No abstract available.

No abstract available.
Rectal Prolapse*

Anemia is one of the important causes compromising normal growth, development, and immune function in children. In outpatient clinics, we can see patients with anemia caused by various etiologies, whether congenital or acquired. Most cases of childhood anemia are caused by incomplete supplements of nutrients for erythropoiesis, despite the improved socioeconomic situation. Sometimes we need to treat the underlying diseases to correct the anemia. Also, we have to evaluate the etiology of anemia according to age, and consider medical treatments on the basis of the underlying causes. In this article, the author reviews pharmacotherapy in childhood anemia.
Ambulatory Care Facilities ; Anemia* ; Child ; Drug Therapy* ; Erythropoiesis ; Humans

BACKGROUND: Photodynamic therapy(PDT) is a type of photochemotherapy that is designed to kill targeted tumor cells. OBJECTIVE: The Clinical effects of PDT were analysed for response rates, post-treatment healing and adverse effects on several cutaneous carcinoma in situ. METHOD: PDT with topical 5-aminolevulinic acid-based irradiation of corresponding 630+5nm light was performed in 6 carcinoma in situ patients who had actinic keratosis, Bowen' disease or cutaneous squamous cell carcinoma. RESULT: In all patients the clinical results were exellent with respect to initial complete responses and cosmetic outcome. CONCLUSION: PDT might be chosen as a first line treament for cutaneous carcimoma in situ.
Carcinoma in Situ* ; Carcinoma, Squamous Cell ; Humans ; Keratosis, Actinic ; Photochemotherapy* ; Skin*

BACKGROUND: The prognosis for children with relapsed acute lymphoblastic leukemia remains dismal. Ifosfamide has previously been shown to be active as a single agent and in combination with doxorubicin, etoposide, and teniposide in pediatric solid tumors, recurrent acute lymphoblastic leukemia and adult acute leukemia. We assessed the efficacy and the toxicity of the drug combination with ifosfamide and etoposide in patients with relapsed refractory acute lymphoblastic leukemia. METHODS: Between April 1995 and May 1996, twenty children aged 1 to 14 years with ALL in Catholic Medical Center, all heavily pretreated and in bone marrow relapse, were enrolled in this study. Drugs were given intravenously each day for 5 days at the following doses ; ifosfamide 1.8 g/m2/day, etoposide 100 mg/m2/day and mesna 1440 mg/ m2/day(as a uroprotectant) ; Cycles were repeated every 28 days for two cycles. RESULTS: 1) Twenty heavily pretreated patients were entered on study. At study entry, seventeen patients were in first relapse, two were in second relapse and one was in third relapse. 2) Six patients(30%) achieved complete remission, and eight patients(40%) achieved partial remission. Overall response rate was 70%. 3) Duration of remission ranged from 30 days to 230 days. 4) The toxicity of the regimen was tolerated. Moderate or severe toxicity evaluated on a per cycle basis included : neutropenia 52.5%, thrombocytopenia 45%, hemorrhagic cystitis 12.5% and mucositis 2.5%. 5) Two patients went on to bone marrow transplantation with histocompatibility matched sibling donors while in remission. CONCLUSION: The combination of ifosfamide and etoposide with mesna uroprotection has significant activity in relapsed refractory childhood lymphoblastic leukemia with tolerable toxicity. We recommended bone marrow transplantation after successful reinduction because of short remission duration of this regimen.
Adult ; Bone Marrow ; Bone Marrow Transplantation ; Child ; Cystitis ; Doxorubicin ; Etoposide* ; Histocompatibility ; Humans ; Ifosfamide* ; Leukemia ; Mesna ; Mucositis ; Neutropenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Recurrence ; Siblings ; Teniposide ; Thrombocytopenia ; Tissue Donors

BACKGROUND: This study was carried out to evaluate the efficacy of desferrioxamine as a chelating agent in iron overloaded patients with severe aplastic anemia due to multiple transfusion. METHODS AND MATERIALS: From Oct. 1995 to Aug. 1996, 15 patients with aplastic anemia, diagnosed from May 1995 to Jan. 1996 at St. Mary's Hospital, who had a transfusional hemosiderosis were included in this study. They received 19 courses of high-dose desfer-rioxamine therapy for 6 days(20 to 30 mg/kg daily as a 24-hour intravenous infusion) . Before and after treatment, we measured serum ferritin, iron, TIBC, 24-hour urinary excretion of iron. RESULTS: 1) The range of iron load before treatment was between 4.5 and 20.0 gram. 2) Because of limit of detection(1,800 microgram/L), it was difficult to compare the changes of serum ferritin level after therapy to those of before therapy. 3) There was no significant differences between the levels of serum iron before and after therapy(214.3+/-62.8 vs 220.0+/-53.3). And there was no significant differences between TIBC before and after therapy(235.8+/-64.6 vs 259.4+/-60.1). 4) Iron/TIBC ratios were significantly deceased after desferrioxamine treatment compared to those of before therapy(0.90+/-0.04 vs 0.85+/-0.04, P<0.001) and mean urinary excretions of iron were increased by high-dose desferrioxamine compared to those by test dose(6.5+/-7.6 vs 29.1+/-14.3, P<0.001) CONCLUSION: High-dose desferrioxamine therapy is very effective for chelating and excretion of iron in iron overloaded patients with severe aplastic anemia due to multiple transfusion. A repeat administration of desferrioxamine is necessary for the iron overloaded patient to eliminate the risk of a transfusional hemosidersis.
Anemia, Aplastic* ; Deferoxamine* ; Ferritins ; Hemosiderosis* ; Humans ; Iron ; Iron Overload

Juvenile idiopathic arthritis (JIA) is a chronic inflammation of joints in pediatric patients. Assessment of JIA disease activity is very difficult, because children cannot definitely describe their pain by themselves due to development of cognitive function during the pediatric period. Assessment of JIA disease activity is useful for quantitative measurement of patient status, monitoring therapeutic response, and disease course over time. This article reviewed objective assessment tool for JIA disease activity and described differences in assessment between adult rheumatoid arthritis and JIA.
Adult ; Arthritis, Juvenile* ; Arthritis, Rheumatoid ; Child ; Humans ; Inflammation ; Joints

PURPOSE: We reviewed the result of allogeneic bone marrow transplantation(BMT) from HLA-identical sibling donors in children with refractory stem cell disorder along with future implication. METHODS: Forty-two children with refractory stem cell disorder received BMT from HLA-identical sibling donors between Nov. 1983 and Feb. 1995. Out of 42 children, 23 cases were severe aplastic anemia(SAA) and 19 cases were refractory leukemias. There were 20 male and 22 female with median age of 13 years (range, 2-17) and median follow-up of 36 months (range, 4-139 months). RESULTS: 1) The overall survival rate of all patients was 73.8%. The survival rate for SAA cases was 87.0%, while that for leukemia was 57.9%. 2) Acute GVHD(> or = grade II) was observed in 16.7% of all patients, 8.7% of SAA patients and 26.3% of leukemia patients, respectively. Chronic GVHD developed in 9.5% of all patients, 4.8% of limited type and 4.8% of extended type. No death was directly attributable to GVHD. 3) The causes of death after allogeneic BMT were graft rejection(7.1%), relapse of leukemia(7.1%), thrombotic thrombocytopenic purpura(4.8%), veno-occlusive disease, sepsis and CMV pneumonia respectively 2.4%. 4) The most common complication except death after allogeneic BMT was herpes zoster(26.2%). The other complications were hemorrhagic cystitis(7.1%), bronchiolitis obliterans and measles respectively 2.4%. CONCLUSIONS: We confirmed that allogeneic BMT is the curable treatment for children with refractory stem cell disorder. The most important factors that influence the result of transplantation are interval between diagnosis and transplantation in severe aplastic anemia and remission state at transplantation in leukemia.
Anemia, Aplastic* ; Bone Marrow Transplantation* ; Bone Marrow* ; Bronchiolitis Obliterans ; Cause of Death ; Child* ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Leukemia* ; Male ; Measles ; Pneumonia ; Recurrence ; Sepsis ; Siblings ; Stem Cells ; Survival Rate ; Tissue Donors ; Transplants

MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) is one of the mitochondrial encephalomyopathy, A rare disease caused by a disturbance of the mitochondrial chain of respiration. MELAS is confirmed by typical light and electron microscopic findings : "ragged red fibers" by modified Gomori trichrome stain on light microscope and numerous abormal mitochondria on electron microscope. We experienced a boy with the characteristic clinical and pathologic findings of MELAS. Our patient demonstrated bilateral basal ganglia calcifications and infarction at right parieto-occipital and thalamic areas on CT and MR We found that MRI was more sensitive and represented the infarcted lesions better than CT. Detection of cerebral insults of MELAS by MRI is important in making decision on patient treatment and also in predicion of the patient prognosis.
Acidosis, Lactic ; Basal Ganglia ; Brain Diseases ; Child* ; Humans ; Infarction ; Magnetic Resonance Imaging ; Male ; MELAS Syndrome* ; Mitochondria ; Mitochondrial Encephalomyopathies ; Muscular Diseases ; Rare Diseases ; Respiration

PURPOSE: This study aimed at determining the detection rate of respiratory viruses and at investigating the risk factors associated with respiratory virus detection in young infants. METHODS: From September 2011 to August 2012, nasopharyngeal swabs were obtained from 227 infants aged < or =90 days with suspected infectious diseases, including sepsis. We performed a retrospective analysis of their clinical characteristics. The prevalence of respiratory viruses in their nasopharyngeal swabs was assayed by real-time polymerase chain reaction (real-time PCR). RESULTS: In total, 157 (69.2%) infants had more than one of the following respiratory viruses: respiratory syncytial virus (n=75), rhinovirus (n=42), influenza virus (n=18), parainfluenza virus (n=15), human metapneumovirus (n=9), coronavirus (n=9), adenovirus (n=4), and bocavirus (n=3). During the same period, bacterial infections were confirmed in 24 infants (10.6%). The detection of respiratory viruses was significantly associated with the presence of cough, a family history of respiratory illness, and a seasonal preference (fall/winter). Using logistic regression analysis, these 3 variables were also identified as significant risk factors. During fall and winter, detection of respiratory viruses was significantly higher in infants who did not have a bacterial infection. CONCLUSION: Respiratory virus is an important pathogen in young infants admitted to a hospital, who are suspected with infectious diseases. Detection of respiratory viruses in young infants was associated with seasonality (fall/winter), presence of respiratory symptoms and a family history of respiratory illness.
Adenoviridae ; Bacterial Infections ; Bocavirus ; Communicable Diseases ; Coronavirus ; Cough ; Humans ; Infant* ; Logistic Models ; Metapneumovirus ; Nasopharynx ; Orthomyxoviridae ; Paramyxoviridae Infections ; Prevalence ; Real-Time Polymerase Chain Reaction ; Respiratory Syncytial Viruses ; Respiratory Tract Infections ; Retrospective Studies ; Rhinovirus ; Risk Factors* ; Seasons ; Sepsis ; Virus Diseases