Ehlers-Danlos syndrome coexisting with juvenile nephronophtisis.

Ehlers-Danlos syndrome (EDS), a heterogeneous disease of the connective tissues, is diagnosed by a triad of symptoms that include skin hyperextensibility, joint hypermobility and connective tissue fragility. Nephronophtisis (NPH) is an autosomal recessive interstitial nephritis leading to terminal renal insufficiency around puberty. The occurrence of these two rare diseases together is unusual.

Nephrotic syndrome revealing a disseminated Langerhans' cell histiocytosis.

Langerhans' cell histiocytosis (LCH) is a clonal disorder of proliferating histiocytes, which can affect various organs. The clinical picture ranges from localized to disseminated disease with multiple organ involvement and severe organ dysfunction. Renal involvement is uncommon and the association with glomerulonephritis is unusual.

[Primary focal segmental glomerulosclerosis in adults: response to treatment and outcome].

Response to therapy of primary focal segmental glomerulosclerosis (FSGS) in adults is poor, most studies demonstrated no response at all. We report our experience from a retrospective study about 22 adults patients. All patients presented proteinuria at admission with a nephrotic syndrome in 86 percent of cases.

[Extramembranous glomerulonephritis and myelodysplastic syndrome].

Membranous glomerulonephritis (MGN) is the main cause of nephrotic syndrome in adults and is usually idiopathic. We report a case of nephrotic MGN associated with a myelodysplastic syndrome (MDS) in a 43 year old man. The initial treatment consisted of oral corticosteroids (1 mg/kg/day).

[Cutaneous tumors during xeroderma pigmentosum in Morocco: study of 120 patients].

Introduction: Xeroderma pigmentosum is a rare recessive and autosomically transmitted genodermatosis. Its cutaneous manifestations are dominated by skin cancers. This investigation aims at studying the epidemiologic, clinic, histologic, therapeutic and evolutive aspects of the skin tumors during xeroderma pigmentosum.