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M Di Lorenzo

Publications and source records attributed to M Di Lorenzo.

At least 19 recordsLinked to original sources
PubMed2006

Putative virulence-related genes in Vibrio anguillarum identified by random genome sequencing.

The genome of Vibrio anguillarum strain H775-3 was partially determined by a random sequencing procedure. A total of 2,300 clones, 2,100 from a plasmid library and 200 from a cosmid library, were sequenced and subjected to homology search by the BLAST algorithm. The total length of the sequenced clones is 1.5 Mbp. The nucleotide sequences were classified into 17 broad functional categories. Forty putative virulence-related genes were identified, 36 of which are novel in V. anguillarum, including a repeat in toxin gene cluster, haemolysin genes, enterobactin gene, protease genes, lipopolysaccharide biosynthesis genes, capsule biosynthesis gene, flagellar genes and pilus genes.

Bacterial Capsules↗
PubMed2003

Strictureplasty: An alternative approach in long segment bowel stenosis Crohn's disease.

BACKGROUND/PURPOSE: Intestinal resection is the most frequent surgical procedure for bowel stenoses in Crohn's disease (CD). Recurrence of strictures, particularly with ileocolonic disease, often requires resection of lengthy segments of bowel, potentially resulting in short bowel syndrome. Different techniques of strictureplasty, such as those described by Mikulicz, Finney and Michelassi, are used in adults. However, these procedures are uncommon in pediatric surgery. The authors report their experience with different techniques of strictureplasty and with their modified Michelassi technique for the surgical treatment of long intestinal strictures caused by CD. METHODS: Five adolescents (2 boys; 3 girls; mean age, 16 age; range, 14 to 20 years) with severe ileocolonic stenoses and intestinal obstruction, not responsive to medical and nutritional therapy, were treated with different strictureplasty techniques. In 3 of them the modified side-to-side Michelassi technique was used. RESULTS: No postoperative complications occurred. After a mean follow-up of 20.5 months (range, 6 to 28 months), patients are free of symptoms with good nutritional status and off steroid therapy. CONCLUSIONS: Strictureplasty is a good and effective surgical option for sparing bowel length in CD patients with extensive intestinal strictures.

Adolescent↗
PubMed2003

Combined approach to functional constipation in children.

BACKGROUND/PURPOSE: The authors' 15-year experience with children shows a high percentage of recurrence of functional constipation (FC) with conventional treatment. These data, confirmed in the international literature, led them to develop a new therapeutic approach. The aim of this study was to achieve intestinal control and avoid recurrence of FC in children through use of medical-psychological treatment. METHODS: The authors studied 25 children (18 boys; mean age, 4.7 years; range, 2.10 to 7), 20% of whom had anal fissure, 30% retentive soiling, 52% pain on defecation, and 88% fecal retention owing to FC. Children and parents were questioned about eating and sleeping habits, school, toilet training, and daily routine. Treatment included increasing water and fiber intake, laxatives, and family therapy including making rules and working on autonomy and paternal role. RESULTS: Mean onset of FC was 3.5 years, after "stressful events" in 88%. The questionnaire shows that 68% lacked parental autonomy and authority; 84% of children decided on their own about eating habits and sleeping; 68% had a "peripheral" father with a mother-child symbiotic relationship. After one month of therapy, 92% of children showed a modification of at least 2 behavioral patterns; after 3 months, 88% had regular bowel movements. During follow-up (range, 6 to 28 months), 48% had 2 or 3 recurrent episodes. After one year, 68% had reinforced the new behavioral patterns with resolution of the pathologic aspects. CONCLUSIONS: A multidisciplinary approach in the treatment of childhood functional constipation showed consistent therapeutic results by making rules and by equalizing family roles.

Cathartics↗
PubMed2001

Value of transthoracic echocardiography in the diagnosis of pulmonary embolism: results of a prospective study in unselected patients.

PURPOSE: Echocardiography is advocated by some as a useful diagnostic test for patients with suspected pulmonary embolism (PE), but its diagnostic accuracy is unknown. The present study was undertaken to determine prospectively the sensitivity and specificity of transthoracic echocardiography in the diagnosis of PE. SUBJECTS AND METHODS: We examined 110 consecutive patients with suspected PE. The study protocol included assessment of clinical probability, echocardiography, and perfusion lung scanning. Pulmonary angiography was performed in all patients with abnormal scans. As echocardiographic criteria to diagnose acute PE, we used the presence of any two of the following: right ventricular (RV) hypokinesis, RV end-diastolic diameter >27 mm (without RV wall hypertrophy), or tricuspid regurgitation velocity >2.7 m/sec. Clinical estimates of PE served as pretest probabilities in calculating, after echocardiography, the posttest probabilities of PE. RESULTS: Pulmonary angiography confirmed PE in 43 (39%) of 110 patients. Echocardiographic diagnostic criteria for PE yielded a sensitivity of 56% and a specificity of 90%. For pretest probabilities of 10%, 50%, and 90%, the posttest probabilities of PE conditioned by a positive echocardiogram were 38%, 85%, and 98%, respectively. The posttest probabilities of PE conditioned by a negative echocardiogram were 5%, 33%, and 81%, respectively. CONCLUSIONS: In unselected patients with suspected PE, transthoracic echocardiography fails to identify some 50% of patients with angiographically proven PE. Although echocardiographic findings of RV strain, paired with a high clinical likelihood, support a diagnosis of PE, the transthoracic echocardiography has to have a better sensitivity to be used as a screening test to rule out PE.

Adolescent↗
PubMed2001

Altered nitric oxide production in the premature gut may increase susceptibility to intestinal damage in necrotizing enterocolitis.

BACKGROUND/PURPOSE: Nitric oxide (NO) mediates enteric smooth muscle relaxation and mucosal protection. The authors have identified an ontogenically determined pattern of enteric NO neural maturation that may render the distal gut of premature piglets susceptible to injury. METHODS: NO synthase (cNOS and iNOS) activities were measured in the developing piglet gut wall and compared with gut from an intraluminal model of necrotizing enterocolitis (NEC) at different times. RESULTS: In premature animals, iNOS activity was significantly higher 3 hours after NEC induction compared with similarly treated 3-day-old piglets. INOS levels continued to rise 6 hours after NEC induction in prematures. Premature animals (labor induced by prostaglandins) failed to show such a rise in iNOS. In 3 day olds, iNOS levels increased significantly 16 hours after injury compared with the 3-hour group. CONCLUSIONS: iNOS production increases in premature piglets with NEC compared with full-term NEC animals and continues to rise in the presence of intestinal damage regardless of developmental status. Maternal administration of prostaglandins attenuates this rise in iNOS activity. Elevated NO production in premature gut may contribute to increased susceptibility to damage in NEC.

Age Factors↗
PubMed2001

The multiple facets of pulmonary sequestration.

PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. METHODS: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.

Abnormalities, Multiple↗
PubMed2000

Repair of the huge umbilical hernia in black children.

Surgical repair of large umbilical hernias may present a challenging surgical problem. The currently described surgical techniques often yield disappointing results. The authors describe a new technique that allows for the repair of the fascial defect and the creation, with the use of a square cutaneous flap, of a neoumbilicus with sufficient depth and a good cosmetic appearance.

Black People↗
PubMed2000

Past and future of biliary atresia.

BACKGROUND: With the advent of liver transplantation the outcome of children with biliary atresia (BA) has improved. Is Kasai hepatic portoenterostomy (KHPE) still a valuable option for the treatment of these patients? METHODS: From 1974 to 1998, 77 patients with biliary atresia have been treated at our institution: 50 girls and 27 boys. RESULTS: Seventy-four patients had a KHPE, and 3 patients had no KHPE because of delay in diagnosis. A total of 65 of 74 patients (88%) had undergone KHPE type I, 4 patients (5.4%) KHPE type II, 3 patients (4%) had a Suruga modification, and 2 patients (2.6%) had a portocholecystostomy. Among the 74 patients, 11 were lost to follow-up and their cases were considered failures. Seventeen of our patients are alive at long-term follow-up after KHPE. Among the 77 patients, 33 (43%) had an orthotopic liver transplantation (OLT). Successful KHPE patients underwent transplant at a mean age of 9 years, and KHPE failed at a mean age of 11 months. A total of 25 of 77 (32%) of patients are alive thanks to OLT. In the cohort, the overall survival rate for the KHPE plus OLT is 42 of 77 (55%). Mortality and morbidity rates were more frequent among the younger patients who had early OLT after KHPE failure. In our series, overall survival rate was improved when the patient had a successful KHPE (P < .001). CONCLUSIONS: Kasai hepatic portoenterostomy (KHPE) continues to be a valuable procedure in the treatment of infants with biliary atresia (BA). Successful KHPE permits transplantation at an age at which mortality and morbidity are decreased leading to a better outcome.

Biliary Atresia↗
PubMed2000

Pulmonary lymphangiectasia revisited.

PURPOSE: Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pulmonary lymphatics. Although case reports are published, little is known about survivors past the neonatal period. METHODS: This is a retrospective review of histologically proven PL in fetuses, infants, and long term survivors since 1965. RESULTS: Eleven children (8 boys, 3 girls) and 8 aborted fetuses (7 male, 1 female) were identified. The fetuses weighed 463.4 g (177 to 681 g). Six were aborted between 19 to 24 weeks of gestation for multiple malformations or anencephaly, and 2 spontaneously aborted: one with PL only, the other with twin-twin transfusion syndrome. Clinical PL was diagnosed between 0 and 11 months of age. Six children died (2 neonatal, 4 within 10 days), 5 survived. Two deaths occurred after cardiac surgery. Among survivors, the symptomatology and frequency of admissions diminished over time. Symptoms included progressive respiratory distress, chronic cough, recurrent pneumonia, bronchial asthma, and choking. One child with bilateral chylothorax was later diagnosed with Noonan syndrome; 2 patients had minor cardiac malformations. Rapid deterioration occurred with mild respiratory infections with only supportive treatment available. Chest x-ray showed marked hyperinflation with interstitial infiltrate. CONCLUSIONS: This is the first long-term study of primary PL and will help counsel parents. Although fatal in the neonatal period, survival is possible if diagnosed past the neonatal period and improvement is expected.

Abortion, Spontaneous↗
PubMed1999

Management of an infant with diffuse bullous pulmonary lesions using high-frequency oscillatory ventilation.

PURPOSE: To describe the anesthetic and ventilatory management of an infant with diffuse pulmonary bullous lesions. CLINICAL FEATURES: Four successive operations were scheduled for an infant with diffuse pulmonary bullous lesions. At the age of seven weeks, conventional positive pressure ventilation during laparotomy for intestinal occlusion led to arterial desaturation. This was corrected by returning to spontaneous respiration and deep inhalation anesthesia with halothane. Based on our ICU experience and due to a potential impaired oxygenation during conventional ventilation, we chose high-frequency oscillatory ventilation (HFOV) for bilateral sequential thoracotomies for bullectomies at the age of five months. We elected the same ventilatory mode for laparotomy for intestinal obstruction secondary to a polyp at the age of six months. This ventilatory mode was combined with total intravenous anesthesia and epidural analgesia and provided optimal oxygenation and ventilation as well as vital signs stability. CONCLUSION: High frequency oscillatory ventilation is a safe technique that may be used in the operating room in cases where conventional ventilation failed to provide satisfactory gas exchange.

Anesthesia, Epidural↗
PubMed1999

Pleuropulmonary blastoma: a rare pathology with an even rarer presentation.

BACKGROUND: Pleuropulmonary blastoma is among the rarest tumors of childhood. Three types have been described: cystic, solid, and mixed. To date, bilateral disease has not been documented. METHODS AND RESULTS: A 5-week-old girl presented with a history of fever. Chest x-ray showed bilateral diffuse cystic lesions. Bowel obstruction developed that required laparotomy. Multiple small bowel polyps were resected. The patient was readmitted 4 months later with deteriorating respiratory status. She underwent sequential thoracotomies for resection of multiple bullae under high-frequency oscillatory ventilation. Small bowel polypectomies were again required because of obstruction. Lung lesions were compatible with pulmonary blastoma but could not be correlated with intestinal polyposis. Bilateral cystic renal lesions were seen on ultrasound scan. Her disease progressed, despite chemotherapy, with the appearance of metastatic iris lesions. She again underwent laparotomies for multiple recurrent generalized small bowel polyps that were causing obstruction. Expanding renal cysts affected kidney function, and she died at 14 months of age. CONCLUSIONS: The rare association between pleuropulmonary blastoma and Wilms' tumor or nephroblastomatosis is known but rarely reported. Lacking pathological evidence, we can only speculate that this was the case. We have been unable to demonstrate any histological association between the renopulmonary and digestive lesions. Despite many unanswered questions, we are likely dealing with a "syndrome" of sorts with a dire outcome, despite aggressive treatments.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1999

Sick sinus syndrome with and without atrial fibrillation: atrial refractoriness and conduction characteristics.

BACKGROUND: Clinical electrophysiology has focused the attention on the electrophysiological properties of the atrial muscle in patients with atrial fibrillation: shortened and inhomogeneous refractoriness and local and regional conduction slowing, as well as prolonged intra- and interatrial conduction disturbances, are well described as electrophysiological parameters associated with the genesis of atrial fibrillation. Patients with sick sinus syndrome are variously included in these studies, but electrophysiological characteristics of patients with sick sinus syndrome alone appear less investigated, even if atrial fibrillation is part of its natural history. The aim of the present study was to define the electrophysiological characteristics of sick sinus syndrome patients with or without paroxysmal atrial fibrillation, compared to subjects without atrial fibrillation and sick sinus syndrome. METHODS: We reviewed the electrophysiological data of 39 patients with sick sinus syndrome (mean age 70 +/- 8 years), who underwent an electrophysiological study in sinus rhythm for the evaluation of the atrial substrate. In 12 patients an associated history of paroxysmal atrial fibrillation was documented. Twenty-seven patients were included in the study with a diagnosis of sinus node dysfunction alone. We also considered as control group 25 subjects (mean age 63 +/- 14 years), referred to our electrophysiological laboratory for unexplained syncope or atrioventricular disturbances. Following pharmacological wash-out and at a drive cycle of 600 ms, effective and functional refractory periods, S1-A1 and S2-A2 latency, A1 and A2 width, and the latent vulnerability index (effective refractory period/A2), were measured. In addition, the P-wave duration during spontaneous sinus rhythm on the surface ECG in D II/V1 leads was measured. RESULTS: Between sick sinus syndrome patients with or without atrial fibrillation, no significant statistical differences in electrophysiological parameters were found. When compared to the control group, sick sinus syndrome patients did not show any differences in effective refractory period (239 +/- 34 vs 250 +/- 29 ms), functional refractory period (276 +/- 28 vs 280 +/- 32 ms), S1-A1 (38 +/- 16 vs 33 +/- 11 ms), and S2-A2 latency (68 +/- 25 vs 63 +/- 25 ms). In contrast, we observed remarkable differences in terms of atriogram duration A1 (60 +/- 20 vs 39 +/- 13 ms, p < 0.001), A2 (95 +/- 34 vs 57 +/- 18 ms, p < 0.001), and effective refractory period/A2 (2.8 +/- 1.2 vs 4.8 +/- 1.7 cm, p < 0.001). Also the duration of the P wave was longer (103 +/- 17 vs 94 +/- 45 ms, p < 0.05). CONCLUSIONS: In sick sinus syndrome patients with or without atrial fibrillation, electrophysiological characteristics appear homogeneous. When compared to the control group, refractoriness was quite similar. In contrast, the most important abnormalities appear based on conduction slowing disturbances, responsible for a low latent vulnerability index. This could explain, at least in part, the tendency of sick sinus syndrome to develop atrial fibrillation as a part of its natural history. At present, the influence of an altered electrophysiological substrate on pharmacological or pacing therapy in patients with sick sinus syndrome is not yet known.

Aged↗
PubMed1999

QT dispersion and early arrhythmic risk during acute myocardial infarction.

BACKGROUND: QT dispersion (maximal minus minimal QT interval calculated on a standard 12-lead electrocardiogram) has been suggested to reflect regional variations of ventricular repolarization and to provide a substrate for reentry ventricular arrhythmias. In this study we evaluate QT dispersion in patients with acute myocardial infarction and assess its relation with early severe ventricular arrhythmias. METHODS AND RESULTS: We studied 101 patients with acute myocardial infarction and a control group of 97 healthy subjects. We determined QT and QTc dispersion on the electrocardiograms performed 12 hours and 3 and 10 days after the onset of symptoms in myocardial infarction patients and on the control group. The average values of QT and QTc dispersion (measured hereafter in milliseconds, ms) were as follows: 70.5 +/- 42.5-87 +/- 46.6 (after 12 hours), 66.5 +/- 37.8-76.9 +/- 43.5 (on day 3), 68.9 +/- 42-76.3 +/- 43.8 (on day 10) and 44 +/- 13.4-54.2 +/- 16.3 (in control group). We observed statistically significant differences in QT and QTc dispersion between the electrocardiogram of normal subjects and each of the three electrocardiograms performed on patients with infarction (p < 0.0005, p < 0.005). We recorded a greater QT dispersion in patients with anterior infarction with respect to those with inferior/lateral infarction (79 +/- 38.6 vs 65.2 +/- 43.16, p < 0.05) and in patients with ejection fraction < 45% (93.1 +/- 28.4 vs 68.3 +/- 34.1 p < 0.005). During the first three days, QT dispersion did not differ in patients treated with thrombolytic agents with respect to those who were untreated, while on day 10 untreated patients showed higher values (74.9 +/- 45.3 vs 60.5 +/- 37.7, p < 0.05). Creatine kinase peak level, sex and age of the patients did not influence QT dispersion. Thirteen patients (12.8%) developed severe ventricular arrhythmias within 72 hours after infarction: 8 patients (7.9%) had ventricular fibrillation and 5 patients (4.9%) had sustained ventricular tachycardia. We found higher early QT and QTc dispersion values in patients who developed severe ventricular arrhythmias (108.8 +/- 63.2 and 125.8 +/- 68.5) with respect to patients who did not (63.3 +/- 32.9 and 80.8 +/- 38.9, p < 0.0005, p < 0.0005). CONCLUSIONS: Our data suggest that QT dispersion: 1) increases during acute myocardial infarction; 2) peaks in the early hours after symptom onset; 3) drops late after infarction in patients treated with thrombolytic agents; 4) is associated with early severe ventricular arrhythmias.

Arrhythmias, Cardiac↗
PubMed1998

Airbags and children: a spectrum of C-spine injuries.

Over 30 children who were improperly restrained or in rear facing safety seats have been reported killed in motor vehicle accidents (MVA) involving airbags. The authors report one minor and two major injuries in properly restrained children in the front passenger seat. In case 1, A 10-year-old seat-belted boy was involved in an MVA (40 km/h) with deployment of both airbags. Physical examination findings showed right hyphema with corneal abrasion, right cheek abrasion and minimal cervical tenderness. C-spine x-ray was normal. He was treated for whiplash and facial burns resulting from contact with hot gas released by the airbags and discharged. In case 2, a 4-year-old boy wearing a lapbelt was in a MVA (20 km/h) with airbag deployment. On arrival, his Glasgow coma score was 3 and he was hemodynamically unstable. Secondary survey after stabilization showed left neck abrasions and ecchymoses, quadraplegia, priapism, and absent rectal tone. C-spine x-ray showed atlanto-occipital dislocation with possible complete spinal cord transection at C1. Aggressive maneuvers were withheld, and the patient was pronounced dead. Autopsy findings confirmed the clinical diagnosis. In case 3, a 3-year-old boy in a forward-facing safety seat was in a MVA (60 km/h) with air bag deployment. The patient was fully awake. C-spine x-rays were normal. Because of fluctuating level of consciousness, he underwent head computed tomography (CT) scan, which demonstrated a posterior fossa subarachnoid hemorrhage and a hematoma posterior to the odontoid, suggesting a ligamentous tear. He remained asymptomatic and was discharged on day 6. A head CT scan at 1 month showed a periosteal reaction in the area of the alar ligament suggestive of partial ligamentous avulsion; this injury was the forerunner of atlanto-occipital dislocation. Airbags deploy by releasing a hot effluent at 300 km/h. Mechanisms of injury include direct contact of hot gas with facial skin and energy transmitted directly from the airbag system to the child's head and neck. These cases illustrate a spectrum of C-spine injuries caused by airbag deployment and support the recommendation that children under 12 years of age travelling in a car equipped with dual airbags be seated in the back.

Accidents, Traffic↗
PubMed1998

A role for H-NS in the regulation of the virF gene of Shigella and enteroinvasive Escherichia coli.

We have investigated the role of H-NS, one of the major components of the bacterial nucleoid, in the expression of the virF gene present on the large virulence plasmid of Shigella and enteroinvasive Escherichia coli in response to different environmental conditions. VirF is an AraC-like protein which activates at least two promoters, virB and virG, both repressed by H-NS. Band shift experiments reveal that the affinity of H-NS for the virF and virB promoters is comparable, while the affinity for the virG promoter is higher. Polyacrylamide gel electrophoresis of three DNA fragments containing the virF, the virB and the VirG promoters demonstrates, in agreement with computer predictions, that they have an intrinsically curved structure, confirming the preference of H-NS for bent DNA. In vivo transcriptional analysis of virF mRNA shows that H-NS negatively controls the expression of virF at 30 degrees C. The expression of a virF-lacZ translational fusion in E.coli wild type and in an hns-defective derivative grown at 30 degrees or 37 degrees C and at pH 6.0 or 7.0 indicates that, in the absence of H-NS, virF expression becomes insensitive to temperature and to limited pH changes. Our results strongly suggest that H-NS controls virF expression by binding to the virF promoter and by repressing its expression at low temperature and at low pH.

Bacterial Proteins↗
PubMed1998

[The role of an electropharmacological transesophageal test in the prevention of paroxysmal atrial fibrillation. Experience with flecainide].

BACKGROUND: The management of patients with paroxysmal atrial fibrillation (AF) is unsuccessful, because AF recurs in about 50% of patients despite an antiarrhythmic treatment. Usefulness of non-pharmacological strategies is available in a limited subset of patients and it does not present a global solution to the problem. At present, treatment with antiarrhythmic agents is the only available tool in patients with AF recurrence. The aim of this study was to assess the predictive value of the electropharmacological transesophageal (TE) test in the management of patients with paroxysmal AF treated by flecainide. METHODS: In 32 patients, ranging in age from 38 to 70 years (mean: 59 +/- 12 years), with documented episodes of paroxysmal AF (mean: 5.6 +/- 3.7 episodes/last year), we performed an electrophysiological transesophageal (TE) test following pharmacological wash-out. An aggressive protocol was used: step A: 10 sec atrial burst at Wenckebach point + 10 bpm, 200 and 250 bpm; step B: 10 sec atrial bursts at 300, 400, 500 and 600 bpm; step C: 8 sec increasing rate burst from 200 to 800 bpm. Induction of sustained AF (> 1 min) was considered the end-point. Patients were treated with flecainide 100 mg bid and a second TE test was performed at the steady-state, with an identical induction protocol and end-point. Based on the response of the second test, patients were divided into responders (R Group: non-inducible AF) and non-responders (NR Group: inducible, sustained AF). Patients were followed-up by periodical controls and contacted by telephone to confirm their clinical status. RESULTS: Sustained AF was induced in 30 patients (94%) at the first TE study. Eight of them dropped-out at the time of the second TE test (6 patients for lack of consent, 1 patient for side-effects and another one for proarrhythmic effects). In the mean follow-up of 15 +/- 6 months, among patients who underwent a second TE test, AF recurrence was documented in 2 out of 14 patients from the R Group and in 7 out of 10 patients from the NR Group (p < 0.01). There were 4 AF episodes in the R Group and 19 in the NR Group (p < 0.001). We did not find significant statistical differences between the two groups in terms of age, sex, body weight, AF episodes/past year, P-wave duration, left atrial dimension, structural heart disease, AF duration at the first TE test and follow-up duration. In five patients from the NR Group with induced AF lasting > 5 min, the percentage of recurrence was 100% and there were 16 AF episodes. Global percentage of patients with recurrence was 37%. CONCLUSIONS: Flecainide is effective in reducing the incidence of AF and results are similar to other antiarrhythmic agents generally used. The electropharmacological TE test might be a useful tool to predict the response to an antiarrhythmic treatment.

Adult↗
PubMed1996

Chromosomal anomalies in newborns with omphalocele.

Omphalocele is the most common congenital abdominal wall defect; its reported incidence is 1 in 4,000 to 5,000 live births. With large defects, the liver is a median organ and lies within the sac (extracorporeal liver [ECL]). With small defects, only bowel or stomach is found outside the abdominal cavity (intracorporeal liver [ICL]). The goal of this study was to determine whether a relationship exists between the sac contents or the timing of diagnosis and the incidence of chromosomal abnormalities or survival among fetuses and newborns with omphalocele. From 1985 to 1995, 83 cases of omphalocele were managed at the authors' institution. In 50 cases the diagnosis was made using prenatal ultrasonography. All patients underwent fetal cardiac echography and amniocentesis. Twenty-four pregnancies were terminated electively because of severe associated anomalies. Of the 59 live births, 41 patients (69%) survived. The incidences of cardiac, chromosomal, and other anomalies were 24% (14), 10% (6), and 21% (16), respectively. Omphalocele with ICL is associated with a better survival rate than omphalocele with ECL (82% v 48%; P < .01) despite the significantly higher rate of karyotype abnormalities (16% v 0%; P < .05). The prognosis was poorer for patients with prenatally diagnosed omphalocele than for those with a postnatal diagnosis (mortality rate, 42% v 21%) because the former group had a higher percentage (70% v 9%) of ECL. Although the incidence of cardiac anomalies was similar for the ECL and ICL groups (33% v 18%), the former had more complex malformations. Death usually occurred in newborns who had neonatal respiratory distress owing to prematurity, or in those with chromosomal or cardiac anomalies. Chromosomal anomalies occurred mainly in cases of small omphaloceles that contained gut only, and it was the major cause of death among this group. In ECL cases, survival was primarily affected by the associated complex cardiac anomalies.

Chromosome Aberrations↗