr Simmons-2 catheter in 103 patients (59+/-8 years [range, 18-80], 78 males). Ninety five (92%) patients had significant coronary artery disease. Subclavian and innominate arteries were moderately tortuous in 25 (24%) patients and aortic arch was elongated, more vertically oriented in 20 (19%). After forming a loop in ascending (n=65, 63%) or descending (n=38, 37%) aorta, the catheter was withdrawn and rotated counterclockwise to engage its tip in left carotid artery and innominate artery subsequently. After the catheter tip was adjusted at each ostia of carotid arteries, contrast material was delivered. In 63 (61%) patients, the procedures were performed on outpatient basis. RESULTS: Bilateral selective carotid angiography was successfully performed in 101 (98%) patients. In two patients with severely tortuous subclavian artery, the catheter was not engaged selectively in left carotid artery. The image quality of the angiograms performed by manual injection was determined satisfactory. Significant carotid stenosis was found in 17 (17%) patients. There were no complications, including thromboembolism and arterial dissection. CONCLUSION: Selective carotid angiography can be performed reliably and safely using a 5 Fr Simmons catheter from the right transradial approach. This technique is useful for evaluation of isolated or associated carotid artery stenosis from the right transradial approach.

3 Case of Shy-Drager Syndrome

Shy-Drager syndrome is a multisystem degenerative disorder of the central nervous system in which progressive autonomic failure such as orthostatic hypotension, urinary or fecal incontinence, impotence and anhidrosis is a main feature. The nonautonomic neurological abnormalities include parkinsonia, cerebellar and pyramidal signs. In 1984 we met 3 such cases at Seoul National University Hospital and present them here with a brief review of literatures.

Combination chemotherapy with 5-fluorouracil and cisplatin for advanced gastric cancer / 대한내과학회지

No abstract available.

The Report of One Case with the Left Atrial Myxoma Complicated with the Cerebral Embolism

We report 56 year old female with the left atrial myxoma diagnosed by M mode and 2-D Echocardiography, to be complicated with the cerebral embolism with review of literatures.

A Cases of Hypertrophic Cardiomyopathy with pathologic Q Wave in EKG

HCM(=Hypertrophic Cardiomyopathy) is a primary cardiac disease and its characteristic morphologic abnormality is a hypertrophied and nondilated left ventriclar in the absence of another cardiac or systemic disease that itself is capable of producing left ventricle hypertrophy. The symptoms of HCM are varied and include dyspnea, orthopnea, fatigue, chest pain, palpitations and impaired consciousness. The pathophysiologic components of the disease process are left ventricular outlofw obstruction, diastolic dysfunction, myocardial ischemia, and arrhythmia. Predicting the clinical course and outcome for individual patients HCM has been difficult because of variability in natural history and the complexity in disease expression. The present report describe a patient with am asymptomatic, pathologic Q wave in whom HCM was diagnosed by echocardiography, MIBI-SPECT, coronary angiography, and left ventriculography.

The Analysis on Overtransfusion of Surgical Patients / 대한마취과학회지

BACKGROUND: Although blood is very important therapeutic agent in bleeding patients, it may transmit disease, cause an adverse reaction in the recipients, raise the cost of patient care. Since the misuse and inappropriate use of blood is common, we are to review the transfusion practice in our hospital and to reduce unnecessary blood transfusion. METHODS: During a 10-month period from June 1994 through March 1995, 347 patients received blood transfusion during the operation. Among them, we reviewed retrospectively the charts of 211 patients available. We analyzed the transfusion pattern of 47 patients whose postoperative hematocrit exceeded 32 percent to determine the magnitude and cause of unnecessary blood transfusion. RESULTS: The patients of postoperative hematocrit over 32 percent were 119 patients out of 211 patients(56.4%). The main cause of overtransfusion was no reevaluation of the patients hematocrit after the prior unit was given. CONCLUSIONS: Guidelines for transfusing patients must be constructed based on acceptable intraoperative hematocrits. Unnecessary transfusion can be decreased when the transfusion done according to the guidelines, insisting on each reevaluation of the hematocrit prior to the administration of unit of blood.

A Case of Chronic Pancreatitis Complicated by Retroperitoneal and Intrasplenic Pseudocysts / 대한소화기내시경학회지

A 69-year-old-male patient was admitted because of left upper quadrant (LUQ) abdominal pain He had hard palpable mass (abaut 15x l0cm) in LUQ abdomen and mild elevated serum alkaline phosphatase (349 IU/L) and amylase (216 U/L) levels at the admission time. Abdominal CT scan revealed huge, well-marginated cystic mass in enlarged spleen with increased densities in dependent portion of cystic mass, about 8x13cm in size. Another small well-capsulated. cystic mass in anterior aspect of spleen was also seen. After ERCP, urgent surgical intervention was done. Final diagnosis was chronic pancreatitis with large intrasplenic (l2x9.5 cm) and retroperitoneal pseudocysts. So we are reporting above case with pertinent review of literatures.

One Case Report with the Occlusion of the Superior Mesenteric Artery and Left Renal Artery Complicated in the Mitral Stenosis

We report one case with the occlusion of the superior mesenteric artery and the left renal artery in the mitral stenosis with the review of the literatures.