[Kikuchi-Fujimoto's disease or histiocytic necrotizing lymphadenitis: A report of two familial cases]. / Maladie de Kikuchi-Fujimoto ou lymphadénite histiocytaire nécrosante : à propos de deux cas familiaux.

INTRODUCTION: The histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto's disease is characterized by a lymph node inflammation whose similarity with systemic lupus is generally admitted. CASE REPORT: Our description of two familial cases aims at raising the hypothesis of the existence of a genetic background in this disease following the example of what is observed in the autoimmune diseases. CONCLUSION: Pathophysiology of Kikuchi-Fujimoto's disease is probably multifactorial and may include predisposing genetic background and a possible infectious triggering event.

[Epileptic seizures and hematemesis in a young patient with sickle cell disease]. / Épilepsie puis hématémèse chez une jeune patiente drépanocytaire.

INTRODUCTION: Sickle cell disease is a multi-faceted disease, which can affect all organs. Here, we report the case of a young woman whose clinical presentation was confusing. CASE REPORT: An 18-year-old patient from Martinique in Caribbean area presented to the emergency room with widespread pain, as part of a vaso-occlusive crisis. She reported being followed for SS sickle cell anemia, with a history of vaso-occlusive crises and exchange transfusions in the past. Her hemoglobin rate was 83g/L. She was treated with opioid analgesics. Then, she presented several generalized tonic-clonic seizures and major episodes of hematemesis, which proved to be simulated by the patient, whose hemoglobin electrophoresis result was finally AS. CONCLUSION: This patient had therefore the Münchausen syndrome, mimicking sickle cell anemia, like eight other cases reported in the literature.

[Idiopathic retroperitoneal fibrosis: a multicentric retrospective study of 30 French cases and follow-up of the renal function]. / Fibrose rétropéritonéale idiopathique : évolution à long terme du pronostic rénal dans une série rétrospective multicentrique de 30 cas.

PURPOSE: Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory disorder, affecting the aorta and the surrounding vessels and tissues. The prognosis is mainly driven by the risks of chronic kidney disease and relapse. Our aim was to assess the prevalence of chronic kidney disease at follow-up. METHODS: We retrospectively reviewed the medical records of patients diagnosed for IRF in Seine-Saint-Denis (France) between 1987 and 2011. We collected informations about presentation, radiologic findings and follow-up. Diagnosis of IRF was confirmed when all the following criteria were met: infiltration of the infrarenal aorta or iliac vessels, absence of aneurysmal dilation, lack of clinical suspicion of malignancy. RESULTS: Thirty patients were identified, with a male/female ratio of 4.9. Mean age was 55±13 years old. The mean creatinine clearance was 66 mL/min/1.73 m(2) and the mean CRP was 45±36 mg/L. In 24 (80%) patients, the location of IRF was periaortic and periiliac. Eleven patients (37%) underwent a diagnostic biopsy, and 14 (47%) required an ureteral procedure. A mean follow-up of 63 months was available for 29 patients: 69% relapsed, 7 developed chronic renal disease (24%), and one died of urinary sepsis. Older age (P=0.023), diabetes (P=0.007), and initial renal insufficiency (P=0.05) were associated with a risk of chronic renal insufficiency. CONCLUSION: The high frequency of relapses and chronic renal disease emphasizes the need of close follow-up in patients diagnosed with IRF.

Impact of a multidisciplinary staff meeting on the quality of antibiotherapy prescription for bone and joint infections in orthopedic surgery.

INTRODUCTION: We studied the impact of a weekly multidisciplinary staff meeting (MSM) on the quality of antibiotherapy for bone and joint infections in orthopedic surgery, as part of professional practice assessment. MATERIALS AND METHODS: We retrospectively studied the file of patients hospitalized for bone and joint infection. We compared antibiotherapy compliance to good use (bacteriology, dose, length of treatment, length of adaptation to microbiology), and outcome at six months for patients with bone and joint infections, before (March 2007 to March 2009) and after (March 2009 to March 2011) implementation of the multidisciplinary staff meeting. We identified 28 patient files (32 infections) before MSM and 26 patient files (28 infections) after MSM. RESULTS: Antibiotherapy was adapted in 47% of cases before MSM, versus 96% after (P<0.0001). The dose was optimum in 72% of infections before MSM, versus 89% after (P=0.11) and the length of antibiotherapy complied with recommendations in 41% of infections before MSM, versus 86% after (P=0.0005). The average time of antibiotic adaptation to the antibiogram changed from 2 days before MSM to 1.7 days after (P=0.43). Forty seven per cent of patients were cured at six months before MSM, versus 57% after (P=0.45); the rate of treatment failure at six months decreased from 25% before MSM to 18% after (P=0.75). CONCLUSION: The effectiveness of antibiotherapy significantly improved concerning the spectrum and treatment duration (P ≤ 0.0005) after implementing MSMs in orthopedic surgery. But the clinical impact at six months was not significant due to the small population sample.

Fish-eye disease: structural and in vivo metabolic abnormalities of high-density lipoproteins.

Fish-eye disease (FED) in humans is characterized by corneal opacities and markedly decreased plasma concentrations of high-density lipoprotein (HDL) cholesterol, apolipoprotein (apo) AI, and apo All, but no tendency to precocious atherosclerosis is present. To elucidate this paradox, the structure of HDL, the potential of serum to promote cholesterol efflux from cultured cells, and the in vivo metabolism of HDL were examined in a 53-year-old woman with a FED syndrome in association with a markedly decreased lecithin:cholesterol acyltransferase (LCAT) activity in HDL due to a mutation of the LCAT gene (Arg158 --> Cys). HDLs isolated by ultracentrifugation were small and enriched in unesterified cholesterol and phospholipids at the expense of cholesteryl esters and proteins. The apolipoprotein content showed an enrichment in apo E and apo AIV, whereas apo AI and apo All were dramatically reduced. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and immunoblotting using specific antibodies showed that the apo E was free or covalently bound to apo All. These particles analyzed by electron microscopy were small and round lipoproteins with a size similar to the smallest fraction of normal HDL3. The potential capacity of the serum to promote efflux from the cells was approximately 40% of control serum levels, but FED HDLs were as efficient as control HDLs in promoting cholesterol efflux from cells. To assess the metabolism of HDL apolipoproteins, in vivo apolipoprotein kinetic studies were performed using endogenous labeling techniques in the patient with FED and three control subjects. All subjects were administered D3-labeled leucine by primed constant infusion for up to 10 hours. The fractional synthetic rates (FSRs) of apo AI and apo All in the patient were 0.674 and 0.594 per day, clearly higher than in controls, 0.210 +/- 0.053 and 0.148 +/- 0.014 per day for apo AI and apo All, respectively. Apo AI and apo All production rates in the patient with FED were normal, 11.32 and 2.62 mg/kg x d, respectively, as compared with those in normal subjects, 11.45 +/- 1.23 and 2.68 +/- 0.17 mg/kg x d. These data established that hypoalphalipoproteinemia in FED was caused by marked hypercatabolism of apo AI and apo All. This hypercatabolism could be the consequence of structural abnormalities due to the selective LCAT deficiency. In conclusion, two steps of reverse cholesterol transport, cholesterol efflux and apo-HDL metabolism, appeared particularly efficient. This efficiency could participate in the absence of premature atherosclerosis in FED patients as regards the low HDL level.

Changes in lipid and lipoprotein levels and Achilles tendon diameters and indices in familial hypercholesterolaemic patients with tendinous xanthomatosis treated by diet and bezafibrate for 2 years.

Thirteen young adult patients suffering from heterozygotic familial hypercholesterolaemia with tendinous xanthomatosis, previously treated with a suitable special diet, were studied to assess the effect of bezafibrate, given for 2 years at a dose of 800 mg/day, on plasma lipid and lipoprotein levels and on changes in size of the Achilles tendon xanthomas. Measurements were made before and at intervals during treatment, the tendinous xanthomas being measured by an echographic procedure to give data on antero-posterior and lateral diameters, thus enabling an Achilles tendon index to be defined. The results confirm the hypolipidaemic activity of bezafibrate, changes in the levels of total cholesterol, triglycerides, lipids and lipoproteins (LDL, VLDL and HDL) being similar in direction and magnitude to those reported previously. A significant regression in the size of the Achilles tendon xanthomas was observed in 11 of the 13 patients, and the regression in the Achilles tendon index correlated significantly with a favourable change in the ratio HDL/LDL + VLDL. It is suggested that, as a result of this objective observation, a favourable effect of bezafibrate treatment would possibly be seen on the anatomical atheromatous lesions which are usual in this type of hyperlipidaemia.

[Effect on lipids, lipoproteins and apoproteins of labetalol prescribed in doses of 400 mg/day in hypertensive patients. Double-blind versus placebo study]. / Effet sur les lipides, lipoprotéines et apoprotéines du labétalol prescrit à la dose de 400 mg/j chez des patients hypertendus. Etude en double aveugle contre placebo.

The effects of labetalol on plasma lipoprotein metabolism were evaluated in a 3-month double-blind drug versus placebo study conducted on 30 consenting hypertensive patients, 15 of whom had normal plasma lipid levels and 15, minor type II hyperlipoproteinaemia; 20 patients received labetalol 400 mg/day and 10 the placebo. All patients remained in stable nutritional status throughout the study. Full clinical examination and blood sampling were carried out 30 days before, and on days 0, 30 and 90 of treatment. Whole blood was collected after 12 hours' fasting and immediately centrifuged prior to determination of plasma lipids (total cholesterol and triglycerides, by enzymatic assay), lipoprotein lipids (HDL, HDL2, HDL3, LDL, VLDL separated by ultracentrifugation in density gradient), apoproteins A1 and B (by laser immunonephelometry) and post-heparin lipoprotein lipase activity (PHLA). Significant changes in heart rate and systolic and diastolic blood pressures were noted in patients under labetalol but not in patients under placebo. Lipid and apolipoprotein levels were similar in both groups on day 0, and no significant variation in lipids, lipoprotein lipids and apolipoproteins were observed after 30 and 90 days of treatment with either labetalol or the placebo. At the end of treatment PHLA was unmodified in the group under placebo and raised in the group under labetalol (p = 0.05). The absence of changes in blood lipid values was found both in patients with normal lipidemia and in those with hyperlipidaemia. This study confirms that labetalol in doses of 400 mg/day has notable anti-hypertensive activity and, as previously reported and in contrast with other beta-blocking agents, is devoid of any adverse effect on lipid metabolism.

[Pseudophlebitis of the lower limbs. A critical study from 102 suspected cases of deep venous thrombosis]. / Les pseudo-phlébites des membres inférieurs. Etude critique à partir de 102 suspicions de thrombose veineuse profonde.

Clinical diagnosis of deep venous thrombosis appears as uneasy, because of inconstant and non-specific symptoms. When studying a phlebitis, the risk to diagnose venous thrombosis is over 50 p. 100. This review of 102 patients, supposed to present with phlebitis, confirms such data. The diagnosis reliability depends mainly on the physician's experience. Examination and clinical research, combined with Doppler data make possible to perform a good diagnosis in 4 cases out of 5. However, in 20 p. 100, phlebography is strongly required, appearing as an essential examination. Analysis of epidemiologic and clinical data demonstrates the importance of certain data as for the diagnosis: female sex, age superior to 60 years, existence of two antecedents and/or a cardiopathy, a complex clinical picture might demonstrate a diagnosis of deep venous thrombosis. Post-phlebitic syndrome and skin infectious pathologies are the main pseudophlebitis etiologies. Popliteal cyst (often mentioned in English literature as a pseudophlebitis factor) is diagnosed in 11 p. 100 of cases.

[Lipids, lipoproteins, arterial accidents and oral contraceptives]. / Lipides, lipoproteines, accidents arteriels et contraceptifs oraux.

PIP: This work reviews lipoprotein metabolism and relationships to atherosclerosis, examines the nature of arterial accidents and lipid modifications that occur with oral contraceptive (OC) use, and assesses the practical consequences for OC prescription. Cholesterol, triglycerides, and phospholipids are not soluble in aqueous milieus, and their transport in plasma is provided by macromolecules comprising a protein part and a lipid part. 5 types of these lipoproteins are distinguished by their relative richness in lipids and protein and by the nature of their proteins. The chylomicrons carry exogenous triglycerides to the peripheral tissues and cholesterol of dietary origin to the liver. Very low density lipoprotein (VLDL) cholesterol is secreted by the liver and transports triglycerides and cholesterol of endogenous origin. Low denisty lipoprotein (LDL) cholesterol originates in the degradation of VLDL cholesterol and transports cholesterol to the cells. High density lipoprotein (HDL) cholesterol is secreted by the liver and intestines or formed in the course of degradation of chylomicrons and VLDL cholesterol. Its role is to carry excess cholesterol in the peripheral tissues to the liver for elimination in the bile. Cholesterol thus follows 2 different pathways in the body: a path from the liver to the peripheral cells, whose markers are LDL and VLDL cholesterol and the plasma apoprotein B, and a path of return of excess cholesterol from the tissues and especially the arteries to the liver, marked by HDL cholesterol and the plasma apoprotein A. Only a proper balance between the 2 flows can prevent an excess of cholesterol in the arteries and the consequent constitution of atherosclerotic lesions. LDL and to a lesser extent VLDL cholesterol are strongly and positively correlated to atherogenic risk, while HDL cholesterol is negatively correlated to risk, independently of other risk factors. Arterial accidents occurring with OC use do not seem to be atheromatous in nature. A study by the Lipid Research Clinics of 2000 OC users and nonusers found that users had higher levels of total cholesterol, of triglycerides, and of LDL and VLDL cholesterol, while the elevation of HDL cholesterol was minimal. The effects of combinations of hormones in OCs depend on their composition. OCs with high or medium doses of estrogen cause an elevation in total cholesterol, triglycerides, and LDL and VLDL cholesterol. HDL cholesterol rises slightly with 19 norsteroids and declines with norgestrel. The ratio of total to HDL cholesterol is on the whole increased. OCs with low estrogen doses induce a decline inHDL cholesterol while the levels of total cholesterol and triglycerides remain unchanged. High dose progestin-only pills induce increases in LDL and decreases in HDL cholestrol. Total cholesterol tends to increases with 19 norsteroids and decline with noregestrel while triglycerides vary slightly. With smaller doses of progestin, less intense effects may be seen. The theoretic atherogenic risk determined by the levels and ratio of total and HDL cholesterol is thus increased with some hormonal combinations. OCs can be prescribed for women with normal lipid balance after a pretreatment lipid profile determination. Lipid balance should be reassessed regularly. OCs are contraindicated in cases of moderate or severe hypercholesterolemia and primary hypo HDLemia. Combined OCs may be used in cases of mild hyperlipoproteinemia in which other contraceptive methods are not possible if regular monitoring is provided.^ieng

Comparative evaluation of the effects of ciprofibrate and fenofibrate on lipids, lipoproteins and apoproteins A and B.

In a double-blind study over a 3-month period, a daily dose of 100 mg ciprofibrate, prescribed in a single administration and a daily dose of 300 mg fenofibrate, prescribed in 3 administrations, significantly reduced the mean values of total cholesterol, LDL cholesterol and VLDL cholesterol, apoprotein B (P less than 0.001) and increased the mean values of HDL cholesterol (P less than 0.01) and total apoprotein A (P less than 0.05). The study, followed-up as an open trial using higher doses (100 or 200 mg/day ciprofibrate, 400 mg/day fenofibrate) tried to demonstrate clearly the benefit of therapy after 9 months with the 2 drugs and to establish the dose-response effects. Comparison of the 2 drugs at the optimal dosages, after 9 months of treatment, showed ciprofibrate to be more effective in increasing HDL cholesterol (P less than 0.05) and apo A (P less than 0.001). No other significant differences in terms of either therapeutic efficacy or biological tolerance became apparent between the 2 drugs. The results obtained in this comparative study were in accordance to those observed in separate trials for ciprofibrate or fenofibrate. Ciprofibrate has the benefit of a long half-life and may also be administered in the form of a single daily dose to patients suffering from major type II hyperlipoproteinaemia.

[Sea-blue histiocytes and storage diseases. 3 cases]. / Histiocytes bleu de mer et maladies de surcharge. Trois observations.

"Sea-blue" with prominent blue granules on Giemsa staining have been described in many diseases. The authors report three cases of storage diseases, in which these particular cells have been found. The significance and pathogenesis of "sea-blue" histiocytes are discussed.

[Dyslipoproteinemic xanthomas]. / Les xanthomes dyslipoprotéinémiques.

The authors recall the clinical and histochemical aspects of xanthomas and their relations with disturbances in serum lipids and lipoproteins. Experiments in animals and clinical studies in humans are then reviewed. In the light of these results, physiopathological mechanisms involved in development of xanthomas and their connections with atherogenesis are discussed.

[Riedel's thyroiditis and retroperitoneal fibrosis. Apropos of a case of multiple fibrosing disease]. / Thyroïdite de Riedel et fibrose rétropéritonéale. A propos d'une observation de maladie multifibrosante.

The authors report a case of a 53 year old woman with Riedel's thyroiditis and retroperitoneal fibrosis. The thyroiditis has been diagnosed a year before the retroperitoneal fibrosis. This one was idiopathic and has been responsible of the death of the patient during an operation performed for ureterolysis. Fifteen other similar cases have been reported in the literature: four of them associated also mediastinal and biliary ducts fibrosis. The existence in one patient of multiple fibrosclerosis locations and the similitude of histological patterns (inflammatory diffuse fibrosis) lead to the concept of a multiple sclerosing disorder involving Riedel's thyroiditis, Ormond's disease, mediastinal fibrosis, sclerosing cholangitis, and the pseudotumors of the orbit. The etiopathogenic processes remain unclear.

[Genetic hypolipoproteinemias]. / Les hypolipoprotéinémies génétiques.

The very rare inherited hypolipoproteinemias are of great help to understand the relations between lipoproteins and atherosclerosis; moreover, in this field, they raise questions, which are discussed in this paper.

[Primary hyperlipoproteinemias. Clinical, biological and physiopathological aspects]. / Les hyperlipoprotéinémies primaires. Aspects cliniques, biologiques et physiopathologiques.

Primary hyperlipoproteinemias are of great interest for the physician and searcher, because of their atherogenic properties; on the other hand, a new type of hyperlipoproteinemia, namely hyperalphaliproproteinemia, seems to be a protective factor against clinical complications of atherosclerosis. The clinical, biological and pathophysiologic aspects of these diseases are studied both from the author's experience and literature data.

[Genetic hypolipoproteinemia]. / Les hypolipoprotéinémies génétiques.

The very rare inherited hypolipoproteinemias are of great help to understand the relations between lipoproteins and atherosclerosis; moreover, they raise questions in this field, which are discussed in this paper.

[Dyslipoproteinemic xanthoma]. / Les xanthomes dyslipoprotéinémiques.

The authors recall the clinical and histo-chemical aspects of xanthomas and their relations with serum lipids and lipoproteins disturbances. The experimental works in animals and in human clinic are then reviewed. At the light of these results the xanthoma development physiopathology and its relation to atherogenesis is considered.