Dr L. Nabarro Professor P.L.Chiodini

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Presentation transcript:

Dr L. Nabarro Professor P.L.Chiodini Current Management of Cystic Echinococcosis; a survey of specialist practice Dr L. Nabarro Professor P.L.Chiodini

What are you doing?

Further investigation Patient 1 34 year old M Abdominal pain 9x9x10cm hepatic cyst ALT 197, ALP257, EO 2 Hydatid IgG positive KEY CONCERN PAIR for type 2 % used Pre procedure BMZ therapy Post procedure BMZ therapy Expert consensus guidance Watch and wait 2.5 Contraindicated (progression) Drug therapy alone Contraindicated (failure) PAIR 12.5 4d to 6 months 3 weeks to 6 months Contraindicated (recurrence) Other percutaneous intervention (PEVAC) 10 1 week to 1 month 1 month to 6 months With drug cover (length not specified) Surgery 62.5 0-6 months 1month to 1 year With drug cover (1 day pre to 1month post) Other Further investigation

Further investigation Patient 1 34 year old M Abdominal pain 9x9x10cm hepatic cyst ALT 197, ALP257, EO 2 Hydatid IgG positive KEY CONCERN PAIR for type 2 % used Pre procedure BMZ therapy Post procedure BMZ therapy Expert consensus guidance Watch and wait 2.5 Contraindicated (progression) Drug therapy alone Contraindicated (failure) PAIR 12.5 4d to 6 months 3 weeks to 6 months Contraindicated (recurrence) Other percutaneous intervention (PEVAC) 10 1 week to 1 month 1 month to 6 months With drug cover (length not specified) Surgery 62.5 0-6 months 1month to 1 year With drug cover (1 day pre to 1month post) Other Further investigation

Further investigation Patient 1 34 year old M Abdominal pain 9x9x10cm hepatic cyst ALT 197, ALP257, EO 2 Hydatid IgG positive KEY CONCERN PAIR for type 2 % used Pre procedure BMZ therapy Post procedure BMZ therapy Expert consensus guidance Watch and wait 2.5 Contraindicated (progression) Drug therapy alone Contraindicated (failure) PAIR 12.5 4d to 6 months 3 weeks to 6 months Contraindicated (recurrence) Other percutaneous intervention (PEVAC) 10 1 week to 1 month 1 month to 6 months With drug cover (length not specified) Surgery 62.5 0-6 months 1month to 1 year With drug cover (1 day pre to 1month post) Other Further investigation

Further investigation Patient 1 34 year old M Abdominal pain 9x9x10cm hepatic cyst ALT 197, ALP257, EO 2 Hydatid IgG positive KEY CONCERN PAIR for type 2 % used Pre procedure BMZ therapy Post procedure BMZ therapy Expert consensus guidance Watch and wait 2.5 Contraindicated (progression) Drug therapy alone Contraindicated (failure) PAIR 12.5 4d to 6 months 3 weeks to 6 months Contraindicated (recurrence) Other percutaneous intervention (PEVAC) 10 1 week to 1 month 1 month to 6 months With drug cover (length not specified) Surgery 62.5 0-6 months 1month to 1 year With drug cover (1 day pre to 1month post) Other Further investigation

Further investigation Patient 1 34 year old M Abdominal pain 9x9x10cm hepatic cyst ALT 197, ALP257, EO 2 Hydatid IgG positive KEY CONCERN PAIR for type 2 % used Pre procedure BMZ therapy Post procedure BMZ therapy Expert consensus guidance Watch and wait 2.5 Contraindicated (progression) Drug therapy alone Contraindicated (failure) PAIR 12.5 4d to 6 months 3 weeks to 6 months Contraindicated (recurrence) Other percutaneous intervention (PEVAC) 10 1 week to 1 month 1 month to 6 months With drug cover (length not specified) Surgery 62.5 0-6 months 1month to 1 year With drug cover (1 day pre to 1month post) Other Further investigation

Patient 2 33 year old M Left lobectomy 1990 with 6 months BMZ 2010: malaise, vomiting Hydatid IgG positive KEY CONCERN Wide variation in drug doses Treatment length: 6 weeks to life long Increasing use of Praziquantel Interval dosing Albendazole Drug % used Dose range Expert Consensus dose Albendazole 64.7 400mg OD/ BD 800mg OD 10-20 mg/kg/d 400mg BD for 6x 21day cycles 10-15mg/kg in two divided doses Mebendazole 2.9 15mg/kg/d 40-50mg/kg in three divided doses Praziquantel Not recommended as a sole agent Combination Albendazole and Praziquantel 29.4 100mg BD 6mg/kg/d 25-50 mg/kg/d 40mg/kg twice a week 2400mg STAT 10-15mg/kg Albendazole in two divided doses 40mg/kg Praziquantel once per week No treatment 2.9 (surgery)

Patient 2 33 year old M Left lobectomy 1990 with 6 months BMZ 2010: malaise, vomiting Hydatid IgG positive KEY CONCERN Wide variation in drug doses Treatment length: 6 weeks to life long Increasing use of Praziquantel Interval dosing Albendazole Drug % used Dose range Expert Consensus dose Albendazole 64.7 400mg OD/ BD 800mg OD 10-20 mg/kg/d 400mg BD for 6x 21day cycles 10-15mg/kg in two divided doses Mebendazole 2.9 15mg/kg/d 40-50mg/kg in three divided doses Praziquantel Not recommended as a sole agent Combination Albendazole and Praziquantel 29.4 100mg BD 6mg/kg/d 25-50 mg/kg/d 40mg/kg twice a week 2400mg STAT 10-15mg/kg Albendazole in two divided doses 40mg/kg Praziquantel once per week No treatment 2.9 (surgery)

Patient 2 33 year old M Left lobectomy 1990 with 6 months BMZ 2010: malaise, vomiting Hydatid IgG positive KEY CONCERN Wide variation in drug doses Treatment length: 6 weeks to life long Increasing use of Praziquantel Interval dosing Albendazole Drug % used Dose range Expert Consensus dose Albendazole 64.7 400mg OD/ BD 800mg OD 10-20 mg/kg/d 400mg BD for 6x 21day cycles 10-15mg/kg in two divided doses Mebendazole 2.9 15mg/kg/d 40-50mg/kg in three divided doses Praziquantel Not recommended as a sole agent Combination Albendazole and Praziquantel 29.4 100mg BD 6mg/kg/d 25-50 mg/kg/d 40mg/kg twice a week 2400mg STAT 10-15mg/kg Albendazole in two divided doses 40mg/kg Praziquantel once per week No treatment 2.9 (surgery)

Patient 2 33 year old M Left lobectomy 1990 with 6 months BMZ 2010: malaise, vomiting Hydatid IgG positive KEY CONCERN Wide variation in drug doses Treatment length: 6 weeks to life long Increasing use of Praziquantel Interval dosing Albendazole Drug % used Dose range Expert Consensus dose Albendazole 64.7 400mg OD/ BD 800mg OD 10-20 mg/kg/d 400mg BD for 6x 21day cycles 10-15mg/kg in two divided doses Mebendazole 2.9 15mg/kg/d 40-50mg/kg in three divided doses Praziquantel Not recommended as a sole agent Combination Albendazole and Praziquantel 29.4 100mg BD 6mg/kg/d 25-50 mg/kg/d 40mg/kg twice a week 2400mg STAT 10-15mg/kg Albendazole in two divided doses 40mg/kg Praziquantel once per week No treatment 2.9 (surgery)

Patient 3 58 year old F Fever and abdominal pain Hydatid IgG positive MRI: type 3A cyst involving diaphragm and lung base KEY POINTS No real guidance in Expert Consensus PAIR contraindicated in lung disease % used Pre procedure BMZ therapy Post procedure BMZ therapy Expert consensus guidance Watch and wait Contraindicated (progression) Drug therapy alone Contraindicated (failure) PAIR 20.6 3 days to 1 month 1 month to 1 year Contraindicated (lung involvement) Other percutaneous intervention 5.9 4 hours- 1 week 1month to 6 months Surgery 73.5 0 to 6 months 1 month to 6 months No guidance

Patient 3 58 year old F Fever and abdominal pain Hydatid IgG positive MRI: type 3A cyst involving diaphragm and lung base KEY POINTS No real guidance in Expert Consensus PAIR contraindicated in lung disease % used Pre procedure BMZ therapy Post procedure BMZ therapy Expert consensus guidance Watch and wait Contraindicated (progression) Drug therapy alone Contraindicated (failure) PAIR 20.6 3 days to 1 month 1 month to 1 year Contraindicated (lung involvement) Other percutaneous intervention 5.9 4 hours- 1 week 1month to 6 months Surgery 73.5 0 to 6 months 1 month to 6 months No guidance

Patient 4 76 year old M, multiple co-morbidities 2 previous surgeries for CE. Previous hepatitis with Albendazole Now abdominal pain and eosinophilia MRCP: hepatic hydatid cyst ERCP: communication between cyst and biliary duct KEY POINTS Scolicidal agent used by 55% Praziquantel as sole agent Low dose Albendazole % used Watch and wait 3.6 Drug therapy alone 10.7 PAIR Other percutaneous intervention 14.3 Surgery 71.5 Drug % used Dose Albendazole 48 200mg OD – 400mg BD Mebendazole 8 50mg/kg/d Praziquantel 12 20-50mg/kg/d Albendazole/ Praziquantel 200mg OD-400mg BD 25-50mg/kg/d Nil 24

Patient 4 76 year old M, multiple co-morbidities 2 previous surgeries for CE. Previous hepatitis with Albendazole Now abdominal pain and eosinophilia MRCP: hepatic hydatid cyst ERCP: communication between cyst and biliary duct KEY POINTS Scolicidal agent used by 55% Praziquantel as sole agent Low dose Albendazole % used Watch and wait 3.6 Drug therapy alone 10.7 PAIR Other percutaneous intervention 14.3 Surgery 71.5 Drug % used Dose Albendazole 48 200mg OD – 400mg BD Mebendazole 8 50mg/kg/d Praziquantel 12 20-50mg/kg/d Albendazole/ Praziquantel 200mg OD-400mg BD 25-50mg/kg/d Nil 24

Patient 4 76 year old M, multiple co-morbidities 2 previous surgeries for CE. Previous hepatitis with Albendazole Now abdominal pain and eosinophilia MRCP: hepatic hydatid cyst ERCP: communication between cyst and biliary duct KEY POINTS Scolicidal agent used by 55% Praziquantel as sole agent Low dose Albendazole % used Watch and wait 3.6 Drug therapy alone 10.7 PAIR Other percutaneous intervention 14.3 Surgery 71.5 Drug % used Dose Albendazole 48 200mg OD – 400mg BD Mebendazole 8 50mg/kg/d Praziquantel 12 20-50mg/kg/d Albendazole/ Praziquantel 200mg OD-400mg BD 25-50mg/kg/d Nil 24

Patient 4 76 year old M, multiple co-morbidities 2 previous surgeries for CE. Previous hepatitis with Albendazole Now abdominal pain and eosinophilia MRCP: hepatic hydatid cyst ERCP: communication between cyst and biliary duct KEY POINTS Scolicidal agent used by 55% Praziquantel as sole agent Low dose Albendazole % used Watch and wait 3.6 Drug therapy alone 10.7 PAIR Other percutaneous intervention 14.3 Surgery 71.5 Drug % used Dose Albendazole 48 200mg OD – 400mg BD Mebendazole 8 50mg/kg/d Praziquantel 12 20-50mg/kg/d Albendazole/ Praziquantel 200mg OD-400mg BD 25-50mg/kg/d Nil 24

Patient 4 76 year old M, multiple co-morbidities 2 previous surgeries for CE. Previous hepatitis with Albendazole Now abdominal pain and eosinophilia MRCP: hepatic hydatid cyst ERCP: communication between cyst and biliary duct KEY POINTS Scolicidal agent used by 55% Praziquantel as sole agent Low dose Albendazole % used Watch and wait 3.6 Drug therapy alone 10.7 PAIR Other percutaneous intervention 14.3 Surgery 71.5 Drug % used Dose Albendazole 48 200mg OD – 400mg BD Mebendazole 8 50mg/kg/d Praziquantel 12 20-50mg/kg/d Albendazole/ Praziquantel 200mg OD-400mg BD 25-50mg/kg/d Nil 24

Conclusions Areas of concern Where next? PAIR - type 2, lung cysts Albendazole Variable doses Unclear length of treatment Interrupted regimens Under treatment in disseminated disease Lack of clarity over praziquantel use Scolicidal agents in biliary fistulae Where next? European CE registry collecting prospective data Clinical trials in particular areas Length of albendazole treatment Role of praziquantel Surgery vs other percutaneous procedures for type 2/3Bs Evidence based, more presciptive guidelines Improved international co-operation: russia- lots of disease. No publications. Lots of unusual practices. More prescriptive guidelines: current guidelines lack information on how long to treat for, role of praziquantel. Evidence is not there but best guess of group of experts is better than best guess of individual physicians thus should be more prescriptive. Global hydatid registry- to collect prospective data on patient groups Clinical trials for particular clinical questions: namely length of albendazole treatment, role of praziquangel, surgery vs pevacs for type 2/3 b