Kyste hydatique du cœur au CHU Erriadh de Constantine (Algérie). Étude rétrospective de 2008 à 2009

The hydatic disease is cosmopolitan and is due to the development of the larva of a small tapeworm called Echinococcus granulosus. Although rare, today, there are many cases of hydatic cyst of the heart. Echocardiography and other physical examinations reveal the fluid collection and also specify its exact location on the heart. Our study focused on patients undergoing surgery for hydatic cysts of the heart during the years 2008 and 2009 in Erriadh teaching hospital, where we have collected seven cases over this period. The young average age of patients, 18 years, favors a high incidence. The sex ratio is 0.14. All the heart walls and cavities were the site of hydatic development. In addition, serology for primitive cardiac hydatidosis provided very low levels of antibodies. The cystic echinococcosis remains a scourge. The cardiac localization remains a very severe disease. The management of patients is very complex. The hydatic cyst of the heart affects teenagers and young adults. It is the main cause for long-term, major complications. In patients from endemic areas and for any patient who developed hydatidosis, it is essential to conduct a systematic search for localization by cardiac echocardiography. This simple measure can manage patients more quickly and avoid complications with socioeconomic consequences.     

Flubendazole in human Echinococcus granulosus hydatidosis. Preoperative care: parasit-pharmacologic study

The effect of flubendazole in human hydatic disease due to E. granulosus has been studied in 22 patients with one or several hydatic cysts of different sites. Flubendazole has been given orally before surgery for a 10 days mean period, at 4 g daily in adults and 1 g daily in children. Flubendazole has been titrated by radioimmuno-assay in sera, urines and bile taken 12 to 24 hours after the last ingestion and in the hydatic cyst (membranes and liquid). The scolex vitality was determined by direct optic microscopy and by intra-peritoneal mouse inoculation. Seven non-treated patients, without hydatic disease, were considered as controls. The flubendazole concentrations in sera (3.01 +/- 3.73 ng/ml) and in urines (7.6 +/- 7.5 ng ml) are low. They are also low in membranes (3.21 +/- ng/mg) and in hydatic liquid, but significantly more elevated in the hepatic localisation than in the pulmonary localisation. The bile concentrations are high (167.9 +/- 133.2 ng/ml). There is no correlation between these concentrations and the amounts of flubendazole administered. The scolex vitality was correlated neither with the amounts of flubendazole administered, nor with the concentrations of flubendazole in the hydatic cyst. Further studies are necessary before judging of the efficacy of flubendazole in the human hydatic disease.     

Spinal hydatidosis. Failure of albendazole

A recession of eighteen months is not enough to evaluate efficacy of albendazole therapy on spine hydatic cyst in spite of a negative specific serology by HAI and electrophoresis. Specific IgE and precipitates system number 5 seem not good tests for the following of evolution. Inoculation of mouse with bone biopsy is an interesting test to evaluate the viability of the cyst.     

Hydatid cyst of the thyroid. Apropos of a case in the Republic of Niger

Thyroid echinococcosis has been rarely found, especially in areas where hydatic cysts are rarely encountered. The authors report on a case of thyroid echinococcosis in a patient living in sahelian Africa (Niger), which is classically known as a region of low endemia for hydatic disease.     

A seminal vesicle cyst complicated with a tumor like nodular mass of benign proliferating prostatic tissue: a case report with ultrastructural and immunohistochemical studies

We report a seminal vesicle cyst complicated with a tumor-like nodular mass of benign proliferating prostatic tissue. The patient was a 53-year-old Japanese man. A cyst of approximately 4.5 cm in diameter was discovered at the left seminal vesicle area. In the inner part of the cyst, a papillary nodular mass of 0.7 cm in diameter was seen. Under the clinical diagnosis of a seminal vesicle cyst with a tumorous mural nodule, the patient underwent resection of the seminal vesicle cyst to rule out the possibility that the nodular mass in the cyst was a neoplasm of an especially malignant nature. Microscopic examination of the excised specimen revealed a small dome-like nodular mass on the luminal surface of the cyst consisting of nodular proliferation of benign tubular gland tissue with various configurations. Conventional histologic, immunohistochemical, and ultrastructural analysis showed the proliferating cells in the nodular mass consisted of the benign prostate type. It is extremely important to differentiate between a benign proliferation and a malignant one, when the nodular mass is found in the seminal vesicle cyst.     

Tracheopathia osteochondroplastica associated with a liver hydatic cyst broken in bronchi

Tracheobronchopathia osteochondroplastica (TBOC) is an unusual respiratory disorder characterized by cartilaginous or bony mucosal nodules in the tracheobronchial tree. It mainly affects men over 50 years old and clinical manifestations are observed when obstructive or infectious complications occur. We report a case of tracheopathia osteoplastica of the upper third of the trachea in a young 35-year-old mal, fortuitously discovered while exploring a biliptysis caused by a broken hydatic cyst of the liver. TBOC can be associated with various metabolic, inflammatory and neoplastic disorders, but its pathogenesis remains unknown. A metaplastic origin is actually the main hypothesis proposed.     

Case report and review: alloimmunization, delayed hemolytic transfusion reaction, and clinically significant anti-Yt(a) in a patient with Beta-thalassemia/sickle cell anemia

A 26-year-old female with Beta-thalassemia/sickle cell anemia was admitted to the hospital with symptoms of a painful crisis. During the next 4 days her hematocrit decreased to 13 percent, and there was reticulocytopenia. She was transfused with four units of red blood cells that were microscopically incompatible, and the hematocrit increased to 29 percent. Eight days later the patient was readmitted with back pain, hemoglobinuria, and a hematocrit of 27 percent. Anti-E, -c, -Jka, and -Yta were identified. The direct antiglobulin test was positive, and the eluate contained anti-c and -Jka. The patient's hematocrit continued to decrease to 14 percent. Transfusions were withheld and the patient recovered uneventfully. Separate 51Cr red blood cell survival studies showed significantly shortened survival of both autologous and R(1)R(1), Jk(a-), Yt(a+) erythrocytes. This case illustrates the complexity of transfusion management in hemoglobinopathy patients.     

Comparative study of nitric oxide (NO) production during human hydatidosis: relationship with cystic fluid fertility

Human hydatidosis is characterized by a prolonged coexistence of Echinococcus granulosus and its host without effective rejection of the parasite. This parasitic infection constitutes a major health problem in Algeria. In this study, we investigated in vivo production of nitrite (NO₂ ^? + NO₃ ^?) in sera of Algerian patients carrying different cyst locations. Nitrite (NO₂ ^? + NO₃ ^?) levels were evaluated by the Griess method. Our results indicated that the levels of nitrite were significantly higher in the sera of hydatic patients than those of healthy controls supporting the involvement of nitric oxide (NO) in antihydatic action. The levels of nitrite in sera of the patients with hepatic hydatidosis were significantly higher than those with pulmonary infection. The lower serum (NO₂ ^? + NO₃ ^?) levels were observed in the relapsing cases. In addition, (NO₂ ^? + NO₃ ^?) levels of fertile hydatic fluids were significantly higher compared to infertile fluids. Our results suggest that the presence of NO products in hydatic fluids seems to be related to the location and the fertility of hydatic cysts. The assessment of protein concentration in hydatic fluids showed that the concentration of proteins was not exclusively dependent on the fertility but on the cyst locations. The assessment of (NO₂ ^? + NO₃ ^?) production in hydatic patients may be a useful tool to evaluate effector mechanisms of NO and clinical manifestations.     

Sickle cell anemia: conclusions from a forensic case report of a young African woman who died after anesthesia

A 20-year old African woman underwent anesthesia for interruption of an unwanted pregnancy. As a consequence of the anesthesia, she went into coma because of an as yet unknown and untested homozygotic state of sickle cell anemia. Her vital functions were maintained for more than 1 year by intensive medicine, but she died finally in multiorgan failure and aspiration pneumonia. Because of the complications under anesthesia and the missing preanesthetic test for hemoglobinopathy, autopsy was conducted in the forensic medicine department and not in the department of pathology. The sickle cell disease was diagnosed by electrophoresis of the blood, by molecular detection of mutation in the hemoglobin gene, as well as by postmortem light and electron microscopy. Sickle cells were found in capillaries of brain, liver, lung, bone marrow, and spleen. Electrophoretic analysis revealed 80.2% HbS in addition to 3.2% HbA₂ and 16.6% HbF, whereas no HbA₀ could be detected in blood, confirming the homozygosity of sickle cell anemia. Because of sickle cell crisis, occluded blood vessels, and severe brain cortex necrosis, the patient died in spite of reanimation and intensive medicine. This case demonstrates that it is still important to realize the possibility of this disease and diagnostic obstacles even in regions where its manifestation is not endemic, as in Northern and Central Europe.     

Adenosquamous carcinoma in a congenital choledochal cyst associated with pancreatico-biliary maljunction

Congenital choledochal cyst is occasionally complicated by carcinomatous transformation, mostly adenocarcinoma. Adenosquamous carcinoma arising in a congenital choledochal cyst is very rare. The author herein reports an adenosquamous carcinoma arising in congenital choledochal cyst associated with pancreatico-biliary maljunction. A 34-year-old man with congenital choledochal cyst and recurrent cholangitis had been followed up, and was admitted to hospital to undergo testing for cancer. Imaging modalities including computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiography showed an elevated lesion in the choledochal cyst. Because clinical cytology of bile indicated malignant cells, pancreatico-duodenectomy, cholecystectomy, and resection of the choledochal cyst were performed. Grossly, the choledochal cyst was type I, and its size was 8 × 10 cm. Anomalous pancreatico-biliary ductal union was recognized. An elevated lesion was recognized in the choledochal cyst. Histologically, the lesion was composed of a squamous cell carcinoma element and an adenocarcinoma element; a gradual transition was recognized between the two. The squamous cell carcinoma element contained microcytic cells with mucins. On immunohistochemistry the adenocarcinoma element and microcytic cells were positive for CEA, but the squamous cell carcinoma element was negative for CEA. Both elements were positive for CA19-9. Ki-67 labeling was 53% in the adenocarcinoma element and 48% in the squamous cell carcinoma element. p53 protein was negative in both elements. At the time of writing, the patient was alive after 25 months without recurrence or metastasis. The present case is the second case of adenosquamous carcinoma arising in congenital choledochal cyst in the English-language literature.     

Pure red-cell aplasia and autoimmune hemolytic anemia in a patient with acute hepatitis A

Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report herein a case of AHA complicated by both PRCA and AIHA. A 49-year-old female with a diagnosis of AHA presented with severe anemia (hemoglobin level, 6.9 g/dL) during her clinical course. A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. Her anemia and liver function test were completely recovered by 9 months after the initial presentation. We review the clinical features and therapeutic strategies for this rare case of extrahepatic manifestation of AHA.     

Trial therapy of inoperable abdominal hydatid cysts by puncture

Hydatidosis is a major public health problem in Tunisia. The infection rate in the normal population is over a 100 times higher than in the generally accepted hyperendemicity level (1,720 in 100,000 as against 13 in 100,000!). The use of drugs of benzimidazole derivates prevents the development of scolices into hydatic cysts and allows to try a non-surgical cure by the punction of hydatid cyst of the sheep liver, i.e. aspiration of the cyst fluid and injection of hypertonic salt solution into the drug treated animal. The good results obtained in a group of 16 animals permitted us to apply this method to two patients whose conditions made surgical intervention dangerous. The results although excellent in the case of a secondary peritoneal cyst were less spectacular in the case of secondary liver cyst of whose wall showed signs of involution.     

Praziquantel in human hydatidosis. Evaluation by preoperative treatment

The efficacy of praziquantel has been studied in human hydatic disease due to E. granulosus, using parasitological and pharmacological criteria of improvement. This evaluation uses a prospective therapeutic trial in 15 patients with one or several hydatic cysts in different sites. Before surgery, nine of these received daily 75 mg/kg of praziquantel in 2 courses of 10 days each. The other six patients are considered as controls. The protoscolices vitality is determined by direct optic microscopy and by intraperitoneal mouse inoculation. According to the results of optic microscopy, praziquantel sterilizes hydatic cysts: 19 sterilized cysts out of 26 coming from treated patients, and 4 out of 11 in control group. This effect seems to be emphasized in hepatic localization. Nevertheless, according to the results of mouse inoculation, praziquantel does not significantly reduce the pathogenicity of inoculated proscolices and germinal layer: 11 mice out of 39 are healthy in the treated group, and 5 out of 15 in controls. Moreover, the determination with a fluorimetric method has not detected praziquantel in the hydatid liquid issued from treated patients. The drug does not pass through the cyst wall, and so, cannot have a scolicidal activity. Praziquantel cannot be considered as a medical treatment in human hydatidosis.     

Red blood cell distribution width index in some hematologic diseases

The red blood cell distribution width index (RDW) was determined in a group of anemic male patients and normal male blood donors. Elevated mean RDW values were found in the anemic patients, with the highest value seen in sickle cell anemia, sickle cell-beta thalassemia, sickle cell trait, beta-thalassemia trait, and iron deficiency in decreasing order of magnitude. The mean RDW of the normal male subjects was 11.3. It was found that the RDW was proportional to the reticulocyte count, with the highest values in the patients with the highest reticulocyte count (sickle cell anemia). One clinical value of the RDW therefore may lie in its capacity for reflecting active erythropoiesis. For example, patients with normal or near-normal hemoglobin and with high RDWs may be suspected of having an elevated reticulocyte count that may indicate a hemoglobinopathy, such as sickle cell trait or thalassemia trait.     

Unrelated Donor Allogeneic Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies Using a Reduced-Intensity Conditioning Regimen and Third-Party Mesenchymal Stromal Cells

Allogeneic hematopoietic stem cell transplantation for patients with a hemoglobinopathy can be curative but is limited by donor availability. Although positive results are frequently observed in those with an HLA-matched sibling donor, use of unrelated donors has been complicated by poor engraftment, excessive regimen-related toxicity, and graft-versus-host disease (GVHD). As a potential strategy to address these obstacles, a pilot study was designed that incorporated both a reduced-intensity conditioning and mesenchymal stromal cells (MSCs). Six patients were enrolled, including 4 with high-risk sickle cell disease (SCD) and 2 with transfusion-dependent thalassemia major. Conditioning consisted of fludarabine (150 mg/m²), melphalan (140 mg/m²), and alemtuzumab (60 mg for patients weighing > 30 kg and .9 mg/kg for patients weighing <30 kg). Two patients received HLA 7/8 allele matched bone marrow and 4 received 4-5/6 HLA matched umbilical cord blood as the source of HSCs. MSCs were of bone marrow origin and derived from a parent in 1 patient and from an unrelated third-party donor in the remaining 5 patients. GVHD prophylaxis consisted of cyclosporine A and mycophenolate mofetil. One patient had neutropenic graft failure, 2 had autologous hematopoietic recovery, and 3 had hematopoietic recovery with complete chimerism. The 2 SCD patients with autologous hematopoietic recovery are alive. The remaining 4 died either from opportunistic infection, GVHD, or intracranial hemorrhage. Although no infusion-related toxicity was seen, the cotransplantation of MSCs was not sufficient for reliable engraftment in patients with advanced hemoglobinopathy. Although poor engraftment has been observed in nearly all such trials to date in this patient population, there was no evidence to suggest that MSCs had any positive impact on engraftment. Because of the lack of improved engraftment and unacceptably high transplant-related mortality, the study was prematurely terminated. Further investigations into understanding the mechanisms of graft resistance and development of strategies to overcome this barrier are needed to move this field forward.     

Increased circulating levels of soluble HLA class I heterodimers in patients with sickle cell disease.

This study examined the presence of a persistent state of low-grade inflammation in sickle cell anemia patients by measuring circulating sHLA-I heterodimers and C-reactive protein during the steady state and after recent crises. Thirty-nine pediatric sickle hemoglobinopathy patients were studied during the steady state and 11 patients were evaluated within 1 month of a painful crisis. A disease severity score was generated for each patient, and soluble HLA-I (sHLA-I) and C-reactive protein levels were determined. Soluble HLA-I was significantly elevated in 55% of the steady-state group and in 36% of the recent-crisis group. The percentage of patients with elevated sHLA-I differed in the various disease subgroups in the steady state: 46% of Hb SS patients, 70% of Hb SC patients, 75% of Hb S beta-thal patients, and 20% of Hb SSF patients. Steady-state and recent-crisis sHLA-I levels were not significantly different. C-reactive protein levels were elevated in 11% of steady-state patients and in 9% of recent-crisis patients. Soluble HLA-I levels did not correlate with C-reactive protein levels or disease severity score, age, hemoglobin, reticulocyte count, platelet count, or white cell count. These results show that the majority of sickle hemoglobinopathy patients have elevated sHLA-I levels during the steady state and after recent crisis, suggesting the presence of chronic inflammation during the steady state.     

Glucose-6-phosphate dehydrogenase deficiency in Mali. Epidemiology and pathological aspects

I,858 semi quantitative G6PD determinations were done in Mali (mostly in the Point G Hospital, in Bamako). 15.7% of the men and 4.5% of the women had a G6PD deficiency. The age did not affect the incidence of the disease. G6PD deficiency was more frequent among the Sonrais than among the other ethnic groups tested. It did not occur more frequently among subjects having the sickling trait of AC hemoglobinopathy but no G6PD was found among subjects having a major hemoglobinopathy (SS or SC). Severe hemolytic anemia was rather infrequent among adults subjects. Hemolysis was induced by drugs--such as dapsone or niridazole--but it was usually mild and time-limited.     

Red cell distribution width in sickle cell disease

Red cell distribution width (RDW), an electronically determined index of anisocytosis, was examined in 60 patients with sickle cell anemia (Hb SS), 28 patients with hemoglobin sickle cell (SC) disease, and seven patients with sickle cell-beta(+) thalassemia (S-thal). All patients were adults and in the steady state of their disease. The RDW was greater in sickle cell patients than in 39 healthy, age and race matched controls without hemoglobinopathy (Hb AA). Patients with sickle cell anemia had higher mean RDW than those with Hb SC disease or with S-thal. The mean RDWs in the latter two disorders were not significantly different. In SS patients, the RDW correlated significantly with the degree of anemia and reticulocytosis. A group of 18 SS patients was studied while in acute painful crisis. Their mean RDW was not different from that in the steady state. Mean WBC and red cell volume, however, were significantly higher during pain crisis.     

Clinical and molecular characterization of novel deletions causing epsilon gamma delta beta thalassemia: Report of two cases

Epsilon gamma delta beta (εγδβ)⁰ - thalassemia is a very rare disorder that results from large deletions in the β-globin gene cluster which abolish all regional globin chain gene expression from that allele. Since it is an exceedingly rare cause of neonatal anemia and is not detected by routine newborn screening, it is usually not suspected clinically and commonly undiagnosed or misdiagnosed. In this study, we describe two patients diagnosed in our hospital with (εγδβ)⁰-thalassemia based on the results obtained from DNA microarray analysis of their peripheral blood. The first patient of mixed European descent presented as a neonate with microcytic hemolytic anemia, hyperbilirubinemia, hypoglycemia and hypothermia, and was found to have a 2.2 Mb loss that included the entire β-globin gene cluster and the locus control region (LCR). The second patient, also of mixed European descent, presented in the neonatal period with anemia, thrombocytopenia and cutaneous extramedullary hematopoiesis, and was found to have a 59 kb loss that included the β-globin LCR, HBE1, HBG1, and HBG2 genes. Both cases highlight the importance of recognizing the clinical features of (εγδβ)⁰-thalassemia and implementing appropriate testing to clarify the diagnosis and manage the condition.     

Compressive neuropathy of the tibial nerve and peroneal nerve by a Baker's cyst: case report

We report a case of Baker's cyst that induced compression of both the tibial and common peroneal nerves. The patient presented with calf atrophy and foot drop over a 6-month period. These signs and symptoms could have been mistaken for those of spinal origin. Based on an electrodiagnostic study and magnetic resonance imaging, compression of nerves by an asymptomatic Baker's cyst measuring 6x4 cm was confirmed. This cyst communicated with the articular joint which was also associated with a medial meniscal lesion. We treated the patient arthroscopically by performing partial medial meniscectomy, and through the posterolateral and the posteromedial portal, decompression of the Baker's cyst was performed. Approximately 6 weeks after the arthroscopic decompression, the cyst recurred. Therefore open resection was performed. At 1-year follow-up, the patient had considerable improvement in motor as well as sensory function and showed no evidence of recurrence. Although the electrodiagnostic studies showed an improvement in symptoms, the patient continued to complain of lower leg weakness owing to delayed diagnosis and cyst decompression. We believe that Baker's cysts should also be considered in the differential diagnoses of patients who present with neuromuscular dysfunction in the calf and leg.