21 years of evolution of primary hepatic neuroendocrine neoplasm in a patient with primary sclerosing cholangitis: A case report.

INTRODUCTION AND IMPORTANCE: Primary hepatic neuroendocrine neoplasms(PHNEN) are among the rarest primitive neuroendocrine neoplasms. Main prognostic factor is histological. We report an unusual case of a PHNEN with 21 years of evolution in the setting of a primary sclerosing cholangitis(PSC). CASE PRESENTATION: A 40 year old man presented in 2001 with clinical signs of obstructive jaundice. CT-scan and MRI showed a 4 cm large hypervascular proximal hepatic mass evoking hepatocellular carcinoma(HCC) or cholangiocarcinoma. Exploratory laparotomy found an aspect of advanced chronic liver disease of the left lobe. Extemporaneous biopsy of a suspicious nodule showed signs of cholangitis. Left lobectomy was performed and postoperatively the patient received ursodeoxycholic-acid and biliary stenting. After 11 years of follow-up, jaundice reappeared with a stable hepatic lesion.A percutaneous liver biopsy was done. Pathology showed a G1 neuroendocrine tumor. Endoscopy, imagery and Octreoscan were normal, supporting the diagnosis of PHNEN. PSC was diagnosed on tumor-free parenchyma. The patient is on liver transplantation waiting list. CLINICAL DISCUSSION: PHNENs are exceptional. Pathology findings, endoscopy and imagery are necessary to rule out an extra hepatic NEN with liver metastasis. While G1 NEN are known for their slow evolution, this 21 year latency is extremely rare. The presence of PSC adds to the complexity of our case. Surgical resection is recommended when possible. CONCLUSION: This case showcases the extreme latency of some PHNEN as well as possible overlap with PSC. Surgery is the most recognized treatment. Liver transplantation seems to be necessary for us, as the rest of the liver shows signs of PSC.

[Primary and isolated phrenic hydatid cyst in Tunisia]. / Kyste hydatique primitif et isolé du diaphragme en Tunisie.

The hydatid cyst of the diaphragm is defined as the development of a hydatid cyst in the diaphragm muscle. The purpose of this study is to identify the specific pathogenic, clinical and therapeutic aspects of this hydatid cyst. It is important to confirm the diagnosis of the location before surgery. We report the case of a patient aged 40 years, in whom the diagnosis of hydatid cyst of the diaphragm was made incidentally in the course of a gastric stromal tumour. The patient was operated by a midline laparotomy. Resection of the protruding dome of hydatid cyst of the diaphragm was carried out along with total gastrectomy. The postoperative course was uneventful. After 12 months, the results were negative. The diagnosis of hydatid cyst of the diaphragm is based on the thoraco-abdominal CT scan. The treatment is surgical based on the topographic features of the cyst.

[Human immunodeficiency virus type 1 subtypes in Djibouti]. / Sous-types du virus de l'immunodéficience humaine de type 1 a Djibouti.

The authors had for aim to study the distribution of HIV-1 subtypes in a cohort of HIV positive patients in the hospital General Peltier of Djibouti. An epidemiological study was made on 40 HIV-1 positive patients followed up in the Infectious Diseases Department over three months. All patients sample were subtyped by genotyping. Thirty-five patients (15 men and 20 women) were found infected by an HIV-1 strain belonging to the M group. Genotyping revealed that - 66% of samples were infected with subtype C, 20% with CRF02_AG, 8.5% with B, 2.9% with CRF02_AG/C and 2.9% with K/C. In fact, Subtype C prevalence has been described in the Horn of Africa and a similar prevalence was previously reported in Djibouti. However our study describes the subtype B in Djibouti for the first time. It is the predominant subtype in the Western world. The detection of CRF02_AG strains indicates that they are still circulating in Djibouti, the only country in East Africa in which this recombinant virus was found. CRF02_AG recombinant isolates were primarily described in West and Central Africa. The presence of this viral heterogeneity, probably coming from the mixing of populations in Djibouti, which is an essential economic and geographical crossroads, incites us to vigilance in the surveillance of this infection.

Pancreaticojejunostomy vs. pancreaticogastrostomy following pancreaticoduodenectomy: results of comparative study.

AIM: Several techniques have been proposed for reconstructing pancreatico-digestive continuity, which the first goal is reducing the rate of pancreatic leakage after pancreaticoduodenectomy. Only a limited number studies have been carried out. Our objective is to compare the results of pancreaticojejunostomy versus pancreaticogastrostomy following pancreaticoduodenectomy. METHODS: This is a retrospective and comparative study about 80 patients who underwent pancreaticoduodenectomy. These patients were divided into two groups: pancreaticojejunostomy (group PJ) and pancreaticogastrostomy (group PG). RESULTS: The PJ group included 39 patients, while 41 patients were included in the PG group. There were no differences between the two groups concerning: patients' demographics, risk factors, indication, mean duration of surgery, texture of pancreatic tissue, need for intraoperative blood transfusion and postoperative prophylactic octreotide. Overall, the mortality postoperative rate was 7.5% (N.=6), the incidence of surgical complications was 50% (51.3% in PJ, 48.8% in PG; P=0.823, not significant). Pancreatic fistula was the most frequent complication, occurring in 17.5% of patients (25.6% in PJ and 9.8% in PG; P=0.062, almost significant). 7.7% of patients who underwent PJ and 14.6% of patients who underwent PG required a second surgical intervention (P=0.326, not significant). There were no differences between the two groups PG and PJ concerning: Postoperative hemorrhage (P=0.63), biliary fistula (P=0.09), acute pancreatitis (P=0.95), delayed gastric emptying (P=0.33). The mean postoperative hospitalisation period stay was similar in both groups (P=0.63) CONCLUSION: There were not any significant differences between the two groups in the overall postoperative complication rate, the incidence of postoperative haemorrhage, biliary fistula, acute pancreatitis, and delayed.

Three Successful Pregnancies in a Patient with Recurrent Cushing's Disease.

The association of pregnancy and Cushing's disease (CD) is rare. A 28-year-old woman was admitted for clinical suspicion of Cushing's syndrome. The investigations confirmed the diagnosis of CD with secondary hypogonadotropic hypogonadism due to an invasive pituitary macroadenoma. The patient underwent transsphenoidal adenomectomy, and histopathology showed an adrenocorticotropic hormone pituitary adenoma. Initial remission of CD ensued, and fertility was restored as the patient had 2 uncomplicated pregnancies. Five years and 10 months after surgery, a third spontaneous pregnancy was confirmed with underlying recurrent CD. Having mild hypercortisolism, CD was managed expectantly. The outcome was a healthy full-term neonate with no maternal complications during pregnancy or labor. Our case highlights the challenge faced by physicians of choosing the optimal approach to active CD in pregnancy. In cases where maternal and fetal complications are mild, conservative approach may be used and specific treatment can be postponed until after delivery.

Intraperitoneal migrating mesh plug wrongfully taken for right colon cancer: A case report.

BACKGROUND: The use of mesh has become nowadays a standard for hernia repairs. It allows a tension-free hernioplasty and has shown that it is an effective way to prevent recurrences. But complications have been described. Intraperitoneal migration of mesh plug is an uncommon complication. CASE REPORT: In this paper we report a case of a 57 year old male who has been operated on 12 years ago, he had a mesh plug repair for a ventral incisional hernia. The mesh migrated into the abdominal cavity and it was wrongfully taken for a locally advanced right colon cancer. Colonoscopy was done and biopsies were taken, but the results were not conclusive. He was operated on. We found the mesh that had migrated and eroded the hepatic flexure. There was a granulation tissue that also included some of the small intestine. There was also an abscess in the abdominal wall. He had an en-bloc resection of a part of the abdominal wall, small intestine and right colon. CONCLUSION: Mesh hernioplasty is a frequent, simple and effective procedure with a low recurrence rate but it can be associated to serious complications such as mesh migration.

Tuberculous severe acute colitis. A case report.

INTRODUCTION: and importance: Intestinal tuberculosis represents 2% of the ten million cases of tuberculosis reported in 2018. Herein, we report a case of tuberculous severe acute colitis. It is a rare and life-threatening condition. Our literature review found only five published cases. It occurs generally in immunocompromised patients. Extended colonic inflammation seems to be the main predictive factor of death. Moreover, an early diagnosis and rapid onset of antituberculous treatment are mandatory to save the patient's life. CASE PRESENTATION: Herein, we present a case of tuberculous severe acute colitis with a review of the reported cases. The patient presented with a severe and idiopathic acute colitis. He was put on broad-spectrum antibiotics and intravenous corticosteroids. At day two, he developed septic shock and colic perforation. Colectomy was performed. Microbiology investigation and pathology examination confirmed tuberculous colitis. CLINICAL DISCUSSION: Tuberculous severe acute colitis occurs generally in immunocompromised patients. Extended colic inflammation seems to be the main predictive factor of death. Moreover, an early diagnosis and rapid onset of antituberculous treatment are mandatory to save the patient's life. However, diagnosis is difficult as symptoms aren't specific. Microbiology and pathology were compulsory to retain colic tuberculosis in all the reported cases. CONCLUSION: Tuberculous severe acute colitis is a challenging and life-threatening condition. It usually occurs in immunocompromised patients. Abdominal CT-scan may evoke the diagnosis. Microbiology and pathology are mandatory to retain the diagnosis. Early diagnosis and onset of antituberculous treatment are compulsory to save the patient's life.

Enterosalpingeal fistula complicating Crohn's disease: Report of two cases and review of the literature.

INTRODUCTION AND IMPORTANCE: Reports of enterosalpingeal fistulae complicating Crohn's disease are scarce. They involve the last ileal loop and lead to a progressive destruction of the salpinx. Usually, no genital symptoms are found. In all the cases reported in the literature, the fistula was diagnosed intra-operatively and resection of the right salpinx was performed without the patient's pre-operative consent. CASE PRESENTATION: We describe 2 cases of women presenting with an Enterosalpingeal fistulae complicating Crohn's disease. Radiological findings allowed a pre-operative diagnosis. Thus, the patients were warned of the right salpinx resection and consent was obtained. CLINICAL DISCUSSION: Enterosalpingeal fistulae complicating Crohn's disease are exceptional. Indeed, to the best of our knowledge, only five cases have been reported till now. In all the reported cases, no genital signs were present. As for our patients who didn't experience such symptoms. Moreover, no radiological evidence of the enterosalpingeal fistula was found in the literature. Consequently, the fistula was always diagnosed intra-operatively. For our patients, radiological findings allowed a pre-operative diagnosis. This permitted to warn them of a possible resection of the fallopian tube. Intra-operative findings were unfortunately conflicting with its preservation. CONCLUSION: Enterosalpingeal fistula is an exceptional complication of the Crohn's disease. No clinical findings are present. The diagnosis should be evoked when the CT-scan or the MRI show an abnormal apposition between the fallopian tube, the last loop and the cecum. Surgical resection of the involved salpinx with the diseased intestinal segment is unfortunately usually needed in a young patient population.

[Assessment of body composition during pulmonary tuberculosis]. / Évaluation de la composition corporelle au cours de la tuberculose pulmonaire.

BACKGROUND: Although the body mass index (BMI) is the most commonly used tool to assess the nutritional status of patients with active tuberculosis (TB), it does not assess changes in body composition. This study aims to assess the contribution of bioelectrical impedancemetry (BIA) for the assessment of body composition during the course of TB compared to BMI and to examine the associated factors. METHODS: Cross-sectional study carried out in patients with active TB at the pulmonology department of CHU la Rabta in Tunis, Tunisia. The nutritional assessment was based on the measurement of BMI and the analysis of body composition by BIA. Malnutrition was accepted when the lean mass index (LMI) was ≤16kg/m2 in men and 15kg/m2 in women. RESULTS: Ninety-five patients were included. According to their LMI, 38 patients were undernourished. The decline in LMI was associated with the severity of TB. Although BMI and LMI were correlated, the use of BMI alone failed to recognize lean mass loss in one in ten patients. CONCLUSION: Undernutrition is frequent in patients with active TB. It is correlated with the severity of the disease. In addition to anti-tuberculosis drugs, nutritional management of these patients is essential.

[Nutritional status and dietary intake in patients with chonic obstructive pulmonary disease]. / Apports nutritionnels et état nutritionnel au cours de la broncho-pneumopathie chronique obstructive.

INTRODUCTION: Undernutrition is a predictor of mortality in chronic obstructive pulmonary disease (COPD). The objectives of our study were to assess nutritional intake in COPD and to study its relationship with disease severity. METHODS: A cross-sectional study that included 66 patients followed for COPD. Patients included had a body composition study and a respiratory and nutritional assessment. RESULTS: The mean age of the population was 66±9 years. The lean body mass index (LMI) was reduced in 26.1% of patients. It was significantly associated with the GOLD group (P=0.04) and significantly correlated with the forced expiratory volume in the first second (FEV1) (P=0.02) and the distance covered during the six-minute walk test (TM6) (P=0.01). A significant difference was found between the caloric intakes and the different GOLD groups (P=0.04). Mean intakes of calories (P=0.002; r=0.07), protein (P=0.01; r=0.16), carbohydrates (P=0.02; r=0.2) and iron (P=0.01; r=0.13) were significantly correlated with the TM6 results. Caloric intake was significantly correlated with LMI (P=0.01; r=0.16), body mass index (P=0.04; r=0.12), FEV1 (P=0.04; r=-0.12) and GOLD stage (0.002). Similarly, protein intake was significantly correlated with LMI (P=0.001; r=0.11), body mass index (P=0.02; r=0.16), FEV1(%) (P=0.001; r=-0.16) and GOLD stage (P=0.002). CONCLUSION: Undernutrition in COPD is caused by decreased food intake and increased resting energy expenditure. Adequate intakes of glucose, protein, fibers, vitamins and zinc are associated with improved ventilatory function.

[Complicated sigmoid tumor: perforated amebiasis]. / Une tumeur sigmoïdienne compliquée: amoebome perforé.

Amoeboma is an inflammatory mass of the colon. It can be an inaugural symptom and thus pose the problem of differential diagnosis with colon cancer. The purpose of this report is to describe the case of a 43-year-old patient who presented with acute abdomen. Physical examination revealed a perforated circumferential mass in the sigmoid colon. Based on a presumptive diagnosis of colonic cancer complicated by perforation, segmental colectomy was performed. Histological examination of the surgical specimen demonstrated colonic amoeboma. The patient was treated using metronidazole. Although rare, amoeboma must be considered in differential diagnosis of cancer of any colonic mass.

Variation of 4 MV X-ray dose rate strongly impacts biological response both in vitro and in vivo.

Whereas an RBE > 1 is described for very low-energy X-ray beams (in the range of 25-50 kV), there is a consensus that the RBE of X-rays (from 0.1 to 3 MeV) is equal to 1, whatever the energy or dose rate of the beam. Comparisons of X-ray beam dose rates are scarce even though these beams are widely used in medical diagnosis or radiotherapy. By using two dose rates (0.63 and 2.5 Gy.min-1) of high-energy X-rays on normal endothelial cells (HUVECs), we have studied the clonogenic assay, but also viability/mortality, cell cycle analysis and measured cellular senescence by flow cytometry, and have performed gene analysis on custom arrays. In order to consolidate these data, we performed localized irradiation of exteriorized small intestine at 0.63 and 2.5 Gy.min-1. Interestingly, in vivo validation has shown a significantly higher loss of weight at the higher dose when irradiating to 19 Gy a small fragment of exteriorized small intestine of C57Bl6J mice. Nevertheless, no significant differences were observed in lesioned scores between the two dose rates, while bordering epithelium staining indicated twofold greater severe damage at 2.5 Gy.min-1 compared to 0.63 Gy.min-1 at one week post-irradiation. Taken together, these experiments systematically show that the relative biological effectiveness of photons is different from 1 when varying the dose rate of high-energy X-rays. Moreover, these results strongly suggest that, in support of clonogenic assay, multiparametric analysis should be considered to provide an accurate evaluation of the outcome of irradiated cells.

Diabetic retinopathy, PAI-1 4G/5G and -844G/A polymorphisms, and changes in circulating PAI-1 levels in Tunisian type 2 diabetes patients.

AIM: The association of altered plasminogen activator inhibitor (PAI)-1 levels and PAI-1 polymorphisms (4G/5G and -844G/A) with diabetic retinopathy (DR) was investigated in 856 type 2 diabetes (T2D) patients, of whom 383 presented with (DR group), and 473 presented without (DWR group), retinopathy. METHODS: PAI-1 4G/5G and -844G/A genotyping were done by PCR-RFLP, and PAI-1 levels were measured by ELISA testing. RESULTS: The genotype distribution of 4G/5G and -844G/A polymorphisms did not deviate from the Hardy-Weinberg equilibrium model among healthy subjects. Higher frequencies of the 4G/4G genotype, and lower frequencies of the -844A allele, -844G/A and -844A/A genotypes, were seen in DR patients, conferring disease susceptibility and protection, respectively. While PAI-1 levels were significantly elevated in the 4G/4G compared with other PAI-1 genotypes, significant differences in PAI-1 levels between DR and DWR patients were seen in the 4G/-844A, 4G/-844G and 5G/-844A haplotype carriers among DR patients. However, comparable distributions of 4G/5G and -844G/A alleles, genotypes and haplotypes, and similar PAI-1 levels, were seen in the proliferative retinopathy (PR) and non-proliferative retinopathy (NPR) patients, indicating that neither PAI-1 variants nor changes in PAI-1 levels were linked to DR severity. Multivariate analyses identified 4G/-844A and 4G/-844G haplotypes as negatively and positively associated, respectively, with DR, but not with DR severity (PR vs NPR) after adjusting for a number of covariates. CONCLUSION: The present study identifies changes in PAI-1 levels and genetic variations at the PAI-1 locus as risk factors for DR, but not DR severity, that may serve as useful markers of increased DR susceptibility.

Cystic echinococcosis in Tunisia: analysis of hydatid cysts that have been surgically removed from patients.

Echinococcosis/hydatidosis caused by Echinococcus granulosus has a widespread distribution in the human population of Tunisia, particularly in the north-west and centre-west of the country. In a recent study, the morphological features, fertility and viability of hydatid cysts that had been excised from patients in Tunis were explored, and the E. granulosus strain or genotype involved in each case was identified from morphology of the protoscolex hooks and the results of molecular genotyping. The hepatic cysts investigated came from 41 patients [31 women and 10 men, with a mean (S.E.) age of 43.41 (14.25) years] who were treated for cystic echinococcosis, by surgery but rarely with chemotherapy, at the La Rabta Hospital in Tunis, in the 12 months ending in June 2008. Most (56%) of these patients originated from rural areas in endemic governorates. Of the 60 hepatic cysts that were studied, 38.3% were located in the right lobe of the liver and 35.0% each involved both hepatic lobes. Almost a third (31.7%) of the excised cysts were degenerating, with the rest considered viable and either multivesicular (38.3%) or univesicular (30.0%). Almost all (93.3%) of the cysts were categorized as fertile, with a mean protoscolex viability of 21.8%. Protoscolex viability was relatively high in the viable univesicular cysts with a visible cyst wall and in the multivesicular and multiseptate cysts with daughter cysts, and lowest in the cysts that appeared to be solid calcified masses. The observed variation in protoscolex viability with cyst type, in cysts excised from patients before any chemotherapy, supports the cyst classification recommended by the World Health Organization but could also be compatible with the imaging-based 'Gharbi' classification. The results of the molecular genotyping showed that all 23 cysts investigated (which came from 20 of the patients) were caused by E. granulosus of the G1 genotype (also known as the 'sheep' or 'sheep-dog' strain).

Textural characterization of media composed of compacted pieces of cardboard and polyethylene using a gas tracer method.

The aim of this work is the experimental determination of effective transport properties of porous media consisting of compacted pieces of cardboard and polyethylene (PE). The proposed method itself is more general and can be applied to many different materials and contexts. Three major transport properties were determined: porosity, tortuosity factor and permeability. Three parameters characterizing the media were varied over a wide range: the bulk density, the size of the elements entering the mix, and the proportion of cardboard and PE in the mix. The properties were measured by means of a specially designed experimental device based on miscible gas tracing. The porosity and tortuosity factor were simultaneously determined by parametric identification, based on the experimental sample output response to an inlet gas concentration step change compared to the results of a direct numerical model. Permeability was calculated in the standard way from the measurement of the pressure drop across the sample. The reproducibility of the measurements was very good. It was found that changing the material density of the medium significantly affects all three structural properties. When the bulk density is varied between 300 and 900 kg m(-3), the tortuosity factor varies in a range as large as 18-8 and the permeability decreases by a ratio of 2-3. The tortuosity factor shows unusual variation, characterized by a decrease when density is increased above 500 kg m(-3). The size of the elements does not significantly affect the structural properties of the medium in the range of parameters studied.

Abdominal metastasis of a parosteal osteosarcoma of the femur: an unusual cause of large-bowel obstruction.

BACKGROUND: Parosteal osteosarcoma is a rare, well-differentiated, predominantly fibro-osseous variant of osteosarcoma. It is regarded as a distinct form of osteosarcoma with better prognosis than conventional osteosarcoma. AIM: We report an unusual case of abdominal mass recurrence of parosteal osteosarcoma of the left distal femur treated eight years previously with wide resection, hip disarticulation and chemotherapy, which presented as an acute abdomen: we discuss the clinical outcomes of this rare entity. CASE PRESENTATION: We present a 54-year-old patient with low-grade parosteal osteosarcoma of the left distal femur. Left total hip disarticulation was indicated after several local relapses of the tumour following extensive resection and chemotherapy. Eight years later, he presented with an acute large bowel obstruction secondary to a compression of the large bowel by an abdominal mass. Abdominal computed tomography showed a large abdominal calcified mass with dilated large bowel loops. During laparotomy, the mass was unresectable. So, the patient underwent emergent colostomy of diversion. Biopsy of the lesion yielded grade III parosteal osteosarcoma material. The patient received adjuvant chemotherapy, but the response was poor: six months later, the patient presented with a peristomal mass and two pulmonary metastases. CONCLUSION: Abdominal recurrence of parosteal osteosarcoma of the distal femur eight years after definitive surgery is rare. This case emphasises the importance of the long-term follow-up of patients with parosteal osteosarcoma.

[Pituitary necrosis and antiphospholipid syndrome]. / Syndrome des anticorps antiphospholipides et nécrose hypophysaire.

Antiphospholipid syndrome (APS) is an acquired thrombotic disorder. It mainly occurs with systemic disease or as a primary disorder. All organs may be involved by thrombosis, but to date the most common endocrine manifestation is chronic adrenal insufficiency. Very few cases of hypopituitarism with primary APS have been reported. We report the case of a 27-year-old woman, victim of a stroke leading to double vision and intracranial hypertension. Magnetic resonance imaging showed a macro-adenoma with hemorrhage of a suprasellar lesion. Hormone assessment showed hyper prolactinemia with positive anticardiolipin antibody. Our case is the second reported associating APS with apoplexy. We discuss the clinical, biological and radiological features observed in our case. We conclude that APS should be searched for whenever a history of adenoma with apoplexy is found associated with recurrent thrombosis.

Bilateral adrenalectomy for severe hypertension in congenital adrenal hyperplasia due to 11beta-hydroxylase deficiency: long term follow-up.

CONTEXT: Bilateral adrenalectomy has been recently proposed as a surgical treatment option for patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. There is however little documented data about the long-term efficiency and potential side effects of this treatment. Patients with 11beta-hydroxylase deficiency (11betaHD) have been also concerned by this new approach. OBJECTIVE: Our objective was to describe our experience with bilateral adrenalectomy as a treatment of severe hypertension in a patient with 11betaHD deficiency and to report the long term follow-up (72 months) results after surgery. PATIENT AND INTERVENTION: A 22-year-old genetically female patient with 11betaHD deficiency was raised as a male because of severe pseudohermaphroditism. The patient has been managed by conventional steroid suppressive therapy and antihypertensive drugs with limited success; hypertension remained uncontrolled and led to severe complications. Bilateral adrenalectomy was offered to him. RESULTS: The intervention was followed by immediate blood pressure normalization and resulted in remarkable clinical improvement. Good compliance with glucocorticoid and androgen substitutive therapies was noted. However, a high 11-deoxycortisol, presumably due to non-ovarian ectopic adrenal rests was noted 24 months after surgery. CONCLUSION: Bilateral adrenalectomy is a safe and efficient method of managing CAH with selected patients. Long-term clinical and biochemical follow-up of patients with CAH treated by bilateral adrenalectomy is needed to earlier detect ectopic adrenal rests.