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1.
Mar Environ Res ; 193: 106293, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38103302

ABSTRACT

Here we provide evidence, along an 8-year period time-series based on multifaceted data from a Mediterranean marine protected area (MPA), whether protection can tackle invasive and range expanding herbivore fishes, and their effects on the algal resource availability, taking into account the population trends of predatory fishes, fisheries catches of herbivore fishes and sea surface temperature (SST) through time. Our findings pointed out that an ineffective in restoring top-down control process MPA may facilitate, rather than alleviate, the sudden and enduring population burst of invasive and range-expanding herbivorous fishes at tipping points of abrupt change. This subsequently results in the deterioration of rocky reef habitats and the depletion of algal resources, with the tipping points of abrupt change for algal and herbivore fish species not overlapping chronologically. As sea temperature increases, ineffective or recently established MPAs may inadvertently facilitate the proliferation of invasive and range-expanding species, posing a significant challenge to management effectiveness and conservation objectives.


Subject(s)
Ecosystem , Herbivory , Animals , Fishes , Fisheries , Temperature , Conservation of Natural Resources
2.
Curr Nutr Rep ; 12(3): 527-544, 2023 09.
Article in English | MEDLINE | ID: mdl-37213054

ABSTRACT

PURPOSE OF REVIEW: Polycystic ovary syndrome (PCOS) is the most common endocrine and metabolic disorder in women of reproductive age worldwide. This disease causes menstrual, metabolic, and biochemical abnormalities such as hyperandrogenism, oligo-anovulatory menstrual cycles, polycystic ovary, hyperleptinemia, insulin resistance (IR), and cardiometabolic disorders, often associated with overweight or obesity and visceral adiposity. RECENT FINDINGS: The etiology and pathophysiology of PCOS are not yet fully understood, but insulin seems to play a key role in this disease. PCOS shares an inflammatory state with other chronic diseases such as obesity, type II diabetes, and cardiovascular diseases; however, recent studies have shown that a healthy nutritional approach can improve IR and metabolic and reproductive functions, representing a valid therapeutic strategy to ameliorate PCOS symptomatology. This review aimed to summarize and collect evidence about different nutritional approaches such as the Mediterranean diet (MedDiet) and the ketogenic diet (KD), as well as bariatric surgery and nutraceutical supplementation as probiotics, prebiotics, and synbiotics, among the others, used in patients with PCOS.


Subject(s)
Diabetes Mellitus, Type 2 , Hyperandrogenism , Insulin Resistance , Polycystic Ovary Syndrome , Humans , Female , Polycystic Ovary Syndrome/complications , Polycystic Ovary Syndrome/drug therapy , Hyperandrogenism/complications , Obesity/complications
3.
Environ Int ; 161: 107143, 2022 03.
Article in English | MEDLINE | ID: mdl-35176575

ABSTRACT

With the advent of the SARS-CoV-2 pandemic, Wastewater-Based Epidemiology (WBE) has been applied to track community infection in cities worldwide and has proven succesful as an early warning system for identification of hotspots and changingprevalence of infections (both symptomatic and asymptomatic) at a city or sub-city level. Wastewater is only one of environmental compartments that requires consideration. In this manuscript, we have critically evaluated the knowledge-base and preparedness for building early warning systems in a rapidly urbanising world, with particular attention to Africa, which experiences rapid population growth and urbanisation. We have proposed a Digital Urban Environment Fingerprinting Platform (DUEF) - a new approach in hazard forecasting and early-warning systems for global health risks and an extension to the existing concept of smart cities. The urban environment (especially wastewater) contains a complex mixture of substances including toxic chemicals, infectious biological agents and human excretion products. DUEF assumes that these specific endo- and exogenous residues, anonymously pooled by communities' wastewater, are indicative of community-wide exposure and the resulting effects. DUEF postulates that the measurement of the substances continuously and anonymously pooled by the receiving environment (sewage, surface water, soils and air), can provide near real-time dynamic information about the quantity and type of physical, biological or chemical stressors to which the surveyed systems are exposed, and can create a risk profile on the potential effects of these exposures. Successful development and utilisation of a DUEF globally requires a tiered approach including: Stage I: network building, capacity building, stakeholder engagement as well as a conceptual model, followed by Stage II: DUEF development, Stage III: implementation, and Stage IV: management and utilization. We have identified four key pillars required for the establishment of a DUEF framework: (1) Environmental fingerprints, (2) Socioeconomic fingerprints, (3) Statistics and modelling and (4) Information systems. This manuscript critically evaluates the current knowledge base within each pillar and provides recommendations for further developments with an aim of laying grounds for successful development of global DUEF platforms.


Subject(s)
COVID-19 , Wastewater-Based Epidemiological Monitoring , COVID-19/epidemiology , Global Health , Humans , Pandemics , SARS-CoV-2 , Wastewater
4.
Environ Pollut ; 266(Pt 1): 115213, 2020 Nov.
Article in English | MEDLINE | ID: mdl-32688110

ABSTRACT

Abundance, composition, and distribution of macro-litter found on the seafloor of the Strait of Sicily between 10 and 800 m depth has been studied using data collected by bottom trawl surveys MEDITS from 2015 to 2019. Three waste categories based on the items use were considered: single-use, fishing-related and generic-use. Over 600 sampling sites, just 14% of these were litter-free. The five-years average density of seafloor litter was 79.6 items/km2 and ranged between 46.8 in 2019 and 118.1 items/km2 in 2015. The predominant waste type was plastic (58% of all items). Regardless of material type, single-use items were a dominant (60% of items) and widespread (79% of hauls) fraction of litter with a mean density of 48.4 items/km2. Fishing-related items accounted for 12% of total litter items. Percentage of dirty hauls and litter density increased with depth. Analysis of the relation density-depth indicates a progressive increase of litter density beyond depth values situated within the interval 234-477 m depending on the litter category. A significant decrease in litter density by categories was observed over the period. Patterns of spatial distribution at the higher depths (200-80 0m) resulted stable over the years. Density hotspots of fishing-related items were found where the fishing activity that uses fish aggregating devices (FADs) is practised and in the proximity of rocky banks. Single-use and generic-use objects densities were greater on the seafloor along main maritime routes than other areas. Comparisons between the percentage of hauls littered with anthropic waste from the mid-1990s against those in 2018-19 highlighted an increase of about 10.8% and 15.3% for single-use items and fishing-related items respectively, and a decrease of 18.6% for generic-use items. This study provides a snapshot of the current situation of littering in the central Mediterranean Sea and represents a solid baseline against which the effectiveness of current and future mitigation strategies of the litter impact on marine environment can be measured.


Subject(s)
Environmental Monitoring , Waste Products/analysis , Animals , Mediterranean Sea , Plastics , Sicily
5.
Gen Comp Endocrinol ; 290: 113393, 2020 05 01.
Article in English | MEDLINE | ID: mdl-31982398

ABSTRACT

In the lacertid Podarcis siculus the reproductive cycle is typically biphasic, with alternate recrudescence (Spring and Fall) and resting (Summer and Winter) phases. This study aimed to shed some light on the role exerted by progesterone during the two recrudescence periods; to this purpose, exogenous progesterone was administered intraperitoneally and the effects on oogonial proliferation, oocyte recruitment, and follicle cells apoptosis were determined. The presence and distribution of progesterone receptors was also investigated by immunohistochemistry and western blotting. Results indicate that progesterone would play different roles and follow different route of action in the two recrudescence periods thus confirming the complexity of the mechanisms controlling oogenesis in this species of vertebrate.


Subject(s)
Lizards , Ovary/physiopathology , Progesterone/metabolism , Animals , Female , Recurrence , Seasons
7.
Commun Agric Appl Biol Sci ; 78(2): 55-63, 2013.
Article in English | MEDLINE | ID: mdl-25145225

ABSTRACT

Chemical and eating quality of citrus fruit changes slowly after harvest, and quality alteration is mainly due to shrinkage, loss of firmness, excessive weight loss and decay rather than a reduction of nutritional value and taste features. Film wrapping may be a suitable means to reduce transpiration and preserve market quality provided film permeability to gases does not lead to: 1) a reduction of in-package O2 partial pressure at a point that would induce anaerobic respiration; 2) an increase of CO2 concentration to toxic levels. This experiment was carried out to study quality changes of 'Sanguinello' oranges treated or not treated with 500 mg/L imazalil (IMZ) and wrapped with continuous, macro- or micro-perforated polyolefinic films. Wrapped and no-wrapped fruit were stored at 20 degrees C and 60% RH for 20 or 30 days. In-package gas composition of the macro-perforated film showed no significant difference compared to air composition, while in-package partial pressure of CO2 and O2 ranged between 4 (continuous film) and 9.8 kPa (micro-perforated films), and 14.8 (continuous film) and 5 kPa (micro-perforated films), respectively. After 30 days of storage weight loss in fruit wrapped with the macro-perforated film was (4.3%) slightly lowerthan un-packed fruit (5%), while in all other packages weight loss never exceeded 0.7%.Quality changes were quite stable over storage in all treatments, although slight but significantly lower levels of total soluble solids and ascorbic acid were detected in micro-perforated films with the lowest degree of perforation. However, the sensory analysis denoted a remarkable decrease of firmness in un-wrapped or wrapped fruit with macro-perforated film, while a moderate build-up of off-flavour, which reduced the eating quality, developed in micro-perforated films. Decay ranged between 6 and 12% in not treated fruit, with the lowest incidence detected in un-wrapped fruit, whereas differences among the different films were not significant. No decay was detected in fruit treated with IMZ. 'Sanguinello' oranges can be stored under retail conditions for a month by the only means of film wrapping without important changes in chemical, eating and marketing quality provided fruit are treated with an effective fungicide to prevent decay and that in-package gas composition is not markedly changed with respect to air.


Subject(s)
Citrus sinensis/chemistry , Food Packaging/instrumentation , Food Packaging/methods , Fruit/chemistry , Plastics/chemistry , Food Packaging/economics , Food Preservation , Food Storage , Fruit/economics , Humans , Permeability , Quality Control , Taste
8.
J Fish Dis ; 29(3): 157-66, 2006 Mar.
Article in English | MEDLINE | ID: mdl-16533301

ABSTRACT

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.


Subject(s)
Fish Diseases/microbiology , Genome, Bacterial/genetics , Vibrio/genetics , Vibrio/pathogenicity , Virulence Factors/genetics , Bacterial Capsules/genetics , Databases, Nucleic Acid , Exotoxins/genetics , Genome, Bacterial/physiology , Genomic Library , Genomics/methods , Molecular Sequence Data , Sequence Homology, Amino Acid , Virulence Factors/classification
9.
J Pediatr Surg ; 38(5): 814-8, 2003 May.
Article in English | MEDLINE | ID: mdl-12720200

ABSTRACT

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.


Subject(s)
Crohn Disease/surgery , Intestinal Obstruction/surgery , Adolescent , Adult , Colon/surgery , Colonic Diseases/etiology , Colonic Diseases/surgery , Crohn Disease/complications , Digestive System Surgical Procedures/methods , Female , Humans , Ileal Diseases/etiology , Ileal Diseases/surgery , Ileum/diagnostic imaging , Ileum/surgery , Intestinal Obstruction/etiology , Male , Radiography , Retrospective Studies
10.
J Pediatr Surg ; 38(5): 819-23, 2003 May.
Article in English | MEDLINE | ID: mdl-12720201

ABSTRACT

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.


Subject(s)
Constipation/therapy , Cathartics/therapeutic use , Child , Child, Preschool , Constipation/etiology , Constipation/psychology , Encopresis/etiology , Female , Humans , Male , Parent-Child Relations , Parenting , Psychotherapy , Secondary Prevention , Surveys and Questionnaires
12.
Am J Med ; 110(7): 528-35, 2001 May.
Article in English | MEDLINE | ID: mdl-11343666

ABSTRACT

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.


Subject(s)
Pulmonary Embolism/diagnostic imaging , Adolescent , Adult , Aged , Aged, 80 and over , Echocardiography, Doppler , Female , Humans , Male , Middle Aged , Probability , Prospective Studies , Reference Standards , Risk Factors , Sensitivity and Specificity
13.
J Pediatr Surg ; 36(5): 700-5, 2001 May.
Article in English | MEDLINE | ID: mdl-11329569

ABSTRACT

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.


Subject(s)
Animals, Newborn/metabolism , Disease Models, Animal , Enterocolitis, Necrotizing/metabolism , Intestinal Mucosa/metabolism , Nitric Oxide Synthase/metabolism , Nitric Oxide/metabolism , Obstetric Labor, Premature/metabolism , Age Factors , Animals , Disease Susceptibility , Female , Gestational Age , Obstetric Labor, Premature/drug therapy , Pregnancy , Prostaglandins/therapeutic use , Swine , Time Factors
14.
J Pediatr Surg ; 36(5): 784-90, 2001 May.
Article in English | MEDLINE | ID: mdl-11329590

ABSTRACT

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.


Subject(s)
Abnormalities, Multiple/pathology , Bronchogenic Cyst/pathology , Bronchopulmonary Sequestration/pathology , Cystic Adenomatoid Malformation of Lung, Congenital/pathology , Scimitar Syndrome/pathology , Abnormalities, Multiple/classification , Abnormalities, Multiple/therapy , Adolescent , Age Factors , Age of Onset , Autopsy , Barium Sulfate , Bronchogenic Cyst/complications , Bronchopulmonary Sequestration/classification , Bronchopulmonary Sequestration/complications , Bronchopulmonary Sequestration/therapy , Child , Child, Preschool , Contrast Media , Embolization, Therapeutic , Fatal Outcome , Female , Humans , Infant , Infant, Newborn , Male , Patient Selection , Prenatal Diagnosis , Radiography, Interventional , Respiratory Insufficiency/etiology , Respiratory Tract Infections/etiology , Retrospective Studies , Scimitar Syndrome/complications , Tomography, X-Ray Computed
16.
J Pediatr Surg ; 35(5): 696-8, 2000 May.
Article in English | MEDLINE | ID: mdl-10813327

ABSTRACT

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.


Subject(s)
Digestive System Surgical Procedures/methods , Hernia, Umbilical/surgery , Black People , Child , Child, Preschool , Esthetics , Female , Follow-Up Studies , Hernia, Umbilical/diagnosis , Hernia, Umbilical/ethnology , Humans , Male , Severity of Illness Index , Surgical Flaps , Treatment Outcome
17.
J Pediatr Surg ; 35(5): 717-20, 2000 May.
Article in English | MEDLINE | ID: mdl-10813333

ABSTRACT

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.


Subject(s)
Biliary Atresia/surgery , Liver Transplantation/trends , Portoenterostomy, Hepatic/trends , Biliary Atresia/diagnosis , Biliary Atresia/mortality , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Liver Transplantation/methods , Liver Transplantation/mortality , Male , Portoenterostomy, Hepatic/methods , Portoenterostomy, Hepatic/mortality , Probability , Quebec , Retrospective Studies , Risk Assessment , Survival Rate , Treatment Outcome
18.
J Pediatr Surg ; 35(5): 796-800, 2000 May.
Article in English | MEDLINE | ID: mdl-10813353

ABSTRACT

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.


Subject(s)
Fetal Diseases/diagnosis , Lung Diseases/congenital , Lung Diseases/pathology , Lymphangiectasis/congenital , Lymphangiectasis/pathology , Abortion, Spontaneous , Abortion, Therapeutic , Autopsy , Female , Fetal Diseases/mortality , Humans , Incidence , Infant , Infant, Newborn , Lung Diseases/mortality , Lymphangiectasis/mortality , Male , Pregnancy , Prognosis , Retrospective Studies , Risk Assessment , Survival Analysis
19.
Can J Anaesth ; 46(10): 970-4, 1999 Oct.
Article in English | MEDLINE | ID: mdl-10522585

ABSTRACT

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.


Subject(s)
High-Frequency Ventilation , Lung Diseases/therapy , Anesthesia, Epidural , Anesthesia, Intravenous , Blood Gas Analysis , Carbon Dioxide/blood , Female , Hemodynamics , Humans , Infant , Lung/diagnostic imaging , Lung Diseases/diagnostic imaging , Lung Diseases/surgery , Oxygen/blood , Radiography , Thoracotomy
20.
J Pediatr Surg ; 34(7): 1057-9, 1999 Jul.
Article in English | MEDLINE | ID: mdl-10442587

ABSTRACT

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.


Subject(s)
Lung Neoplasms/diagnosis , Pulmonary Blastoma/diagnosis , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy, Needle , Fatal Outcome , Female , Humans , Infant , Lung Neoplasms/drug therapy , Lung Neoplasms/pathology , Pulmonary Blastoma/drug therapy , Pulmonary Blastoma/pathology , Tomography, X-Ray Computed
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