August 1993 | J E Sansom, P Jardine, P W Lunt, W H Schutt, C T C Kennedy
The text discusses two main topics: the role of fat in female recognition and the overlap between Proteus and Klippel-Trenaunay syndromes, as well as an asymptomatic metastatic small bowel carcinoid case.
Fat makes recognition more likely in females than in males. Early recognition based on physical appearance can help avoid serious complications like pancreatitis in individuals and their families. This is linked to familial partial lipodystrophy, an X-linked dominant syndrome, and lipoatrophic diabetes, which are associated with lipodystrophy and diabetes.
The case describes a patient with features of both Proteus and Klippel-Trenaunay syndromes. Proteus syndrome is characterized by asymmetrical overgrowth, epidermal nevi, port-wine stains, and macrodactyly. Klippel-Trenaunay syndrome involves port-wine stains, limb hypertrophy, and bony overgrowths. The patient had hemihypertrophy, macrodactyly, epidermal nevi, and port-wine stains, which are characteristic of Proteus syndrome. However, complete syndactyly of the second and third toes and absence of plantar soft tissue hypertrophy are more consistent with Klippel-Trenaunay. Recurrent venous thrombosis, which is common in Klippel-Trenaunay, occurred in the limb not affected by hemihypertrophy, suggesting possible overlap between the two syndromes.
The text also presents a case of an asymptomatic 76-year-old woman with extensive metastatic small bowel carcinoid. The patient was found to have a 10 cm liver lesion and a malignant neuroendocrine tumor. After surgery, including ileal resection, right hemihepatectomy, and metastasectomies, the patient recovered well and remained asymptomatic one year later.
The text references multiple studies and cases to support these findings.The text discusses two main topics: the role of fat in female recognition and the overlap between Proteus and Klippel-Trenaunay syndromes, as well as an asymptomatic metastatic small bowel carcinoid case.
Fat makes recognition more likely in females than in males. Early recognition based on physical appearance can help avoid serious complications like pancreatitis in individuals and their families. This is linked to familial partial lipodystrophy, an X-linked dominant syndrome, and lipoatrophic diabetes, which are associated with lipodystrophy and diabetes.
The case describes a patient with features of both Proteus and Klippel-Trenaunay syndromes. Proteus syndrome is characterized by asymmetrical overgrowth, epidermal nevi, port-wine stains, and macrodactyly. Klippel-Trenaunay syndrome involves port-wine stains, limb hypertrophy, and bony overgrowths. The patient had hemihypertrophy, macrodactyly, epidermal nevi, and port-wine stains, which are characteristic of Proteus syndrome. However, complete syndactyly of the second and third toes and absence of plantar soft tissue hypertrophy are more consistent with Klippel-Trenaunay. Recurrent venous thrombosis, which is common in Klippel-Trenaunay, occurred in the limb not affected by hemihypertrophy, suggesting possible overlap between the two syndromes.
The text also presents a case of an asymptomatic 76-year-old woman with extensive metastatic small bowel carcinoid. The patient was found to have a 10 cm liver lesion and a malignant neuroendocrine tumor. After surgery, including ileal resection, right hemihepatectomy, and metastasectomies, the patient recovered well and remained asymptomatic one year later.
The text references multiple studies and cases to support these findings.