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Review Question - QID 104606

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QID 104606 (Type "104606" in App Search)
A 3-year-old girl is brought to her pediatrician by her mother, who is concerned that her daughter has developed puffy eyes and swelling in her feet. Physical examination confirms bilateral lower extermity edema to the mid-calf. The patient's medical history is significant for acute hepatitis B infection at the age of 9 months that was acquired through perinatal transmission. Laboratory studies are obtained and show a serum albumin level of 2.2 g/dL and 4+ protein and no blood on urinalysis. Hepatitis B serologies are also obtained and show positive HBsAg and positive anti-HBc IgM antibodies. Which of the following is the most likely diagnosis in this patient?

Minimal change disease

36%

14/39

Focal segmental glomerulosclerosis

10%

4/39

Membranous glomerulonephritis

51%

20/39

IgA nephropathy

0%

0/39

Poststreptococcal glomerulonephritis

0%

0/39

Select Answer to see Preferred Response

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This patient is suffering from nephrotic syndrome. Although minimal change disease is the most common overall cause of nephrotic syndrome in children, the combination of chronic hepatitis B infection and nephrotic syndrome in this patient makes membranous glomerulnoephritis the most likely diagnosis.

Membranous glomerulonephritis presents similar to other nephrotic syndromes, with generalized edema noted on examination as well as hypoalbuminemia and gross proteinuria. Causes can include infections (Hepatitis B & C, syphilis, malaria), occult malignancy, lupus, certain medications (gold, penicillamine). Membranous glomerulonephritis is most commonly treated with steroids and cyclophsophamide. The disease course may be progressive with a waning response to steroid therapy; however, more commonly, the disease is associated with slow recovery and relatively low rates of persistent asymptomatic proteinuria.

Lam et al. discuss the management of pregnant mothers and infants with hepatitis B infection. When hepatitis B is passed from mother to infant in the peripartum period, the child has a 90% chance of becoming a chronic carrier of the disease. To minimize this outcome, mothers should undergo prenatal screening for hepatitis B infection. If the mother is infected, infants should receive hepatitis B immune globulin and vaccination within 12 hours of birth. Additionally, all infants should be vaccinated against hepatitis B prior to being discharged from the hospital.

Levy et al. discuss the development of membranous nephropathy after perinatal Hepatitis B infection. The time between infection with hepatitis B infection and subsequent development of kidney disease is variable and unknown. The authors discuss a case study in which a newborn acquired hepatitis B infection in the peripartum period and subsequently developed proteinuria at 6 years of age.

Illustration A shows the process of immune complex deposition that occurs in membranous glomerulonephropathy. Illustration B is an electron microscopy image showing the classic 'spike and dome' pattern seen with membranous glomerulonephritis.

Incorrect Answers:
Answer 1: Although minimal change disease is the most common cause of nephrotic syndrome in young child, the coexistence of hepatitis B infection in this patient makes membranous glomerulonephritis the more likely diagnosis.
Answer 2: FSGS is associated with HIV infection, not Hepatitis B, and commonly occurs in young African American males with refractory hypertension.
Answer 4: IgA nephropathy (Bergers disease) is a nephritic syndrome; this patient's laboratory results are more consistent with a nephrotic syndrome.
Answer 5: Poststreptococcal glomerulonephritis is a nephritic syndrome; this patient's laboratory results are more consistent with a nephrotic syndrome. Additionally, there is no history of preceding impetigo or pharyngitis.

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