OBJECTIVE: The study aimed to describe portal stenting for postoperative portal occlusion with delayed (≥ 3 months) variceal bleeding in the afferent jejunal loop. MATERIALS AND METHODS: Eleven consecutive patients (age range, 2–79 years; eight men and three women) who underwent portal stenting between April 2009 and December 2015 were included in the study. Preoperative medical history and the postoperative clinical course were reviewed. Characteristics of portal occlusion and details of procedures were also investigated. Technical success, treatment efficacy (defined as disappearance of jejunal varix on follow-up CT), and clinical success were analyzed. Primary stent patency rate was plotted using the Kaplan-Meier method. RESULTS: All patients underwent hepatobiliary-pancreatic cancer surgery except two children with liver transplantation for biliary atresia. Portal occlusion was caused by benign postoperative change (n = 6) and local tumor recurrence (n = 5). Variceal bleeding occurred at 27 months (4 to 72 months) and portal stenting was performed at 37 months (4 to 121 months), on average, postoperatively. Technical success, treatment efficacy, and clinical success rates were 90.9, 100, and 81.8%, respectively. The primary patency rate of portal stent was 88.9% during the mean follow-up period of 9 months. Neither procedure-related complication nor mortality occurred. CONCLUSION: Interventional portal stenting is an effective treatment for delayed jejunal variceal bleeding due to portal occlusion after hepatobiliary-pancreatic surgery.
Biliary Atresia ; Child ; Esophageal and Gastric Varices ; Follow-Up Studies ; Hemorrhage* ; Humans ; Liver Transplantation ; Male ; Methods ; Mortality ; Portal Vein* ; Recurrence ; Stents* ; Treatment Outcome ; Varicose Veins*

No abstract available.
Ataxia* ; Immunoglobulin G* ; Ophthalmoplegia*

The pneumonia severity index (PSI) and CURB-65 are widely used tools for the prediction of community-acquired pneumonia (CAP). This study was conducted to evaluate validation of severity scoring system including the PSI and CURB-65 scores of Korean CAP patients. In the prospective CAP cohort (participated in by 14 hospitals in Korea from January 2009 to September 2011), 883 patients aged over 18 yr were studied. The 30-day mortalities of all patients were calculated with their PSI index classes and CURB scores. The overall mortality rate was 4.5% (40/883). The mortality rates per CURB-65 score were as follows: score 0, 2.3% (6/260); score 1, 4.0% (12/300); score 2, 6.0% (13/216); score 3, 5.7% (5/88); score 4, 23.5% (4/17); and score 5, 0% (0/2). Mortality rate with PSI risk class were as follows: I, 2.3% (4/174); II, 2.7% (5/182); III, 2.3% (5/213); IV, 4.5% (11/245); and V, 21.7% (15/69). The subgroup mortality rate of Korean CAP patients varies based on the severity scores and CURB-65 is more valid for the lower scores, and PSI, for the higher scores. Thus, these variations must be considered when using PSI and CURB-65 for CAP in Korean patients.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Asian Continental Ancestry Group ; Cohort Studies ; Community-Acquired Infections/*mortality ; Female ; Humans ; Intensive Care Units ; Male ; Middle Aged ; Pneumonia/*mortality ; Prospective Studies ; Republic of Korea ; *Severity of Illness Index ; Young Adult

Carnitine (beta-hydroxy-gamma-trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic jaundice and poor weight gain, and who responded dramatically to oral carnitine supplementation.
Carnitine ; Esters ; Fatty Acids ; Humans ; Infant, Newborn ; Jaundice, Obstructive ; Live Birth ; Weight Gain

Carnitine (beta-hydroxy-gamma-trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic jaundice and poor weight gain, and who responded dramatically to oral carnitine supplementation.
Carnitine ; Esters ; Fatty Acids ; Humans ; Infant, Newborn ; Jaundice, Obstructive ; Live Birth ; Weight Gain

PURPOSE: Early identification of neonatal sepsis is a global issue because of limitations in diagnostic procedures. The objective of this study was to compare the diagnostic accuracy of neutrophil CD64 and C-reactive protein (CRP) as a single test for the early detection of neonatal sepsis. METHODS: A prospective study enrolled newborns with documented sepsis (n=11), clinical sepsis (n=12) and control newborns (n=14). CRP, neutrophil CD64, complete blood counts and blood culture were taken at the time of the suspected sepsis for the documented or clinical group and at the time of venipuncture for laboratory tests in control newborns. Neutrophil CD64 was analyzed by flow cytometry. RESULTS: CD64 was significantly elevated in the groups with documented or clinical sepsis, whereas CRP was not significantly increased compared with controls. For documented sepsis, CD64 and CRP had a sensitivity of 91% and 9%, a specificity of 83% and 83%, a positive predictive value of 83% and 33% and a negative predictive value of 91% and 50%, respectively, with a cutoff value of 3.0 mg/dL for CD64 and 1.0 mg/dL for CRP. The area under the receiver-operating characteristic curves for CD64 index and CRP were 0.955 and 0.527 (P<0.01), respectively. CONCLUSION: These preliminary data show that diagnostic accuracy of CD64 is superior to CRP when measured at the time of suspected sepsis, which implies that CD64 is a more reliable marker for the early identification of neonatal sepsis as a single determination compared with CRP.
Blood Cell Count ; C-Reactive Protein ; Humans ; Infant, Newborn ; Neutrophils ; Phlebotomy ; Prospective Studies ; Sensitivity and Specificity ; Sepsis

Congenital hypogonadotropic hypogonadism is one of the causes of pubertal failure and primary amenorrhea, it is related to uterine hypotrophy. If the uterus is extremely hypotrophied, it is difficult to identify in imaging studies and can be misdiagnosed as a structural anomaly of internal genitalia. We report a case of extreme uterine hypotrophy in 18-year-old girl with primary amenorrhea that was finally diagnosed as hypogonadotrophic hypogonadism. The patient was initially suspected of Mullerian agenesis owing to the invisible uterus. After 4 months of treatment with estrogen, she showed significant growth of the uterus.
Adolescent ; Amenorrhea ; Estrogens ; Female ; Genitalia ; Humans ; Hypogonadism ; Uterus

Congenital hypogonadotropic hypogonadism is one of the causes of pubertal failure and primary amenorrhea, it is related to uterine hypotrophy. If the uterus is extremely hypotrophied, it is difficult to identify in imaging studies and can be misdiagnosed as a structural anomaly of internal genitalia. We report a case of extreme uterine hypotrophy in 18-year-old girl with primary amenorrhea that was finally diagnosed as hypogonadotrophic hypogonadism. The patient was initially suspected of Mullerian agenesis owing to the invisible uterus. After 4 months of treatment with estrogen, she showed significant growth of the uterus.
Adolescent ; Amenorrhea ; Estrogens ; Female ; Genitalia ; Humans ; Hypogonadism ; Uterus

Giant congenital melanocytic nevi are very rare skin lesions with an estimated prevalence of 1 in 20,000 live births, and have high risk of malignant melanoma development and leptomeningeal melanocytosis. Hence, its early and exact diagnosis in the neonatal period is important and essential. Only seven cases of giant congenital melanocytic nevi have been reported in Korea, of which none of the cases were associated with hemangioma. Herein, the authors describe a case of giant congenital melanocytic nevi with huge hemangioma with the pathologic findings that exhibited benign and provide a literature review.
Hemangioma ; Humans ; Infant, Newborn ; Korea ; Live Birth ; Melanoma ; Nevus, Pigmented ; Prevalence ; Skin

Hemangiopericytoma is a rare vascular tumor that usually occurs in adults. The tumor is believed to originate from pericytes that are closely related to the capillary walls. Congenital hemangiopericytoma is a more rare disease that occurs in approximately 0.03% of all heamngiopericytomas. Herein, we describe a 1-month-old male newborn with huge congenital hemangiopericytoma in the retroperitoneum that exhibited a typical morphological vascular pattern.
Adult ; Capillaries ; Hemangiopericytoma ; Humans ; Infant, Newborn ; Male ; Pericytes ; Rare Diseases