Traitement chirurgical d’une anémie hémolytique auto-immune

Introduction

Auto-immune hemolytic anemia (AIHA) is a rare cause of anemia, characterized by autoantibodies directed against self red blood cells. It can be primary or secondary, in particular due to lymphoproliferative diseases.

Expression particulière d’une hydatidose péritonéale

Hydatid cyst is a parasitic disease, common in Mediterranean countries. It is still endemic in Morocco, where the incidence is between 4.5 and 6.9% (Santé 2000;10(4):255–60; Chirurgie 1991;117(10):854–9). The liver and lungs are the organs most commonly involved, and peritoneal echinococcosis, either primary or secondary, is rare. The principal uncertainty concerns the mechanism of spread of echinococcal larvae to the peritoneum. The most reliable hypothesis is that liver and lungs can be bypassed through precapillary anastomosis between pre- and postparenchymal circulation (ANZ J Surg 2005; 75 (11):986–91). The presenting symptoms are often misleading, and the combination of clinical history, imaging findings and serologic test results usually helps the diagnosis. Hydatid disease should be considered in the differential diagnosis of all cystic retroperitoneal and intraperitoneal masses. Surgery remains the best curative or palliative treatment for peritoneal echinococcosis, although anthelmintics can be an effective alternative. We report the case of a 16-yearold patient with primary peritoneal echinococcosis who was seen in the department of gastroenterology and hepatology at the Mohamed VI university hospital complex in Marrakech. The presenting symptoms were atypical. The diagnosis was based on histological findings. The course after chemotherapy led to degenerative changes in the lesions without recurrence after a follow-up of 32 months. This case illustrates the mechanism of pathogenesis and difficulties in diagnosis and treatment of this endemic disease in our country.     

Une périmyocardite révélatrice d’une granulomatose éosinophilique avec polyangéite : une nouvelle observation

Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis characterized by necrotizing arteritis, eosinophilic infiltration and extravascular granuloma; that may involve several organs. Cardiac involvement is the first cause of death in this vasculitis. These include myocarditis, pericarditis, coronary heart disease, dysrhythmias, and rarely valvular involvement. We report the observation of CSS revealed by acute perimyocarditis.     

Cystadénome mucineux de l’appendice mimant une tumeur duodénale

Mucocele of the appendix is a rare lesion, characterized by an accumulation of mucus. Localization of appendicular mucocele under the liver is uncommon. We report a case of a 56-year-old man, with duodenal tumor revealed on abdominal ultrasonography and computed tomography. The preoperative diagnosis showed duodenal leiomyoma. Surgical exploration revealed a voluminous appendicular mucocele, which comprised the duodenal wall, because it was amucocele of an appendix under the liver. Ileocecal resection was performed with uneventful postoperative course after 30 months. Histopathologic examination showed a benign cystadenoma. Clinical and radiological appearance of this rare lesion could be unusual preoperatively and are often confusing. We discuss the diagnostic and therapeutic problems raised by this lesion. Surgical treatment requires a complete resection. Prognosis depends on histological study.     

L’éosinophile,une cible pour les thérapeutiques du futur

Bronchial asthma is a common disease, of which most of patients are adequately treated by standard therapy. However, a small group of patients with asthma has severe disease that cannot be controlled by conventional strategies (Inhaled corticosteroids and bronchodilatator). The central role of Th2 lymphocytes and cytokines (Interleukin-4, interleukin-5), as well as eosinophils has been highlighted in asthmatics individuals. Eosinophils are generally regarded as a particularly harmful element in the asthmatic airways, and chemokines and proeosinophilic cytokines may play a central role in this condition. Several novel strategies have now been tested in animal models of asthma including inhibitors of proeosinophilic cytokines. The challenge, now, is to establish their therapeutic interest in human asthma.     

Syndrome d’activation macrophagique associé à une granulomatose et une infiltration lymphocytaire T CD4 diffuse révélant une nocardiose disséminée et une infection pulmonaire à Streptomyces spp

IntroductionHemophagocytic lymphohistiocytosis (HLH) is a rare syndrome frequently secondary to infectious disease, especially in immuno-compromised patients. We report a HLH secondary to disseminated nocardiosis and Streptomyces spp pulmonary infection.Case reportA 69-years-old women had recent subcutaneous nodules of the forearms and loins associated with peripheral neuropathy and pulmonary nodule of the right upper lobe. Cutaneous biopsy revealed granuloma. Cutaneous lesions worsened and the patient developed a HLH with probable cardiac and neurological involvement, associated with cutaneous granulomatosis and diffuse polyclonal lymphocyte proliferation. Nocardia PCR was positive in cutaneous biopsy. Pulmonary samples revealed Streptomyces in culture and Nocardia in PCR. The evolution under antibiotic treatment was favorable.ConclusionRecent diagnosis of HLH without obvious etiology should lead to etiological investigation, including the search for infections with slow-growing bacteria such as Nocardia or Streptomyces spp.     

Végétation tricuspidienne au cours d’une maladie de Still,une manifestation non infectieuse rare de lésion intracardiaque

Introduction

Adult-onset Still's Disease is a rare multisystemic inflammatory disease characterized by fever, maculo-papular erythematous rash and arthralgia. Adult-onset Still's disease is a diagnosis of exclusion.

Pancréatite aiguë inaugurale d’une vascularite à IgA de l’adulte : une manifestation inhabituelle

Introduction

IgA vasculitis is a systemic small vessel leukocytoclastic vasculitis characterized by skin purpura, arthritis, abdominal pain and nephritis. Most of the abdominal complications are due to edema and hemorrhage in the small bowel wall, but rarely to acute secondary pancreatitis.

Maladie de Gaucher de type 1 simulant une thrombopénie immunologique : intérêt de l’hyperferritinémie et de l’hypergammaglobulinémie dans le bilan d’une thrombopénie isolée

IntroductionGaucher disease type 1 is a rare genetic disease. It can cause thrombocytopenia. Current guidelines do not support bone marrow examination in front of isolated thrombocytopenia if no evidence suggests malignant hemopathy. This strategy aiming at sparing unnecessary investigations makes such rare diseases more difficult to diagnose.Case reportA 31-year-old woman was diagnosed with immune thrombocytopenia according to current guidelines. She presented later with mild splenomegaly. Bone marrow aspirate smears showed Gaucher cells. Gaucher disease was then confirmed. Looking backward, initial biological clues (hyperferritinemia, hypergammaglobulinemia) should have enabled to consider the diagnosis.ConclusionGaucher disease type 1 can be responsible for apparently isolated thrombocytopenia. The disease must be looked for if the thrombocytopenia is associated with unexplained hypergammaglobulinemia or hyperferritinemia. Diagnosing immune thrombocytopenia without bone marrow sample requires to systematically pay attention to any clinical or biological abnormality, not to ignore rare differential diagnoses.     

Volvulus gastrique récurrent révélant un hémangio-endothéliome malin de l’antre. A propos d’une observation

Gastric volvulus is an abnormal rotation of the stomach on its axis which acute form requires emergent care. Nevertheless, unusual symptoms of this disease at presentation, often lead to delay diagnosis. We report a case of acute gastric volvulus revealing a malignant hemangio endothelioma of the antrum in a 62 years old man. He presented recurrent signs of gastric outlet obstruction mimicking Borchardt’s syndrome because endoscopy of the upper digestive tract or nasogastric probe were not possible to carry out. Use of CT scan was also helpful to set up diagnosis of volvulus leading to laparotomy which highlighted the antral tumour. So, partial gastrectomy was performed and postoperative course was uneventful. After four months, the clinical course was good and a gain of 3 kg of weight was noted.     

Cancer de l’?sophage au Sénégal: revue d’une série de 72 cas

Aim

The cancer of the oesophagus because of its poor prognosis is a terrifying digestive tumour. The goal of this work was to appreciate the epidemiologic characteristics, anatomo-clinics and to become it of a series of patients.

Patients and methods

It was about a retrospective analysis of 72 records of patients in the same team, followed between January 2004 and December 2007.

Results

Squamous cell carcinoma accounted for 94.4% of cancers of the oesophagus during this period. The average age of the patients was 48 years with extremes of 16 and 86 years. The diagnosis was late with six months average time of the clinical symptomatology evolution. The general state was faded with an index of performance WHO superior to 2 at 48.6% of the patients. The high alcohol consumption and smoking, principal exogenic toxic factors risk was found only in 16.6% of the cases. Achalasia and Plummer-Vinson syndrome, squamous cell carcinoma specific factors of risk, were present in 5.6% of the cases. Gastrointestinal oesophageal reflux disease, indirect risk factor of adenocarcinoma, was found in 9.7% of the cases. The disease was advanced with 64% of stages III and IV TNM in classification (UICC 2002). The rate of resecablity was 27.8%. None of our patients had profited from neo-adjuvant treatment. Overall survival at two years was estimated at 2.8%.

Conclusion

The pejorative prognosis seemed to be raised by the late diagnosis in our series. An improvement of survival would pass by an early detection. A barium transit in front of all odynophagia, followed by an endoscopic examination if required must be our standard. The mechanism of the viral carcinogenesis by human papillomavirus types 16 and 18 evoked but not completely elucidated is a way of research to decrease the incidence of the cancer of the oesophagus in population exposed to these viruses.     

Primo-infection à cytomégalovirus : une cause infectieuse d’infarctus splénique

Introduction

Cytomegalovirus-associated thrombosis has been extensively reported in the medical literature, mainly in immune-compromised patients. However, the association with splenic infarcts has rarely been reported.

Sténose de l’artère rénale à distance d’une dénervation rénale pour HTA résistante

The onset of renal artery stenosis following a renal denervation is rare and occurs in the first few months after renal denervation. We report the onset of renal artery stenosis a long time after the renal denervation for resistant hypertension. This is a 74 year-old patient who stopped smoking in 1980 and who was treated for dyslipidemia with a revascularized coronary artery disease in 2011, a well-stabilized peripheral arterial disease since 2001, a stable asymptomatic carotid atheroma and a good kidney function. His hypertension known since 1995 became resistant. After the control of renal arteries by angio-CT scan, he had a renal denervation in October 2012. His blood pressure decreased 3 months later confirmed by self-blood pressure monitoring (SBPM) and ambulatory blood pressure monitoring (ABPM) with a CT scan with a non-significant renal artery stenosis in January 2014. He remained normotensive under treatment until July 2015 but his hypertension became uncontrolled at the end of 2015 then resistant and severe confirmed by SBPM in April 2017, despite a 5-drug antihypertensive treatment associated to atorvastatin and clopidogrel confirmed by SBPM in April 2017. A left post-ostial renal artery stenosis with decrease in size of left kidney and cortex as compared to 2011 was detected at CT and treated by angioplasty. It was associated with a rapid decrease in blood pressure but unfortunately a new increase related to a restenosis occurred at the end of 2017, which justified a new angioplasty. Discussion about the etiology and the management of this renal post-denervation late stenosis.