Predictors of long-term respiratory insufficiency of exomphalos major.

INTRODUCTION: Exomphalos major (EM) is associated with significant morbidity and even mortality with an important risk of long-term pulmonary disease. AIM: To assess the outcomes of exomphalos in a single tertiary pediatric unit and to identify prognostic factors for patients with respiratory insufficiency who still require ventilatory assistance at six months. MATERIAL AND METHODS: All infants admitted to our institution over a 10-year period (2005 to 2015) with exomphalos were retrospectively reviewed. EM was defined when the abdominal wall defect measured >= 5 cm and/or contained liver within the sac. Data were collected on patient demographics, prenatal course and imaging, birth information, immediate and long-term outcomes. Those with long-term respiratory insufficiency were identified as the primary outcome and reviewed to assess prognostic factors. A p value of ≤0.05 was regarded as significant. Data are quoted as median(range). RESULTS: A total of 46 infants were diagnosed with exomphalos during the study period, with most (n = 30, 65%) defined as exomphalos major. Respiratory complications occurred in 16 (35%) with 8 (50%) of these requiring long-term (≥6 months) mechanical ventilation and 5 (31%) required a tracheostomy. On univariate analysis, resuscitation at birth (p = 0.0004), birth weight <3000 g (p = 0.008), use of nitric oxide (p = 0.004), high frequency oscillatory ventilation (HFOV) (p = 0.001), pulmonary hypoplasia (p<0.0001) and pulmonary hypertension (PHTN) (p = 0.02) were significantly associated with respiratory insufficiency. The strongest predictive model for ventilation support at six months was resuscitation at birth in combination with PH (OR = 1.57). Five infants (11%) died at 5(1-122) days. CONCLUSIONS: In patients with EM, the presence of pulmonary hypertension along with resuscitation at birth are the most important prognostic factors for long-term respiratory insufficiency. Acknowledgement of these factors allows for better parental counselling regarding respiratory outcomes.

Development and optimization of osmotically controlled drug delivery system for poorly aqueous soluble diacerein to improve its bioavailability.

In an attempt to improve the low oral bioavailability of Diacerein (DCN), the combination of a ternary solid dispersion and an asymmetric osmotic pump system had been designed to enhance solubility and to control DCN delivery. Ternary DCN solid dispersion was prepared by melting fusion method using surfactant polymers, and carrier (Pluronic® PF127, Solutol® HS15, and PEG 35 K) and this DCN solid dispersion powder with the proper amount of excipients were compressed and coated with Opadry®CA to develop a Semi-Permeable and Asymmetric Osmotic Pump tablets. The ternary DCN solid dispersion by using surfactant polymers (Pluronic® F127 and Solutol® HS 15) with a ratio of 1:1 was displayed market significant improvement in saturated solubility (70.2 ± 4.14 µg/ml) and fast dissolution rate (Q60min = 79.28 ± 3.1% and IDR5 min = 5.25 ± 0.19 ml/min) in comparison to pure DCN. Moreover, the optimized asymmetric osmotic pump tablet with following parameters; 3% w/v Opadry® CA coat concentration, 1% w/w HPMC E15 gelling polymer and 35.8%w/w NaCl Osmogen concentration, was displayed control release of DCN at zero-order kinetic (R2 = 0.977) for up to 24 h(s). The in-vivo study conducted on rabbits was revealed a significant enhancement in the bioavailability of the optimized osmotic pump (28.84 ± 3.32 ng.hr/ml) compared to DCN dispersion (10.39 ± 1.45 ng.hr/ml). In conclusion, the approach of enhancing solubility and wet-ability in accompany with optimized asymmetric osmotic pump system could serve as a promising delivery system and a way to improve the bioavailability of poorly aqueous soluble drugs.

Coeliac screening in a high-risk population: paediatric type 1 diabetes-a review of current guidelines and practice.

BACKGROUND AND AIMS: Coeliac disease (CD) is more common in those with type 1 diabetes mellitus (T1DM) and may be asymptomatic despite the presence of intestinal histological changes. Optimal screening practice guidelines differ internationally. We undertook a retrospective audit to determine the efficacy of current screening practice for CD in T1DM in our centre. METHODS: All children and adolescents < 16 years, diagnosed with T1DM in our service and continuing to attend the service in January 2017 were included. Data on CD screening was collected and compared to current NICE, NASPGHAN and ESPGHAN guidelines. RESULTS: Of the 355 patients attending our service, 253 attended from T1DM diagnosis and all had CD screening performed in our centre. In 37 of 253 patients, IgA-TTG was positive, providing a cumulative prevalence of 14.6%. Of these, 31(83.78%) with an elevated TTG on screening had no recorded gastrointestinal symptoms or CD-related clinical signs. Of the 35 TTG plus EMA-positive patients, 22/35 (59.46%) had diagnostic endoscopic biopsy. Nineteen (83.4%) had CD confirmed, 1 (4.54%) had negative biopsy and 2 (9%) had equivocal, non-diagnostic changes. CONCLUSIONS: Timely diagnosis of CD can prevent chronic ill health in affected individuals, and in patients with T1DM, CD is an independent risk factor for increased morbidity and mortality. Given the high prevalence of atypical symptoms and silent CD in those with T1DM, in this and other studies, and the benefits of detection and treatment of CD, screening is essential. Large-scale data collection allowing for the development of evidence-based guidelines is required.

Incidence of Low-Grade Testicular Injury in Orchidectomy Specimens Post-testicular Torsion.

INTRODUCTION: Following detorsion and orchidopexy for testicular torsion, predominantly animal studies have reported a risk of autoimmune and reperfusion injury to the contralateral testis. As a result, when testicular viability is compromised, orchidectomy is readily performed. This practice increases the likelihood of testes with potentially reversible injury being excised. We aim to determine the incidence of such occurrences and review the available evidence for and against early orchidectomy when testicular viability is doubtful. MATERIALS AND METHODS: Data for a 15-year period from two pediatric institutions on testicular torsion in children younger than 16 years were reviewed. Using a previously published grading system, the orchidectomy specimens in this cohort with early low-grade injury were analyzed. Low-grade injury suggests the possibility of restitutio ad integrum implying restoration of exocrine and endocrine function of the affected testes. RESULTS: Between both institutions, 222 scrotal explorations were performed for testicular torsion; 20 neonatal and 202 outside the neonatal period (age range [median]: 1-28 days [3 days] and 3 months-16 years [13 years], respectively). Of these scrotal explorations, 17 neonatal and 66 nonneonatal orchidectomies were required (85 vs. 33%, respectively; p < 0.0001). From these orchidectomy specimens, 5 (6%) were found to have low-grade injury. The ages of these five children ranged from 9 to 16 years (median 15, mean 13.6 years). Their symptom duration ranged from 8 to 37 hours (median 14, mean 18 hours) and two of these children had a preoperative ultrasound documenting no flow to the testis. CONCLUSION: The finding of histopathological features that may represent salvageability of a torted testis occurs relatively rarely. Because of this possibility, appropriate intraoperative steps to check for reperfusion must be undertaken prior to orchidectomy. More evidence for the use of antioxidants and tunica albuginea decompression to improve testes salvage rates is required. The potential for exocrine and endocrine function if partial testicular atrophy occurs and the evidence for contralateral autoimmune testicular damage in pre- and postpubertal males require further investigation.

Management of recurrent tracheoesophageal fistulas: a systematic review.

INTRODUCTION: The management of a recurrent tracheoesophageal fistula (RTEF) includes either open surgery (OS) or an endoscopic treatment (ET); the ideal option is unclear. We aim to comparatively review all published treatment options, and outcomes, for managing RTEF. MATERIALS AND METHODS: A literature search was performed using the keywords "recurrent tracheoesophageal fistula." All English language articles describing the management of RTEF in children were reviewed. A synthesis of the relevant data is presented in a descriptive form due to the heterogeneity of the included articles. RESULTS: A total of 44 papers between 1955 and 2013 described 165 patients; 57 ET and 108 OS. Of the 57 ET patients, there was an 84% success rate compared with 93.5% of 108 OS patients; the failed ET cases were all successfully treated by OS. The refistulation rate after OS was 21% and an average of 1.1 (range 1-2) procedures were required. After ET, the refistulation rate was 63% and an average of 2.1 (range 1-6) treatments were required for success; these results were reported after a maximum follow-up of 9 years and 23 years for ET and OS, respectively. The major complications after OS were 17 (16%) leaks and 4 (3.7%) deaths, while for ET 3 (5%) suffered respiratory distress postoperatively and there was 1 (1.7%) death. CONCLUSION: OS for RTEF has a low morbidity and mortality, a higher success rate, and requires fewer treatments than an endoscopic repair. The ideal ET is undecided but it remains a viable alternative provided treatment failures are anticipated and prompt redo treatments initiated to prevent ongoing respiratory morbidity.

Wandering spleen: a rare cause of mesenteroaxial gastric volvulus.

Gastric volvulus is a rare cause of acute abdomen in children. Usually it is associated with defects such as diaphragmatic hernia, hiatal hernia, eventration, and paralysis of the diaphragm. We report an extremely rare case presenting with acute intractable vomiting and abdominal distention. Passage of a nasogastric tube relieved the symptoms. A history of the ingestion of a large bolus of chewing gum and a suboptimal contrast study were misleading, and there was a delay in diagnosis of 3 days. Later, repeat upper GI fluoroscopy with contrast medium identified mesenteroaxial volvulus of the stomach. Exploratory laparotomy additionally revealed a wandering spleen. Derotation of the stomach with anterior gastropexy was performed. To our knowledge, wandering spleen in association with gastric volvulus has been mentioned only three times before in the literature.