Page 248 - Medicine and Surgery
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                   244 Chapter 6: Genitourinary system


                     Goodpasture’s disease (anti-Glomerular Basement  Investigations

                     Membrane (GBM) disease).                       Urinedipstickispositiveforbloodandmaybepositive
                     Systemic diseases such as systemic lupus erythe-  for protein. Urine microscopy may demonstrate the

                     matosus (SLE), Henoch–Sch¨ onlein purpura (HSP),  dysmorphic red blood cells and red cell casts, which
                     vasculitis (Wegener’s granulomatosis, polyarteritis),  indicate a nephritic urine.
                     malignanthypertensionandhaemolytic-uraemicsyn-     Patientsshouldbeinvestigatedasforacuterenalfailure
                     drome (HUS).                                 (see page 234).
                                                                  Renal biopsy is required in most cases to help iden-

                   Pathophysiology                                tify the underlying cause, deomonstrate the pattern of
                   Proliferation of endothelial cells and mesangial cells, or  disease, indicate prognosis and guide management.
                   vasculitis, leads to occlusion of the capillary lumen, re-     FBC and peripheral blood film particularly to look for
                   duced blood flow, oliguria and acute renal failure. Dam-  thrombocytopenia, and evidence of haemolysis.
                   aged glomeruli leak red blood cells causing microscopic     More specific tests which are useful in nephritic
                   (occasionally macroscopic) haematuria. The low GFR  syndrome include:
                   also leads to activation of the renin–angiotensin sys-  1 ANA and anti-dsDNA (anti-double stranded DNA
                   tem, exacerbating hypertension. Proteinuria may also be  antibody is specific for SLE)
                   present.                                       2 ANCA - Anti-neutrophil cytoplasmic Antibody
                     Focal nephritis: When less than 50% of the glomeruli
                                                                    (found in vasculitides such as Wegener’s)
                     are affected this usually manifests as haematuria  3 Anti-GBM antibody
                     with or without minor proteinuria. The majority of  4 Complement C 3 and C 4 – these are low in certain
                     glomeruli are unaffected so renal failure is minimal or  conditions.
                     absent.                                      5 ASO and anti-DNAase – these are evidence of a pre-
                     Diffuse nephritis: If more than 50% of the glomeruli
                                                                    ceding streptococcal infection
                     are affected then oliguria and acute renal failure re-  6 Serum cryoglobulins
                     sults. If diffuse nephritis is severe (with crescents
                     in most of the glomeruli) then rapidly progressive
                                                                Management
                     glomerulonephritis results.
                                                                This is as for acute renal failure. Urgent treatment of
                                                                the underlying cause is often needed to prevent perma-
                   Clinical features                            nent loss of renal function and early referral to a renal
                   The full nephritic syndrome includes haematuria, pro-  physician is necessary.
                   teinuria, hypertension and oedema (from salt and water
                   retention), oliguria and uraemia, but the features are
                   variably present. Often, the patient is unwell and there  Acute diffuse proliferative
                   may be features of the underlying illness, for exam-  glomerulonephritis
                   ple haemoptysis with Goodpasture’s syndrome, rash,
                                                                Definition
                   joint pains, a preceding infection, e.g. diarrhoea or a
                                                                A diffuse global glomerular disease, which is immune
                   sore throat. Headache and loin pains are common non-
                                                                complex mediated and usually precipitated by a preced-
                   specific features. Salt and water retention can lead to
                                                                ing infection.
                   hypertensive encephalopathy and pulmonary oedema.
                                                                Incidence
                   Macroscopy/microscopy
                                                                The commonest glomerulonephritis worldwide, falling
                   The kidneys are oedematous, swollen, with scattered pe-
                                                                in the United Kingdom.
                   techiae sometimes seen. The microscopic appearances
                   are described in greater detail in section on Glomeru-
                   lar Disease (see page 240) and under each individual  Age
                   condition.                                   Anyage, peak in schoolchildren.
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