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  • 1
    ISSN: 1432-1076
    Keywords: Key words Hyperinsulinism ; Hypoglycaemia ; Diazoxide ; Sulphonylureas ; Management
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Primary persistent hyperinsulinaemic hypoglycaemia of infancy is rare. Diazoxide treatment remains the mainstay of medical therapy in long-term management. We reviewed 77 cases of primary persistent hyperinsulinism in neonates and infants who were treated with diazoxide and studied criteria predictive of therapeutic efficacy. The only criterion identified was age at manifestation. All but 1 of the 31 neonatal cases were unresponsive to diazoxide. Responsiveness increased with age: 12 of 39 early-infantile cases, and all seven late-infantile cases were diazoxide-responsive. In responders, a diazoxide dose of 10–15 mg/kg per day was always effective, suggesting an “all or none” response. Diazoxide-resistant hyperinsulinism is characterized by its severity with higher plasma insulin levels. The analysis of 46 surgically treated patients showed that the efficacy of diazoxide is not related to the aetiology of the pancreatic lesions. In six cases, after many years of management, diazoxide treatment was stopped without recurrence of hypoglycaemia. Conclusion Diazoxide is an efficient treatment in the long-term management of most persistent hyperinsulinaemic hypoglycaemia of infancy revealed in infants and children but is usually ineffective in neonatal forms. Drug efficacy does not correlate with anatomical lesions. Medical treatment can sometimes be stopped after many years of management without recurrence of disease manifestations.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Over a 4-year period, we managed four children with alarming haemangiomas (two cases of Kasabach-Merritt syndrome and two life-threatening haemangiomas). Systemic steroid therapy was ineffective. Other treatments (radiotherapy, anti-platelet drugs) were also ineffective in the Kasabach-Merritt patients. On the basis of recent reports on the effects of interferon on endothelial cells, we used alpha-2 interferon therapy, but obtained no response.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Hirschsprung disease (HSCR) is a frequent congenital disorder (1 in 5,000 newborns) of unknown origin characterized by the absence of parasympathetic intrinsic ganglion cells of the hindgut. Taking advantage of a proximal deletion of chromosome 10q (del 10q11.2–q21.2) in a patient with total ...
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 2 (1987), S. 86-92 
    ISSN: 1437-9813
    Keywords: Esophageal stenosis ; Congenital ; Dysphagia ; Malformation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A series of 20 patients with congenital esophageal stenoses (CES), including 4 with tracheobronchial remnants, 6 with membranous diaphragm, and 10 with fibromuscular stenosis, is presented. The experience acquired by treating these patients is compared to 95 cases from the literature and a definition of CES is proposed. The 35% association of CES woth other anomalies, especially esophageal atresia, is analyzed and some difficulties in the treatment of this rare anomaly are discussed.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Intensive care medicine 15 (1989), S. S57 
    ISSN: 1432-1238
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1238
    Keywords: Key words Congenital diaphragmatic hernia ; Antenatal diagnosis ; Postnatal management ; High-frequency oscillatory ventilation ; Intensive care unit ; Neonate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective: a) To analyze the influence of a new management strategy on the outcome of neonates with antenatally diagnosed congenital diaphragmatic hernia (CDH); b) to determine early prognosis respiratory factors with the new strategy.¶Design: Retrospective study. Setting: Level III perinatal center. Patients and method: Between 1985 and 1997, 51 consecutive neonates with antenatally diagnosed CDH were admitted to our level III neonatal intensive care unit. Before 1992 (period 1; n = 19), we used conventional mechanical ventilation and early surgery requiring transfer. Since 1992 (period 2; n = 32), we prospectively tested a new approach including (a) systematically use of high-frequency oscillatory ventilation (HFOV) regardless of the initial clinical severity, (b) delayed surgery following stabilization requiring transfer to a different surgical unit, but (c) no transfer of unstable patients with surgery under HFOV in our neonatal intensive care unit (n = 10). The two cohorts were comparable in terms of potential ante and postnatal prognostic indicators. Results: Survival was improved with the new strategy: 21/32 (66 %) vs. 5/19 (26 %); P 〈 0.02. This improvement between periods 1 and 2 was due to a decrease in both preoperative and postoperative deaths in the later period. The better survival during period 2 was associated with the appearance of very late deaths, frequent pleural effusions, and the survival of more severe forms having evolved to a chronic respiratory insufficiency. Survivors were ventilated for longer time with longer duration of oxygen supplementation. The best oxygenation index (OI), alveolar arterial difference and oscillation amplitude (P/P) during the first 24 h, but not the best PaCO2, were the most reliable prognostic indicators during period 2. An OI ≤ 10 with a P/P ≤ 55 cmH2O was associated with a very good prognosis (94 % survival). Conclusions: The prognosis of antenatally diagnosed CDH was improved by systematic HFOV on admission, no systematic transfer, and delayed surgery. This improvement is associated with modification of postnatal outcome.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-2323
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé Treize enfants âgés de 2 à 16 ans ont subi une résection subtotale de l'intestin grêle dont 10 pour volvulus de sa partie moyenne. Tous ont survécu leur croissance s'étant effectuée normalement. Le total en années de la nutrition parentérale qu'ils ont reçu s'élève à 36 ans. Dans 7 cas, où la longueur du segment intestinal restant variait entre 30 et 120 cm, il fut possible d'arrêter l'alimentation parentérale 30 mois en moyenne après la résection. En revanche si la résection est quasi totale, la longueur du segment de grêle conservé étant inférieure à 20 cm, le maintien permanent de l'alimentation parentérale est inévitable à moins que la transplantation intestinale ne devienne possible. Grâce à l'alimentation parentérale organisée à domicile, la qualité de vie de ces sujets est presque normale.
    Abstract: Resumen Trece niños de edades entre los 2 y los 16 años han sido sometidos a resección subtotal del intestino delgado, como consecuencia de vólvolus del intestino medio en 10 casos. Todos los niños se encuentran vivos y su crecimiento es normal; ellos representan 36 años-pacientes acumulados de nutrición parenteral y 11 años de nutrición enteral a tasa constante. En siete casos en quienes el intestino residual media entre 30 y 120 cm, fué posible terminar todo tipo de soporte nutricional artificial a los 30 meses, en promedio, después de realizada la resección intestinal. Por el contrario, si la resección fué casi total con menos de 20 cm de intestino residual, la dependencia vitalicia de nutrición parenteral es inevitable, a menos que el transplante intestinal llegue a ser factible. Mediante la nutrición parenteral cíclica realizada a domicilio, la calidad de la vida de estos pacientes llega a ser casi normal.
    Notes: Abstract Thirteen children, aged 2 to 16 years, had a subtotal resection of small bowel, following a mid-gut volvulus in 10 cases. All children are alive, and their growth is normal; 36 cumulative patient-years of parenteral nutrition and 11 years of constant-rate enteral nutrition were performed. In 7 cases, in which residual small bowel varied between 30 to 120 cm, termination of all artificial nutritional support was possible 30 months in mean after intestinal resection. In contrast, if resection was near total with less than 20 cm remaining, life-long dependence on parenteral nutrition is unavoidable unless intestinal transplantation becomes feasible. With cyclic parenteral nutrition at home, the patients' quality of life is near normal.
    Type of Medium: Electronic Resource
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