Response patterns in adventitial layer of Echinococcus granulosus sensu stricto cysts from naturally infected cattle and sheep.

Cystic echinococcosis is a zoonotic disease caused by the metacestode of Echinococcus granulosus sensu lato. The disease is characterized by the development of cystic structures inside viscera of the intermediate host, mainly liver and lungs. These cysts are formed by three layers: germinal, laminated, and adventitial layer, the latter being the local host immune response. Metacestodes that develop protoscoleces, the infective stage to the definitive host, are termed fertile, whereas cysts that do not produce protoscoleces are termed non-fertile. Sheep usually harbor fertile cysts while cattle usually harbor non-fertile cysts. Adventitial layers with fibrotic resolution are associated to fertile cysts, whereas a granulomatous reaction is associated with non-fertile cysts. The aim of this study was to analyze cellular distribution in the adventitial layer of fertile and non-fertile E. granulosus sensu stricto cysts found in liver and lungs of cattle and sheep. A total of 418 cysts were analyzed, 203 from cattle (8 fertile and 195 non-fertile) and 215 from sheep (64 fertile and 151 non-fertile). Fertile cysts from cattle showed mixed patterns of response, with fibrotic resolution and presence of granulomatous response in direct contact with the laminated layer, while sheep fertile cysts always displayed fibrotic resolution next to the laminated layer. Cattle non-fertile cysts display a granulomatous reaction in direct contact with the laminated layer, whereas sheep non-fertile cysts display a granulomatous reaction, but in direct contact with the fibrotic resolution. This shows that cattle and sheep cystic echinococcosis cysts have distinct local immune response patterns, which are associated to metacestode fertility.

[A CASE OF FAMILIAL PULMONARY AND HEPATIC ECHINOCOCCOSIS)].

Echinococcus granulosus still remains a widespread disease. Moreover, there is a large number of patients in whom it is diagnosed rather late because its clinical presentation can be unspecific and symptoms vary. We report a familial case of the disease. Its specific feature was the onset with the bilateral pulmonary process and late involvement of the liver. This excluded other pulmonary diseases, such as pneumonia, tuberculosis or cancer.

Pulmonary hydatid cysts: the naturally occurring models for rupture.

BACKGROUND: Pulmonary hydatid cysts are recognized to have high rate of rupture compared to those in other affected organs. To identify risk factors associated with endobronchial rupture, we prospectively assessed 32 patients with hydatid cysts. There were 21 males and 11 females, with a mean age of 32 ± 15 years (range 9 to 65 years). METHODS: All patients undergoing thoracotomies for hydatid cysts were included. Demographic data, site, size, and whether cysts were ruptured or intact, were reviewed. Intraoperatively, bronchial fistula diameters were measured. A stepwise multiple logistic regression model was used to analyze the results. RESULTS: Seventeen (53.1%) patients presented with ruptured cysts (group 1) and 15 with intact cysts (group 2). There was a significant difference in mean fistula diameter between groups: 6.16 ± 2 mm in group 1 vs. 0.34 ± 0.19 mm in group 2 (p ≤ 0.0001), which was identified as the only significant risk factor associated with cyst rupture. CONCLUSION: At the fistula site, the intracystic pressure is unopposed, leading to herniation of the endocyst membrane, disruption of its integrity, and rupture. Therefore, we postulate that this scenario in combination with other coexisting factors could be the possible mechanism for cyst rupture in group 1. This concept may also explain the pathogenesis of the high rate of rupture of pulmonary hydatid cysts. Accordingly, we consider these cysts a naturally occurring model for rupture, which should be treated surgically as soon as the diagnosis is made, to avoid complications.

Non-invasive determination by cardiovascular magnetic resonance of right ventricular-vascular coupling in children and adolescents with pulmonary hypertension.

BACKGROUND: Pediatric pulmonary hypertension (PH) remains a disease with high morbidity and mortality in children. Understanding ventricular-vascular coupling, a measure of how well matched the ventricular and vascular function are, may elucidate pathway leading to right heart failure. Ventricular vascular coupling ratio (VVCR), comprised of effective elastance (Ea, index of arterial load) and right ventricular maximal end-systolic elastance (Ees, index of contractility), is conventionally determined by catheterization. Here, we apply a non-invasive approach to determining VVCR in pediatric subjects with PH. METHODS: This retrospective study included PH subjects who had a cardiovascular magnetic resonance (CMR) study within 14 days of cardiac catheterization. PH was defined as mean pulmonary artery pressure (mPAP) ≥ 25 mmHg on prior or current catheterization. A non-invasive measure of VVCR was derived from CMR-only (VVCRm) and compared to VVCR estimated by catheterization-derived single beat estimation (VVCRs). Indexed pulmonary vascular resistance (PVRi) and pulmonary vascular reactivity were determined during the catheterization procedure. Pearson correlation coefficients were calculated between PVRi and VVCRm. Receiver operating characteristic (ROC) curve analysis determined the diagnostic value of VVCRm in predicting vascular reactivity. RESULTS: Seventeen subjects (3 months-23 years; mean 11.3 ± 7.4 years) were identified between January 2009-August 2013 for inclusion with equal gender distributions. Mean mPAP was 35 mmHg ± 15 and PVRi was 8.5 Woods unit x m2 ± 7.8. VVCRm (range 0.43-2.82) increased with increasing severity as defined by PVRi (p < 0.001), and was highly correlated with PVRi (r = 0.92, 95 % CI 0.79-0.97, p < 0.0001). Regression of VVCRm and PVRi demonstrated differing lines when separated by reactivity. VVCRm was significantly correlated with VVCRs (r = 0.79, CI 0.48-0.99, p <0.0001). ROC curve analysis showed high accuracy of VVCRm in determining vascular reactivity (VVCR = 0.85 had a sensitivity of 100 % and a specificity of 80 %) with an area under the curve of 0.89 (p = 0.008). CONCLUSION: Measurement of VVCRm in pediatrics is feasible. Pulmonary vascular non-reactivity may be contribute to ventricular-vascular decoupling in severe PH. Therapeutic intervention to maintain a low vascular afterload in reactive patients may preserve right ventricular functional reserve and delay the onset of RV-PA decoupling. Use of VVCRm may have significant prognostic implication.

[Up-to-date approaches in treatment of hydatid disease of the liver in childhood].

Sixty children (age 3-15 years) with hydatid disease of the liver were treated. The patients were divided into 2 equal groups (control and main groups).The hydatid disease of the liver was revealed in 22 (33.6%) patients, combined hydatid disease of the liver and lung were noted in 38 (66.4%) patients. The "capitonnage" of the residual cavity was applied in the control group and omentoplasty--in the main group. Chemotherapy with Nemazol (dose 10-15 mg/kg/day) was carried out. In order to reduce the negative influence of Nemazol on patients and to accelerate reparation processes of liver parenchyma Vobenzyme (2-3 pills/3 times/day) was applied. The number of complications was 23.2 % in the control group. The adhesive bowel obstruction was noted in 6.6% of patients, bleeding and jaundice in 3.3%, the residual cavity suppuration in 10%. Complications were registered in the main group in 6.6% of children (the adhesive bowel obstruction in 3.3%, preservation of the residual cavity after a year in 3.3%). The number of relapses in the control group was 6 (12%), there were no relapses in the main group. Thus the procedure of preoperative and postoperative chemotherapy allowed avoidance of the development of relapses of hydatid disease of the liver. Omentoplasty is the most rational method of treatment of residual cavity in surgery of hydatid disease of the liver in children.

Simultaneous heart and bilateral lung hydatid cyst operated in a single session.

A 48 year-old man was admitted to our hospital because of coughing with dispnoea and chest pain. On physical examination he showed marked respiratory difficulty, with a respiratory rate of 25 breaths per minute, and his jugular veins were mildly distended. Transthoracic echocardiography showed a cystic mass located in the diaphragmatic surface of the right ventricular wall without any protrusion into the ventricular cavity. These cardiac and also bilateral pulmonary hydatic cysts were demonstrated by thoracic CT imaging. There were three hydatic cysts which were located in the right middle lobe medial segment (20 mm × 20 mm) and two of them were located in the left lower lobe laterobazal segment (15 mm × 15 mm and 17 mm × 14 mm). Extracorporeal bypass via median sternotomy was used and all components of hydatid cysts in heart and lungs were removed in same session. Patient recovered well. So one-stage surgery by median sternotomy is an excellent approach for cardiac and lung cyst hydatid.

[The surgical treatment of hydatid disease of liver and lungs: the state of art].

Results of surgical treatment of 65 patients with the combined hydatid disease were analyzed. Lung invasion was combind with liver cysts in 57 patients, spleen -- in 2 patients, soft tissues of the thorax - in 2 patients and retroperitoneal cysts were found in 3 patients. Lung echinococcectomy with the use of minithoracotomy proved to be a method of choice. The access permits early recovery and shorter rehabilitation period, allowing to perform the second liver surgery on the 3--7th day after the first operation.

[The recurrent lung hydatid cysts in children].

The treatment results of 199 children with lung echinococcosis was performed. 78 (39.2%) had complicated forms of the disease. The organ-preserving operations were predominately performed: traditional echinococcectomy in 177 (89%) and videothoracoscopic echinococcectomy in 15 (7.5%). Lobe resection was performed in 2 patients. 5 patients cured without surgery thank to bronchial cyst evacuation. The long-term follow-up data were obtained from 163 (82%) of patients. Reccurense of the hydatid disease was registered in 8 (4.9%) patients. The study demonstrated the importance of immediate surgery after the cyst rupture with pleural dissemination and early recurrence diagnostics, which allows conservative treatment.

Giant viable hydatid cyst of the lung revealed by hiccups.

Hydatid cystic disease is still a significant clinical problem in endemic countries. Hydatid cysts are usually located in the liver and lung. The resulting large cysts in the lung are a special clinical entity called giant hydatid cysts. We present a case of this rare entity: An 18-year-old woman presented with three month history of hiccups and progressive dyspnea. Chest X-ray revealed a very large homogenous opacity of the left lung. A diagnosis of giant hydatid cyst was made intra operatively; the patient was treated surgically using cystotomy and capitonnage without post-operative complications. This report illustrates that the hydatid cyst of the lung may occasionally present with signs of mediastinal compression.

Immunoglobulin G subclass responses to recombinant Em18 in the follow-up of patients with alveolar echinococcosis in different clinical stages.

In this study, we compared the sequential responses of immunoglobulin G (IgG) subclasses to the diagnostic antigen Em18 in sera from patients with alveolar echinococcosis. A total of 225 sera from 36 patients at different clinical stages according to the WHO-PNM staging system were tested. The antibody responses were measured for cohorts with resected and unresected parasitic lesions by enzyme-linked immunosorbent assays (ELISA). Total IgG and, to a lesser extent, IgG4 antibody levels against Em18 correlated with all PNM stages before treatment, whereas levels of IgG2 were low and IgG3 was undetectable. Antibody kinetics, however, depended on the treatment rather than on the PNM stage. For some patients, after curative surgery, IgG1 antibodies dropped below the cutoff earlier than other antibodies, followed by total IgG and IgG4 within 18 months. For some patients with recurrences after surgery, IgG1 and IgG4 reappeared, whereas patients with unresectable lesions but stable disease showed steady declines in the levels of all antibodies, and IgG1 became undetectable in some patients. Additional testing of IgE responses to Em18 showed constantly low levels at all stages and in all cohorts.

Eighteen-year experience with echinococcosus of the heart: clinical and echocardiographic features in 14 patients.

We present a retrospective analysis of clinical symptoms and echocardiographic features in 14 patients having cardiac hydatic cysts and screened during the 18 last years, with surgical correlations in 13. The heart was the sole location of the cyst in six patients. The revealing symptoms were precordial pain (six patients), dyspnea (two patients). A systolic murmur of false pulmonic stenosis was present in three patients. In 13 patients, two-dimensional echocardiographic images of hydatic cysts were those of masses of liquid content with a well-contrasted capsule, which was single in eight patients and multiple in five, one of which had a honeycomb appearance. The mass was solid and calcified in one patient. The outlines of the cysts were particularly sharp on transesophageal echocardiograms (six patients). The cyst size ranged from 1.8 to 11 cm in diameter. Cysts were located in the intramyocardium in 10 patients (left ventricle in four patients, interventricular septum in four patients, right ventricle in two patients), the pericardium in three and the mediatinum in one. There were a Doppler pulmonary valve gradient in three patients. Computed tomography (eight patients) and coronary arteriography (seven patients) have no additional diagnosis value than echocardiography. Nuclear magnetic resonance imaging (three patients) was helpful in determining extracardiac extension of the cysts. Transthoracic and transesophageal two-dimensional echocardiography have a central role in diagnosing cardiac hydatic cysts.