The prognostic factors for extrahepatic biliary atresia (EHBA) after Kasai portoenterostomy include the patient's age at portoenterostomy (age), size of bile duct in theporta hepatis (size), clearance of jaundice after operation (clearance) and the surgeon's experience. The aim of this study is to examine the most significant prognostic factor of EHBA after Kasai portoenterostomy. This retrospective study was done in 51 cases of EHBA that received Kasai portoenterostomy by one pediatric surgeon. For the statistical analysis, Kaplan-Meier method, Logrank test and Cox regression test were used. A p value of less than 0.05 was considered to be significant. Fifteen patients were regarded as dead in this study, including nine cases of liver transplantation. There was no significant difference of survival to age. The age is also not a significant risk factor for survival in this study (Cox Regression test; p = 0.63). There was no significant difference in survival in relation to the size of bile duct. However, bile duct size was a significant risk factor for survival (Cox Regression test; p = 0.002). There was a significant difference in relation to survival and clearance (Kaplan-Meier method; p = 0.02). The clearing was also a significant risk factor for survival (Cox Regression test; p = 0.001). The clearance of jaundice is the most significant prognostic factor of EHBA after Kasai portoenterostomy.
Bile Ducts ; Biliary Atresia* ; Humans ; Jaundice ; Kaplan-Meier Estimate ; Liver Transplantation ; Prognosis ; Retrospective Studies ; Risk Factors

Acquired port-wine stain is rare. It mimics a congenital port-wine stain morphologically but the pattern of onset is acquired after birth. It represents a progressive ectasia of vessels located in the superficial vascular plexus. The exact mechanism is unknown but some reported cases have occurred after trauma. We herein report five cases of acquired port-wine stains and they were idiopathic in nature.
Dilatation, Pathologic ; Parturition ; Port-Wine Stain*

PURPOSE: The purpose of this study was to evaluate and compare the scintigraphic findings and diagnostic accuracy of double-phase Tc-99m sestamibi scan in primary and secondary hyperparathyroidism (HPT). MATERIALS AND METHODS: We retrospectively reviewed 16 cases of primary (18 lesions) and 11 cases of secondary HPT (44 lesions) who underwent Tc-99m-sestamibi scan before the surgical intervention. Scan was performed using LEM camera (Siemens, Germany) after the injection of 740MBq of Tc-99m sestamibi. Routine image consisted of baseline and 3-hour delayed images and each image was obtained using both parallel and pine hole collimator. The study population was 27 patients (male/female=5/22, age: 49.1+/-10.8). RESULTS: Eighteen lesions of primary HPT consisted of 13 adenomas and 5 hyperplasias, while all lesions of secondary HPT were hyperplasias. Among the case of primary HPT, we could detect all the lesions of 13 adenomas but only 2 lesions of 5 hyperplasias (40%) could be detected by double phase scintigraphy. Three cases of primary lesion showed decreased uptake in delayed images compared with baseline. The sensitivity, specificity, positive predictive value and accuracy of primary and secondary HPT were 58.8% (10/17), 83.3% (10/12), 83.3% (10/12), 75.9% (22/29), and 37.5% (15/40), 50% (2/4), 88.2% (15/17), 38.6% (17/44), respectively. Overall sensitivity, specificity, positive predictive value and accuracy were 43.9% (25/57), 75% (12/16), 86.2% (25/29), and 53.4% (39/73). There were no statistical difference between the weight of primary and secondary HPT lesion (p>0.05). CONCLUSION: Tc-99m sestamibi scan is fairly good modality to detect parathyroid lesion in patient with primary HPT before the surgical intervention. However, since some of cases may reveal decreased uptake in delayed image, a careful attention to the findings of baseline images may be helpful. Still the low accuracy of sestamibi scan in diagnosis of secondary HPT prohibits routine use of it for this disease.
Adenoma ; Diagnosis ; Humans ; Hyperparathyroidism, Primary ; Hyperparathyroidism, Secondary* ; Hyperplasia ; Parathyroid Glands ; Radionuclide Imaging* ; Retrospective Studies ; Sensitivity and Specificity

It has been widely accepted that complete surgical resection of hepatoblastoma is essential for long-term survival. But unfortunately less that 50% of hepatic tumors in children can be totally removed at the time of diagnosis. This report is to present the experience of successful resection of hepatoblastoma after concurrent radiotherapy with transarterial chemoinfusion in a child. We believe this modality of treatment enables complete resection of unresectable hepatoblastoma, which is resistant to the systemic chemotherapy.
Child ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Radiotherapy*

Transhiatal gastric transpositions were performed in two case of long gap esophageal atresia without tracheoesophageal fistula. The patients were a 12 months old female and an 18 months old male. Stamm type gastrostomies were performed at other hospitals in both cases. The stomach was mobilized preserving the right gastric artery, the right gastroepiploic artery and spleen. The proximal and distal esophageal pouches were excised by transcervical and transhiatal route, respectively. The mobilized stomach was pulled up to the neck through esophageal hiatus and posterior mediastinal route. The esophagogastrostomy, the only one anastomosis of this procedure, was performed in the neck. There was no clinical evidence of anastomotic leakage, stricture, regurgitation, difficulty of gastric emptying, hoarseness or respiratory problem. Transhiatal gastric transposition seems to be a safe and easy alternative surgical procedure for esophageal replacement in long gap esophageal atresia.
Anastomotic Leak ; Arteries ; Constriction, Pathologic ; Esophageal Atresia* ; Female ; Gastric Emptying ; Gastroepiploic Artery ; Gastrostomy ; Hoarseness ; Humans ; Infant ; Male ; Neck ; Spleen ; Stomach ; Tracheoesophageal Fistula

BACKGROUND: The claudins are a family of transmembrane proteins associated with tight junctions and they are critical for maintaining cell-to-cell adhesion in sheets of epithelial cells. However, their role in the progression of cancer remains largely unexplored. The aims of this study were to evaluate the expression patterns of claudin-1 and -4 in benign lesions and invasive ductal carcinomas (IDC) of the breast, and relationships between the expression of these markers and the clinicopathological characteristics in IDC patients. METHODS: We examined the claudin-1 and -4 protein expressions by performing immunohistochemical stainings in 54 benign lesions and 120 IDCs via the tissue microarray method. We evaluated the correlation between the expression of these markers and the clinicopathological characteristics of IDC. RESULTS: The expressions of claudin-1 (p=0.099) and -4 (p=0.000) were up-regulated in IDCs as compared with benign lesions. The claudin-1 expression correlated with the loss of estrogen receptor (p=0.036) and progesterone receptor (p=0.011). The claudin-4 expression correlated with lymph node metastasis (p=0.043), the nuclear grade (p=0.030), the histologic grade (p=0.007), and the loss of estrogen receptor (p=0.001) and progesterone receptor (p= 0.029). CONCLUSIONS: These results suggest that claudin-1 and -4 may play a significant role in the carcinogenesis of IDC of the breast and these may represent novel markers for this disease.
Breast* ; Carcinogenesis ; Carcinoma, Ductal* ; Claudin-1* ; Claudin-4 ; Claudins ; Epithelial Cells ; Estrogens ; Humans ; Immunohistochemistry ; Lymph Nodes ; Neoplasm Metastasis ; Receptors, Progesterone ; Tight Junctions

Anti-leucine-rich glioma-inactivated 1 (LGI-1) encephalitis is clinically characterized by subacute cognitive decline and frequent seizures including fasciobrachial dystonic seizures. A 67-year-old man visited the emergency department due to the recurrent episodes of seizures of 4 days duration. At the time of the visit, he showed hyponatremia at Na 121 mg/dL. His symptoms were relieved by 3% sodium chloride and water restriction. Here we report a case of anti-LGI-1 encephalitis with worsening symptoms associated with hyponatremia.

BACKGROUND: The p73 is a recently identified homologue of the tumor suppressor gene, p53, and it has been found to induce apoptosis and inhibit cell proliferation. However, its role in the development of tumors is unclear. This study examined the expression of p73 in patients with non-small cell lung carcinomas (NSCLCs) to determine its clinical significance and association with the expressions of p53, pRb, and mdm2. METHODS: A total of 183 NSCLCs were analyzed immunohistochemically using a tissue microarray. RESULTS: The p73 protein was expressed in the cell nuclei in 156 (85.2%) out of the 183 cases. There was no correlation between the p73 expression and the clinicopathological variables. However, there was a correlation between the p73 expression and the mdm2 and pRb expressions. Multivariate Cox survival analysis identified tumor size and lymph node metastasis to be independent prognostic factors, but the p73 expression was not found to be associated with the patients' survival. CONCLUSIONS: p73 is commonly expressed in NSCLC and it might, in conjunction with pRb and mdm2, be involved in the development of these tumors.
Apoptosis ; Carcinoma, Non-Small-Cell Lung ; Cell Nucleus ; Cell Proliferation ; Genes, Tumor Suppressor ; Humans ; Lung* ; Lymph Nodes ; Neoplasm Metastasis ; Prognosis

We underwent 3 cases of laparoscopic splenectomy in pediatric patients of hereditary spherocytosis and had excellent outcome. Average operation time was 100 minutes and it was longer than average operation time of traditional open splenectomy in pediatric patients of hereditary spherocytosis (83 minute), but average hospitalization day was 3 days and it was shorter than compared group (6.2 days). Advantages of laparoscopic surgery were appeared in this study. Excellent outcome of these cases will help further performance of laparoscopic splenectomy in children.
Child* ; Hospitalization ; Humans ; Laparoscopy ; Splenectomy*

Animals ; Bone Marrow ; Bone Regeneration ; Calcium Phosphates ; Fibrin ; Humans ; Osteogenesis ; Platelet-Rich Plasma ; Rabbits ; Skull ; Transplants