PEDIATRICS.....PART TWO....RARE FEVERS

Today, we'll cover some of the rarer disorders...causing fever in the pediatric/adolescent
populations....which can be encountered in practice...and are often covered on the Boards.


Q: Which are the "common" HEREDITARY PERIODIC FEVER SYNDROMES?


A: There are three disorder, which one should consider "outside" of the common
causes of fever...when seeing a patient for recurrent fever, without a secondary
cause and explanation.

1. FAMILIAL MEDITERRANEAN FEVER
2. HYPER- IgD SYNDROME
3. FAMILIAL HIBERNIAN FEVER a.k.a TNF Receptor-Associated Periodic Syndrome.



Q: What is FAMILIAL MEDITERRANEAN FEVER (FMF)?


A: It is an inherited illness, causing recurrent fevers and "ITIS"-es. Eg. peritonitis,
pleuritis, arthritis and rarely pericarditis. Occurs predominantly Mediterranean
descendants....Italians/Greeks/Cypriots/Turks/Armenians/Jews.
TREATMENT: COLCHICINE....will also prevent potential Amyloidosis.



Q: What is the HYPER-IgD SYNDROME?


A: It is rare illness, producing recurrent fever in children in, the first year of life.
These can be triggered by vaccination/stress. Occurs mainly in Northern European,
Dutch, French children. Besides the fever, common symptoms are vomiting/diarrhea
arthralgias, maculopapular rash and cervical adenopathy.
DIAGNOSIS: is established by: Serum IgD level > 100 IU/ml. TX: is empiric.



Q: What is FAMILIAL HIBERNIAN FEVER, a.k.a TNF Receptor-Associated Periodic
Syndrome?


A: It is also a hereditary condition, casuing recurrent fevers, arthralgias, and rash occuring
in Scottish, Irish descendants...but is not limited to this ethnic group. This is more common
in adolescents...before age 20. SYMPTOMS: Fever, with myalgias and extremity swelling.
Diagnosis: Low levels of type 1 TNF receptor ( < 1 ng/ml)....between attacks.
TREATMENT: PREDNISONE and ETERNACEPT.


Of note...both Familial Mediterranean Fever and Familial Hibernian Fever can be
complicated by AMYLOIDOSIS!


Thank you for your time...we'll be back shortly.