HPI 14yo previously healthy male who was diagnosed with otitis media 3 weeks prior to admission, treated with azithromycin. He seemed to recover after the treatment until 1 week prior to admission when he woke up with a swollen face. His mom thought this was allergies, so she gave him Benadryl along with his once daily Claritin. His swelling decreased throughout the day but was back the next morning. This continued for the next few days. Two days prior to admission, he did a lot of walking at the zoo and developed swelling in his legs afterward. This worsened over a couple days. His mother took him to the family physician where he was found to have gained 19 lbs and had systolic BP in 150s. He was also short of breath. He was referred to the Emergency Department at an outside hospital where he was requiring oxygen and was admitted. On the third day of hospitalization, he started to complain of peripheral vision changes. Therefore, he was transferred to PCH PICU. PMH History of multiple ear infections
No meds
Allergic to Augmentin FH None significant SH Lives with parents and two siblings Physical Exam VS: T: 37.1. HR: 108. RR: 20. BP: 132 /80 . SaO2 96 % on Nasal Cannula at 0.5 LPM. Weight: 58.00kg (41%ile), Height 169 cm (59%ile)
GENERAL: laying in bed, mildly irritated with providers but no distress, interacting normally for age and situation HEAD: normocephalic, atraumatic. EYES: both pupils briskly reactive to light, anisicoria L pupil about 1.5mm > R pupil NOSE: no discharge or obstruction. OROPHARYNX: moist mucus membranes, no mucosal lesions NECK: supple without lymphadenopathy or tenderness to palpation. CARDIOVASCULAR: normal rate, rhythm, and S1/S2, without murmur or gallop. Pulses appropriate. Capillary refill time <2 seconds. LUNGS: clear to auscultation bilaterally, diminished air flow in the bases with some scattered crackles, no retractions. ABDOMEN: soft, non-tender, non-distended with active bowel sounds and no masses or hepatosplenomegaly. No fluid wave or ascites appreciated EXTREMITIES: all extremities warm and well perfused. Edema in lower extremities bilaterally, non pitting, no nail pits or nail bed abnormalities, no swelling in hands BACK: small scar on left flank GENITOURINARY: (Attending exam with charge nurse Brian Vincent) normal uncircumcised TIV male genitalia, testes descended bilaterally with normal cremasteric reflex, no scrotal swelling or rash, normal sphincter tone NEUROLOGIC: awake and alert, GCS 15, grossly normal strength and tone, patellar tendon reflexes normal. SKIN: no rashes or petechiae on skin
Patient summary: 14yo previously healthy male with facial and extremity edema, hypertension, shortness of breath, and vision changes. Differential diagnosis Renal Poststreptococcal GN MPGN IgA nephropathy SLE HSP Alport syndrome Hepatitis B associated GN Endocarditis associated GN Post-infectious GN HUS Renovascular disease Nephrotic syndrome Neuro Intracranial hemorrhage Intracranial mass PRES HACE Respiratory Pneumonia Pulmonary edema HAPE Cardiac Heart failure Primary hypertension Coarctation of the aorta ID Pneumonia Meningitis Other Pheochromocytoma Ingestion Conversion disorder
Imaging CXR: Bilateral pleural effusions and pulmonary edema
Head CT: Multiple intracranial hemorrhages
Brain MRI: 3 cm area of abnormal signal and hemorrhage involving the occipital lobe and a 2.2 cm area of abnormal signal with punctate hemorrhage involving the right parietal lobe.
Labs Prot/Cr 0.4 Lactate 1.8 CBC: WBC 5.7, Hgb 12.8, Hct 38.8, Plt 321 (N 55% L 31.3% M 9.1% E 3.8%) CRP 0.1 CMP: Na 141, K 5.1, Cl 112, C02 24, Gluc 69, BUN 17, Cr 0.87 Ca 8.8, Prot 6.6, Alb 3.3, Total Bili 0.5, ALP 166, ALT 47, AST 29 TSH 3.14 Urine Macro: Hgb large, Prot 2+ ESR 22 PT 13.9/INR 1.1 C3 25L, C4 18
RFP: Na 140, K 4.7, Cl 111, C02 21, Gluc 137, BUN 17, Cr 1.02, Alb 2.8, Ca 8.2, Phos 5.2
Further labs ASO 540 Anti-DNAse B 2120 ANCA negative
4 days after admission: Cr 0.88
Poststreptococcal Glomerulonephritis Most common cause of acute nephritis in children Most commonly in ages 5-12 Specific nephritogenic strains of group A beta-hemolytic streptococcus Spectrum of asymptomatic, microscopic hematuria to full- blown acute nephritic syndrome (red to brown urine, proteinuria, edema, hypertension, and acute kidney injury) Poststreptococcal GN Pathophysiology: Glomerular immune complex disease triggers complement activation and inflammation Deposition of streptococcal antigens in the glomerulus and subsequent immune complex formation
Usually developed Between 1-3 weeks following GAS pharyngitis Between 3-6 weeks following GAS skin infection
Presenting symptoms:
Edema (67%) Gross hematuria (30-50%) Hypertension (50-90%) caused be salt and fluid retention
Other causes of GN Membranoproliferative GN May be indistinguishable from post-infectious GN Hematuria, hypertension, proteinuria, and hypocomplementemia Persistent urinary abnormalities and hypocomplementemia beyond 4-6 weeks IgA nephropathy Shorter time period between antecedent illness and hematuria (less than 5 days) HSP Other clinical findings (purpura, abdominal pain) No decrease in complement SLE Both C3 and C4 are low (only C3 is low in PSGN) Work-up Urinalysis Hematuria, with or without red cell casts Varying degrees of proteinuria Pyuria Blood C3 is significant depressed in first 2 weeks of disease (in contrast to lupus nephritis) Anti-DNAse B and ASO elevation Culture Recent positive throat or skin culture Renal biopsy If C3 remains low after 6 weeks, suggestive of MPGN Recurrent episodes of hematuria suggesting IgA nephropathy Progressive increase in creatinine Treatment Supportive therapy Treatment of volume overload with anti-hypertensives and loop diuretics Sodium and water restriction Dialysis (rare) Antibiotics if persistent streptococcal infection Time course Diuresis usually begins within 1 week Serum creatinine returns to baseline within 3-4 weeks Hematuria resolves in 3-6 months Proteinuria lingers (15% at 3 years, 2% at 7-10 years) Prognosis >90% have normal or only mildly decreased kidney function 5-18 years after presentation Can go on to develop hypertension or renal insufficiency in adulthood Complications Encephalopathy (PRES) Pulmonary edema Intracranial hemorrhage (very few reports in the literature)
References Up To Date Chiu CY et al. Poststreptococcal glomerulonephritis with pulmonary edema presenting as respiratory distress. Pediatr Nephrol. 2004 Nov;19(11):1237-40. Zaki SA and Shanbag P. Unusual presentation of poststreptococcal glomerulonephritis as posterior reversible encephalopathy syndrome. J Pediatr Neurosci. 2014 Jan;9(1):42-4.