Unusual cause of recurrent macroscopic hematuria in an adolescent girl: Questions
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CLINICAL QUIZ
Unusual cause of recurrent macroscopic hematuria in an adolescent girl: Questions Abir Boussetta 1,2 & Manel Jellouli 1,2 & Raja Aouaidia 2,3 & Rim Goucha 2,4 & Taher Gargah 1,2 Received: 1 October 2020 / Accepted: 15 October 2020 # IPNA 2020
Keywords Child . Gross hematuria . Fever . Hypertension . Granulomatous interstitial nephritis . IgA deposits
Case summary A 14-year-old female was hospitalized for exploration of recurrent gross hematuria and hypertension. She was born of a non-consanguineous marriage and had no notable personal or family history of kidney or inflammatory disease. Since last year, the patient had presented 3 episodes of gross hematuria managed as cystitis. She was admitted to our pediatric nephrology department with a complaint of gross hematuria, fever, and heart palpitations. She denied any vomiting, cough, and abdominal or joint pain. Physical examination of the patient revealed the following: height, 168 cm (75th–90th percentile); weight, 101 kg (> 97th percentile) with gross hematuria and confirmed hypertension at 150/73 mmHg (95th percentile 125/82 mmHg), heart rate was 80/min with a regular rhythm and normal heart auscultation. Examination of her respiratory system revealed no abnormality. She had no edema and normal diuresis. Skin examination showed itchy papules on the legs. The remainder of the examination was normal. Her blood and urine reports at the time of admission are shown in Table 1.
The answers to these questions can be found at https://doi.org/10.1007/ s00467-020-04845-4. * Abir Boussetta [email protected] 1
Pediatric Nephrology Department, Charles Nicolle Hospital, Tunis, Tunisia
2
Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia
3
Department of Internal Medicine A, Charles Nicolle Hospital, Tunis, Tunisia
4
Nephrology Department, Mongi Slim Hospital, Tunis, Tunisia
Renal ultrasonographic examination revealed that both kidneys were large (123 mm) (mean normal kidney size for age and height: right 80–107 mm, left 87–116 mm) without signs of obstruction. In view of suspected lupus nephritis, the patient was planned for a kidney biopsy which showed non-caseating granulomatous interstitial nephritis with interstitial inflammatory cell infiltrate, and severe tubular inflammation. Glomeruli lesions were rare. Immunofluorescence revealed that the patient had mesangial IgA deposits, with rare interstitial deposits of C3. Images of the kidney biopsy are shown in Fig. 1. Questions 1. What are the likely possible diagnoses in this patient in view of the histopathological findings? 2. How would you investigate and manage this patient further? 3. What is the mechanism of recurrent hematuria in this condition?
Pediatr Nephrol Table 1 Laboratory investigations on admission
Hemogram
Hemoglobin 10.4 g% Leucocyte count 6200/mm3 Polymorphs 3310, lymphocytes 2120 Platelet count 317000/ mm3 Positive, IgG (++) Albumin 1+, red cells: 28 * 106/ml
Direct Coombs test Urine routine and microscopy
Leukocytes: 320,000/ml Urine protein Serum elect
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