[Pepper's syndrome: report of two cases at the Charles de Gaulle University Pediatric Hospital Center, Ouagadougou (Burkina Faso)]. / Le syndrome de Pepper: à propos de deux cas observés au Centre Hospitalier Universitaire Pédiatrique Charles de Gaulle de Ouagadougou (Burkina Faso).
Pan Afr Med J
; 28: 189, 2017.
Article
in Fr
| MEDLINE
| ID: mdl-29599887
ABSTRACT
Pepper's syndrome is a neuroblastoma that metastasizes to the liver. It affects infants younger than six months of age. It can regress spontaneously and is associated with a favorable prognosis in 80% of cases. Given its rarity, we here report two cases of Pepper's syndrome observed at the Charles de Gaulle university pediatric hospital center, Ouagadougou (Burkina Faso). Our study involved two female infants in whom the disease manifested as an increase in abdominal volume, hepatomegaly and signs of respiratory distress. Ultrasound enabled diagnosis, which was based on the nodular appearance of the liver in both cases and determination of the primary tumor in one case. Urinary catecholamine test confirmed the diagnosis in one case. Both patients died from complications related to liver compression, chemotherapy (in one of the cases) and lack of treatment (in the other case).
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Respiratory Insufficiency
/
Fingers
/
Hepatomegaly
/
Intellectual Disability
/
Microcephaly
/
Muscle Hypotonia
/
Myopia
/
Obesity
Type of study:
Diagnostic_studies
/
Prognostic_studies
Limits:
Female
/
Humans
/
Newborn
Country/Region as subject:
Africa
Language:
Fr
Journal:
Pan Afr Med J
Year:
2017
Document type:
Article
Affiliation country:
Burkina Faso