The clinical and imaging spectrum of findings in patients with congenital lobar emphysema.

Congenital lobar emphysema (CLE) is an important cause of infantile respiratory distress that may require surgical intervention. We retrospectively reviewed the clinical and imaging findings in eight infants with CLE. In our series, CLE was more common in females, predominantly involved the right lung, often presented with an opaque lobe from retained fetal lung fluid, and frequently involved the lower lobes, multiple segments or lobes, and both lungs. Most patients with CLE were diagnosed and managed on the basis of clinical and plain radiographic findings alone. Computed tomography, and occasionally ventilation/perfusion scintigraphy, were helpful in confirming the diagnosis and in guiding management decisions in several cases; bronchoscopy showed that stenosis of the right mainstem bronchus was the cause of CLE in one case. Three patients experienced progressive worsening of respiratory distress and required surgical resection of the affected lobe for cure; the remaining five patients were managed medically with eventual remission of symptoms.     

The computed tomography mucous bronchogram sign

Mucous secretions filling the bronchial tree may be identified on computed tomography (CT) as low-density, treelike branching structures within consolidated or collapsed lungs similar to air bronchograms. These mucous bronchograms may occur from mucoid impaction of the bronchi distal to an obstructing lesion of the bronchus, most notably bronchogenic carcinoma, and from conditions that cause impaired mucociliary transport. In most cases, thin-section CT (5 mm) will demonstrate the presence or absence of an obstructing tumor involving the bronchus and will allow a correct assessment as to the cause of the mucous bronchograms; however, on occasion mucus within the central bronchi may result in a false-positive CT diagnosis of obstructing tumor. The demonstration of mucous bronchograms within a thoracic mass indicates that the mass is consolidated or collapsed pulmonary tissue and allows distinction from pleural disease.     

Fractures of the craniovertebral junction associated with other fractures of the spine: overlooked entity?

In a review of 155 craniovertebral fractures (occiput-C1-C2), 40 of these had associated fractures and/or dislocations or subluxations elsewhere in the spine. This rather common occurrence, one of four, has not been emphasized in the recent literature, indicating that the radiologic examination should not stop after the craniovertebral fracture is identified. Furthermore, in 13 patients, neurologic deficits were encountered that in all instances were from associated lower-level fracture. From this experience it was believed that a minimum of anteroposterior and lateral views of the entire spine should be obtained in patients in whom a craniovertebral fracture is found, especially if neurologic deficits are present. The other sites of injury were in the lower cervical spine in 17 patients, in the thoracic spine in five, in the lumbar spine in two, and in the sacrococcygeal spine in two patients. Eight patients had three or more levels of fracture.     

Mechanism-based combination telomerase inhibition therapy

Inhibition of telomerase is an exciting therapeutic target, since it is required for the long-term proliferation of most cancer cells but not present in most somatic cells. However, effective telomerase inhibitors have yet to be tested in clinical trials. In this issue of Cancer Cell, Seimiya and coworkers explore inhibiting tankyrase, an enzyme involved in making telomeres accessible to telomerase. Adding a partial inhibition of tankyrase to a partial inhibition of telomerase drove cancer cells into crisis and death. The combination of tankyrase and telomerase inhibitors may offer new opportunities for realizing the promise of telomerase inhibition therapy.     

Stage II-B carcinoma of the cervix complicated by bilateral pelvic kidneys

BACKGROUND: The simultaneous occurrence of advanced cervical cancer and a pelvic kidney is uncommonly reported. It is an interesting clinical problem because the pelvic kidney lies within the radiation field. CASE: A patient found to have bilateral pelvic kidneys and stage II-B cervical cancer underwent an anterior exenteration. This was complicated by urinary conduit leakage and eventual nephrectomy for hemorrhage. She has been followed for 14 months with normal renal function and no evidence of disease. CONCLUSION: A primary surgical approach to stage II-B cervical carcinoma was offered to this patient because of bilateral pelvic kidneys. Anastomotic breakdown with urinary leakage after an ileal conduit remains a significantly morbid problem.     

Development and testing of patient satisfaction measure for inpatient psychiatry care

Patient satisfaction is one of the most important indicators for service excellence. Investigations have been done with population-specific patient satisfaction tools for psychiatric patients; however, there are few published measures for evaluating inpatient care. We developed and tested a 15-item instrument to evaluate the interdisciplinary care model and therapeutic interventions. Results demonstrated reliability and validity of the tool.     

Spontaneous immortalization of clinically normal colon-derived fibroblasts from a familial adenomatous polyposis patient

Normal human diploid cells do not spontaneously immortalize in culture, but instead enter replicative senescence after a finite number of population doublings. Ablation of key checkpoint arrest or cancer-suppressor genes, through dominantly inherited germline mutation (p53+/-, Li-Fraumeni) or viral oncogene expression (SV40 large T, HPV16/18, and E6/E7) can lead to escape from senescence, additional doublings, and entrance into crisis phase, where immortal clones emerge at low frequency. In the vast majority of cases, telomerase is reactivated and telomeres are stabilized. Here we describe the spontaneous immortalization of clinically normal fibroblasts derived from colonic stroma of a familial adenomatous polyposis (FAP) patient. The preimmortal (C26C) and the spontaneously immortalized derivative (C26Ci) cells are heterozygous for a characterized germline mutation in exon 15 of the adenomatous polyposis coli gene. Immortalization was accompanied by spontaneous reactivation of endogenous telomerase and establishment of telomeres at presenescent lengths. Normal checkpoint behavior is retained and a diploid karyotype is maintained. These cells provide a valuable new addition to the limited number of spontaneously immortalized human cell types, particularly fibroblast cells, and will be useful in experimentally determining the functional pathways in neoplastic development and in the identification of potential molecular targets for cancer chemoprevention.