Results of serotyping for this case are still pending, but the possibility that this infection was caused by a vaccine-preventable serotype underscores the importance of ensuring vaccine understanding among both community and specialist pediatricians. Table?2. Pneumococcal immunization recommendations in nephrotic syndrome thead th align=”center” colspan=”4″ rowspan=”1″ ACIP recommendations for PCV13 and PPSV23 administration in children aged 6C18 years old with immunocompromising conditions (including nephrotic syndrome) hr / /th th rowspan=”1″ colspan=”1″ /th th align=”left” rowspan=”1″ colspan=”1″ PCV13 /th th align=”left” rowspan=”1″ colspan=”1″ PPSV23 /th th align=”left” rowspan=”1″ colspan=”1″ PPSV23 /th /thead Patients who have NOT previously received PPSV231 dose1st dose 8 weeks after PCV13 dose2nd dose 5 years after 1st PPSV23 dosePatients who have previously received PPSV231 dose 8 weeks after last PPSV23 doseN/Aalready received 1st dose5 years after 1st PPSV23 dose Open in a separate window ACIP, Advisory Committee on Immunization Practices; PCV13, 13-valent pneumococcal conjugate vaccine; PPSV23, 23-valent pneumococcal polysaccharide vaccine. This case highlights the diagnostic challenges that pHUS presents, and the importance of early recognition. cell glycoproteins [3]. This process, known as T-activation, then prospects to IgM binding from circulating IgM anti-T antibodies, and the clinical syndrome of HUS (Physique?1) [4]. Open in a separate windows Fig.?1. A mechanism of endothelial cell injury in Streptococcal Pneumoniae associated hemolytic uremic syndrome. Treatment of pHUS is with antibiotics with activity against and supportive care. If necessary, transfusion of washed blood products is preferable to avoid increasing the levels of preformed anti-T antibodies, which are high in unwashed products. Anecdotal evidence supports the use of plasma exchange with 5% albumin replacement, and avoiding new frozen plasma (FFP) due to preformed anti-T antibodies in the pooled product [5C7]. Case statement A 12-year-old female with steroid-resistant nephrotic syndrome presented to the emergency department with fever, shortness of breath and cough. On exam she was tachycardic and tachypneic, requiring 3 L of supplemental oxygen. She was given 1 L of normal saline bolus intravenously. A chest X-ray recognized bilateral pulmonary edema. She met criteria for sepsis [8]. She was started on vancomycin and cefotaxime, and admitted to our pediatric intensive care unit for further management. Past medical history was amazing for the diagnosis of nephrotic syndrome at the age of 5 years. Although in the beginning responsive to steroids she suffered several relapses when the steroid dose was tapered. At the age of 6 years a renal biopsy showed findings consistent with minimal switch disease. Genetic screening for inherited nephrotic syndromes recognized a heterozygous, non-coding mutation in the (at 8 h, and a nasopharyngeal swab PCR BRD7552 was positive for parainfluenza type 2. Overnight the patient developed oliguria, the creatinine increased from 1.4 to 2.4 mg/dL, and the hemoglobin decreased from 11.4 to 7.4 g/dL (Table?1). Acthar? and tacrolimus were discontinued. She was transfused Rabbit Polyclonal to FZD1 one unit of unwashed packed red blood cells (pRBCs) and started on continuous veno-venous hemodiafiltration due to worsening kidney function and pulmonary edema. Table?1. Pertinent laboratory data contamination [11]. Evidence of T-antigen exposure (direct Coombs test, polyagglutination test or peanut lectin agglutination test) can help support the diagnosis, but is not mandatory [12]. The patient met diagnostic criteria for pHUS, and additionally experienced a positive direct Coombs test (Table?1). While disseminated intravascular coagulation (DIC) with multiorgan failure was considered as an alternative diagnosis, BRD7552 it was eventually rejected due to evidence of autoimmune hemolysis (schistocytes, positive Coombs), and normal fibrinogen level. In the beginning the mildly prolonged prothrombin time/activated partial thromboplastin time (PT/aPTT) hinted toward the possibility of DIC, but coagulation abnormalities are common in pHUS, and DIC is typically not considered without BRD7552 significant PT/aPTT elongation along with decreased fibrinogen. One unique aspect of this case was the patient’s age and risk factors. The median age of pHUS reported in the literature ranges from 13 to 24 BRD7552 months [1, 13, 14]. Four cases of pHUS in adults have been reported [15C18]. BRD7552 Two of these patients experienced undergone splenectomy, and so underlying immunodeficiency is usually presumably a risk factor for development of pHUS at an older age. Nephrotic syndrome is usually a well-known cause of secondary immunodeficiency due to multiple factors including edema, urinary loss of immunoglobulins and match factors, and secondary effects of cytotoxic therapies [19C21]. in particular is usually a major cause of sepsis and peritonitis in nephrotic syndrome [22], yet this is the first reported case of pHUS in a patient with nephrotic syndrome. Another potential risk factor in this patient was her exposure to immunosuppressive brokers. Tacrolimus has a well-known association with HUS, but does not typically cause a positive Coombs test. Also, in a review of 16 cases of tacrolimus-induced HUS, the average time from initiating tacrolimus to disease onset was 7.1 months, while our patient had been treated.