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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2012  |  Volume : 23  |  Issue : 5  |  Page : 1024-1027
Severe pneumococcal hemolytic uremic syndrome in an 8-month-old girl

1 Department of Pediatric Nephrology, Charles Nicolle Hospital, Tunis, Tunisia
2 Department of Nephrology, Charles Nicolle Hospital, Tunis, Tunisia

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Date of Web Publication13-Sep-2012


The hemolytic uremic syndrome (HUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure, represents one of the major causes of acute renal failure in infancy and childhood. The typical form occurring after an episode of diarrhea caused by Escherichia coli is the most frequent in children. Other microorganisms also may be responsible for HUS, such as Streptococcus pneumoniae, which causes more severe forms of the disease. We report an 8-month-old girl who presented with pneumonia and subsequently developed HUS. Renal biopsy showed characteristic lesion of thrombotic microangiopathy and extensive cortical necrosis. She was managed with peritoneal dialysis but did not improve and developed severe sepsis due to staphylococcal peritonitis, resulting in the death of the patient. Streptococcus pneumoniae-induced HUS is uncommon, but results in severe disease in the young. There is a high risk of these patients developing end-stage kidney disease in the long term.

How to cite this article:
Gargah T, Cherif M, Goucha-Louzir R, Lakhoua MR. Severe pneumococcal hemolytic uremic syndrome in an 8-month-old girl. Saudi J Kidney Dis Transpl 2012;23:1024-7

How to cite this URL:
Gargah T, Cherif M, Goucha-Louzir R, Lakhoua MR. Severe pneumococcal hemolytic uremic syndrome in an 8-month-old girl. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2022 Oct 2];23:1024-7. Available from: https://www.sjkdt.org/text.asp?2012/23/5/1024/100885

   Introduction Top

Hemolytic and uremic syndrome (HUS), one of the common causes of acute renal failure in pediatric practice, is characterized by a triad of microangiopathie hemolytic anemia, thrombocytopenia and acute renal failure. [1] It occurs classically after a diarrhea due to  Escherichia More Details coli and Shigella dysenteriae, rarely in the con­text of Streptococcus pneumoniae infection. The diarrhea-associated HUS is the one usually considered as a typical HUS. [2] HUS due to pneumococcus (pHUS), termed as atypical HUS, is a more severe disorder and is characterized by high rates of morbidity and mortality. It has dif­ferent epidemiologic, therapeutic and prognos­tic characteristics. More than 80 cases of pHUS have been previously reported in the literature who presented with pneumococcal pneumonia or meningitis.

We report here a case of a child who had pneumococcal pneumonia and developed severe HUS, which was further complicated by septicemia.

   Case Report Top

An 8-month-old female patient was presented with a seven-day history of fever and produc­tive cough related to pneumopathy treated with cefotaxime and vancomycin. She was referred in our department because she developed respiratory distress and acute renal failure with anuria. There was no history of diarrhea or urinary infections. Family history was non-contributory. On physical examination, we noted an altered general state, obnubilation, mottling of skin, generalized edema and anuria. Body tempe­rature was 38.6°C, pulse rate 120/min, respi­ratory rate 66/min and blood pressure 110/60 mmHg. Chest examination revealed diffuse crackling rales. Her oxygen saturation breathing room air was 93% and capillary refill time was less than 3 s. Chest radiography showed lower lobe pneumonia with minimal pleural effusion.

Blood investigations showed a hemoglobin le­vel of 7.2 g/dL, white cell count 27,000/mm 3 , platelet count 90,000/mm 3 and C-reactive pro­tein of 206 mg/L. The plasma concentrations of urea and creatinine, which were normal before the onset of HUS, increased to 26 mmol/L and 280 μmol/L. The peripheral smear showed schistocytes (fragmented red cells) and thrombocytopenia.

Blood culture grew colonies of Streptococcus pneumoniae sensitive to both cefotaxime and vancomycin. Direct Coomb's test and peanut agglutinin tests were negative. Serum low-density lipoprotein level was normal at 120 U/L. the serum complement level revealed C3 fraction lowered to 0.73 g/L, C4 fraction and CH50 were normal, 0.39 g/L and 103%, respectively. Ultrasound showed both kidneys to be slightly hyperechoic.

Peritoneal dialysis was started, whereby the edema and hypertension improved, but diuresis did not occur and serum creatinine level re­mained high. Renal biopsy was performed du­ring the second week after the normalization of blood pressure and thrombocytopenia. The histological findings observed were those of a thrombotic microangiopathy [Figure 1] associa­ted with extensive cortical necrosis [Figure 2] and [Figure 3]. Subsequently, the patient developed staphylococcal peritonitis and severe septicemia from which the patient did not recover and expired.
Figure 1: Arteriolar and glomerular thombosis (Masson's trichromic stain).

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Figure 2: The coexistence of thrombotic microangiopathy and cortical necrosis (Masson's trichrome stain).

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Figure 3: Cortical necrosis (Masson's trichrome stain × 80).

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   Discussion Top

HUS in children occurs mostly due to infe­ction from enterohemorrhagic Escherichia coli and Shigella dysenteriae type 1. Rarely, it occurs due to invasive Streptococcus pneumoniae, which was first described by Fischer and colleagues in 1971. [3] It is estimated that pHUS accounts for 3-5% of all HUS cases. [4],[5]

All Streptococcus pneumoniae can produce neuraminidase, and are able to stimulate T­antigen. Patients with Streptococcus pneumoniae-associated disease have detectable serum neuraminidase activity, while patients with pneumococcal disease without HUS do not have detectable activity. [6],[7] This diagnostic test was not available in our hospital; however, the patient had Streptococcus pneumoniae isolated from blood cultures and had associated micro-angiopathic hemolytic anemia, thrombocytopenia, acute renal failure and specific histological lesions, which confirmed the diagnosis.

Pneumococcal HUS usually affects healthy children under 24 months. [4],[5] Streptococcus pneumoniae is commonly present with either menin­gitis or pneumonia before the development of pHUS. Intervals between onset of pneumococcus-related symptoms and the development of HUS have been reported to range from one day to two weeks, but mostly occur within one week. [5],[6] Empyema was the most common extra-renal complication of Streptococcus pneumoniae-associated HUS, and was reported to be around 50-70%. [4],[5],[6] Our child was eight months old; she developed HUS one week after pneumonia and she did not have empyema.

The majority (75-100%) of Streptococcus pneumoniae-associated HUS patients required dialysis therapy for the management of acute renal failure, and the duration was around ten to 25 days. About 8-30% of the patients went on to develop end-stage renal disease. [4],[5],[6] The 8-month-old girl was dialysed during 30 days. Control of the Streptococcus pneumoniae infec­tion must be achieved early and adequately in order to improve renal prognosis. For our pa­tient, in spite of an adopted antibiotic treatment, renal biopsy showed extensive cortical necrosis.

Disorders of complement regulation are, nu­merically, the most important. Some cases of complement dysfunction appear to respond to plasma therapy. The guideline proposes urgent and empirical plasmapheresis and replacement with whole plasma fraction for the first month after diagnosis. [8] This should be undertaken only in specialized pediatric nephrology centers where appropriate medical and nursing skills are avai­lable. Our patient had low serum C3 level; plasmapheresis was planned but was not done due to lack of resources.

Neuraminidase released by the pneumococci may cleave N-acetylneuraminic acid residues on red blood cells (RBCs), leading to exposure of the T cryptantigen and polyagglutinability of RBCs, a process known as T activation. Data suggest a pathogenic role of exposed T antigens on glomeruli interacting with naturally occur­ring anti-T in the development of renal dys­function in Phus. By reducing the levels of anti-T and neuraminidase, plasma exchange may have a role in the treatment of severe cases of pHUS. [9]

The use of washed blood products is prefe­rable in case of blood transfusion, as the pre­sence of plasma may prolong hemolysis through the action of a neuraminidase and can worsen the manifestations of HUS or exhibit life-threatening complications. [3],[4],[6]

The prognosis for patients with pHUS is poorer than for typical HUS; the mortality rate is reported to be 19-50% and approximately 50% of the surviving patients have long-term renal dysfunction. Early recognition of HUS, transfusion with washed blood products, plasma exchange, dialysis and adequate treatment of in­fection could save lives and decrease sequelae. [3],[8]

pHUS is a disease that should be better known because its incidence may be increasing, espe­cially in young infants. Anti-pneumococcal vac­cination may be reduce its risks. This case high­lights two important facts: the possibility of occurrence of pHUS in young infants on one hand and the fact that the histological study is a valuable pointer of prognosis on the other.

   References Top

1.Kaplan BS, Cleary TG, Obrig TG. Recent advances in understanding the pathogenesis of haemolytic uremic syndromes. Pediatr Nephrol 1990;4:276-83.  Back to cited text no. 1
2.Siegler RL. The haemolytic uremic syndrome. Pediatr Clin North Am 1995;42:1505-29.  Back to cited text no. 2
3.Fischer K, Poschmann A, Oster H. Molyse beischwere pneumonie infolge neuraminidase wirkung. Monatsschrift fur kinderheilkunde 1971;119:2-8.  Back to cited text no. 3
4.Lee CF, Liu SC, Chen JP, Sheu JN. Pneumococcal pneumonia with empyema and haemolytic uremic syndrome in children: Report of three cases. J Microbiol Immunol Infect 2006;39:348-52.  Back to cited text no. 4
5.Huang YH, Lin TY, Wong KS, et al. Haemolytic uremic syndrome associated with pneumococcal pneumonia in Taiwan. Eur J Pediatr 2006;165:332-5.  Back to cited text no. 5
6.Vaderkooi OG, Kellner JD, Wade AW, et al. Invasive streptococcus pneumoniae infection causing haemolytic uremic syndrome in chil­dren: Two recent cases. Can J Infect Dis 2003; 14:339-43.  Back to cited text no. 6
7.Chautemps N, Milési C, Cambonie G, et al. Pneumococcal haemolytic uremic syndrome: About 2 cases. Arch pediatr 2008;15:1206-10.  Back to cited text no. 7
8.Hopkins CK, Yuan S, Lu Q, Ziman A, Gold­finger D. A severe case of atypical haemolytic uremic syndrome associated with pneumococcal infection and t activation treated successfully with plasma exchange. Transfusion 2008;48: 2448-52.  Back to cited text no. 8
9.Ariceta G, Besbas N, Johnson S, et al. European paediatric study group for HUS guideline for the investigation and initial therapy for diarrhea-negative haemolytic uremic syndrome. Pediatr nephrol 2009;24:687-96.  Back to cited text no. 9

Correspondence Address:
Tahar Gargah
Department of Pediatric Nephrology, Boulevard 9 Avril, Charles Nicolle Hospital, Tunis, 1009
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.100885

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  [Figure 1], [Figure 2], [Figure 3]

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