Polysyndactyly and trigonocephaly with partial agenesis of corpus callosum.

Several malformation syndromes which include polysyndactyly and craniofacial anomalies have been described. We report a case of an 11-month-old boy with a pattern of anomalies including polysyndactyly, trigonocephaly, partial agenesis of corpus callosum and dysmorphic facies.

[Pseudo-occlusions of the internal carotid artery]. / Le pseudo-occlusioni della carotide interna.

The pseudo-occlusion (P.O.) of the internal carotid artery is defined as an atheromatous lesion causing a high-grade stenosis, which describes a peculiar angiographic finding ("string sing" or "slim sign"). The authors report their experience with 6 P.O. (angiographically diagnosed) that had been found in 16 months. In all these cases, whenever there was a clinical e/or B-scan ultrasound suspect, angiographic recommendations for the detection of that lesion have been applied. One of these 6 patients, clinically asymptomatic, refused surgery, remaining asymptomatic for cerebral ischemia during the successive 16 months; angiographic control evidenced an unmodified P.O. The other 5 patients, clinically symptomatic, underwent surgical correction: in one, intraoperative finding was a total carotid occlusion. In the other 4 patients the P.O. was confirmed and an endoarterectomy has been done. No immediate or later complications have been noted (clinical and ultrasonographic average follow-up time was 12 months). The experience described by the authors leeds to some considerations: a) the carotid P.O. is not so rare; specially if adequate angiographic technique has been employed; b) the non-invasive studies could not reliably distinguish a P.O; c) intraoperative findings do not confirm, always, angiographic ones but a fibrous, chronic and totally occluded internal carotid artery; d) the evolution of P.O. in a complete carotid occlusion is very probable but not obligatory, and if this happens it needs long period of time; consequently the surgical correction of P.O. could be justified but not urgently; e) the surgical correction of the P.O. can be done without particular difficulty and its outcome is so similar to those obtained from the routine carotid surgery.

["Late" obstruction induced by physical exertion in 15 boys with asthma caused by food aeroallergens in 2 periods of the year (winter and summer)]. / Studio della ostruzione "late" da sforzo fisico in 15 ragazzi con asma da aeroallergeni alimentari in due periodi dell'anno (inverno ed estate).

The aim of this study was the observation of the presence of the "late" phase of obstruction after free run in subjects responsive either to aeoallergens either to food allergens but with withdrawal of food allergens from diet in comparison with control group of children which did not have the food allergens exclusion. The study was executed in February and in June in the same children's group having the purpose to observe the variation of this phase in June with a great pollination of graminacee. The results showed the presence of a late phase of obstruction of magnitude similar to the quick phase which begun from III to VII h. but was no greater than -20% FEV1 fall. The control group showed in many children the fall of -20% FEV1 fall. The group of children responsive to the graminacee with food allergen's exclusion showed in June a greater obstructive late phase in comparison to February but the obstruction never was greater than -20% FEV1 fall.

[Nasal conduction and ventilation: study of 30 children with food allergy before and after adenoidectomy]. / Conduzione nasale e ventilazione: studio su 30 bambini con allergia alimentare prima e dopo adenoidectomia.

UNLABELLED: Observed the results of an investigation on the trouble of breathing in 1350 children, looked at the trouble's frequence (450 positive and 400 dubious), the Authors studied the obstruction of the high and peripheral, air-ways and the associated auditive troubles in relation with aerial allergens and foods allergens. METHODS: For the diagnosis of food's allergens: broncho-stimulation test by foods; rhinomanometry with Broms' formula, spirometry and Rresp exam, audiometric and impedentiometric exam. RESULTS: About 30 children with air-ways obstruction and positive at the bronchostimulation test to food's allergens 7 were Prick and Rast positive to aeroallergens'; 12 showed total obstruction of nasal pipes and audiometric and impedentiometric troubles, 15 obstruction of one only nasal pipe, 3 absence of obstruction. At the spirometric check and of Rresp in the 63.3% of children was associated obstruction of peripheral air-ways and in the 26.6% laryngeal obstruction. The only nasal obstruction was present in 10% of children, in the 48% was associated to conjunctivitis. In 12 children with audiometric and impedentiometric damage who were adenoidectomized two months after the surgical intervention the nasal condition showed evident improvement but in 5 children after one year the nasal obstruction showed itself again. The food allergens exclusion caused disappearance of nasal, laryngeal and peripheral obstructive syndrome and normalization of audiometric and impedentiometric values in the adenoidectomized subjects and in 95% of staying children.

[Study of the activity of ambroxol in children with aspecific bronchial hyperreactivity]. / Studio sull'attività dell'Ambroxol in bambini con iperreattività bronchiale aspecifica.

The authors examined the action of non-specific bronchial reactivity to methacholine (IBA) before and after treatment with ambroxol in a group of a children with clinic symptomatology of recurrent cough and/or rhinitis. The little patients are selected from a pediatric population that, besides the clinical data mentioned above, had a high probability to manifest nonspecific bronchial reactivity for which clinical and anamnestic data predisposed. In all patients were carried out an allergologic examination with the execution of Prick test and determination of total IgE. The results obtained and discussed suggest a favorable employ of the drug that is able to normalize the functional condition of the bronchial mucous membrane and of the ciliary apparatus which alteration are frequently present in subjects with high non-specific bronchial reactivity.

[Treatment of acute respiratory infections in childhood with a new cephalosporin, ceftriaxone]. / Trattamento delle infezioni acute respiratorie in età pediatrica con una nuova cefalosporina, il ceftriaxone.

Results of the comparison between tobramicine and ceftriaxone (new long-acting 3rd generation cephalosporin) in the treatment of pediatric acute respiratory tract infections are referred. Treatment with ceftriaxone is judged more advantageous both for efficacy and for saving the number of administered doses.

[Results of nasal brushing in the study of ciliary conformation and function in chronic bronchopneumopathies in childhood]. / Risultati dell'aplicazione del brushing nasale per lo studio della conformazione e funzione ciliare nelle broncopneumopatie croniche infantili.

UNLABELLED: For the frequent remark in childhood of relapsing bronchitis and/or broncho-pneumonic sickness we resolved to use for the differential diagnosis the nasal brushing method according to Rutland (for samples of ciliated epithelium from the deep surface of the inferior nasal turbinate using a 2 mm diameter nasal brush. The samples were used for ultrastructural study of cilia. RESULTS: Children studied 22:14 (6-14) y.old with relapsing bronchitis, 4 with relapsing broncho-pneumonitis, 1 Nemaline myopathy (n.m.) with heavy respiratory insufficiency; 3 Werdnig Hoffmann syndromes (W.H.). Control group of 4 normal children. In the control group and 14 relapsing bronchitis normal tubular pattern (9 + 2) and ciliary conformation were present; in the relapsing broncho-pneumonic syndrome group, 2 children presented deficiency of some brace dynein arms = chronic inflammation; in the Nemaline myopathy we noted total lack of interior dynein arms with random cilia orientation; in the 3 W.H. = normal cilia conformation. The authors noted for the heavy respiratory insufficiency of the Nemaline myopathy the presence of immotile cilia and discuss possibility that in all Nemaline myopathy syndromes this association may be present (a new syndrome?). The W.H. myopathy is characterized by normal cilia aspects and the respiratory insufficiency is caused by muscular respiratory insufficiency secondary to anterior spinal corn horn. In the Nemaline myopathy the same genetic defect can act in muscular fibres and on dynain arms (cytoskeleton). The technique described is suitable for processing epithelial brushings from other parts of the respiratory tract.(ABSTRACT TRUNCATED AT 250 WORDS)

[Incidence of wheezing asthma, rhinitis, urticaria and conjunctivitis in a pediatric emergency department]. / L'incidenza di wheezing-asma, tosse, rinite, orticaria, congiuntiviti in un dipartimento di emergenza pediatrica.

The Authors considered the incidences of the urgencies for allergic pathology arrived at Santobon Hospital during January-December 1992. In the period of twelve months a whole of 42.702 children came to Hospital; among them 4.359 showed an allergic pathology with a clear prevalence for wheezing and asthma. In addition it was observed too that an allergic mainly affected male children.

[Gastric mucosa changes caused by anti-inflammatory agents in childhood]. / Alterazioni mucosali gastriche da assunzione di farmaci antinfiammatori in età pediatrica.

Nonsteroidal anti-inflammatory drugs (NSAIDs) and steroids are very commonly prescribed. Morbidity and mortality from nonsteroidal anti-inflammatory drugs (NSAID) and steroids continue to be a significant health problem. In this paper are reported: 1) the biological insights into injurious effects of NSAIDs and steroids on mucosal protection and repair; 2) our clinical experience in the diagnosis and the management of children affected by secondary ulcer disease induced by therapeutic doses of NSAIDs and steroids; 3) the guidelines in the prevention of the NSAIDs and steroids-induced gastric damage.

[Results of a screening for atopy and aspecific bronchial hyperreactivity in 6-year-old children]. / Risultati di uno screening dell'atopia e della iperreattività bronchiale aspecifica in bambini di 6 anni di età.

In the course of a clinic-anamnestic study performed from November 1984 to April 1985 by the collaborators of USL 40 Maternal-Infantile Service, among the children (1569) of the first primary school, 622 children has been selected for possible, probable and verified asthma. For making deeper the diagnosis, 358 children had gone to the respiratory allergologic section of division of pneumology and respiratory allergology of Cardarelli Hospital. The control group was formed from 12 children without atopic familiarity and without asthmatic tendency. Among the 358 children, 127 were asymptomatic but with familiar anamnesis positive for atopy; 219 had or had suffered allergic symptomatology. The authors had studied all children by Prick-test with a standard battery of allergens and with by methacholine aspecific bronchial reactivity test. The familiar tendency to atopy was present in 81.1% of studied children, with prevalence of maternal tendency (41,83%) in comparison with this of ascendents and collaterals. Among the asymptomatic subjects with atopic familiarity only 27.56% was skin positive for one or more allergens, while 59.82% of symptomatic subjects was skin positive. The bronchial aspecific reactivity with methacholine was increased in the 62% of asymptomatic and in the 84.69% of symptomatic subjects. The aspecific bronchial reactivity level in mean was higher in the children with bronchial asthma but also the subjects with rhinitis and chronic cough has showed a higher level in comparison with adults affected of similar symptomatology. In 8 children the authors have studied the reproduction of PD20 methacholine which was very high.

[Changes in the motor apparatus of the bronchial cilia. Comparative study of two types of myopathies: nemaline myopathy and the Werdnig-Hoffmann syndrome]. / L'alterazione dell'apparato motore delle ciglia bronchiali. Studio in rapporto a due diversi tipi di miopatia: la nemaline myopathy e la sindrome di Werdnig-Hoffmann.

The Authors during an extensive applications of nasal brushing in infancy according to Rutland and Cole, remarked--first time in literature--the contemporary presence in a child of the nemaline myopathy syndrome and immotile cilia syndrome. Supposing the possibility that in other myopathy may be present the same ciliary immotile syndrome, they studied with nasal brushing three cases of Werdnig-Hoffman syndromes: but they presented normal ciliary conformation. The Authors suppose that in case in future it should be found in other cases of nemaline myopathy the contemporary presence of dinein arms lack, it should be demonstrated that this syndrome takes derivation from a genetic alteration both of the muscular apparatus and the ciliary mobility system of bronchial epithelius.

[Conservative endoscopic treatment for achalasia of the esophagus. Our experience]. / II trattamento endoscopico conservativo dell'acalasia esofagea. Nostra esperienza.

The Authors report their experience in the endoscopic treatment using pneumatic dilatation for 18 patients with achalasia, seen in the period January 1992 August 1995 at the Laboratory of Digestive Endoscopy of the Institute of General Thoracic and Vascular Surgery of the University of Parma. Ten were males and 8 females, age range 23-79 years (average age 48 yrs.). The most common symptoms was dysphagia, all patients were radiologically and manometrically studied confirming the clinical and endoscopic diagnosis. All underwent pneumatic dilatation under endoscopic control. In all, 36 dilations were carried out without registering complications related to the method. A maximum follow-up of 30 months confirmed a good-excellent outcome in 89% of cases. In 3 cases a gastroesophageal reflux symptomatology was registered, but successfully treated medically. The Authors conclude that the pneumatic dilation for achalasia is to be considered the treatment of choice, leaving the surgical approach only for cases resistant to such treatment.