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Le syndrome de Pepper: à propos de deux cas observés au Centre Hospitalier Universitaire Pédiatrique Charles de Gaulle de Ouagadougou (Burkina Faso)
| Content Provider | Semantic Scholar |
|---|---|
| Author | Kam, Madibèlè Douamba, Sonia Nagalo, Kisito Dao, Lassina Kouéta, Fla Lougué, Claudine Yé, Diarra |
| Copyright Year | 2017 |
| Abstract | Le syndrome de Pepper est une forme metastatique hepatique du neuroblastome. C'est une entite specifique du nourrisson de moins de six mois qui a la particularite de pouvoir regresser de facon spontanee avec un pronostic favorable dans 80% des cas. A cause de sa rarete, nous rapportons deux cas du syndrome de Pepper observes au Centre Hospitalier Universitaire Pediatrique Charles de Gaulle de Ouagadougou (Burkina Faso). Il s'agissait de deux nourrissons de sexe feminin chez qui la symptomatologie de la maladie se traduisait par une augmentation du volume abdominal, une hepatomegalie et des signes de lutte respiratoire. L'echographie a permis de poser le diagnostic par l'aspect nodulaire du foie dans les deux cas et la determination de la tumeur primitive dans un cas. Le dosage des catecholamines urinaires a confirme un cas. L'evolution etait fatale chez les deux patientes du fait des complications de compression par le foie, des complications de la chimiotherapie dans l'un des cas et l'absence de traitement dans l'autre cas. Mots cles: Neuroblastome, syndrome de Pepper, enfant English Title: Pepper's syndrome: report of two cases at the Charles de Gaulle University Pediatric Hospital Center, Ouagadougou (Burkina Faso) English 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). Keywords: Neuroblastoma, Pepper's syndrome, infant |
| File Format | PDF HTM / HTML |
| DOI | 10.11604/pamj.2017.28.189.11901 |
| PubMed reference number | 29599887 |
| Volume Number | 28 |
| Alternate Webpage(s) | https://www.ajol.info/index.php/pamj/article/download/167309/156735 |
| Alternate Webpage(s) | https://doi.org/10.11604/pamj.2017.28.189.11901 |
| Journal | The Pan African Medical Journal |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |