Craniopharyngiomas

Craniopharyngiomas are rare benign epithelial tumors, arising from the pituitary stalk or gland and developing in the sellar and suprasellar region, affecting both adults and children. Incidence is 0.5 to 2 new cases per million inhabitants. Clinical features essentially include visual disturbances, endocrine deficiencies, and neurological signs. Initial signs are often visual loss and increased intracranial pressure in children, growth and pubertal delay in teenagers, visual disturbances or cognitive impairment in adults. Diagnosis is made on MRI and CT scan, demonstrating a sellar or suprasellar tumor, heterogeneous, with frequent calcifications. Craniopharyngiomas can be classified depending on their locations from the sella, the diaphragma sellae, and upon their origins from the pituitary stalk or the infundibulum. They can also be classified depending on the location from the optic chiasm and the third ventricle. This classification allows surgical series comparison, which is of importance since developments and extensions of the tumor can explain surgical difficulties. The management of this lesion is still controversial. Because it is an extra-cerebral benign lesion, the ideal goal of treatment should be complete tumor removal with improvement of altered visual functions, minimal deterioration of endocrine function, and no neuropsychological impairment. But the situation of the tumor, its relationship with third ventricle, hypothalamus, optic tract, vascular structures make its removal often difficult. However, great progresses have been realized in surgical treatment, resulting in a dramatic improvement of the prognosis of craniopharyngiomas. Nowadays, one can expect total removal in 60 to 70% of patients, subtotal removal in 20 to 30%, and partial removal in 10%. When total removal is impossible, radiotherapy may reduce the risk of a poor evolution. Recurrences are a problem in 15% of patients with total removal, 35% in subtotal removal, 70% in partial removal. If radiotherapy has not been performed as first treatment, it is efficient in 80% of recurrences. Long term follow up is necessary in these patients, due to medical management of endocrine, visual and psychological problems, and risk of late recurrence. With close involvement in this management, most of patients may enjoy a good outcome, 80% being able to return to normal active life.     

A survey of specialist paediatric dental services in Sweden: results from 2003, and trends since 1983

The child population in Sweden has changed dramatically during the last 20 years. Changes have also occurred within the Public Dental Service (PDS), regarding the provision of dental care to children and adolescents. All these changes may affect the referral pattern and provision of specialist dental care for children and adolescents. OBJECTIVES: The primary aim of this study was to survey the services provided by specialists in paediatric dentistry in Sweden during 2003. A secondary aim was to compare the results with previous surveys. METHODS: A Web-based survey was sent to all 34 specialist paediatric dentistry clinics and was answered by all clinics. Data were compared with results from the surveys performed in 1983, 1989, and 1996. RESULTS: The number of paediatric dentists had been relatively constant over the last 20 years, whereas the number of children referred to paediatric dentists had increased by 28% since 1983. It was estimated that 1.3% of all children in Sweden are treated at a specialist paediatric dental clinic in 2003. Dental treatment need in combination with behaviour management problems (BMP) was the main reason for referral and occurred in 37% of all referrals. The proportion of medically compromised children/children with disabilities had increased from 6% in 1983 to 22% in 2003. The number of patients treated using sedation and general anaesthesia had increased since 1983, and particularly since 1996. CONCLUSIONS: Despite improvements in dental health among children and adolescents in Sweden during the last 20 years, an increasing number of children are referred for specialist paediatric dental treatment. There is an urgent need to increase the number of specialist paediatric dentists in Sweden in order to ensure the continuation of high quality of dental care for children and adolescents.     

Paroxysmal hemicrania in a family

Paroxysmal hemicrania (PH) is a trigeminal autonomic cephalgia, characterised by unilateral attacks responsive to indomethacin. There are no published reports of a family history in PH. We report a mother and daughter both with PH. The daughter and her sister also had migraine.     

Asthma onset prior to multiple sclerosis and the contribution of sibling exposure in early life

Higher sibling exposure is associated with a reduced risk of asthma and other T helper 2 (Th2)-type disorders, possibly through a beneficial effect of higher infection load. The effect on Th1 disorders such as multiple sclerosis (MS) is less clear. Here we examine the association between asthma and MS, taking into account early life sibling exposure. A population-based case-control study in Tasmania, Australia based on 136 cases of magnetic resonance imaging (MRI)-confirmed MS and 272 community controls, matched on sex and year of birth. Study measures include cumulative exposure to total, older or younger siblings by age 6 years, history of doctor-diagnosed asthma and serological IgG responses to herpes viruses. MS cases were more likely (P = 0.02) than controls to have asthma which began before age of onset of MS symptoms compared to the corresponding age for controls. The absence of younger sibling exposure by age 6 years potentiated (P = 0.04) the association between asthma and MS. Compared to those with younger sibling exposure and no asthma, the adjusted odds ratio for MS for those with asthma and no younger sibling exposure was 7.22 (95% CI: 2.52, 20.65). Early life sibling exposure was associated with altered IgG serological responses to Epstein-Barr virus (EBV) and herpes simplex virus 1 (HSV1) in adulthood. Reduced early life sibling exposure appeared to contribute to the excess of asthma among MS cases by the time of MS onset. MS development may reflect factors that relate to a general immuno-inflammatory up-regulation of immune activity as well as disease specific factors. The link between early life sibling exposure and the immune response to herpes group viral antigens is consistent with a protective role for early life infections.     

Is pure red cell aplasia a new extra digestive manifestation of celiac disease?

INTRODUCTION: Celiac disease is an autoimmune enteropathy that appears on a predisposed genetic background. Its clinical presentation has been extended those last years by varied extradigestive manifestations. EXEGESIS: This is a case report of a twenty-year-old woman who presented simultaneously a celiac disease and pure red cell aplasia. The IgA and IgG antigliadin antibodies as well as antinuclear, anti-DNA, anti-SSA and anti-SSB antibodies were detected, without any clinical symptom supporting the diagnosis of systemic lupus erythematosus or Sj?gren syndrome. Thoracic CT-scan reveals a thymus enlargement and biopsy concludes to thymus hyperplasia at histological examination. Pure red cell aplasia regressed after gluten-free diet, corticoid therapy and thymectomy. CONCLUSION: This observation, added to the three others paediatric cases previously published in the literature concern an uncommon association between pure red cell aplasia and celiac disease. However, they are not enough to conclude to a direct link between these two disorders. Pure red cell aplasia could represent un new dysimmune manifestation occurring in celiac disease, but this will need to be confirmed with others cases.     

Treatment delay and the risk of prolonged status epilepticus

Factors contributing to the duration of a single convulsive seizure > 5 minutes were analyzed in 157 children. The medically treated episodes were compared with seizure episodes resolving without treatment (n = 27). Major differences were in age (p = 0.016) and etiology (p = 0.003), and the association between treatment delay and response became significant after 30 minutes when this was analyzed as a single variable (p = 0.003) in Cox regression analysis.     

Validity and reproducibility of a computerised tool for assessing the iron, calcium and vitamin C intake of Belgian women

OBJECTIVE: To determine the relative validity of a newly developed iron intake assessment tool, designed specifically to assess iron, calcium and vitamin C intake. DESIGN: Estimates of iron, calcium and vitamin C intake from a computerised iron intake assessment tool compared with those from 11-day estimated dietary records. SETTING: Region of Ghent (N= +/- 225,000), a city in Flanders, the Dutch-speaking part of Belgium. SUBJECTS: In all, 50 women aged 18-39 y, participating in a large-scale epidemiological study on iron intake and iron status. MAIN RESULTS: Mean dietary iron intake from the 11-day food record, the unadjusted dietary iron intake assessment tool and the adjusted tool was, respectively, 10.5 +/- 2.7, 10.4 +/- 4.3 and 9.6 +/- 2.9 mg. For the different nutrients, the correlation coefficients vary from 0.45 to 0.60 for adjusted intake. The mean difference of iron intake by the two methods (0.8 +/- 2.9 mg) did not differ significantly from zero. The new method correctly classified 38% (iron), 38% (calcium) and 58% (vitamin C) of the subjects to the correct tertile. The correlation coefficients ranged from 0.48 for adjusted vitamin C intake to 0.73 for adjusted calcium intake between two administrations. CONCLUSION: The newly developed instrument can be used to assess mean group intakes of iron, calcium and vitamin C in women consuming a Western diet. However, since the ranking capability of the new tool is rather weak, further refinement of the tool is required to produce a robust method for assessing iron, calcium and vitamin C intakes of individuals. SPONSORSHIP: This research was supported by the Belgian National Fund for Scientific Research/Flemish Division (Fund No G.0152.01).