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

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

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

PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of infancy(PHHI). METHODS: Medical records of 10 patients(9 males and 1 female, mean age:40.4+/-1.5 months) who were diagnosed as PHHI and underwent pancreatectomy from 1988 to 2000 were reviewed. Clincal and biochemical data were recorded. Subjects were classified arbitrarily into early-onset or late-onset group according to age of onset. Pathologic appearance of pancreas was divided into 2 forms:diffuse or focal. The former had a focal pancreatic adenomatous hyperplasia and the latter was characterized by increased number of betacells with similar distribution seen in normal neonates. RESULTS: One patient had focal, and nine had diffuse lesions. After near-total pancreatectomy, 4 patients(40.0%) showed complete response, 4(40.0%) had persistent hypoglycemia, and 2(20.0%) developed diabetes mellitus. As neurological sequelae, 6 patients(60.0%) had persistent seizures, and 6(60.0%) had delayed motor and speech development. No clinical or biochemical factors related to postoperative outcome were found. CONCLUSION: This data indicate that early diagnosis of patients who present with hypoglycemic symptoms in infancy, especially early in life, and development of more effective therapy are warranted, because there is no clinical or biochemical factor predicting final outcome after near-total pancreatectomy and only 40% of patients with PHHI remained euglycemic after surgery with possible severe neurological sequelae.
Age of Onset ; Congenital Hyperinsulinism* ; Diabetes Mellitus ; Early Diagnosis ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Infant, Newborn ; Male ; Medical Records ; Pancreas ; Pancreatectomy* ; Seizures

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

PURPOSE: The aim for this study was to investigate clinical manifestation of seasonal influenza A and B during the 2012 winter season in Wonju, South Korea. Their clinical and laboratorial characteristics and effect of oseltamivir were compared and analyzed. METHODS: Children under the age of 18 years who visited the Wonju Severance Christian Hospital with fever or acute respiratory symptoms and who were diagnosed with influenza A or B by rapid antigen test from nasopharyngeal swab were selected for the study. The medical records of patients were retrospectively reviewed. RESULTS: Influenza A was detected in 374 patients (83.7%), and influenza B in 72 (16.6%). The incidence of influenza A was highest in February (n=186), while that of influenza B was highest in March (n=36). The most common symptoms were fever (n=434, 97.1%) and cough (n=362, 81.0%). No significant differences were observed between influenza A and B in symptoms and laboratory data. Patients who had used oseltamivir within 2 days showed statistically lower admission rate, shorter admission duration, and lower incidence of pneumonia. CONCLUSION: This study found no statistical difference between influenza A and B, in symptoms, progression, and laboratory test, but those who were treated with oseltamivir given within 2 days of the onset of fever experienced more positive outcomes.
Child ; Cough ; Fever ; Gangwon-do ; Humans ; Incidence ; Influenza, Human* ; Korea ; Medical Records ; Oseltamivir ; Pneumonia ; Retrospective Studies ; Seasons*

Sacral pressure sores have been treated by a variety of surgical methods. complete treatment needs wide excision and coverage with healthy tissue which has constant and sufficient blood supply. Use of gluteus maximus muscle flap with or without overlying skin is a revolutionary method because of the reliability of blood supply. However, it is technically a little bit complicated, and future reconstruction for recurrent decubitus is especially limited in paraplegic patients. The development of gluteal perforator-based flap with para-sacral perforator introduce a new treatment modality for the sacral pressure sores. Total 10 cases of sacral pressure sores were treated with gluteal perforator-based flap. There were minimal postoperative complications except wound dehiscence in one case. This flap has a many advantage of no transection or sacrifice of the gluteus maximus muscle, elevation time for the flap is short, reliable blood flow of the perforator, large rotation arc and no post-operative hindrance to walking in patients who are not paraplegic. The disadvantages of this perforator-based flaps are the anatomical variation in the location of perforators and the need for technically careful dissection.
Humans ; Postoperative Complications ; Pressure Ulcer* ; Skin ; Walking ; Wounds and Injuries

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

No abstract available.
Diabetes Mellitus* ; Diabetic Ketoacidosis* ; Humans ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prednisolone*

To validate the diagnostic significance of red cell indices in non-anemic iron deficiency and iron deficiency anemia, complete blood count, serum iron, total iron binding capacity, and serum ferritin were measured in 208 middle school girls between 13 and 15 years of age. We used Reciever Operatin Characteristic (ROC) curve to compare the diagnostic significances of various red cell indices (MCV, MCH, MCHC, RDW). We also established the ideal cutoff values of red cell indices for the diagnosis of iron deficiency anemia. The results were as follows: 1) The prevalence of iron deficiency anemia and non-anemic iron deficiency were 4.8% and 11.1% respectively. 2) For the diagnosis of iron deficiency anemia, MCV had the highest diagnostic capacity, and MCH was the secondly useful one. 3) For the diagnosis of non-anemic iron deficiency. MCV had the highest diagnostic capacity, but the sensitivity and specificity of the 4 red cell indices(MCV, MCH, MCHC, RDW) were so low that they could not be used as screening or confirmative tests. 4) The sensitivity and specificity of MCV were 1 for the diagnosis of iron deficiency anemia(cutoff value: MCV=79 fl). In case of MCH, the sensitivity was. 1 and the specificity was 0.995 for the diagnosis of iron deficiency anemia(cutoff value: MCH=25pg). In case of MCHC, the sensitivity was 1 and the specificity was 0.69 for the diagnosis of iron deficiency anemia (cutoff value: MCH-33g/dl). In case of RDW, the sencitivity was 0.9 and the specificity was 0.96 for the diagnosis of iron deficiency anemia(cutoff value: RDW=13.5%). With above results, we could conclude that MCV and MCH were very useful screening tests for iron deficiency anemia and MCV could be used as a confirmative test of iron deficiency anemin.
Anemia, Iron-Deficiency* ; Blood Cell Count ; Diagnosis ; Erythrocyte Indices* ; Female ; Ferritins ; Humans ; Iron* ; Mass Screening ; Prevalence ; ROC Curve* ; Sensitivity and Specificity