1/5. Focal segmental glomerulosclerosis: a need for caution in live-related renal transplantation.Focal segmental glomerulosclerosis (FSGS) has increasingly been recognized to occur in a familial pattern. We have observed the development of biopsy-confirmed FSGS and subsequent end-stage renal disease (ESRD) in one live related kidney donor and ESRD without biopsy in another. Both donors had family members with ESRD secondary to FSGS. Both donors were apparently healthy by routine physical examination, urinalysis, and serum creatinine at the time of evaluation as live related donors. We believe these cases emphasize the need for great caution when evaluating siblings as potential live related donors.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/5. Chronic inflammatory demyelinating polyradiculoneuropathy and severe peripheral oedema: a renal explanation.Inflammatory demyelinating neuropathies have been associated with membranous and focal sclerosing glomerulonephritis. Here we describe a 58 year old man with a clinical history, physical examination and laboratory investigations consistent with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), who also had severe lower limb and sacral oedema resistant to medical therapy. Mild proteinuria was present and a renal biopsy showed features consistent with focal sclerosing glomerulonephritis (FSGN). The patient's weakness and oedema did not respond to i.v. immunoglobulin or plasmapheresis but responded to high dose oral prednisone. The oedema was not explained by immobility, hypoproteinaemia or local factors. The occurrence of the oedema in a person with CIDP and FSGN and its improvement with prednisone, together with improvement in CIDP and FSGN, suggests that it was immune mediated, possibly due to increased capillary permeability. The presence of renal disease in patients with inflammatory demyelinating neuropathies may be more common than currently realised.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/5. Secondary focal segmental glomerulosclerosis in non-obese patients with increased muscle mass.BACKGROUND: Secondary focal segmental glomerulosclerosis (FSGS) is a pattern of glomerular injury mediated by hyperfiltration and other adaptive structural-functional responses. We describe 3 non-obese patients with elevated body mass index (BMI) owing to increased muscle mass who had renal biopsy findings favoring a form of secondary FSGS. methods: Clinical and pathologic data were obtained on 3 patients with 1) renal biopsy findings of focal segmental and/or global glomerulosclerosis with glomerulomegaly; 2) BMI > or = 30; 3) body fat percentage < 20%; 4) "highly muscular" appearance, and 5) proteinuria > or = 1 g/d without nephrotic syndrome. 24-hour urine creatinine excretion was used to estimate lean body mass and percentage body fat. RESULTS: The 3 patients were males (age 38 - 48 years) employed in jobs requiring strenuous physical activity. BMIs ranged from 30.4 - 32.1 kg/m2 with body fat percentages of 12.9 - 16.8%. creatinine clearances at time of biopsy ranged from 113 - 208 ml/min. Renal biopsies showed focal segmental and/or global glomerulosclerosis affecting a minority of glomeruli with glomerular hypertrophy and minimal (mean 15%) foot process effacement. Treatments included angiotensin-converting enzyme inhibitor, angiotensin ii receptor blocker, or weight loss. Over a mean follow-up time of 24.3 months, serum creatinine remained stable and proteinuria decreased in all patients. CONCLUSIONS: Non-obese patients with increased BMI due to elevated muscle mass are at risk of developing a secondary form of FSGS that resembles obesity-related glomerulopathy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/5. Concurrent FSGS and Hodgkin's lymphoma: case report and literature review on the link between nephrotic glomerulopathies and hematological malignancies.BACKGROUND: The link between the nephrotic syndrome (NS) and malignancy was first described in 1922. In solid tumors, the NS is most often due to membranous glomerulonephropathy, whereas in common hematological malignancies, minimal-change disease predominates. Focal segmental glomerulosclerosis (FSGS) is among the least frequently reported renal lesion associated with malignancy. methods: We report a case of the simultaneous diagnoses of FSGS and Hodgkin's lymphoma, and review the literature on various nephrotic glomerulonephropathies associated with common leukemia and lymphoma. RESULTS: Although nephrotic glomerulonephropathies rarely occur in association with acute leukemia, they have often been described in chronic lymphocytic leukemia (CLL). Membranoproliferative glomerulonephropathy and membranous glomerulonephropathy are the most common lesions observed in CLL. Nephrotic glomerulonephropathies have also been well documented among patients with lymphomas, in particular, Hodgkin's lymphoma. While minimal-change disease is most commonly found in association with Hodgkin's lymphoma, more diverse and complex renal lesions are associated with non-Hodgkin's lymphoma. FSGS remains a rare association with hematological malignancies. CONCLUSIONS: Nephrotic glomerulonephropathies are not only linked to solid-organ tumors, but also to hematological malignancies. A thorough evaluation, including a physical examination for lymphadenopathy and organomegaly, as well as a hematological evaluation, must be performed in all patients presenting with nephrotic glomerulonephropathies.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/5. cyclosporine A (CyA)-induced decrease of serum gonadotropin levels in a case of Klinefelter's syndrome.We report a case of Klinefelter's syndrome who developed a decrease of serum gonadotropin levels, particularly LH, after CyA treatment for complicated focal glomerulosclerosis (FGS). A 38-year-old man suffering from general malaise and pretibial edema was diagnosed FGS by renal biopsy in October 1988, and was referred to our hospital for further evaluation and treatment for FGS in December 1988. He was not married, and closer anamnesis revealed that he had had impaired seminal ejaculation from the age of 30. The physical examination showed 37% obesity, scanty body hair, pretibial edema and small bilateral testes (3.0 x 1.5cm). Laboratory findings included marked proteinuria (5.3g/day) and mild renal dysfunction (serum creatinine 1.3mg/dl, glomerular filtration rate 57.2ml/min). Endocrinologically, high basal levels of LH and FSH (133.6mIU/ml and 93.7mIU/ml, respectively) and the hyperresponses of LH and FSH to LH-RH stimulation were found, but the other pituitary hormone levels, thyroid and adrenal status, were in the normal range. In testicular biopsy, nodularly proliferated leydig cells and no seminal tubules could be seen. The chromosome analysis showed 47,XXY karyotype, which confirmed the diagnosis of Klinefelter's syndrome in this patient. From 9 January 1989, CyA (6mg/Kg.day) was orally administered for 4 weeks in order to treat for FGS. After CyA administration, basal levels of LH and FSH remarkably decreased, particularly LH, and their decrease lasted for at least 6 weeks after cessation of CyA (final levels; LH 28.2mIU/ml, FSH 69.8mIU/ml). On the other hand, serum testosterone level was low normal or slightly under normal, and no apparent changes could be seen during CyA treatment.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |