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Pepper's syndrome: report of two cases at the Charles de Gaulle University Pediatric Hospital Center, Ouagadougou (Burkina Faso).

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).
PMID
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Authors

Mayor MeshTerms
Keywords

Neuroblastoma

Pepper’s syndrome

infant

Journal Title the pan african medical journal
Publication Year Start




PMID- 29599887
OWN - NLM
STAT- MEDLINE
DCOM- 20180405
LR  - 20180405
IS  - 1937-8688 (Electronic)
VI  - 28
DP  - 2017
TI  - [Pepper's syndrome: report of two cases at the Charles de Gaulle University
      Pediatric Hospital Center, Ouagadougou (Burkina Faso)].
PG  - 189
LID - 10.11604/pamj.2017.28.189.11901 [doi]
AB  - 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).
FAU - Kam, Madibele
AU  - Kam M
AD  - Service de Pediatrie Medicale, CHU Pediatrique Charles de Gaulle, Ouagadougou,
      Burkina Faso.
FAU - Douamba, Sonia
AU  - Douamba S
AD  - Service de Pediatrie Medicale, CHU Pediatrique Charles de Gaulle, Ouagadougou,
      Burkina Faso.
AD  - UFR SDS, Universite Ouaga I Pr. Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.
FAU - Nagalo, Kisito
AU  - Nagalo K
AD  - Service de Pediatrie Medicale, CHU Pediatrique Charles de Gaulle, Ouagadougou,
      Burkina Faso.
AD  - UFR SDS, Universite Ouaga I Pr. Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.
FAU - Dao, Lassina
AU  - Dao L
AD  - Service de Pediatrie Medicale, CHU Pediatrique Charles de Gaulle, Ouagadougou,
      Burkina Faso.
AD  - UFR SDS, Universite Ouaga I Pr. Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.
FAU - Koueta, Fla
AU  - Koueta F
AD  - UFR SDS, Universite Ouaga I Pr. Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.
AD  - UFR SDS, Universite Ouaga I Pr. Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.
FAU - Lougue, Claudine
AU  - Lougue C
AD  - UFR SDS, Universite Ouaga I Pr. Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.
AD  - Service de Radiologie, CHU Pediatrique Charles de Gaulle, Ouagadougou, Burkina
      Faso.
FAU - Ye, Diarra
AU  - Ye D
AD  - Service de Pediatrie Medicale, CHU Pediatrique Charles de Gaulle, Ouagadougou,
      Burkina Faso.
AD  - UFR SDS, Universite Ouaga I Pr. Joseph Ki-Zerbo, Ouagadougou, Burkina Faso.
LA  - fre
PT  - Case Reports
PT  - Journal Article
TT  - Le syndrome de Pepper: a propos de deux cas observes au Centre Hospitalier
      Universitaire Pediatrique Charles de Gaulle de Ouagadougou (Burkina Faso).
DEP - 20171031
PL  - Uganda
TA  - Pan Afr Med J
JT  - The Pan African medical journal
JID - 101517926
RN  - Cohen syndrome
SB  - IM
MH  - Burkina Faso
MH  - Developmental Disabilities/diagnosis/physiopathology
MH  - Fatal Outcome
MH  - Female
MH  - Fingers/*abnormalities/physiopathology
MH  - Hepatomegaly/*etiology
MH  - Hospitals, University
MH  - Humans
MH  - Infant, Newborn
MH  - Intellectual Disability/*diagnosis/physiopathology
MH  - Microcephaly/*diagnosis/physiopathology
MH  - Muscle Hypotonia/*diagnosis/physiopathology
MH  - Myopia/*diagnosis/physiopathology
MH  - Obesity/*diagnosis/physiopathology
MH  - Respiratory Insufficiency/*etiology
PMC - PMC5871256
OTO - NOTNLM
OT  - Neuroblastoma
OT  - Pepper's syndrome
OT  - infant
EDAT- 2018/03/31 06:00
MHDA- 2018/04/06 06:00
CRDT- 2018/03/31 06:00
PHST- 2017/02/05 00:00 [received]
PHST- 2017/09/04 00:00 [accepted]
PHST- 2018/03/31 06:00 [entrez]
PHST- 2018/03/31 06:00 [pubmed]
PHST- 2018/04/06 06:00 [medline]
AID - 10.11604/pamj.2017.28.189.11901 [doi]
AID - PAMJ-28-189 [pii]
PST - epublish
SO  - Pan Afr Med J. 2017 Oct 31;28:189. doi: 10.11604/pamj.2017.28.189.11901.
      eCollection 2017.