3.A Case of Langerhans Cell Histiocytosis with Hypothalamic Mass.
Woe Sook YOON ; Ji Hae KANG ; Yoo Rah HONG ; Jae Sun PARK ; Bong Kwon CHUN ; Mi Hee JUNG ; Chang Woo MOON
Korean Journal of Pediatric Hematology-Oncology 2005;12(1):131-137
A 14-month-old girl presented with petechial skin lesions and polydipsia was diagnosed as Langerhans cell histiocytosis (LCH) and responded fairly well to multiple chemotherapies using vincristine, cyclophosphamide, and prednisone. 3 years later, relapses were more common with short periods of remissions in spite of using more intensive therapy with vinblatine and etoposide. At age of 4.5, sudden weight gain and abnormal behavior led to MRI study and revealed an hypothalamic mass. Radiation of 1, 800 cGy was given to the mass and followed by a 75% decrease in measuring and remission of the obesity. Although, there was no evidence of tumor progression in the hypothalamus, she died of sepsis due to systemic progression of the disease at age of 5. LCH commonly present with the symptoms of diabetes insipidus, but hypothalamic mass is not common. We report this case with a brief review of literatures.
Cyclophosphamide
;
Diabetes Insipidus
;
Drug Therapy
;
Etoposide
;
Female
;
Histiocytosis, Langerhans-Cell*
;
Humans
;
Hypothalamus
;
Infant
;
Magnetic Resonance Imaging
;
Obesity
;
Polydipsia
;
Prednisone
;
Recurrence
;
Sepsis
;
Skin
;
Vincristine
;
Weight Gain
4.Increased Activity of Insulin-like Growth Factor binding Protein-4 Protease in H-mole Patients.
Woo Seok SEO ; Dong Won BYUN ; Ji Oh MOK ; Ji Sung YOON ; Yeo Joo KIM ; Hyung Kyu PARK ; Chul Hee KIM ; Sang Jin KIM ; Kyo Il SUH ; Myung Hi YOO ; Hae Hyeog LEE ; Soo Kyoon RAH
Journal of Korean Society of Endocrinology 2004;19(4):346-357
BACKGROUND: Hydatidiform mole (H-mole) is characterized by the neoplastic proliferation of trophoblasts. Only 1~10% of patients with partial H-mole will develop a trophoblastic tumor, but 18~29% of those with complete H-mole will develop a persistent trophoblastic tumor. Therefore, the early diagnosis and monitoring after operation of an H-mole disease are very important. Recently, the pregnancy associated plasma protein-A (PAPP-A) was proved to have a similar role as that of IGF binding protein-4 (IGFBP-4) protease, which has shown an increasing function in fetal growth and development by degradation of IGFBP-4 and an increase in IGF in the serum during pregnancy. Our hypothesis is "the H-mole, which shows placental hyperplasia will also have an IGFBP-4 protease activity, which may be used as in the early diagnosis and monitoring of H-mole disease". METHODS: Serum samples from 6 non-pregnant, 18 pregnant (5 in the 1st trimester, 10 in the 2nd, and 3 in the 3rd), 12 postpartum women and 3 H-mole patients(2 with complete H-mole and 1with partial H-mole) were collected and measured for the -HCG, IGF and PAPP-A levels and IGFBP-4 protease activities by a IGF-II ligand blot analysis and electrophoresis method. The IGFBP-4 protease activity of the serum during normal pregnancy was compared with that of H-mole disease. RESULTS: The results from the in vitro protease assays using recombinant IGFBP-4 determined that IGFBP-4 proteolysis was significantly increased during the first (56%) and second trimesters (90%), but reached a plateau by the third trimester (94%). In H-mole disease diagnosed 11 weeks after conception, the IGFBP-4 proteolytic activity was 97%, which was nearly the same as at terminal pregnancy. This activity gradually decreased to 75% at 1 week, 58.7% at 2 and 33% at 3 weeks after the operation. The -HCG was also decreased from 490,400 to 123,822.7, 1,352.3, and 128.5 mIU/mL at 1, 2 and 3 weeks after the operation, respectively. The PAPP-A level also gradually decreased from 34.87 to 25.5, 12.0 and 2.7 g/mL 1, 2 and 3 weeks after the operation, respectively. However, the IGF decreased from 238.3 to 172.9 ng/mL 1 week after the operation, but increased to 251.4 and 295 ng/mL at 2 and 3 weeks after the operation, respectively. CONCLUSION: These results demonstrated that the IGFBP-4 protease activity was significantly increased during pregnancy, and was extremely elevated durimg the early stages of H-mole disease, but gradually decreased after removal of molar tissue. Therefore, measuring the IGFBP-4 protease activity may play an important role in the early diagnosis and monitoring of H-mole disease
Early Diagnosis
;
Electrophoresis
;
Female
;
Fertilization
;
Fetal Development
;
Humans
;
Hydatidiform Mole
;
Hyperplasia
;
Insulin-Like Growth Factor Binding Protein 4
;
Insulin-Like Growth Factor II
;
Molar
;
Plasma
;
Postpartum Period
;
Pregnancy
;
Pregnancy Trimester, Second
;
Pregnancy Trimester, Third
;
Pregnancy-Associated Plasma Protein-A
;
Proteolysis
;
Trophoblastic Neoplasms
;
Trophoblasts
5.Increased Activity of Insulin-like Growth Factor binding Protein-4 Protease in H-mole Patients.
Woo Seok SEO ; Dong Won BYUN ; Ji Oh MOK ; Ji Sung YOON ; Yeo Joo KIM ; Hyung Kyu PARK ; Chul Hee KIM ; Sang Jin KIM ; Kyo Il SUH ; Myung Hi YOO ; Hae Hyeog LEE ; Soo Kyoon RAH
Journal of Korean Society of Endocrinology 2004;19(4):346-357
BACKGROUND: Hydatidiform mole (H-mole) is characterized by the neoplastic proliferation of trophoblasts. Only 1~10% of patients with partial H-mole will develop a trophoblastic tumor, but 18~29% of those with complete H-mole will develop a persistent trophoblastic tumor. Therefore, the early diagnosis and monitoring after operation of an H-mole disease are very important. Recently, the pregnancy associated plasma protein-A (PAPP-A) was proved to have a similar role as that of IGF binding protein-4 (IGFBP-4) protease, which has shown an increasing function in fetal growth and development by degradation of IGFBP-4 and an increase in IGF in the serum during pregnancy. Our hypothesis is "the H-mole, which shows placental hyperplasia will also have an IGFBP-4 protease activity, which may be used as in the early diagnosis and monitoring of H-mole disease". METHODS: Serum samples from 6 non-pregnant, 18 pregnant (5 in the 1st trimester, 10 in the 2nd, and 3 in the 3rd), 12 postpartum women and 3 H-mole patients(2 with complete H-mole and 1with partial H-mole) were collected and measured for the -HCG, IGF and PAPP-A levels and IGFBP-4 protease activities by a IGF-II ligand blot analysis and electrophoresis method. The IGFBP-4 protease activity of the serum during normal pregnancy was compared with that of H-mole disease. RESULTS: The results from the in vitro protease assays using recombinant IGFBP-4 determined that IGFBP-4 proteolysis was significantly increased during the first (56%) and second trimesters (90%), but reached a plateau by the third trimester (94%). In H-mole disease diagnosed 11 weeks after conception, the IGFBP-4 proteolytic activity was 97%, which was nearly the same as at terminal pregnancy. This activity gradually decreased to 75% at 1 week, 58.7% at 2 and 33% at 3 weeks after the operation. The -HCG was also decreased from 490,400 to 123,822.7, 1,352.3, and 128.5 mIU/mL at 1, 2 and 3 weeks after the operation, respectively. The PAPP-A level also gradually decreased from 34.87 to 25.5, 12.0 and 2.7 g/mL 1, 2 and 3 weeks after the operation, respectively. However, the IGF decreased from 238.3 to 172.9 ng/mL 1 week after the operation, but increased to 251.4 and 295 ng/mL at 2 and 3 weeks after the operation, respectively. CONCLUSION: These results demonstrated that the IGFBP-4 protease activity was significantly increased during pregnancy, and was extremely elevated durimg the early stages of H-mole disease, but gradually decreased after removal of molar tissue. Therefore, measuring the IGFBP-4 protease activity may play an important role in the early diagnosis and monitoring of H-mole disease
Early Diagnosis
;
Electrophoresis
;
Female
;
Fertilization
;
Fetal Development
;
Humans
;
Hydatidiform Mole
;
Hyperplasia
;
Insulin-Like Growth Factor Binding Protein 4
;
Insulin-Like Growth Factor II
;
Molar
;
Plasma
;
Postpartum Period
;
Pregnancy
;
Pregnancy Trimester, Second
;
Pregnancy Trimester, Third
;
Pregnancy-Associated Plasma Protein-A
;
Proteolysis
;
Trophoblastic Neoplasms
;
Trophoblasts
6.Deep Venous Thrombosis and Heterotopic Ossification in the Patients with Traumatic Spinal Cord Injury.
Ueon Woo RAH ; Hwa Sook KIM ; Hae Won MOON ; Il Young LEE ; Jae Ho EOM ; Jong Bin LEE
Journal of the Korean Academy of Rehabilitation Medicine 2003;27(3):349-354
OBJECTIVE: To investigate the incidence, time of onset, and risk factors of deep vein thrombosis asssociated with heterotopic ossification in patients with spinal cord injury. METHOD: The medical records of 201 patients with spinal cord injury were reviewed. Duplex ultrasound and/or venography were used for the diagnosis of deep vein thrombosis and 3 phase bone scan and/or plain radiologic studies were used for the diagnosis of heterotopic ossification. RESULTS: Whereas the incidence of heterotopic ossification and deep vein thrombosis in this population were 10.0% and 4.5%, respectively, 55.5% of the individuals with deep vein thrombosis developed heterotopic ossification. The overall incidence of coexistence of deep vein thrombosis and heterotopic ossification was 2.5%. The significant difference between the occurrence of heterotopic ossification and deep vein thrombosis in this SCI population reached statistical significance (Fisher's exact test p<0.005). CONCLUSION: The results of this study suggest that there exists an association between the occurrence of deep vein thrombosis and heterotopic ossification following SCI.
Diagnosis
;
Humans
;
Incidence
;
Medical Records
;
Ossification, Heterotopic*
;
Phlebography
;
Risk Factors
;
Spinal Cord Injuries*
;
Spinal Cord*
;
Ultrasonography
;
Venous Thrombosis*
7.The Effect of Body Mass Index on Nerve Conduction Studies.
Kyong Seok RHIE ; Il Yung LEE ; Ueon Woo RAH ; Hae Won MOON ; Kyong Mi KIM
Journal of the Korean Academy of Rehabilitation Medicine 2002;26(3):316-320
OBJECTIVE: To determine whether there is a difference in nerve conduction studies depend on the body mass index (BMI) of subjects METHOD: Twenty normal healthy volunteers were enrolled for the study. A routine usual sensory and motor nerve conduction study and a sensory nerve conduction study using the near nerve needle technique were performed. BMI was calculated as weight (kg) divided by height (m) squared. In order to evaluate the effect of BMI on the various measure ments of the nerve conduction study, one-way analysis of variance (ANOVA) was used. RESULTS: The sensory nerve amplitudes of median, ulnar and sural nerves correlated significantly (p<0.05) with BMI. However, no correlation was noted between BMI and sensory nerve amplitude by near nerve needle technique. There was no statistical differences noted in the measurements of latency of examined motor and sensory nerves neither the velocity of examined motor nerves. CONCLUSION: In clinical practice, the effect of BMI should be taken into account when the interpretation of abnormal sensory nerve study has to be soli.
Body Mass Index*
;
Healthy Volunteers
;
Needles
;
Neural Conduction*
;
Obesity
;
Sural Nerve
;
Weights and Measures
8.Effects of Psyllium Husk for the Management of Neurogenic Bowel in Chronic Spinal Cord Injured Persons.
Kyong Mi KIM ; Ueon Woo RAH ; Il Yung LEE ; Hae Won MOON ; Seon Hee IM ; Kyong Seok RHIE
Journal of the Korean Academy of Rehabilitation Medicine 2002;26(5):533-538
OBJECTIVE: The purposes were to determine the baseline colon transit time (CTT) and to assess the effect of dietary fiber (psyllium husk) on neurogenic bowel function in chronic spinal cord injured (SCI) persons. METHOD: Eleven chronic spinal cord injured persons with upper motor neuron type neurogenic bowel were participated. Personal interview were carried out for all studied subjects at pre and post treatment period. The baseline colon transit time (CTT) were measured for the right (rCTT), left (lCTT), rectosigmoid (rsCTT) colons as well as for the entire colon using radio-opaque markers. After 4 weeks treatment of psyllium husk, the subjects were reevaluated for their CTTs and the results were compared to the pretreatment values. RESULTS: The mean age of the subjects was 33.9+/-11.0 years and the level of injury ranged from C3 to T10. The mean duration after SCI was 22.6 months (6~47 months). The rCTT, lCTT, rsCTT and tCTT were not affected after the treatment of psyllium husk. Also their bowel care patterns and satisfaction were unaffected by the treatment. CONCLUSION: The results of this study suggest that the use of psyllium husk in chronic spinal cord injured persons do not show the same effect on bowel function as has been previously reported in general population with idiopathic constipation.
Colon
;
Constipation
;
Dietary Fiber
;
Humans
;
Motor Neurons
;
Neurogenic Bowel*
;
Psyllium*
;
Spinal Cord Injuries
;
Spinal Cord*
9.Suprascapular Nerve Injury Restricted to the Infraspinatus Muscle by a Ganglion Cyst: A case report.
Dae Seop SHIM ; Ueon Woo RAH ; Hae Won MOON ; Il Yung LEE ; Jae Ho EOM
Journal of the Korean Academy of Rehabilitation Medicine 2002;26(6):819-821
Suprascapular nerve injury restricted to the infraspinatus muscle is an uncommon condition. We present a 29 year-old man who complained of right shoulder pain and weakness for 8 months. Physical examination revealed atrophy of right infraspinatus muscle and T2 weighted MRI of the right shoulder revealed a mass with homogeneous high intensity at the spinoglenoid notch and atrophy of right infraspinatus muscle. EMG study revealed abnormal spontaneous activities and reduced recruitment pattern of motor units in right infraspinatus muscle. Symptoms improved after the excision of a mass which was a ganglion cyst by pathologic examination. We were reporting a case of suprascapular nerve injury restricted to the infraspinatus muscle.
Adult
;
Atrophy
;
Ganglion Cysts*
;
Humans
;
Magnetic Resonance Imaging
;
Physical Examination
;
Shoulder
;
Shoulder Pain
10.Clinical diagnostic analysis of 28 cases of malaria diagnosed in Pusan.
Seung Hwan PARK ; Jong Sik HWA ; Ho Rim RAH ; Hae Woong CHUNG
Journal of the Korean Academy of Family Medicine 2001;22(9):1409-1416
BACKGROUND: Pusan is geographically far away from the indigenous area of malaria. It is the area where the tourists and foreigners travel through frequently because of the port. We investigated the situation in Pusan concerning malaria through the examination of malaria patients who contracted it recently. We found the ways to decrease erroneous diagnosis presuming malaria on the basis of these studies. METHODS: We retrospectively analyzed the medical records of 28 confirmed cases of malaria with the chief complaint of repeated high fever, who were admitted and treated in the Pusan Adventist Hospital and Pusan Samsun Hospital from June 1997 to August 2000. RESULTS: 25 cases were indigenous and 3 patients were imported cases contracted overseas. Peripheral blood smears revealed Plasmodium vivax in all indigenous cases, whereas 2 were P. falciparum and 1 case was P. vivax in the imported cases. Yeonchon-goon(9 cases) was the most prevalent area in the indigenous cases. Africa and Southeast Asia(3 cases) were the contracted areas in the imported cases. 27 cases were men and 1 case was a woman among the 28 cases, and the incidence was high peak in 8-10 month. Most of the patients(72.1%) were in the their 20's, and most of all had worked in the military bases near the demilitarized zone(DMZ). Thrombocytopenia and leukopenia were found more frequently by the laboratory findings. 23 of the cases(82%) were suspected to be malaria, before we confirmed it by peripheral blood smears. CONCLUSION: The number of patients with indigenous malaria from the other areas in Korea and patients with malaria from foreign countries will be expected to increase. Before we confirmed the malaria by peripheral blood smears, the cases suspected of malaria were 82.1%. This is a high diagnostic rate due to careful history taking and physical examination. The doctors decreased the rate of erroneous diagnosis of malaria and should be careful in the choice of the proper drugs.
Africa
;
Busan*
;
Diagnosis
;
Emigrants and Immigrants
;
Female
;
Fever
;
Humans
;
Incidence
;
Korea
;
Leukopenia
;
Malaria*
;
Male
;
Medical Records
;
Military Facilities
;
Physical Examination
;
Plasmodium vivax
;
Retrospective Studies
;
Thrombocytopenia
Result Analysis
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