Standing and walking age in children with idiopathic clubfoot: French physiotherapy versus Ponseti method.

PURPOSE: A mild delay in gross motor milestones and walking age has been reported in infants with clubfoot. The influence of different treatments on motor development has been poorly investigated. Some parents and physical therapists express concern that the Ponseti method (PM) and its constraints (abduction brace, casts) would affect development more than the French physical therapy method (FM) due to greater immobilization and lesser stimulation.The purpose of this study was to evaluate achievement of three motor milestones (pull-to-standing, cruising and independent walking) in two groups of clubfoot patients treated at two experienced institutes respectively with the PM and FM. METHODS: In all, 52 consecutive infants (full-term at birth, mean age at beginning of treatment 24.3 days (sd 10), mean Dimeglio score 12 (sd 3.4)) were prospectively enrolled (26 patients per centre) and followed up to walking age recording milestones. RESULTS: The two groups were not different in terms of age at the beginning of treatment (p = 0.067) and rate of tenotomy. Age at tenotomy was significantly lower in the PM group (p = 0.000). Severity (p = 0.004) and number of bilateral cases (p = 0.012) were higher in the PM group. A non-significant difference was found for age of achievement of pull-to-standing (p = 0.109), cruising (p = 0.253) and independent ambulation (p = 0.349) between the two groups. Overall, milestones were achieved approximately two months later than normal population. Sex, severity, laterality and need of tenotomy were not found to significantly influence milestones. CONCLUSION: Our results confirmed that infants with clubfoot are expected to have a minimum delay in motor development. Infants treated with the PM and those treated with the FM did not show significant differences in gross motor milestones achievement at walking age. LEVEL OF EVIDENCE: Level II - Prospective comparative therapeutic studies.

Interobserver reliability of Diméglio and Pirani score and their subcomponents in the evaluation of idiopathic clubfoot in a clinical setting: a need for improved scoring systems.

PURPOSE: Diméglio (DimS) and Pirani (PirS) scores are the most commonly used scoring systems for evaluation of clubfoot, with many centres performing both. Interobserver reliability of their global score has been rated high in a few studies, but agreement of their subcomponents has been poorly investigated. The aim of the study was to assess interrater reliability of global scores and of items in a clinical setting and to analyse overlapping features of the two scores. METHODS: Fifty-six consecutive idiopathic clubfeet undergoing correction using the Ponseti method were independently evaluated at each casting session by two trained paediatric orthopaedic surgeons using both scores. Interobserver reliability of collected data was analysed; a kappa coefficient > 0.60 was considered adequate. RESULTS: For DimS and PirS, the Pearson correlation coefficients were 0.87 and 0.91 (p < .0001) respectively, and kappa coefficients were 0.23 and 0.31. Among subcomponents, kappa values were rated > 0.60 only for equinus and curvature of lateral border in PirS; muscular abnormality in DimS was rated 0.74 but a high prevalence index (0.94) indicated influence of scarce prevalence of this feature. All other items showed k < 0.60 and were considered to be improved.For overlapping features: posterior and medial crease showed similar agreement in the two systems, items describing equinus and midfoot adduction were much more reliable in PirS than in DimS. CONCLUSIONS: In a clinical setting, despite a high correlation of evaluations for total scores, the interobserver agreement of DimS and PirS was not adequate and only a few items were substantially reliable. Simultaneous use of two scores seemed redundant and some overlapping features showed different reliability according to criterion or scale used. Future scoring systems should improve these limitations. LEVEL OF EVIDENCE: Level I - Diagnostic studies.

Hyaluronan derivatives bearing variable densities of ferulic acid residues.

A synthetic procedure has been developed to conjugate ferulic acid (FA) to an important natural polysaccharide derivative such as hyaluronic acid (HA). The activation of FA with 1,1'-carbonyldiimidazole (CDI) has been investigated. Two reactive intermediates, namely monoimidazolide 2 [i.e. (E)-3-(4-hydroxy-3-methoxyphenyl)-1-(1H-imidazol-1-yl)prop-2-en-1-one] and bisimidazolide 3 [i.e. (E)-4-(3-(1H-imidazol-1-yl)-3-oxoprop-1-enyl)-2-methoxyphenyl 1H-imidazole-1-carboxylate] were characterized from the point of view of their structure and reactivity. The ready isolation of bisimidazolide 3 and its reactivity support its potential usefulness in the feruloylation of molecular or macromolecular materials bearing hydroxyl moieties. Bisimidazolide derivative 3 has been found to be an effective reagent in the feruloylation of HA to give HAFA graft copolymers showing different grafting degrees (GD), which could be modulated by varying the reaction conditions. A series of HAFA derivatives showing different GD values has been prepared and submitted to an extensive macromolecular and rheological characterization in order to ascertain that the grafting of HA with FA does not degrade the polysaccharide backbone and to evaluate the role of GD in affecting solubility and rheological properties. The results suggested that relatively low GD values were sufficient to confer physical cross-linking capabilities resulting in the features of a strong gel of HAFA dispersions.

Corneal sensitivity in keratoconus after penetrating and deep anterior lamellar keratoplasty.

PURPOSE: To evaluate recovery of corneal sensitivity in the graft following penetrating keratoplasty (PK) or deep anterior lamellar keratoplasty (DALK) in patients with keratoconus. MATERIALS AND METHODS: 113 eyes of 113 patients (39 females, 74 males) were examined. All eyes had undergone corneal grafting: 39 PK, 74 DALK. Corneal sensitivity tested using the Cochet-Bonnet esthesiometer was evaluated the day before and 3 days, 3, 6, 12 and 24 months after surgery. The corneal sensitivity recovery was associated with surgical techniques (PK or DALK), age and sex of recipient and donor, cadaver time and type of preservation of the cornea. RESULTS: The average percentage of recovery of corneal sensitivity was 91% at 2 years. Surgical techniques and other qualitative and quantitative variables did not affect the statistically significant recovery of corneal sensitivity. CONCLUSIONS: In both surgical techniques, PK and DALK, we noticed a good recovery of corneal sensitivity in 2 years.

Selective disarrangement of the rostral telencephalic cholinergic system in heterozygous reeler mice.

Reelin (RELN) is a key molecule for the regulation of neuronal migration in the developing CNS. The reeler mice, which have spontaneous autosomal recessive mutation in the RELN gene, reveal multiple defects in brain development. Morphological, neurochemical and behavioral alterations have been detected in heterozygous reeler (HR) mice, suggesting that not only the presence, but also the level of RELN influences brain development. Several studies implicate an involvement of RELN in the pathophysiology of neuropsychiatric disorders in which an alteration of the cholinergic cortical pathways is implicated as well. Thus, we decided to investigate whether the basal forebrain (BF) cholinergic system is altered in HR mice by examining cholinergic markers at the level of both cell body and nerve terminals. In septal and rostral, but not caudal, basal forebrain region, HR mice exhibited a significant reduction in the number of choline acetyltransferase (ChAT) immunoreactive (ir) cell bodies compared with control mice. Instead, an increase in ChAT ir neurons was detected in lateral striatum. This suggests that an alteration in ChAT ir cell migration which leads to a redistribution of cholinergic neurons in subcortical forebrain regions occurs in HR mice. The reduction of ChAT ir neurons in the BF was paralleled by an alteration of cortical cholinergic nerve terminals. In particular, the HR mice presented a marked reduction of acetylcholinesterase (AChE) staining accompanied by a small reduction of cortical thickness in the rostral dorsomedial cortex, while the density of AChE staining was not altered in the lateral and ventral cortices. Present results show that the cholinergic basalo-cortical system is markedly, though selectively, impaired in HR mice. Rostral sub-regions of the BF and rostro-medial cortical areas show significant decreases of cholinergic neurons and innervation, respectively.

Volume transmission and wiring transmission from cellular to molecular networks: history and perspectives.

The present paper deals with a fundamental issue in neuroscience: the inter-neuronal communication. The paper gives a brief account of our previous and more recent theoretical contributions to the subject and also reports new recent data that support some aspects of our proposal on two major modes of communication in the central nervous system: the wiring and the volume transmission. There exist two competing theories on inter-neuronal communication: the neuron doctrine and the theory of the diffuse nerve network, supported by Cajal and Golgi, respectively (see their respective Nobel Lectures). The present paper gives a brief account of a view on inter-neuronal communication in the brain, the volume and wiring transmission concept that to a great extent reconcile these two theories. Thus, the theory of volume and wiring transmission are summarized and its recent developments that allow to extend these two modes of communication from the cellular network to the molecular network level is also briefly illustrated. The explanatory value of this broadened view is further enhanced by our recent proposal on the existence of a Global Molecular Network enmeshing the entire central nervous system. It may be interesting to note that also the Global Molecular Network theory is reminiscent of the old reticular theory of Apathy. Finally, the so-called 'tide hypothesis' for diffusion of signals in the brain is briefly discussed and its possible extension to the molecular level is for the first time introduced. Early indirect evidence supporting volume transmission in the brain was the discovery of transmitter-receptor mismatches. Thus, as an experimental part of the present paper a new approach to evaluate transmitter-receptor mismatches is given and evidence for inter-relationships between temperature micro-gradients and mismatches is provided.

Stereoselective metabolism of mexiletine in Chagasic women with ventricular arrhythmias.

Following a week of racemic mexiletine HCl at 200 mg tid (2x100 mg capsules), stereoselective aliphatic hydroxylation was studied in eight Chagasic women with chronic ventricular arrhythmias (52-67 yrs) with no history of renal or hepatic diseases. Blood samples were collected at dose interval up to 24 h of drug administration. Plasma concentrations of R(-) and S(+) mexiletine (MEX) and its metabolite hydroxymethylmexiletine (HMM) were determined by HPLC after derivatization with chiral reagent. The differences between R(-) and S(+) enantiomers were compared by paired t-test. Results are mean (95% CI). The following differences (p < 0.05) between R(-) and S(+) enantiomers, respectively, were found: MEX AUCss(0-8) 2.34 (1.84-2.85) vs 2.55 (1.97-3.13) microg.ml(-1) x h(-1); MEX CL/f 11.27 (7.77-14.77) vs 10.46 (7.18-13.74)ml.min(-1).Kg(-1); HMM Cmax 38.26 (24.3-52.22) vs 16.73 (10.1-23.29)ng.ml(-1); HMM Tmax 4.71 (2.67-6.76) vs 3.29 (1.24-5.33) h and HMM AUCss(0-8) 253.50 (165.39-341.61) vs 103.70 (69.51-137.90)ng.ml(-1).h(-1). The AUCss(0-8) ratio R(-)/S(+) for MEX was 0.93 (0.87-0.98) while for HMM was 2.50 (2.16-2.85). Distribution of MEX and HMM enantiomers were not significantly different. In this study we demonstrate that kinetic disposition of mexiletine exhibits stereoselectivity in vivo and that aliphatic hydroxylation is favored for R(-) mexiletine in Chagasic women with ventricular arrhythmias.

[Reconversion after Hartmann's procedure. Our experience]. / Ricanalizzazione dopo Hartmann. Nostra esperienza.

An increasing number of intestinal reconversion after Hartmann have been performed in recent years, especially due to improved surgical techniques and progressively lengthened lifespan. The authors report 33 cases of intestinal recanalization of 100 interventions according to Hartmann from 1984 to 1996 (21 not neoplastic pathologies, 12 neoplasias). The variables considered included: patient age, type of disease requiring intervention according to Hartmann, oncologic characteristic of patients with neoplasia, interval between the two interventions, preoperative examinations performed, morbidity and mortality after reconversion. Furthermore, the fundamental indications for reconversion are described, in particular in patients with neoplasias (CEA, transanal echo, total body Ct, anal manometry). The low frequency of preoperative complications, zero mortality, satisfactory long-term follow-up (only one patient with neoplastic relapse) indicate that colon-rectal reconversion can also be performed in the elderly and patients with neoplasias with favorable prognosis.

Congenital circumflex coronary artery fistula with drainage into the left ventricle.

Congenital coronary fistula is a rare heart defect, which consists of a communication between a coronary artery and a cardiac chamber of coronary vessel. Most such fistulae drain into a right heart chamber or into the pulmonary artery. Congenital left coronary artery-left ventricle fistula is even more uncommon. In a search of the literature, we were able to find only 15 other cases of congenital left coronary artery-left ventricle fistula. We describe here the clinical picture of a patient with left circumflex coronary artery draining into the left ventricle, and the successful surgical repair of the lesion. As far as we know, such a case has never before been reported in detail.

[Superior vena cava compression syndrome]. / Síndrome de compressão da veia cava superior.

The superior vena cava syndrome that causes difficulties on venous return to the heart is a uncommon clinical entity and actually, more than 90% of the cases are secondary to a malignant intrathoracic process, rather than the intrinsic diseases of the aorta that is a rare etiology of the compression. The present report documents an aortic ascending atherosclerotic aneurysm that developed a superior vena cava compression syndrome. The literature was reviewed, stressing some important aspects about the etiology, physiopathology, diagnostic procedures, prognostic and treatment of this syndrome.

[Percutaneous transluminal coronary angioplasty. Analysis of 305 dilatations]. / Angioplastia coronária transluminal percutânea. Análise de 305 dilatações.

PURPOSE: To show the initial experience of this service with the technique of percutaneous transluminal coronary angioplasty. PATIENTS AND METHODS: Between january 1988 and december 1989, 305 coronary angioplasties were performed in 280 patients with age range of 33 to 82 years (being 49% above 60 years old) and 71% male. Among those, 212 (75%) experienced clinical picture of stable angina and 68 (25%) of unstable angina or myocardial infarction. Patients were divided in three groups: group A--those with lesions of 70% or worse in more than one vessel or in the same vessel with or without any other vessel total occlusion (multiple lesions or multiple vessels: 36 patients; group B--those with lesions of 70% or worse in one vessel with at least one occluded vessel: 64 patients; group C--those with lesions of 70% or worse isolated in a single vessel: 180 patients. RESULTS: Of the 280 treated patients, 229 were considered successful (75%). There has been 22 complications (7%) and 9 deaths (3%). The late follow-up (from 1 to 24 months), displayed 28 new procedures based on clinical suspicion of restenosis. Obstruction or occlusion were present in 16 of the later, with 10 being selected to undergo new procedure, one of those twice. The remaining 12 patients received clinical or surgical treatment. CONCLUSION: Aside from the fact that early and midterm results indeed are promising, the procedure is not safe from immediate risks, and limiting late restenosis. Nevertheless, the lesions might be redilated by the same technique, with success rates comparable to the initial one.