Arnold-Chirai malformation type II is congenital disorder which consists of downward displacement into upper cervical spinal canal of parts of the cerebellum, 4th ventricle, and medulla oblongata. We experienced two cases of Arnold-Chiari malformation with lumbar meningomyelocele, hydrocephalus. We confirmed the cases by brain C-T and report with brieft review of the lilterature.
Arnold-Chiari Malformation* ; Brain ; Cerebellum ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Hydrocephalus ; Medulla Oblongata ; Meningomyelocele ; Spinal Canal

The Dandy-Walker syndrome is a developmental disorders of the brain characterized by cystic deformity of the 4th ventricle and agensis of the cerebellar vermis. Other systemic anomalies and chromosomal abnormalities are associated with this syndrome. We are experienced a case in a 9 months old male infant who presented initially with frequent vomiting, low birth weight, On the physical examination, a prominent occiput, palpable mass below the right upper quadrant, pulmonary valve stenosis, congenital dislocation of the hips, ventral flexion of fingers, clubfoots and the rocker-bottom deformities of feet were present. On the chromosomal study, there were chromosomal polymorphisms in a thickened C-band of chromosome No. 1 by C-banding method. The brain CT revealed a large, thin-walled, low density mass of CSF without enhancement in the posterior fossa, showing upward displacement of cerebellar hemisphere with absent inferior vermis(or associated with dysplastic cerebellar hemisphere). A brief review of the related literatures were included in this report.
Brain ; Chromosome Aberrations* ; Clubfoot ; Congenital Abnormalities ; Dandy-Walker Syndrome* ; Dislocations ; Fingers ; Foot ; Hip ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Male ; Physical Examination ; Pulmonary Valve Stenosis ; Vomiting

Teramethylammonium(TMA) in one of the synthetic compounds of nicotine that act at ganglionic site. The major action of TMA consists of initial stimulation followed by a more persistent depression of all autonomic ganglia by binding to a cholinergic receptor. It is well believed that the level of membrane potential in arterial smooth muscle is an important regulator of tension development. Depolarization and hyperpolarization by only few millivolts results in significant changes in tension. In general, the agents of vascular smooth muscle induce vascular relaxaion. The present study was undertaken to elucidate the effect of TMA on vascular contractility in the isolated rabbit thoracic aorta with or without endothelial cell, and mechanisms involved in the change of vascular contractility by TMA. The results obtained are summarized as follows ; 1) In the presence of endothelial cell, TMA induced a relaxtion of the aorta precontracted with norepinephrine but induced a contraction in the aorta without endothelial cells, indicating that in the rabbit aorta, relaxations produced by TMA were the endothelium-dependent. 2) The addition of inhibitor such as methylene blue, hemoglobin, hydroquinone and p-bromophenacyl bromide during the TMA-induced relaxation reversed the contractile tension to a level similar to or higher than that before the addition of TMA in rabbit thoracic aorta.This relaxation effect of TMA suggest that the TMA-inducdd relaxation in rabbit aorta is due to the release of endotheline derived relaxing factor(EDRF). 3) Relaxation induced by TMA was antagonized by atropine and thus the TMA does seem to act on the muscarinic receptors. 4) TMA reduced the norepinephrine-induced Ca++ influx into rabbit smooth muscle membrane. From the above results, it may be concluded that TMA-induced vacular relaxation in rabbit aorta is due to the release of EDRF. Mechanism involved in the relaxation induced by TMA may be the stimulation of soluble guanylate cyclase and increased tissue cGMP concentrations.
Aorta ; Aorta, Thoracic* ; Atropine ; Depression ; Endothelial Cells ; Endothelins ; Endothelium ; Endothelium-Dependent Relaxing Factors* ; Ganglia, Autonomic ; Ganglion Cysts ; Guanylate Cyclase ; Membrane Potentials ; Membranes ; Methylene Blue ; Muscle, Smooth ; Muscle, Smooth, Vascular ; Nicotine ; Norepinephrine ; Receptors, Muscarinic ; Relaxation

Fractures of the humeral neck are relatively common, but their anatomical complexities make closed reduction difficult sometimes, and in case of reduction it is tough to maintain the position. Even open reduction of the fracture also faces multiple problems including poor functional recovery of the shoulder joint. The Department of Orthopedic Surgery of Seoul Adventist Hospital developed a device of external fixation for 13 humeral neck fractures. We used this method for displaced and unstable fractures of humeral neck from December 1987 to March 1989. Satisfactory results were obtained in 11 cases and poor results in 2 cases. We think our method is a effective useful way of treatment for humeral neck fractures, and its main advantages are as follows:1. Using proximal and distal bars, fracture can be easily reduced and firmly maintained. 2. Active motion exercise can be started early and functional recovery of the shoulder joint can be highly predictable. 3. It is quite convenient for treating combined fracture or external wound of same extremity.
Extremities ; Methods ; Neck ; Orthopedics ; Seoul ; Shoulder Joint ; Wounds and Injuries

Urinary protein per se causes renal tubular injury and stimulates immunologic reaction. The extent of proximal tubular injury can be estimated by measuring the amount of 24 hours urinary beta2-microglobulin (U beta2-MG). The aim of this study was whether U beta2-MG level could predict the response to the initial steroid treatment for the patients with minimal change nephrotic syndrome (MCNS). We analyzed 58 adult patients (33 M, 25 F), aged 33+/-15 years (range 16-76), with biopsy-proven MCNS treated with 40 to 60mg of oral prednisolone daily up to 16 weeks. The responsiveness (44 cases) inculded complete and partial remission or steroid resistance (14 cases). No difference was found between the steroid responsive and resistant group with regard to age, BUN, serum creatinine, albumin, cholesterol, and urinary protein. The levels of U beta2- MG (microgram/g creatinine) were 250808+/-478917 and 1648+/-2386 in resistan ia Kwang-Yul Chang, et al.:Prediction of Steroid Responsiveness in Adult Minimal Change Nephrotic. Syndrome Using Urinary beta2-Microglobulint group and responsive group, respectively (P<0.05). The cut off value was 400microgram/ g creatinine with 78% of sensitivity and 48% of specificity. The likelihood ratio for the resistance was 2.5 to 2.8 with the U beta2-MG levels over 400 ug/g creatinine and was 0.2 to 0.3 below that value. Multivariate analysis confirmed that higher U beta2- MG level was associated with a lower likelihood of steroid responsiveness, independent of age, sex, creatinine, serum protein, and urinary protein. This study showed that the pretreatment U beta2- MG level may be used to identify subgroup of patients with MCNS who are more likely to be responsive to initial steroid treatment.
Adult* ; Cholesterol ; Creatinine ; Humans ; Multivariate Analysis ; Nephrosis, Lipoid* ; Prednisolone ; Sensitivity and Specificity

Cryosupernatant which is the residual plasma fraction after removing cryoprecipitate has been used for plasma exchange in thrombotic thrombocytopenic purpura and hemolytic uremic syndrome replacing the fresh frozen plasma. Recently, the unusually large yon Willebrand factor multireefs (ULvWFM) has been observed in patients with refractory or chronic relapsing hemolytic uremic syndrome as well as thrombotic thrombocytopenic purpura which disappeared by infusion or plasma exchange with cryosupernatant, and infusion of fresh frozen plasma, the largest multimers of yon Willebrand factor were replenished that it might be a cause of refractoriness. This study was conducted to investigate the characteristics of home-made cryosupernatant from thawed fresh frozen plasma and its thehrapeutic effect in a hemolytic uremic syndrome patient. The level of fibrinogen, coagulation factor VIII, vWF antigen, and ristocetin cofactor activity was decreased and yon Willebrand factor multimers were barely seen in cryosupernatant than those of in fresh frozen plasma. A hemolytic uremic syndrome patient tried with exchange and infusion of cryosupernatant showed excellent recovery. It is concluded that home-made cryosupernatant shares many of the features of fresh frozen plasma except factor VIII, especially von Willebrand factor multimers, and thus it could be a useful alternative to fresh frozen plasma in case of refractory hemolytic uremic synydrome.
Factor VIII ; Fibrinogen ; Hemolytic-Uremic Syndrome ; Humans ; Plasma Exchange* ; Plasma* ; Purpura, Thrombotic Thrombocytopenic ; von Willebrand Factor

PURPOSE: To analyze treatment outcome of curly toe and to suggest appropriate treatment modality according to the degree of deformity. MATERIALS AND METHODS: 26 patients with 37 curly toes were subject to study. The mean age was 20 months (range 6 to 42 months), and one neglected case aged 28 years. Grade 1 were treated with strapping. Grade 2 and 3 were treated with open flexor tenotomy. The degree of deformity was analyzed by utilizing grade which Hamer and others once used previously, and the condition of pre- and post-treatment was evaluated. RESULTS: Of 18 cases with preoperative condition grade 1, all cases had favourable results which showed 16 cases remaining in grade 1 and 2 cases becoming almost normal. The average grade was 0.88 at the last follow-up. Of 19 cases with preoperative condition grade 2 or grade 3, 5 cases had remained mild rotational deformity with grade 1, and 14 cases were recovered to normal. The average grade was 2.57 preoperatively and 0.26 postoperatively. CONCLUSIONS: It is very important to assess the degree of deformity before deciding treatment method for curly toe. In case of grade 2 or grade 3, an open flexor tenotomy has proven to be effective treatment. Surgical treatment may provide favorable results regardless of age, whenever it is performed in case with persistent severe deformity.
Congenital Abnormalities* ; Follow-Up Studies ; Humans ; Tenotomy ; Toes* ; Treatment Outcome

Background: Periodontitis is reported to be associated with preterm birth (spontaneous preterm labor and birth). Gastroesophageal reflux disease (GERD) is common during pregnancy and is expected to be related to periodontitis. However, little research has been done on the association among preterm birth, GERD and periodontitis. This study uses popular machine learning methods for analyzing preterm birth, GERD and periodontitis. Methods: Data came from Anam Hospital in Seoul, Korea, with 731 obstetric patients during January 5, 1995 - August 28, 2018. Six machine learning methods were applied and compared for the prediction of preterm birth. Variable importance, the effect of a variable on model performance, was used for identifying major determinants of preterm birth. Results: In terms of accuracy, the random forest (0.8681) was similar with logistic regression (0.8736). Based on variable importance from the random forest, major determinants of preterm birth are delivery and pregestational body mass indexes (BMI) (0.1426 and 0.1215), age (0.1211), parity (0.0868), predelivery systolic and diastolic blood pressure (0.0809 and 0.0763), twin (0.0476), education (0.0332) as well as infant sex (0.0331), prior preterm birth (0.0290), progesterone medication history (0.0279), upper gastrointestinal tract symptom (0.0274), GERD (0.0242), Helicobacter pylori (0.0151), region (0.0139), calcium-channel-blocker medication history (0.0135) and gestational diabetes mellitus (0.0130). Periodontitis ranked 22nd (0.0084). Conclusion GERD is more important than periodontitis for predicting and preventing preterm birth. For preventing preterm birth, preventive measures for hypertension, GERD and diabetes mellitus would be needed alongside the promotion of effective BMI management and appropriate progesterone and calcium-channel-blocker medications.

No abstract available.
Azithromycin* ; Pregnancy* ; Scrub Typhus*

BACKGROUND: Idiopathic acute transverse myelitis (ATM) is an inflammatory or demyelinating illness of unknown etiology. It is usually monophasic, though recurrences at the same spinal level of the initial attack have been reported. In Korea, the spinal cord is the most commonly affected site of multiple sclerosis (MS) and its clinical features often resemble those of ATM. As the prognoses of these two conditions are different, it is important to distinguish them from each other. The purpose of this study was to evaluate spinal MRI findings that could help differentiate ATM from myelopathic MS. METHODS: A total of 50 MRIs obtained less than four weeks after the onset of symptoms in 13 patients with ATM and 24 patients with clinically definite MS were reviewed retrospectively. The diagnostic criterion for ATM was a rapid onset of spinal motor and sensory dysfunction referable to a distinct spinal cord level with sphincter dysfunction, reaching its peak within 4 weeks, in the absence of pre-existing neurological diseases and spinal cord compression. For MS, Poser's criteria were used. We analyzed the lesion length, location, cross-sectional area, cord morphology, and contrast enhancement patterns in each of the groups. RESULTS: Characteristic MRI findings of ATM included; 1) centrally located hyperintensity (86%) occupying more than half of the cross-sectional area of the cord (100%), 2) thoracic preponderance in lesion location (thoracic: 7, cervicothoracic:3, cervical: 1), 3) lesion length of more than 3 vertebral segments (100%), 4) cord swelling (73%), and 5) focal or diffuse enhancement (90%). Most MRI findings of myelopathic MS (MMS) were not different from those of ATM, especially in Asian-type MS. Different MRI features of MMS from those of ATM included: 1) peripherally located hyperintensity occupying less than half of the cross-sectional area of the cord (20.8%), 2) lesion length less than two vertebral segments (33.3%), 3) normal cord morphology (25%), 4) no enhancement (36.1%), and 5) multifocality (22.2%), which were more commonly found in Western-type MS. CONCLUSIONS: Although there were some differences of spinal MRI features between ATM and MMS, it was not easy to differentiate them by MRI findings alone. The findings suggest that Asian-type MS has unique MRI characteristics similar to those of ATM and different from those of Western-type MS.
Humans ; Korea ; Magnetic Resonance Imaging* ; Multiple Sclerosis* ; Myelitis, Transverse* ; Prognosis ; Recurrence ; Retrospective Studies ; Spinal Cord ; Spinal Cord Compression