The case presentation of Gregory Barens

1. Case presentation of Gregory barens
by Nathiya
• Gregory brought to children’s hospital , with
• HIGH FEVER
• COUGH
• DYSPNEA

2. • On physical examination , he was pale , looked dehydrated ,
breathing rapidly with flaring nostrils.
• Respiratory rate : 62/min (normal :20/min)
• Pulse :120/min (normal : 60-80/min)
• Blood pressure :90/60mmHg (normal )
• Auscultation : crackles on lower left lobe
of left lung
• Diagnosis : LOBAR PNEUMONIA

4. Laboratory Results :
• WBC count – 19000 cells (normal-4000-7000 cells)
• 87% neutrophils (normal -60%)
• Infection with gram positive cocci : streptococcus pneumoniae
• No history of allergy to penicillin
Treatment:
• Intravenous ampicillin (1g every 6 hours)
• 4th day – RS 40/min ; WBC count 9000 ; temp 37.5 C
• 9th day – no fever ; WBC count 7000

5. When ampicillin was replaced by penicillin,
Symptoms like
• Puffy eyes
• Large hives(urticaria) on abdomen
• Anti histamine Benadryl was given orally& penicillin was discontinued
• Two hours later wheezing was heard all over lungs which responded to
inhalation of beta2-adrenergic agent albuterol
• He also had reddened eyes owing to inflamed conjunctiva
• Swelling around mouth

6. • Lymph nodes (cervical , axillary , inguinal) and spleen – enlarged
• Ankles and knee joints – swollen & painful
• Raised WBC with predominant lymphocytes (72%)
• Plasma cells were detected in blood smear
• RBC sedimentation rate 30mm/h
• Serum C1q & C3 levels
DIAGNOSIS : SERUM SICKNESS & given BENADRYL and
NAPROSYN(a non-steroidal anti-inflammatory agent)

7. • Rash and swellings became worse
• Purpuric lesions on feet
• No blood in stool
• Later Gregory became agitated , disoriented , couldn’t recognize his parents
• Examination of CSF : presence of inflammatory cells
• Increased protein concentration
• EEG: circulation of blood in posterior part of brain
• Biopsy of purpura : moderate edema & IgG and C3 deposition

9. TYPE III HYPERSENSITIVITY
• Type III hypersensitivity occurs when
there is accumulation of immune
complexes (antigen-antibody complexes)
that have not been
adequately cleared by innate immune
cells, giving rise to an inflammatory
response and attraction of leukocytes.
Such reactions progressing to the point
of disease produce immune complex
diseases.

13. Serum sickness
• When an antigen is injected intravenously , the immune complexes formed
can be deposited at various sites
• When deposited in
• The synovial tissue inflammation of joints
Arthritis
• The kidney glomeruli glomerulonephritis
• The endothelium of blood vessels vasculitis

14. Serum from the blood of immunized animals
contained ANTITOXIN

15. • Although horse serum is no longer used , other foreign proteins are still
administered to the patients
• The most common cause of serum sickness is antibiotics esp. penicillin and
its derivatives , which act as HAPTENS
• These drugs bind to proteins (CARRIERS) elicit rapid and strong
IgG antibody response
• Antigen may be exogenous or endogenous

16. Symptoms of serum sickness can include:
•Fever.
•General ill feeling.
•Hives.
•Itching.
•Joint pain.
•Rash.
•Swollen lymph nodes.

17. • Urticaria, is a prominent feature of the rash, implying a role for histamine
derived from mast-cell degranulation.
• The mast-cell degranulation is triggered by the ligation of cell surface
FcγRIII by IgG-containing immune complexes.
• Serum sickness after a second dose of antigen follows the kinetics of a
secondary antibody response and the onset of disease occurs typically
within a day or two.
• Serum sickness is nowadays seen after the use of anti-lymphocyte
globulin, employed as an immunosuppressive agent in transplant
recipients, & also, rarely, after the administration of streptokinase, a
bacterial enzyme that is used as a thrombolytic agent to treat patients
with a myocardial infarction or heart attack.

18. Immune complexes in blood vessels

22. Two reasons why small immune complexes would be
cleared more slowly than large complexes
• Small complexes activate complement less effectively than large complexes because C1
must bind to several Fc regions before it is activated. The avidity of complexes for
binding to phagocytes will also be increased when several Fc regions are available to
bind to the Fc receptors on a phagocyte. If this were not so, monomeric antibody would
block binding of complexes to Fc receptors.
• The formation of complexes is followed by an abrupt fall in total hemolytic complement.
• The clinical signs of serum sickness that develop are due to granular deposits of antigen–
antibody and C3 forming along the glomerular basement membrane (GBM) and in small
vessels elsewhere.
• As more antibody is formed and the reaction moves into antibody excess, the size of the
complexes increases and they are cleared more efficiently.

24. Treatment:
• He was started on anti-inflammatory corticosteroid prednisone
• Symptoms improved progressively
• Joint swelling and splenomegaly resolved
• He was discharged after 7 days of onset of serum sickness on a slowly
decreasing course of prednisone and Benadryl
• He had no IgE antibodies against penicillin or ampicillin detected by both
immediate hypersensitivity skin test and RAST
• Instruction: Gregory should never be given penicillin, penicillin derivatives or
cephalosporins