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

[Richard Wasserman]

Colleagues:

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, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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