[Shwachman's syndrome].
BACKGROUND
Shwachman syndrome has been recognized as the second most frequent cause of pancreatic insufficiency in children and its is related to hematological and bony abnormalities.


OBJECTIVE
To know the main clinical symptoms, analytical data, radiological and sonographic findings in the Shwachman syndrome.


METHODS
We present a 1 year 4 months old girl with Shwachman syndrome. We reviewed the clinical history taking into consideration height/weight relationship according to percentiles and physical examination. Analytical data: blood counts, fetal hemoglobin, vitamin B12 and folate levels, bone marrow examination, stool cultures and examination for ova and parasites, transaminase levels, sweat electrolytes, serologic tests for viral hepatitis. Radiological studies: bone series and bone age, abdominal sonography and computed tomography. Liver and intestinal biopsies.


RESULTS
In this patient we confirmed the presence of exocrine pancreatic insufficiency, dysfunction of the bone marrow and bony abnormalities typical in Shwachman syndrome.
Zárate Mondragón
F E
FE
Servicio de Gastroenterología y Nutrición Pediátrica, Instituto Nacional de Pediatría, Col. Insurgentes Cuicuilco.
Ramírez Mayans
J A
JA
Cervantes Bustamante
R
R
Mora Tiscareño
M A
MA
Mata Rivera
N
N
Rodríguez Lizárraga
E
E
D001842:Bone and Bones / Q000002:abnormalities* / Q000000981:diagnostic imaging; D010188:Exocrine Pancreatic Insufficiency / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D007223:Infant; D009503:Neutropenia / Q000150:complications* / Q000175:diagnosis; D013577:Syndrome; D014057:Tomography, X-Ray Computed
Síndrome de Shwachman.