Molecular genotyping of Echinococcus granulosus using formalin-fixed paraffin-embedded preparations from human isolates in unusual tissue sites.

Cystic echinococcosis (CE) caused by Echinococcus granulosus remains a serious problem worldwide for issues relating to public health and the economy. The most predominantly affected sites are the liver and the lungs, but other organs such as the heart, the spleen and the peritoneum can also be infected. Access to cysts from uncommon sites has limited genomic and molecular investigations. In the present study, genotypes of E. granulosus sensu lato were identified from formalin-fixed paraffin-embedded tissues (FF-PETs) implicated in human CE. Tissue samples were obtained from 57 patients with histologically confirmed CE. DNA samples were analysed using Egss 1 polymerase chain reaction (PCR) specific to the mitochondrial 12S rRNA gene of E. granulosus sensu stricto. All cysts were typed as E. granulosus sensu stricto with up to 35% of the liver and 16.6% of lungs being the most frequently infected, and up to 48.4% of samples being from rare sites. No correlation was found between cyst site and either the gender or the age of patients. This study demonstrates the possibility of exploiting atypical cysts using FF-PET samples and highlights the predominance of E. granulosus sensu stricto species in the Tunisian population, even in unusual infection sites.

[Visceral leishmaniasis in immunocompetent adults. About six cases]. / La leishmaniose viscérale de l'adulte immunocompétent. À propos de six cas.

OBJECTIVE: Studying the epidemiological variations of visceral leishmaniasis in Tunisia and proving the importance of parasitological investigations to raise the diagnosis. PATIENTS AND METHODS: Six patients hospitalised during the period between January 1998 and January 2009 at Fattouma Bourguiba Teaching Hospital in Monastir, five men and an only one woman, aged from 26 to 70 years old, originating from the central and eastern regions of the country. Epidemiological, clinical, biological and therapeutic data were obtained from the patient's medical files. RESULTS: The major clinical symptoms were fever, weakness and spleen enlargement. Biological data revealed the presence of anaemia in every case and leucopoenia associated or not associated with thrombopenia in four cases. The diagnosis of visceral leishmaniasis was confirmed by the identification of the parasite in the blood or in the bone marrow. All patients were treated with two courses of antimoniate of meglumine separated by a 6-week interval. The outcome was positive and the patients were cured. CONCLUSION: Visceral leishmaniasis is increasing among adults in Tunisia. Moreover, it is spreading outside its epidemiological area in the north to reach the central and southern regions. It should be raised when fever and spleen enlargement occur. Biological data are hardly specific. Diagnosis is based on finding the parasite in human fluids, mainly by molecular techniques. The rapid establishment of a specific treatment is vital.

[Retrospective study of the distribution and the fertility of hydatid cysts in the child in Tunisia]. / Étude rétrospective de la distribution et de la fertilité des kystes hydatiques chez l'enfant en Tunisie.

PURPOSE OF THE STUDY: Our aim was to study the distribution and the fertility of the hydatid cysts in function of the age and the sex of patients and to identify the strain(s) responsible(s) of the children hydatidosis. PATIENTS AND METHODS: We have analyzed a total of 241 cysts coming from 195 children aged 2 to 16 years operated in the CHU F. Bourguiba of Monastir during the period from November 1999 to December 2009. For each cyst, the localization and the fertility of the métacestode as well as age, sex and origin of the patient are listed. Identification of strains was carried out by PCR/RFLP and has targeted the ribosomal gene ITS1. RESULTS: The lung was the primary localization of cyst (61.8%) followed by the liver (34.85%). The greatest number of cases is observed in the age groups 4-9 years (138 cases) where children's infection is more frequent in the male than in the female sex. The fertility of the cyst was independent of its site or its size and no incidence of age of children was detected. The G1 sheep strain is responsible for the contamination of children. CONCLUSION: The cystic echinococcosis described as a young adult disease may actually observed at any age and remains a serious problem of public health in Tunisia.

[Myalgia in familial Mediterranean fever]. / Myalgies au cours d'une fièvre méditerranéenne familiale.

INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of fever and painful episodes of sterile peritonitis, pleuritis and arthritis. Among rare symptoms of the disease, muscular manifestations, first described in 1945, sometimes as one of the main clinical manifestations or as its sole feature should be recognized. We present a patient with FMF in whom severe myalgia were predominant. CASE REPORT: An 18 year-old Tunisian boy treated with corticosteroids for an "inflammatory myopathy" in another institution was admitted for abdominal pain. FMF was suspected because of a history of paroxysmal abdominal pain with fever from the age of 5 leading two times to laparotomy and one attack of left knee arthritis at the age of 14. FMF diagnosis was confirmed genetically, corticosteroids were tapered and a treatment with colchicine was started. Two years and a half later, he was admitted for severe and incapacitating myalgia of the upper and lower limbs without fever nor abdominal pain that responded well to rest and colchicine. Myalgia was then definitively attached to FMF. CONCLUSION: Three clinical patterns of myalgia are now well identified in FMF: the spontaneous pattern as observed in our patient, the exercise-induced pattern and the protracted febrile myalgia syndrome. The three patterns differ in the severity of pain, grade of fever and duration of the episode.

[Cystic echinococcosis in children in Tunisia: fertility and case distribution of hydatid cysts]. / L'échinococcose hydatique de l'enfant en Tunisie: fertilité et localisation des kystes.

Cystic echinococcosis, which commonly starts during childhood or adolescence, is a serious problem of public health in Tunisia. For 121 children (161 cysts), the localization and fertility of cysts as well as viability of their protoscoleces were determined. Results indicated that the lung was the primary localization of cyst (59%) followed by the liver (35%). Children's infection is more frequent in male than in female (sex ratio 1.96) and the greatest number of cases is observed in the 4-9 year age groups (94 cases). The fertility of the cyst was independent of its site or its size and no incidence of age of children was detected. Nevertheless, the fertility rate is higher in females than in males for the liver localization.

[Takayasu's arteritis associated with acute myeloblastic leukemia]. / Association artérite de Takayasu et leucémie aiguë myéloblastique.

INTRODUCTION: We present one patient with acute myeloblastic leukemia diagnosed two months after the onset of Takayasu's arteritis. EXEGESIS: A 21-year old woman with a previous history of erythema nodosum and episcleritis was admitted for a left cervical mass. Diagnostic imaging showed an aneurism of the left extracranial internal carotid and a stenosis of the left subclavian artery. Histological findings of the carotid aneurism revealed a granulomatous giant cell arteritis consistent with Takayasu's arteritis. Two weeks after, she was discharged, elevated white cell count (440.000/mm3 ) was disclosed. A bone marrow aspirate documented an acute myeloid leukemia. The patient died of intracerebral hemorrhage. CONCLUSION: Leucocytoclastic vasculitis and polyarteritis nodosa occur in acute myeloid leukemia, but the association with Takayasu's arteritis is new. In our knowledge, only two documented cases of Takayasu's arteritis in association with acute myeloblastic leukemia have been published.

Genetic differences between Tunisian camel and sheep strains of the cestode Echinococcus granulosus revealed by SSCP.

Ovine and dromedary Echinococcus granulosus isolates from Tunisia were identified as G1 and G6 strains based on polymorphism of the mitochondrial cytochrome C oxydase CO1. Single strand conformation polymorphism (SSCP) was used in order to examine the genetic variation within and between Tunisian G1 and G6 strains and to estimate the extent of selfing. The dromedary isolates are genetically distinct from sheep isolates (high value of genetic variation between populations: Fst= 0.46). No significant deficiency in heterozygotes was found in sheep isolates, whereas heterozygote deficiency (suggesting selfing) was found in a limited number of camel isolates.

[Behçet's disease in Tunisia. Clinical study of 519 cases]. / La maladie de Behçet en Tunisie. Etude clinique de 519 cas.

PURPOSE: To describe clinical characteristics of Behçet's disease in Tunisia. METHODS: It's a retrospective and multicentric study conducted by the Tunisian society of internal medicine. Inclusion criteria were those of the international study group. Were also included patients without international study group criteria but with at least one manifestation among arthritis, venous thrombosis or neurological manifestation with oral and genital ulceration or oral ulceration and skin lesions. RESULTS: Five hundred and nineteen patients were included. 87.5% of them fulfilled the international criteria. The male to female ratio was 2,7. The mean age was 28.7+/-9.3 years at onset and 32.7+/-9.2 years at diagnosis. The incidence of each manifestations was as follows: oral ulcers: 100%, genital ulcers: 87.5%, pseudo-folliculitis: 67.6%, erythema nodosum: 17.5%, positive pathergy test: 51%, joint involvement: 55%, uveitis: 32.2%, vein thrombosis: 24.9%, arterial aneurysms: 3.9%, neurological involvement: 11.6%. The frequency of HLA B51 antigen was 35% among the 187 patients tested. There was no difference in the manifestations of the disease between patients having B51 and those lacking it. Venous thrombosis (29.8 vs 11.4%), arterial involvement (4.4 vs 1.4%) and uveitis (37.5 vs 17,9%) were significantly more frequent in men whereas erythema nodosum (22.9% vs 15.6%) and joint involvement (70,7 vs 49.9%) more frequent in women. The mean follow up was 6,1+/-5.7 years. Mortality rate was 2.3% in our series. CONCLUSION: Our study confirms the androtropism of the disease in Mediterranean and Middle east countries. Positive pathergy test and venous thrombosis were more frequent in our study, like those from Mediterranean region. Whereas, ocular and neurological involvement were quite less frequent in our series.

Molecular evidence of ovine (G1) and camel (G6) strains of Echinococcus granulosus in Tunisia and putative role of cattle in human contamination.

Three hundred and seventy-two cysts coming from 50 humans, 166 cattle, 153 sheep and 3 camels were collected in order to establish some epidemiological molecular information in Tunisia for the first time. The analysis by PCR-RFLP of ITS1 sequence showed that all the human, ovine and bovine cysts were due to the common sheep strain of Echinococcus granulosus. The sequencing of the CO1 gene of 37 isolates confirm the G1 genotype of this strain. For seven of these isolates, we found the mutation C56T which is present in the three principal intermediate hosts: human (three cysts), cattle (three cysts) and sheep (one cyst). With regard to the G1 genotype, we identified three other point mutations. The camel strain G6 is uniquely found in the three camels isolates and not in the other intermediate hosts analysed. The fertility of the bovine cyst represents 48% that means that this host is involved in a bovine-dog cycle and consequently represents a reservoir of sheep strain in Tunisia. Our results confirm the importance of the prophylaxis measures in order to disrupt the cycle of transmission sheep-dog in Tunisia. Nevertheless, the supervision of bovine infection should be reinforced because this intermediate host may constitute an important link with the human contamination.

Genetic relationships between sheep, cattle and human Echinococcus infection in Tunisia.

Allozyme variation at seven polymorphic loci (GPI, EST, MDH, MPI, DIA, PEP, PGM) was studied to examine genetic variation within and between sheep, cattle and human populations of Echinococcus granulosus in Tunisia. A high degree of genetic similarity was shown between the cysts of the three host origins. Nevertheless, whereas, the ovine and human samples were highly similar, the cattle samples were slightly different genetically. We conclude that humans are mostly infected by parasites originating from sheep liver. The intense deficiency in heterozygotes was partly artefactual (Wahlund effect) and partly due to self-fertilisation.

Massive pleural effusion in systemic lupus erythematosus: thoracoscopic and immunohistological findings.

Small to moderate, bilateral pleural effusions are common during the course of systemic lupus erythematosus (SLE). These are related to several complications, particularly, congestive heart failure, nephrotic syndrome, pulmonary embolism or SLE itself. Thoracoscopy performed for a massive unilateral pleural effusion in a patient with SLE and inferior vena cava thrombosis revealed several small nodules on the visceral pleura. Immunofluorescence studies of biopsy samples showed immunoglobulin deposits confirming the lupus-related origin of the pleuritis.

[Wegener's granulomatosis and pregnancy. A case]. / Granulomatose de Wegener et grossesse. Une observation.

Pregnancy concomitant with Wegener's granulomatosis is extremely rare: so far, only four cases have been published. The authors report a fifth case where the disease appeared during the post-partum period, a situation which has already been noted in two of the published cases. Interruption of a subsequent pregnancy was followed by a flare-up of the disease resulting in the patient's death. This suggests that post-partum and post-abortum are probably instrumental in the onset and deterioration of Wegener's granulomatosis. The two patients previously reported who received immunosuppressants combined with corticosteroids had no flare-up after delivery. It seems permissible to prescribe such a therapeutic combination before and after delivery or abortion, especially since the fear of foetal toxicity from these drugs seems to be exaggerated.

[Multiple hydatid cysts of the thigh: the role of magnetic resonance imaging]. / Hydatidose multiple de la cuisse: place de l'imagerie par résonance magnétique.

Hydatid cysts of the thigh are uncommon in our country despite a high frequency of hydatic disease which is estimated to affect 5.33% of the general population. Diagnosis is usually made late in the clinical course. Ultrasonography, CT scan and magnetic resonance imaging are the most helpful diagnostic tools. We report a case of a 76-year-old female smoker who had undergone surgery for a hydatic cyst of the right lung at the age of 56 years. In June 1997, the patient was admitted for decompensation of chronic obstructive lung disease. Physical examination revealed a 6 cm painless mass in the right thigh which had developed progressively over the last year. Ultrasonography showed multiple hydatic cysts. Magnetic resonance imaging confirmed the diagnosis, showing interconnected multivesicular cystic formations. Medical treatment was given due to respiratory failure and patient refusal of a surgical procedure.

[Pulmonary embolism of hydatic origin]. / Embolie pulmonaire d'origine hydatique.

BACKGROUND: The right ventricle is an exceptional localization for hydatic cysts. There is a risk of hydatic embolism and chronic or acute cor pulmonale. CASE REPORT: A 63-year-old-man with an uneventful history was hospitalized for dry cough, exercise-induced dyspnea and bloody expectorations which had developed over the previous year. Multiple and bilateral opacities were visualized on the standard chest x-ray and the right border of the heart showed a bulge in the middle portion. Signs of right-sided hypertrophy were seen on the ECG. Imaging findings led to the diagnosis of multiple organ hydatiasis involving the lung, the liver the mediastinum and a ruptured hydatic cyst in the right ventricle. The cavogram revealed defect images in the superior vena cave and the pulmonary angiogram confirmed the diagnosis of hydatic embolism. Medical treatment was given but the patient died 8 months after diagnosis. DISCUSSION: Hydatic pulmonary embolism generally occurs after rupture of a hydatic cyst in the right ventricle or due to venous migration of daughter vesicles to the right heart then the pulmonary artery. Clinical manifestations are not specific although hemoptisy is the most frequent sign. Positive diagnosis, guided by echocardiographic findings, is based on the pulmonary arteriogram. Prognosis is particularly poor and depends of the patient's general status as well as the number and size of the embolized vessels. Survival rate is poor. Open heart surgery is indicated in localized forms.

[Costal chondrosarcoma. 4 cases]. / Chondrosarcome costal. 4 observations.

BACKGROUND: Tumors of the rib cage are uncommon and malignant in 29% of the cases. Chondrosarcoma predominates, accounting for 40% of all cases of malignant costal tumors. CASE REPORTS: Four patients (3 women, 1 man, mean age 28.2 years) were hospitalized for costal chondrosarcoma. Pain and tumefaction dominated the clinical presentation. Calcifications suggested the diagnosis in 3 cases. Curative surgery was performed in all cases. Postoperative radiotherapy was unable to improve prognosis in 2 patients. DISCUSSION: Chondrosarcoma of the ribs is characterized by a strong potential for invasive extension. Diagnosis is suspected on the basis of imaging findings and confirmed at pathology. Surgery is required. Chemotherapy and radiotherapy do not improve prognosis significantly.

[Chronic septic granulomatosis in a young girl manifesting as invasive pulmonary aspergillosis and flaccid paraplegia]. / Granulomatose septique chronique chez une fillette revelee par une aspergilose pulmonaire invasive et une paraplegie flasque.

UNLABELLED: The invasive Pulmonary Aspergillosis (IPA) is the principal cause of death in patients with chronic granulomatosis disease (CGD). It can happen before age of one and can reveal the (CGD). Usually, the transmission of GSD is linked to x in 65% of cases. We report, the case of a girl aged 3 years issued from consanguine marriage referred to hospital for hemoptysia. We note that at 40 days she presents an suppurate adenitis. She is hypotrophic at--3DS without respiratory problem, she have a splenomegaly. The radiography chest note an alveolar and interstitial opacity bilateral, apical right and basal left. There is a biological inflammatory syndrome. During the evolution, the child presents a paraplegia secondary to compression myelitis. A D5 laminectomy in urgency was done. The vertebral Biopsia, the serology and the culture isolate fumigatus aspergillus the GCD is evoqued confirmed by the NBT test who was negative. IN CONCLUSION: --We have to look for GCD in front of pulmonary aspergillus.--The vertebra lesion is secondary to pulmonary lesion.--Our observation is noticeable by the recessive autosomal transmission who was seen in 35% of cases, and by the neurologic complication rarely reported.

[Reexpansion pulmonary edema]. / Oedèmes pulmonaires à vacuo.

Reexpansion pulmonary edema is an uncommon complication which sometimes occurs after evacuation of a large amount of air or fluid from the pleural space. We report two cases that illustrate the diversity of the clinical expression, severe in one case and latent in the other. The pathophysiology of reexpansion pulmonary edema remains obscure. Increased pulmonary capillary permeability, favored by previous atelectatic parenchyma and rapid reexpansion appears to be the main cause. Treatment is basically preventive. Curative treatment is based on adequate oxygenation and circulation. Lower aspiration pressure and oxygenation were sufficient in our patients. Severe clinical prognosis has been reported in the literature with a 15 to 20% mortality despite use of mechanical ventilation in particularly serious situations.

[Broncho-pulmonary cancer associated with pulmonary tuberculosis. Report of 4 cases]. / Association cancer broncho-pulmonaire et tuberculose pulmonaire. A propos de 4 cas.

The association between bronchopulmonary carcinoma and pulmonary tuberculosis would not be fortuitous but related to increased susceptibility to opportunistic infections and tuberculosis in cancer patients. We present four cases demonstrating the gravity of the situation and the difficulties encountered in diagnosis and treatment. Diagnosis of tuberculosis in patients with bronchopulmonary carcinoma requires pathological evidence from histology biopsies or bacteriology samples. The diagnosis is further complicated in early stage neoplasms. In case of tuberculosis, surgical treatment of bronchopulmonary carcinoma may have to be postponed or even contraindicated. Inversely, chemotherapy and radiotherapy may favor extension of the tuberculosis.

[Tuberculosis of the thoracic wall. Presentation of 4 personal cases and review of the literature]. / La tuberculose de la paroi thoracique. Mise au point à partir de 4 observations personnelles et d'une revue de la littérature.

The thoracic wall is an uncommon localization for tuberculosis, accounting for an estimated 1 to 5% of all cases of bone and joint tuberculosis which themselves account for 15% of all extrapulmonary localizations. Four patients were hospitalized for tuberculosis of the thoracic wall. The first patient, aged 22 years, had sternal tuberculosis with multiple bone localizations. The second patient was 37 years old and had a cold abscess of the peristernal soft tissues with several other bone localizations devoid of clinical manifestations. A third 37-year-old patient had a cold intercostal abscess revealed by a mass in the upper left quadrant of the left breast. In the last patient, 50 years old, tuberculosis of the ribs was associated with a cold tumor of the knee. Together with cases reported in the literature, these observations demonstrate the difficult diagnostic situation presented by tuberculosis of the thoracic wall. Treatment is based on long-duration multi-drug therapy. Surgical resection is rarely indicated. Cure was achieved in our four cases.

[Localized tracheobronchial amyloidosis. A new case studied with x-ray computed tomographic and nuclear magnetic resonance. Review of the literature]. / Amylose trachéo-bronchique localisée. Une nouvelle observation avec étude tomodensitométrique et en résonance magnétique nucléaire. Revue de la littérature.

We report a new case of localized primary tracheobronchial amyloidosis revealed by haemoptysis and effort dyspnea. The CT scan image was a homogeneous and circumferential thickening of the bronchial wall measuring 282 on the Hounsfield scale. At magnetic resonance imaging the amyloid substance was a slightly less intense than fat tissue in T1 and T2-weighted sequences. Treatment using Yag laser photoresection stabilized the lesions with one year follow-up. The review of the literature enables us to summarize the clinical, endoscopic, morphological, prognostic and therapeutic features of tracheobronchial amyloidosis, as well as pathogenic hypotheses and relationship with tracheobronchopathia osteoplastica.