• 116. Identification of pharmacological chaperones for feline and human GM1 gangliosidosis

      Rigat, Brigitte; Michael, Tropak; Crushell, Ellen; Daphne, Benedict; Martin, Douglas; Stuetz, Arnold; Callahan, John; Mahuran, Don (2009)
      WORLD (Research in Lysosomal Disease)meeting 2009
    • Accidental Ingestion of Magnetic Spheres in Children

      Zgraj, O; Awadalla, S (Irish Medical Journal, 2015-05)
      Magnetic foreign body ingestion can have a very serious sequale if multiple or combined with another metal object inside the abdomen. We report 2 cases of ingestion of rare-earth magnets with a very different consequences. This adds to the world’s literature on this topic.
    • Acute functional deterioration in a child with cerebral palsy.

      Smyth, Elizabeth; Kaliaperumal, Chandrasekaran; Leonard, Jane; Caird, John; Department of Paediatrics, Children's University Hospital, Dublin, Republic of Ireland. (2012)
      We describe a case of acute functional deterioration in a 13-year-old girl with severe spastic diplegia (GMFCS III) and a new diagnosis of diffuse intrinsic pontine glioma (DIPG). She presented with acute deterioration in mobility and motor function over 1 month, which was associated with dysarthria, dysphagia and behavioural change. Her mother had noticed subtle functional deterioration over the 2 months prior to this. Her physiotherapist who was concerned about her acute functional deterioration referred her for emergency review. Neurological imaging revealed a diffuse pontine lesion consistent with DIPG. She was subsequently referred to oncology. She deteriorated further, clinically, over the next few days and following discussion with the team; her family opted for palliative treatment, given the poor prognosis associated with DIPG.
    • Acute mastoiditis in children: presentation and long term consequences.

      Glynn, F; Osman, L; Colreavy, M; Rowley, H; Dwyer, T P O; Blayney, A; Department of Otorhinolaryngology Head and Neck Surgery, Children's University Hospital, Temple Street, Dublin 2, Ireland. fglynn@rcsi.ie (2008-03)
      Acute mastoiditis, a destructive bacterial infection of the mastoid bone and air cell system, is relatively uncommon today but remains a potentially serious condition. There is a lack of information in the literature regarding the long term otological problems that children may face following an episode of this condition.
    • Analgesic control after hip arthroscopy: a randomised, double-blinded trial comparing portal with intra-articular infiltration of bupivacaine.

      Baker, Joseph F; McGuire, Ciara M; Byrne, Damien P; Hunter, Kim; Eustace, Nick; Mulhall, Kevin J; Department of Orthopaedic Surgery, Sports Surgery Clinic, Dublin - Ireland. (2011-06-07)
      Abstract: The optimum anaesthetic and analgesic management following hip arthroscopy is yet to be determined. There is, in addition, some concern over the use of intraarticular local anaesthetic. We compared the analgesic efficacy of intra-articular infiltration compared with portal infiltration of bupivacaine following hip arthroscopy. Patients were randomised to receive either 10ml of 0.25% bupivacaine either into the joint or around the portal sites following completion of surgery. 73 patients were recruited (40 intra-articular). The portal infiltration group required significantly more rescue analgesia immediately after surgery (2.33mg vs.0.57mg, p=0.036). Visual Analogue Scale pain scores were not significantly different at 1 and 2 hours following surgery, but at 6 hours the portal group had significantly lower VAS scores (p=0.0036). We believe that the initial pain following surgery results from capsular injury and this explains the need for more rescue analgesia in the portal infiltration group. Further work is needed to establish the ideal regimen. A combination of portal and intra-articular infiltration may be the most efficacious.
    • Animal attack: an unusual case of multiple trauma in childhood.

      O'Grady, E; Doyle, M; Fitzgerald, C W R; Mortell, A; Murray, D (Irish Medical Journal, 2014-11)
      A 2½ year old girl attended our facility following attack by a tapir at a city zoo. She sustained multiple injuries including a forearm laceration and multiple perforating wounds to her abdominal wall. She had several procedures, including bowel resection, performed under the care of the General Paediatric Surgery and Plastic Surgery teams and was treated with a course of IV antibiotics. She recovered well and to date has suffered no long-term adverse outcome.
    • Anorexia Nervosa (AN) in inpatients at a children s hospital (2005-2011)

      O’Reilly, M; Carr, C; Boylan, C; Anglim, M; Houlihan, B (Irish Medical Journal (IMJ), 2014-02)
      AN is a serious mental illness best treated in the community 1 . Those with critically low weight require hospitalisation. There is little published research on AN in Ireland. The aim of this audit was to evaluate the Irish experience. The mean age on admission was 13.5 yrs which is 6mo earlier than 2002 figures. Boys represented 6/20 (30%) of admissions. On admission girls were more underweight than boys (0.4th centile V 9th centile for BMI). This was despite girls presenting to hospital sooner than boys post onset of symptoms. Aside from low weight, over-exercising and food restricting were the most common presenting features. Inpatient weight restoration is successful with a mean weekly weight gain of 930g which is within the recommended range of 500-1000g/wk. Mean hospital stay was 38 days.
    • Antibiotic prophylaxis in children with vesicoureteric reflux: has RIVUR answered all our questions?

      Teoh, CW; Awan, A (Irish Medical Journal, 2015-04)
      The interest in urinary tract infections (UTI) and vesicoureteral reflux (VUR), which is the retrograde flow of urine from the bladder into the ureter, started gaining pace in the late 1950s after the introduction of voiding cystourethrography. 1 VUR is present in one third of children presenting with febrile urinary tract infections (UTI) and is associated with renal scarring. 1 In an effort to reduce renal scarring as a result of VUR, various surgical repairs of the vesicoureteral junction, and later endoscopic injection techniques were introduced. 2 With the emerging understanding of the strong association between UTI and VUR, antibiotic prophylaxis gained popularity after an observational study showed that VUR improved with long-term, low-dose antibiotic treatment. 3 Smellie et al found that prophylaxis significantly reduced the number of UTI recurrences compared to before prophylaxis. 4 The use of antibiotic prophylaxis became standard practice after two large prospective randomised trials comparing surgical intervention and antibiotic prophylaxis found no difference between the two interventions in relation to UTI recurrence and new renal damage. 5-7 Endoscopic and surgical treatments were reserved for severe reflux.
    • Arousal responses in babies at risk of sudden infant death syndrome at different postnatal ages.

      Dunne, K P; Fox, G P; O'Regan, M; Matthews, T G; General Hospital, Castlebar, Co. Mayo. (1992-03)
      Hypercarbic and hypoxic arousal responses during sleep were measured in healthy term infants, infants where a previous sibling died from sudden infant death syndrome (SIDS) and infants suffering a clearly defined apparent life threatening event (ALTE) requiring vigorous or mouth to mouth resuscitation. Groups of infants were tested at approximately one, six and 13 weeks postnatally. Arousal was defined as gross body movement with eyes opening and moving or crying. Hypercarbic arousal was by step increases in F1 Co2 until arousal occurred or until endtidal (PETCO2) reached 8.7 KpA (65 mm Hg) Hypoxic arousal was by step decreases in FIO2 until arousal occurred or until an FIO2 of 0.15 had been maintained for 20 minutes. There was no difference in hypercaribic arousal threshold with age in any group. Hypercarbic arousal threshold was significantly higher in siblings (mean 53.4, 53.6, 54.7 mmHg. [7.12, 7.14, 7.29 KPA] at 0, 6, 13 postnatal weeks) compared to controls (mean 50.9, 52.3, 53.0mm Hg. [6.78, 6.97, 7.29 KPS respectively). ALTE infants differed only at 12 weeks having a significantly lower threshold (51.0mmHg. [6.80 KPA] V 53.0mm Hg. (7.06 KPA]) compared to controls. There was no difference in hypoxic arousal response with age in any group. An arousal response to hypoxia occurred in only 22% of ALTE infants and 40% of siblings compared to 67% of normal infants. Deficient sleep arousal, especially to hypoxia, is common in infants and especially those considered at increased risk from SIDS. This deficiency is present in the first postnatal week and did not vary overy the first three months of postnatal life.
    • Aspergillus flavus sinusitis in ALL.

      Keane, Colm; Colreavy, Michael; Lynch, Maureen; O'Gorman, Peter; Department of Haematology, Princess Alexandra Hospital, Brisbane, Australia. colm_keane@health.qld.gov.au (2009-02)
    • Assessment of biochemical profiles during metabolic decompensation in 14 patients with Maple Syrup Urine Disease

      Knerr, I; Crushell, E; Kozdoba, O; Mayne, PD; Deverrell, D; Monavari, A; Treacy, E (2011-09)
      Society for the study of Inborn Errors of Metabolism Annual Symposium Geneva Sept 2011
    • Baby walkers in Europe time to consider a ban

      Murphy, A; Nicholson, A J (Irish Medical Journal, 2011-03)
    • Basiliximab induced non-cardiogenic pulmonary edema in two pediatric renal transplant recipients.

      Dolan, Niamh; Waldron, Mary; O'Connell, Marie; Eustace, Nick; Carson, Kevin; Awan, Atif; Department of Nephrology, Children's University Hospital, Dublin, Ireland. dolanniamh@eircom.net (2009-11)
      We report two cases of non-cardiogenic pulmonary edema as a complication of basiliximab induction therapy in young pediatric renal transplant patients identified following a retrospective review of all pediatric renal transplant cases performed in the National Paediatric Transplant Centre, Childrens University Hospital, Temple Street, Dublin, Ireland. Twenty-eight renal transplantations, of which five were living-related (LRD) and 23 were from deceased donors (DD), were performed in 28 children between 2003 and 2006. In six cases, transplantations were pre-emptive. Immunosuppression was induced pre-operatively using a combination of basiliximab, tacrolimus and methylprednisolone in all patients. Basiliximab induction was initiated 2 h prior to surgery in all cases and, in 26 patients, basiliximab was re-administered on post-operative day 4. Two patients, one LRD and one DD, aged 6 and 11 years, respectively, developed acute non-cardiogenic pulmonary edema within 36 h of surgery. Renal dysplasia was identified as the primary etiological factor for renal failure in both cases. Both children required assisted ventilation for between 4 and 6 days. While both grafts had primary function, the DD transplant patient subsequently developed acute tubular necrosis and was eventually lost within 3 weeks due to thrombotic microangiopathy and severe acute antibody-mediated rejection despite adequate immunosuppression. Non-cardiogenic pulmonary edema is a potentially devastating post-operative complication of basiliximab induction therapy in young pediatric patients following renal transplantation. Early recognition and appropriate supportive therapy is vital for patient and, where possible, graft survival.
    • Batteries not included

      Hand, F; McDowell, D; Gillick, J (Irish Medical Journal, 2014-01)
      We report two cases of oesophageal lodgement of ingested button batteries (BB) in young children. In one case the diagnosis and subsequent treatment was made in a timely fashion and the patient suffered no sequelae. In the second case there was a delay in diagnosis and the patient subsequently suffered both early and late complications. The purpose of this report is to highlight the importance of the correct management of suspected BB ingestion.
    • BCG-oma

      O'Loughlin, A; O'Donnell, B (2009-03)
      American Academy of Dermatology Meeting
    • Bevacizumab compared with Diode Laser in stage 3 posterior retinopathy of prematurity: a 5 year follow-up

      O’Keeffe, N; Murphy, J; O’Keeffe, M; Lanigan, B (Irish Medical Journal, 2016-02)
      We conducted a prospective randomized study to compare outcomes of intravitreal Bevacizumab versus diode laser in thirty eyes of fifteen premature babies with zone 1 or posterior zone 2 retinopathy of prematurity (ROP). We recorded complications, regression/reactivation of ROP, visual outcome, refractive error and systemic complications. The Bevacizumab-treated eyes showed rapid regression of the ROP with resolution of plus disease and flattening of the ridge at 48 hours post injection. In 3 Bevacizumab-treated eyes, reactivation occurred and were treated with laser (3 eyes) or a further Bevacizumab injection (1 eye). Of the diode laser treated eyes, one showed progression and was treated with Bevacizumab. At 5-year follow-up, good outcomes were observed in both treatment groups. However, less myopia was found in the Bevacizumab compared with the diode laser treated eyes.
    • Biochemical and Glycomic effects of diet relaxation in Classical Galactosaemia

      Coss, K; Coman, D; Brown, A; Hendroff, U; Carolan, C; Mayne, PD; Walsh, O; Struwe, W; Rudd, P; Treacy, E (2010-09)
      Society for the Study of Inborn Errors of Metabolism Annual Symposium Istanbul
    • Bringing the Board of Directors on Board with Quality and Safety of Clinical Care

      Temple Street Children’s University Hospital; HSE Quality Improvement Division (Health Service Executive, 2018-08)
      The genesis of this project was about bringing the Temple Street Children’s University Hospital Board of Directors on a journey, which would result in the Board holding the hospital Executive accountable for the quality of clinical care delivered. It was a collaboration between the Board, the Project Team and the HSE Quality Improvement Division. Governing Boards of healthcare organisations are responsible for their organisations’ performance (HSE 2017). Prior to this project Temple Street Children’s University Hospital (TSCUH) Board of Directors received operational information on access, efficiency, human resources and finance indicators through a monthly balanced score card report, while the quality indicators were reported quarterly. Data on the score card were presented using a red, amber and green speedometer with an associated line chart, which demonstrated if the desired target was achieved.