[CIS PIDD] [cis-pidd] Recurrent Sepsis Syndrome

[wolska]

Hi RichardIt is realy intruiging case. What about cellular immunity?Did you consider systemic autoinflammatory diseases?Do you have any photos of edema? how does it looks like ?-- Beata Wolska - KuśnierzOddział ImmunologiiIP-CZD Al.Dzieci Polskich 2004-730 Warszawatel. 22 815 73 85fax. 22 815 73 82bwolska at interia.plOd: "Richard Wasserman" <drrichwasserman at gmail.com>Do: "CIS-PIDD" <cis-pidd at lyris.dundee.net>; Wysłane: 19:28 Czwartek 2015-04-30Temat: [cis-pidd] Recurrent Sepsis SyndromeColleagues:I need help with a 23 yo male admitted for the 6th episode of culture negative sepsis syndrome since he had H1N1 pneumonia requiring mechanical ventilation about one year ago. The episodes are characterized by high fever but no focal findings. Hypotension develops in the first 24-36 hours and requires fluid resuscitation and pressors. Initial WBC ~4500 and left shifted. Increases to ~10,000 in the first few days. CRP increases to ~60. He is always treated with multiple antibiotics at presentation and blood cultures, fungal blood cultures, respiratory cultures and respiratory virus assays have been negative. He improves over three to five days and is discharged. The episodes are not temporally related (either before or after) to his IGIV.I apologize for the length of his history but he has had multisystem disorders that I don’t understand and may stimulate a productive thought.Immunologic – At age 4, he an evaluation for recurrent infection (see below) that showed low normal immunoglobulin concentrations and poor response to pneumococcal polysaccharide vaccine.  After prophylactic antibiotics failed, he improved with IGIV. At age 14 IGIV was stopped and he deteriorated. Reevaluation showed hypogammaglobulinemia. IGIV was restarted and bacterial infections have been a minor, occasional problem.Infection - Chronic otitis as an infant evolved to recurrent sinusitis and conjunctivitis. Bacterial chest infections have been rare. There have been many episodes of Candida esophagitis related to steroid and antibiotic use. At age 14, he had a respiratory tract infection the resulted in symptoms that were only partially bronchodilator responsive. Hospitalization was prolonged. One year later, he developed parainfluenza 3 pneumonia and was hospitalized. At age 22, he developed H1N1 pneumonia and required mechanical ventilation. He has returned to baseline of limited activity tolerance.Respiratory – There is long standing cough, chest tightness and shortness of breath that responds partially to high dose bronchodilators and steroids. Cough worsens with minimal activity. Viral infections are the major trigger and recovery is prolonged. FEV1 has always been normal without bronchodilator responsiveness. Chest CT when he is not actively infected is normal.Neurologic - Partial complex and Jacksonian seizures with visual disturbances and additional minor motor components. At the onset, seizures were totally disabling with dozens of seizures per day. Uncontrolled seizures required intubation and drug induced paralysis on several occasions. During that time, he developed alexia that has resolved after about one year of intensive retraining in reading and writing. The patient’s mother observed that his seizure frequency and severity improved after IGIV infusions. Neurologic problems have responded to high dose IGIV, pulse steroids and mycophenolate but not to infliximab or etanercept. Studies for autoantibodies associated with neurologic disease have been negative. Repeated MRI's have been completely normal.Peripheral edema – At age 14 he developed dependent peripheral edema that is partially responsive to diuretics. There is neither abdominal nor pelvic compression nor vascular disease on Doppler exam. His cardiologist and endocrinologist believe that the problem is neither cardiac nor endocrine. An evaluation for dysautonomia was negative.GI - Motility studies showed esophageal dysmotility, gastroparesis and atony of the descending colon. Diet is limited because of food allergy and multiple non-IgE mediated food intolerances and is supplemented with elemental formula. About 60% of calories are via TPN. Cholecystectomy at age 15, polyposis of the gall bladder.Renal – Hypertension and electrolyte wasting of unknown etiology.Endocrine – He was thought to have steroid induced adrenal suppression but a stimulation test performed after several years without prolonged steroid therapy showed no cortisol production. He is maintained with physiologic steroid replacement. Family history – he is an only child. Mother has SLE.Thank you,Richard-- Richard L. Wasserman, MD, PhDDallasAllergyImmunology7777 Forest Lane, Suite B-332Dallas, Texas 75230Office (972) 566-7788Fax (972) 566-8837Cell (214) 697-7211


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