RENAL CASE 6.1 Nephrotic Syndrome

SECTION 6 | RENAL

Sandra Benavides

LEARNING OBJECTIVES CHIEF COMPLAINT: Follow-up for kidneys 1. Identify common History of Present Illness: A 17½-year-old Hispanic male diag- presenting signs nosed with nephrotic syndrome at 5 years of age presented to and symptoms of nephrology clinic for follow-up. His initial years of diagnosis nephrotic syndrome. were characterized by frequently relapsing nephrotic syndrome. 2. Recognize and Approximately 3 years ago, he was treated with cyclosporine develop therapeutic for a period of time due to frequent relapses. However, when he strategies for the had a relapse while on cyclosporine, he developed severe uremia complications of and neurologic symptoms of uremia within 3 months of treat- nephrotic syndrome. ment. Thereafter, the cyclosporine was discontinued and he was 3. Design a monitoring started on CellCept. On that, he was free of edema for a few years. plan for efficacy However, he recently recovered from a major relapse characterized and toxicity of by anasarca, a very high hemoglobin (up to 19 g/dL), and hypo- pharmacologic natremia. He was admitted to the local children’s hospital, first therapy for nephrotic rehydrated with saline and then eventually with 25% albumin. syndrome. He was released but then readmitted shortly thereafter because 4. Determine of difficulty in controlling the relapse. He received multiple doses appropriate of Solu-Medrol infusions and completed a four-dose course of outcomes for rituximab infusions. He went into a prolonged remission after considering the that for approximately 2 years. However, he became nonadherent discontinuation with his medications and was seen last month for relapse. At that of pharmacologic time, he was found to be hemoconcentrated and was readmitted therapy in nephrotic to the hospital for 5 days; he continued to have complications syndrome. related to hyponatremia, decreased urine output, and marked 5. Identify important anasarca. He received two doses of Solu-Medrol and one dose of health maintenance albumin followed by Lasix, which he seemed to respond to, and education points then he underwent the rituximab treatment (two doses on this for patients with course). The patient, however, was released a week ago even nephrotic syndrome. though his anasarca remained because of psychosocial issues. He requires Lasix once a day and feels that the edema has somewhat improved but not subsided. His mother states that he started to have a cold during this time.

Review of Systems: Patient complains of swelling and mild abdominal discomfort but no respiratory distress. He reports a runny nose and headache but no sore throat, fever, or cough.

Past Medical History: Nephrotic syndrome (10 years); allergic rhinitis (4 years)

Past Surgical History: None

49 Pediatric Pharmacotherapy Self Assessment 50

MEDICATION HISTORY

Medication Sig Start Date End Date Taking Authorizing Provider

Mycophenolate 250 mg po bid 2 yr prior 1 wk ago No Dr. Abbott

Mycophenolate 500 mg po bid Dose increased at 1 wk ago Yes Dr. Abbott last admission Vitamin D 1,000 units po daily 1 yr prior Yes Dr. Abbott

Montelukast 5 mg po hs 4 yr Yes Dr. Borerro

Prednisone 60 mg po daily 2 yr prior Yes Dr. Abbott

Enalapril 10 mg po daily 1 yr Yes Dr. Abbott

Simvastatin 10 mg po daily 1 yr Yes Dr. Abbott

Ibuprofen 400 mg po q 6 hr as 1 wk Yes Self-administered needed for headache

Furosemide 20 mg po daily 1 wk Yes Dr. Abbott

Social History: Lives with parents and GU: No scrotal swelling attends school, denies alcohol and drug use Musculoskeletal: Pitting edema 2+ up to his Family History: No known history of kidney knees disease Skin: Skin showed keloids over both ears from Immunizations: UTD, negative for seasonal piercings influenza vaccine (refuses) Neurological: Nonfocal Allergies: NKDA LABORATORY DATA PHYSICAL EXAM BASIC METABOLIC PANEL BP 138/94 mm Hg | Pulse 106 beats per min Component Value Range Wt 77.8 kg (between 75th and 85th percentile) Glucose 84 60–110 mg/dL Ht 179.5 cm (between 50th and 75th percentile) BUN 7 7–17 mg/dL BMI 24.1 kg/m2 (75th percentile) Sodium 143 137–145 mmol/L General Appearance: Alert, no facial swelling, Potassium 3.8 3.6–5.0 mmol/L but significant pitting edema on both legs and Chloride 106 98–107 mmol/L ascites CO2 27 22–30 mmol/L HEENT: Mild pharyngeal injection, some Creatinine 0.52 0.60–1.00 mg/dL rhinorrhea, no lymphadenopathy; eyes normal; Calcium 9.6 8.4–10.2 mg/dL had an eye exam this year and it was WNL Phosphorus 3.4 2.5–4.5 mg/dL Neck: The neck is supple, no lymphadenop- Albumin 2.1 3.5–5 g/dL athy, no thyromegaly CBC Heart: RRR, no murmur WBC count 3.8 4.5–11.0 x 103/µL RBC count 4.73 4.30–5.10 million/µL Lungs: Lungs sound clear, no wheezes, no rales Hemoglobin 14.3 11.4–15.4 g/dL Abdomen: Soft, positive for ascites but not Hematocrit 43.1 36.0% to 49.0% tender Platelets 220 150–450 x 103/µL