Atypical pulmonary carcinoid tumour in a 28-year-old nonsmoker with Prader-Willi syndrome
| Autor: | V. Anagnostakou, A. Kokkori, M. Argiriou, I. Nenekidis, J. Kokotsakis, Georgios T. Stathopoulos, Charalambos Zisis, P. Dedeilias |
|---|---|
| Rok vydání: | 2011 |
| Předmět: |
Pulmonary and Respiratory Medicine
Anamnesis medicine.medical_specialty medicine.diagnostic_test business.industry medicine.disease Gastroenterology Obesity Surgery Pulmonary carcinoid tumour Polyphagia Blood pressure Internal medicine Wheeze medicine medicine.symptom Chest radiograph business Body mass index |
| Zdroj: | European Respiratory Journal. 38:1230-1233 |
| ISSN: | 1399-3003 0903-1936 |
| DOI: | 10.1183/09031936.00034711 |
| Popis: | To the Editors: In recent years, the life expectancy of patients with Prader–Willi syndrome (PWS) has increased, unveiling several cases of early-onset cancer and raising the question whether the condition predisposes to tumourigenesis. We encountered a unique occurrence of pulmonary atypical carcinoid in a young never-smoker with PWS. A 28-yr-old, morbidly obese male with PWS was admitted as a candidate for gastric bypass. He was a lifetime nonsmoker, and his height, weight and body mass index were 1.78 m, 175 kg, and 55.2 kg·m−2, respectively. Polyphagia, obesity and a failure to achieve psychosocial milestones became apparent at the age of 5 yrs, when PWS was diagnosed based on clinical findings and a de novo deletion of the proximal region of chromosome 15 (del(15)(q11–q13)). Type II diabetes mellitus was diagnosed at the age of 17 yrs, and dietary control along with administration of sulfonylurea was initiated. The remainder of the anamnesis included low levels of testosterone, which were treated with monthly intramuscular depot injections of testosterone enanthate (250 mg). On admission, the patient reported mild shortness of breath, nonproductive cough and malaise of indeterminate origin. A physical exam revealed markedly increased subcutaneous fat, picking scars, hypoplastic external genitalia and a polyphonic wheeze over the right hemithorax. Pulse was regular and of normal rate, diastolic blood pressure was elevated (98 mmHg) and haemoglobin oxygen saturation was 96% in room air. Electrocardiography was normal. Laboratory data, including blood cell counts, routine serum biochemistry and coagulation times, were within normal limits, except for fasting plasma glucose (178 mg·dL−1) and lipids (total cholesterol 233 mg·dL−1; high-density lipoprotein cholesterol 32 mg·dL−1; triglycerides 322 mg·dL−1). A pre-operative chest radiograph, performed routinely but also prompted by the patient’s wheeze, showed a round, regular-shaped opacity of soft-tissue quality measuring … |
| Databáze: | OpenAIRE |
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