Supportive & palliative interventions in motor neurone disease: what we know from current literature?

Although there is no cure for motor neurone disease (MND), the advent of supportive interventions including multidisciplinary care (MDC) has improved treatment interventions and enhanced quality of life (QOL) for MND patients and their carers. Our integrative review showed evidence-based MDC, respiratory management and disease-modifying therapy that have improved the outcomes of patients diagnosed with MND. Supportive approaches to nutritional maintenance and optimization of symptomatic treatments, including management of communication and neuropsychiatric issues, improve the QOL for MND patients.

End-of-life Characteristics and Palliative Care Provision for Patients With Motor Neuron Disease.

Motor neuron disease (MND) is a neurodegenerative disease and manifested as progressive decline in physical, respiratory, swallowing and communication function, and ultimately death. Traditional model of care was fragmented and did not match with multifacet needs of patients and carers. Furthermore, there could be lack of integrated care at end of life for patients with MND in most lower- and middle-income countries or in places with inadequate palliative care (PC) coverage.

Cardiac Mechanics in Children with Bronchopulmonary Dysplasia.

Background: Disruption of pulmonary vasculogenesis occurs in bronchopulmonary dysplasia (BPD). Increased impedance to pulmonary flow secondary to abnormal vascular development may affect ventricular mechanics.

Objectives: We aimed to test the hypothesis that cardiac mechanics are altered in prematurely born children with BPD.

Cost-benefit analysis of hyperphenylalaninemia due to 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency: for consideration of expanded newborn screening in Hong Kong.

Objectives: To evaluate the cost-benefit of implementing an expanded newborn screening programme for hyperphenylalaninemias due to 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency in Hong Kong.

Setting: Regional public hospitals in Hong Kong providing care for cases of inborn errors of metabolism.

Methods: Implementational and operational costs of a new expanded mass spectrometry-based newborn screening programme were estimated.

Expanded Prader-Willi syndrome due to chromosome 15q11.2-14 deletion: report and a review of literature.

We report on a male infant with de novo unbalanced t(5;15) translocation resulting in a 17.23 Mb deletion within 15q11.2-q14 and a 25.

Risk factors associated with outcomes of very low birthweight infants in four Asian countries.

Aim: The study aims to determine the risk factors associated with mortality and necrotising enterocolitis (NEC) among very low birthweight infants in 95 neonatal intensive care units in the Asian Network on Maternal and Newborn Health.

Methods: This is a cross-sectional study using an international collaborative database of 17,595 very low birthweight infants admitted within 28 days of birth between 2003 and 2006 in four Asian countries. Information on the mortality and morbidity of neonates admitted to the neonatal intensive care units was recorded.

Bacillus bloodstream infections in a tertiary perinatal centre: an 8-year study.

Background: Isolates of nonanthrax Bacillus species in clinical samples are frequently considered as contaminants. However, there were case reports describing Bacillus sepsis among infants, associated with high mortality and morbidity.

Methods: We performed a retrospective review of the clinical and epidemiological features of Bacillus bacteremia at our neonatal intensive care unit from January 2002 to December 2009.

Bordetella bronchiseptica Pneumonia in an Extremely-Low-Birth-Weight Neonate.

Bordetella bronchiseptica, a gram-negative coccobacillus, is a common veterinary pathogen. In both domestic and wild animals, this bacterium causes respiratory infections including infectious tracheobronchitis in dogs and atrophic rhinitis in swine. Human infections are rare and have been documented in immunocompromised hosts.

Gastric pneumatosis in a premature neonate.

Gastric pneumatosis is extremely rare during infancy. It has been reported in association with necrotizing enterocolitis or congenital abnormalities such as pyloric stenosis. Here, we report a case of gastric pneumatosis in a premature neonate on synchronized nasal intermittent positive pressure ventilation.

Review of primary hypothyroidism in very low birthweight infants in a perinatal centre in Hong Kong.

Aims: To review the incidence and risk factors of primary hypothyroidism in very low birthweight (VLBW) infants in our centre and to assess their neurodevelopmental outcome.

Method: Retrospective, descriptive review of VLBW infants with primary hypothyroidism from 1 January 2000 to 31 December 2008 in a perinatal centre. Case control comparisons for neurodevelopmental outcome at 18 months, nested to a prospective VLBW cohort (Vermont Oxford Database).

Neurodevelopmental outcome of severe neonatal hemolytic hyperbilirubinemia.

We recruited 128 neonates with hyperbilirubinemia over a 5-year period (1995-2000) to study the short- and long-term effects of hemolytic hyperbilirubinemia on the auditory brainstem pathway and neurodevelopmental status. These children were divided into two groups: (1) a hemolytic group (n = 29; ABO incompatibility [n = 19], Rh incompatibility [n = 1], glucose-6-phosphate dehydrogenase deficiency [n = 8] and both ABO incompatibility and glucose-6-phosphate dehydrogenase deficiency [n = 1]) and (2) a nonhemolytic group (n = 99). All received phototherapy.

Short- and long-term outcome of severe neonatal nonhemolytic hyperbilirubinemia.

We studied the effects of hyperbilirubinemia on brainstem auditory pathways and neurodevelopmental status in 99 full-term neonates with severe nonhemolytic hyperbilirubinemia (total serum bilirubin level = 301 to 500 micromol/L) born between 1995 and 2000. These were divided into three groups: group 1, moderate hyperbilirubinemia (n = 30; mean maximum total serum bilirubin = 320.7 micromol/L or 18.

Randomized trial comparing two natural surfactants (Survanta vs. bLES) for treatment of neonatal respiratory distress syndrome.

Exogenous surfactants have been used as an effective treatment of neonatal respiratory distress syndrome (RDS). Different preparations of surfactant carry different biophysical and clinical properties. To study the response pattern and treatment outcome of two natural surfactants (bLES and Survanta) for the treatment of RDS, we conducted a randomized clinical trial at the neonatal unit of a university teaching hospital.

Perinatal outcome in large-for-gestational-age infants. Is it influenced by gestational impaired glucose tolerance?

Objective: To examine the relationship between the World Health Organization category of impaired glucose tolerance (IGT) (two-hour value of the 75-g oral glucose tolerance test at 8-10.9 mmol/L) and outcome in large-for-gestational age (LGA) infants to determine whether IGT affects perinatal morbidity in addition to affecting infant size.

Study Design: A retrospective study was performed on 461 LGA newborns (birth weight > 90th percentile) from singleton pregnancies delivering after 36 completed weeks in a 12-month period to determine the difference in perinatal outcome between nondiabetic pregnancies (n = 382) and pregnancies with diet-treated IGT (n = 79).