[Complex congenital clubfoot: a case report]. / Pie equinovaro congénito complejo: presentación de un caso.

The Ponseti method is the ideal treatment for the management of congenital clubfoot of any etiology; it corrects the deformity in more than 90% of cases. However, a small percent of idiopathic clubfoot cases, known as complex clubfoot, do not respond properly to this treatment and thus a modification of the original technique described by Doctor Ponseti for manipulating the forefoot is required. We present herein the case of a patient with complex congenital clubfoot treated during her first year of life with the modified Ponseti method with good results.

Fibrodisplasia osificante progresiva / Fibrodysplasia ossificans progressiva: a case report

Se presenta el reporte de un caso de fibrodisplasia osificante progresiva en una paciente de 17 años de edad, quien fue valorada por primera vez a los 6 años. La ausencia de herramientas de terapéuticas que permitan modificar el curso de esta rara condición, hace que su reconocimiento temprano sea en extremo importante, para así evitar la realización de procedimientos quirúrgicos y biopsias innecesarias que aumenten el número de lesiones y limitaciones de estos niños

Amputación en el tratamiento de las malformaciones congénitas de los miembros inferiores en niños: Serie de casos. Experiencia de diez años / Amputation surgery and congenital deficiencies in children legs

En este estudio se evaluaron los pacientes que requirieron amputaciones por malformaciones congénitas en miembros inferiores en el Instituto de Ortopedia Infantil Roosevelt, durante los últimos diez años. Se incluyeron 62 pacientes, a los que se les realizó amputación en algún nivel de miembros inferiores, intervenidos entre enero de 1990 y diciembre de 2000. El promedio de edad fue de 6 años (0 a 18 años). Los casos se distribuyeron según el diagnóstico de nivel de amputación, compromiso uni o bilateral, forma del muñón, aplicaciones, tipo de marcha, satisfacción de los padres con el procedimiento y tiempo para adquirir autonomía en la marcha. Los diagnósticos más frecuentes fueron la hemimelia peronera tipo II (35,5 por ciento) y la hemimelia tibial tipo I, (21,0 por ciento); el tipo de amputación más frecuente fue la amputación del pie (35,5 por ciento), el compromiso bilateral se encontró en 16,1 por ciento y la complicación más frecuente fue la infección en 19,4 por ciento

Efectividad del calzado ortopédico pronador en el tratamiento postoperatorio de niños con pie equino varo congénito idiopático rígido / Effectiveness of the corrective shoes in the posoperative treatment of equinovarus deformities in children

El uso de calzado ortopedico en el tratamiento postoperatorio del pie equino varo congenito idiopatico (PEVCI) es motivo de controversia. Con el fin de conocer la efectividad del uso del calzado con modificaciones especiales (botas pronadoras) en el manejo postoperatorio del paciente con PEVCI, a quienes se les efectuó una liberación posterointerna ampliada (LIPA), se realizó un estudio experimental clinico controlado ciego, que comparó dos intervenciones: al primer grupo se le formuló zapato ortopedico (botas pronadoras), al segundo se le asigno zapato normal. Para la evaluación se utilizaron los enterizos de Turco modificados. Los resultados se clasificaron como buenos regulares y malos. Se presenta un analisis interno con el objeto de detectar diferencias entre los grupos. Hasta el momento no se han encontrado diferencias por lo que se autoriza la continuación del estudio para definir con mayor precisión la efectividad de la intervención

[Postischemic dysfunction and persistent ischemia in the early postinfarction period. Evaluation by echo-dobutamine-atropine test]. / Disfunzione postischemica e ischemia persistente nel postinfarto precoce. Valutazione con stress-test con eco-dobutamina atropina.

AIM: To study post-ischemic dysfunction and persistent ischemia in early post-infarction, by means of Echo-dobutamine-Atropine stress test (ECHO-DOB). Methods. We studied 138 patients (pts) aged < or = 75 yrs (mean 59.2 +/- 9.8) at their first uncomplicated myocardial infarction (AMI), treated with systemic thrombolysis. All pts underwent the test within 2 weeks since the onset of the attack and they were reevaluated in follow-up 3 months later. Under Echo and ECG monitoring, ECHO-DOB was performed according to EDICS protocol. Low doses was infused (5-10 mcg/Kg/min every 3') to assess the viability and high doses (20 > or = 40 mg/Kg/min + Atropine) to assess a possible persistent ischemia. The Wall Motion Score Index (WMSI) was used for the semiquantitative analysis of kinesis in a model of left ventricle divided into 16 segments. coronary angiography was performed within 30 days from the AMI, in 82.3% of pts. Results. Low dose of Dobutamine (DOB) induced in 92/136 (67.6%) pts an improvement in the contractile dysfunction in the region of necrosis; in 31.5% (29/92) it remained until the end of the test, suggesting the presence of viable myocardium, In absence of myocardium "at risk". High doses DOB induced worsening of contractile dysfunction in 64 pts (47%); in 40 (62.5%) viability had been previously detected (viable but ischemic myocardium); In 30 (75%) it was homozone or adjacent (viable but ischemic myocardium in the region of the coronary artery or in the tributary vessels correlated to the necrosis). In 24/64 (37.5%) pts the absence of modification of WMSI (no improvement and no worsening), suggested the presence of necrotic tissue in the tributary region of the vessel of necrosis. The significant improvement in kinesis revealed in 92 pts, by the follow-up control was confirmed in 64 (p < 0.001) (69.5%). Forty-four pts without hyperkinesia showed no significant improvement compared with the base line (stunned or hibernating myocardium). The sensitivity of low doses for viable myocardium (gold standard follow-up) was 69%; specificity was 77%. The sensitivity of high doses for ischemia (gold standard coronary arteriography) was 78%; specificity was 100%. No major side effects during the Dobutamine Atropine test were observed. CONCLUSIONS: The ECHO-DOB test in the post-infarction period allow the assessment of the functional significance of what is the objectified by coronary angiography and identifies pts at risk of more serious events. An integration of the test with the angiographic data can direct towards more suitable therapeutical or surgical choices.

Relationship between HDL-cholesterol and apolipoprotein A1 and the severity of coronary artery disease.

INTRODUCTION: We investigated whether apolipoprotein A1 (ApoA1) could better identify patients with severe vessel damage than HDL-cholesterol in a population with recent acute myocardial infarction (AMI). METHODS: One hundred and forty-one male patients (mean age: 54.3 +/- 7.3 years) who had undergone coronary angiography within 3 weeks of an AMI were studied. Coronary angiography data were evaluated both anatomically (as single, double or multivessel disease) and by means of the Gensini score. The main lipid parameters were evaluated on admission to the Coronary Care Unit. RESULTS: ApoA1 and the ApoA1/ApoB ratio (A1/B) mean values were significantly (P < 0.05) different when patients were divided on the basis of HDL-cholesterol cut-off value (35.0 mg.dl-1); when divided on the basis of Gensini score (< or = 18 or > 18), there was a statistically significant difference for ApoA1 (P < 0.01), HDL-cholesterol and A1/B (P < 0.05): only this latter was able to discriminate between the two groups. The mean ApoA1 and A1/B (P < 0.01), Gensini score (P < 0.001) and total cholesterol/HDL-cholesterol (P < 0.05) values of patients in the single-vessel disease group differ from those both of double- and multivessel disease groups. A1/B is also able to discriminate between single- and double-vessel disease. DISCUSSION: Our results confirm that ApoA1 and ApoA1/ApoB ratio are better than HDL-cholesterol in assessing the severity of coronary damage.

[Identification of ischemic and/or viable myocardium in early post-infarction period using echocardiographic imaging technique. Comparison of 2 tests: echo-dipyridamole and echo-dobutamine]. / Identificazione di miocardio ischemico e/o vitale nel post-infarto precoce mediante tecnica di imaging ecocardiografico. Confronto fra due test: eco-dipiridamolo ed eco-dobutamina.

AIM OF THE STUDY: To compare two stress tests: Dipyridamole-echocardiography (ECHO-DIP) and Dobutamine-echocardiography (ECHO-DOB) with angiographic data (still "gold standard") in early postinfarction period in order to identify ischemic and/or viable myocardium (considering the echocardiographic follow-up as "gold standard"). METHODS: 40 consecutive patients (pts), mean age 56, at their first uncomplicated acute myocardial infarction (AMI) treated with thrombolytic agents were studied. All underwent coronary angiography. RESULTS: The ECHO-DIP test resulted positive in 17 pts for transient regional asynergy (homozone 13/17; heterozonal positivity 4/17). The mean basal Wall Motion Score Index (WMSI) was 0.46 +/- 0.30 and at asynergy was 0.58 +/- 0.33 (p < 0.001). The ECHO-DOB test resulted positive in 20 pts; mean basal WMSI was 0.42 +/- 0.31 and at asynergy 0.55 +/- 0.35 (p < 0.001). Both tests were positive in 14 pts; the site of regional asynergy was the same in each test. During ECHO-DOB hypercinesia appeared in 27/39 pts (WMSI form basal 0.42 +/- 0.31 to 0.22 +/- 0.21 p < 0.001). Recovery of contractile function seems to identify viable myocardium: viable tissue shows early functional recovery during ECHO-DOB infusion. In 14 pts it remained until the end of the test, and in 12 it was transient, denoting the presence of myocardium "at risk". Remote (3- or 6-month) clinical and echocardiographic follow-up were carried out in all pts (25 undergoing medical therapy and 15 after PTCA or coronary bypass). In 26 pts with hyperkinesia at ECHO-DOB, basal echocardiogram revealed improvement of WMSI from 0.42 +/- 0.31 to 0.32 +/- 0.29 (p < 0.001). CONCLUSIONS: "Passive" ECHO-stress tests in the early postinfarction period are easy to perform and free of major risks, they allow pts at risk due to residual ischemia to be revealed with a high degree of specificity and sensitivity. The improvement of kinesis during inotropic stimulus of Dobutamine suggests stunned or hibernating myocardium.

[Azlocillin in the treatment of pulmonary infections in patients with cystic fibrosis: plasma concentrations and therapeutic indications]. / L'azlocillina nel trattamento delle infezioni polmonari dei pazienti affetti da fibrosi cistica: concentrazioni plasmatiche e indicazioni terapeutiche.

Azlocillin plasma concentrations have been studied in 10 cystic fibrosis patients suffering from chronic pulmonary infections with Pseudomonas aeruginosa. Patients were given single i.v. doses of 100 e 200 mg/kg body weight as intravenous infusion over 30 minutes. Azlocillin plasma levels have been assayed by a rapid, sensitive and precise high performance liquid chromatographic method. After the dose of 100 mg/kg body weight concentrations of azlocillin decreased below the therapeutic concentrations after three hours; dose of 200 mg/kg was followed by plasma concentrations in the therapeutically desirable range during the 6-8 hours study period. The pharmacokinetic analysis offers further evidence of the dose-dependent nature of azlocillin elimination. Higher dosage of 200 mg/kg body weight and monitoring of plasma drug levels are recommended in the therapy of patients with cystic fibrosis.

[Sensitivity and specificity of antigliadine antibodies in the diagnosis of celiac disease in a population of children in Toscana]. / Sensibilità e specificità degli anticorpi antigliadina nella diagnosi di celiachia in una popolazione toscana in età pediatrica.

The aim of this study was to determine the value of IgA and IgG antigliadin antibody test (AGA) for screening, diagnosis and follow-up of coeliac disease. A rapid, simple, sensitive and accurate immunosorbent assay (ELISA) was used to determine AGA IgA and IgG in the sera of patients with untreated coeliac disease (I stage), coeliac patients in gluten withdrawal, healthy controls, children with gastroenterological disorders other than coeliac disease and children with constitutional short stature. In the I stage of coeliac disease the AGA IgA gave a sensitivity of 90.9% and a specificity of 97.9%, the IgG assay resulted in a sensitivity of 100% and a specificity of 82.3%. AGA IgG resulted positive in 17.7% of control disease group, but it's interesting to remark that they had a mean level significantly lower than in coeliac patients. On gluten free diet the titres of AGA IgA returned to normal value in three months, while the AGA IgG showed a slower decrease.

Hemodynamic changes during acetate dialysis, bicarbonate dialysis and hemofiltration.

Twenty uremic patients submitted to three different dialytic procedures (6 patients to acetate dialysis, 8 patients to bicarbonate dialysis, 6 patients to hemofiltration) were monitored in respect to the hemodynamic parameters recorded with a thermistor Swan-Ganz catheter. During acetate dialysis there was an increment of cardiac index (CI) up to positive values (+4.8%), while resistance index (RI) decreased progressively until it reached -16.5%. During bicarbonate dialysis CI, after an initial fall, remained constant and RI increased (+8.6%). During hemofiltration CI decreased constantly throughout the entire procedure while RI increased until +12.9%. We concluded that during diffusive transport the buffer used plays an important role in determining hemodynamic responses to fluid removal; during convective transport vascular resistance increases in spite of acetate.

Biofiltration in chronic uremia: evaluation of long-term efficiency and hemodynamic tolerance with Swan-Ganz catheter.

Biochemical data and clinical tolerance were evaluated in eight uremic patients treated by Biofiltration (BF) for 5-20 months. In four patients hemodynamic parameters were monitored with a Swan-Ganz catheter during a session of BF. BF provides long-term biochemical safety and improved tolerance to fluid removal.

[Frequency of isolation and drug resistance of bacterial strains isolated from respiratory material in patients with cystic fibrosis]. / Frequenza di isolamento e chemioresistenza di stipiti batterici isolati da materiali respiratori in pazienti affetti da fibrosi cistica.

During 1985, 251 respiratory samples from 61 patients were examined at the tuscan cystic fibrosis Center (Florence), and isolated strains were tested against various antimicrobial drugs. Pseudomonas aeruginosa and Staphylococcus aureus were the predominant pathogens isolated. Infections caused by St. aureus were the commonest during the first years of life, whereas those caused by Ps. aeruginosa, occasional at early age, became chronic and the main source of infection with growth. Sensitivity testings against antibiotics confirmed that Ps. aeruginosa isolated from cystic fibrosis patients has a high percentage of resistant strains. The most active drugs were ceftazidime and aztreonam. An increased resistance to aminoglycosides was observed.

[Pharmacokinetics of oral theophylline. Comparison between asthmatic and cystic fibrosis patients]. / Farmacocinetica della teofillina orale. Confronto tra pazienti asmatici e pazienti con fibrosi cistica.

The pharmacokinetics of oral theophylline at steady-state were comparatively investigated in 13 asthmatic patients and in 10 patients with cystic fibrosis (CF). In all patients, the drug was administered twice daily as slow-release tablets. The total daily dose of theophylline ranged from 10.8 to 29.4 mg/kg/day. For each patient, the time-course of theophylline steady-state plasma levels was studied after the morning dose. Six serial plasma samples were drawn at 0, 2, 4, 6, 8, and 12 h after dosing. Model-independent methods were used for calculating the pharmacokinetics parameters (area under the curve and clearance). The clearance values (mean +/- SD) calculated in the two patient groups were significantly different (asthmatic patients: clearance = 61.2 +/- 15.6 ml/h/kg; CF patients: clearance = 86.3 +/- 22.8 ml/h/kg; P = 0.007). It has previously been shown that the clearance of theophylline after single dose is increased in CF patients. Our study confirms this finding under steady-state conditions and demonstrates that higher theophylline doses are on the average required to treat patients with FC.

[Spiro-ergometric evaluation of a group of healthy young subjects: sedentary females, sedentary males and practicing male sports amateurs (jogging)]. / Valutazione spiroergometrica effettuata in gruppi di giovani sani: femmine sedentarie, maschi sedentari e maschi praticanti sport amatoriale (jogging).

In order to assess the qualitative and quantitative differences in physical performance, twenty sedentary females (Group A), twenty sedentary males (Group B) and twenty amateur sportsmen who jogged 45-60 min 2-3 times a week (Group C), have been evaluated by spiroergometric test on bicycle ergometer. At the submaximal level of 90 Watts the cardiorespiratory efficiency, as reflected by the values of double product (DP) and ventilatory equivalent (VE) was statistically different in the three Groups (A less than B less than C). At the maximal work the females, compared to sedentary males, achieved lower values (P less than 0.001) of maximal working capacity (MWC), total work (TW), systolic blood pressure (SBP), maximal O2 uptake (VO2 max), O2 pulse (O2P) and a greater (P less than 0.001) energetic cost (EC = HRmax/TW): sedentary males, compared to amateur sportsmen, achieved lower values (P less than 0.001) of MWC, TW, VO2 max, O2P and greater values (P less than 0.001) of VE, EC, HR, and DP (P less than 0.02). The lower VO2 max in females can be mainly ascribed to the lower stroke volume which in turn correlates with smaller body surface area. The greater VO2 max and O2P max of amateur sportsmen can be ascribed to larger stroke volume and muscular O2 uptake brought about by jogging. Furthermore the females, compared to sedentary males, have achieved VO2 max and O2P max that were 29% lower, a TW 43% lower, while EC was 73,4% higher. The sedentary males, compared to amateur sportsmen, have achieved lower values of VO2 max (-14,3%), O2P max (-17,5%) and TW (-31,3%) with DP and EC 8,3% and 50,7% higher, respectively. On the grounds of these data sedentary subjects, particularly females, pay a high energetic cost during physical activity. The practice of an aerobic sport such as jogging, may notably increase cardiovascular and respiratory efficiency.