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Entity Clinical Course Prognosis

Postinfectionglomerulonephritis (GN). Onsetoccurs 1014 d after acute illness,commonly streptococcal.

Characteristics include acuteonset, tea-colored urine, mild tosevere renal insufficiency, andedema.

Acute phase is usually over in 2wk. Complete resolution occursin 95% of cases. Severity ofrenal failure and hypertension

varies. Microhematuria maypersist to 18 mo.Hypocomplementemia resolvesin 130 d.

Excellent. Chronic disease israre. Severe proteinuria, atypicalpresentation or course, orpersistent hypocomplementemia

suggest another entity.

Membranoproliferative GN.Presentation ranges from mildmicrohematuria to acute GNsyndrome. Diagnosis is made byrenal biopsy. Etiology is unknown.Types I and II are most common.Lesion is chronic.

Course can be mild to severe(rapid deterioration in renalfunction); may mimicpostinfection GN. Proteinuriacan be severe. Complementdepression is intermittent topersistent. Hypertension isusually significant.

Type I may respond tocorticosteroids. Type II (densedeposit disease) is less treatable;function decrease varies fromimmediate to as long as 15 y in3050% of untreated cases.

IgA nephropathy. Classicpresentation is asymptomaticgross hematuria during acuteunrelated illness, withmicrohematuria between episodes.Occasional instances of acute GNsyndrome occur. Etiology isunknown. Diagnosis is made bybiopsy.

90% of cases resolve in 15 y.Gross hematuric episodesresolve with recovery from acuteillness. Severity of renalinsufficiency and hypertensionvaries. Proteinuria occurs inmore severe, atypical cases.

Generally good; a smallpercentage develops chronicrenal failure. Proteinuria in thenephrotic range is a poor sign.There is no universally acceptedmedication. (Corticosteroids maybe useful in severe cases.)

Henoch-Schnlein purpura GN.Degree of renal involvementvaries. Asymptomaticmicrohematuria is most common,

but GN syndrome can occur. Renalbiopsy is recommended in severecases; it can provide prognosticinformation.

Presentation varies with severityof renal lesion. In rare cases,may progress rapidly to seriousrenal failure. Hypertension

varies. Proteinuria in thenephrotic range and severedecline in function can occur.

Overall, prognosis is good.Patients presenting with > 50%reduction in function orproteinuria exceeding 1 g/24 h

may develop chronic renal failure.Severity of renal biopsy picturecan best guide approach in suchcases. There is no universallyaccepted medication.

GN of systemic lupuserythematosus (SLE).Microhematuria and proteinuria arerarely first signs of this systemicdisease. Renal involvement varies,but severe GN may ensue withremissions and exacerbationsthroughout the course.

Renal involvement is mild tosevere. Clinical complexitydepends on degree of renalinsufficiency and other systemsinvolved. Hypertension issignificant. Manifestations of theseverity of the renal lesion guidetherapeutic intervention.

Renal involvement accounts formost significant morbidity in SLE.Control of hypertension affectsrenal prognosis. Medication isguided by symptoms, serology,and renal lesion. End-stage renalfailure can occur.

Hereditary GN (eg, Alportsyndrome). Transmission isautosomal-dominant/X-linked, withfamily history marked by end-stagerenal failure, especially in youngmales. Deafness and eyeabnormalities are associated.

There is no acute syndrome.Females are generally lessaffected but are carriers.Hypertension and increasingproteinuria occur with advancingrenal failure. There is no knowntreatment.

Progressive proteinuria andhypertension occur early, withgradual decline in renal functionin those most severely affected.Disease progresses to end-stagerenal failure in most males.

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Table 221. Glomerular diseases encountered in childhood.