Medical Mystery — The Answer
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《新英格兰医药杂志》
To the Editor: The medical mystery in the July 1 issue1 involved 50-year-old identical twins (Figure 1A). The patient is the twin on the left side of the photograph. He has acromegaly caused by a growth hormone–hypersecreting pituitary macroadenoma. He presented with visual-field impairment, and magnetic resonance imaging showed a pituitary macroadenoma with extrasellar extension (Figure 1B shows a coronal section, and Figure 1C a sagittal section). A few months earlier, obstructive sleep apnea had been diagnosed. The history also included headache, enlargement of the feet, increased sweating, and joint stiffness. The patient's serum insulin-like growth factor level was elevated, at 56.5 nmol per liter (mean for age, 17.0 nmol per liter), and an oral glucose-tolerance test showed a failure to suppress growth hormone (5 mU per liter; normal value, <2 mU per liter).
Figure 1. Acromegaly in a 50-Year-Old Man.
Panel A shows the identical twins; the twin on the left has acromegaly caused by a growth hormone–hypersecreting pituitary macroadenoma. Panel B shows a coronal section and Panel C a sagittal section of the macroadenoma on magnetic resonance imaging.
The patient's coarse facial features were evident in comparison with the facial features of his identical twin. The acral enlargement (nose and lips) and enlargement of the facial soft tissue (skin folds and infraorbital puffiness) are striking. Few patients with acromegaly seek care owing to the very slow progression. The patient underwent transsphenoidal surgery, with visual-field improvement. Complete relief from symptoms was not attained, and treatment with a somatostatin analogue was started, which reduced the patient's headache, fatigue, and sweating.
Willy-Anne Nieuwlaat, M.D.
Gerlach Pieters, M.D., Ph.D.
University Medical Center Sint Radboud
6500 HB Nijmegen, the Netherlands
Editor's note: We received 2655 responses to this medical mystery: 57 percent from physicians in practice, 19 percent from physicians in training, 12 percent from medical students, and 12 percent from other readers. Responses were received from 86 countries. Sixty-four percent of respondents correctly diagnosed acromegaly in the twin in the white shirt; 23 percent suggested other endocrinopathies such as hypothyroidism, Hashimoto's thyroiditis, and Cushing's syndrome. Other, less common diagnoses included Paget's disease, the superior vena cava syndrome, lung cancer, chronic obstructive pulmonary disease, chronic renal failure, nephrosis, amyloidosis, Dilantin (phenytoin) toxicity, leprosy, scleroderma, myotonic dystrophy, Parkinson's disease, and hemochromatosis. Two percent of respondents suggested that the twin in the red shirt had Bell's palsy — he did not.
References
Nieuwlaat W-A, Pieters G. A medical mystery -- which twin is the patient? N Engl J Med 2004;351:68-68.
Figure 1. Acromegaly in a 50-Year-Old Man.
Panel A shows the identical twins; the twin on the left has acromegaly caused by a growth hormone–hypersecreting pituitary macroadenoma. Panel B shows a coronal section and Panel C a sagittal section of the macroadenoma on magnetic resonance imaging.
The patient's coarse facial features were evident in comparison with the facial features of his identical twin. The acral enlargement (nose and lips) and enlargement of the facial soft tissue (skin folds and infraorbital puffiness) are striking. Few patients with acromegaly seek care owing to the very slow progression. The patient underwent transsphenoidal surgery, with visual-field improvement. Complete relief from symptoms was not attained, and treatment with a somatostatin analogue was started, which reduced the patient's headache, fatigue, and sweating.
Willy-Anne Nieuwlaat, M.D.
Gerlach Pieters, M.D., Ph.D.
University Medical Center Sint Radboud
6500 HB Nijmegen, the Netherlands
Editor's note: We received 2655 responses to this medical mystery: 57 percent from physicians in practice, 19 percent from physicians in training, 12 percent from medical students, and 12 percent from other readers. Responses were received from 86 countries. Sixty-four percent of respondents correctly diagnosed acromegaly in the twin in the white shirt; 23 percent suggested other endocrinopathies such as hypothyroidism, Hashimoto's thyroiditis, and Cushing's syndrome. Other, less common diagnoses included Paget's disease, the superior vena cava syndrome, lung cancer, chronic obstructive pulmonary disease, chronic renal failure, nephrosis, amyloidosis, Dilantin (phenytoin) toxicity, leprosy, scleroderma, myotonic dystrophy, Parkinson's disease, and hemochromatosis. Two percent of respondents suggested that the twin in the red shirt had Bell's palsy — he did not.
References
Nieuwlaat W-A, Pieters G. A medical mystery -- which twin is the patient? N Engl J Med 2004;351:68-68.