3.A Case of Prader-Willi Syndrome with FUO Diagnosed at Infancy.
Jun Ho KIM ; Jin Hwa JEONG ; Sung Min CHO
Journal of the Korean Pediatric Society 2000;43(8):1142-1148
Prader-Willi syndrome(PWS) was first described by Prader et al in 1956. This syndrome is characterized by diminished fetal activity, low birth weight, infantile hypotonia with feeding problem, temperature instability, early onset of childhood hyperphagia with consequent obesity, short stature, hypogonadism and mental retardation. The deletion of chromosome 15(del 15(qll-13)) was reported by Ledbetter in 1981, which was thought to be of paternal origin. Recently, such micro- deletion may be diagnosed by fluorescence in situ hybridization(FISH) that recognizes specific DNA base sequence. We experienced a Prader-Willi syndrome confirmed by FISH in an infant that had hypotonia, growth retardation, feeding difficulty and FUO since 1 month of age. We report this case with a brief review and related literature.
Base Sequence
;
DNA
;
Fetal Movement
;
Fluorescence
;
Humans
;
Hyperphagia
;
Hypogonadism
;
Infant
;
Infant, Low Birth Weight
;
Infant, Newborn
;
Intellectual Disability
;
Muscle Hypotonia
;
Obesity
;
Prader-Willi Syndrome*
4.A Case of Fryns Syndrome.
Jun Ho KIM ; Jin Hwa JEONG ; Sung Min CHO
Journal of the Korean Pediatric Society 2000;43(9):1269-1273
Fryns syndrome is a lethal syndrome of multiple congenital anomalies first described by Fryns et al in 1979. A recently developed major diagnostic criteria includes abnormal face, small thorax with widely spaced hypoplastic nipples, distal limb and nail hypoplasia, lung hypoplasia with diaphragmatic hernia, central nervous system anomalies and congenital heart disease. The pathogenesis of Fryns syndrome is not clear. Of the major immediate life-threatening abnormalities of this syndrome, lung hypoplasia associated with diaphragmatic hemia has usually proven to be fatal. We report a case of Fryns syndrome, which has the prenatal ultrasonographic findings of Dandy-Walker malformation and renal hypoplasia.
Central Nervous System
;
Dandy-Walker Syndrome
;
Extremities
;
Heart Defects, Congenital
;
Hernia, Diaphragmatic
;
Lung
;
Nipples
;
Thorax
5.A Case of Polymorphous Low Grade Adenocarcinoma in Submandibular Gland.
Hun Po CHO ; Young Min LEE ; Jun Ki LEE ; Jun Ho PARK
Korean Journal of Otolaryngology - Head and Neck Surgery 2014;57(11):778-782
Polymorphous low-grade adenocarcinoma (PLGA) is a distinctive salivary gland neoplasm with a propensity to arise from minor salivary glands, particularly of the palate. Reports of PLGA have increased with the establishment of specific histopathological criteria characterizing the PLGA, but there are only a few reports of major salivary gland origin. PLGA shares histological features with adenoid cystic carcinoma, so histopathologic diagnosis is essential and immunohistochemistry is an important tool when making differential diagnosis. The treatment of choice is wide surgical excision, and long-term follow up is necessary to evaluate local recurrences. We herein report a case of PLGA arising in a submandibular gland with a review of the relevant literature.
Adenocarcinoma*
;
Carcinoma, Adenoid Cystic
;
Diagnosis
;
Diagnosis, Differential
;
Follow-Up Studies
;
Immunohistochemistry
;
Palate
;
Recurrence
;
Salivary Gland Neoplasms
;
Salivary Glands
;
Salivary Glands, Minor
;
Submandibular Gland*
6.A Case of Immunoglobulin G4-Related Sialadenitis and Dacryoadenitis.
Kyoung Kyu LEE ; Hun Po CHO ; Young Min LEE ; Jun Ho PARK
Korean Journal of Otolaryngology - Head and Neck Surgery 2013;56(7):444-447
Hypertrophy of salivary gland was developed by sialadenitis, sialolithiasis, autoimmune diasease and other tumorous conditions. Mikulicz's disease has been used to describe symptomless hypertrophy of the salivary gland and lacrimal gland. In the past, it was thought that Mikulicz's disease is included within the diagnosis of Sjogren's syndrome. However, Mikulicz's disease represents Immunoglobulin G4 (IgG4)-related hypertrophy of salivary gland and lacrimal gland by an elevated level of IgG4 in the serum of the patient and good responsiveness to glucocorticoid, eventually leading to recovery of gland function. We have experienced a case of IgG4-related sialadenitis and dacryoadenitis with bilateral hypertrophy of parotid, submandibular and lacrimal glands. We report this case with a brief review of the literature.
Dacryocystitis
;
Humans
;
Hypertrophy
;
Immunoglobulin G
;
Immunoglobulins
;
Lacrimal Apparatus
;
Salivary Gland Calculi
;
Salivary Glands
;
Sialadenitis
;
Sjogren's Syndrome
7.Development of biocompatible dressing material made of collagen and amniotic membrane and wound healing experiment in rat.
Kang Min AHN ; Ji Ho LEE ; Ui Lyong LEE ; Jong Ho LEE ; Jong Won LEE ; Sung Po KIM ; Eun Kyung YANG ; Ki Ho KIM
Journal of the Korean Association of Oral and Maxillofacial Surgeons 2006;32(3):189-199
<0.05). 4. Histologic examination revealed that AM protected leukocyte infiltration and epithelial migration was nearly completed at 4 weeks. Terudermis(R) group showed mild neutrophil infiltration until 2 weeks and completion of epithelization at 4 weeks. Control group showed massive leukocyte infiltration until 4 weeks. 5. Microvessels were increased sharply at 1 week and control group at 1 and 4 week showed significant differences with Terudermis(R) group of same interval(p<0.05) but no differences were found with AM group(p<0.05). CONCLUSION: EGF and EGF-R were well preserved in freeze-dried AM. AM attached to collagen acted as excellent biologic dressing which had similar effect with Terudermis(R). AM showed anti-inflammatory action and healing was completed at 4 weeks after full-thickness skin defect.
Amnion*
;
Animals
;
Bandages*
;
Biological Dressings
;
Collagen*
;
Epidermal Growth Factor
;
Leukocytes
;
Microvessels
;
Neutrophil Infiltration
;
Rats*
;
Skin
;
Wound Healing*
;
Wounds and Injuries*
8.Absorbable Guided Bone Regeneration Membrane Fabricated from Dehydrothermal Treated Porcine Collagen
Kang Mi PANG ; Han Wool CHOUNG ; Sung Po KIM ; Eun Kyung YANG ; Ki Ho KIM ; Soung Min KIM ; Myung Jin KIM ; Jeong Won JAHNG ; Jong Ho LEE
Journal of the Korean Association of Maxillofacial Plastic and Reconstructive Surgeons 2011;33(2):112-119
9.Effects of Thiopental on the Adhesion of Polymorphonuclear Neutrophils and Myocardial Function in an Ischemia/Reperfusion Isolated Guinea Pig Heart Model.
Sung Uk CHOI ; Hun CHO ; Choon Hak LIM ; Hye Won LEE ; Hae Ja LIM ; Suk Min YOON ; Seong Ho CHANG ; Po Soon KANG
Korean Journal of Anesthesiology 2003;44(5):709-716
BACKGROUND: The adhesion of polymorphonuclear neutrophils (PMNs) to the coronary endothelium is an crucial step in PMN-mediated reperfusion injury. The purpose of this study was to investigate whether thiopental inhibits the postischemic coronary vascular adhesion of PMNs, and results in reduced postischemic myocardial dysfunction in isolated guinea pig hearts. METHODS: Hearts (n = 6-8/group) were isolated from male-guinea pigs and perfused with modified Krebs-Henseleit solution. After isolation, hearts were stabilized for 10 minutes, perfused for 15 minutes, allowed an ischemic period for 30 minutes, and a reperfusion period of 60 minutes (C group). In the P group, a bolus of 1x10(6) PMNs was infused 2 minutes after reperfusion, and in the T group additional thiopental was infused 5 minutes after the start of reperfusion, and PMNs were infused on 2 minutes after reperfusion. PMN adhesion (%), LVDP, LVEDP, +/-dP/dt, HR, and CF were measured pre- and postischemia. RESULTS: The addition of thiopental (25 microM) to the perfusate reduced the postischemic coronary vascular adhesion of PMNs (72.5+/-4.5% vs 40.0+/-7.4%, P < 0.05) and prevented postischemic myocardial dysfunction compared with group P (73.5+/-6.9% vs 48.4+/-3.0%, P < 0.05). CONCLUSIONS: Postischemic myocardial dysfunction is significantly more pronounced after PMN infusion. Thiopental reduced the postischemic coronary vascular adhesion of PMNs and attenuated the myocardial dysfunction, which was responsible at least in part, for the cardioprotective effect.
Animals
;
Endothelium
;
Guinea Pigs*
;
Guinea*
;
Heart*
;
Ischemia
;
Neutrophils*
;
Reperfusion
;
Reperfusion Injury
;
Swine
;
Thiopental*
10.The Effect of Metoclopramide on the Time from Disapprearance of 1 Hz Single Twitch Height to Reappearance of that following injection of Succinylcholine.
Sung Sook OH ; Po Sun KANG ; Il Ok LEE ; Nan Suk KIM ; Suk Min YOON ; Sang Ho LIM ; Young Suk CHOI
Korean Journal of Anesthesiology 1990;23(6):979-983
Although it has been accepted that metoclopramide provides the effects of antiemetics, enhance- ment of intestinal motility and acts as antagonist on dopamine receptor, there is still controversy over inhibitory action of plasma cholinesterase. We examined the effect of preoperative intravenous administration of metoclopramide 10mg (group 2,4) on the duration of neuromuscular block induced by an intubating dose (1 mg/kg) of succinylcholine. Forty female adult patients were devided into four groups, scheduled for cesarean section (group 3,4) or other elective operations (group 1,2). The 1 H2single twitch height of the adductor pollicis muacle was recorded on paper to 1 H2single twitch stimulus by a nerve stimulator (Accelograph). The patients in group 2 and 4 received metoclopramide 10 mg i.v followed by succinylcholine 1mg/kg, and the patients in group 1 and 3 received succinylcholine 1mg/kg alone. The time from disappearance of 1 H, single twitch height to reappearance of that did not significantly differ between group l and 3. However, there was significant prolongation of the time from disappearance of 1H2single twitch height to reappearance of that in patients who received metoclopramide followed by succinylcholine in group 2 and 4. Also, we examined the effect of preoperative intravenous administration of metoclopramide 10mg on the duration of neuromuscular block by 0.5mg/kg of succinylcholine in twenty female patients for elective operations (group 5,6). There was no significant prolongation of the time from disappearance of 1 H, single twitch height to reappearance of that.
Administration, Intravenous
;
Adult
;
Antiemetics
;
Cesarean Section
;
Cholinesterases
;
Female
;
Gastrointestinal Motility
;
Humans
;
Metoclopramide*
;
Neuromuscular Blockade
;
Plasma
;
Pregnancy
;
Receptors, Dopamine
;
Succinylcholine*