Craniofacial structures and dental development in three patients with Nager syndrome

In Finland, 3 patients have been diagnosed with Nager syndrome (NS) during the last 17 years. Thus the incidence for NS in Finland is 3:1,000,000. The craniofacial structures and dental development of these patients were studied clinically and radiographically at the age of 3-4 years, and compared to age-matched controls and to the norms of the Finnish population. The striking structural finding was a severely short, retrognathic and posteriorly rotated mandible. Especially the ramus was deficient; its height was, on average, less than one-third of that of the control group. All children were tracheostomized neonatally. At the age of 3-4, the lower pharyngeal airway was still severely obstructed or completely closed. Nasopharyngeal airway was wide and the soft palate was missing in all patients. All patients had a complete deciduous dentition, but agenesis of permanent teeth (ranging from 2-10 missing teeth) was observed in each patient. Accelerated dental development was found in two subjects. Condylar ankylosis or severely limited mouth opening were observed.The present findings give new information and quantify earlier observations of craniofacial structures and dental development in NS. Analysis of facial structures suggests that if surgical intervention is needed to enable better breathing, the goal of the structural correction should be aimed at the most deficient structure, namely the ramus height. As a result of severe dentofacial deviation, a treatment process through the growth requires multidisciplinary teamwork of surgeons, pediatrists, orthodontists and prosthodontists.     

Hospitalized children's descriptions of their experiences with postsurgical pain relieving methods

The purpose of this study was to describe children's (aged 8-12 yr) experiences with postsurgical pain relieving methods, and their suggestions to nurses and parents concerning the implementation of pain relief measures in the hospital. The data were collected by interviewing children (N = 52) who were inpatients on a pediatric surgical ward in the university hospital of Finland. Content analysis was used to analyze the data. The children rated the intensity of pain on a visual analogue scale. The results indicated that all of the children used at least one self-initiated pain relieving method (e.g. distraction, resting/sleeping), in addition to receiving assistance in pain relief from nurses (e.g. giving pain killers, helping with daily activities) and parents (e.g. distraction, presence). The children also provided suggestions, especially as it relates to nurses (e.g. creating a more comfortable environment), regarding the implementation of effective surgical pain relief. However, some cognitive-behavioral and physical methods were identified that should be implemented more frequently in clinical practice. Furthermore, most children reported their worst pain to be severe or moderate, which indicates that pain management in hospitalized children should be more aggressive.     

Total laryngopharyngectomy with circumferential reconstruction: Helsinki institutional study

European Archives of Oto-Rhino-Laryngology - Surgical complications after total laryngopharyngectomy (TLP) are common, reconstruction is challenging, and patients often lose their ability to...     

The Importance of Measuring Competency-Based Outcomes: Standard Evaluation Measures Are Not Surrogates for Clinical Performance of Internal Medicine Residents

Background: Despite recent emphasis on educational outcomes, program directors still rely on standard evaluation techniques such as tests of knowledge and subjective ratings. Purposes: To assess the correlation of standard internal medicine (IM) residency evaluation scores (attending global evaluations, In-Training examination, and Mini-Clinical Examination Exercise) with documented performance of preventive measures for continuity clinic patients. Methods: Cross-sectional study of 132 IM residents attending an IM teaching clinic, July 2000 to June 2003, comparing standard evaluations with chart audit. Results: Mean resident performance ranged from 53% (SD = 24) through 89% (SD = 20) across the 6 preventive measures abstracted from 1,102 patient charts. We found weak and mostly not significant correlations between standard measures and performance of preventive services. Conclusions: Standard measures are not adequate surrogates for measuring clinical outcomes. This supports the Accreditation Council for Graduate Medical Education's recommendations to incorporate novel Toolbox measures, like chart audit, into residency evaluations.     

Incidence and prevalence of diagnosed coeliac disease in Finland: Results of effective case finding in adults

Objective. The majority of patients with coeliac disease remain undetected world-wide. Finland has aimed at a high detection rate by training health personnel, and has advocated serologic screening for known coeliac disease risk groups. The purpose here was to assess whether this approach has been effective in clinical practice. Material and methods. The study was based on a prospective database of new coeliac patients aged 16 years or more, to whom the Social Insurance Institution had paid monthly compensation for the additional cost of maintaining a gluten-free diet since 2002. To obtain this compensation the diagnostic criteria for coeliac disease, including biopsy findings, had to be attested in a statement from a physician. The incidence and prevalence rates were calculated until the end of 2006. The total population aged 16 years or more was 4.31 million. Results. In 2004–06, a total of 5020 persons (64% female) receiving a new dietary grant were identified through the database. The mean annual incidence of proven coeliac disease was thus 39 per 100,000 individuals. Altogether, 23,553 persons received the dietary grant. This gives a nation-wide point prevalence of adult coeliac disease of 0.55% (0.70% F, 0.38% M). There was a regional variation from 33 to 49 per 100,000 in the annual incidence and from 0.41% to 0.72% in the prevalence of coeliac disease. Conclusions. To the best of our knowledge, these figures for proven coeliac disease are the highest reported. Increased alertness to the condition and active case finding has made this efficient diagnostics possible.