Ataxia in children: think about vitamin E deficiency ! (comment on: ataxia in children: early recognition and clinical evaluation).

A recent article from Pavone et al. published in the Italian Journal of Pediatrics entitled «Ataxia in children: early recognition and clinical evaluation¼ made an exhaustive overview of the large spectrum of pediatric ataxias. However, we would underline the importance of considering hereditary ataxia due to isolated vitamin E deficiency as a specific and treatable cause of child ataxia. We present a short clinical and therpeutic synopsis of this peculiar genetic etiology, frequently encountred in the mediterranean region.

Pan-European survey of the topical ocular use of cyclosporine A.

OBJECTIVE: To assess medical practices surrounding the use of topical ocular cyclosporine A across European Union nations. METHODS: Key stakeholders (ophthalmologists, hospital pharmacists, regulatory health authorities) from European Union member states were interviewed by telephone using a semi-structured, open-ended questionnaire. Ophthalmologists responded to questions about practice patterns of cyclosporine A use (prescription frequency, indication, dosage), pharmacists about cyclosporine A formulations (composition, manufacturing process, quality control, distribution), and the regulatory authorities about market authorization and pharmacovigilance for various cyclosporine A products. RESULTS: Over the years, cyclosporine A use for ophthalmic indications has increased across all European Union nations. Prevalence of cyclosporine A use was heterogeneous, with Belgium, France, Germany, Italy, Portugal, Spain and the United Kingdom reporting the highest frequency. Compounded cyclosporine A formulations and other cyclosporine A products were prescribed through temporary authorization on a compassionate use or named-patient basis. Cyclosporine A was prescribed for dry eye disease, atopic and vernal keratoconjunctivitis, corneal graft rejection, and other autoimmune and inflammatory diseases. Concentrations of prescribed topical cyclosporine A ranged between 0.05-2% and formulations were instilled 1-6 times daily. Interviewed stakeholders expressed concern regarding, (1) paucity of product information, (2) lack of standardized manufacturing processes and quality control of cyclosporine A formulations, and (3) poor regulation and pharmacovigilance of ocular cyclosporine A-based products. CONCLUSIONS: Medical practice surrounding ocular cyclosporine A use in European Union nations differs based on variations in concentration, dosage, prescription indication, formulation, availability and distribution, manufacturing, quality, and regulatory monitoring.

Ocular tolerability and efficacy of a cationic emulsion in patients with mild to moderate dry eye disease - a randomised comparative study.

PURPOSE: The purpose of this study was to compare the safety and efficacy of a new cationic emulsion (CE) with a formulation of polyvinyl alcohol and povidone (PVA-P) for the treatment of mild to moderate dry eye disease. METHODS: This was a multicenter, open-label, comparative study. Patients were randomised to receive CE (Cationorm) or PVA-P (Refresh) (1:1). The following objective criteria were assessed to compare the two eye drops: tear Break-up Time (TBUT), Schirmer's test, lissamine green staining (Van Bijsterveld score), corneal fluorescein staining (Oxford scale) and oculopalpebral examination, on D7 and D28 (end of study). At these visits, ocular symptoms and safety were also assessed. RESULTS: Seventy-nine patients were randomised: CE: 44 patients; PVA-P: 35 patients. At D28, improvement was significantly better for TBUT [CE: 1.7 ± 2.4 s; PVA-P: 0.6 ± 1.8 s; P=0.015] and for the Van Bijsterveld score [CE: -1.4 ± 1.2; PVA-P: -0.9 ± 1.2; P=0.046] in the CE group. The same applied for the palpebral erythema score (P=0.023), overall efficacy assessed by the investigators (P<0.001), and symptoms not related to eye drop instillation (P=0.021). Improvement was observed from D7. No difference was observed between the two treatments with regard to ocular safety. CONCLUSION: These results suggest that in patients with mild to moderate dry eye, Cationorm, in addition to its moisturizing and lubricating properties, also helps stabilize the tear film due to its oily component. This study demonstrates the benefit of this new pharmaceutical form for the treatment of mild to moderate dry eye disease.

Attached and unattached fractions of short-lived radon decay products in outdoor environments: effect on the human respiratory system.

The authors developed a model for determining the alpha- and beta-activities per unit volume of air due to radon ((222)Rn), thoron ((220)Rn) and their decay products attached and unattached to the aerosol in the outdoor air at the workplace in natural conditions at different locations in Morocco by using both CR-39 and LR-115 type II solid-state nuclear track detectors. In addition, the percentage of (218)Po, (214)Pb and (214)Po radionuclides attached to the aerosols and the unattached fraction f(j) for different values of the attachment rate were evaluated. Radon and thoron concentrations in outdoor air of the studied different locations were found to vary from 9.20±0.8 to 16.30±1.50 Bq m(-3) and 0.22±0.02 to 1.80±0.20 Bq m(-3), respectively. The committed equivalent doses due to the radon short-lived progeny (218)Po and (214)Po attached and unattached to the aerosol air were evaluated in different tissues of the respiratory tract of the members of the public from the inhalation of outdoor air.

Concentrations of 222Rn, 220Rn and their decay products measured in outdoor air in various rural zones (Morocco) by using solid-state nuclear track detectors and resulting radiation dose to the rural populations.

Alpha and beta activities per unit volume of air due to radon ((222)Rn), thoron ((220)Rn) and their progenies were measured in the outdoor air at different locations in Morocco by using both CR-39 and LR-115 type II solid-state nuclear track detectors (SSNTDs). In addition, the radon concentration was continuously measured in one location by using the methods with SSNTDs and AlphaGuard counter. The influence of the geological and meteorological conditions as well as phosphate and building material dust on the radon concentration in the outdoor air of the areas studied was investigated. The committed equivalent doses due to (218)Po and (214)Po radon short-lived progeny were evaluated in different tissues of the respiratory tract of the members of the public from the inhalation of outdoor air. The annual effective dose due to radon short-lived progeny from the inhalation of outdoor air by the members of the rural population was estimated.

[Operative risk factors and polyvalvular cardiac surgery]. / Facteurs de risque opératoire en chirurgie cardiaque polyvalvulaire.

The aim of this study was to identify risk factors for morbidity and mortality in cardiac valvular surgery. Two hundred and fifty-four patients underwent polyvalvular surgery between May 1996 and December 1998. The mean age was 36.8 years (range 4-66 years) and the group comprised 148 women. Two subgroups were defined: the first (184 patients), characterised by associated mitral and tricuspid valve disease: the second (70 patients), characterised by associated mitral and aortic valve disease with a tricuspid lesion in about half the cases. The risk factors of morbidity and mortality analysed were: Clinical: previous history of cardiac failure, NYHA classification, atrial fibrillation and cardiothoracic index. Doppler echocardiographic: left ventricular end systolic dimension > 50 mm, left ventricular and diastolic dimension > 70 mm, fractional shortening < 28%, ejection fraction < 40%: systolic pulmonary artery pressure > 30 mmHg. Haemodynamic: capillary wedge pressure > 20 mmHg, systolic pulmonary artery pressure > 30 mmHg. The authors identified three statistically significant risk factors for operative morbidity and mortality in polyvalvular cardiac surgery: Previous history of one or more episodes of cardiac failure NYHA functional Classes III or IV fractional shortening < 28%. The morbidity and mortality were lower when patients were operated before these poor prognostic factors were observed.

Arterial complications of the thoracic outlet syndrome: fifty-five operative cases.

Between January 1, 1969, and December 31, 1984, 55 operative procedures were carried out in 47 patients to correct subclavian-axillary artery lesions resulting from compression at the thoracic outlet. The most common causes of compression were a long cervical rib (27) and an anomalous first rib (15). Presenting features included claudication, vasomotor phenomena, digital gangrene, and acute limb-threatening ischemia. A combined supraclavicular and infraclavicular approach was preferred. Decompression was best achieved by excision of the cervical rib and the first rib and division of all soft tissue elements. The most common methods of arterial repair were resection-anastomosis (23) and replacement of vein graft (11). Embolic occlusions were frequently present (35). Axillary emboli were amenable to direct revascularization at the time of subclavian artery repair. If possible, more-distal embolic occlusions were managed without recourse to embolectomy catheter manipulations. The mean follow-up was 5 years 8 months (range 4 months to 16 years). Patients were assessed clinically, and the arterial repair was monitored by Doppler ultrasonography, B-mode scanning, and digital subtraction angiography. Of the 39 patients available for follow-up, 35 had no symptoms and four had residual claudication. There were no amputations. In the remaining cases the subclavian-axillary artery segment showed no hemodynamic or anatomic abnormality.

[Interruption of the vena cava inferior. A comparative study of the Adams de Weese clip and the Kimray Greenfield filter]. / Interruption de la veine cave inférieure. Etude comparée du clip d'Adams de Weese et du filtre de Kimray Greenfield.

Efficacy of two methods for interruption of inferior vena cava was compared after insertion of 95 Adams de Weese clips by the sub or trans-peritoneal route and of 72 Greenfield filters usually by a jugular approach. Introducing Greenfield's filter to produce caval interruption did not require modification of operative indications. In contrast, it allowed caval blockade to be performed in more elderly patients, generally in a poorer condition, at the price of higher mortality, due more to the clinical circumstances of the interruption rather than the caval blockade itself. Postoperative follow up showed fewer caval thromboses after Greenfield's filter (15%) than after the pericaval clip (35%).

[Talo-calcaneal continuity. Myth or reality?]. / Le couple talo-calcanéen. Mythe ou réalité?

On the basis of two separate biomechanical studies of talus and calcaneus, the authors show: the absolute continuity of the trabecular structure of these bones, the mechanical behaviour characterised as "fragile" for the talus and "intermediate" for the calcaneus, superficial strains underload: by photoelastic studies, they are show to be in reversed phases both on their lateral and medial sides. Authors conclude that these two bones act together as sectional across which collect dispach and absorb transmitted forces during gate.

[Anatomic and histologic study of the human ileocecal valve. Developmental aspects as a function of age]. / Etude anatomique et histologique de la valve ileo-caecale humaine. Aspects évolutifs en fonction de l'âge.

Based on gross anatomy and histological studies of thirty specimens ranging from nineteen weeks of gestational age to eighty-two years old, the authors present an analytical study of the ileocecal valve. The results confirm the evolutive aspect of the ileocecal valve with age, but, however seems to demonstrate a functional regression correlated with the frequency of its incontinence in adulthood.

[Method for studying internal tensions at the intervertebral junction in extreme postures. Description of experimental material and preliminary results]. / Méthode d'étude des contraintes internes des éléments de la jonction intervertébrale en postures extrêmes. Description du matériel expérimiental et résultats préliminaires.

The authors describe a device for measuring the resultant tensions existing at the intervertebral junction (or its constitutive elements) in all spine attitudes. The reported method has been applied to the L4-L5 junction of a spine taken from the corpse of a 50 years old man. Although incomplete, the results suggest the following points: --the L4-L5 intervertebral junction seems to be very deformable, particularly in the sagittal plane; --the intervertebral disk is probably an elastic support which sustains shear stresses during flexion, extension and bending movements in the frontal plane; --during flexion, articular processes seem to be movement leaders as well as supports; --ligaments seem to play a small mechanical role, but their proprioceptive function seems essential.

[Study of the talus and calcaneus using microdurometry, penetrometry and compression]. / Etude du talus et du calcaneus par microdureté, pénétrométrie et compression.

The authors give the results of various methods such as hardness micrometry, indentation tests and the radiographic study of fracture patterns, which they have applied to the talus and to the calcaneus. It appeared to them that: --these techniques outline the phenomenon of rupture behaviour of both talus and calcaneus; --the talus behaves as a fragile type of material whereas the calcaneus is of the intermediate type. The authors therefore think that these methods can be of great interest in a better understanding of the biomechanical properties of bone.