Female patient, 3 years 10 months old, with a history of severe mitral stenosis and recurrent pneumonia, who motivated nine hospitalizations in three years, none of them severe in nature or requiring ICU therapy.
He was admitted to the basic care area with a diagnosis of febrile syndrome under study, with a history of decay and short-term fever.
He developed shock and purpuric lesions in the inguinal area within a few hours.
Lived to the critical care area (CC) with compromised consciousness, fever (T° 38.2 °C), hypotension (mean arterial pressure 56 mmHg), purpura and reticularis generalized.
On admission to ACC examination, severe lactacidemia (venous gasses with pH 7.0, BE-18, lactate 11 mmol/1, (VN = 0.5-2.2 mmol/1) and leukocyte count was abnormal 36%.
All these findings were compatible with severe septic shock. Mechanical ventilation, invasive monitoring and volume resuscitation were initiated (130 ml/kg on the first day), fresh transfusion of platelets (blood clotting unit), 30 ml/kg frozen).
She received supplementation with corticosteroids and calcium, alkalinization and continuous insulin infusion.
Antibiotic therapy with vancomycin (60 mg/kg/day), clindamycin (40 mg/kg/day) and ceftriaxone (100 mg/kg/day).
In the first 24 hours, vasoplegic septic shock refractory to vasopressants and terlipressin was evident.
Episodes with multiple abdominal syndrome, which responded to depression, multiple organ dysfunction syndrome (hepatic, renal, disseminated intravascular coagulation DIC), which required continuous hemostasis.
There was no significant progression of the lesions, but persisted in shock.
In view of these antecedents, it was performed on the second day of hospitalization echogram and abdominal CT, which showed absence of spleen and bilateral adrenal volume increase, supporting the diagnosis of Waterhouse Friderichsen syndrome.
Howell-Jolly corpse was found in the blood smear.
Then 2 g/kg of immunoglobulin ev was administered.
High frequency oscillatory ventilation was initiated for ARDS refractory to mechanical ventilation, responding to the prolonged prone position.
A blood culture was positive for S. pneumonicte, serotype 19 F, penicillin resistant (MIC 4.0 μg/ml) and sensitive to cefoxime (MIC 0.75 μg/ml).
The patient was hemodynamically stable but had a bilateral cerebral hemorrhagic infarction with development of hemoventricle and severe endocraneal hypertension, which precipitated his death on the eighth day of admission.
Necropsy revealed necrotizing bilateral adrenal ventriclesgia, bilateral hemorrhagic pneumonia, acute mitral valve endocarditis, generalized septic emboli, left parietal intraparenchymatous hemorrhage and intraventricular hemorrhage.
The absence of the spleen (congenital asplenia) was confirmed.
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Immunological and imaging studies performed on family members yielded normal results.
