Morning Report

September 4th, 2013 Carrie Johnson

HPI
In Urgent Care in August You see a 3 yo Caucasian female with fever, sore throat and swollen lymph nodes for 2 days Fever reach 104 on day of presentation Complaining of throat pain, discomfort with swallowing Decreased oral intake Also complains of generalized stomach ache; no vomiting or diarrhea

HPI continued
Some body aches, head aches intermittently No altered mental status No cough, congestion, rhinorrhea, ear pain, or snoring. No joint pain, rash, night sweats, weight loss. No dysuria, no back pain Using ibuprofen which seems to help fevers, pain

 PMH/PSH:
Bilateral Grade III VUR, no prophylaxis, no history of UTI. Has had a few similar episodes of lymph node enlargement and fever fairly regularly throughout past year. Always with a negative strep test. Had blood work done at PCPs office, slight elevation of WBC (2021), normal differential, platelet, hemoglobin, normal UA/culture. Received a course of antibiotics each time. Between episodes feels well.

Unremarkable social history and family history. No sick contacts, no daycare. No medication use other than Ibuprofen during illness

Physical Exam
Temp 102 F, HR 110, RR 20, BP 86/52 Wt: 73%ile for age, Ht: 84%ile for age Gen: Well nourished, non-toxic HEENT: EOMI, no conjunctival injection, TMs normal, nares clear. Posterior OP with erythema, thin tonsillar exudate. No lesions, masses, or ulcerations. Discomfort with swallowing. Neck: Full ROM, tender bilateral submandibular lymph node enlargement (1.5 cm). No overlying erythema CV: RRR, no murmurs, rub, or gallop. Good perfusion Resp: CTAB, no wheezing or crackles, no increased WOB Abd: soft, no tenderness to palpation, normoactive BS Extrem: full ROM, nontender Skin: no rashes

Differential
3 year old female with recurrent episodes of fever and tender lymph node enlargement.

Differential
ID Lymphadenitis Pharyngitis -Group A strep (carrier in home?) - viral (rhinovirus, parainflu, coronavirus, adenovirus,
coxsackievirus, Human metapneumovirus)

- H flu, M catarrhalis, strep pneumo, mycoplasma Retropharyngeal Abscess Prevertebral Abscess Atypical mycobacterium Bartonella Henselae EBV/CMV/HIV
Onc Lymphoma Neuroblastoma Heme Cyclic Neutropenia

Rheum/Imm -Serum Sickness -PFAPA -Familial Mediterranean fever -Tumor Necrosis Factor Receptor-associated Periodic Syndrome (TRAPS) -Hyperimmunoglobulin D Syndrome -SLE -Sarcdoidosis -Histiocytic necrotizing lymphadenitis (Kikuchi Disease)

More Past Medical History

When digging through the chart more First presented with similar illness with high fever and swollen lymph nodes nine months ago. Since then, doctors office visit every 4-8 weeks for same symptoms. Always with a negative rapid strep and culture. Often diagnosed with Cervical lymphadenitis or AOM. Received 5 courses of antibiotics Resolution of symptoms in between Patient lovingly nicknamed her lymph nodes my rocks, and anticipated their reappearance every 4-6 weeks.

Periodic Fever with Aphthous stomatitis, Pharyngitis, and Adenitis

Abrupt onset of high fever (38.9-41.1C), malaise, irritability, sore throat. Aphthous ulcers in first 12-24 hours on lips or buccal mucousa Pharyngitis (+/-) exudate Tender cervical adenopathy May also have HA, myalgia, abdominal discomfort

Natural Course of Illness

Onset age 2 to 5 years old Benign, self limited (unknown etiology) Mean duration of attacks ranges from 4.5 to 8 years. With time, episodes are of shorter duration and occur less frequently. Within 5-7 years of onset, most children asymptomatic. Limited cases occurring in adulthood

Laboratory Findings
May have mild leukocytosis (15-19), Elevation of ESR ( 49 +/- 30 mm/hr), CRP increase (~ 2.5 to 7 mg/dl). No increased presence of ANA above baseline population estimates ( 1 in 30 children) All lab values normalize between episodes Strep test should be negative.
If positive may represent benign carriage

Diagnosis
Based on Clinical Presentation 3+ episodes of fever no more than 5 days at regular intervals (on average Q 3-6 weeks) Pharyngitis + aphthous ulcers OR tender cervical LAD Normal growth parameters, good health between episodes (no B symptoms)

Treatment
Optional NSAIDS, tylenol for fevers Tonsillectomy (Controversial)
Small study (N 39): Remission in 63%,

Prednisone (benefit vs. harm)

Single dose of 1-2 mg/kg has dramatic response on fever, pharyngitis within hours Repeated doses shorten the interval between attacks in 25% of cases Can try Colchicine to extend interval between attacks but effect is short lived.

Cimetidine (Case Reports)

20-40 mg/kg divided BID May help with reducing or eliminating recurrent episodes Continue drug therapy for 6 months if initial benefit

PFAPA must be excluded when

Family history of recurrent fever (consider hereditary autoinflammatory syndromes) Elevated CRP/ESR between attacks (suggests chronic inflammation) Atypical symptoms (are not consistent with previous pattern) Neutropenia (consider cyclic neutropenia)

Cyclic Neutropenia
Rare Onset in first year of life Recurring fevers and neutropenia ANC <200 (very consistent time frame for the individual; range 14-35 days) Familial pattern (AD inheritance) Symptoms: malaise, fever, aphthous stomatitis, gingival/mucosal infection, sub/cutaneous infections

Periodic Fever/Autoinflammatory Syndromes

References
Donald Goldsmith. Periodic Fever Syndromes. Pediatrics in Review Vol 30 No.5, May 2009. Shashi Sahai. Lymphadenopathy. Pediatrics in Review 2013;34;216. DOI: 10.1542/pir.34-5-216 Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic Fever syndrome in children Journal of Pediatrics. 1999; 135: 15. Shai Padeh, MD. Periodic Fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA syndrome). Uptodate.com