A Case of Infantile Nephrotic Syndrome associated with Neuroblastoma

Nephrotic syndrome in the first year of life, characterized by renal dysfunction and proteinuria, is associated with a heterogeneous group of disorders. These disorders are often related to genetic mutations, but the syndrome can also be caused by a variety of other diseases. We report an infant with nephrotic syndrome associated with a neuroblastoma. A 6-month-old girl was admitted with a 10% weight loss over 10 days and nephrotic-range proteinuria. She was ill-looking, and her blood pressure was higher than normal for her age. Her cystatin-C glomerular filtration rate was decreased, and levels of plasma renin, aldosterone, and catecholamines were elevated. Renal ultrasonography and abdominal computed tomography showed a retroperitoneal prevertebral mass encasing both renal arteries and the left renal vein. The mass was partially resected laparoscopically, and the pathologic diagnosis was neuroblastoma. Findings on a simultaneous renal biopsy were unremarkable. The patient was treated with chemotherapy and several anti-hypertensive drugs, including an alpha blocker. Two months later, the mass had decreased in size and the proteinuria and hypertension were gradually improving. In an infant with abnormal renin-angiotensin system activation, severe hypertension, and nephrotic-range proteinuria, neuroblastoma can be considered in the differential diagnosis.

Experiences with Endoscopic Interventions for Variceal Bleeding in Children with Portal Hypertension: A Single Center Study / 대한소아소화기영양학회지

PURPOSE: The aim of this study was to compare the efficacy and safety of band ligation and injection sclerotherapy in the endoscopic treatment of children with variceal bleeding. METHODS: The study population included 55 children, all of whom were treated at the time of endoscopic diagnosis of esophageal varices at Asan Medical Center, Seoul, Korea, between January 1994 and January 2011. The primary outcomes included initial success rates and duration of hemostasis after endoscopic management (band ligation vs. injectionsclerotherapy). RESULTS: The mean age was 6.7+/-5.2 years and the mean follow-up time was 5.4+/-3.7 years. The most common cause of esophageal varices was biliary atresia. Of 55 children with acute variceal bleeding, 39 had band ligation and 16 had injection sclerotherapy. No differences between groups were observed in terms of the size, location, and presence of red color sign. The success rates of band ligation and sclerotherapy in the control of acute bleeding episodes were 89.7% and 87.5%. The mean duration of hemostasis after endoscopic intervention was 13.2+/-25.1 months. After one year, 19 of 39 patients (48.7%) treated with band ligation and 7 of 16 patients (43.8%) with injection sclerotherapy had experienced rebleeding episodes. Complications after the procedures were observed in 10.3% and 18.8% of children treated with band ligation and injection sclerotherapy. CONCLUSION: The results of our current study suggest that band ligation and injection sclerotherapy are equally efficient treatments for the control of acute variceal bleeding and prevention of rebleeding.

Unidirectional Valve Patch Closure for Ventricular Septal Defect with Severe Pulmonary Hypertension: A Case Report / 대한흉부외과학회지

The surgical closure of VSD in patient with severe pulmonary hypertension has been considered a difficult problem for surgeons, because sudden hemodynamic change after closure of the defect could bring on high perioperative mortality. Recently, it was reported that UVP (unidirectional valve patch), which allows some blood to flow from right to left in case of acute right heart failure, is effective in improving the postoperative hemodynamics after closing septal defects. This 42-year old woman had suffered from VSD for 20 years and recently complained of worsening exertional dyspnea for three months, and was diagnosed of a large VSD (2.0 cm in diameter) with severe pulmonary hypertension (116/38 mm Hg), equal to systemic arterial pressure. We could successfully close VSD with severe pulmonary hypertension using one UVP and the other UVP for the creative ASD to be prepared against possible acute right heart failure. She was discharged on the fourteenth postoperative day and has been well for twelve months with spontanenous closure of UVP patch at the ninth postopeative month.