Evaluation of chronic rhinosinusitis management using the SNOT-22 in adult cystic fibrosis patients.

OBJECTIVES: Sinonasal disease (SND) is a common finding in subjects with Cystic Fibrosis (CF). Even though it does not play a key role in the life expectancy of these patients, it increases the morbidity rate of CF, with deterioration of the quality of life and of general health status. The aim of this study is the evaluation of the impact of sinonasal disease and Extensive Endoscopic Sinus Surgery (eESS) on the quality of life (QOL) of CF patients by means of the 22 item Sino-Nasal Outcome Test (SNOT-22) and define if there is a correlation between SNOT-22 and the Lund-Mackay score. PATIENTS AND METHODS: We performed a prospective study in 33 CF patients with medically refractory SND. The extent of polyposis was graded both endoscopically and on computed tomography images in accordance with the Lund-Mackay score. All patients underwent eESS. The SNOT was administered in order to assess the quality of life related to SND both before surgery and during follow-up at 6 months, 1 year and 2 years postoperatively. RESULTS: The SNOT-22 and Lund-Mackay score showed a remarkable mean improvement during follow-up. The differences between the initial examination and last score were significant (p < 0.001), but there was no statistic correlation between SNOT-22 and Lund-Mackay. CONCLUSIONS: More attention should be paid to sinonasal symptoms in CF patients and SNOT-22 is a simple and effective tool for this purpose. This score is also important for indicating the outcome of the eESS procedure. In all patients SNOT showed a significant improvement in QoL.

Cervical lymphadenopathies signaling thyroid microcarcinoma. Case study and review of the literature.

BACKGROUND: Some lateral cervical lymphadenopathies may lead to the discovery of papillary microcarcinomas (PMC) of the thyroid that are not radiologically apparent. This relatively rare clinical situation raises questions about the diagnostic approach to chronic cervical lymphadenopathy and the impact of lymph node metastasis on PMC prognosis. PURPOSE OF THE ARTICLE: To study the epidemiologic, clinical, and prognostic criteria of cases of lymphadenopathy that signaled PMC. PATIENTS AND METHODS: A retrospective study of 167 consecutive cases of PMC compared with 13 cases where a cervical mass signaled other forms of PMC. RESULTS: The mean age was 48.5 years, the ratio of men to women was 5:8, and the mean PMC size was 5.5mm. These data did not differently significantly from those of the other PMC cases. The preoperative imaging found fluid content in six cases, with microcalcifications in three cases. All cases were treated by modified radical neck dissection on the side with the lymphadenopathy and total thyroidectomy with central neck dissection. The lymphadenopathy included a ruptured capsule in five cases and was accompanied by central lymph node metastases in three cases. Thyroid capsule involvement was significantly more common in cases of PMC discovered due to lymphadenopathy than in other cases of PMC (69% versus 9.7%, respectively; p<0.001). The mean follow-up was 7.3 years. There were no deaths due to PMC signaled by lymphadenopathy. Two cases of lymph node recurrence after 8 and 10 years were controlled by another surgery and radioactive iodine treatment. CONCLUSION: Any chronic cervical mass should suggest the possibility of thyroid origin, especially in cases with cystic content or microcalcifications in subjects with no particular risk factors. An ultrasound of the thyroid should be done, as well as a fine needle aspiration biopsy of the lymphadenopathy with a thyroglobulin assay. Treatment is the same as for any thyroid carcinoma, and results in a good oncological outcome, despite the possibility of lymph node recurrences.

Growth and pubertal disorders in neurofibromatosis type 1.

The first textbook of Pediatric Endocrinology in the early 1950s reported an association of neurofibromatosis type 1 (NF1) and precocious puberty (PP) and/or short stature. Recent studies have indicated that children with NF1 grow normally until puberty; thereafter height velocity and relative height (SDS or percentiles) decreases with respect to healthy peers, reaching a mean adult height close to the 25th percentile for the general population. Moreover, the percentage of patients with true short stature (<3rd percentile) increases from childhood (5%) to late puberty (20-30% in literature, 18% in our study), and final height is significantly below the genetic target and predicted adult height calculated just before or at the beginning of puberty. Finally, among the shortest patients (<10th percentile) there is a high incidence of severe complications, such as CNS tumors, huge plexiform neurofibromas and severe scoliosis. Precocious puberty is a frequent complication of NF1, and occurs mainly in association with optic pathway tumors (OPT); however, occasionally it has been reported in the absence of optic gliomas, probably with a similar incidence as in the general population. GnRH agonist therapy must be decided individually as in some patients further growth could be normal and/or treatment would not improve final height. In the presence of early pubertal signs, an OPT must be ruled out. In addition to PP, delayed puberty has been frequently reported in NF1. In a study of 123 girls with NF1, we found that the mean age at menarche (13.0 +/- 1.9 yr) was later than in their mothers (12.7 +/- 1.4 yr) and in the general population (12.4 +/- 1.2 yr; p <0.05), with a very high incidence of delayed menarche (>2 SD): 16% vs 6.8% (mothers) vs 3.4% (controls) (p <0.01). In conclusion, growth and puberty present unusual patterns in NF1, often with true pathological findings increasing medical and psychological problems.