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Biomedical subjects

A Messineo

Publications and source records attributed to A Messineo.

At least 19 recordsLinked to original sources
PubMed2003

Mutation analysis of the RET gene in total intestinal aganglionosis by wave DNA fragment analysis system.

BACKGROUND/PURPOSE: Total intestinal aganglionosis (TIA) extending from the duodenum to the rectum is the most rare form of Hirschprung's disease (HSCR) and usually is fatal. RET is the major gene associated with HSCR, and germline mutations of this gene account for up 50% of familial and up to 15 to 20% of sporadic cases in HSCR. The aim of this study was to investigate DNA variants in the RET gene in TIA patients using the WAVE DNA Fragment Analysis System. METHODS: Genomic DNA was extracted from whole blood samples from 6 patients with TIA. Polymerase chain reaction (PCR) amplification of the 21 exons of RET was performed using published oligonucleotide primers. Heteroduplexes were followed by the WAVE DNA Fragment Analysis System with the DNASep cartridge. RESULTS: WAVE system technology detected 16 variants in the RET gene in the 6 patients with TIA. Three patients had a significant mutation in exon 8, 11, and 15, respectively. Thirteen RET polymorphic variants also were detected in the 6 patients, with L746L variant in exon 13 occurring in 4 patients. CONCLUSIONS: WAVE system technology is an efficient method for the detection of DNA sequence variants. Our findings suggest that not only RET mutations but also RET polymorphic variants may contribute to the occurrence of TIA.

Amino Acid Substitution↗
PubMed2001

Lung volume reduction surgery in lieu of pneumonectomy in an infant with severe unilateral pulmonary interstitial emphysema.

A male infant with a prenatal diagnosis (at 20 weeks' gestation) of cystic adenomatoid malformation was delivered after 38 weeks' gestation (birth weight, 3 kg) and admitted to the neonatal intensive care unit. During the first few days of life, he developed mild respiratory distress; a chest radiograph and computed tomography scan showed multiple cystic areas in the left lower lobe with hyperinflation and herniation of the upper lobe across the midline. At 3 weeks of age, a left lower lobectomy was performed for presumed cystic malformation. To our surprise the pathology reports revealed pulmonary interstitial emphysema. The postoperative chest radiograph was unchanged, and mechanical ventilation was necessary and required progressively increasing ventilatory settings to provide adequate support. High-frequency oscillatory ventilation and selective right bronchus intubation failed to improve lung function. After 3 weeks, a left thoracotomy was repeated and lung volume reduction was performed with removal of 50' of the peripheral hyperinflated parenchyma. Postoperative recovery was rapid; the child was weaned from the ventilator after 3 days and discharged after 3 weeks. Follow-up chest X-rays showed a normally expanded right lung with mediastinal structures back to midline and a small left lung. Favorable results persisted at 3 years of follow-up. This first and successful experience with lung volume reduction in a neonate suggests that infants who need removal of a large portion of lung parenchyma to achieve adequate ventilation and gas exchange, lung volume reduction surgery should be considered as an alternative to pneumonectomy.

Cystic Adenomatoid Malformation of Lung, Congenita↗
PubMed2001

The treatment of internal anal sphincter achalasia with botulinum toxin.

Internal anal sphincter (IAS) achalasia is a disorder of defecation in which the IAS fails to relax. Botulinum toxin (BT), which has been successfully used to relax the anal and lower esophageal sphincters, was injected twice into the IAS of one adolescent and three infants with manometric, radiologic, and in 2 cases histochemical diagnosis of anal achalasia: in the adolescent a third injection was necessary. Spontaneous defecation was achieved in all patients following the second injection. In one case a diagnosis of short-segment Hirschsprung's disease was obtained after the second injection. Local infiltration of BT into the IAS proved effective in the treatment of IAS achalasia. Double-blind studies and longer follow-up periods are needed to better evaluate these preliminary results and define the limits of this promising therapy.

Botulinum Toxins, Type A↗
PubMed2001

Glomerular permeability after surgical trauma in children: relationship between microalbuminuria and surgical stress score.

OBJECTIVES: To determine whether there is an increase of urinary albumin during and after surgical trauma and investigate a possible relationship between microalbuminuria and the severity of surgical stress. DESIGN: Prospective study. SETTING: University hospital pediatric intensive care unit. PATIENTS: Forty consecutive children scheduled for elective surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Microalbuminuria/urinary creatinine ratio (MACR) was measured before, during, and after elective moderate or major surgical procedures. The Oxford Surgical Stress Score (SSS) was determined for each patient at the end of the operation, and its relationship with maximum deviation of MACR from baseline values was investigated. MACR showed a progressive increase during surgery and a decrease afterward, reaching preoperative values in most cases within 24 hrs after the end of surgery. There was a significant correlation between the increase in MACR and severity of the surgical trauma as measured by SSS. Two patients showed a rise in MACR after the initial postoperative normalization before clinical appearance of a surgical complication and one patient showed a persistent rise in MACR before clinical appearance of a septic complication. None of the other patients showed any rise in MACR after postoperative normalization, and they all had an uneventful recovery. CONCLUSIONS: MACR rises during and after major or moderate elective surgery in children. There is a significant positive correlation between severity of surgical trauma and capillary permeability in pediatric patients. Microalbuminuria, as an index of capillary permeability, may be an early sign of incipient complications and assist in the identification of those patients whose condition will deteriorate. The test is a cheap, blood-sparing, easy-to-perform bedside procedure that may have a useful role in clinical practice for evaluating the effect of surgical trauma on capillary permeability in children.

Albuminuria↗
PubMed1999

Pulmonary barotrauma in congenital diaphragmatic hernia: a clinicopathological correlation.

BACKGROUND/PURPOSE: The high mortality rate in congenital diaphragmatic hernia (CDH) has been ascribed to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). One of the principal treatment strategies has been the use of hyperventilation to reverse ductal shunting, but the wisdom of this approach is being questioned because of parenchymal lung injury from high inflation pressures. The authors hypothesize that the use of hyperventilation to reverse or prevent ductal shunting would result in ventilator-induced lung injury, which would be evident on postmortem examination. A retrospective review of clinical and autopsy information was conducted. METHODS: Clinical and autopsy information gathered for a previously published series of 223 infants with CDH presenting in the first 24 hours of life was reviewed. Autopsy and clinical data were analyzed from 68 of 101 nonsurvivors who died with severe hypoxemia. RESULTS: Sixty-two of 68 cases (91%) had evidence of diffuse alveolar damage and hyaline membrane formation, which was more evident in the ipsilateral lung. Forty-four (65%) infants had pneumothoraces, and 4 infants had interstitial fibrosis. Pulmonary hemorrhage was seen in 35 cases (50 maximum peak inspiratory pressure [mean +/- SD] was 40.4+/-7.9 cm H2O and lowest modified ventilatory index [respiratory rate x peak airway pressure] was 2323+/-836). The degree of pulmonary hypoplasia was evaluated by lung weight with the ratio of the observed combined lung weight to the expected lung weight based on birth weight and gestational age. The ratio based on birth weight was 57%+/-25%, and the ratio based on gestational age was 60%+/-26%. Twenty-one infants (35%) had nonpulmonary anomalies. The most significant was a 10% incidence of congenital heart disease. Apart from this, lethal nonpulmonary anomalies were rare. CONCLUSION: These results suggest that lung injury secondary to mechanical ventilation plays an important role in the mortality rate of patients with CDH, which may become increasingly significant when there is underlying pulmonary hypoplasia.

Barotrauma↗
PubMed1998

Inflammatory pseudotumors of the lung in children: conservative or aggressive approach?

BACKGROUND: Inflammatory pseudotumors (inflammatory myofibroblastic tumors) of the lung are myofibroblastic lesions of controversial nosology. The concept that these are benign lesions has been recently questioned given their capacity for local invasion and recurrence. PROCEDURE AND RESULTS: We observed five children with inflammatory pseudotumors of the lung in whom the tumor was resected using the most limited excision possible. Unusually, in all five cases the tumor was localized in the right upper lobe: Three were parenchymal, and the others involved the bronchus. The three parenchymal masses underwent wedge resection, whereas the two bronchial lesions required ex-cision followed by a bronchoplasty and an up-per lobectomy, respectively. In all, the pathology showed a complete resection, and the patients had uneventful recoveries. Follow-ups, including CT and bronchoscopies, ranged from 4 to 8 years (mean of 6.4 years) with no signs of recurrence. CONCLUSIONS: Our limited experience supports the idea that excisional surgery is the treatment of choice for inflammatory pseudotumors of the lung. Such excisions, both diagnostic and curative, carry minimal risks and avoid unnecessary mutilation. Closely monitored follow-up is mandatory, as the natural history of this lesion is not yet well understood.

Adolescent↗
PubMed1997

Endoscopic dilation of acquired tracheobronchial stenosis in infants.

Acquired tracheobronchial strictures in infants are associated with significant morbidity. Their treatment requires different types of therapeutic procedures depending on the severity and location of the stenosis. We successfully dilated five children (ages between 1 and 23 months) with acquired tracheobronchial (stenoses two carinal and three of the mainstem bronchi) using a new technique. Gruentzig balloon catheters of different caliber were introduced into the tracheal lumen of apneic children under endoscopic vision. Three infants required four dilations and in the other two cases only two procedures were necessary. One complication occurred during dilation and resulted in a pneumothorax caused by a partial-bronchial disruption. At follow-up (range, 18-36 months; mean, 23.4 months) all five children were clinically improved. In the four cases without complications the chest x-rays were normal and the last bronchoscopy showed tracheobronchial trees with normal calibers. The child who developed a pneumothorax during attempted dilation recovered. In this child, dilation of the right upper lobe bronchus was unsuccessful. Dilation was attempted without visualization of the bronchus and the stricture. Our limited experience confirms that pneumatic balloon catheters can successfully treat acquired tracheobronchial stenoses in infants. To avoid complications, strict criteria need to be applied in the selection of cases with tracheobronchial stenoses.

Bronchi↗
PubMed1997

Congenital diaphragmatic hernia--a tale of two cities: the Toronto experience.

PURPOSE: The optimal therapy for congenital diaphragmatic hernia (CDH) is evolving. This study analyzes the results of treatment of CDH in a large tertiary care pediatric center using conventional and high-frequency oscillatory ventilation (HFOV) without extracorporeal membrane oxygenation (ECMO) contrasting these with a parallel study from a similar large urban center using conventional ventilation with ECMO. METHODS: Between 1981 and 1994, 223 consecutive neonates who had CDH diagnosed in the first 12 hours of life were referred for treatment before repair. Conventional ventilation was used with conversion to HFOV for refractory hypoxemia or hypercapnia, and a predicted near 100% mortality rate. ECMO was used in only three patients, all of whom died. A retrospective database was collected. Thirty-one clinical variables were tested for their association with the outcome. Common ventilatory and oxygenation indices were tested for their prognostic capability. RESULTS: Apgar scores, birth weight, right-sided defects, pneumothorax, total ventilatory time, and the use of high frequency oscillatory ventilation were the only variables associated with outcome. A modified ventilatory index and postductal A-aDo2 were strong prognostic indicators. From 1981 to 1984 surgery was performed on an emergency basis. Since 1985 surgery was deferred until stabilization had been achieved. This resulted in a shift in the mortality from postoperative to preoperative with no change in total survival. HFOV did not alter the overall survival. Results of autopsies performed (70%) showed significant pulmonary hypoplasia and barotrauma as the primary causes of death. The survival was 54.7%. CONCLUSION: Conventional ventilation with HFOV produced equal survival to conventional ventilation with ECMO in two comparable series. Pulmonary hypoplasia was the principle cause of death. This continued high mortality at both centers suggests that new therapies are required to improve outcomes.

Extracorporeal Membrane Oxygenation↗
PubMed1995

A simple exam to screen superficial masses: fine-needle aspiration cytology.

Fine-needle aspiration cytology (FNAC) is now considered a useful tool in the evaluation of adult patients with mass lesions. We reviewed the experience with FNAC in our Paediatric Surgical Department. One hundred and eleven FNACs were performed in children with a superficial mass and no definite diagnosis. All the exams were done by the same physician using a fine needle and no anesthesia. We routinely used May-Grüwald-Giemsa and Papanicolaou staining. Patients age ranged from 20 days to 17 years, with a mean age of 6.5 years. A clinically benign pathology was cytologically confirmed in 90 cases (81%). All children did well at follow-up. Malignancy was diagnosed in eight cases (7.2%) and in all was confirmed with a surgical biopsy. In nine children (8.1%) the specimen was considered insufficient for definite diagnosis. In four cases (3.6%) the pathologist diagnosed a possible malignancy that was excluded at surgical biopsy. The sensitivity was 100% and the specificity was 96%. Our experience confirms that FNAC is a fast, cheap, simple, and accurate diagnostic method and should be used for screening in all children with doubtful superficial masses.

Adolescent↗
PubMed1995

The safety of home tracheostomy care for children.

Home tracheostomy care avoids prolonged hospitalization for tracheostomized children who must remain cannulated for a long time; however the safety aspects of this form of management are still under discussion. Since 1987 the authors have tutored parents in tracheostomy care. Once parental training was complete and the necessary equipment (aspirator, humidifier, etc) provided, children in stable condition were discharged and had period follow-up as outpatients. Thirty-four children had a total of 710 months in home care; 11 of them had decannulation. The only reported complications were two partial obstructions of the cannula and two accidental decannulations. The low number of documented accidents and the parents' profound appreciation in having their children at home indicate that patients with a tracheostomy in need of long-term care can be safely managed at home.

Accidents↗
PubMed1994

Tracheoplasty without stent, using preshaped cryopreserved cartilage allografts in neonatal pigs.

The management of congenital and acquired laryngotracheal stenosis in children often includes tracheoplasty with different materials. Autologous cartilage is the most commonly described graft for tracheal reconstruction. In 12 1-week old piglets a tracheal defect was created and repaired using cryopreserved allografts sculpted before preservation into a newly designed shape. In half the animals (group 1), a free pericardial autograft was interposed on the luminal surface of the cartilage graft. A stent was not used in any animal. The animals grew normally and were killed after 2 months. The grafted tracheas showed maintenance of their structural support, and no evidence of narrowing of the grafted area in comparison to the trachea above the graft. The lumen was lined by ciliated respiratory epithelium. There was no identifiable pericardium at the time of sacrifice and no differences between the two groups. This study shows that in neonatal pigs, cartilage allografts, sculpted before cryopreservation, are a suitable alternative to autografts to cover tracheal defects and can be used without stenting. No advantages were identified when interposing a free pericardial graft.

Animals↗
PubMed1994

Tracheomalacia.

Tracheomalacia describes a generalized or localized weakness of the trachea that results in excessive narrowing of the tracheal lumen during expiration or whenever intrathoracic pressure increases. Severe forms of tracheomalacia are characterized by life-threatening apneic spells, inability to extubate the airways, and episodic pneumonia. Aortopexy has proved to be an effective method of treatment for most of these conditions when seen in children. When aortopexy fails to prevent tracheal obstruction, a support applied to the external circumference of the trachea can be useful to prevent airway collapse.

Child↗
PubMed1992

Juvenile hemangiomas involving the thoracic trachea in children: report of two cases.

Two 3-month-old girls presented with posterior mediastinal juvenile hemangioma (JH), a benign tumor rarely found in the mediastinum. Incomplete resections were performed. Over 4 and 7 months, respectively, the hemangiomas recurred and grew through the tracheal wall into the tracheal lumina, reducing the airway by 90% in one case and 70% in the other. The recurrences were resected, including five tracheal rings in the first case and four in the second. In case 1 the tumor recurred in the trachea 2 months later. A tracheostomy was performed, and corticosteroids were administered for 6 months. The tumor involuted and the patient was decannulated after 18 months; she is doing well 7 years later. The patient in case 2 has been well in the 18 months since her second operation. Airway obstructions can be treated by tracheal resection when conservative management fails.

Airway Obstruction↗
PubMed1992

Clinical factors affecting mortality in children with malrotation of the intestine.

In children with symptoms secondary to malrotation of the intestine, a retrospective statistical study was undertaken to identify factors associated with an increased risk of mortality. Between 1964 and 1989, laparatomy was performed on 182 children. For study purposes, the children were divided into three groups. Group I included 71 patients with an obstruction only in the duodenum. The remaining 111 children, all of whom had midgut volvulus, were further divided; those without gut necrosis (79) into group II, and those with necrosis (32) into group III. One child each died in groups I and II, and 15 in group III. In all children we evaluated the relationship between mortality and age at presentation, presence of associated serious abnormalities, time from onset of symptoms to surgery, and the presence of necrotic bowel. For group III, we considered the influence of percentage of bowel resected on mortality. In these 182 children the factors associated with an increased risk of mortality were presence of necrosis (P < .0001), presence of other abnormalities (P = .0008), and younger age (P = .0084). Time from onset of symptoms to surgery was not associated with statistically increased risk of mortality. The 1% mortality noted in children without intestinal necrosis (group I and II) was related to associated abnormality. For group III the estimated probability of survival ranged from .999 for patients with 10% of intestinal necrosis to .351 for whose with 75% of intestinal necrosis, assuming the best prognostic conditions (patient older than 3 months with no associated serious abnormalities).

Abnormalities, Multiple↗
PubMed1992

Repair of long tracheal defects with cryopreserved cartilaginous allografts.

Tracheoplasties with various autografts (cartilage, periosteum, pericardium) have been used in the treatment of long-segment tracheal stenosis. Previous studies have shown that cartilage allografts survive transplantation on a long-term basis in various sites of the body. In this study we set out to determine if cryopreserved cartilage and cryopreserved tracheal allografts would survive when used to cover tracheal defects in animals. A rectangular defect (2.8 +/- 0.3 cm long and incorporating 50% of tracheal circumference) was created in the thoracic trachea of 18 piglets. The defect was covered with the excised tracheal segment in 6 (group A, control group), with a cryopreserved tracheal allograft in 6 (group B), and with a cryopreserved cartilage allograft harvested from the scapula in 6 (group C). The allografts were cryopreserved, by a standard slow-freezing technique, at -80 degrees C for more than 21 days. All animals survived the grafting procedure and were killed after 2 months. None had signs of airway obstruction. Using the trachea above the defect as the standard, the mean sagittal narrowing of the airway in the repaired trachea was 0.4 mm in group A, 0.7 mm in group B, and 0.6 mm in group C; the coronal diameter in normal and grafted trachea was similar. The lumen of all grafts was lined by regenerating respiratory epithelium, and cilia were seen in many. Some cartilage was reabsorbed in group A and B but cartilage islands were present in all. In group A, reabsorption of cartilage was minimal. These findings suggest that segments of trachea or cartilage allografts can be cryopreserved, stored, and, subsequently, used when necessary for tracheoplasty.

Animals↗
PubMed1992

Severe tracheomalacia associated with esophageal atresia: results of surgical treatment.

This study reviews our experience from 1977 to 1991 with 32 children born with esophageal atresia and tracheoesophageal fistula (EA-TEF) who later underwent surgery for severe tracheomalacia. Indications for surgery included dying spells (21), inability to extubate the airway (nasotracheal tube 5, tracheotomy 3), and recurrent pneumonia (3). Diagnosis was confirmed by chest x-ray, esophagogram, and bronchoscopy. Aortopexy was performed in 31 of 32 children; a splint without aortopexy was used in one older girl. Splinting was also necessary in two of the 31 at the initial operation, when aortopexy failed to prevent tracheal collapse. There were four initial failures. Currently, 29 children are well (median follow-up, 6.6 years). Two have a tracheostomy in place, and one, who had a vascular ring divided after aortopexy, died at home of unknown cause 1 month later. These findings indicate that aortopexy provides long-term relief of severe symptoms of tracheomalacia associated with EA-TEF in almost all affected children. When aortopexy fails, the insertion of an airway splint may succeed; otherwise, tracheostomy is necessary.

Aorta↗