A very rare cause of acute kidney injury: Questions
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CLINICAL QUIZ Avery rare cause of acute kidney injury: Questions Malgorzata Mizerska-Wasiak & Katarzyna Jankowska & Anna Adamowicz-Salach & Maria Roszkowska-Blaim Received: 25 September 2013 /Revised: 21 October 2013 /Accepted: 24 October 2013 # IPNA 2013 Keywords Anaemia . Acute kidney injury Case report A boy aged 1 year and 11 months was admitted to a paediatric haematology department due to a 2-day his- tory of fever of 39 °C, vomiting, diarrhoea, jaundice and general malaise. On admission, the patient was in overall good clinical condition, without fever or symptoms of a respiratory tract infection. Physical examination revealed slight yellowing of the skin and sclerae, and mild hepatomegaly (1 cm below the costal arch). Laboratory testing showed anaemia with a haemoglobin level of 6.1 g/dL, platelet count of 218,000/ mm 3 (normal reference range 250,000-400,000/mm 3 ), bio- chemical features of haemolysis, including relative reticulo- cyte count of 32.5 %, increased levels of lactate dehydroge- nase at 5,926 U/L (normal reference range up to 920 U/L), iron at 234 ug/dL (normal reference range up to 145 ug/dL), ferritin at 1,309.98 ng/mL (normal reference range up to 140 ng/mL) and total bilirubin at 4.4 mg/dL, as well as elevated inflammation markers, including a C- reactive protein level of 5.4 mg/dL (normal reference range up to 1 mg/dL) and leukocyte count of 15,000/ mm 3 . The blood film showed anizocytosis, myelocytes and spherocytes but no schistocytes. The direct anti- globulin test, routinely performed with immunoglobulin (Ig) G antibodies, was negative. Additional testing showed acute kidney injury, with a creatinine level of 1.3 mg/dL, a glomerular filtration rate estimated using the Schwartz formula of 64 mL/min/1.73 m 2 and a urea level of 191 mg/dL. Serum sodium and potassium levels, as well as arterial blood gases were normal. Urinalysis showed proteinuria of 135 mg/dL, with three to four erythro- cytes per field of view, and numerous haemoglobin deposits. Abdominal ultrasonography revealed enlarged kidneys (length 70–77 mm) with increased echogenicity. Based on the overall clinical picture and laboratory findings, autoimmune haemolytic anaemia was suspected. Paroxysmal nocturnal haemoglobinuria was excluded based on normal CD59 and CD66b expression on granulocytes and CD55 and CD59 expression on erythrocytes. The repeated antiglobulin test was positive and showed the presence of complement C3d fragments on red blood cells (RBCs), and biphasic haemolysins were detected in the serum. Parvovirus B19 infection was excluded (based on negative results of anti-PV B19 IgG and anti-PV B19 IgM testing), as were Epstein– Barr virus and cytomegalovirus infections. The management included transfusion of filtered and irra- diated packed RBCs (on three occasions) and protecting the patient from cold. Further laboratory testing performed during the hospitalisation period showed increases in creatinine and urea levels to 1.7 and 199 mg/dL, respectively, on the second day The answers to these questions can be found at http://dx.doi.org/10.1007/ s00467-013-2680-8 M. Mizerska-Wasiak : K. Jankowska : M. Roszkowska-Blaim Department of Paediatric Nephrology, Medical University of Warsaw, Warsaw, Poland A. Adamowicz-Salach Department of Paediatric Haematology and Oncology, Medical University of Warsaw, Warsaw, Poland M. Mizerska-Wasiak (*) Department of Pediatrics and Nephrology, Medical University of Warsaw, Marszalkowska 24, 00-576 Warsaw, Poland e-mail: [email protected] Pediatr Nephrol DOI 10.1007/s00467-013-2678-2
Transcript of A very rare cause of acute kidney injury: Questions
CLINICAL QUIZ
Avery rare cause of acute kidney injury: Questions
Malgorzata Mizerska-Wasiak & Katarzyna Jankowska &
Anna Adamowicz-Salach & Maria Roszkowska-Blaim
Received: 25 September 2013 /Revised: 21 October 2013 /Accepted: 24 October 2013# IPNA 2013
Keywords Anaemia . Acute kidney injury
Case report
A boy aged 1 year and 11 months was admitted to apaediatric haematology department due to a 2-day his-tory of fever of 39 °C, vomiting, diarrhoea, jaundiceand general malaise.
On admission, the patient was in overall good clinicalcondition, without fever or symptoms of a respiratory tractinfection. Physical examination revealed slight yellowing ofthe skin and sclerae, and mild hepatomegaly (1 cm below thecostal arch). Laboratory testing showed anaemia with ahaemoglobin level of 6.1 g/dL, platelet count of 218,000/mm3 (normal reference range 250,000-400,000/mm3), bio-chemical features of haemolysis, including relative reticulo-cyte count of 32.5 %, increased levels of lactate dehydroge-nase at 5,926 U/L (normal reference range up to 920 U/L),iron at 234 ug/dL (normal reference range up to 145 ug/dL),ferritin at 1,309.98 ng/mL (normal reference range up to
140 ng/mL) and total bilirubin at 4.4 mg/dL, as wellas elevated inflammation markers, including a C-reactive protein level of 5.4 mg/dL (normal referencerange up to 1 mg/dL) and leukocyte count of 15,000/mm3. The blood film showed anizocytosis, myelocytesand spherocytes but no schistocytes. The direct anti-globulin test, routinely performed with immunoglobulin(Ig) G antibodies, was negative.
Additional testing showed acute kidney injury, with acreatinine level of 1.3 mg/dL, a glomerular filtration rateestimated using the Schwartz formula of 64 mL/min/1.73 m2
and a urea level of 191 mg/dL. Serum sodium and potassiumlevels, as well as arterial blood gases were normal. Urinalysisshowed proteinuria of 135 mg/dL, with three to four erythro-cytes per field of view, and numerous haemoglobin deposits.Abdominal ultrasonography revealed enlarged kidneys(length 70–77 mm) with increased echogenicity.
Based on the overall clinical picture and laboratoryfindings, autoimmune haemolytic anaemia wassuspected. Paroxysmal nocturnal haemoglobinuria wasexcluded based on normal CD59 and CD66b expressionon granulocytes and CD55 and CD59 expression onerythrocytes.
The repeated antiglobulin test was positive andshowed the presence of complement C3d fragments onred blood cells (RBCs), and biphasic haemolysins weredetected in the serum. Parvovirus B19 infection wasexcluded (based on negative results of anti-PV B19IgG and anti-PV B19 IgM testing), as were Epstein–Barr virus and cytomegalovirus infections.
The management included transfusion of filtered and irra-diated packed RBCs (on three occasions) and protecting thepatient from cold.
Further laboratory testing performed during thehospitalisation period showed increases in creatinine and urealevels to 1.7 and 199 mg/dL, respectively, on the second day
The answers to these questions can be found at http://dx.doi.org/10.1007/s00467-013-2680-8
M. Mizerska-Wasiak :K. Jankowska :M. Roszkowska-BlaimDepartment of Paediatric Nephrology, Medical University ofWarsaw, Warsaw, Poland
A. Adamowicz-SalachDepartment of Paediatric Haematology and Oncology,Medical University of Warsaw, Warsaw, Poland
M. Mizerska-Wasiak (*)Department of Pediatrics and Nephrology, Medical University ofWarsaw, Marszalkowska 24, 00-576 Warsaw, Polande-mail: [email protected]
Pediatr NephrolDOI 10.1007/s00467-013-2678-2
of hospital stay, along with platelet count reduction to 139,000/mm3. Parameters of renal function normalised on thetenth day of treatment. Two weeks after the discharge, all ofthe blood count and renal function parameters were normal,with no anaemia noted. At that time, the haemoglobin levelwas 12.7 g/dL, and direct antiglobulin test results continued tobe positive.
Questions
1. What is the diagnosis?2. What is the pathogenesis of acute kidney injury in this
disease?3. What are the diagnostic and therapeutic aspects that need
to be considered?
Pediatr Nephrol
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