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Glomerular diseases present clinically in several different ways, depending on the nature and severity of the primary disease and the extent to which the normal physiological functions of the glomerulus are perturbed.1,2 Some children with glomerulonephritis (GN) are found incidentally to have microscopic hematuria or proteinuria when checked by routine urinalysis but are otherwise asymptomatic. At the other extreme, children may become critically ill with oligoanuric rapidly progressive GN in need of urgent dialysis. A few glomerular diseases are inherited (see Chapter 473), but most forms of GN are acquired and are generally considered to be immunologically mediated. There are three classical clinical syndromes that develop from glomerular injury: acute and chronic glomerulonephritis, defined by the triad of hematuria, hypertension, and azotemia; nephrotic syndrome, defined by proteinuria and hypoalbuminemia; and hemolytic uremic syndrome, defined by microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency.

The pathophysiological sequence of events that lead to the development of the nephritic triad (hematuria, azotemia, and hypertension) are shown in Figure 472-1. In each of the clinical entities with glomerular proliferation, the inflammation process leads to decreased glomerular perfusion, resulting in compromised renal function and retention of salt and water with potential development of hypertension and edema.

Figure 472-1.

Approach to the evaluation of a child with glomerulonephritis, based on serum C3 level. ANCA, antineutrophil cytoplasmic antibody; ASPGN, acute poststreptococcal glomerulonephritis; HIV, human immunodeficiency virus; MPGN, membranoproliferative glomerulonephritis; SLE, systemic lupus erythematosus.

Approach to a Patient with Glomerulonephritis

The patient with glomerular disease presents clinically with a constellation of features that may include hematuria, proteinuria, hypertension, edema, and renal insufficiency. The urinary sediment is characterized as active when dysmorphic erythrocytes and cellular casts are present. A series of questions guides the initial diagnostic and management plan.1

  1. 1. Does this patient have acute or chronic GN? Many patients with chronic GN appear relatively asymptomatic until the disease is advanced. Clues of chronicity include significant anemia, evidence of renal osteodystrophy (abnormal bone radiographs or an elevated PTH level), or small echogenic kidneys on ultrasound examination. Acute onset of severe hypertension often causes neurological symptoms such as headaches and seizures, while long-standing hypertension of insidious onset may be clinically silent, but left ventricular hypertrophy may be present.

    2. Does this patient have isolated renal disease, or are extrarenal organ systems involved? A careful systems review and physical examination will help determine whether the investigation should move in the direction of primary (acquired) GN or toward multisystem disease associated with GN (Table 472-1). Relevant extrarenal involvement may be clinically silent. For example, postinfectious serological studies (such as a streptozyme or anti–hepatitis B or anti–hepatitis C antibodies) may be indicated if infection-associated GN is a possibility. When patients present with vasculitis caused by Wegener’s granulomatosis, involvement of the lung ...

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