Sara Scranton, MD/PGY-2

History
22 month old male
H/O complex febrile seizures (2 prior admissions) and

RSV bronchiolitis (prior hospitalization one month ago) Family is Chin-speaking only, from Burma Patient born and raised in Utah Immunized

History
3 days of high fevers and erythematous rash
3 days cough, congestion Febrile seizure 3 days ago with first fever

Decreased activity, increase fussiness, poor PO

Nose bleeds X 6 mos, increased frequency recently Dad concerned about bruising on arms/legs appeared

3 days ago Bloody stool one episode 2 days ago, now diarrhea Vomiting X 3 episodes in 2 days

Presentation to ED
Vitals: T 40, HR 190, RR 48, BP 77/44, sat 100% RA
Normal Exam except for: Fussy

Macrocephalic
Course breath sounds Hepatosplenomegaly Bruising on Extremities Erythematous rash on extremities

Labs in ED

LABORATORY: ABNORMAL - CBC w/ diff: WBC 14.7, Hct 32.7, MCV 74, Plts 215, 25 Bands, 44% N, 38.1% L - CMP: Na 134, K 3.4, Cl 105, CO2 16, Glu 133, BUN 21, Cr 0.55, Ca 8.2, Prot 6.5, Alb 3.2, Bili 0.4, Alk Phosph 343, ALT 145, AST 161 - PT 15.8, INR 1.3, PTT 41, Fibrinogen 840 - Blood gas: 7.43 pH, 24 pCO2, PO2 62, HCO3 16.1, Base Def 7 - Urine: 10 WBC, 10 RBC, Epi>30, Urate Cryst 3+, Urine micro 100+ protein,

leukesterase +

- Stool Guaiac +, VRP: RSV + - CRP 24.2, ESR 107

Imaging in ED
IMAGING: NORMAL -CT Brain without contrast: Intracranial contents

normal. -CT Abdomen and pelvis with contrast: Normal abdominal and pelvis CT. Appearance of the liver, spleen, kidneys, adrenal gland, pancreas, and gallbladder is normal. -XR Chest: perihilar bronchial wall thickening with some streaky perihilar opacities. These findings may be seen in the setting of viral infection or reactive airways disease.

Physical Exam on Admission

PHYSICAL EXAM: ON THE FLOOR T: 38.2 . HR: 150 . RR:48. BP: 108 / 64 . SaO2 94 % on Room Air. GENERAL: Very fussing, difficult to perform exam. Moans and cries. Is overall alert and active. HEAD: macrocephalic, atraumatic, EYES: normal red reflex and pupillary reflexes bilaterally, extraocular movements intact, conjugate gaze, no conjunctival injection, no drainage, EARS: tympanic membranes gray on the left side and slightly erythematous on the right side, normal light reflex and landmarks, no effusion or perforation, NOSE: no discharge or obstruction. OROPHARYNX: moist mucus membranes, tonsils without exudate, pharyngeal erythema and slightly erythematous tounge, white lesions on tongue and buccal mucosa NECK: cervical lymphadenopathy more prominant in the posterior cervical region, palpable pea to marble sized nodes, non tenderness to palpation.

CARDIOVASCULAR: tachycardia, rhythm, and S1/S2, without murmur

or gallop. Pulses appropriate. Capillary refill time 3 seconds.

Physical Exam on Admission

LUNGS: clear to auscultation bilaterally, good air flow, no retractions, mild crackles bilaterally.
ABDOMEN: soft, non-tender, distended with active bowel sounds and no masses. However there were palpable bowel loops. No splenomegaly. Hepatic edge palpated 2 cm below the costal margin.

EXTREMITIES: all extremities warm and well perfused. No cyanosis or clubbing. Mild edema of hands and feet. BACK: no abnormalities noted
GENITOURINARY: Normal penis and scrotum without edema, testes palpated. Mild diaper rash. No other lesions or rashes. No anal lesions or fissures. NEUROLOGIC: awake and alert, cranial nerves II-XII grossly intact, grossly

normal strength and tone, patellar tendon reflexes normal. SKIN: Erythematous palpable macular rash on the arms and legs. Lesions average of 1-2 cm in diameter, mostly round and some nodular feeling. They are non-tender. Diffuse tiny petechia on feet. Skin peeling around his finger nails.

Differential Diagnosis?

Kawasaki HSP Autoimmune Hepatitis Rheumatic Fever HSV or EBV Hepatitis Toxins Tylenol or Anticonvulsant Leukemia Metabolic Disorder Vitamin K Deficiency Thalassemia ARF Endocarditis

Next Day
Blood Culture Grew: Gram + Cocci in 13 hours
Results: Group A Strep

Streptococcal Toxic Shock Syndrome (STSS)

OR Toxic Shock-Like Syndrome (TSLS)

Streptococcus pyogenes
AKA Group A Strep
Most Common Clinical manifestations: pharyngitis,

impetigo, cellulitis, toxic shock syndrome, necrotizing fasciitis

Complications of poststreptococcal infection:

rheumatic fever, glomerulonephritis

Streptococcal Toxic Shock Syndrome (STSS)

More than 120 distinct serotypes or genotypes of group

A beta-hemolytic streptococci (Streptococcus pyogenes) have been identified Most cases of STSS are caused by strains producing at least 1 of several different pyrogenic exotoxins, most commonly SPE A The toxins act as superantigens that stimulate production of TNF and other inflammatory mediators that cause capillary leak and other physiologic changes, leading to hypotension and organ damage.

Toxic Shock Syndrome

Toxic Shock Syndrome with Group A Strep usually less

likely to cause typical sun-burn like rash as Staph dose

This patient was diagnosed with erythema nodosum

secondary to the Group A Strep as his rash (confirmed on dermatology biopsy)

Toxic Shock Syndrome

Diagnosis is a Clinical Case Definition
CDC no longer has a definition: This page has been removed. CDC does not have information on Toxic Shock Syndrome. For other disease topics, please see the CDC homepage. RedBook Definition based off of : The Working Group on Severe Streptococcal Infections. Defining the group A streptococcal toxic shock syndrome: rationale and consensus definition. JAMA. 1993;269(3):39039.

Toxic Shock Syndrome

Step 1: Isolation of group A

streptococcus (Streptococcus pyogenes)

1A:From a normally sterile site (eg, blood, cerebrospinal fluid, peritoneal fluid, or tissue biopsy specimen) 1B:From a nonsterile site (eg, throat, sputum, vagina, open surgical wound, or superficial skin lesion)

Toxic Shock Syndrome

Step 2: Clinical signs of severity 2A: Hypotension: systolic lower than the fifth percentile for age in children

BP 84/39 is 5th % for his age/height presented with BP 77/44 in ED

AND

Toxic Shock Syndrome

2B: Two or more of the following signs: Renal impairment: creatinine concentration at least 2 times the upper limit of normal for age Coagulopathy: platelet count 100 000/mm3 or less or disseminated intravascular coagulation Hepatic involvement: elevated AST, ALT, or total bilirubin concentrations at least 2 times the upper limit of normal for age Adult respiratory distress syndrome A generalized erythematous macular rash that may desquamate Soft tissue necrosis, including necrotizing fasciitis or myositis, or gangrene

Toxic Shock Syndrome

An illness fulfilling criteria IA and IIA and IIB can be

defined as a definite case. An illness fulfilling criteria IB and IIA and IIB can be defined as a probable case if no other cause for the illness is identified.

Toxic Shock Syndrome

Of note this is Old CDC Criteria (still found on Wikipedia) In either case, diagnosis is based strictly upon CDC criteria modified in 1981 after the initial surge in tampon-associated infections.:[1] Body temperature > 38.9 C (102.02 F) Systolic blood pressure < 90 mmHg Diffuse rash, intense erythroderma, blanching with subsequent desquamation, especially of the palms and soles Involvement of three or more organ systems:

Gastrointestinal (vomiting, diarrhea) Mucous membrane hyperemia (vaginal, oral, conjunctival) Renal failure (serum creatinine > 2 times normal) Hepatic inflammation (AST, ALT > 2 times normal) Thrombocytopenia (platelet count < 100,000 / mm) CNS involvement (confusion without any focal neurological findings)

Toxic Shock Syndrome

The incidence of GAS-mediated TSS is highest among

young children and the elderly, although STSS can occur at any age. Of all cases of invasive streptococcal infections in children, fewer than 5% are associated with documented STSS. Mortality rates are substantially lower for children than for adults with GAS-mediated STSS.

Treatment
Fluid management to maintain adequate venous return

and cardiac filling pressures to prevent end-organ damage Anticipatory management of multisystem organ failure Parenteral antimicrobial therapy at maximum doses with the capacity to:
Kill organism with bactericidal cell wall inhibitor (eg, beta-

lactamaseresistant antimicrobial agent) Decrease enzyme, toxin, or cytokine production with protein synthesis inhibitor (eg, clindamycin)

Immune Globulin Intravenous may be considered for

infection refractory to several hours of aggressive therapy or in the presence of an undrainable focus or persistent oliguria with pulmonary edema

Treatment
Once GAS infection has been identified, antimicrobial therapy

can be changed to penicillin and clindamycin. Intravenous therapy should be continued until the patient is afebrile and stable hemodynamically and blood culture results are negative. The total duration of therapy is based on duration established for the primary site of infection. Aggressive drainage and irrigation of accessible sites of infection ASAP. If necrotizing fasciitis is suspected then immediate surgical exploration or biopsy is crucial to identify deep soft tissue infection that should be dbrided immediately. The use of Immune Globulin Intravenous (IGIV) can be considered as adjunctive therapy of STSS or necrotizing fasciitis if the patient is severely ill, although randomized trials to assess efficacy have not been performed. Dosing is unknown.

References
RedBook Online
MayoClinic.com CDC.nih.gov