[Primary sclerosing cholangitis in children - clinical manifestations and diagnostic difficulties]. / Pierwotne stwardniajace zapalenie dróg zólciowych u dzieci - obraz kliniczny oraz trudnosci diagnostyczne.

Primary sclerosing cholangitis is a rare chronic disease of intra- and extrahepatic bile ducts, which causes cholestasis with inflammation and fibrosis ultimately resulting in biliary cirrhosis. The review focuses on clinical manifestations and diagnostic difficulties in primary sclerosing cholangitis in children.

[Pulmonary metastases in children with solid tumours--own experiences]. / Przerzuty do pluc w guzach litych u dzieci--obserwacje wlasne.

BACKGROUND: The most frequent reasons of relapses in solid tumours among children are lung metastases. AIM: Analysis of lung metastatic cases among children with solid tumours treated from 1995-2005. MATERIAL AND METHODS: 26 lung metastatic cases (17 males, 9 females) were analysed. At the moment of the diagnosis lung metastases were present in 19.2% of patients while in the rest (80.8%) occurred during and after treatment. The most often lung metastases were recognised in osteosarcoma (15-57.8%) and carcinoma embryonale (3-11.6%). Secondary metastases in lungs occurred within 4-48 months after the diagnosis. In 57.7% were bilateral. 36 thoracotomies (average 1.7/ a child) were performed. The after-surgery chemotherapy for tumour recurrence was introduced in each case. RESULTS: In the analysed group 14 (53.8%) children are alive with the overall survival time 8-120 months. The rest 12 (46.2%) are dead with the survival time 6-24 months. The statistically significant difference was found in comparison of complete surgery versus incomplete (p=0.02), no significance was found in primary or secondary metastases (p=0.27). Time of occurrence was statistically insignificant (p=0.26). CONCLUSIONS: The occurrence of metastases in children solid tumours worsened the prognosis. The active search for lung metastases at the moment of diagnosis, treatment and follow-up combined with complete surgery procedures may prolong survival. There is a need to find new methods of lung metastases treatment.

[Assessment of treatment results in children with malignant liver tumours in own experience]. / Ocena wyników leczenia dzieci z nowotworami zlosliwymi watroby na podstawie badan wlasnych.

AIM: The assessment of malignant liver tumours in children treated in our centre between years 1985-2004 has been made in order to analyze the prognostic factors and improvement in survival rate. MATERIAL AND METHODS: 17 patients with malignant liver tumours were followed-up. There were 10 (58,8%) patients with hepatoblastomas, 5 (29,4%) hepatocarcinomas, 1 (5,9%) undifferentiated embryonal sarcoma and 1 (5,9%) rhabdomyosarcoma. Primary metastatic disease was recognized in 3 cases as: hepatic vascular involvement, lungs, femoral bone and lymph nodes of liver hilus metastases. All patients underwent preoperative chemotherapy. Tumour resection was attempted in 13 (76,5%) cases; it was complete within adequate resection margins in 11 (64 7%). In 3 cases biliary fistulas occurred after surgery. Secondary metastases appeared in lungs, lymph nodes of liver hilus and central nervous system in 4 cases. RESULTS: Twelve patients are alive with median follow-up 34,0 mths, five died with median survival time 16,0 mths. Total excision of liver tumour had no statistical significance in lifetime prolongation (p =0,12). Survival rate was statistically longer in patients without metastatic disease (p=0,028). CONCLUSIONS: Complete surgical excision had no statistical significance in increasing survival time in liver tumour patients. Metastatic disease had statistical significance in shortening overall survival of patients with liver tumours. Unsatisfactory results in hepatocarcinoma treatment in children dramatically demonstrate the need for new treatment approaches.