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                   258 Chapter 6: Genitourinary system


                   Clinical features                              development of ARF, including hypercalcaemia and
                   Usuallypresentsasapainoracheinthebackorabdomen  dehydration. The mechanism of development of
                   which may be exacerbated by drinking large amounts  ‘myeloma kidney’ is via a direct toxic effect on re-
                   of fluid, for example it may become symptomatic for  nal tubular cells and blockage of the tubules and col-
                   the first time in students who drink large quantities of  lecting ducts by the paraprotein. In addition, patients
                   beer.Theremaybeapalpableloinmass.Occasionallythe  may develop amyloidosis and renal tubular acidosis as
                   hydronephrosis is so marked that it can mimic ascites.  complications of multiple myeloma. See page 490.
                     In some cases, it is asymptomatic and diagnosed in-     Amyloidosis: This condition may be systemic or con-
                   cidentally when an ultrasound is performed for another  fined to the kidneys and is an important cause of
                   reason. Congenital cases may be found on antenatal ul-  glomerulardisease.Itcancauseproteinuria,nephrotic
                   trasound scan, or in childhood during investigation of  syndrome and renal failure. See page 513.
                   urinary tract infections.                        Henoch–Sch¨ onleinpurpura(HSP):Thissystemicvas-
                                                                  culitis causes a purpuric rash, abdominal pains, joint
                   Complications                                  pains and a glomerulonephritis that is indistinguish-
                   Infectionofthedilatedsystemduetourinarystasis,renal  able from IgA nephropathy. This is unsurprising, as
                   stones and renal failure.                      HSP is characterised by tissue deposition of IgA con-
                                                                  taining immune complexes. See page 381.
                                                                    Systemic lupus erythematosus: This multisystem con-
                   Investigations
                                                                  nective tissue disease commonly affects the kidneys.
                   An IVU shows a dilated renal pelvis (often grossly) with
                                                                  SLE is the great mimicker of almost every type of
                   normal, undilated ureters. There is delayed passage of
                                                                  glomerulonephritis from minimal change disease, to
                   contrast, which is not overcome by administration of
                                                                  membranous nephropathy, to proliferative glomeru-
                   diuretics. If obstruction is in doubt, further investiga-
                                                                  lonephritis. See page 365.
                   tions as for urinary tract obstruction (see above) may be
                                                                    Systemic sclerosis: This connective tissue disorder can
                   needed.
                                                                  cause acute or chronic renal disease due to the dam-
                                                                  age caused to the renal arterioles. The only treatment
                   Management
                                                                  known to be of benefit is ACE inhibitors. See page 367.
                   If the PUJ obstruction is causing symptoms or renal     ANCA-associated vasculitis: This includes Wegener’s
                   impairment, reconstruction of the renal pelvis (pyelo-
                                                                  Granulomatosis and Polyarteritis Nodosa which can
                   plasty) can be undertaken so that the renal pelvis drains
                                                                  cause rapidly progressive glomerulonephritis, with
                   into the ureter, by excising the narrowed segment.
                                                                  acute renal failure. Early treatment with immunosup-
                                                                  pression regimes such as plasmapheresis, high dose
                   Prognosis                                      steroids and cyclophosphamide can improve renal
                   It is not possible to predict how much function will re-  function. See pages 124 and 379.
                   coverandasmallproportionofpatientscontinuetohave
                   pain.
                                                                Thrombotic thrombocytopenic
                                                                purpura – haemolytic uraemic
                   The kidney in sytemic disease                syndrome
                     Hypertension: See page 73.                 Definition

                     Diabetes mellitus: This is a common cause of renal  Haemolytic uraemic syndrome (HUS) and thrombotic

                     disease and accounts for a large number of patients  thrombocytopenicpurpura(TTP)areprobablytwosep-
                     whoneed dialysis. See page 451.            arateentities,buthavesuchsimilarclinicalpresentations
                     Multiple myeloma: Many patients with multiple  and findings that they are usefully considered together.

                     myeloma will develop renal complications such as  When acute renal failure is predominant it is called
                     proteinuria, acute or chronic renal failure and amy-  HUS, and when neurological problems are predominant
                     loidosis. There are several contributing factors to the  it is called TTP. Often both ends of the spectrum are
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