1.Clinical Characteristics of Symptomatic Hypocalcemic Infants.
Joon Young SONG ; Young Lim SHIN ; Han Wook YOO
Journal of Korean Society of Pediatric Endocrinology 2002;7(1):95-104
PURPOSE: The purpose of this study was to evaluate clinical manifestation, etiology and prognosis of hypocalcemic infants who were admitted with seizure. METHODS: We reviewed medical records of 32 infants admitted at the Asan Medical Center with hypocalcemic seizure retrospectively. We classified patients into vitamin D deficiency group(n=7, 21.9%), transient hypoparathyroidism group(n=4, 12.5%), relative hypoparathyroidism with hyperphosphatemia group(n=16, 50%), and others(n=5, 15.6%) according to the laboratory results. RESULTS: Of the 32 patients, 29 patients were improved. There were no differences in gestational age and birth weight among the three groups. In the vitamin D deficiency group, age of onset was later than those of the transient hypoparathyroidism group and relative hypoparathyroidism with hyperphosphatemia group(51.6+/-2.7 vs 8.3+/-.5, 8.2+/-.6 days). In the age when all laboratory results were normalized, transient hypoparathyroidism group was younger than those of vitamin D deficiency group and relative hypoparathyroidism group(33.2+/-4.6 vs 93.6+/-8.5, 77.1+/-2.4 days). In the total treatment period, relative hypoparathyroidism with hyperphosphatemia group was longer than those of vitamin D deficiency group and transient hypoparathyroidism group(68.9+/-3.5 vs 42.0+/-5.0, 25.0+/-4.3 days). Others included two 22q11.2 deletion syndrome patients, a congenital hypoparathyroidism, a pseudohypoparathyroidism, and an early neonatal hypocalcemia. CONCLUSION: Transient hypoparathyroidism and hyperphosphatemia were major causes of neonatal hypocalcemia. And high calcitonin and peripheral organ resistance to parathyroid hormone act on hypocalcemia. In infants after one month, vitamin D deficiency was also an important cause of hypocalcemia. Most of the patients were improved within 1-2 months after proper management, but relative hypoparathyroidism with hyperphosphatemia group needed longer treatment. So, it is necessary to perform a systematic study for several complex causes that explain above fact.
Birth Weight
;
Calcitonin
;
Chungcheongnam-do
;
DiGeorge Syndrome
;
Gestational Age
;
Humans
;
Hyperphosphatemia
;
Hypocalcemia
;
Hypoparathyroidism
;
Infant*
;
Medical Records
;
Parathyroid Hormone
;
Prognosis
;
Pseudohypoparathyroidism
;
Retrospective Studies
;
Seizures
;
Vitamin D Deficiency
2.Modulation of collagen gene expression by colchicine.
Young Wook RYOO ; Kyu Suk LEE ; Joon Young SONG
Korean Journal of Dermatology 1993;31(5):696-701
BACKGROUND: Recent clinial observations have suggested that colchicine, which is in frequent use in gout can affect the conneciive tissue metabolism in skin and other ti.ues. OBJECTIVE: This study suggest that further development of colchiine might provide a novel means of modulating collagen gen expression in patients with fibrotic disease. METHOD: We examed the effect of colchicine on the expres, on of collagen, fibronectin and collagenase gene by skin filroblast culture hy Nort.hern and dot-blot iybridization. Result : The rate of transcription of genomic DNA corresponding o type I collagen and libvonectin was reduced in colchicine-treated cultures but collagenase was not. reduced. Canclusion : The microti.ikile disruptive agent, colchicine, reduced the expression of type I collagen and fibronectin in a dose-(lependent manner. This st.udy suppor that colchicine is one of the promising antifibrogenic drugs curvently being tested as a treatment, of hun an fibrotic disease.
Colchicine*
;
Collagen Type I
;
Collagen*
;
Collagenases
;
DNA
;
Fibroblasts
;
Fibronectins
;
Gene Expression*
;
Gout
;
Humans
;
Metabolism
;
Skin
4.Detection of Mycobacterium leprae in Tissue and Blood by Polymerase Chain Reaction.
Kyu Suk LEE ; Kwang Youl OH ; Young Wook RYOO ; Min Ho SUH ; Joon Young SONG
Annals of Dermatology 1994;6(2):130-135
BACKGROUND: Methods to detect and quanitify Mycobacterium leprae(M. leprae)are needed for studies involving the epidemiology, pathogenesis, and chemotherapy of leprosy. Serological assays and skin tests lack the sensitivity and specificity to serve as diagnostic tool for M. leprae infection. The polymerase chain reaction(PCR) based on the selective amplification of an 530-bp frangment of the gene encoding the proline-rich antigen of M. leprae was performed with sections of fixed or frozen biopsy samples from leprosy patients. OBJECTIVE: This study was done to investigate the applicability of PCR for the detection of low numbers of M. leprae in tissues and peripheral blood. METHODS: The PCR was used to amplify a 530-base-pair M. leprae DNA with the thermoxtable Taq DNA polymerase. RESULTS: The In frozen skin tissues and peripheral blood of leprosy patients. relatively high detection rates of PCR products was achieved by using direct gel analysis as well as Southern blot hybridization. CONCLUSION: These results suggest that PCR amplification for the detection of M. leprae may be useful for the epidemiologic study of large papulations as well as coinical astudies on the individual patients.
Biopsy
;
Blotting, Southern
;
DNA
;
Drug Therapy
;
Epidemiologic Studies
;
Epidemiology
;
Humans
;
Leprosy
;
Mycobacterium leprae*
;
Mycobacterium*
;
Polymerase Chain Reaction*
;
Sensitivity and Specificity
;
Skin
;
Skin Tests
;
Taq Polymerase
5.Extracellular Matrix, TGF - beta Gene and Ha-ras Oncogene Expression in Type I Neurofibromatosis.
Jae Bong JUNG ; Ho June KWON ; Young Wook RHU ; Kyu Suk LEE ; June Young SONG
Korean Journal of Dermatology 1997;35(2):249-257
BACKGROUND: Neurofibroma, the hallmark of neurofibromatosis, is a cutaneous or subcutaneous lesion, with a variable clinical presentation. Histologically, neurofibroma consists of proliferation of nerve derived cellular elements, together with an abundant, collagenous extracellular matrix. Specifically, neurofibroma has been shown to contain 30-50% collagen in its matrix. Objective 5. METHODS: We examined the expression of extracellular matrix genes (collagen, fibronectin, laminin), TGF-b mRNA and Ha-ras oncogene mRNA by using Northern and slot-blot hybridization and immunoperoxidase stains. Result: In Northern blot analysis, Ha-ras and TGF-b genes revealed respectively, 8.8kb and 2. 5kb sized mRNA transcripts in neurofibroma. These parameters were normal in the control. The expression of these genes were 1.9, 2.0 fold increased in neurofibroma. In slot-blot analysis, expression of type I collagen showed fibronectin genes to be 2,401+210, 540+43, respectively, in neurofibroma. So there were 3.7 fold, 2.1 fold, differences respectively, compared to the normal control. However, there were no significant changes of type IV collagen and laminin Bl mRNA levels between neurofibroma and normal skin tissues. Irnmunoperoxidase staining by rnonoclonal anti type IV collagen antibody in neurofibroma showed type IV collagen to be diffusely and weakly stained in tissue. On staining by monoclonal anti-laminin antibody, laminin was stained in a matrix and around vessels. CONCLUSION: The increased expression of extracellular matrix genes may suggest that there is a subpopulation of fibroic cells in neurofibroma which are stimulated by TGF-b. Ha-ras genes which might have accumulated with the differentiation of neural tissue may be related to the pathogenesis of neurofibroma tissue formation. Further studies are needed to determine whether the other factors are related to the pathogenesis of neurofibroma.
Blotting, Northern
;
Collagen
;
Collagen Type I
;
Collagen Type IV
;
Coloring Agents
;
Extracellular Matrix*
;
Fibronectins
;
Genes, ras*
;
Laminin
;
Neurofibroma
;
Neurofibromatoses*
;
RNA, Messenger
;
Skin
6.A Case of Atypical Lupus Vulgaris Developing at a Skin Graft Site.
Bo Sung SON ; Ho June KWON ; Young Wook RYOO ; Kyu Suk LEE ; Joon Young SONG
Annals of Dermatology 1997;9(2):147-150
Lupus vulgaris is most prevalent on exposed parts, especially the face but can also develop on exetremities. Lupus vulgaris originates from tuberculosis elsewhere in the body by hematogenous, lymphatic, or contiguous spread. A 19-year-old male patient came to our department. The patient had had many recurrent oozing and verrucous plaques and crusts on the left foot for one year. A skin biopsy from the lesion on the left dorsum of the foot showed scattered well defined granulomas consisting of the epithelioid cell clusters with Langerhans and foreign body type giant cells in the mid dermis. Caseation necrosis was slight. There were no bacilli on AFB staining. The multi test CMI for tuberculin was highly positive. A chest X-ray did not show any abnormal findings. The presence of Mycobacterium tuberculosis DNA was demonstrated by polymerase chain reactions (PCR) for detection of mycobacterial DNA from a routinely prepared paraffin-embedded skin specimen. Herein we report a very atypical case of lupus vulgaris confirmed by PCR.
Biopsy
;
Dermis
;
DNA
;
Epithelioid Cells
;
Foot
;
Giant Cells, Foreign-Body
;
Granuloma
;
Humans
;
Lupus Vulgaris*
;
Male
;
Mycobacterium tuberculosis
;
Necrosis
;
Polymerase Chain Reaction
;
Skin*
;
Thorax
;
Transplants*
;
Tuberculin
;
Tuberculosis
;
Young Adult
7.Value of color ultrasonography in anetnatal prediction of nuchal cord.
Young Won PARK ; Young Wook YOON ; Jae Sung CHO ; Kyeong SEO ; Chan Ho SONG
Korean Journal of Obstetrics and Gynecology 1993;36(7):2517-2522
No abstract available.
Nuchal Cord*
;
Ultrasonography*
8.Plasma Glucose and Insulin Changes during IV Theophyline Therepy in Preterm Infants with Apnea.
Young Wook YOON ; Yeon Kyun OH ; Eun Hee LEE ; Jung Youl SONG
Korean Journal of Perinatology 1998;9(1):26-30
PURPOSE: The purpose of this study was to evaluate the effects of theophylline in preterm infants with apnea on glucose homeostasis and insulin values. METHOD: In this prospective study, level of glucose and insulin were measured from peripheral blood of 8 neonates(1,450+/-114gm, 31+/-2.1week), who were admitted from April 1, 1997 to July 30, 1997 in Neonatal Intensive Care Unit of Wonkwang University Hospital, for apnea of prematurity(> 20 sec with bradycardia and/or cyanosis) were given aminophylline intravenously. Blood samples were collected at pretreatment, posttreatment 2hours, 1-2days, 3-4days, 5-7days and posttreatment 48hours, and compare to those of the 8 control neonates(1,711+/-232gm, 32+/-1.7week). RESULTS: The results were as follows: 1) Plasma glucose values were significantly higher in the treatment group than those of the control group at 1-2days(104.67+/-20.39mg/dL vs 83.43+/-15.86mg/dL) and 3-4days(111.0+/-32.39mg/dL vs 79.25+/-14.03mg/dL)(p<0.05). 2) Plasma glucose values which were increased at 1-2days(104.67+/-20.39mg/dL)and 3-4days(111.0+-32.39mg/dL), were significantly higher than pretreatment values(66.33+/-31.19mg/ dL)(p<0.02), but were not increased to the level of hyperglycemia(> 125mg/dL). 3) The mean posttreatment glucose levels drawn at 48hours after discontinuation of theophylline was significantly decreased to the values of pretreatment values compared to those of the 1-2days and 3-4days(p<0.01). 4) Insulin concentrations were also increased insignificantly when blood glucose were increased in the treatment group compared with control group. CONCLUSION: In conclusion, intravenous administration of theophylline produces hyperglycemia significantly, including an increase in a serum insulin. But, clinically significant hyperglycemia(> 125mg/dL) was not noted. So, plasma glucose may not need to be monitored in preterm apneic infants receiving theophylline. But, further studies are need to elucidate the effect of theophylline considering the serum toxic level of theophylline.
Administration, Intravenous
;
Aminophylline
;
Apnea*
;
Blood Glucose*
;
Bradycardia
;
Glucose
;
Homeostasis
;
Humans
;
Hyperglycemia
;
Infant
;
Infant, Newborn
;
Infant, Premature*
;
Insulin*
;
Intensive Care, Neonatal
;
Plasma*
;
Prospective Studies
;
Theophylline
9.A Case of Squamous Cell Carcinoma Arising in the Mature Cystic Teratoma with Direct Invasion to Transverse Colon and Jejunum.
Do Sang LEE ; Moo Hyung SONG ; Wook KIM ; Il Young PARK ; Jong Man WON
Journal of the Korean Society of Coloproctology 1998;14(1):149-152
Malignant degeneration of mature cystic teratoma has been reported in 1~3% of cases, usually between the age of 30 and 70 years with a peak incidence of 40~60 years. The most common malignancy developing in such tumors is squamous cell carcinoma arising in a mature cystic teratoma. Hirakawa reported two patients with benign teratomas diagnosed 25 and 32 years prior to surgery for malignancy and Dorothea reported a patient who was diagnosed as benign teratoma 50 years prior to operate for carcinoma. But there has been no report of squamous cell carcinoma arising in the mature cystic teratoma with direct invasion to gastrointestinal tract. We report a case of squamous cell carcinoma with direct invasion to transverse colon and jejunum in 62-year-old female who was diagnosed as mature cystic teratoma 20 years ago.
Carcinoma, Squamous Cell*
;
Colon, Transverse*
;
Female
;
Gastrointestinal Tract
;
Humans
;
Incidence
;
Jejunum*
;
Middle Aged
;
Teratoma*
10.A Case of Cornelia de Lange syndrome.
Byoung Hong AHN ; Song Nyeon CHOI ; Young Wook KIM ; Ki Bok KIM
Journal of the Korean Pediatric Society 1990;33(2):234-240
No abstract available.
De Lange Syndrome*