A rare complication of pauci-immune crescentic glomerulonephritis in a child: Questions
- PDF / 535,769 Bytes
- 2 Pages / 595.276 x 790.866 pts Page_size
- 19 Downloads / 192 Views
CLINICAL QUIZ
A rare complication of pauci-immune crescentic glomerulonephritis in a child: Questions Sidharth Kumar Sethi 1 & Abhyuday Rana 2 & Shyam Bihari Bansal 2 & Alka Rana 3 & Dinesh Kumar Yadav 2 & Kritika Soni 2 & Marie-Agnès Dragon-Durey 4 & Rupesh Raina 5 & Vijay Kher 2 Received: 22 August 2020 / Accepted: 15 September 2020 # IPNA 2020
Keywords Child . Pauci-immune crescentic glomerulonephritis . Anemia . Hypertension . Thrombocytopenia . Thrombotic microangiopathy . aHUS
Case summary A 6-year-old female born of non-consanguineous marriage with no previous comorbidities was referred to our center with rapidly progressive kidney failure. She reported having a minor upper respiratory tract infection 1 month ago followed by progressive anasarca. There was no history of rash, abdominal pain, overt hematuria, dyspnea, cough, palpitations, joint swelling/pain, or neurological deficits. Prior to the referral, her evaluation done externally showed serum creatinine (sCr) of 3 mg/dL with a urine protein to creatinine ratio of 1.2 g/g. Urinalysis showed microscopic hematuria. Serological evaluation was unremarkable with normal C3 and C4 levels (130 mg/dL and 30 mg/dL) and negative ASO, anti-DNase, ANA, anti-GBM, anti-dsDNA, antiSmith, anti-RNP, anti-Ro, anti-La, anti-MPO, and anti-PR3 antibodies. Serological evaluation for HIV, hepatitis B, and C was The answers to these questions can be found at https://doi.org/10.1007/ s00467-020-04786-y. Both Sidharth Sethi and Abhyuday Rana shall be first authors. * Sidharth Kumar Sethi [email protected] 1
Pediatric Nephrology, Kidney Institute, Medanta the Medicity, Haryana 122001 Gurgaon, India
2
Kidney Institute, Medanta the Medicity, Haryana 122001 Gurgaon, India
3
Department of Pathology , Medanta the Medicity, Haryana 122001 Gurgaon, India
4
Université de Paris, Hôpital Européen Georges Pompidou, APHP, INSERM UMRS1138, Paris, France
5
Pediatric Nephrology, Akron Children’s Hospital, Cleveland, OH, USA
negative. ANCA by immunofluorescence and anti-GBM antibodies were negative. She was transfused with a unit of packed RBCs at the local center due to severe anemia (Hb-5.1 g/dL). On admission, her physical examination was unremarkable except for hypertension (BP 144/86 mmHg), anasarca, and pallor. Laboratory evaluation at our center showed anemia (Hb 6.2 mg/dL) and elevated levels of sCr (5.2 mg/dL), urea (372 mg/dL), and LDH (854 U/L), and normal complement levels were noted (C3 89 mg/dL, C4 26 mg/dL). Kidney ultrasound revealed normal-sized kidneys with enhanced echogenicity, consistent with parenchymal pathology. Chest X-ray showed no abnormalities. Considering rapidly progressive glomerulonephritis, she was administered a dose of intravenous cyclophosphamide (500 mg/m2), and pulse methylprednisolone therapy (30 mg/kg intravenous daily for 3 days) was started in addition to diuretic and antihypertensive medications. A kidney biopsy was then obtained, and fifteen glomeruli were sampled for light microscopy. Cellular and fibrocellular crescents were seen in 8 an
Data Loading...
