Spontaneous artery dissection: is it part of the spectrum of autosomal dominant polycystic kidney disease?
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Biomedical subjects
Publications and source records attributed to D Chauveau.
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OBJECTIVES: Polycystic liver disease is sometimes responsible for chronic symptoms linked to hepatomegaly which can result in acute complications such hemorrhage or infection of cysts. The aim of this retrospective study was to evaluate the results of partial hepatic resection in patients with symptomatic or complicated polycystic liver disease. METHODS: Twelve patients (11 women and one man, mean age 49) with diffuse polycystic liver disease were treated by partial liver resection (left lateral lobectomy in 7, left hepatectomy in 4, and extended right hepatectomy in 1). Four patients had terminal renal failures and three had chronic haemodialysis. Median follow-up was 34 months. RESULTS: Ascites occurred postoperatively in 10 patients (83%) and was long-lasting (> 2 weeks) in 5; all patients with end-stage renal failure had long-lasting ascites. One of them died on the 40th postoperative day of ascites infection. Another patient with end-stage renal failure died two years postoperatively from chronic disabling ascites and malnutrition while awaiting kidney transplantation. The 10 other patients were markedly improved after partial liver resection, including a marked decrease in hepatomegaly, and the disappearance of chronic symptoms and cystic complications. This beneficial effect was incomplete in the two surviving patients with end-stage renal failure until kidney transplantation was performed. CONCLUSION: These results suggest that partial liver resection is a highly effective treatment in patients with symptomatic polycystic liver disease, preferably before the onset of end-stage renal failure.
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The outcome and consequences of pregnancy in women with impaired renal function are still debated. To assess the benefit of recent advances in coordinated obstetrical and nephrologic management, we analyzed fetal and maternal outcome of 43 pregnancies in 30 women with various types of primary renal disease and moderate to severe renal failure at conception defined by serum creatinine concentration (Scr) ranging from 0.11 to 0.49 mmol/l. All pregnancies took place during the 20-year period from 1975 through 1994 and were prospectively followed jointly by our Nephrology Unit and Obstetric and Neonatology Units of University Hospitals. Of the 43 pregnancies (45 fetuses), 13 ended in fetal death (including 5 first-trimester abortions and 8 fetal deaths beyond the 20th gestational week). There were 32 live births, a success rate of 82% not considering first-trimester abortions. Successful pregnancies were significantly more frequent in the last decade than in the preceding one (91 vs 65%, p = 0.05). Overall live birth rate was higher in pregnancies started with Scr < 0.20 mmol/l than in those with Scr > 0.20 mmol/l (80% vs 53%, p = 0.02). The upper preconception Scr value associated with a successful fetal outcome was 0.27 mmol/l. Hypertension was the major factor of fetal prognosis, as the relative risk of fetal loss was 10.6 times higher when hypertension was present at conception or early in pregnancy than when blood pressure was spontaneously normal or well-controlled by therapy. An accelerated course toward end-stage renal failure was observed in 7 patients (23%), all of whom had severe hypertension and heavy proteinuria at conception. We conclude that fetal outcome in patients with impaired renal function has been improved in recent years, due to advances in obstetrics and neonatology, improved blood pressure control and close co-operation between nephrologists and obstetricians, but that a risk of fetal loss and of accelerated deterioration of maternal renal disease still persists when Ccr at conception is lower than 25-30 ml/ min/1.73 m2.
OBJECTIVE: To try to define when and what type of operation should be performed in von Hippel-Lindau disease (VHL) with renal involvement. MATERIAL AND METHODS: Between 1985 and July 1996, 43 patients with renal involvement of VHL disease were followed in our department. We decided to operate on patients with one or several solid or complex tumours greater than 2.5 cm in diameter, by means of conservative surgery (tumour excision removing a layer of healthy parenchyma) when the kidney did not contain more than 5 or 6 tumours or radical nephrectomy in the other cases. Subsequent follow-up consisted of CT, chest x-ray and renal function tests every 6 months. RESULTS: Twenty-nine patients with a mean age of 33.6 years were operated. 21 patients (29 kidneys) were treated by conservative surgery: in situ in 20 cases and ex vivo in 9 cases; 3 cases were treated by bilateral radical nephrectomy, 4 by unilateral radical nephrectomy and 1 by resection of the prominent dome for urinary tract obstruction. Four cases of acute arterial thrombosis were observed following ex vivo surgery, requiring nephrectomy in every case. No serious complications were observed with in situ conservative surgery. A new lesion in the renal parenchyma remaining after conservative surgery was observed in 5 cases during follow-up (mean: 29 months). No local recurrence was observed after radical nephrectomy. Two nonoperated patients developed metastatic disease. CONCLUSION: Conservative treatment is usually possible: either simple surveillance, in the absence of any solid or complex tumours larger than 2.5 cm in diameter, or conservative surgery for tumours larger than 2.5 cm in diameter, with no more than 6 tumours per kidney; this attitude requires six-monthly follow-up by CT of the renal parenchyma.
Sixteen laparoscopic cyst fenestrations for symptomatic adult polycystic liver disease (APLD) were performed in 13 women, including four patients who had had previous attempts at treatment (percutaneous sclerotherapy in two and fenestration via a laparotomy in two). The median number of cysts deroofed was 32 (range 18-58) during the 13 primary procedures. There was no in-hospital death. Postoperative transient ascites occurred in six patients. After operation 11 patients experienced immediate relief of symptoms but during a median follow-up of 26 (range 6-49) months, eight of these patients developed recurrent symptoms. Two patients underwent three repeat laparoscopic fenestrations, at which time perihepatic adhesions were rare and no complication occurred. Because repeat procedures may be performed, laparoscopic fenestration appears to be useful for the treatment of symptomatic APLD. However, it is less effective than fenestration at open surgery or liver resection and should be employed only in patients with predominantly large cysts.
Reflux nephropathy is one of the most prevalent renal diseases and a leading cause of chronic renal failure in women-of childbearing age. To evaluate the issue and possible complications of pregnancy in women with reflux nephropathy, we retrospectively analyzed fetal and maternal outcome in 158 women who had 375 pregnancies between 1965 and 1994. The overall fetal death rate was 10.2% and tended to decrease in the 1985 to 1994 decade as compared to the preceding period (8.4% vs. 12.6%). The relative risk of fetal death was 4.8 times greater hypertension was present at conception than in normotensive patients. Fetal death rate was also higher in patients with impaired renal function that in those with serum creatinine < 0.11 mmol/liter at conception (36.7% vs. 7.7%, P < 0.0001). Urinary tract infection accounted for frequent morbidity but seldom resulted in fetal mortality. Maternal renal disease was unaffected by pregnancy, excepted for 4 of the 21 patients with pre-existing renal failure who exhibited an irreversibly accelerated course after pregnancy. We conclude that pregnancy is essentially successful and uneventful in patients diagnosed with reflux nephropathy who have normal blood pressure and preserved renal function, whereas the fetal prognosis is more compromised and there is a risk of accelerated progression of maternal renal disease when serum creatinine concentration is in excess of 0.22 mmol/ liter. This suggests that women with reflux nephropathy should preferably conceive before having reached that stage of renal failure.
Renal involvement in von Hippel-Lindau (VHL) disease has emerged as the most prevalent cause of death in this hereditary disorder. In a group of 43 VHL patients (23 unrelated families) with renal lesions we examined whether severity of renal disease is affected by parental inheritance and VHL subtype (1, without pheochromocytoma; 2, with pheochromocytoma). We also tested whether and how nephron-sparing surgery could be applied. Renal involvement comprised multiple cysts and bilateral and multifocal carcinomas (RCC) which were detected by screening in 38 patients, at 30.5 (14 to 62) years of age. The severity of the renal disease was similar in VHL type 1 (79% of the pedigrees) and 2 (21%). It was not influenced by the sex of the carrier. Twenty-nine patients were operated on at a mean age of 33.6 years: 21 patients (28 kidneys or 61% of all operated kidneys) underwent nephron-sparing surgery, 4 had complete ablation of involved kidneys and thus required dialysis, 3 had uninephrectomy and 1 had cyst fenestration. Vascular thrombosis was the most severe early complication. It occurred in 4 of 9 kidneys treated by ex vivo surgery. During a median follow-up of 29 months, local recurrence occurred in 5 of 21 (24%) patients treated by nephron-sparing surgery, whereas 2 developed metastasis. Chronic renal failure (creatinine > 120 mumol/liter) affected 11 patients; in 9 of them, it was due to sequelae of surgery. In conclusion, screening of RCC and nephron-sparing surgery are of value in VHL patients. However, indications of ex vivo surgery should be drastically restricted and renal sequelae are not uncommon. Renal followup is required because of the risk of recurrence.
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We report our local experience of two patients with type 1 primary hyperoxaluria (PH1) who received successful isolated cadaver kidney transplantation. The indication of isolated renal transplantation exists for PH1, but it must probably be restricted to the less severe forms of this disease. Recipient and donor selection is crucial.
One hundred eighty-seven native kidneys in 96 patients were examined with color Doppler ultrasound (US) to (a) determine the color Doppler US characteristics of renovascular disorders and (b) assess the value of color Doppler US in detection of such disorders. Correlative angiography or computed tomography was performed in 94 patients. The following renovascular disorders were found: renal artery (RA) stenosis (40 cases), RA thrombosis (13 cases), RA aneurysm (four cases), renal vein thrombosis (three cases), arteriovenous fistula (three cases), peripheral infarction (one case of bilateral infarcts), and distal occlusive disease (three cases). One case of aortal coarctation was also found. Color Doppler US failed to demonstrate the proximal main RA in 25% of cases (among 193 RAs total including supernumerary RAs). The sensitivity and specificity of color Doppler US for the detection of RA stenosis or thrombosis were 89% and 99%, respectively. Color Doppler US thus appears to be effective in the diagnosis of renovascular disease in native kidneys.