[Gadolinium-enhanced thoracic CTA: retrospective analysis of image quality and tolerability in 45 patients evaluated prior to the description of nephrogenic systemic fibrosis]. / Angioscanographie thoracique au gadolinium en TDM multicoupe du thorax: analyse rétrospective de la qualité image et de la tolérance chez 45 patients évalués antérieurement à la description de la fibrose systémique néphrogénique.

PURPOSE: To assess the accuracy and torerability of gadolinium-enhanced thoracic CTA using a 64 MDCT compared to a 16 MDCT. Because this study was started prior to the description of NSF, particular attention was paid to long-term follow-up of the patient population. MATERIALS AND METHODS: The study protocol was approved by the ethics committee of our institution and informed consent was obtained from all patients. Fourteen patients (Group 1) (9 males and 5 females; mean age: 64.3 years) with contraindication to the administration of iodinated contrast material underwent thoracic CTA (collimation: 32 x 2 x 0.6 mm; pitch: 1.2) with gadolinium administration (0.5 mml/ml) at 0.4 mmol/kg injected at 6 ml/sec with evaluation of clinical and biological tolerability of the gadolinium based contrast agent. Results from this patient population were compared to results from a population of 31 patients (21 males; 10 females; mean age: 63.2 years) (Group 2) imaged on a 16 MDCT. All patients were folloowed-up for a mean time of 22.6 months. RESULTS: Using a mean contrast volume (standard deviation) that was not significantly different (Group 1: 54.8+/-11 ml; Group 2: 53.4+/-6.9 ml) (p=0.94), patients in Group 1 underwent complete thoracic CTA whereas patients in Group 2 underwent CTA of only the middle third of the thoracic region. All CTA examinations were diagnostic for Group 1 and Group 2 patients; however, evaluation of subsegmental vessels was possible in a significantly larger proportion of patients in Group 1 (10/14; 72%) compared to Group 2 (6/31; 19%) (p=0.003). Mean attenuation values within pulmonary arterial branches were similar for Groups 1 and 2 (central arteries: 194.5+/-51.3 HU vs 180.6+/-53.8 HU; p=0.38) (lobar arteries: 208.5+/-52.5 HU vs 189.9+/-60.1 HU; p=0.33) (segmental arteries: 220.4+/-50.4 HU vs 201.5+/-54.7 HU; p=0.42). Transient alteration of renal function was recorded in one patient from Group 1 with severe pre-existing chronic renal failure. No change in renal function was observed for Group 2 patients. No case of NSF was reported in patients with pre-existing renal failure at the time of enrollment. CONCLUSION: The use of gadolinium-based contrast agent for thoracic CTA using a 64 MDCT provides diagnostic quality examinations in all patients with improved image quality compared to a 16 MDCT. No complication other than transient alteration of renal function was observed. Because the likelihood of developing NSF may vary with the type of gadolinium-based contrast agent used, the least toxic agent should be used.

Adult Fanconi syndrome secondary to light chain gammopathy. Clinicopathologic heterogeneity and unusual features in 11 patients.

Fifty-seven cases of Ig light chain-associated Fanconi syndrome (FS) have been reported so far, mostly as isolated reports. The pioneering work by Maldonado and associates (35), who reviewed the first 17 cases in 1975, led to the unifying concept that patients with FS and Bence Jones proteinuria have a special form of plasma cell dyscrasia characterized by slow progression of the tumor and by prominent crystal formation in proximal tubule cells, in the absence of myeloma casts in the distal tubule. We carefully reappraised these characteristics in a series of 11 patients. Ten renal biopsy specimens were available for electron microscopy, adding to the 15 previously reported cases with ultrastructural studies. Moreover, 10 of the kappa light chains could be entirely or partially sequenced and tested for their resistance to cathepsin B, a lysosomal protease present in proximal tubule cells. Our series showed an unexpected clinicopathologic heterogeneity. Seven patients presented with the typical clinical and pathologic features of FS and low-mass myeloma or monoclonal gammopathy of undetermined significance (MGUS), in keeping with Maldonado et al's description. Crystals in bone marrow cells were detected in patients of this group, only. Three patients who presented with full-blown FS exhibited, however, the characteristic features of myeloma cast nephropathy in the setting of high-mass myeloma. One patient of this group also had numerous crystals in proximal tubule cells. The eleventh patient had complete FS with MGUS, but no crystals in proximal tubule cells even after electron microscopy. Contrasting with the clinicopathologic heterogeneity, genetic and biochemical analyses of the light chains showed a striking homogeneity. First, they all were of the kappa type. Second, 8 of 9 belonged to the V kappa I variability subgroup, which indicates that FS light chains are related by the sequence of their variable regions. Third, the 8 V kappa I light chain sequences most likely originated from only 2 germline genes, LCO2/012 and LCO8/018. Fourth, all 5 LCO2/012-derived sequences presented an unusual hydrophobic or nonpolar residue at position 30. These sequence peculiarities may account for unusual physicochemical properties of the light chains including the resistance of their variable domain V kappa to proteolysis by cathepsin B, observed in 7 of 9 patients in our series, while light chains isolated from patients with myeloma cast nephropathy are completely digested. Resistance of V kappa to proteolysis in FS patients can explain the accumulation of the light chain in the endocytotic compartment of the proximal tubule cells, leading to impairment of proximal tubule functions.

Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment.

BACKGROUND: Systemic capillary leak syndrome (SCLS) is a rare condition characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse swelling, weight gain, and renal shut-down. From the first publication in 1960, only 34 cases have been reported. OBJECTIVE: To collate enough patients to observe the natural history of the disease and evaluate the efficacy of empiric treatments. DESIGN: Multicentric retrospective study. RESULTS: Thirteen patients (6 women and 7 men) were collated with a mean follow-up of 6.4 years. Eight patients are still alive after a mean of 5.6 years (range 1 to 15). Three patients out of the 11 who were not lost to follow-up died; 1 during an attack and 2 because of a progression towards multiple myeloma. CONCLUSIONS: Our series shows an improvement in the prognosis of SCLS due most likely to improved management during attacks. Some patients' disease could evolve into a multiple myeloma. Treatment is still empiric and no prophylactic therapy, including terbutaline associated with aminophylline, has clearly proven its efficacy.

Effects of fish oil rich in polyunsaturated fatty acids on hyperlipidemia of hemodialysis patients.

Hemodialysis (HD) patients have a high incidence of hyperlipidemia. Hypertriglyceridemia is the most frequent abnormality encountered. It results mainly from a defect in the degradation of triglycerides. The aim of this study was to evaluate the efficacy of a fish oil (Max-EPA) rich in polyunsaturated fatty acids (PUFA), such as eicosapantenoic acid (EPA), on lipid abnormalities of hemodialysis patients. Thirteen hyperlipidemic HD patients were investigated (7 males, 6 females; mean age 57 years; mean duration of HD 72 months). None were diabetic or treated with antihypertensive drugs. All patients had hypertriglyceridemia (greater than 200 mg/dl) and an increase (greater than 1) in the ratio of serum apolipoprotein B to serum apolipoprotein A1 (ApoB/ApoA1). They received for one month, 6 g/day of Max-EPA providing 1 g of EPA. After treatment, serum triglyceride levels fell by 38% from 231 +/- 40 (SD) mg/dl to 140 +/- 38 mg/dl (P less than 0.01). Total cholesterol did not change significantly (before therapy 241 +/- 33 mg/dl, after therapy 249 +/- 38 mg/dl). Apolipoprotein A1 levels (116 +/- 17 mg/dl) were not modified after therapy, 117 +/- 11 mg/dl. Apolipoprotein B decreased significantly from 182 +/- 26 mg/dl to 150 +/- 21 mg/dl after treatment (P less than 0.01). The ApoB/ApoA1 ratio showed a significant decrease from 1.56 +/- 0.26 to 1.3 +/- 0.16 after therapy (P less than 0.01). Also, the greatest reductions were found in the patients who had both the highest serum triglyceride levels and the highest ApoB/ApoA1 ratios. No side effects were observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Liver transplantation eliminates insulin needs of a diabetic patient.

Organ transplantation and subsequent therapeutic agents may induce or worsen preexisting diabetes mellitus. We report the case of a diabetic patient whose insulin needs disappeared after liver transplantation. Non insulin-dependent diabetes mellitus was diagnosed when she was 47, and was treated by hypoglycemic drugs and then insulin. Chronic post-hepatitis C cirrhosis was diagnosed at the age of 55 and required liver transplantation 2 years later. During the postoperative course, the insulin doses required to maintain normal glucose levels progressively decreased, and insulin became completely unnecessary by the 29(th) postoperative day. After insulin was stopped, glucose levels remained within normal ranges for the 5-year-long follow-up, despite the worsening of a preexisting diabetic nephropathy and the occurrence of a diabetic retinopathy. This case highlights the fact that liver transplantation may eliminate insulin needs in a diabetic patient but also shows that degenerative complications may occur despite apparent remission of diabetes.

Nephrotic syndrome cured by removal of malignant gastric stromal tumor.

Malignant tumors have been associated with the development of nephrotic syndrome. We report a 73-year-old man with nephrotic syndrome who had a malignant stromal gastric tumor. Curative resection of the sarcoma resulted in remission of the nephrotic syndrome.

[Better understanding of atheromatous ischemic renal vascular disease]. / Mieux comprendre la pathologie vasculo-rénale ischémique athéromateuse.

Atheromatous ischaemic renal vascular disease (AIRVD) comprises ischaemic renal disease, atheromatous disease of the large arteries and intra-renal atheromatosis. Cholesterol emboli and lesions of nephroangiosclerosis are often associated, affecting the two kidneys. It is an increasingly common cause of chronic renal failure in an aging population, affecting 12 to 14% of new patients requiring dialysis in the United States. Atheromatous stenoses are very progressive with a risk of renal atrophy; they are a marker of polyvascular disease, often detected during other angiographic investigation. Hypertension secondary to the stenosis, still incorrectly called renovascular hypertension, is, however rare, affecting less than 0.5% of hypertensives. For economic reasons, it is important to select patients who need complementary investigation. In view of the absence of specific signs of the pathology, the "presumptive" diagnosis is based on a range of clinical and biological results, especially in a high risk context. The method of investigation varies from team to team, depending on the availability of equipment, the experience of the operators and the patient himself. Duplex Doppler, spiral angioscan and magnetic resonance angiography are the most pertinent investigations for the management of AIRVD. When the diagnosis of renal artery stenosis has been made, the problem of revascularisation, the objective of which is to preserve or restore the functional nephronic mass, has to be treated to prevent progression to end stage renal failure. Although epidemiological and physiopathological evidence is in favour of revascularisation, only renal salvage procedures are imperative. Apart from these indications, the clinical benefits of revascularisation have not yet been demonstrated. In all cases, the control of associated risk factors is essential to maintain the success of revascularisation and slow down the progression of atheromatous disease.

[Are demographic characteristics of hypertensive patients different with renal artery stenosis?]. / Les caractéristiques démographiques des hypertendus sont-elles différentes en présence d'une sténose artérielle athéromateuse rénale?

UNLABELLED: Atheromatous renal disease is more and more involved in end stage renal failure in polyatheromatous patients. The goal of this work was to study the demographic differences between hypertensive patients with renal artery stenosis (RAS) or without RAS. METHOD: Between November 1995 and July 1997, 49 hypertensive patients were included consecutively for a suspicion of RAS. Age, sex, hypertension history, tabagism, cardiovascular heredity, body mass index, diabetes history, hypercholesterolemia, kalmia, serum creatinine, creatinine clearance, systolic blood pressure (SBP), diastolic blood pressure (DBP) and pulse pressure (PP) were studied. A renal echo-doppler and a renal TDM were performed in all patients. A renal arteriography was performed in 23 patients with a RAS suspicion after the two morphologic exams. The demographic characteristics of both clinical groups control (group 1) and patients with unilateral RAS (group 2), were compared. STATISTICS: Descriptive analysis, Wilcoxon test, Khi 2 test, spearman correlation (p < 0.05). RESULTS: Group 2 patients were significantly older than group 1 patients (p = 0.008) with a mean age difference of 8 years. Creatinine clearance was lower in patients suffering from RAS with a mean difference of 23 mL/min between groups 1 and 2 (p = 0.0007) but we also had to take into account the negative correlation existing between creatinine clearance and age (r = 0.63; p = 0.0001) when interpreting these results. The DBP was lower (p = 0.03) and the PP higher (p = 0.01) in group 2. The SBP was higher in group 2 but this result was not significant. Mean differences in SBP and PP between group 1 and group 2 were 6 mmHg and 15 mm Hg respectively. Smoking was more common in group 2 (p = 0.04). The history of hypertension, cardiovascular heredity, sex ratio (M/F), body mass index and prevalence of diabetic were comparable between the two clinical groups. CONCLUSION: Although there were demographic differences between the two clinical groups, no clinical or biological variable could be used alone to identify which patients suffered from renal stenosis, because the distribution of these variables did not differ significantly between the two groups and the effective was small. Then, we thought that Krijnen's predictive rule is interesting in the screening of hypertensive patients with RAS suspicion.

[Muscle infarction. An unknown complication of diabetes mellitus]. / L'infarctus musculaire. Une complication méconnue du diabète.

INTRODUCTION: Diabetic muscle infarction (MI) is a rare and little-known complication of diabetes mellitus. CASE REPORT: We report a case of relapsing MI in which magnetic resonance imaging (MRI) suggested the diagnosis. A 53-year-old man with multi-complicated type II diabetes mellitus was admitted to our unit for illness and deep tumefaction of the right thigh. Because of unconclusive MRI, a muscular biopsy of the lesion was performed and MI confirmed. Three months after, a left relapse of MI occurred. Immediate treatment with immobilization and heparinotherapy permitted a rapid recovery. CONCLUSION: About 70 previously reported cases are reviewed. The mean age at presentation was about 40 years. MI was usually seen in patients with long-standing diabetes with multiple end organ microvascular complications. Homo- or heterolateral recurrences are reported in almost half of the patients. MRI is the best imaging technique for suggesting the diagnosis.

[Silicone inclusions in the spleen in a patient under chronic hemodialysis]. / Inclusions de silicone dans la rate chez une hémodialysée chronique.

A 67-year-old woman with chronic renal failure, in periodic hemodialysis, presented an hemolytic anemia in relation to splenic sequestration. Light Microscopic examination of the spleen revealed multiple granulomatous reaction. Numerous macrophages contained inclusions which were non-birefringent and non-staining with routine stains. Electron microscopy, scanning electron microscopy and energy dispersive X-Ray analysis were performed and revealed silicone. Its origin was silicone tubing of the hemodialysis equipment. Pathogenic effects of silicone are discussed.

[Retinochoroidal involvement of type II membranoproliferative glomerulonephritis. An angiographic study with indocyanine green]. / Atteinte rétinochoroïdienne dans les glomérulonéphrites membrano-prolifératives de type II (GNMP II). Une étude angiographique au vert d'indocyanine.

PURPOSE: Type II measangiocapillary glomerulonephritis is related to dense deposits within the glomerular basal membrane and the basal membrane of the pigment epithelium (Bruch's membrane). Being a vasculitis, an angiographic study by indocyanine green (ICG) could possibly enlarge the semiologic features of this disease. METHODS: The indocyanine green angiographic changes in 3 patients with predialitic renal failure due to type II measangiocapillary glomerulonephritis (MCGN II) (dense deposit disease) are reported. A complete ophthalmologic examination, electroretinography, electro-oculography, fluorescein and indocyanine green angiography (ICG) were performed for each patient. RESULTS: Abnormal hyperfluorescent dots were seen on the same part of the fundus on both fluorescein and ICG angiography, though these locations were different for each of the three patients. These results seem to link the deposits to vascular changes within the choriocapillaris, which opposes them to drusen encountered in age related macular degeneration (ARMD). It appears that the choriocapillary lesions could be similar to the glomerular disease. CONCLUSION: Beyond the diagnostic challenges related to the nephrologic disease, it is known that subretinal neovascularization occurs in some cases of MCGN II, although the pathophysiologic mechanism of the deposits is probably not the same as in ARMD. Therefore, ICG angiography should be performed when MCGN II is first known, serving as an initial examination for further follow-up.

[Exchangeable electrolytes, glycoregulation and growth hormone in one case of Bartter's syndrome (author's transl)]. / Electrolytes échangeables, glycorégulation et hormone de croissance au cours d'un syndrome de Bartter.

Total body water (T.B.W), extra-cellular fluid volume (E.C.F.), exchangeable sodium (NaE) and potassium (KE) were measured in one typical case of Bartter's syndrome with growth retardation. Intracellular potassium depletion was associated with a slight impaired carbohydrate tolerance and with decreased growth hormone response to arginine infusion and insulin-induced hypoglycemia. These observations demonstrate that impairement of growth hormone responses to stimulation occur in Bartter's syndrome with potassium depletion.

[The prognostic value of raised uric acid in the blood in arterial hypertension in pregnancy. 58 cases (author's transl)]. / Valeur pronostique de l'hyperuricémie dans les hypertensions artérielles de la grossesse. A propos de 58 observations.

The authors have carried out research in 58 cases to see whether the findings of high uric acid levels in the blood are of prognostic value in all cases of arterial hypertension in pregnancy whatever the aetiology. The material studied consisted of 30 true cases of toxaemia of pregnancy, 13 cases of recurrent toxaemia and 15 cases of the vasculo-renal syndrome on top of the pre-existing pathological condition. In 37 cases where the blood uric acid level was lower than 300 micromoles there were only 4 minor complications. Serious complications were found in 6 cases in which the patients had a blood uric acid level highthan 480 micromoles. This study confirms the prognostic value of blood uric acid levels. The estimation of these levels, however, seems to be of less value in making a differential diagnosis between the various kinds of toxaemia and hypertension found in pregnant women due to different aetiologies.

[Postpartum renal cortical necrosis]. / La nécrose corticale rénale du post-partum.

The authors report two cases of post-partum cortical renal necrosis. The diagnosis was made on clinical and biological criteria (severe, prolonged oliguria, the signs of intra-vascular coagulation defects, a rise in L.D.H. and anaemia with micro-angiopathy. It is confirmed by selective renal arteriography and renal punch-biopsy. There is a threefold method of treatment: --of the kidney condition by repeated haemodialysis and diuretics; --antihypertensive treatment; --treatment aimed at the aetiological pathology (heparin, anti-platelet aggregation and fresh plasma). Functional recovery of the kidney was obtained in both cases (after an interval of 1 year and an interval of 1 1/2 year). The authors review the diagnostic, physiopathological, morphological and therapeutic elements in the condition of cortical renal necrosis.

[The influence of present therapeutic methods and especially of beta-blockers on fetal and maternal prognosis in hypertensive syndromes in pregnancy. 43 case records (author's transl)]. / Influence des thérapeutiques actuelles et notamment des bêta-bloquants sur le pronostic foetal et maternel des syndromes hypertensifs de la grossesse. A propos de 43 observations.

The authors studied 43 cases of arterial hypertension in pregnancy in an attempt to determine the efficiency and safety of different anti-hypertensive drugs. The patients were divided into two major groups: arterial hypertension which revealed itself during pregnancy (true toxaemias of pregnancy and relapsing toxaemias), and arterial hypertensions which were added on to a pre-existing pathology (arterial hypertension, diabetes, chronic nephritis). The cases in these different classes were then divided into two definite groups according to the need for therapy: the first group was treated by rest and hydrallazine as a single therapeutic agent. In the second group multiple agents were needed because of the arterial hypertension, and one was a beta-blocker. Complications were found particularly in the second group of true toxaemias of pregnancy where unfortunately 5 fetal deaths occurred that were attributable to the severity of the hypertension more than to the beta-blockers, which were administered for longer and in higher doses without major complications in recurrent toxaemias and pre-existing arterial hypertension cases.

[Epidemiologic data on arterial hypertension in pregnancy]. / Données épidémiologiques sur l'hypertension artérielle gravidique.

A retrospective study of 241 case histories of essential hypertension in pregnancy treated in the Salengro Maternity Hospital of Lille from 1976 to 1981 was carried out. Looking at 450 readings of blood pressure in pregnancy it has been possible to work out a profile for these patients. These patients have as singular factors: They are often fat or very fat. They often have raised cholesterol levels in the blood. They often have a family history of hypertension or of diabetes. Many use oestro-progestogen birth control pills and had trouble in glucose regulation far more than women who had normal blood pressures in pregnancy.

[Hematologic changes induced by extracorporeal circulation]. / Modifications hématologiques induites par la circulation extracorporelle.

The plasma and cellular changes seen during the use of extracorporeal circulatory circuits define the system's degree of haemocompatibility. Heparin is still very much used to prevent activation of the blood clotting mechanisms and to reduce their effects. The fall in concentration of the clotting factors and their inhibitors is usually moderate; it is due to haemodilution, particularly important in cardiac surgery and during plasma exchanges. Fibrinolysis is often activated. In cardiac surgery, it is seen in nearly 20% of cases straight after the end of the ECC, and in nearly 80% of cases during the ECC. In all cases of resin haemoperfusion, there is an early transitory fibrinolytic burst, seen only rarely during haemodialysis and plasma exchanges. This phenomenon is usually well controlled by the natural inhibitors; it can be prevented by antifibrinolytic drugs. Cellular changes show the same trends during cardiac surgery, haemoperfusion and haemodialysis. Thrombopaenia is seen within a few minutes starting of ECC. It is caused by platelet activation, with aggregate formation; these are then trapped by the microcirculation. Leukopaenia occurs at the same time, later followed by rebound; complement activation could be the main cause by forming aggregates of polymorphonuclear cells and monocytes. Intravascular mechanical haemolysis reaches significant levels only in a few cardiac surgical procedures. The great speed of activation of the plasma and platelet enzyme systems by the ECC circuits explains these early changes. They are not only due to direct effects of the physiological circulatory characteristics and to contact with artificial surfaces, but also to plasma-cell interactions and to the patients' reaction to these first alterations.