Primer caso clínico de Síndrome de Pai en México / First clinical case of Pai Syndrome in México

Descrito por primera vez en 1987, el Síndrome de Pai se considera una variante rara de la displasia fronto-nasal. Consiste en el fallo del cierre de la línea media y sus signos son encéfalo-cráneo-faciales. Los hallazgos que conforman el síndrome son: la presencia de una variedad de pólipos (intranasal, cutáneos y alveolar del maxilar superior), fisura ósea y labiopalatina en la línea media, lipoma intracraneal y agenesia parcial o total del cuerpo calloso. Su causa es desconocida y su presentación esporádica. La incidencia se estima en 1 de cada 20.000 a 40.000 recién nacidos, siendo el sexo femenino el más afectado. El objetivo de este artículo es presentar el primer caso clínico documentado en México con estas características, y de acuerdo al último caso publicado en 2014 por Mee Hong, es el número 38 de la literatura mundial (AU)

Described by the first time in 1987, Pai's Syndrome is considered a rare variant of the displasia fronto-nasal. It consists of the fault of the closing of the middle line and his signs are encephalo-craneo-facial. The findings of the syndrome are the presence of a variety of polyps (intranasal, cutaneous and alveolar of the upper jaw), bony and lip-palate cleft in the middle line, intracranial lipoma and partial or total agenesia of the corpus callosum. The etiology of this syndrome is not known, and its presentation is sporadic. The incidence is estimated in 1 of every 20.000-40.000 newborn children, being the most affected feminine sex. The aim of this article is to present the first clinical case reported in Mexico with these characteristics and that in agreement to the last case published in 2014 for Mee Hong, it is number 38 of the world literature (AU)

[The use of hypertonic sea-water solution in patients after surgery of the nose and paranasal sinuses]. / Zastosowanie hipertonicznego roztworu wody morskiej u chorych po leczeniu operacyjnym nosa i zatok przynosowych.

UNLABELLED: Surgical operations on the nose are very common otorhinolaryngological procedures. The surgical outcome depends not only on the performance of the procedure itself but also on the postoperative care of the nasal cavities. Clinicians continue their search for agents which would promote cleansing of the nasal cavities and regeneration of the nasal mucosa postoperatively. THE AIM OF THE STUDY: was a retrospective evaluation of the effect of the product Marimer Hypertonic on symptoms in patients who had undergone nasal surgery (septoplasty, functional endoscopic sinus surgery, surgical removal of nasal polyps). MATERIAL AND METHOD: The medical records included details of full history and physical examination on postoperative days 1, 7 and 14.The medical records of 120 patients were randomly selected from all records. The records were consecutively selected using the following criteria: 1-Operation type; septoplasty group A and B, FESS group C and D or classical surgical removal of nasal polyps group E and F , 2- Use (group B, D, F) or non-use (group A, C, E) of the hypertonic sea water solution aerosol for nasal douching (Marimer Hypertonic 2.2% sea-water solution). All patients included in the postoperative follow-up at the ENT Outpatient Clinic assess their symptoms by completing a standard self-assessment chart. The symptoms include nasal obstruction, discharge and crusting in the nasal cavities, pain in the nose and paranasal sinuses, reduction of smell, and general health (disease severity) and are assessed on day 1 after removal of nasal packing and on treatment days 7 and 14. At the same time, a clinician assesses their condition, including general health, nasal blockage, discharge and crusting in the nasal cavities, appearance of the nasal mucosa, and sense of smell evaluated by a scratch test using a smell book and a felt-tip pen test. RESULTS: A statistically significance difference in the severity of disease between group A (control) and group B (using Marimer Hypertonic) indicates a statistically significant beneficial effect of nasal douching with Marimer Hypertonic after septoplasty. As early as day 7 after surgery, the disease severity rated by the participants was reduced by 58% in patients usining Marimer Hypertonic compared to 25% in controls. On day 14, the reduction was by 84% and by 51% respectively. The use of Marimer Hypertonic after septoplasty produced a much faster relief of nasal obstruction/blockage as assessed by both patients and clinicians. In the opinion of patients using Mariner Hypertonic, on day 7 and day 14 nasal obstruction/blockage was reduced by 73% and by 87% respectively while in controls the corresponding values were 14% and 48%. Nasal douching with Marimer Hypertonic produced faster relief of discomfort caused by the accumulation of abnormal nasal discharge and crusting. In the assessment of patients from the control group, 14 days after surgery abnormal nasal discharge and crusting persisted at a level similar to that reported immediately after septoplasty. In the group using Marimer Hypertonic, the symptoms reported by patients subsided by 58% and 88% by day 7 and day 14 respectively. As assessed by clinicians using rhinoscopy and endoscopy, the improvement of nasal discharge and crusting was significantly faster and more effective in patients using Marimer Hypertonic (by 70% on day 7 and 95% on day 14) compared to controls (15% and 45% respectively). The use of Marimer Hypertonic after FESS produced a much faster relief of nasal obstruction/blockage as assessed by both patients and clinicians. In the opinion of patients using Mariner Hypertonic, on day 7 and day 14 nasal obstruction/blockage was reduced by 73% and by 87% respectively while in controls the corresponding values were 23% and 61%. Nasal douching with Marimer Hypertonic was found to produce an obvious statistically significant beneficial effect resulting in the relief of discomfort caused by crusting and accumulation of abnormal nasal discharge after FESS. By day 7 the symptoms reported by patients subsided by 78% in patients using Marimer Hypertonic and by 88% in controls. By day 14 the symptoms decreased by 93% and 47 % respectively. As assessed by clinicians using rhinoscopy and endoscopy, the improvement of nasal discharge and crusting was significantly faster and more effective in patients using Marimer Hypertonic (by 84% on day 7 and 95% on day 14) compared to controls (11% and 26% respectively). The use of Marimer Hypertonic after polypectomy produced a much faster relief of nasal obstruction/blockage as assessed by both patients and clinicians. On day 7 nasal obstruction/blockage was reduced by 73% in patients using Marimer Hypertonic and by 14% in controls. On day 14 the corresponding values were 89% and 50%. CONCLUSIONS: The findings of the present study support the investigators' opinion that the use of hypertonic sea-water solution, i.e. the product Marimer Hypertonic should be recommended for nasal douching after corrective nasal septal surgery (septoplasty), functional endoscopic sinus surgery (FESS) and surgical removal of nasal polyps (polypectomy). The use of Marimer Hypertonic facilitates maintenance of normal patency of the nasal cavities, reduces the amount of abnormal nasal discharge and crusting and speeds up healing of iatrogenic injuries of the mucous membrane of the nose.

[Rehabilitation therapy after nasal endoscopic surgery for nasal polyp and chronic sinusitis patients].

OBJECTIVE: To analyze the possible factors affecting the results of nasal endoscopic surgery in nasal polyp and chronic sinusitis patients and to discuss the ways to improve the results of operation. METHOD: Retrospective analysis was done in 226 patients followed up more than 3 year. The analysis was focused on patients' occupation, personal habits, extent of persisting on rehabilitation therapy after operation and clinical recovery status. RESULT: The patients who work in the environment rich of irritating substance and dust,the patients having unhealthy habits (e. g: spicy food and alcohol addicting) and the patients who did not take or insist on rehabilitation therapy after operation (e. g: local and systemic medication, nasal lavage and cleaning up of surgical cavities under nasal endoscope) had higher frequency of relapse and quicker recurrence. Rehabilitation therapy was the most important factor affecting the results of operation. CONCLUSION: Insisting on systemic rehabilitation therapy after operation is a occupation protection and kicking the unhealthy personal habits can prevent and reduce the recurrence of nasal polyp and chronic sinusitis after nasal endoscopic surgery.

[The efficacy of subterranean therapy in the salt chamber of Kinga Spa in Wieliczka for patients suffering from allergic rhinitis]. / Skutecznosc leczenia subterraneoterapia alergicznego niezytu nosa w komorach solnych Szpitala Uzdrowiskowego Kinga w Wieliczce.

42 patients (27 women and 15 men aged 14-65 years) suffering from atopic and non atopic rhinitis were submitted for 24 days to the subterraneo-therapy in salt chambers of Kinga Spa in Wieliczka during the exacerbation of their illness. 20 patients suffered from pollen or dust mites allergy and the next 22 patients from non atopic rhinitis with recurrent nasal polyposis. In the first week and at the end of the therapy rhinoscopy, peak nasal inspiratory flow and smell were evaluated as well as daily symptoms score was noted in each treated patient. The results of performed observations indicate that unrelatively to the type of the rhinitis: 1. the nasal symptoms (running nose, sneezing, block of the nose) resolved directly during the stay of the patient in the salt chamber but the more constant effect was visible with the time of the therapy. 2. an increase in the peak nasal expiratory flow was connected with the decrease of nasal mucosal edema, 3. the smell disturbances disappeared in the patients together with the decrease of mucosal edema. The observed changes seem to be connected with the influence of the salt chambers' microclimate on the cells osmolarity disturbances in the nasal mucosa provoked by the non infectious chronic inflammation.