Case report: POEMS syndrome in childhood.
POEMS syndrome is a rare multisystem disorder, which is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. We report a 15-year-old girl with POEMS syndrome, who developed growth retardation, delayed puberty, gradually increasing abdominal distention, brown skin pigmentation, hypogonadism, hepatosplenomegaly, lympadenomegaly, monoclonal gammopathy, and anemia. The patient dramatically responded to pulse steroid therapy. To our knowledge, this patient is one of the youngest reported cases of POEMS syndrome.
Sevketoglu
Esra
E
Department of Pediatrics, Bakirkoy Dr. Sadi Konuk Research and Training Hospital, Istanbul, Turkiye. caglaes@yahoo.com
Hatipoglu
Sami
S
Ayan
Inci
I
Dogan
Oner
O
Salihoglu
Bahar
B
D000005:Abdomen / Q000473:pathology; D000293:Adolescent; D000740:Anemia / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006130:Growth Disorders / Q000175:diagnosis; D018380:Hematopoietic Stem Cell Transplantation; D006529:Hepatomegaly / Q000175:diagnosis; D006801:Humans; D007006:Hypogonadism / Q000175:diagnosis; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000628:therapy*; D010265:Paraproteinemias / Q000175:diagnosis; D010859:Pigmentation Disorders / Q000175:diagnosis / Q000628:therapy; D011237:Predictive Value of Tests; D011628:Puberty, Delayed / Q000175:diagnosis; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
D013256:Steroids