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Many patients with illnesses that once were fatal at birth or during childhood now survive into adult life. This article considers four respiratory illnesses of early life in which long-term survival now occurs frequently: cystic fibrosis, diaphragmatic hernia, esophageal atresia-tracheoesophageal fistula, and bronchopulmonary dysplasia. In cystic fibrosis, although the median age at death is now 25 years, chronic pulmonary infection due ultimately to the abnormal composition and clearance of airway mucus is still the usual cause of death. Earlier survivors of congenital diaphragmatic hernia had only minor diminution of perfusion and ventilation of the lung on the side of the hernia as adolescents or young adults; however, as infants with greater degrees of pulmonary hypoplasia have successful repair of their hernias, more long-term respiratory impairment will probably be found. The esophageal atresia tracheoesophageal fistula complex leaves all esophagi and many tracheas permanently abnormal; recurrent aspiration, repeated pneumonia, and an unduly collapsible trachea are the result, although symptoms may be few. Survivors of bronchopulmonary dysplasia have decreased exercise capacity, wheezing, and recurrent pneumonia, although their chest radiographs may become normal or almost normal.
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PMID:Caldwell Lecture. Respiratory problems of early life now allowing survival into adulthood: concepts for radiologists. 835 26

Currently two genetic pulmonary disorders can be diagnosed before birth: alpha-1-antitrypsin deficiency and mucoviscidosis. For the latter there are two possible diagnostic techniques: first a study of the intestinal enzymes of the amniotic fluid, a reliable method only at the 18th week, and also a study of DNA markers (ADN) of the trophoblastic cells using molecular biological techniques: this can be performed from the 10th to 11th week of pregnancy but presupposes a family study in which there is already a subject suffering from the disorder. Foetal echocardiography enables various pulmonary abnormalities to be detected: pleural effusion, cyst, pulmonary hypoplasia and other disorders. This technique however has some limits, at least at present. Most often these severe malformations are revealed at birth such as respiratory distress or stillbirth. Adenomatous cystic malformations or congenital lobar emphysema, a posterior diaphragmatic hernia, and oesophageal atresia with oesophagotracheal fistula are the most frequent and are curable surgically.
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PMID:[Prenatal diagnosis and genetic diseases of the lung]. 326 Oct 24

In recent years patients with cystic fibrosis (CF) have experienced longterm survival and have demonstrated a number of intra-abdominal complications. This report evaluates the intra-abdominal complications seen in 69 of 189 children with cystic fibrosis from 1972 to 1983. Forty-one patients were boys and twenty-eight girls. Complications occurred in 36 neonates, with meconium ileus (MI) noted in 33 and giant cystic meconium peritonitis (GCMP) in 3. Meconium ileus equivalent occurred in seven older children presenting with bowel obstruction. In addition, rectal prolapse occurred in 12, inguinal hernia in 10, intussusception in 3, cholelithiasis in 3, GE reflux in 4, stress ulcer in 1 and appendicitis in 1. Three infants with GCMP survived resection and enterostomy. Infants with MI were divided into simple (15) or complicated (18) cases. Nonoperative therapy using gastrografin enema was successful in three of eight with simple MI. Operative enterotomy and irrigation was successful in three cases while resection and enterostomy was done in nine. MI was complicated by atresia, volvulus and/or perforation in 18 cases requiring resection and anastomosis or enterostomy. Survival for MI was 86% compared to 36% in 25 MI patients treated in the previous two decades. Meconium ileus equivalent was successfully managed using gastrografin enema in five of seven children. Only 3 of 12 children with rectal prolapse required repair. Two cases of intussusception were reduced while one required resection. Three of 10 children had hernia recurrence due to chronic pulmonary problems.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Intra-abdominal complications of cystic fibrosis. 404 71

Is an acute bronchial, obstructive disease of the infant caused mainly by the respiratory syncytial virus. It appears epidemically preceded by infections of the upper respiratory ducts, followed by coughing, dysnea, expiratory sibilants, suprasternal and subcostal during inspiration and radiologic evidences of choneking. In the differential diagnosis the physician must consider pulmonary dysgenesis, diaphragmatic hernia, congenital lobar emphysema, congenital cardiopathy, pneumothorax, obstruction due to foreign body, asthmatic crisis and fibrocystic disease. Fundamentally, two diagnoses should be discarded: 1) dyspenic bacterial bronchopneumonic syndrome; 2) prime infection T. B. bronchopneumonia with bronchiolitic syndrome.
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PMID:[Bronchiolitis]. 742 29

Lobar transplantation represents a therapeutic option for children and some adults with severe end-stage pulmonary disease. Six patients including two neonates, three children, and one adult underwent lobar transplantation. Ages ranged from 17 days to 21 years. Transplant procedures were unilateral in the neonates and two of the children and bilateral in the child and adult who had cystic fibrosis. The donor lobes were from cadavers in the two neonates and living related donors in the children and the adult. Unilateral grafts involved use of the right upper lobe in the 12-year-old patient with bronchopulmonary dysplasia; right middle lobe with a ventricular septal defect repair in the 4-year-old patient with Eisenmenger's syndrome, left upper lobe in the 28-day-old patient with primary pulmonary hypertension, and the right upper and middle lobes in the 17-day-old patient with diaphragmatic hernia. Bilateral lobar transplantations were performed with the right lower and left lower lobes in the two patients with cystic fibrosis (aged 13 and 21 years). The two neonates underwent emergency transplantation with the use of extracorporeal membrane oxygenation as a bridge. Perioperative survival was 83%, with only the 4-year-old patient with ventricular septal defect/Eisenmenger's syndrome dying early. No airway complications were observed. The unilateral grafts received most of the blood flow as shown by perfusion scanning (range 74% to 99%). Living related donor complications included prolonged air leaks (> 6 days) in two patients. In urgent situations, such as an infant requiring extracorporeal membrane oxygenation, and in the existing milieu of donor shortage, lobar transplantation (living related or cadaveric) is a surgically feasible procedure and can provide a donor source in the limited time frame of these clinical situations. Bilateral lobe transplantation may be a viable option for patients with cystic fibrosis and life-threatening respiratory decompensation.
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PMID:Lobar transplantation. Indications, technique, and outcome. 807 33

We report data from 25 microsurgical aspirations of the epididymis on 22 men. There were 14 men with congenital absence of the vas, 6 with failed vasoepididymostomy, 1 with adult cystic fibrosis and 1 with a childhood hernia repair. The specimens were used for assisted reproductive technologies, including in vitro fertilization and tubal embryo transfer. The protocol for aspiration, ovulation induction and sperm processing evolved during the course of these studies, and the patients were classified into 2 groups on the basis of methodology. Seventeen procedures were performed for the in-house in vitro fertilization team but 8 other procedures were done for nearby in vitro fertilization centers, and the final prepared sperm samples were transported as part of our mobile program. Group 1 patients underwent standard aspiration techniques, standard ovulation induction and sperm processing by wash and swim up. Among this group there were no fertilizations or pregnancies with 8 in-house and 2 transported specimens. Group 2 patients had leuprolide suppression before ovulation induction, direct intratubular aspiration and a complex sperm preparation, including pentoxifylline stimulation, mini-Percoll filtration and incubation with human follicular fluid. Among this group there were 2 fertilizations and 1 pregnancy with 9 in-house cases, and 3 fertilizations and 2 pregnancies with 6 transported specimens. These results suggest that a mobile program for microsurgical aspirations of sperm from the epididymis and in vitro fertilization or tubal embryo transfer is feasible within the framework of a strict protocol.
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PMID:Microsurgical aspiration of sperm from the epididymis: a mobile program. 847 39

When infants with recurrent wheezing have a clinical course inconsistent with asthma, an extensive list of alternative diagnoses needs to be considered. Anatomic malformations, such as congenital heart disease, laryngotracheomalacia, and diaphragmatic hernia, should be considered for immediate medical stabilization and early surgical correction. Life-threatening infections such as bacterial epiglottitis, retropharyngeal cellulitis, and viral myocarditis require prompt intervention. A careful history and physical examination reveal important diagnostic clues that, in this case, prompted a directed evaluation to rule out common masqueraders of asthma such as foreign body aspiration, cystic fibrosis, gastroesophageal reflux, viral pneumonitis, or pulmonary tuberculosis. On occasion, such a search is unrevealing and a diagnostic challenge remains. In those situations, judicious use of modern technology to scrutinize anatomic (high-resolution computed tomography) and functional (infant pulmonary function tests) pathology, and justifiable invasive procedures such as bronchoscopy and lung biopsy, uncover the true diagnosis, allowing for optimal management.
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PMID:A wheezy infant unresponsive to bronchodilators. 916 57

The spectrum of surgical diseases in patients with cystic fibrosis (CF) has not been comprehensively studied. A retrospective review of 792 consecutive patients with CF presenting over a 25 year period (1970-1994) was made to determine the incidence of operations, procedures performed, complications encountered, and impact on physical development and pulmonary function tests (PFTs). A total of 191 operations were performed on 130 (16%) of the 792 patients; 98 operations (51%) were abdominal, 58 (30%) thoracic, and 31 (16%) hernias; 64 were male, and 66 female; average age was 14 +/- 10 years. Complications occurred in 12 (16%); 9 deaths were from progressive respiratory failure, 2 from superficial wound infections, and 1 from an episode of line sepsis. In the first 15 years, 9 complications occurred in 126 operations vs. 3 in 73 operations during the last 10 years. Operations were classified as emergent, urgent, or elective. Of the 9 deaths, 8 occurred after emergent or urgent operations (4 abdominal and 4 thoracic), while 1 death occurred following elective herniorrhaphy. For each subgroup, (abdominal, thoracic, and hernia), there was no difference in height/weight indicies, peak flow, forced vital capacity (FVC), forced expired volume in 1 sec (FEV(1)), or FEV(1)/FVC ratio when comparing 1 year preoperation and 1 year postoperation. In conclusion, patients in this high-risk population were operated on with few complications, but when a complication occurred it tended to be pulmonary and fatal (4.7% of all operations). Furthermore, operations did not cause significant deteriorations in PFTs and they did not cause these children to fall off their expected age-adjusted growth curves.
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PMID:Surgical experience in patients with cystic fibrosis: a 25-year perspective. 1180 46

Acute appendicitis is rare in term neonates. In most of the reported cases, it is seen as a complication of necrotizing enterocolitis, Hirschsprung's disease, cystic fibrosis, meconium plug, inguinal hernia, umbilical hernia, Group B Streptococcal septicemia and chorioamnionitis. A surviving term male newborn with isolated acute appendicitis with perforation is reported. A high index of suspicion of acute appendicitis, early surgery and the importance of a thorough search for a perforation in cases of neonatal acute abdominal distention is stressed. Literature of this rare condition is reviewed briefly.
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PMID:Perforated acute appendicitis in a term neonate. 1510 22

A 34-year-old woman with cystic fibrosis presented with bilateral femoral hernias, which were found to be retrovascular at operation. The hernias were not amenable to conventional open or laparoscopic repair, and were repaired using pre-peritoneal mesh inserted deep to transversalis fascia. The anatomical basis and management of uncommon variants of femoral hernia are discussed.
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PMID:Bilateral retrovascular femoral hernia. 1863 43


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