12 research outputs found
Diagnosis and treatment of thyroid cancer in children in the multicenter analysis in Poland for PPGGL
Introduction: Differentiated thyroid carcinoma (DTC) in
children presents different biological behavior in comparison
to adults. Authors presents preliminary results of multicenter
analysis concerning incidence, diagnostics and treatment
of DTC in children.
Material and methods: The study is a retrospective analysis
of 107 pediatric patients from 14 academic centers based
on the data from 2000 to 2005 obtained by questionnaire in
hospitals involved in the treatment of DTC in children.
Results: Papillary thyroid cancer was diagnosed in 83 children,
follicular thyroid cancer in 10 children and medullary
thyroid cancer in 14 children. Incidence of DTC in children
was estimated between 18 and 23 cases per year. The biggest
group of patients consisted of children between 11 and
15 years of age, with girls to boys ratio 3.3 : 1. Clinically DTC
in children presented most often as solitary thyroid nodule.
Cervical lymphadenopathy was observed in 42% of patients.
Intraoperative verification indicated metastatic nodes
in 50% of children. Low stage DTC predominated (T1
in 36% and T2 in 26% of children). One step surgery was performed in 65% of children with DTC, two step surgery
in 25% of patients. I131 therapy was undertaken in 80% of
children. Lung metastases were indicated in post therapeutic
studies in 14% of children with DTC. Prophylactic thyroidectomies
were performed in 79% of children in the group
of patients with MTC and RET gene mutations.
Conclusions: The necessity of introduction of unified therapeutic
standard in children with DTC in Poland is underlined.Wst臋p: Zr贸偶nicowane raki tarczycy (DTC, differentiated thyroid
carcinoma) wyst臋puj膮 u dzieci rzadko. Wi臋kszo艣膰 przypadk贸w
wykrywanych jest w wieku 11-17 lat. W odr贸偶nieniu
od doros艂ych DTC u dzieci prezentuj膮 odmienne zachowanie
biologiczne. Ma艂a liczba przypadk贸w DTC
w poszczeg贸lnych o艣rodkach oraz wzgl臋dnie 艂agodny ich
przebieg utrudniaj膮 ocen臋 wyst臋powania i leczenia DTC
u dzieci w Polsce, uzale偶niaj膮c j膮 od wysi艂k贸w w艂o偶onych
w uzyskanie rzetelnych danych. Autorzy przedstawiaj膮
wst臋pne wyniki analizy wieloo艣rodkowej dotycz膮ce wyst臋powania,
diagnostyki i leczenia DTC u dzieci.
Materia艂 i metody: Podj臋te badania s膮 retrospektywn膮 analiz膮
obejmuj膮c膮 lata 2000-2005, opart膮 na danych z historii
chor贸b uzyskanych z ankiet rozes艂anych do o艣rodk贸w dla
dzieci i doros艂ych podejmuj膮cych leczenie DTC. Do analizy
zg艂oszono 107 pacjent贸w z 14 o艣rodk贸w akademickich
w Polsce. Analizie poddano wiek i p艂e膰 dzieci z DTC, wielko艣膰
i lokalizacj臋 zmian w tarczycy, sposoby rozpoznawania
DTC, rodzaje i zakres wykonywanych zabieg贸w operacyjnych
oraz leczenie uzupe艂niaj膮ce izotopem J131.
Wyniki: Raka brodawkowatego stwierdzono u 83 dzieci,
p臋cherzykowego u 10 dzieci, a rdzeniastego u 14 dzieci. Cz臋sto艣膰
wyst臋powania DTC u dzieci w Polsce waha艂a si臋 mi臋dzy
18 a 23 przypadkami rocznie. W wojew贸dztwach: mazowieckim
i po艂膮czonych wielkopolskim i lubuskim wykazano
w okresie 2000-2005 wy偶sz膮 (24 i 25) cz臋sto艣膰 wyst臋powania
DTC, w pozosta艂ych wojew贸dztwach wykazywano
od 2 do 10 przypadk贸w DTC. Najwi臋ksz膮 grup臋 pacjent贸w
stanowi艂y dzieci w wieku 11-15 lat, a stosunek dziewcz膮t do ch艂opc贸w wynosi艂 3,3 : 1. Klinicznie DTC prezentowa艂y
si臋 najcz臋艣ciej jako pojedyncze guzki tarczycy. Limfadenopati臋
szyjn膮 w badaniu klinicznym stwierdzono
u 42% pacjent贸w, a 艣r贸doperacyjnie u 50% dzieci. U wi臋kszo艣ci
pacjent贸w dominowa艂y ni偶sze stopnie zaawansowania
DTC (T1 u 36% i T2 u 26% dzieci). Operacje jednoetapowe
wykonano u 65% dzieci, operacje dwuetapowe u 25%
dzieci, a profilaktyczne tyreoidektomie u 79% dzieci z grupy
pacjent贸w z rakiem rdzeniastym tarczycy (MTC, medullary
thyroid cancinoma) i mutacj膮 genu Ret. Leczenie izotopowe
J131 podj臋to u 80% dzieci. Przerzuty do p艂uc w scyntygrafii
poterapeutycznej wykazano u 14% dzieci z DTC.
Wnioski: We wnioskach podkre艣la si臋 konieczno艣膰 wdro偶enia
na terenie ca艂ego kraju ujednoliconego i ocenianego
na podstawie obiektywnych przes艂anek sposobu post臋powania
z dzie膰mi z DTC
MARS procedure as a bridge to combined liver-kidney transplantation in severe chromium-copper acute intoxication: a paediatric case report
Liver regeneration in living-related donors after harvesting of liver segments II and III or II, III and IV
Backgroun
Cross-cutting view of current challenges in paediatric solid organ and haematopoietic stem cell transplantation in Europe: the European Reference Network TransplantChild.
The low prevalence of European paediatric transplanted patients and scarcity of resources and expertise led to the need for a multidisciplinary network able to improve the quality of life of paediatric patients and families requiring a solid organ or haematopoietic stem cell transplantation. The European Reference Network (ERN) TransplantChild is one of the 24 ERNs established in a European legal framework to improve the care of patients with rare diseases. ERN TransplantChild is the only ERN focused on both solid organ and haematopoietic stem cell paediatric transplantation, based on the understanding of paediatric transplantation as a complex and highly specialised process where specific complications appear regardless the organ involved, thus linking the skills and knowledge of different organ disciplines. Gathering European centres of expertise in paediatric transplantation will give access to a correct and timely diagnosis, share expertise and knowledge and collect a critical mass of patients and data that increases the speed and value of clinical research outcomes. Therefore, the ERN TransplantChild aims for a paediatric Pan-European, Pan-transplant approach