clinical conundrum
DESCRIPTION
Clinical Conundrum. Stephanie Kuhlmann, MD, FAAP Brooke R. Mason, MD, FAAP KU Pediatric Hospitalist Group Wesley Medical Center Wichita, KS. Chief Complaint. 7 year old female presents with 3 day history of fever up to 104.9 and myalgia. History of the Present Illness. Fevers x 3 days - PowerPoint PPT PresentationTRANSCRIPT
Stephanie Kuhlmann, MD, FAAPBrooke R. Mason, MD, FAAP
KU Pediatric Hospitalist GroupWesley Medical Center
Wichita, KS
7 year old female presents with 3 day history of fever up to 104.9 and myalgia
• Fevers x 3 days• L shoulder and L thigh pain now resolved upon
admission• Febrile episode 3 weeks ago (temp 104)
• refused to bear weight at the time • Evaluated at an ER in Wichita the prior evening
• Diagnosis: viral illness - received IVF and sent home• Recurrent fevers since infancy
• every 2-3 months• lasts 1-2 days now 3-4 days
POSITIVES• CONSTITUTIONAL: fever• GI: constipation, decreased
appetite• RENAL: decreased UOP• MS: thigh pain, shoulder pain• HEME: occasional epistaxis
NEGATIVES• CONSTITUTIONAL: weight loss• HEENT: headache, vision
changes, sore throat, sores in mouth, ear pain
• NECK: swollen lymph nodes, stiffness
• PULM: cough or respiratory symptoms
• GI: abdominal pain, nausea, vomiting, diarrhea
• SKIN: rashes or skin changes• MS: swelling or redness of
joints, swelling of extremities• HEME: no easy bruising
• Medical Conditions:– FTT as infant associated with diarrhea
• tested negative for Celiac but noted partial IgA deficiency
• Hospitalizations: None• Surgeries: None• Meds: Tylenol/Motrin• Developmental History:
– Mild speech delay and receiving speech therapy
• Social History• Military family
• Recent move from North Dakota to Montana to Kansas
• Cat and dog at home• No camping or recent international travel
• Family History• No autoimmune disease• Mom and MGM with SVT
• VITALS: T 101.4 R, HR 151, RR 20, BP 114/59, SaO2 100% on RA, Wt 20.1 kg (20th%), Ht 122 cm (50th%)
• GEN: Alert and oriented, non toxic appearing • HEENT: TMs, conjunctiva and pharynx without erythema, no oral lesions, no
nasal congestion or discharge • NECK: Supple, no stiffness • CHEST: CTAB • CV: Tachycardic, but regular rhythm, no murmur, adequate pulses and cap refill • ABD: Soft, NT, no masses, no HSM • MS: No joint swelling, no decreased ROM, no tenderness along L thigh• EXT: Pink, warm, well perfused , no edema• SKIN: No rashes or lesions• NEURO: No deficits, strength appropriate• LYMPH: No palpable nodes throughout
• CMP normal• UAM negative• CBC
– WBC 8.7– Hgb 10.7– Platelets 164K– 80% neutrophils– 12% lymphs– 15% monos– MCV and RDW WNL
• Infectious (osteomyelitis , pyomyositis, TB, EBV/CMV, HIV)
• Rheum/inflammatory (SLE, JIA)• Periodic fever syndrome (PFAPA, TRAPS, FMF,
NOMID, cyclic neutropenia)• Oncology (leukemia, bone tumor)• Endo (thyroid)
• Received Tylenol PO, Zofran PO, NS bolus of 400 mL
• CBC, CMP, CRP, ESR, CPK, iCa, Mg, Phos, TSH, ANA ordered
• CBC with Hgb 10.6, MCV nml; remainder normal• Blood culture (negative)• ESR, CRP (mild to moderate elevation; 47.2 and 27)• CPK (WNL, 39)• UA (2+ ketones otherwise unremarkable)• ANA (negative)• Thyroid studies (WNL)
• Monitored in hospital overnight • Tmax 105 F• Complained briefly of abdominal pain with a
benign exam • Low blood pressure overnight with SBP of 73
• not tachycardic, well perfused, good urine output• fluid bolus and her BP normalized
• Ordered HIV PCR, Mono spot, Quantiferon• Film of L femur • Consulted ID
“This is presumptive Periodic Fever based on the history of fairly regularly occurring
episodes of fever once a month for 2-3 years and well between the episodes.”
• One small ulcer like lesion on her lip• SBP 73-107, HR 105-135, Tmax 104• Albumin dropped to 2.7 gm/dL• Platelets decreased to 120k • Hgb decreased to 9.1 gm/dL• Ferritin, LDH, iron, haptoglobin ordered• Question if bone marrow biopsy needed• EBV and CMV serology sent
• Continued to be intermittently febrile, (Tmax 104)
• SBP 93-102, HR 98-138• Developed exudative pharyngitis,• Shotty cervical lymphadenopathy• Hgb 9.8 gm/dL, platelets 156 K, WBC 5.9• Strep screen and culture
• Afebrile after midnight• SBP WNL• Developed 2nd lesion on lip• Exudative pharyngitis, anterior and posterior
cervical adenopathy• Given dose of 2 mg/kg of prednisolone for
presumed PFAPA• ANA negative, CMV serology and EBV IgM
WNL• Dismissed to home
• Periodic Fever, Aphthous stomatitis, Pharyngitis, and Adenitis
• Other findings: • Malaise, headache, abdominal pain, arthralgia, HSM,
leukocytosis, and elevated acute phase reactants. • Symptoms begin around 2–6 yr of age • Lasts 4–6 days, regardless of antipyretic or antibiotic
treatment• Occurs at a frequency of 8–12 episodes/yr.
• Frequency and intensity of the episodes diminish over time
• Etiology and the pathogenesis unknown. • Response to a single dose of prednisone (1–2
mg/kg) with prompt resolution of symptoms within 24 hr
• Complete resolution has also been reported after tonsillectomy
• No long term sequelae