To sum up the experiences and results of surgical treatment for recurrent rectal carcinoma after rectectomy, pelvic exenterations were carried out for 49 patients with the disease and the survival rate was estimated. No death occurred in our group. The resect rate was 91 8%, and the operative complication rate was 12 2%. The 3 year survival rate was 85 5%, and the 5 year survival rate was 60 6%. Pelvic exenteration can improve the survival rate for recurrent rectal carcinoma after rectectomy. Urinary reconstruction and repair of pelvic peritoneum are important for reducing operative complications.

Posterior Cortical Atrophy is a group of neurodegenerative disorders characterized by early, prominent and progressive impairment of visuospatial and visuoperceptual functions in the context of relatively preserved memory and insight in the early phases. Initial visual symptoms are vague, compelling patients to seek ophthalmologic consult. They present with simultagnosia and spatial disorientation, which are often missed by routine ophthalmologic and neurologic exams, causing delay in diagnosis. As the disease progresses, Posterior Cortical Atrophy ultimately leads to a more diffuse pattern of cognitive dysfunction. The underlying pathology is believed to be Alzheimer’s Disease and a greater level of amyloid plaques is correlated with earlier clinical symptoms of Posterior Cortical Atrophy. The clinical features of reported cases are heterogenous, leading to a classification of different variants and underlying pathologies. We report the serial clinical, cognitive and imaging data of a variant of Posterior Cortical Atrophy primarily affecting the dorsal stream.
Neuropsychological Tests

PURPOSE: Idiopathic thrombocytopenic purpura (ITP) is a relatively common hematological disease in children. It generally occurs after exposure to a common viral infection episode; however, it may occasionally follow immunization with measles, measles-mumps-rubella (MMR), hepatitis B (HBV), influenza, diphtheria-tetanus-pertussis (DTP), or chickenpox vaccines. In this study, the incidence, clinical characteristics, and treatment outcome of vaccination-associated ITP were investigated and compared with non-vaccination-associated ITP. METHODS: The admission records of 105 pediatric ITP patients between 0-14 years of age admitted to Department of Pediatrics, Wonkwang University Hospital from January 1994 to July 2007 were retrospecitively reviewed. Patients were grouped into a vaccination-associated group and a non-vaccination-associated group according to vaccination history within the previous 1 month, and various clinical features between the two groups were statistically analyzed. RESULTS: Thirteen patients (12%) had a preceding vaccination. Eight had received DTP vaccination, 2 had received hepatitis B, and 1 each had received influenza, MMR, and Japanese B encephalitis vaccination. However, none of the patients had a recurrent thrombocytopenia after subsequent vaccinations. In the vaccination-associated group, the age was significantly lower, anemia was more common, and the risk period with blood platelet count <20x10(9)/L was significantly shorter than for the in non-vaccination-associated group. Also, wet purpura was less prominent and the remission within 1month was more frequently achieved in the vaccination-associated ITP group. CONCLUSION: Vaccination-associated ITP patients showed mild symptoms with a more benign and shorter lasting course than non-vaccination-associated ITP patients. Moreover, platelet count assessment at the time of the next immunization may not be necessary.
Anemia ; Chickenpox ; Child ; Encephalitis, Japanese ; Hematologic Diseases ; Hepatitis B ; Humans ; Immunization ; Incidence ; Influenza, Human ; Measles ; Pediatrics ; Platelet Count ; Purpura ; Purpura, Thrombocytopenic ; Purpura, Thrombocytopenic, Idiopathic ; Thrombocytopenia ; Treatment Outcome ; Vaccination ; Vaccines

PURPOSE: The association between iron deficiency anemia and febrile convulsion in infants has been examined in several studies with conflicting results. Therefore, the authors aimed to evaluate the precise relationship involved. METHODS: In this case-control study, the authors assessed 100 children with a diagnosis of febrile convulsion, aged between 9 months and 2 years, during January 2007 to July 2009. The control group consisted of 100 febrile children without convulsion; controls were closely matched to the cases by age, gender, and underlying disease. RESULTS: The mean ages of the febrile convulsion and control group were 16.3+/-7.4 and 15.8+/-6.1 months, respectively, and the two groups had no differences in clinical features. Iron deficiency anemia (Hb <10.5 gm/dL) was more frequent in the febrile convulsion group than in the control group, although there was no statistical significance. Unexpectably, the RDW (red blood cell distribution width) was significantly lower and the MCNC (mean corpuscular hemoglobin concentration) was significantly higher among seizure cases than among the controls (P<0.05). There is no statistical difference between simple and complex febrile groups in the clinical and laboratory profiles. On multiple logistic regression analysis, iron deficiency anemia was more frequent, but the RDW was lower, among the cases with febrile convulsion, compared with the controls. Conclusions: Our study suggests that the iron deficiency anemia is associated with febrile convulsion, and screening for iron deficiency anemia should be considered in children with febrile convulsions.
Aged ; Anemia, Iron-Deficiency ; Blood Cells ; Case-Control Studies ; Child ; Hemoglobins ; Humans ; Infant ; Iron ; Logistic Models ; Mass Screening ; Seizures ; Seizures, Febrile

PURPOSE: Soluble transferrin receptor (sTfR) is a truncated extracellular form of the membrane transferrin receptor produced by proteolysis. Concentrations of serum sTfR are related to iron status and erythropoiesis in the body. We investigated whether serum sTfR levels can aid in diagnosis and treatment of iron deficiency anemia (IDA) in children. METHODS: Ninety-eight patients with IDA were enrolled and were classified according to age at diagnosis. Group 1 comprised 78 children, aged 6-59 months, and group 2 comprised 20 adolescents, aged 12-16 years. RESULTS: In group 1, patients' serum sTfR levels correlated negatively with mean corpuscular volume; hemoglobin (Hb), ferritin, and serum iron levels; and transferrin saturation and positively with total iron binding capacity (TIBC) and red cell distribution width. In group 2, patients' serum sTfR levels did not correlate with ferritin levels and TIBC, but had a significant relationship with other iron indices. Hb and serum sTfR levels had a significant inverse relationship in both groups; however, in group 1, there was no correlation between Hb and serum ferritin levels. In 30 patients of group 1, serum sTfR levels were significantly decreased with an increase in Hb levels after iron supplementation for 1 month. CONCLUSION: Serum sTfR levels significantly correlated with other diagnostic iron parameters of IDA and inversely correlated with an increase in Hb levels following iron supplementation. Therefore, serum sTfR levels can be a useful marker for the diagnosis and treatment of IDA in children.
Adolescent ; Anemia, Iron-Deficiency* ; Child* ; Diagnosis* ; Erythrocyte Indices ; Erythropoiesis ; Ferritins ; Humans ; Iron ; Membranes ; Proteolysis ; Receptors, Transferrin* ; Transferrin

Giant cell tumors acount for 4% of all bone tumors and usually occur in the epiphyses of long bones and rarely involve the head and neck region, representing about 9% of them. The incidence of giant cell tumor arising in Paget's disease, which is characterized by a bizarre and greatly exaggerated skeletal turnover, is probably less than 0.2%. We experienced a case of giant cell tumor in the bilateral maxilla with Paget's disease of bone, which was treated by excision and curettage of the tumor mass via endoscopic approach.
Curettage ; Epiphyses ; Giant Cell Tumors* ; Giant Cells* ; Head ; Incidence ; Maxilla* ; Neck ; Osteitis Deformans

OBJECTIVETo observe the effect of total flavonoids from Scutellaria amoena on the experimental arrhythmia.

METHODExperimental animals anesthetized with 10% chloral hydrate were evenly randomized into control group, positive control group, and low-dose, middle-dose and high-dose total flavonoids groups. The experimental arrhythmia ouabain-induced in guinea pigs and barium chloride or calcium chloride-induced in rats were observed and detected respectively. The result was converted into cumulative dosage of ouabain, in guinea pig model. In rat model, the duration of arrhythmia were detected.

RESULThold dosage of ventricular premature heat (VP) and ventricular fibrillation( VF) ouabain-induced in guinea pigs was markedly elevated, and the duration of ventricular tachycardia (VT) barium chloride-induced and VF calcium chloride-induced in rats was postponed by total flavonoids from S. amoena.

CONCLUSIONTotal flavonoids from S. amoena has obvious protective effect on drug-induced arrhythmia.

Animals ; Anti-Arrhythmia Agents ; administration & dosage ; Arrhythmias, Cardiac ; drug therapy ; Disease Models, Animal ; Female ; Flavonoids ; administration & dosage ; Guinea Pigs ; Humans ; Male ; Plant Extracts ; administration & dosage ; Rats ; Rats, Sprague-Dawley ; Scutellaria ; chemistry

BACKGROUND: Influenza-associated myositis (IAM) is a rare and poorly recognized complication of influenza infection in children, and is characterized by acute onset of severe pain in the lower extremities and a refusal to ambulate walk. We sought to understand the association between IAM and influenza B infection and to investigate its clinical and laboratory characteristics in affected children. METHODS: Influenza B-associated myositis (IBAM) cases diagnosed in the pediatrics department of Wonkwang University Hospital from January 2010 and March 2016 were analyzed retrospectively. RESULTS: Medical records of affected children were examined, and clinical characteristics and laboratory findings were recorded. Of the 536 children diagnosed with influenza B infection, 47 children complained of bilateral calf pain with or without gait disturbance. All children exhibited elevated serum aspartate aminotransferase (AST) level. The median serum creatine kinase (CK) and lactate dehydrogenase (LDH) levels, reportedly elevated in myositis, were 2,597 IU/L and 678 IU/L, respectively. While the immunofluorescence test results were negative for some patients, the polymerase chain reaction test results indicated influenza B infection in all 47 children. At the time of hospital discharge, the patients' symptoms had resolved, and their CK levels had improved. CONCLUSION: IBAM was generally benign and short, and although the blood AST, CK, and LDH levels were markedly high, the erythrocyte sedimentation rate and C-reactive protein levels were normal. Further, the duration of IBAM symptoms correlated with the duration of fever. The IBAM-associated clinical and laboratory findings are highly characteristic and may allow its rapid diagnosis during the influenza season.
Aspartate Aminotransferases ; Blood Sedimentation ; C-Reactive Protein ; Child ; Creatine Kinase ; Diagnosis ; Fever ; Fluorescent Antibody Technique ; Gait ; Humans ; Influenza B virus ; Influenza, Human ; L-Lactate Dehydrogenase ; Lower Extremity ; Medical Records ; Myalgia ; Myositis ; Pediatrics ; Polymerase Chain Reaction ; Retrospective Studies ; Seasons

OBJECTIVETo detect potential mutations of OTC gene in a male infant affected with ornithine transcarbamylase deficiency.

METHODSGenomic DNA were isolated from peripheral blood samples of family members and 100 healthy individuals. Potential mutations of the 10 exons of OTC gene were screened with PCR and Sanger sequencing.

RESULTSA homozygous missense mutation c.917G>C in exon 9, which results in p.R306T, was identified in the infant. Sequencing of the mother and two female members of the family indicated a heterozygous status for the same mutation. The same mutation was not found in other members of the family and 100 healthy controls.

CONCLUSIONA missense mutation c.917G>C in the OTC gene is responsible for the pathogenesis of the disease. Identification of the mutation can facilitate prenatal diagnosis and genetic counseling for the family.

Computational Biology ; Female ; Humans ; Male ; Mutation ; Ornithine Carbamoyltransferase ; genetics ; Ornithine Carbamoyltransferase Deficiency Disease ; diagnosis ; genetics ; Sequence Analysis, DNA

This study was aimed to investigate the changes of cytokine contents in single donor platelets (SDPs) collected by using MCS(+), Trima, Amicus blood cell separators during storage. 18 portions of SDPs were collected by MCS(+), Trima, Amicus blood cell separators, were preserved in standard condition of blood bank, the levels of cytokines such as IL-8, RANTES, CD154, TGF-beta(1) and VEGF were detected by ELISA at 1, 3, 5, 7 days during storage. The results showed that the levels of IL-8, RANTES, CD154, TGF-beta(1) and VEGF in SDPs collected by blood cell separators MCS(+), Trima, and Amicus gradually increased with prolonging of time during storage, but the increase of IL-8 level in SDPs collected by MCS(+) separator was significant difference from SDPs collected by Trime and Amicus separators (p < 0.05). It is concluded that the all collected SDPs mentioned above express IL-8, RANTES, CD154, TGF-beta(1) and VEGF during storage, and their cytokine levels show a tendency to increase with prolonging of time during storage, apheresis platelets with less leukocytes express IL-8 lower.
Blood Platelets ; metabolism ; CD40 Ligand ; blood ; Cell Separation ; methods ; Chemokine CCL5 ; blood ; Cytokines ; blood ; Humans ; Interleukin-8 ; blood ; Platelet Count ; Specimen Handling ; Transforming Growth Factor beta ; blood ; Vascular Endothelial Growth Factor A ; blood