Etude des anastomoses entre la veine rénale gauche et les plexus intra-rachidiens

Résumé Des anastomoses entre la veine rénale gauche et les plexus rachidiens existent dans environ 80% des cas (16 cas sur 20); elles sont importantes dans 25% des cas (5 cas). Le siège de leur origine est fixe: le plexus du trou de conjugaison de l'espace L1–L2 reçoit généralement ces voies anastomotiques.Ces anastomoses ont une grande importance, puisqu'elles constituent la seule voie collatérale de sûreté du rein dans 7 cas sur 20. Leur hypertrophie, dans certains cas pathologiques, peut être responsable de troubles neurologiques par compression, réversibles après ligature des anastomoses hypertrophiées.     

Clinical follow-up of the first SF-1 insufficient female patient

ObjectiveSteroidogenic factor 1 (SF-1/NR5A1) plays a crucial role in regulating adrenal development, gonad determination and differentiation, and in the hypothalamic-pituitary control of reproduction and metabolism. In men (46, XY), it is known that mutations in SF-1/NR5A1 gene cause a wide phenotypic spectrum with variable degrees of undervirilization. In recent years, the role of SF-1 in the ovarian function was increasingly discussed and alterations in the gene were related to primary ovarian insufficiency. We describe the follow-up of a 46, XX affected woman with a SF-1 mutation and by comparing our case with the known manifestations reported in the literature, we try to further elucidate the function of SF-1 in the ovary.ResultsDuring infancy, adrenal insufficiency was the only clinical sign of the loss-of-function as ovarian development and function seemed normal. To date, this young woman aged 16.5 years shows normal growth, normal BMI and psychomotor development, has a normal puberty and regular menstruation.ConclusionThis report shows one, to date uniquely described, phenotypic variant of SF-1 mutation in a 46, XX affected person with adrenocortical insufficiency but no ovarian dysfunction nor disturbance of pubertal development. To follow the natural history of SF-1 mutation in a 46, XX individual will further shed light on its role in the ovarian function and thus will help to counsel affected patients in future.     

Management of accessory renal arteries during aneurysm repair in a patient with end-stage renal failure

The presence of accessory renal artery is a frequent anatomic variation that can challenge abdominal aortic aneurysm (AAA) repair. Here, we show an image of an abdominal aortic aneurysm extended to multiple accessory renal arteries in a patient known for an end-stage renal failure. This case raises the questions of the criteria that should be taken in consideration for an optimal management of accessory renal artery during AAA repair.     

Fibrose systémique néphrogénique

Nephrogenic systemic fibrosis is a recently described entity that occurs in patients with advanced renal failure. Its cause is probably toxic. The patients develop skin thickening, which is usually symmetrical on the limbs and sometimes extend to the trunk. Joint contractures and muscle sclerosis confine the patients to wheelchair. Systemic involvement may occur and includes cardiomyopathy, pulmonary fibrosis and diaphragmatic paralysis. The diagnosis is confirmed by the association of skin fibrosis and a cellular infiltration composed of CD34+ fibrocytes. Prognosis is severe with many deaths, rarely directly related to the disease. An improvement of lesions is possible, especially in case of resolution of the renal insufficiency. Several treatments have been evaluated, but none has shown consistent benefit. The toxic culprit is likely to be the gadolinium ions (Gd⁺⁺⁺), released from some contrast agents used in nuclear magnetic resonance imaging. Evidence of the responsibility of Gd⁺⁺⁺ is based on epidemiologic, biochemical and experimental data. Recommendations have been published for patients with renal insufficiency requiring a nuclear magnetic resonance imaging. If they are followed and efficient, it is likely that nephrogenic systemic sclerosis will disappear.     

Hypertension artérielle,atteinte rénale et génétique chez le sujet noir : mise au point

The incidence and prevalence of hypertension is markedly elevated in Afro-American populations vs Caucasians. The development of end-stage renal disease is also more frequent in Afro-American subjects, independently of blood pressure control. As compared to Caucasians, Afro-American subjects have a higher risk of end-stage renal disease when they are infected with HIV or have lupus. For decades, these data remained mysterious. Within the last 3 years, results from studies in the field of genetics and infectious diseases have transformed our view on this problem. The aim of this paper is to explain how these results have changed our understanding of hypertension and its consequences in Afro-American subjects.     

Hyperuricemia in sickle cell disease in France

Purpose

Hyperuricemia has been reported to be a common feature of sickle cell disease occurring between 32 to 41% of the patients, in studies conducted during the 1970's. Since then, this notion has been rarely challenged. The objective of this study was to assess the prevalence of hyperuricemia and gout in adult patients with sickle cell disease in France.

Methods

Between May 2007 and March 2009, serum and urinary urate concentration, creatininemia and hemogram were prospectively assessed in all consecutive sickle cell patients, followed in our sickle cell disease centre. All subjects were in a clinically steady state. Clinical acute gout history was also recorded.

Results

Sixty-five patients (mean age 31 ± 10.3 years) were investigated. Mean uric acid serum level was 281.6 ± 74 μmol/L. Hyperuricemia was evidenced in six patients only (9.2%) (95% IC: 3.5-19.0). None of the patient had a medical history of acute gout. Patients in the higher serum uric acid tertile concentration had higher serum creatinine level (62.3 ± 17.1 μmol/L vs 51.5 ± 12.6 μmol/L, P < 0.01), lower fractional excretion of urate (4.5% vs 6.8%, P < 0.03) and higher reticulocyte count (median 219 500/mm³ vs 144 000/mm³, P = 0.08) compared to the other patients.

Conclusion

Hyperuricemia and gout are not a clinical problem in sickle cell disease in our country. Nevertheless, our findings indicate that kidney function has to be fully explored if serum uric acid level is elevated or significantly deteriorates during follow-up. Serum uric acid level could be an early marker of renal dysfunction in sickle cell disease patients.     

Adrenal tuberculosis: case report

Isolated adrenal tuberculosis is rare, and represents between 1-2% of the etiologies of adrenal masses called incidentalomas. A 32-year-old woman, without notable medical history, was hospitalized for pain in the left hypochondrium, lasting for two months in a context of apyrexia and weight loss amounted to 5 kg. Clinical examination was normal, but abdominopelvic CT objectified bilateral adrenal hypertrophy predominantly left with bilateral linear calcifications. The chest radiograph was normal, adrenal hormones were normal. The research of BK in sputum and urine were negative on direct examination and culture. The tuberculin was 12 mm and HIV status was negative. A left adrenal biopsy was done and histopathological study of tuberculous lesions was found confirming caseofolliculaire adrenal tuberculosis. The patient has been treated with antibacillaire with favorable evolution. In light of this observation, the authors make the point on this rare disease.