[Severe Schonlein-Henoch nephritis in adults. A report of twenty cases]. / Les néphropathies sévères du purpura rhumatoïde de l'adulte. A propos de vingt observations.

Twenty patients with severe Schönlein-Henoch nephritis were selected on a histologically basis of diffuse proliferative endo- and/or extracapillary glomerulonephritis during a period of 12 years in Champagne-Ardenne. There were 15 men and 5 women, mean age 44.1 years. An infectious history was found in 40%, an urinary tract cancer in 15%. In all cases there was purpura, in 80% joint pain and in 50% digestive symptoms. Clinical presentation at diagnosis included, in all cases, hematuria (gross in 50%) and proteinuria (of nephrotic range in 80%); there was hypertension in 60% and renal failure in 80%. Histology found, in all cases, mesangial IgA and often C3 deposits, with a diffuse endocapillary proliferation in 10%, extra-capillary proliferation in 30% and both endo-extracapillary in 60%; 45% of the patients had crescents in greater than 50% of glomeruli. The outcome, after steroid and immunosuppressive treatment, was end-stage renal failure in 25%, moderate renal failure in 20%, or normal renal function in 55% with a mean follow-up period of 4.6 years. These severe nephritis were associated with repetitive and often necrotic purpura, frequent joint pain and severe digestive symptoms. The analysis of initial renal presentation confirmed the bad prognosis of nephrotic syndrome, renal failure and especially hypertension, which were well correlated with the severity and diffusion of proliferative lesions. Despite a worse known prognosis, these nephritis responded to an aggressive and early treatment.

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.

[Paraplegia and dissection of the abdominal aorta after closed trauma. Apropos of a case. Current review of the literature (1982-1993)]. / Paraplégie et dissection de l'aorte abdominale après traumatisme fermé. A propos d'un cas. Revue de la littérature récente (1982-1993).

A case of dissection with thrombosis of the subrenal abdominal aorta after blunt trauma led to paraplegia and bilateral ischaemia of the lower limbs in addition to acute abdominal signs. A review of the recent literature (1982-1993) revealed 32 reported cases. The patients were predominantly male and most often victims of an automobile accident (18 cases) or crushing trauma (6 cases). The clinical picture associated diverse degrees of abdominal signs, ischaemia and sensorial-motor impairment of the lower limbs. The diagnosis was established immediately on D0 in only 18 cases, early on days 1 to 7 in 4 cases and was late (day 8 to day 30) in 5 cases or very late (beyond day 30) in 6 cases. The difficulty in immediate diagnosis was related to the absent or incomplete vascular symptomatology or the late onset of the first signs. When a lesion of the aorta was suspected, an arteriography, angioscanner or peroperative exploration led to diagnosis. Neurological signs were frequent (10 cases including 8 with paraplegia) and generally related to ischaemia of the peripheral nerves. They may lead to denate from the diagnosis of vascular lesions. Fractures of the intima (17 cases) was the most frequent aortic lesion which also involved fracture of the media in a number of cases. Dissection was associated in 7 cases and complete or partial thrombosis of the aorta in 7. False aneurysms observed in 6 cases are the usual pathological form in cases of late diagnosis. Nearly all of the lesions were subrenal. Damage to abdominal organs was frequently observed.(ABSTRACT TRUNCATED AT 250 WORDS)

[Acquired multicystic disease of the renal sinus]. / Maladie multikystique acquise du sinus rénal.

A case of multilocular parapelvic cyst in a 62 years old male patient is reported herein. The features and differential diagnosis of this offently incidental disposis are discussed. The specificity of computed tomography eliminates the need for invasive diagnostic procedures.

[Retroperitoneal rhabdomyosarcoma revealed by acute kidney failure in children. Report of a case]. / Rhabdomyosarcome rétropéritonéal chez un enfant révélé par une insuffisance rénale aiguë. A propos d'un cas.

Retroperitoneal sarcomas are rare tumors representing 0.1% to 0.2% of all malignant tumors. They are usually revealed by an abdominal mass (33%), possibly associated with pain (41%). Edema of the lower limbs is reported in 10% of cases. Acute renal failure with anuria by bilateral ureteral compression, associated with invasion of the inferior vena cava has not been previously reported.