Repigmentation d’un vitiligo universalis déclenchée par l’hémodialyse

Vitiligo is an acquired depigmenting disorder. Vitiligo universalis is a rare form responsible for significant aesthetic damage. To date, the exact pathogenesis remains unknown. Its treatment, a real challenge, consists rather in removing the still pigmented areas. We report a case of a patient followed for stable vitiligo universalis from an early age who presented with repigmentation shortly after initiation of hemodialysis.     

Métastases osseuses d’un épendymome d’emblée de double localisation. À propos d’un cas

A case of cerebral and sacral ependymoma revealed by cerebellar syndrome and sciatica in a 68-year-old man is reported. Bone metastases were diagnosed seven months after surgical excision of the brain tumor.     

Thrombose d’oxygénateur en l’absence de résistance à l’héparine mais en présence d’une maladie de Vaquez

A 55-year-old male with a history of positive HIV serology and polycythemia vera underwent coronary artery bypass graft surgery with normothermic extracorporeal circulation. Following heparin administration the activated clotting time (ACT) was 633 seconds (Hemocron^® with kaolin). Lower than expected arterial and venous oxygen partial pressures together with high pressure (350 mmHg) in the arterial line upstream of the oxygenator were observed. Because of these signs the oxygenator was changed during the procedure. The outcome was uneventful. Electronic microscopic examination of the oxygenator membrane and thermic exchanger revealed fibrin and platelet deposits. Similar cases are described in the literature during polycythemia vera. Therefore the prevention might be a preoperative treatment with antiplatelet therapy in polycytemia vera.     

30 ans d’anastomose iléoanale

Résumé / Abstract

30 ans d’anastomose iléoanale     

Insuffisance rénale aiguë révélatrice d’un lymphome rénal. À propos d’un cas

Acute renal failure, as the initial manifestation of lymphoma, has been reported only in a few cases. In this work, we report the case of a 28-year-old women admitted for acute renal failure. Her physical examination detected bilateral kidney enlargement. Laboratory evaluation revealed a serum creatinine value 218 μmol/l. A 24-hour urine collection analysis allowed the detection of 1 g of protein. No red cells were found after urinanalysis. Renal ultrasound showed massively enlarged kidneys. Renal biopsy of the kidney and pathologic examination showed diffuse infiltration of the interstitium with lymphocytes and atypical cells positive for CD20 markers. A diagnosis of diffuse large B-cell type non-Hodgkin lymphoma was made. However, investigations revealed the presence of two others sites of lymphoma: gastric and ophthalmic. The patient's renal function and kidney size as well as the other lymphoma locations were normalized after the initiation of chemotherapy.     

Techniques d’anastomoses iléoanales avec réservoir

Complete proctocolectomy with ileoanal pouch anastomosis is the gold standard for the treatment of familial adenomatous polyposis and ulcerative colitis. In Crohn’s disease, this technique may be considered for selected patients free of anal or small bowel lesions. The two main techniques used for ileoanal anastomosis are the manual anastomosis following mucosectomy (Parks’ procedure, the reference technique), and the stapled anastomosis, the most used procedure worldwide. A third technique exists also, consisting in rectal eversion with section on the dentate line (Hautefeuille procedure); this technique avoid conservation of the anal transitional zone in stapled ileoanal anastomosis, and the risk of incomplete mucosectomy associate with Parks’ procedure. Finally, it is possible today to perform this intervention by laparoscopic approach, provided the operating team has the required training.     

Les maladies ossifiantes d’origine génétique

Inherited ossifying diseases are relatively uncommon diseases leading to a great disability and life-threatening complications. Fibrodysplasia Ossificans Progressiva (FOP) is characterized by the association of skeletal abnormalities mainly in great toes, and ossifications in tendons and muscles, resulting in the formation of a “second skeleton”. The transmission is based on a dominant autosomal pattern, but most cases are sporadic. BMP dysregulation seems to be the main underlying mechanism of the heterotopic ossifications. The genetic basis remain controversial between a mutation on chromosome 4 or 17. Progressive Osseous Heteroplasia (HOP), more recently described, shares some similarities with Albrights hereditary osteodystrophy. In HOP, the ossifications progressively developed from the dermis to the deeper layer. The genetic abnormality involved the GNAS 1 gene leading to an inactivation of the alpha subunit of the G protein-complex. Radiological pattern and physipathological mechanisms are different in FOP and POH. In FOP, muscles’ fibroblasts are transformed into bone progenitor cells with enchondral ossification. In HOP fat cells are involved in a process of intramembranous ossification. Some therapeutic approaches have been tried : angiogenesis inhibition, mast cell inhibition; others remained in project : BMP 4 inhibition ; but actually there is no proved efficacy of any of them.     

Transpiration stimulée afin d’améliorer l’équilibre hydro-sodé chez les patients hémodialysés : à propos d’un cas

Obtaining the desired dry weight in dialysis patients is challenging once residual diuresis has disappeared, considering the trend of increasing dietary salt intake and shortening dialysis time over the last 40 years. We describe the case of a 55-year-old patient of Sudanese origin, who presented excessive interdialytic weight gain and hypertension on maintenance hemodialysis. After failure of conservative measures, a therapy of daily hot water baths of 30 minutes each on non-dialysis days was introduced. All clinical parameters improved, including potassium profile. In this article, we review the history, pathophysiological mechanisms, efficacy and possible side effects of this interesting, somewhat forgotten technique.     

Atteinte des derniers nerfs crâniens révélatrice d’une métastase occipitovertébrale d’un cancer du col de l’utérus

A 37-year-old woman had a histologically documented occipitovertebral metastasis from an invasive epidermoid carcinoma of the uterine cervix. The metastasis was found three months after the primary and was treated by local radiation therapy followed by systemic chemotherapy. Radiation therapy was highly effective in relieving the pain but was not associated with changes in magnetic resonance imaging findings. Lymph node involvement and local extension occurred. The patient died one year after the diagnosis of the metastasis. Involvement of the occipitovertebral junction by uterine cervix carcinoma metastasis is exceedingly rare. The relevant literature is reviewed.