Baseline Quality of Life in Pediatric Patients With Low-flow Vascular Malformations.

OBJECTIVE: The aim of this study was to quantify and describe baseline patient and parent-proxy health-related quality of life scores in patients with low-flow vascular malformations at a single, tertiary-care vascular anomalies clinic. STUDY DESIGN: This is a retrospective study of data collected on patients with low-flow vascular malformations between the ages of 2 to 25 who were seen at a single, tertiary-care center vascular anomalies clinic. A total of 266 patients are included in this study. RESULTS: Patients with lymphatic malformations report decreased quality of life scores as compared with venous malformations in the emotional, psychological, school, and social domains. Patients with lower extremity malformation report decreased quality of life scores as compared with head/neck, trunk, upper extremity, and multifocal malformations; most notably in the physical domain. CONCLUSIONS: Treatment of low-flow vascular malformations should aim to improve patient quality of life. The use of standardized health-related quality of life measures in this study quantifies baseline quality of life scores among patients with low-flow vascular malformations.

The impact of sarcopenia on outcomes in patients with inflammatory bowel disease undergoing colorectal surgery.

INTRODUCTION: Sarcopenia is an independent predictor of poor post-operative outcomes following major surgery. Inflammatory bowel disease (IBD) consists of Crohn's disease (CD) and ulcerative colitis (UC), which are chronic inflammatory conditions involving the gastrointestinal system. Evidence demonstrates that up to 60% of patients with IBD have sarcopenia. Despite advances in medical management, more than 15% of patients with UC and 80% with CD eventually require surgical intervention. The primary aim of the study was to assess the impact of sarcopenia on post-operative anastomotic leak rates. METHODS: A retrospective cohort study of patients at Royal Perth Hospital who underwent major abdominal surgery for management of IBD between January 2010 and December 2020 was performed. Sarcopenia was assessed according to the skeletal muscle index at the level of the third lumbar vertebrae using the cut off 52.4 and 38.5 cm2 /m2 for men and women, respectively. RESULTS: A total of 147 patients were included. The prevalence of sarcopenia within the study population was 36.1%. Patients with sarcopenia were significantly taller (P = 0.025) and were more likely to be on pre-operative steroid medications (P = 0.045). There was no difference in the remaining baseline characteristics between sarcopenic and non-sarcopenic patients. Patients with sarcopenia were more likely to develop a post-operative anastomotic leak (OR:11.303, 95% CI, 1.53-83.51, P = 0.017), grade IV complications (OR:6.79, 95%CI:1.1-43.6, P = 0.044) and require total parenteral nutrition (TPN) (OR:3.212, 95% CI:1.3-8.1, P = 0.013). CONCLUSIONS: Sarcopenia significantly increases the risk of major post-operative complications for patients with IBD undergoing colorectal surgery.

Thrombogenic superior vena cava syndrome from long-standing central venous access in a 5-year-old patient treated with balloon-expandable stents.

Thrombogenic superior vena cava syndrome is an uncommon, dangerous complication of long-standing central venous catheter use. The increased use of central venous catheters has resulted in more non-malignant cases of superior vena cava syndrome across all age groups. We present a 5-year-old male with superior vena cava syndrome associated with acute onset of severe upper extremity and facial swelling, dyspnea, and a right subclavian central venous catheter malfunction. The patient was ultimately treated with percutaneous stenting of the superior vena cava with balloon-expandable Palmaz stents following unsuccessful angioplasty, catheter-directed thrombolysis, and percutaneous thrombectomy. This case highlights a relatively uncommon complication in children from long-term central venous catheter access and describes an emerging, minimally-invasive therapeutic alternative that allows for preservation of age-appropriate superior vena cava luminal diameter as patients grow.

Radioembolisation for treatment of pediatric hepatocellular carcinoma.

Transarterial radioembolisation with yttrium-90 (TARE-Y90), a catheter-directed therapy, has been used extensively in adults to treat primary and secondary hepatic malignancies. To our knowledge, the use of this palliative technique has not been described in children. We present two children with unresectable hepatocellular carcinoma (HCC) treated with TARE-Y90.

Prevention of neural hypersensitivity after acute upper limb burns: Development and pilot of a cortical training protocol.

BACKGROUND: Acute burn patients suffer pain and secondary hyperalgesia. This alters movement patterns and impairs function. Non-pharmacological methods of treatment are limited and lack rigorous testing and evidence for use. The treatment in this case series was designed to direct conscious attention to, and normalise sensation of, the injured limb in pain free way. The aim of the study was to describe a cortical training programme (CTP) in acute upper limb burn patients and to investigate the efficacy, safety and feasibility of the protocol. METHOD: The study is a descriptive case series (n=6). Study tasks engaged sensory and motor nerves to influence the perception of the injured area. Visual and tactile inputs to maintain and, or normalise the homuncular map were central to the intervention. RESULTS: One patient, who commenced the study without resting pain, responded negatively. The remaining five patients had reduced pain and fear avoidance behaviours with associated improvement in arm function. CONCLUSIONS: The CTP approach is safe and feasible for use with acute burn patients where pain is reported at rest. Comparative studies are required to determine the relative efficacy of the program to usual interventions and the patients who may benefit from the technique.

Development of a biotic ligand model to predict the acute toxicity of cadmium to Daphnia pulex.

The goal of this study was to develop a biotic ligand model (BLM) to predict the acute toxicity of cadmium to Daphnia pulex. Organisms were cultured in moderately soft water and standard 48h acute toxicity tests were used to determine EC50s in various water chemistries where the effects of Ca(2+), Na(+), Mg(2+), Cl(-), K(+), pH, and two sources of natural organic matter (Suwannee River and Nordic Reservoir) were evaluated. Overall, toxicity responses were consistent with the free-ion activity model and the principles inherent in the BLM. Increases in Ca(2+) resulted in higher EC50s, indicating that Cd(2+) competes with Ca(2+) for uptake at the biotic ligand. Similar cation competition effects were observed when Mg(2+) was varied but with a less pronounced protective effect relative to Ca(2+). Changes in Na(+) and K(+) concentrations had no significant effect on Cd toxicity. EC50 values did not change significantly when pH was adjusted over a range from 8.0 to 6.1. Additions of natural organic matter resulted in elevated dissolved organic carbon (DOC) concentrations that significantly reduced Cd bioavailability via complexation of Cd(2+). An existing biotic ligand model (HydroQual BLM ver 2.2.3) was tested for its ability to predict acute Cd toxicity to D. pulex. Once the BLM was adjusted for the relatively sensitivity of D. pulex the protective effects of Ca and DOC could be predicted reasonably well but other test chemistries did not match with measured EC50s. Binding constants derived from the test results (logK(CaBL) of 4.1, logK(MgBL) of 3.7, logK(HBL) of 6.1 and logK(CdBL) of 7.0) were used to develop a modified BLM for the effects of Cd on D. pulex that accounted for the moderating effect of Ca and Mg on acute toxicity but overestimated the protective effect of DOC.

Development of a biotic ligand model for the acute toxicity of zinc to Daphnia pulex in soft waters.

The goal of this study was to develop a biotic ligand model (BLM) for the acute toxicity of zinc to the Daphnia pulex in soft water. In different tests Ca (as CaSO(4)), Na (as NaCl), Mg (as MgSO(4)), K (as KCl), pH (using the buffer 3-(N morpholino)-propanesulphonic acid (MOPS)) and dissolved organic carbon (DOC) were altered to determine possible effects on the 48h EC50 for Zn. Increases in waterborne Ca(2+) had a protective effect on Zn toxicity, suggesting that this ion competes with Zn and that they share a common site of biological uptake. Increased waterborne Mg(2+) also reduced Zn toxicity, but to a lesser degree compared with Ca(2+). No significant effects of other cations on EC50 for Zn were observed, indicating that the toxicity of Zn in D. pulex is not linked to Na(+) and K(+). Increasing DOC concentrations resulted in higher EC50 values for Zn due to the complexation of Zn by organic matter in solution and the resulting reduction of free Zn(2+) ion concentrations. Tests to characterize the effect of pH on Zn toxicity showed a small rise in EC50 values between pH 6.3 and 7.1 and no further change as pH was increased to 8.0. Two existing BLM implementations for acute Zn effects on D. magna were tested to determine their applicability to D. pulex in soft water. The existing models underestimated the protective effect of Ca(2+) and Mg(2+) but accurately predict the effects of DOC. A modified BLM, with revised equilibrium constants for competitive cation effects was developed. This study shows that the acute effects of Zn on D. pulex in soft water can be characterized and incorporated into a predictive BLM.