A rare case of tubulointerstitial nephritis and uveitis (TINU) syndrome: Questions
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CLINICAL QUIZ
A rare case of tubulointerstitial nephritis and uveitis (TINU) syndrome: Questions Douglas J. Stewart 1
&
Thivya Sekar 2 & Kjell Tullus 1
Received: 11 April 2020 / Accepted: 22 April 2020 # IPNA 2020
Case summary A 12-year-old male presented with anorexia and 8 kg of weight loss over a 4-week period, during which time he experienced recurrent generalised headaches. He subsequently developed symptoms of a sore throat, non-productive cough, lethargy, myalgia, drenching night sweats, and low-grade fevers that were non-periodic in nature. He reported some dryness of his eyes. He was initially managed with paracetamol at home but was then admitted to hospital. He had normal urine output and blood pressure was less than the 90th percentile for age and height. There was no recent travel history and family contacts had been well. His father was diagnosed with Sjögren’s syndrome in early adulthood, managed with oral steroids. A paternal uncle had a diagnosis of focal and segmental glomerulosclerosis (FSGS), managed with perindopril and atorvastatin. The young person had a background of mild asthma and recurrent croup as an infant but was otherwise well. He had not taken any ibuprofen or herbal remedies in the lead up to, or during, his current illness. On admission to hospital, he was found to have a creatinine of 146 μmol/L which rose to a peak of 211 μmol/L (estimated GFR 28 mL/min/1.73 m2 [modified Schwartz, k = 36.5]) over the course of 9 days. His CRP was elevated at 94 mg/L, as was The answers to these questions can be found at https://doi.org/10.1007/ s00467-020-04590-8. * Douglas J. Stewart [email protected] 1
Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK
2
Department of Histopathology, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK
his ESR at 126 mm/h. Lactate dehydrogenase (LDH) rose from 211 to 478 U/L during admission. Haemoglobin and platelet count remained normal throughout. White cell count was normal and serial blood films showed a slight left shift of neutrophils and mild reactive monocytosis. Eosinophil count and coagulation screen were normal. Liver function tests and serum albumin were normal. Urinalysis was positive for low levels (1+) of glucose, blood, and protein. Urine microscopy showed white cells < 10 × 106/L and red cells < 1 × 106/L. There was significant tubular proteinuria with a urine retinol-binding protein (RBP) to creatinine ratio of 5550 μg/ mmol (reference range 3.9–32.0 μg/mmol). Urine albumin to creatinine ratio was 34.4 mg/mmol. Immunological testing showed normal C3 and C4, and the young person was ANA and ANCA negative, with rheumatoid factor (RF) < 20 U/mL. Immunoglobulin A, G, and M levels were all within normal limits. Serum angiotensin-converting enzyme (ACE) was 28 U/L (reference range 29–112 U/L). A urinary tract ultrasound was performed, demonstrating that both kidneys were above the 95th percentile for age (right 11.2 cm and left 11.6 cm) wi
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