Prediction of Resistance to Standard Intravenous Immunoglobulin Therapy in Kawasaki Disease

BACKGROUND AND OBJECTIVES: Ten to twenty percent of children with Kawasaki disease (KD) do not respond to initial intravenous immunoglobulin (IVIG) treatment. If untreated, approximately 15% to 25% of KD patients have complications. The aim of this study was to find useful predictors of responsiveness to initial IVIG treatment in KD. SUBJECTS AND METHODS: We retrospectively reviewed medical records of 91 children diagnosed with KD at Myong Ji Hospital from March 2012 to April 2014. Before and after (24 hours to 36 hours) IVIG treatment, the following laboratory data were obtained: hemoglobin (Hb) level, white blood cell count, proportion of neutrophil, lymphocyte and eosinophil, platelet count, erythrocyte sedimentation rate (ERS), C-reactive protein (CRP), creatine kinase (CK), creatine kinase MB (CK-MB), and N-terminal pro-brain natriuretic peptide (NT-proBNP). Subjects were then divided into two groups: IVIG-responsive or IVIG-resistant. RESULTS: Of 91 patients, 11 (12%) required retreatment. By univariate analysis, before-IVIG laboratory parameters of white blood cell count, % neutrophil, ERS, CRP, sodium, CK, CK-MB, and NT-proBNP were significantly different between IVIG-responsive and IVIG-resistant patient groups. In the after-IVIG laboratory parameters, Hb level, white blood cell count, % neutrophil, % lymphocyte, CRP, CK, CK-MB, and NT-pro-BNP were significantly different between the two groups. While the mean-differences were not statistically significant, fractional change (FC)-CRP and FC-% neutrophil showed significant difference. By multivariate analysis, FC-CRP was confirmed to be an independent predictor for initial IVIG resistance. CONCLUSION: Fractional change-C-reactive protein might be a useful and important value for predicting initial IVIG resistance in KD patients.

Endovascular Therapy of Left Subclavian Artery Stenosis in Patients with Left Internal Mammary-Coronary Artery Bypass Graft

PURPOSE: We wanted to evaluate the clinical usefulness of percutaneous transluminal angioplasty (PTA) and stenting of left subclavian artery (LSA) stenosis in the patients with a left internal mammary artery (LIMA)-coronary artery bypass graft. MATERIALS AND METHODS: From September 1998 to November 2005, significant proximal LSA stenoses were treated with PTA and stenting in 22 patients (15 men and 7 women) who had a prior LIMA-coronary artery bypass graft or who were willing to undergo LIMA-coronary artery bypass grafting. The technical success rates, complications and restenosis during the follow-up period were retrospectively evaluated. RESULTS: Six patients had a prior LIMA bypass graft and 16 patients were treated before their coronary artery bypass surgery. The etiology of the LSA stenosis was atherosclerosis in all patients. Four patients were treated with PTA only, and stents were placed in 18 patients. Technical success was achieved in all patients. An embolism in the proximal LIMA occurred after stenting in one patient, and the LIMA was recanalized with transcatheter thrombolysis. During a mean follow-up of 30 months, only one patient was found to have recurrent LSA stenosis 29 months after stenting and this patient was successfully managed with angioplasty. CONCLUSION: Endovascular therapy is useful and efficacious for the treatment of LSA stenosis in patients with a LIMA-coronary artery bypass graft.

A Case of Desmoplastic Trichilemmoma / 대한피부과학회지

Desmoplastic trichilemmoma is a rare variant of trichilemmoma characterized by typical features of trichilemmoma with abundant desmoplastic stroma. We report herein a 54-year-old man presented with 2-year history of a solitary bean sized verrucous nodule on the occipital scalp. Histologically, the tumor showed a well-defined lobular growth extending from the epidermis into the dermis with abundant central desmoplastic stroma. Epithelial lobules at the margins of the neoplasm showed the typical features of trichilemmoma composed of glycogen-rich clear cells and peripheral palisading columnar cells. In contrast, at the center the cells assume a more random pattern of cords and strands traversed by the hyaline stroma, mimicking invasive carcinoma. It was diagnosed desmoplastic trichilemmoma by the distinctive histologic appearance and totally excised.

A Case of Epithelioid Angiosarcoma of the Scalp / 대한피부과학회지

Angiosarcoma is a rare malignant tumor of endothelial cell origin. Histopathologically, varied differentiation may be observed even within the same tumor. Epithelioid angiosarcoma is one subset of poorly differentiated angiosarcoma which is a rare variant. We report a case of epithelioid angiosarcoma of the scalp in a 59-year-old man. The patient had a painful solitary, 2x3 cm sized, dome-shaped, firm, erythematous to purplish nodule with crust on the vertex of the scalp. Histopathologically, the tumor was chiefly composed of solid sheets of atypical epithelioid cells with prominent eosinophilic cytoplasm, a large vesicular nuclei, and occasional intracytoplasmic vacuoles. Well to moderately differentiated areas with irregular vascular channel-like structures lined by atypical hyperchromatic endothelial cells were also seen in the peripheral areas of the tumor. Immunohistochemically, the tumor cells were positive with CD31 which is sensitive marker of endothelial cell.

A Case of Adult-onset Urticaria Pigmentosa / 대한피부과학회지

Mastocytosis is a heterogenous group of disorders characterized by abnormal growth and accumulation of mast cells in one or more organ systems. Urticaria pigmentosa represents the most common form of cutaneous mastocytosis in children and adults. We report a case of adult-onset urticaria pigmentosa in a 53-year-old man. The patient had pruritic multiple scattered well-demarcated brownish macules and papules on whole body for 4 years. Histopathologic examination showed diffuse and perivascular infiltration of lymphocytes and cuboidal mast cells in the upper dermis. Any sign or symptom of systemic involvement was not detected.

A Clinical Study of Skin Problems of The Amputee / 대한피부과학회지

BACKGROUND: There has been no clinical study of skin problems of the amputee in Korea and at Veterans Hospital, we have experienced so many skin problems of amputees. OBJECTIVE: We tried to observe skin problems of amputees and to relate them with sweating, poor hygiene, poor fit of socket, mechanical trauma, and duration of wearing prosthesis. METHODS: 125 extremity amputees who visited the Seoul Veterans Hospital during the period of 5 years from Jan. 1994 to Dec. 1998 were analyzed clinically. RESULTS: The study results are summarized as follows: 1. Among the 125 extremity amputees, the number of male patients was 124(99.2%) and that of female patients was 1(0.8%). The age distribution showed a peak incidence in the 7th decade(30.4%), followed by the 5th decade(28.8%), 6th decade(24.8%), and the mean age was 56.4 years. 2. The most common type of amputation was below the knee amputation(74 cases), the second was above the knee amputation(37 cases). 3. The duration of wearing prosthesis was above 20 years in 88 patients(70.4%), 16-20 years in 15(12%), 11-15 years in 11(8.8%), and 1-5 years in 6(4.8%). 4. The skin problems of the amputees were superficial fungal infection, chronic ulcer, intertriginous dermatitis, callus, corn, verrucous hyperplasia, verruca which were 63 cases(50.4%), 26 cases(20.80%), 17 cases(13.6%), 6 cases(4.8%), 5 cases(4%), 5 cases(4%), 3 cases(2.4%) respectively. 5. The predisposing factors of the dermatoses were mechanical trauma(84 cases) which acted as a cause of the chronic ulcer, intertriginous dermatitis, corn and callus, sweating(80 cases) and poor hygiene(11 cases) as those of superficial fungal infection, and poor fitting(6 cases) as that of verrucous hyperplasia. CONCLUSION: Superficial fungal infection and chronic ulcer were the main skin problems of the amputee. The longer the duration of wearing prosthesis, the more skin problems developed. The mechanical trauma and sweating were the major predisposing factors of stump dermatoses, especially of chronic ulcer and superficial fungal infection. Amputation is only the beginning of a long period of rehabilitation, and the treatment of the patient and the affected skin goes on for the rest of the patient's life. This clinical study suggests that the dermatologist must be concerned of the care of amputee's skin problems.

A Case of Chronic Actinic Dermatitis / 대한피부과학회지

Chronic actinic dermatitis was recently proposed as a syndrome including photosensitive eczema, photosensitivity dermatitis, actinic reticuloid, and persistent light reactivity. It is defined on the basis of 3 criteria, including clinically persistent eczematous eruption on sun-exposed skin, histologically consistent with chronic eczema, and photobiologically decreased MED to UVB. We report herein a 61-year-old man presented with 2-year history of erythematous to brownish lichenified papules and plaques on sun-exposed areas and shown decreased minimal erythema doses to both UVB and UVA. Biopsy specimen showed consistent with chronic eczema. He was treated with topical therapies of topical steroids and intralesional injection of triamcinolone, and oral anti-histamine, with avoidance of exposure to sun.

The Effectiveness of Mycophenolate Mofetil(MMF) in the Prevention of Acute Rejection and Graft Outcome in Clinical Renal Transplantation / 대한이식학회지

In this study, We evaluated the safety and efficacy of mycophenolate mofetil (MMF) for the prevention of acute rejection episodes when given in combination with cyclosporine and corticosteroids during the postoperative period in living donor kidney transplantation. 212 patients were enrolled; 106 patients received triple therapy with azathioprine (control group) and another 106 patients received triple regimen with MMF 2 g/day (study group). There was two subgroup in the study group. Of the study group, one subgroup was 90 patients treated MMF after acute rejection episode or an increase in serum creatinine level above 2.0 mg/dl (MMF conversion) and another group was 16 patients with primary MMF therapy. We made no demographic difference between study and control groups with patient selection for reducing bias. There was no statistical differences in serum creatinine level between two groups at 2 years after transplantation. There were 12 (13.3%), 3 (18.8%) acute rejection episodes in the MMF conversion and primary MMF therapy groups and 36 (33.7%) in the control group with statistic difference (p=0.01). 5 patients (5.6%) in the MMF-treated group lost their graft versus 16 patiens (15%) in the AZA-treated group. The 2-year graft survival rate was 97.4% in the study group and 83.9% in the control group. There was signficant difference in 2-year graft survival (p=0.003). The adverse effects of MMF were opportunistic infection, leukopenia, abdominal pain and diarrhea. MMF in combination with cyclosporine and prednisone was superior to a standard immunosuppressive regimen including azathioprine. Taken together, the data indicated that MMF will be a valuable addition to the list of immunosuppressants available for the prevention and treatment of acute renal rejection after renal transplantation. Its final place in clinical transplantation will be determined by further analysis of future, randomized-prospective studies, and by broadening experience with this important addition to the immunosuppressive regimen.

PTBD Spiral CT Cholangiography: Utility in Patients with Extrahepatic Biliary Obstruction

PURPOSE: The purpose of this study is to assess the utility of PTBD spiral CT cholangiography, after infusion of contrast media through a PTBD tube, for evaluation of a biliary lesion after emergency PTBD due to severe jaundice. MATERIALS AND METHODS: Forty patients with emergency PTBD due to extrahepatic biliary obstruction were transferred to our clinic and prospectively studied. The causes of obstruction were 17 extrahepatic cholangiocarcinomas (including three Klatskin's tumors), seven pancreatic head carcinomas, six calculous diseases of the common bile duct, six periampullary lesions, two ampulla of Vater carcinomas, one gall bladder carcinoma with invasion of the common hepatic duct, and one cholangitis. Diagnosis was on the basis of pathologic, radiologic, and clinical findings. Pre-contrast CT scanning was performed. After the infusion of contrast media (iothalamate : normal saline=1:10) through a PTBD tube, spiral CT scans were obtained. After IV infusion of contrast media (Ultravist, 100cc), early- and delayed-phase spiral CT scans were obtained at 45 and 210 seconds, respectively, with an interscan interval of 5mm. 3-D CT cholangiograms were then reconstituted. Spiral CT without infusion of contrast media through a PTBD tube and PTBD spiral CT cholangiography were performed in 14 cases. The level of extrahepatic biliary obstruction was categorized as either upper, middle, or lower third. In 21 surgically confirmed cases, we evaluated the accuracy with which the level and cause of obstruction was determined; levels and causes during surgery and by as seen on PTBD cholaniography were compared. RESULTS: The levels of obstruction diagnosed on PTBD spiral CT cholangiography and on 3-D CT cholangiography corresponded in all cases to the levels during surgery and on PTBD cholangiography [upper third (n=7), middle third (n=12), lower third (n=21)], and the level diagnosed on spiral CT without infusion of contrast media through a PTBD tube corresponded to the level during surgery in ten of 14 cases. The cause of obstruction diagnosed on PTBD spiral CT cholangiography corresponded to pathologic findings in 19 of 21 cases. In 15 cases, 3-D CT cholangiography was diagnostically helpful. CONCLUSION: PTBD spiral CT cholangiography is a useful diagnostic method for determining the level and cause of biliary obstruction.

Evaluation of Lateral Margin of Left Lobe of the Liver on CT Scan: Focus on Perisplenic Extension

PURPOSE: The perisplenic extension of the left lobe of the liver can be misinterpreted as a splenic or perisplenic lesion on ultrasonography(US) and computed tomography(CT). The purpose of our study is to classify thelateral margin of the left lobe of the liver into three types and to evaluate the incidence and the relationship between each type and abnormal liver on CT scan. MATERIAL & METHODS: A total of 515 abdominal CT scans frompatients over 15 years old were retrospectively evaluated. Liver contours were divided into three types on the basis of degree of the left lateral extension of left lobe of the liver. Type A was defined as the lateral extension of the left lobe of liver to the medial portion of the stomach, type C as the perisplenic portion, andtype B as between the two types. Each type was further divided into normal and abnormal liver groups based onclinical, CT, surgical & patholigic findings and evaluated on its ratio of normal and abnormal liver, intrahepatic diseases associated with an abnormal liver and statistical significance between a normal and abnormal liver. RESULTS: The incidence of the three types of liver among the 515 patients was 360(69.9%), 121(23.5%) and 34(6.6%) patients in type A, B and C, respectively. Type C showed normal liver in six patients, which was 2.7% of allnormal livers(221/515) and abnormal liver in 28 patients, which was 9.5% of all abnormal livers(294/515). Type Ashowed normal liver in 49.7%, abnormal liver in 50.3% and there was not statistically significant difference between normal and abnormal liver(P>0.05). Type B showed normal liver in 29.8% and abnormal liver in 70.2% ; type C showed normal liver in 17.6%, abnormal liver in 82.4% and there was a statistically significant difference between normal & abnormal liver(P<0.001). The space occupying lesion(SOL) was most common(52.6%) in all theabnormal livers and hepatoma was the most common disease in the SOL(47.2%). In the abnormal type C liver, SOL(58%)and diffuse hepatopathy(32.8%) were most common and hepatoma and liver cirrhosis were the most common diseases,respectively. CONCLUSION: The perisplenic extension of the left lobe of the liver(type C) was seen in 6.6% of abdominal CT scans, and was frequently associated with abnormal liver conditions including SOL. Careful interpretation is needed to differentiate from splenic or perisplenic disease.

Radiologic Findings of Hemophilic Arthropathy of the Knee: Focusing on MR Imaging & Plain Radiography

PURPOSE: To evaluate the characteristic MR findings of hemophilic arthropathy of the knee. MATERIALS & METHOD: Seven knees in six patients with hemophilia (five hemophilia A and one hemophilia B) were retrospectively studied with MR images and plain radiographs. Patients were aged between 2 and 20 years (mean, 11) and all had a clinical history of repeated hemarthrosis. MR images of the knee were analyzed with respect to intra- andextra-articular hemorrhage, the state of synovial tissue, articular cartilage, bone, menisci, and ligaments. RESULTS: Synovial hypertrophy and articular cartilage destruction were revealed in all seven knees ; pannus wasfound in four, and was seen as low signal intensity on T1-weighted image and high signal intensity on T2-weighted images. All five instances of synovial hypertrophy and pannus were enhanced. Joint effusion, presented in five ofseven knees, demonstrated slightly low signal intensity on T1-weighted image and high signal intensity on T2-weighted images, and was associated with peripheral low signal intensity of hemosiderin. Subchondral and marginal erosion was seen in six cases, patellar deformity in three, meniscal damage in four and cruciate ligament damage in one case. CONCLUSION: MR is superior to plain radiography in demonstrating chronic repeated hemarthrosis (manifested as thick intra-articular effusion), hemosiderin, synovial hypertrophy, erosion ordestruction of articular cartilage and bone, and meniscal or cruciate ligament injury of hemophilic arthropathy ofthe knee. MR is therefore thought to be a useful imaging study for accurate evaluation of hemophilic arthropathyof the knee.

Ureteroscopic stone removal / 대한비뇨기과학회지

Rigid ureteroscopy is now established technique for the management of ureteral stones. We evaluated the experiences of rigid ureteroscopy retrospectively at our institutions from October 1990 to June 1991, 30 ureteroscopies were performed for the removal of ureteral stones and one ureteroscopy for the removal of Kock pouch stones. In 23 cases(74.2%), the stones were removed successfully. The likelihood of failure was greater for stones larger than 10mm and above the lower ureter. Among the reasons for failure to remove stones. the failure to insert the ureteroscope into the ureteral orifice was the most common. Complications were developed in 11 cases but managed conservatively in all cases. We agree that ureteroscopic removal of stone is the treatment of choice for lower ureteral stone of smaller than 10mm in size with proven efficacy and safety.

A case of giant renal artery aneurysm associated with arteriovenous fistula / 대한비뇨기과학회지

A 48-year-old man presented with gross hematuria, hypertension and right flank pain. Angiography revealed unusual giant renal artery aneurysm associated with arteriovenous fistula. After nephrectomy, symptoms and signs were subsided. Herein we report this case with brief review of the literatures.

A case of eosinophilic cystitis / 대한비뇨기과학회지

Eosinophilic cystitis is an uncommon inflammatory process seen in children and adult which was recognized in 1960. The disease is similar to interstitial cystitis. tuberculosis and bladder neoplasms in the clinical manifestations and should be differentiated from them. We herein report a case of eosinophilic cystitis in a 60-year-old woman which was confused. with bladder carcinoma with brief review of the literatures.

5 cases of stones occurred in Kock pouch / 대한비뇨기과학회지

During follow-up of Kock pouch, we found 5 cases of stones in the pouches (3 cases in male and 2 cases in female). Follow-up period was ranged from 34 to 62 months. The size of the stones varied from 0.5 to 4.3cm. 4 cases of stones were managed by endourological procedures, using ultrasonic and electrohydraulic lithotripsy. A case fail to manage by endourological procedure end so needs open operation. No complication occurred during or after the procedure. Spinal anesthesias were necessary in 5 patients. The stones were usually developed around suture materials near efferent nipple valve. The major component of the stones was struvite. We suggest that causes of stone development in Kock pouch may be suture materials and infected residual urine.

A Case of Cutaneous Hemorrhagic Bullous Eruptions in Lymphoma of the Small Intestine / 대한피부과학회지

Authors experienced a 59-year-old male patient with the lymphoma of the small iritestine which involved the adjoining mesenteric lymph nodes, the parietaI pleura, and the skin. The skin lesions, simulating necrotizing angiitis, were deep purple colored, asymptomatic, tense, hemorrhagic bullae with soft consistency on the both thighs and left infraorbital area. He had extranodal type, and diffuse, poorly differentiated lymphocytic lymphoma by the Rappaport classification, and stage IV by the Ann Arbor staging classification. After about 50 days of clinical manifestations, he was died mainly due to massive intestinal bleeding.

The Simplifed Hair Baiting Technique and its Practical Application / 대한피부과학회지

In order to avoid contamination attributed to Vanbreuseghem hair haiting technique in the laboratory, the author contrived the following modified method. After adding 100ml of antibiotics solution (mixture of 500mg of Cyclohexamide, 20, 000u of Penicillin and 40mg of Streptomycin in 1,000ml of distilled water) in PVC phial containing 100gm of soil, it was gently agitated for an hour and lefted to be settled for 12 hours. The suspended solution obtained and a batch of horse hair were randomly distributed on a Petri dishfitted with blue cloths, then cultured at 24C'. The overall results obtained from this modified method were not as satisfying as that of the Vanbreuseghem method but a few advantages such as simplicity of the procedure and significantly lower laboratory contamination implicated its practicality. Furthermore, this method was also found to be useful in the first isolation of infected animal hairs, hair perforation tests and as a substitution for artificial media. For the cross mating, a patch of blue cloths was laid on top cf moist sterilized soil evenly spreaded on a Petri dish. Then hair-baiting procedure was performed and good results were obtained.

Trends in Scabies for 12 Years / 대한피부과학회지

In order to appreciate the incidence of scabies, the authors conducted an observation of ll,848 cases of scabies in 152,254 outpatients at Chilgok Catholic Skin Clinic from January 1968 to December 1979 and the following results were obtained; The incidence of scabietic patients was 7.78% among total outpatients and the annual frequency increased progressively from 1972 (3.86%), showed maximal incidence in 1975(12. 35%), and thereafter decreased. In sexual distribution, males predominated over female 1.9: 1. In age distribution, 2nd and 3rd decades occupied 64 of total scabietic patients. The monthly distribution of total scabietic patients was highest in December (10.85%) and lowest in August (4.57%) but no seasonal variation was observed. Over half(54.9%) of 5,273 scabietic patients visited within 2 months after their infection. 1,031 cases(10.27%) of 10, 036 scabietic patients revealed the complications such as infection (5.04%), eczematization (3.37%) and nodule (1.86%).

Two Cases of Onychomycosis due to Aspergillus repens / 대한피부과학회지

Onychomycosis caused by the genus aspergillus is extremely rare in Korea and only one case due to A. sydowi has been reported. We experienced two casea of onychomycosis infected with A. repens, which were healthy adults. The first case was a 29 year-old male who developed thickening and dark discoloratian of 1st, and, 3rd and 5th toe-nails of left foot from about 5 months on. The second case was a 27 year-old male who developed thickening and yellow-whitish discoloration of the nail of right thumb from about 3 rnonths on. In both cases, direct examination of the affected nails showed many thick bluish hyphae and spore chains, and A. repens was isolated. Our cases were first reported through the Korean literature.

Tuberous Sclerosis Occuring through 3 Generations / 대한피부과학회지

Tuberous sclerosis is a complex neurocutaneous disease inherited as an autosomal dominant pattern, which is characterized by facial angio-fibroma, mental retardation and seizures. Many cases have been reported in the literatures, but familial cases are relatively rare. We present 9 cases of tuberous sclerois occuring througb 3 generations, which are 3 cases of angiofibroma, mental retardation and seizures, 2 cases of facial angiofibroma and seizures, 1 case of mental retardation and seizures and 3 cases who showed facial angiofibroma only. Two brothers in this family reveald icthyosis vulgaris without tuberous sclerosis.