Steriods: A good thing in Childhood MPGN ? By Brent Lee Lechner CPT, MC, USA

History 10 year old White Female presents to PCP: Periorbital edema during trip to Netherlands, which started 15 days prior visit Dark red urine the day of the visit along with ankle edema Dipstick of Urine: Protein 3+, Large Blood No Hypertension, Creatinine at 0.7 mg/dl Sent to Peds Nephrology within 2 days

History No rashes, no joint pain, and no fevers No sore throat, no skin infections Strep. culture taken negative Fatigue and decreased appetite URI symptoms prior to trip to Netherlands Runny nose, cough and congestion Zithromax given for sinusitis

Past History PMHx: PSHx: FHx: Imm: UTD All: NKDA Few otitis media No UTI No renal stones No admission to hospital PSHx: None FHx: No ESRD No Renal Stones No Hematuria No Autoimmune Disease Imm: UTD All: NKDA

Physical Examination T: 37.1 C P:81 BP: 132/82 R:18 PO2:100% Head: NC, 0 lesions Eyes: PERRLA, EOMI, B Fundo:Nml B eyelid Edema 2+/4 Ears: B Clear TM Nose: Non-swollen turbinates Throat: Non-red, clear, 0 exudate Neck: Supple, 0 mass, 0 JVD 0 Stiffness Chest: RRR, S1, S2, 0 murmur Lungs: B CTA good air movement 0 Wheezing 0 lymph nodes Abdomen: ND/NT, Soft, BS(+) 0 HSM Ext: 0 c/c; From; Cap Refill<2s Edema Ankles: 2+ pitting edema B No edema in UE Skin: 0 Rashes; 0 Impetigo

LABS CBC CHEM 10 11.9 6.7 339 133 108 15 36.9 3.7 21.0 0.8

LABS Urinanalysis Urine Micro pH 6.0 SG 1.030 Ketones (-) Glucose (-) Nitrates (-) LE (-) Protein 3+ Blood Large Urine Micro TNTC RBCs 10-20 RBC cast/ hpf 50+ Dysmorphic RBCs/hpf 5-7 Granular Casts/hpf

Labs C3: 16 C4: 29 ANA < 1:40 Strep Serologies: Negative Albumin 1.9 mg/dl Hepatitis Screen (-) HIV screen (-) Urine Protein to Creatinine ratio: 8.8

Army Air Ambulance

History One Month: Daily journal and school nurse recording BP increasing BP, more edema, weight gain and some ascites Slightly elevated creatinine to 1.4 mg/dl Repeat C3 showed low C3 at 20 Anemia worsening at Hct: 24.4% Admitted to the Hospital BP control with medication Renal Biopsy

Army CSH

Follow Up History Started on Prednisone 60 mg qd after IV Solumedrol pulse in the hospital Developed Shingles Treated with Valcyclovir Attempt to reduce Prednisone to qod Unsuccessful Creatinine increased to 2.2 mg/dl

Follow Up History Two weeks after steriod reduction: Creatinine rose to 2.8mg/dl and 3.1 mg/dl Admission to hospital Massive Ascites developed Creatinine rose to 4.7 mg/dl, despite pulse steriods Patient started on Peritoneal Dialysis Prednisone 60 mg qd

Continued History One week of peritoneal dialysis Patient responded to Steroids and creatinine reduced to 1.7 mg/dl Several more attempts to reduce to Prednisone qod: unsuccessful Bump in her creatinine with return to baseline after Prednisone dose resumed Occasional readmission to hospital due to massive ascites and uncontrolled HTN

Humanitarian Relief

MPGN Type II Children > 5 years of age, Male=Female Caucasians and reports of familial clusters 95% low plasma C3 50% Anemic 50 % 10-year renal survival (Cameron et al) 1983 West CD 1980 Alternate day 60 mg/m2 Prednisone Retrospective study 71 patients: Renal Survival at 10 year mark 82, 56% after 20 years

Do steroids aid in the regression of MPGN ? “Regression of Membranoproliferative GN Type II (DDD): Observations in Six Children” McEnery and Adams AJKD 1988

Do steroids aid in the regression of MPGN ? “Regression of Membranoproliferative GN Type II (DDD): Observations in Six Children” McEnery and Adams AJKD 1988

More Humanitarian Relief

Do steroids aid in the regression of MPGN ? “Treatment of MPGN with qod prednisone – a report of The International Study of Kidney Disease in Children” Tarish (1992) Ped Nephrology Controlled, double-blinded Study Years: 1970-1980 Ages: 5-17 80 Children End Point: Increase in creatinine 0.4 mg.dl

Do steroids aid in the regression of MPGN ? “Treatment of MPGN with qod prednisone – a report of The International Study of Kidney Disease in Children” Tarish (1992) Ped Nephrology

Do steroids aid in the regression of MPGN ? “Treatment of MPGN with qod prednisone – a report of The International Study of Kidney Disease in Children” Tarish (1992) Ped Nephrology

Do steroids aid in the regression of MPGN ? “Treatment of MPGN with qod prednisone – a report of The International Study of Kidney Disease in Children” Tarish (1992) Ped Nephrology

Cadets At Yankee Stadium