1.Factors affecting Final Adult Height in Turner Syndrome.
Min Ho JUNG ; Jin Soon HWANG ; Eun Young KIM ; Sei Won YANG
Journal of Korean Society of Pediatric Endocrinology 1999;4(1):64-70
Purpose : Shortness is the most frequent and quite disturbing characteristics of patients with Turner syndrome. The aim of this study was to evaluate the factors affecting final adult height(FAH) in these patients. METHODS : The study group was comprised of 19 patients who were diagnosed as Turner syndrome and attained FAH. We analyzed the influences of various factors on FAH in GH treated group with those in GH untreated group. Results : Nineteen patients were enrolled; thirteen received GH treatment and six did not. The mean duration of GH treatment was 24.3 months(range : 9 to 50 months), and the mean dosage of GH was 0.98+/-0.35IU/kg/wk in GH treated group. The mean growth velocity during GH treatment was 5.6+/-1.8 cm/yr, which was significantly higher than that during pretreatment period(P<0.05). In GH treated group, the mean chronological age, bone age, mean height, and height SD score at GH therapy were 13.7+/-1.7yr, 11.3+/-1.9yr, 129.7+/-7.9cm, and -4.1+/-1.1, respectively, which were not statistically different from those at diagnosis of GH untreated group. In GH treated group, the mean FAH and FAH SD score were 144.8+/-5.0cm, and -3.2+/-0.9, respectively, which showed no significant difference compared with those of GH untreated group. Analyzing the factor affecting FAH in all Turner girls of both groups together, parental height, chronological age, bone age, and bone age delay at diagnosis(or at the initiation of GH therapy) were not related to FAH. Height and height SD score at diagnosis(or at the initiation of therapy) were positively related to FAH(P<0.05, r=0.72). CONCLUSION : The results suggest that GH treatment dose not improve FAH in patients with Turner syndrome, despite increased growth velocity during GH treatment, which might come from intermittern GH therapy. This should be remained to be clarified with more Turner patients who attained FAH.
Adult*
;
Diagnosis
;
Female
;
Growth Hormone
;
Humans
;
Parents
;
Turner Syndrome*
2.A Case of Vascular Leak Syndrome During Induction Chemotherapy of Acute Lymphoblastic Leukemia (T Cell Type) .
Kye Wool KANG ; Hwang Min KIM ; Won Kyu CHOI ; Baek Keun LIM ; Chang Hun LEE
Journal of the Korean Pediatric Society 2001;44(9):1041-1046
Vascular leak syndrome(VLS) is characterized by endothelial damage, which causes extravasation of plasma proteins and fluid from capillaries into the extravascular space. It has been suggested that the increased vascular permeability is the result of an IL-2-induced suppression of endothelin-1 secretion by endothelial cells, an IL-2-induced activation of the complement cascade or TNF-alpha release from IL-2-activated T-cells. A 13-year-old male patient visited our hospital with abdominal pain. He was diagnosed with acute lymphoblastic leukemia(T cell type) by bone marrow study on his 2 nd day in hospital. On the 3 rd day of induction chemotherapy(prednisone, L-asparaginase, vincristine, intrathecal methotrexate), pulmonary edema and pleural effusion, ascites and generalized edema developed and lasted for 53 days without responding to supportive care. The laboratoy finding was that TNF-alpha was increased without evidence of infection and hypoalbuminemia was noted. It was suggested that the patient's clinical feature was induced by VLS.
Abdominal Pain
;
Adolescent
;
Ascites
;
Blood Proteins
;
Bone Marrow
;
Capillaries
;
Capillary Permeability
;
Complement System Proteins
;
Edema
;
Endothelial Cells
;
Endothelin-1
;
Humans
;
Hypoalbuminemia
;
Induction Chemotherapy*
;
Male
;
Pleural Effusion
;
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
;
Pulmonary Edema
;
T-Lymphocytes
;
Tumor Necrosis Factor-alpha
;
Vincristine
3.Von Recklinghausen' s Disease with Plexiform Neurofibroma , Giant Pigmentation , and Skeletal Abnormalities.
Sang Min HWANG ; Sung Ku AHN ; Beom Joo LEE ; Won Soo LEE ; Eung Ho CHOI
Korean Journal of Dermatology 1995;33(6):1179-1183
Plexiform neurofibroma is considered a pathognomic of Von Recklinghousen's disease, which involves the deep and large nerve trunk. These are large irregular nerve fascicles which result from an increase in endoneural matrix within individual nerve facicles, without an increased number of nerve fibers. We experenced a case of Von Recklinghausen's disease in a 24 year-old male who had variable cutaneous skeletal, and CNS lesions. He presented multiple neurofibromas, cafe-au-lait spots, and axillary freckles as common cutaneous lesions of NF-I and giant pigmentation, sacral hypertrichosis, and plexiform neurofibroma as unusual cutaneous lesions. Also he had a scoliosis, bowing deformity of the humerous and wedging deformity of the body of the 5th cervical spine as a skeletal manifestation and cortical calcification in the occipital area as a CNS manifestation.
Cafe-au-Lait Spots
;
Congenital Abnormalities
;
Humans
;
Hypertrichosis
;
Male
;
Melanosis
;
Nerve Fibers
;
Neurofibroma, Plexiform*
;
Neurofibromatoses
;
Neurofibromatosis 1
;
Pigmentation*
;
Scoliosis
;
Spine
;
Young Adult
4.A Case of Miescher Syndrome with Insulin-Resistant Diabetes Mellitus.
Byung Min CHOI ; Jong Kwang LEE ; Kee Hwang YOO ; Joo Won LEE ; Soon Kyum KIM
Journal of the Korean Pediatric Society 1994;37(9):1292-1295
Miescher syndrome comprises congenital acanthosis nigricans, hypertrichosis, failure to thrive and short stature, dysmorphism especially of the jaws and oral cavity, insulin-resistant diabetes mellitus, and a characteristic general appearance. This report concerns a rare case of 12-year-old girl having insulin resistant diabetic mellitus with Miescher syndrome. The relevant literature was reviewed.
Acanthosis Nigricans
;
Child
;
Diabetes Mellitus*
;
Failure to Thrive
;
Female
;
Humans
;
Hypertrichosis
;
Insulin
;
Jaw
;
Mouth
5.Two cases of virus associated hemophagocytic syndrome.
Jae Hee HAN ; Hyun Chul LEE ; Hwang Min KIM ; Jong Soo KIM ; Kyung Won LEE
Journal of the Korean Pediatric Society 1993;36(10):1458-1465
Virus associated hemophagocytic syndrome, class ll histiocytoses, characterized by high fever, severe constitutional symptoms, abnormal liver function and coagulation, perigheral blood pancytopenia and histiocytic hyperplasis with prominent hemophagocytosis in bone marrow and lymph nodes has been reported and associated with active viral infection. It is non-malignant and reversible. It must be differentiated from histiocytic medullary reticulosis because of the inappopriateness of immunosuppressive of cytotozic therapy which is the therapeutic method for HMR, but is contraindicated in the treatment of VAHS. This paper describes two patients whose clinicopathology was compatible with the diagnosis of virus associated hemophagocytic syndrome.
Bone Marrow
;
Diagnosis
;
Fever
;
Histiocytosis
;
Humans
;
Liver
;
Lymph Nodes
;
Lymphohistiocytosis, Hemophagocytic*
;
Pancytopenia
6.The Effects of Nutritional Education Program on Nausea and Vomiting, Anorexia, Food Intake, and Nutritional Status of GI Cancer Patients Undergoing Chemotherapy.
Journal of Korean Oncology Nursing 2010;10(1):38-47
PURPOSE: The purpose of this study was to identify the effects of nutritional education program (NEP) on nausea and vomiting, anorexia, food intake, and nutritional status among GI cancer patients undergoing chemotherapy. METHODS: A nonequivalent, non-synchronized posttest design was used. Study subjects were conveniently selected 30 patients (15 subjects for each group) who were undergoing chemotherapy. The subjects of the experimental group received NEP which consisted of 2 educations and 1 counselling while they were hospitalized, and telephone counselling with nutritional supports after discharge for four times. RESULTS: The score of anorexia was lower and the amount of food intake was higher in the experimental group than those of the control group. However, there were no significant differences in levels of nausea and vomiting, weight, hemoglobin, albumin, and total protein level between the experimental and the control group. CONCLUSION: The findings demonstrate that the NEP was effective in alleviating anorexia and improving subjective food intake of GI cancer patients undergoing chemotherapy. These findings suggest that NEP can be a useful nursing intervention for preventing nutritional disorders for patients with GI cancer after chemotherapy.
Anorexia
;
Child
;
Eating
;
Hemoglobins
;
Humans
;
Nausea
;
Nutrition Disorders
;
Nutritional Status
;
Nutritional Support
;
Telephone
;
Vomiting
7.A case of transient diabetes mellitus and diabetic ketoacidosis induced by L-asparaginase and prednisolone administration in a patient with relapsed acute lymphocytic leukemia.
Won Kyu CHOI ; Mee Kyung NAMGOONG ; Har Yong LEE ; Hwang Min KIM ; Jong Soo KIM
Journal of the Korean Pediatric Society 1991;34(9):1316-1322
No abstract available.
Diabetes Mellitus*
;
Diabetic Ketoacidosis*
;
Humans
;
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
;
Prednisolone*
8.A Mitochondrial Mutation in Leber's Hereditary Optic Neuropathy.
Jeong Min HWANG ; Hye Won PARK
Journal of the Korean Ophthalmological Society 1995;36(12):2218-2224
Leber's hereditary optic neuropathy is caused by a single nucleotide change in the mitochondrial deoxynucleic acid(mtDNA). We identified a single guanine to adenine transition mutation in the mitochondrial DNA at nucleotide position 11778(Wallace mutation)in a 13 year old boy. To our knowldge, this is the first report confirming mtDNA mutation in Korea. This would be very helpful for the correct diagnosis of optic neuritis, optic neuropathy and optic atrophy of unknown etiology as well as for genetic counselling in the future.
Adenine
;
Adolescent
;
Diagnosis
;
DNA, Mitochondrial
;
Guanine
;
Humans
;
Korea
;
Male
;
Optic Atrophy
;
Optic Atrophy, Hereditary, Leber*
;
Optic Nerve Diseases
;
Optic Neuritis
9.A Clinical Study of Rosacea.
Tae Hyun KIM ; Sang Min HWANG ; Won Soo LEE ; Sung Ku AHN ; Eung Ho CHOI
Korean Journal of Dermatology 2000;38(5):583-588
No Abstract Available.
Rosacea*
10.A Case of Pruritic Folliculitis of Pregnancy.
Sang Min HWANG ; Eung Ho CHOI ; Won Soo LEE ; Sung Ku AHN
Korean Journal of Dermatology 1998;36(1):156-159
Pruritic folliculitis of pregnancy of pregnancy is a specific dermatoses of pregnancy which is characterized by erythematous follicular papules and pustules between the fourth and ninth month of pregnancy. It has usually resolved by 4 weeks of postpartum and has no adverse implications for mother and baby. We report a case of pruritic folliculitis of pregnancy in a 24-year-old primigravida woman who had had monomorphic erythematous follicular papules and pustules on her anterior chest wall and back. Histopathological findings of erythematous papules showed acute folliculitis and perifolliculitis. The skin lesions improved rapidly with 2 weeks of delivery without treatment.
Female
;
Folliculitis*
;
Humans
;
Mothers
;
Postpartum Period
;
Pregnancy*
;
Skin
;
Skin Diseases
;
Thoracic Wall
;
Young Adult