What is Known About Critical Congenital Heart Disease Diagnosis and Management Experiences from the Perspectives of Family and Healthcare Providers? A Systematic Integrative Literature Review.

The experience of diagnosis, decision-making and management in critical congenital heart disease is layered with complexity for both families and clinicians. We synthesise the current evidence regarding the family and healthcare provider experience of critical congenital heart disease diagnosis and management. A systematic integrative literature review was conducted by keyword search of online databases, MEDLINE (Ovid), PsycINFO, Cochrane, cumulative index to nursing and allied health literature (CINAHL Plus) and two journals, the Journal of Indigenous Research and Midwifery Journal from 1990. Inclusion and exclusion criteria were applied to search results with citation mining of final included papers to ensure completeness. Two researchers assessed study quality combining three tools. A third researcher reviewed papers where no consensus was reached. Data was coded and analysed in four phases resulting in final refined themes to summarise the findings. Of 1817 unique papers, 22 met the inclusion criteria. The overall quality of the included studies was generally good, apart from three of fair quality. There is little information on the experience of the healthcare provider. Thematic analysis identified three themes relating to the family experience: (1) The diagnosis and treatment of a critical congenital heart disease child significantly impacts parental health and wellbeing. (2) The way that healthcare and information is provided influences parental response and adaptation, and (3) parental responses and adaptation can be influenced by how and when support occurs. The experience of diagnosis and management of a critical congenital heart disease child is stressful and life-changing for families. Further research is needed into the experience of minority and socially deprived families, and of the healthcare provider, to inform potential interventions at the healthcare provider and institutional levels to improve family experience and support.

Smoking in pregnancy is a key factor for sudden infant death among Maori.

AIM: To examine the sudden unexpected death in infancy (SUDI) disparity between Maori and non-Maori in New Zealand. METHODS: A nationwide prospective case-control study ran from March 2012 to February 2015. Exposure to established SUDI risk factors was analysed to investigate the disparity experienced by Maori. Infant ethnicity was based on mother's ethnicity. Maori ethnicity was prioritised. Non-Maori includes Pacific, Asian, NZ European and Other. RESULTS: There were 137 cases and 649 controls. The Maori SUDI rate was 1.41/1000 live births compared to 0.53/1000 for non-Maori. Parents/caregivers of 132 cases (96%) and 258 controls (40%) were interviewed. Smoking in pregnancy was associated with an equally increased SUDI risk for Maori (adjusted OR = 8.11, 95% CI = 2.64, 24.93) and non-Maori (aOR = 5.09, 95% CI = 1.79, 14.47), as was bed-sharing (aOR = 3.66, 95% CI = 1.49, 9.00 vs aOR = 11.20, 95% CI = 3.46, 36.29). Bed-sharing prevalence was similar; however, more Maori controls smoked during pregnancy (46.7%) than non-Maori (22.8%). The main contributor relating to increased SUDI risk for Maori/non-Maori infants is the combination of smoking in pregnancy and bed sharing. CONCLUSION: The association between known SUDI risk factors, including bed sharing and/or smoking in pregnancy and SUDI risk, is the same regardless of ethnicity. Maori infants are exposed more frequently to both behaviours because of the higher Maori smoking rate.

Incontinentia pigmenti in 22-month old Afro-Caribbean fraternal twin girls: a case report / Incontinencia pigmentaria en gemelas afrocaribeñas de 22 meses: un reporte de caso

Incontinentia pigmenti is a rare X-linked dominant condition characterized by cutaneous, neural, ocular and dental manifestations. The condition has mainly been reported in Caucasian females. The aim of this case report is to highlight the clinical presentation in Afro-Caribbean twin girls. The girls demonstrated abnormal hair distribution, pigmented limbs and torso, small conical or missing teeth with delayed dental eruption.

La incontinencia pigmentaria es una rara condición dominante ligada al cromosoma X, caracterizada por manifestaciones cutáneas, nerviosas, oculares y dentales. La condición ha sido reportada principalmente en mujeres caucásicas. El objetivo de este reporte de caso es resaltar su presentación clínica en gemelas afrocaribeñas. Las chicas mostraron una distribución anormal del cabello, extremidades y torso pigmentados, pequeños dientes cónicos o ausentes con retraso en el brote dental.

Respiratory health outcomes 1 year after admission with severe lower respiratory tract infection.

Severe lower respiratory infection (LRI) is believed to be one precursor of protracted bacterial bronchitis, chronic moist cough (CMC), and chronic suppurative lung disease. The aim of this study was to determine and to describe the presence of respiratory morbidity in young children 1 year after being hospitalized with a severe LRI. Children aged less than 2 years admitted from August 1, 2007 to December 23, 2007 already enrolled in a prospective epidemiology study (n = 394) were included in this second study only if they had a diagnosis of severe bronchiolitis or of pneumonia with no co-morbidities (n = 237). Funding allowed 164 to be identified chronologically, 131 were able to be contacted, and 94 agreed to be assessed by a paediatrician 1 year post index admission. Demographic information, medical history and a respiratory questionnaire was recorded, examination, pulse oximetry, and chest X-ray (CXR) were performed. The predetermined primary endpoints were; (i) history of CMC for at least 3 months, (ii) the presence of moist cough and/or crackles on examination in clinic, and (iii) an abnormal CXR when seen at a time of stability. Each CXR was read by two pediatric radiologists blind to the individuals' current health. Results showed 30% had a history of CMC, 32% had a moist cough and/or crackles on examination in clinic, and in 62% of those with a CXR it was abnormal. Of the 81 children with a readable follow-up X-ray, 11% had all three abnormal outcomes, and 74% had one or more abnormal outcomes. Three children had developed bronchiectasis on HRCT. The majority of children with a hospital admission at <2 years of age for severe bronchiolitis or pneumonia continued to have respiratory morbidity 1 year later when seen at a time of stability, with a small number already having sustained significant lung disease.

Incontinentia Pigmenti in 22-month Old Afro-Caribbean Fraternal Twin Girls. A Case Report.

Incontinentia pigmenti is a rare X-linked dominant condition characterized by cutaneous, neural, ocular and dental manifestations. The condition has mainly been reported in Caucasian females. The aim of this case report is to highlight the clinical presentation in Afro-Caribbean twin girls. The girls demonstrated abnormal hair distribution, pigmented limbs and torso, small conical or missing teeth with delayed dental eruption.

Reperfusion strategies in the management of extremity vascular injury with ischaemia.

BACKGROUND: Extremity injury with ischaemia is the most common pattern of vascular trauma and is a challenge for surgeons who must make decisions about the timing and mechanism of limb reperfusion. In modern military conflicts, effective use of limb tourniquets and rapid transport of the injured have increased the number of casualties who reach a medical service with potentially survivable vascular trauma. This report provides a review of extremity ischaemia and reperfusion following vascular trauma. METHODS: A review was undertaken of extremity vascular injury with ischaemia, including a focus on adjuncts aimed at reducing reperfusion injury and improving neuromuscular recovery and limb salvage. RESULTS: Findings from basic and clinical research support the need to restore perfusion to an ischaemic limb as soon as possible in order to achieve optimal neuromuscular recovery. Large-animal studies demonstrate that haemorrhagic shock worsens the impact of ischaemia on the neuromuscular structures of the limb and reduces the ischaemic threshold to as little as 1 h. Surgical adjuncts such as vascular shunts, fasciotomy, regional limb cooling and ischaemic conditioning may reduce the severity of ischaemic injury. Medical therapies have also been described including hypertonic saline, statins and ethyl pyruvate, which reduce the inflammatory response following limb reperfusion. CONCLUSION: Contemporary translational research refutes a casual approach to extremity vascular injury with ischaemia, instead emphasizing expedited reperfusion. Surgical and medical adjuncts exist to expedite reperfusion and mitigate reperfusion injury. Additional research and development of these adjuncts is necessary to improve quality or functional limb salvage after vascular trauma.

Oral health of children with intractable epilepsy attending the UK National Centre for Young People with Epilepsy.

AIM: To investigate the oral health of children with intractable epilepsy attending the UK National Centre for Young People with Epilepsy. STUDY DESIGN AND METHODS: 39 children and adolescents with intractable epilepsy at a residential school, the UK National Centre For Young People With Epilepsy (NCYPE) were age, gender and ethnicity matched with 39 healthy children from local schools in Surrey (England). Dental examinations were completed for indices for both the primary and permanent dentitions comprising decayed, missing and filled teeth and surfaces, plaque index, gingivitis index, developmental enamel defects, and incisor tooth trauma. RESULTS: There was no significant difference in the dmfs, dmft, DMFS or DMFT in the children with epilepsy compared with the controls. There was a significantly greater mean plaque score associated with permanent teeth in the children with epilepsy 68.0 SD+/- 31.5, compared with the control children, 142.9 SD+/- 23.2, p<0.0001. The mean +/- SD gingivitis score was significantly greater in the children with epilepsy 47.9+/-33.8, compared with the control children, 15.85+/-21.8, p<0.001. A significantly greater number of children with epilepsy had experienced anterior tooth trauma, 54% in all, compared with the controls, 12.5% p<0.0001. Although children with epilepsy had greater mean plaque and gingivitis scores, the prevalence of dental caries was low. Children and teenagers with intractable epilepsy were more likely to have sustained dental trauma than controls. CONCLUSIONS: A dental service aimed at early attention to anterior tooth trauma is needed. In addition, there is an ongoing need for improving the oral hygiene of these individuals to prevent the development of periodontal disease in later life.

Dental emergencies presenting to a university-based paediatric dentistry clinic in the West Indies

OBJECTIVES: The aims of the present study were to investigate the type and prevalence of dental emergencies presenting at a teaching hospital paediatric emergency clinic in Trinidad, and to describe the socio-demographic factors related to the use of the service. DESIGN AND METHODS: The authors used a prospective study of consecutive dental patients presenting to a paediatric emergency clinic. Data recorded included type of presenting emergency and socio-demographic variables. RESULTS: Data were available for 309 participants; 47 percent were male and 53 percent female. The average age of the participants was 8.66 years (SD = 3.75 years; range = 1-16 years). Seventy-three percent of the participants' parents were involved in manual work or unemployed, and 21 percent were in nonmanual/professional employment; the occupation was not known in 6 percent of cases. Caries-related problems accounted for 74 percent of emergencies. Dental trauma mostly affected the upper permanent incisor teeth, with concussion, subluxation and intrusion being the most commom injuries. CONCLUSION: Dental emergencies presenting to this university-based clinic were predominantly related to caries and trauma. The service was more frequently utilized by children in the mixed dentition stage, children from lower socioeconomic groups and those living in the local area. The frequency of caries-related problems indicates the need for more community-based preventive strategies, including encouraging greater attendance for routine dental care and dental health education. Strategies for oral health promotion should be developed to prevent dental trauma (AU)

Dental emergencies presenting to a university-based paediatric dentistry clinic in the West Indies.

OBJECTIVES: The aims of the present study were to investigate the type and prevalence of dental emergencies presenting at a teaching hospital paediatric emergency clinic in Trinidad, and to describe the socio-demographic factors related to the use of the service. DESIGN AND METHODS: The authors used a prospective study of consecutive dental patients presenting to a paediatric emergency clinic. Data recorded included type of presenting emergency and socio-demographic variables. RESULTS: Data were available for 309 participants; 47% were male and 53% female. The average age of the participants was 8.66 years (SD = 3.75 years; range = 1-16 years). Seventy-three per cent of the participants' parents were involved in manual work or unemployed, and 21% were in nonmanual/professional employment; the occupation was not known in 6% of cases. Caries-related problems accounted for 74% of emergencies. Dental trauma mostly affected the upper permanent incisor teeth, with concussion, subluxation and intrusion being the most common injuries. CONCLUSION: Dental emergencies presenting to this university-based clinic were predominantly related to caries and trauma. The service was more frequently utilized by children in the mixed dentition stage, children from lower socioeconomic groups and those living in the local area. The frequency of caries-related problems indicates the need for more community-based preventive strategies, including encouraging greater attendance for routine dental care and dental health education. Strategies for oral health promotion should also be developed to prevent dental trauma.

Cryopreservation of embryogenic tissue of a range of genotypes of sweet potato (Ipomoea batatas [L] Lam.) using an encapsulation protocol.

Embryogenic tissue of nine sweet potato [Ipomoea batatas (L.) Lam] genotypes from Asia, Africa and the Americas was established from in vitro axillary buds on Murashige and Skoog medium supplemented with 2,4-dichlorophenoxyacetic acid or 2,4,5-trichlorophenoxyacetic acid. Embryogenic aggregates, 1.0-2.0 mm in diameter, were encapsulated in alginate gel, precultured on medium containing elevated levels of sucrose and dehydrated prior to rapid freezing in liquid nitrogen. The maximum survival of embryogenic tissue ranged from 4% to 38%, depending on the genotype. With the incorporation of a slow-cooling step, survival was generally much higher than that obtained after rapid freezing alone. Five of eight genotypes tested with this protocol gave survival percentages in excess of 55%, and a further two in excess of 33%, all after evaporative dehydration. The most effective sucrose treatment(s), however, varied with the genotype.

Management of severe bronchiolitis: indications for ventilator support.

AIM: Bronchiolitis is a common respiratory illness in children. We reviewed our experience of children under one year presenting to an intensive care unit with a clinical diagnosis of bronchiolitis in order to determine if ethnicity, prematurity, arterial carbon dioxide tension or nasopharyngeal aspirates positive for respiratory syncytial virus were related to the need for ventilator assistance. METHOD: A review of the charts of all infants with bronchiolitis admitted to the paediatric intensive care unit from December 1991 to February 1994 was undertaken. RESULTS: There were 94 infants. Ventilator assistance was given to 24 children--nine children had nasopharyngeal continuous positive airway pressure and 15 children required intermittent positive pressure ventilation. There was no difference in ethnic mix between the respiratory support group (Maori 45%, Pacific Islands 30%, other 25%) and those children managed conservatively (Maori 40%, Pacific Islands 36%, other 24%). Fifteen of the 24 infants who needed ventilator support were born prematurely. The mean (corrected) age of infants who required respiratory support was 1.79 (SD2.98) months compared to 3.32 (SD2.58) months for those infants who did not (p < 0.01). We were able to match 19 of the 24 infants who required ventilator support by age, sex and ethnicity with a nonventilated child. There was no significant difference in admission PaCO2 between groups (7.7 SD 1.5 vs 8.1 SD 1.5 kPa) or highest PaCO2 in the first 24 hours for nonventilated children and preintubation PaCO2 in ventilated children (8.6 SD1.3 vs 8.9 SD 1.9kPa). Nasopharyngeal aspirates were positive for respiratory syncytial virus in 39 patients. Respiratory support was required for 13 children who had positive RSV aspirates and for nine children who were not RSV positive (NS). CONCLUSION: Infants with bronchiolitis that were premature were not likely to need respiratory support. Ethnicity, arterial PaCO2 and positivity for RSV were not related to the need for ventilator assistance.

The niche of the gill parasite Dactylogyrus banghami (Monogenea: Dactylogyridae) on Notropis stramineus (Pisces: Cyprinidae).

Distribution of the monogenean Dactylogyrus banghami on the gills of the fish Notropis stramineus (Cyprinidae) was described by calculation of mean relative positions and Levins' niche breadths on the linear spatial resource gradients gill filament length and gill arch length. Thirty fish with 276 worms were examined; only 1 of the fish had an additional gill parasite species (Trichodina sp). Worms were more broadly and evenly distributed across the length of the gill arch than they were on the filament (breadths of 0.91 and 0.67, respectively). Mean worm positions were near the center of both resources: 54% of the distance from the arch cartilage on the filament, and 54% from the ventral end of the arch itself. The results are considered consistent with predictions about the niche structures of species in unsaturated noninteractive specialist communities.

The roles of ecological and evolutionary influences in providing structure to parasite species assemblages.

Parasite species assemblages currently are thought to range from isolationist to interactive, their dynamic properties being related to the number of species and types of hosts involved. The literature contains few experimental tests of this concept, however, and many of the host/parasite systems studied to date are not amenable to experimental manipulation. In this review, the presence of a parasite species, in a sample of host individuals, is considered to be an evolutionary phenomenon, but the parasite's population structure is considered to be an ecological one. Studies that allow evaluation of these 2 influences are comparative in nature and include data from a series of homogeneous samples of host populations. A lottery model is presented, in which hosts acquire their assemblages of parasites by Monte Carlo type sampling from multiple kind arrays; the major structuring influence is the relative probability of becoming infected by various parasite species. Claims of parasite species interaction need to be supported by studies showing departures from the predictions of this model. The species density and infraassemblage diversity index distributions are recommended as quantitative tools useful in such work.

Host stadium specificity in the gregarine assemblage parasitizing Tenebrio molitor.

Reciprocal cross-stadia experimental infections were used to demonstrate stadium specificity within the gregarine assemblage parasitizing Tenebrio molitor, the yellow mealworm. Gregarina cuneata, Gregarina polymorpha, and Gregarina steini are characteristic parasites of larval T. molitor. Gregarina niphandrodes is a characteristic parasite of adult T. molitor. Experimental infections were produced in all homologous host-parasite combinations. No infection was produced in heterologous or cross-stadia combinations. This study introduces the concept of separate, distinct parasite niches corresponding to separate life cycle stages and established by known, predictable life cycle events within a single host species.