Patients with cystic echinococcosis in the three national referral centers of Mongolia: A model for CE management assessment.

BACKGROUND: Mongolia is one of the endemic countries for cystic echinococcosis (CE), a zoonotic disease caused by the larval stage of Echinococcus granulosus. The goal of this study is to describe the current clinical management of CE in Mongolia, to capture the distribution of cyst stages of patients treated, and to contrast current practice with WHO-IWGE expert consensus. METHODS: Hospital records of CE patients treated between 2008 and 2015 at the three state hospitals and fulfilling the inclusion criterion 'discharge diagnosis CE' (ICD 10 code B.67.0-67.9) were reviewed. Demographical, geographical, clinical and ultrasonography (US) data were extracted and analyzed. The annual surgical incidence was estimated. The digital copies of US cyst images were independently staged by three international experts following the WHO CE cyst classification to determine the proportions of patients which ideally would have been assigned to the WHO recommended treatment modalities surgery, percutaneous, medical (benzimidazole) treatment and watch & wait. RESULTS: A total of 290 patient records fulfilled the inclusion criteria of the study. 45.7% of patients were below 15 years of age. 73.7% of CE cysts were located in abdominal organs, predominantly liver. US images of 84 patients were staged and assessed for interrater-agreement. The average raw agreement was 77.2%. Unweighted Kappa coefficient and weighted Kappa was 0.57 and 0.59, respectively. Mean proportions of images judged as stages CE1, CE2, CE3a, CE3b, CE4 and CL were 0.59, 0.01, 0.19, 0.08, 0.03 and 0.11, respectively. 40 cysts met the inclusion criteria of treatment modality analysis. The mean proportions of cases with a single cyst assigned to medical, percutaneous treatment, surgery and watch & wait were 52.5% (95% CI 42-65), 25.8% (95% CI 15-30), 5.1% (95% CI 0-10) and 3.3% (95% CI 0-10), respectively. 13.3% (95% CI 5-25) of cysts were staged as CL and therefore assigned to further diagnostic requirement. CONCLUSION: WHO CE cyst classification and WHO-IWGE expert consensus on clinical CE management is not implemented in Mongolia. This results in exclusively surgical treatment, an unnecessary high risk approach for the majority of patients who could receive medical, percutaneous treatment or observation (watch & wait). Introduction of WHO-IWGE expert consensus and training in ultrasound CE cyst staging would be highly beneficial for patients and the health care services.

Cystic echinococcoses in Mongolia: molecular identification, serology and risk factors.

BACKGROUND: Cystic echinococcosis (CE) is a globally distributed cestode zoonosis that causes hepatic cysts. Although Echinococcus granulosus sensu stricto (s.s.) is the major causative agent of CE worldwide, recent molecular epidemiological studies have revealed that E. canadensis is common in countries where camels are present. One such country is Mongolia. METHODOLOGY/PRINCIPAL FINDINGS: Forty-three human hepatic CE cases that were confirmed histopathologically at the National Center of Pathology (NCP) in Ulaanbaatar (UB) were identified by analysis of mitochondrial cox 1 gene as being caused by either E. canadensis (n=31, 72.1%) or E. granulosus s.s. (n=12, 27.9%). The majority of the E. canadensis cases were strain G6/7 (29/31, 93.5%). Twenty three haplotypes were identified. Sixteen of 39 CE cases with data on age, sex and province of residence were citizens of UB (41.0%), with 13 of the 16 cases from UB caused by E. canadensis (G6/7) (81.3%). Among these 13 cases, nine were children (69.2%). All pediatric cases (n  =  18) were due to E. canadensis with 17 of the 18 cases (94.4%) due to strain G6/7. Serum samples were available for 31 of the 43 CE cases, with 22 (71.0%) samples positive by ELISA to recombinant Antigen B8/1 (rAgB). Nine of 10 CE cases caused by E. granulosus s.s. (90.0%) and 13 of 20 CE cases by E. canadensis (G6/7) (65.0%) were seropositive. The one CE case caused by E. canadensis (G10) was seronegative. CE cases caused by E. granulosus s.s. showed higher absorbance values (median value 1.131) than those caused by E. canadensis (G6/7) (median value 0.106) (p  =  0.0137). CONCLUSION/SIGNIFICANCE: The main species/strains in the study population were E. canadenis and E. granulossus s.s. with E. canadensis the predominant species identified in children. The reason why E. canadensis appears to be so common in children is unknown.