Iranian Urology and Renal Transplantation Center

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Unusual presentation and progression of emphysematous pyelonephritis in a 9 year old boy.

Abbas Basiri, Hamid Reza Abdi, Nastaran Mahmoudnejad AND Mohammad Hossein soltani
Pediatric Urology
Urology Nephrology Research CenterShahid Labbafinejad HospitalShahid Beheshti Medical UniversityTehraniran .
child - emphysematous pyelonephritis - pleural effusion - silent behavior


In literature review, EPN in children has been reported only in a 12 year old boy renal transplant recipient who presented with septic appearance but we are going to report a 9 year old boy with EPN who had neither diabetes nor immunedeficiency in past history.

Main Description

Case presentation

The patient is a 9 year old boy admitted in another hospital because of left flank pain and low grade fever without urinary symptoms. Urine analysis revealed pyuria and urine culture was positive for E – coli, so after one week antibiotic therapy for treatment of urinary tract infection (UTI), his symptoms relieved and he discharged with good status. After three weeks, he came to our emergency unit with sever left flank pain whitout fever and urinary symptoms. He was hemodinamically stable: PR: 78 ,  RR: 18 , Blood Pressure : 95/60  , Axillary Temprature : 37.6. In physical examination, we found left CVA tenderness and slightly palpable mass in left flank. Urine analysis showed pyuria and pertinent laboratory values included serum ceratinin : 0.8 mg/dl ,  blood sugar : 97 and serum white blood cell count : 19200 . Ultrasonography revealed sever hydronephrosis  consist of concentrated and heterogenous fluid  probably due to pyonephrosis and 3 cm stone in renal pelvis and air traping space in upper calyx  probably due to emphysematous pyelonephritis. Computerized tomography (CT SCAN) confirmed the diagnosis of emphysematous pyelonephritis.






The patient underwent left nephrectomy and final pathology was xanthogranulomatous pyelonephritis with perinephric inflammation. Culture of fluid in left kidney after operation was positive for klebsiella pneumonia and blood culture was negative. Four days after operation, because of dry cough, chest x ray was obtained and it showed pleural effusion in lower part of left hemithorax. Chest CT scan confirmed massive pleural effusion  but the patient had good status and no fever and leukocytosis.




Exudative fluid was drainaged using chest tube and its culture was positive for streptococous viridians. Also evaluation for tuberculosis and fungal infection was negative. Using intravenous broad spectrum antibiotic and aggressive hydration, the patient discharged with good status after 15 days and in 2 month follow up, significant improvement in appetite, general condition and weight gaining was happened. Several serologic tests for detection of immunedeficiency problems were performed that all of them were normal.




EPN as a threatening and jeopardizing disease exclusively seen in adults with background of diabetes or obstruction or anatomical abnormality, so happening of this disease in children with normal immune system is not reported. Emphysematous pyelonephritis is almost confined to patients with DM who constitute more than 90% of all cases with it. While in non-diabetic patients EPN is invariably associated with ureteric obstruction. The most common clinical manifestations of EPN (fever, flank pain, and pyuria) were nonspecific and not different from the classic triad of upper UTI other than EPN. usually EPN is a sudden onset , progressive and life threatening process that associated with extensive changes in vital signs ,  level of concioussness and metabolic status but  happening of EPN in this child with slow progression and minimal changes in metabolic state  may be exposed the other faces of this infection. The diagnosis of this condition depends on the characteristic radiological findings of gas within the collecting system, renal parynchema and/or peri-nephric tissue. Although there are different modalities of treatment including medical, surgical or both, It is a life-threatening infection and mortality rates can be as high as 70% to 80%. it seems that long term period of EPN in this patient  may  be  led  to creation a systemic inflammatory response in surrounding organs such as producing the left parapneumonic effusion, this event shows that EPN can be a result of chronic infective process and comparing to the previous articles , it is not usual behaviour . Although regarding to the existence a stone in left Ureteropelvic junction , urinary obstruction is the main reason for occurance of this disease but  finally we can´t clarify what is the main origin of this disease in patient ; lung or kidney ? We think that this patient is an unusual case because of presentation in pediatric age, concomitant with exudative pleural effusion and uncommon clinical behaviour.