Proteus Syndrome: Report of Two Cases With Pelvic Lipomatosis

Costa, T Rodríguez; Fitch, Naomi; Em, Azouz

doi:10.1542/peds.76.6.984

Cited by 74 publications

(1 citation statement)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, surgical reconstruction may improve the physical appearance and emotional state of children with PS [ 21 ]. Mental retardation and seizures may be part of this syndrome, but the average intelligence usually remains normal [ 22 , 23 ]. Nevertheless, the patient did not manifest any history of seizures or mental retardation.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of an Asymmetric Overgrowth Syndrome in a Kenyan African Child: A Case Report and Review of Literature

Okello¹,

Mutio²,

Masiga³

et al. 2022

Cureus

View full text Add to dashboard Cite

Body overgrowth may be generalized or may affect certain areas. We present a seven-year-old African male with progressive, asymmetric, postnatal overgrowth of the left side of his face and left upper limb. The impression of proteus syndrome (PS) was made based on established clinical diagnostic criteria presented in the literature. Our objective is to highlight the diagnosis based on clinical features, investigations, and a multidisciplinary approach to the management of proteus syndrome.

show abstract

Section: Discussionmentioning

confidence: 99%

A Rare Case of an Asymmetric Overgrowth Syndrome in a Kenyan African Child: A Case Report and Review of Literature

Okello¹,

Mutio²,

Masiga³

et al. 2022

Cureus

View full text Add to dashboard Cite

show abstract

Hemihypertrophy, hemimegalencephaly, and polydactyly

Reardon¹,

Harding²,

Winter³

et al. 1996

Am. J. Med. Genet.

View full text Add to dashboard Cite

W e present clinical and neuropathological details of a patient with hemihypertrophy and hemimegalencephaly who may have Proteus syndrome. The observation of polysyndactyly in the case indicates either that polysyndactyly is a rare manifestation in Proteus syndrome, or that a separate condition, mimicking Proteus syndrome and pursuing a similar clinical course, might exist. 0 1996 Wiley-Liss, Ine.

show abstract

Elattoproteus syndrome: Delineation of an inverse form of Proteus syndrome

Happle

1999

Am. J. Med. Genet.

View full text Add to dashboard Cite

A 7-year-old boy had partial lipohypoplasia and patchy dermal hypoplasia involving large areas of his body. These areas of deficient growth were similar to those described in many cases of Proteus syndrome. Paradoxically, however, he had only few and rather mild lesions of disproportionate overgrowth. This unusual case is taken as a clue to postulate the Elattoproteus syndrome, an inverse form of Proteus syndrome. The paradoxical coexistence of hyperplastic and hypoplastic lesions may reflect a twin spot phenomenon. The patient would carry at the Proteus locus one allele giving rise to overgrowth of tissues (Pleioproteus allele--from Greek pleion, meaning plus), whereas the other allele would cause deficient growth of tissues (Elattoproteus allele--from Greek elatton, meaning minus). At an early stage of embryogenesis, somatic recombination would give rise to two different populations of cells homozygous for either allele. From a heuristic point of view, one may postulate the existence of the Elattoproteus syndrome, a purely inverse form of Proteus syndrome that would develop in the absence of the Pleioproteus allele.

show abstract

Proteus Syndrome: Report of Two Cases With Pelvic Lipomatosis

Cited by 74 publications

References 0 publications

A Rare Case of an Asymmetric Overgrowth Syndrome in a Kenyan African Child: A Case Report and Review of Literature

A Rare Case of an Asymmetric Overgrowth Syndrome in a Kenyan African Child: A Case Report and Review of Literature

Hemihypertrophy, hemimegalencephaly, and polydactyly

Elattoproteus syndrome: Delineation of an inverse form of Proteus syndrome

Contact Info

Product

Resources

About