A complicated Glenn procedure: risk factors and association with adverse long-term neurodevelopmental and functional outcomes.

OBJECTIVES: To determine potentially modifiable risk factors for a complicated Glenn procedure (cGP) and whether a cGP predicted adverse neurodevelopmental and functional outcomes. A cGP was defined as post-operative death, heart transplant, extracorporeal life support, Glenn takedown, or prolonged ventilation. METHODS: All 169 patients having a Glenn procedure from 2012 to 2017 were included. Neurodevelopmental assessments were performed at age 2 years in consenting survivors (n = 156/159 survivors). The Bayley Scales of Infant and Toddler Development-3rd Edition (Bayley-III) and the Adaptive Behavior Assessment System-2nd Edition (ABAS-II) were administered. Adaptive functional outcomes were determined by the General Adaptive Composite (GAC) score from the ABAS-II. Predictors of outcomes were determined using univariate and multiple variable linear or Cox regressions. RESULTS: Of patients who had a Glenn procedure, 10/169 (6%) died by 2 years of age and 27/169 (16%) had a cGP. Variables statistically significantly associated with a cGP were the inotrope score on post-operative day 1 (HR 1.04, 95%CI 1.01, 1.06; p = 0.010) and use of inhaled nitric oxide post-operatively (HR 7.31, 95%CI 3.19, 16.76; p < 0.001). A cGP was independently statistically significantly associated with adverse Bayley-III Cognitive (ES -10.60, 95%CI -17.09, -4.11; p = 0.002) and Language (ES -11.43, 95%CI -19.25, -3.60; p = 0.004) scores and adverse GAC score (ES -14.89, 95%CI -22.86, -6.92; p < 0.001). CONCLUSIONS: Higher inotrope score and inhaled nitric oxide used post-operatively were associated with a cGP. A cGP was independently associated with adverse 2-year neurodevelopmental and functional outcomes. Whether early recognition and intervention for risk of a cGP can prevent adverse outcomes warrants study.

Near-fatal pediatric methemoglobinemia secondary to intentional sodium nitrite ingestion.

Methemoglobinemia is the result of inappropriate oxidation of hemoglobin iron groups, leading to a failure of oxygen transport and delivery, resulting in a clinical state of refractory hypoxia. Methemoglobin levels above 70% are often considered fatal. Acquired methemoglobinemia can be caused by a variety of substances, including sodium nitrite, a commercially available food preservative and color fixative. This report describes a patient presenting with a methemoglobin level of 83% secondary to intentional sodium nitrite ingestion. The methemoglobin level recorded is amongst some of the highest found in surviving patients.

Diffuse scarring alopecia in a female pattern hair loss distribution.

We describe three cases of hair loss in a female pattern hair loss (FPHL) distribution with histologic features of lichen planopilaris (LPP). All patients had a history of diffuse, gradual hair loss in a Christmas tree pattern that clinically presented as FPHL on gross and dermoscopic examination. Notably, there were no characteristic clinical signs of LPP and no histologic features of FPHL. These cases are most consistent with cicatricial pattern hair loss (CPHL). This relatively new entity is similar to fibrosing alopecia in a pattern distribution (FAPD) in that they are both scarring alopecias confined to a FPHL distribution, but CPHL lacks the clinical signs of perifollicular erythema and perifollicular keratosis seen in FAPD. These three cases may present an early, subtle form of CPHL and will be of interest to clinicians and histopathologists alike.

Basal cell carcinoma and squamous cell carcinoma growth rates and determinants of size in community patients.

BACKGROUND: Cutaneous basal cell carcinomas (BCCs) and squamous cell carcinomas (SCCs) have poorer outcomes if treated when large. OBJECTIVE: We sought to estimate the growth rate of BCCs and SCCs and examine the relationship of personal, pathway, and cancer factors with cancer size (diameter). METHODS: We surveyed patients, pathology, and treatment for invasive BCCs and SCCs in 1 Australian region in 2000 through 2001. RESULTS: BCC size increased with increasing time since first noticed. Relative to mean size at 0 to 2 months, the mean size ratio was 1.10 at 2 to 8 months and increased steadily to 1.81 at 5 to 10 years (P < .001). Few BCCs were untreated beyond 10 years. There was no consistent evidence that SCC size increased with increasing time. Larger BCCs were independently associated with older age, male sex, no skin checks by a physician, aggressive tumor type, ulceration and lesion-associated scar tissue, and larger SCCs with male sex, skin checks by a physician every 1 to 3 months, and location on limbs. LIMITATIONS: Patient recall of dates and lack of thickness for SCCs are limitations. CONCLUSION: Earlier diagnosis of BCCs, perhaps through skin checks by a physician, may reduce their size and improve outcome. SCC size did not evidently increase with time.

Antimicrobial efficacy and in vivo toxicity studies of a quaternized biopolymeric flocculant.

This study evaluated the antibacterial spectrum and safety of a chemically modified biopolymeric flocculant (TMB) against waterborne pathogens. The biopolymer previously characterized as polysaccharide with flocculating activity is produced extracellularly by the bacterium Klebsiella terrigena. The amino sugars on the polymer were chemically modified by quaternization, which resulted in N,N,N trimethyl biopolymer (TMB). Quaternization was effective in imparting biocidal activity to TMB against five selected waterborne pathogens, namely, Aeromonas hydrophila, Yersinia enterocolitica, Salmonella typhimurium, Listeria monocytogenes and Escherichia coli O157:H7. 99.999% inactivation was achieved with S. typhimurium at a dose of 60 µg ml(-1) of TMB within 60 min at the ambient temperature, followed by other pathogens. Haemotological, histopathological and general examinations indicated no adverse effects in Swiss albino mice fed with the quaternized biopolymer (120 mg kg(-1) body weight(-1) day(-1)) over a period of 30 days. These results suggested that TMB was tolerated well without any signs of toxicity and may have potential application as a safe, antimicrobial bioflocculant for both removing and inactivating waterborne pathogens.

Development of a quaternized chitosan with enhanced antibacterial efficacy.

The antibacterial activity of a water-soluble chitosan derivative prepared by chemical modification to quaternary ammonium compound N,N,N-trimethylchitosan (TC) was investigated against four selected waterborne pathogens: Aeromonas hydrophila ATCC 35654, Yersinia enterocolitica ATCC 9610, Listeria monocytogenes ATCC 19111 and Escherichia coli O157:H7 ATCC 32150. An inactivation of 4 log CFU/ml of all waterborne pathogens was noted for the quaternized chitosan as compared with chitosan over a short contact time (30 min) and low dosage (4.5 ppm) at ambient temperature. A marked increase in glucose level, protein content and lactate dehydrogenase (LDH) activity was observed concurrently in the cell supernatant to be a major bactericidal mechanism. The results suggest that the TC derivative may be a promising commercial substitute for acid-soluble chitosan for rapid and effective disinfection of water.

Pediatric Acute Respiratory Distress Syndrome and Tracheal Injury in a Patient Requiring Extracorporeal Membrane Oxygenation Following Cement Aspiration: A Case Report.

BACKGROUND: Ingestion and aspiration of caustic substances is a common problem in pediatrics and carries the risk of associated aspiration pneumonitis, laryngeal injury, and esophageal injury. Extracorporeal membrane oxygenation (ECMO) has been used to support adults with acute respiratory distress syndrome (ARDS) from aspiration of cement dust, however, literature outlining pediatric management in cases of alkali lung and airway injuries is lacking. CASE SUMMARY: A 6-year-old boy presented with ARDS from cement aspiration requiring high-pressure ventilation. He had further complications of tracheal injury with subsequent pneumomediastinum secondary to the alkali burn. He required ECMO to facilitate repeat bronchoscopy for cement particle washout and to enable recovery from ARDS and tracheal injury. CONCLUSION: This case highlights the need to perform early bronchoscopy and gastrointestinal endoscopy for injury assessment and foreign body removal in alkali burns. It also emphasizes the value of ECMO support for respiratory failure and facilitating bronchoalveolar lavage when it is not otherwise tolerated.

Setting up a Rescue Extracorporeal Life Support Program.

Extracorporeal life support (ECLS) is a high-risk, lifesaving medical treatment that is typically limited to centers that can support a comprehensive ECLS program. Rescue programs can bridge the gap in care between ECLS centers and other tertiary pediatric centers without cardiac surgical and comprehensive ECLS support. We describe how our pediatric center without cardiac surgery successfully partnered with an established ECLS center to develop a Rescue ECLS Cannulation Program. This formalized program provides cannulation and stabilization by a specialized team at the presenting hospital before being transported to a partner hospital. This article outlines how we established our unique Rescue ECLS Cannulation program. We outline the planning, development, and implementation of the program and describe the unique aspects contributing to successful implementation including longitudinal training, staged program evolution, and a bundled approach to care. We also describe the patients who we have cannulated since its inception. Rescue ECLS Cannulation Programs provide access to consistent, high-quality, and lifesaving care to critically ill patients at sites without the resources to support a full ECLS program.

The intractable problems with brain death and possible solutions.

Brain death has been accepted worldwide medically and legally as the biological state of death of the organism. Nevertheless, the literature has described persistent problems with this acceptance ever since brain death was described. Many of these problems are not widely known or properly understood by much of the medical community. Here we aim to clarify these issues, based on the two intractable problems in the brain death debates. First, the metaphysical problem: there is no reason that withstands critical scrutiny to believe that BD is the state of biological death of the human organism. Second, the epistemic problem: there is no way currently to diagnose the state of BD, the irreversible loss of all brain functions, using clinical tests and ancillary tests, given potential confounders to testing. We discuss these problems and their main objections and conclude that these problems are intractable in that there has been no acceptable solution offered other than bare assertions of an 'operational definition' of death. We present possible ways to move forward that accept both the metaphysical problem - that BD is not biological death of the human organism - and the epistemic problem - that as currently diagnosed, BD is a devastating neurological state where recovery of sentience is very unlikely, but not a confirmed state of irreversible loss of all [critical] brain functions. We argue that the best solution is to abandon the dead donor rule, thus allowing vital organ donation from patients currently diagnosed as BD, assuming appropriate changes are made to the consent process and to laws about killing.

Rapid Brain MRI Use in a Pediatric Emergency Department.

Investigators from the University of Pittsburgh (Department of Emergency Medicine and Division of Pediatric Radiology) and Feinberg School of Medicine (Division of Emergency Medicine) studied the rates of neuroimaging (rapid brain MRI [rMRI], head CT [HCT], and full MRI) before and after implementation of four rapid MRI protocols in their ED.