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R Makdassi

Publications and source records attributed to R Makdassi.

At least 19 recordsLinked to original sources
PubMed2004

[Inaugural unilateral adrenal hematoma of an antiphospholipid syndrome].

INTRODUCTION: A primary antiphospholipid syndrome is a very rare cause of adrenal haemorrhage. OBSERVATION: A 51 year-old man presented with a unilateral adrenal haemorrhage, enhanced by the prescription of Synacthène during the 4 days that preceded. There was no adrenal deficiency but the immunological control revealed the presence of anti-phospholipid antibodies. After 2 years of follow-up, adrenal controls have not shown any underlying tumour or endocrine insufficiency. COMMENTS: Adrenal involvement is described in the anti-phospholipid syndrome and may present in the form of adrenal deficiency in the case of occasionally only microscopic bilateral haemorrhages. Furthermore, Synacthène is known to induce adrenal haemorrhages although this complication remains rare. Moreover, any unilateral adrenal haemorrhage requires subsequent follow-up for several months or even years in order to eliminate any underlying tumour and to control the absence of any adrenal deficiency if the involvement is bilateral.

Adrenal Gland Diseases↗
PubMed2004

[Muscular haematoma in Henoch-Schönlein purpura].

INTRODUCTION: Henoch-Schonlein purpura is one of the most frequent systemic vasculitis in children. In adults, muscle involvement is extremely rare and not very well characterized. We report a case of Henoch-Schonlein purpura with severe skin and renal involvement in witch multiple intramuscular haematoma leaded to severe anaemia. Histological examination confirms the muscle localization of the disease. EXEGESIS: A 68 years old man treated by oral anticoagulation for multiple venous thrombosis, was admitted with necrotic vasculitis of the skin, abdominal pain and segmental IgA glomerulopathy. The diagnosis of Henoch-Schonlein purpura was rapidly made and intensive steroid therapy started. After rapid improvement, a haemorrhagic shock due to voluminous intramuscular haematoma was diagnosed by MRI. Histological examination of the muscle, confirms the localization of the disease. CONCLUSION: Intramuscular haematomas are very uncommon in Henoch-Schonlein purpura. There are usually a consequence of muscular immune complex vasculitis. In our patient, high dose corticosteroid was not unable to control the disease.

Aged↗
PubMed2003

[Acute renal insufficiency secondary to interferon].

Alpha interferon is increasingly used in the treatment of malignancies and viral hepatitis. Renal involvement after its use consist in more cases on proteinuria. We report one case of acute renal failure without nephrotic syndrome but with tubulointerstitiel infiltration by mononuclear cells in 65 old man who had before interferon therapy 151 mumol/l of serum creatinine. Interferon was administrated for digestive carcinoid neoplasia and bone and liver metastasis. Outcome was improvement of renal function after withdrawn of interferon, dialysis and steroid treatment.

Acute Kidney Injury↗
PubMed2003

[Amyloidosis and pleural calcification].

INTRODUCTION: Pulmonary diseases during the course of generalised amyloidosis are principally represented by tracheobronchial involvement and diffuse parenchymal localizations. CASE REPORT: The authors report the case of a 66-year-old woman presenting with pleural amyloidosis in the context of generalised amyloidosis. Thoracoscopy performed in the investigation of recurrent transudative pleural effusions found evidence of an inflamed parietal pleura with areas of calcification. Pleural biopsies confirmed amyloid infiltration pleural ossification. Talc pleurodesis was performed. CONCLUSIONS: The authors' conclusion is that, meeting an unexplained pleural effusion even transudative during the course of a generalised amyloidosis, the thoracoscopy is the diagnosis key test as it allows moreover a pleural pleurodesis to be performed.

Aged↗
PubMed2002

[Chronic interstitial nephritis induced by 5-aminosalicylic acid: a new case report].

5-aminosalicylic acid (5-ASA) used for the treatment of inflammatory bowel disease is known to induce chronic interstitial nephritis (CIN). However, the frequency of occurrence and the spectrum of severity of 5-ASA-induced CIN are not known. In this paper, we report a new case of CIN induced by 5-ASA in a patient treated for about 7 years for a Crohn disease. After that 5-ASA was discontinued and prednisone therapy started, renal function improved partially. About 30 observations of CIN induced by the use of 5-ASA in patients treated for inflammatory bowel disease have already been published. None were published in patients treated for other diseases such as arthritis rheumatism. The review of the literature suggests that the prognosis is poor and correlates with the duration of treatment, the cumulative dose and the level of renal impairment at diagnostic. We believe that the control of the renal function in patients treated by 5-ASA must be regular and prolonged.

Adult↗
PubMed2000

[Collapsing glomerulopathy and cytomegalovirus, what are the links?].

BACKGROUND: Collapsing glomerulopathy is a form of focal and segmental glomerulosclerosis which occurs preferentially in black people. It causes severe nephrotic syndrome and quickly progresses towards end-stage renal failure. CASE REPORT: We report the case of a 16-year-old black girl from Guadeloupe who was admitted for tetanus and edema in 1996. She had hypoparathyroidism, renal failure and a nephrotic syndrome as well as cytomegalovirus infection. Renal biopsy showed collapsing glomerulopathy. The renal function improved on glucocorticoid and ganciclovir therapy and her serum creatinine stabilized around 250 mumol/l for two years. DISCUSSION: Collapsing nephropathy is the cellular type of focal and segmental glomerulosclerosis. The main etiology is the human immunodeficiency virus. A viral infection may be involved in its pathogenesis. Other viruses could be linked with this nephropathy. This case report relates a case associated with a cytomegalovirus viruria. The clinical course might be related with the antiviral treatment.

AIDS-Associated Nephropathy↗
PubMed2000

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.

Adult↗
PubMed1999

[Extracapillary glomerulonephritis with anti-myeloperoxidase antibodies in 2 patients with systemic scleroderma treated with penicillamine D].

BACKGROUND: Side-effects, including autoimmune disorders, are frequent with D-penicillamine therapy. Proteinuria is observed in 10% of the patients, often secondary to extramembranous glomerulopathy. Necrotizing extracapillary glomerulonephritis is however exceptional. CASE REPORTS: Two patients with systemic sclerodermia were treated with D-penicillamine for 7 and 14 years. Both developed necrotizing extracapillary glomerulonephritis with anti-myeloperoxidase antibodies (anti-MPO), associated with hemorrhagic alveolitis in one case. Partial regression of the renal failure was obtained after withdrawal of D-penicillamine and combination treatment with prednisone and cyclophosphamide. DISCUSSION: Extracapillary glomerulonephritis or a lung-kidney syndrome are frequently associated with anti-MPO antineutrophil cytoplasm antibodies (ANCA). In systemic sclerodermia, the presence of anti-MPO appears to define a group of patients at risk of pauci-immune extracapillary glomerulonephritis or a lung-kidney syndrome. In addition, the presence of ANCA in patients with renal failure would suggest extracapillary glomerulonephritis rather than sclerodermic microangiopathy. Development of extracapillary glomerulonephritis with anti-MPO in patients who are taking D-penicillamine suggests that inductor mechanisms other than D-penicillamine are involved in the pathogenesis of these glomerulopathies.

Aged↗
PubMed1999

[Factors in the progression of renal insufficiency during the 2 years preceding the use of dialysis].

UNLABELLED: The respective contribution of sex, type of nephropathy, degree of proteinuria, blood pressure, protein and sodium daily intake, lipid profile, protidemia, hemoglobinemia, acidosis and CaPO4 product on the rate of renal failure progression is debated. The link between these parameters and the decrease of creatinine clearance, delta Ccr (according to Cockroft) was assessed in uni and multivariate analysis in a population of 49 patients (26 men, 23 women; age 60 +/- 15 years, weight 73 +/- 15 kg) selected out of 173 presently treated hemodialysis patients on the basis of availability of a quarterly follow-up for two years before starting dialysis. The patients were advised a moderate protein and salt restriction which could be retrospectively assessed (on urinary excretion of urea and sodium) at respectively 0.82 g/kg/day and 6.5 g/day. The two years delta Ccr was 14 +/- 14 ml/min. It was not different in men and women (specially when expressed in % of initial value). This decrease in Ccr was neither significantly different in glomerular disease (17 +/- 8, n = 14), diabetic nephropathy (12 +/- 6, n = 7), nephroangiosclerosis (15 +/- 8, n = 5), interstitial nephritis (12 +/- 10, n = 14), and PKD (11 +/- 12, n = 9). Patients with antihypertensive drugs (n = 42) had a faster progression than those without drugs (n = 7): delta Ccr = 15 +/- 14 vs 7 +/- 7 ml/min (p < 0.05) in spite of comparable blood pressure but with higher proteinuria. Linear regression of delta Ccr with the initial and two year averaged values of the quantitative parameters showed a significant positive link for both values with cholesterol, hemoglobin and proteinuria and a negative one with protidemia. A positive link was observed with the initial value of bicarbonate and the two year mean of diastolic and mean blood pressures. No link at all was observed with urea and Na excretion, CaPO4 product and triglycerides. Multiple regression disclosed a significant link only for protidemia (negative with both initial and two years averaged value), diastolic BP (only for the two year averaged value and hemoglobinemia (for the initial value). When the patients were classified according to a threshold value of their protidemia, DBP, hemoglobinemia, and cholesterolemia those with the combination of two risk factors of progression (pro-tidemia < 66 g/l, DBP > or = 90 mmHg, hemoglobinemia > 11 g/dl, proteinuria > 3g/d, CT > 5 mmol/l) had a significantly greater decrease of Ccr than those with the three other combinations at the exception of the association of low protidemia with DBP. CONCLUSION: 1. diastolic hypertension and low protidemia are the two most important factors predicting progression of renal failure; 2. a predictive synergy was furthermore pointed out between on one hand low protidemia and diastolic hypertension and on the other hand proteinuria and cholesterol; 3. on the contrary, anemia attenuates progression linked to low protidemia, diastolic hypertension, proteinuria and high cholesterol.

Acidosis↗
PubMed1999

Risk factors of renal failure progression two years prior to dialysis.

AIM: The respective contribution of sex, type of nephropathy, degree of proteinuria, blood pressure, protein and sodium daily intakes, blood lipid profile, protidemia, hemoglobinemia, acidosis and CaPO4 product on the rate of renal failure progression is debated. PATIENTS AND METHODS: The link between these parameters and the decrease of creatinine clearance, deltaCcr (according to Cockroft) was assessed in uni- and multivariate analysis in a population of 49 patients (26 women; age 60+/-15 years, weight 79+/-15 kg) selected out of 173 presently treated hemodialysis patients on the basis of availability of a quarterly follow-up for 2 years before starting dialysis. The patients were advised a moderate protein and salt restriction which could be retrospectively assessed (on urinary excretion of urea and sodium) at, respectively, 0.82 g/kg/day and 6.5 g/day. RESULTS: The 2-year deltaCcr was 14+/-14 ml/min. It was not different in men and women. This decrease in Ccr was neither significantly different in gomerular disease (17+/-8, n = 14), diabetic nephropathy (12+/-6, n = 7), nephroangiosclerosis (15+/-8, n = 5), interstitial nephritis (12+/-10, n = 14), and PKD (11 +/-12, n = 9). Patients with antihypertensive drugs (n = 42) had a faster progression than those without drugs (n = 7): deltaCcr = 15+/-14 vs 7+/-7 ml/min (p < 0.05) in spite of comparable blood pressure but with higher proteinuria. Linear regression of deltaCcr with the initial and 2-year averaged values of the quantitative parameters showed a significant positive link for both values with cholesterol, hemoglobine and proteinuria and a negative one with protidemia. A positive link was observed with the initial value of bicarbonate and the 2-year mean of diastolic and mean blood pressures. No link at all was observed with urea and Na excretion, CaPO4 product and triglycerides. Multiple regression disclosed a significant link only for protidemia (negative with both initial and 2-year averaged value), diastolic BP (only for the 2-year averaged value and hemoglobinemia (for the initial value). When the patients were classified according to a threshold value of their protidemia, DBP, hemoglobinemia, and cholesterolemia those with the combination of 2 risk factors of progression (protidemia > or = 66 g/l, DBP > or = 90 mmHg, hemoglobinemia > 11 g/dl, proteinuria > or = 3 g/d, CT > 5 mmol/l) had a significantly greater decrease of Ccr than those with the 3 other combinations at the exception of the association of low protidemia with DBP. CONCLUSION: Diastolic hypertension and low protidemia are the 2 most important factors predicting progression of renal failure. A predictive synergy was furthermore pointed out between low protidemia or diastolic hypertension with proteinuria and cholesterol. On the contrary anemia attenuates progression linked to low protidemia, diastolic hypertension, proteinuria and high cholesterol.

Anemia↗
PubMed1997

Steady-state captopril renography: continuous monitoring of the captopril-induced increase in 99mTc-MAG3 mean parenchymal transit time in renovascular hypertension.

Steady-state captopril renography (SSCR) is an original technique for assessing the captopril-induced increase in technetium-99m mercaptoacetyltriglycine (99mTc-MAG3) mean parenchymal transit time (MPTT) in kidneys affected with functional renal artery stenosis (RAS). The steady-state parenchymal activity achieved by constant infusion of 99mTc-MAG3 is directly linked to the MPTT of the radiopharmaceutical. This steady-state parenchymal activity was continuously monitored from 15 min before to 60 min after a single dose of captopril in order to detect possible disruption of the steady state. SSCR was performed in 11 hypertensive patients with unilateral RAS and in two with RAS of a solitary kidney before renal revascularization (RR). In four of these patients, an additional SSCR was performed after RR. Of the ten patients whose hypertension was cured or improved by RR, one presented an uninterpretable SSCR and six presented a positive SSCR on the affected side. Control SSCR performed in four of these six patients was bilaterally negative. SSCR was also bilaterally negative in the three patients who showed no blood pressure response to RR. These preliminary results tend to indicate that, in spite of the stability of pre- and post-captopril hydration and data acquisition conditions allowed by this steady-state technique, the sensitivity is lower than expected. However, the reason for the false-negative results does not seem to be inherent to SSCR.

Adult↗
PubMed1997

[Transesophageal echographic demonstration of ulcerated atheromatous plaques of the thoracic aorta responsible for cholesterol emboli].

The authors present of case of a 61-year-old man suffering from cholesterol emboli, in whom transoesophageal echocardiography revealed complex atheromatous lesions of the thoracic aorta. There is growing emphasis, at the present time, on the concept of triggering factors with the multiplication of endovascular radiological investigations, the more widespread availability of cardiac surgery and the use of anticoagulants and fibrinolytics. The prognosis is poor, treatment is only palliative and preventive measures are therefore essential.

Aorta, Thoracic↗
PubMed1997

[Thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome in adults. Apropos of 27 cases].

UNLABELLED: We report a series of 27 patients included on the basis of either thrombotic microangiopathy (TMA) at renal histology (13 cases) or, in the absence of histology, non-immunological hemolytic anemia with schizocytes and thrombopenia (14 cas). The etiopathogenic treatment consisted in the administration of antiagregating agents (in all patients except 3 of group I because of the severity of thrombopenic), corticosteroids (1 case), intravenous immunoglobulins (2 cases) fresh frozen plasma (FFP) without plasma exchange (PE) in 7 cases and PE with FFP in 13 patients. According to the 6 months outcome, 4 groups were considered I: death due to neurological damage; II: chronic hemodialysis; III: partial renal recovery; IV: complete renal recovery. COMMENTS AND CONCLUSIONS: a/Patients with neurological complications have poor prognosis in spite of minor renal involvement and use of PE whose indication is validated in these cases. b/When renal involvement predominates, accelerated hypertension is linked to arteriolar or mixte type of TMA, exposes to an increased risk of hemorrhagic complications of the renal biopsy (4 out fo 5) which questions the usefulness of such biopsy (group II). c/TMA may precede cancer. It has per se a favorable outcome even when metastases are already present, warranting aggressive treatment.

Adolescent↗