Author/Editor		Kern, Izidor; Osolnik, Katarina; Tomič, Viktorija; Uršič, Viktor
Title		Whipplova bolezen
Translated title		Whipple's disease
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 78, št. 1
Publication year		2009
Volume		str. 13-8
Language		slo
Abstract		Background Whipple’s disease is a chronic, multisystemic disease caused by a microorganism named Tropheryma whipplei. Disease is rare, so it presents a diagnostic problem to clinicians. Long-term treatment with antibiotics is successful. We report two patients with longstanding complaints and accurate histopathological diagnosis further on confirmed by molecular biology test. Methods Both patients presented with malaise, weight loss and laboratory parameters of chronic infection. Since rheumatological, haemathological and infective causes of complaints were ruled out, patients were admitted to our hospital. Histopathological examination in both cases confirmed Whipple’s disease and antibiotic treatment was started. Polymerase chain reaction using specific oligonucleotide primers for 16S rRNA was performed on formalin-fixed, paraffin-embedded tissue samples of both patients and peripheral blood of one patient. Results One patient presented with advanced disease and despite antibiotic treatment he died with signs of pulmonary arterial hypertension. The other patient was diagnosed early in the course of the disease and antibiotic treatment was effective. In tissue samples of both patients we found PAS-positive intracellular bacilli. By PCR method we detected a specific gene segment of Tropheryma whipplei in tissue samples and peripheral blood. Conclusions Presented two cases confirm the rarity of Whipple’s disease and illustrate the variety of nonspecific clinical findings which lead to late diagnosis. Histopathological changes are very specific on PAS staining. Molecular biology test aids in disease confirmation.
Summary		Izhodišča Whipplova bolezen je kronična, večsistemska bolezen, ki jo povzroča bacil Tropheryma whipplei. Bolezen je redka, zato jo težko prepoznamo. Dolgotrajno antibiotično zdravljenje je uspešno. V prispevku prikazujemo dva bolnika z večletnimi težavami, zanesljivo histopatološko diagnozo in potrditvijo z molekularnobiološkim testiranjem. Metode Pri obeh bolnikih so izstopali slabo počutje, hujšanje, laboratorijski kazalci kroničnega vnetja. Po izključevanju revmatološkega, hematološkega in infekcijskega vzroka težav sta bila napotena v Bolnišnico Golnik ‐ KOPA. Pri obeh smo s histopatološkim pregledom dokazali Whipplovo bolezen in uvedli antibiotično zdravljenje. V vzorcih tkiva obeh bolnikov, fiksiranih v formalinu in vklopljenih v parafin ter periferne krvi drugega bolnika, smo opravili test verižne reakcije s polimerazo s specifičnimi oligonukleotidnimi začetniki za zaporedje 16S rRNK. Rezultati Pri prvem bolniku je bolezen napredovala in je bolnik kljub antibiotičnemu zdravljenju umrl zaradi pljučne arterijske hipertenzije. Pri drugem je bila bolezen odkrita dovolj zgodaj, da je bilo antibiotično zdravljenje uspešno. V obeh primerih smo v tkivnih vzorcih dokazali PAS-pozitivne intracelularne bacile. V vzorcih tkiva in periferne krvi smo z verižno reakcijo s polimerazo pomnožili del gena 16S rRNK, značilnega za bakterijo Tropheryma whipplei. Zaključki Opisana primera potrjujeta, da je Whipplova bolezen redka. Zaradi pestre, neznačilne klinične slike jo pogosto pozno ali prepozno diagnosticiramo. Histopatološka slika je zelo značilna, ker lahko s PAS dokažemo prisotnost povzročitelja. Molekularnobiološki test zanesljivo potrdi etiologijo bolezni.
Descriptors		WHIPPLE'S DISEASE POLYMERASE CHAIN REACTION RNA, RIBOSOMAL, 16S MIDDLE AGE