Primary Autosomal Recessive Distal Renal Tubular Acidosis Caused by a Common Homozygous SLC4A1 Mutation in Two Lao Families.

Primary distal renal tubular acidosis (dRTA) caused by mutations of the SLC4A1 gene, which encodes for erythroid and kidney isoforms of anion exchanger, shows marked difference in inheritance patterns and clinical features in different parts of the world. While the disease shows autosomal dominant inheritance without any red cell morphological abnormalities in the temperate countries, it is almost invariably recessive, and often accompanies red cell morphological abnormalities or hemolytic anemia in the tropics, especially in Southeast Asia. Here, we report three patients with autosomal recessive (AR) dRTA, presenting with typical findings of failure to thrive and rickets, from two unrelated Lao families. The mutational analyses revealed that all three patients harbored the same homozygous SLC4A1 mutation, p.Gly701Asp. Adequate supplementation of alkali and potassium resulted in remarkable improvement of growth retardation and skeletal deformities of the patients. This is the first case report of Lao patients with AR dRTA caused by SLC4A1 mutations.

Congenital heart disease at Laos Children's Hospital: Two year experience.

BACKGROUND: Management of congenital heart disease (CHD) in developing countries is challenging because of limited access to health-care facilities and socioeconomic limitations. The aim of this study was to describe the recent experience with CHD at Children's Hospital, Vientiane, Laos, the only pediatric referral hospital in the country. METHODS: From July 2013 to November 2015, 1009 echocardiograms were carried out in 797 individuals who visited Children's Hospital, in whom CHD was identified in 213. Demographic characteristics, echocardiography and age at first CHD diagnosis on echocardiogram were compared by residential area. RESULTS: Among the 213 patients, the most frequent anomalies, in descending order, were ventricular septal defect, atrial septal defect, patent ductus arteriosus, and tetralogy of Fallot. Moderate or severe CHD requiring surgery or intervention was detected in 137 patients; median age at initial diagnosis was 6.0 months (IQR, 1.5-29.8). Among those with moderate or severe CHD, 89 patients were from rural areas and 48 from the capital area. The proportion of patients from rural areas older than 1 and 3 years at diagnosis was 46.1% and 32.6%, respectively. In contrast, patients from the capital area were diagnosed at a significantly earlier age: 16.7% at age >1 year and 6.2% at age >3 years (both P < 0.001 vs rural areas). CONCLUSIONS: Diagnosis of CHD requiring surgery is delayed in Laos, especially in rural areas. Better education and training of local medical staff, and greater access to medical advice are required to improve CHD care in Laos.

Childhood encephalitis in the Greater Mekong region (the SouthEast Asia Encephalitis Project): a multicentre prospective study.

BACKGROUND: Encephalitis is a worldwide public health issue, with a substantially high burden among children in southeast Asia. We aimed to determine the causes of encephalitis in children admitted to hospitals across the Greater Mekong region by implementing a comprehensive state-of-the-art diagnostic procedure harmonised across all centres, and identifying clinical characteristics related to patients' conditions. METHODS: In this multicentre, observational, prospective study of childhood encephalitis, four referral hospitals in Cambodia, Vietnam, Laos, and Myanmar recruited children (aged 28 days to 16 years) who presented with altered mental status lasting more than 24 h and two of the following minor criteria: fever (within the 72 h before or after presentation), one or more generalised or partial seizures (excluding febrile seizures), a new-onset focal neurological deficit, cerebrospinal fluid (CSF) white blood cell count of 5 per mL or higher, or brain imaging (CT or MRI) suggestive of lesions of encephalitis. Comprehensive diagnostic procedures were harmonised across all centres, with first-line testing was done on samples taken at inclusion and results delivered within 24 h of inclusion for main treatable causes of disease and second-line testing was done thereafter for mostly non-treatable causes. An independent expert medical panel reviewed the charts and attribution of causes of all the included children. Using multivariate analyses, we assessed risk factors associated with unfavourable outcomes (ie, severe neurological sequelae and death) at discharge using data from baseline and day 2 after inclusion. This study is registered with ClinicalTrials.gov, NCT04089436, and is now complete. FINDINGS: Between July 28, 2014, and Dec 31, 2017, 664 children with encephalitis were enrolled. Median age was 4·3 years (1·8-8·8), 295 (44%) children were female, and 369 (56%) were male. A confirmed or probable cause of encephalitis was identified in 425 (64%) patients: 216 (33%) of 664 cases were due to Japanese encephalitis virus, 27 (4%) were due to dengue virus, 26 (4%) were due to influenza virus, 24 (4%) were due to herpes simplex virus 1, 18 (3%) were due to Mycobacterium tuberculosis, 17 (3%) were due to Streptococcus pneumoniae, 17 (3%) were due to enterovirus A71, 74 (9%) were due to other pathogens, and six (1%) were due to autoimmune encephalitis. Diagnosis was made within 24 h of admission to hospital for 83 (13%) of 664 children. 119 (18%) children had treatable conditions and 276 (42%) had conditions that could have been preventable by vaccination. At time of discharge, 153 (23%) of 664 children had severe neurological sequelae and 83 (13%) had died. In multivariate analyses, risk factors for unfavourable outcome were diagnosis of M tuberculosis infection upon admission (odds ratio 3·23 [95% CI 1·04-10·03]), coma on day 2 (2·90 [1·78-4·72]), supplementary oxygen requirement (1·89 [1·25-2·86]), and more than 1 week duration between symptom onset and admission to hospital (3·03 [1·68-5·48]). At 1 year after inclusion, of 432 children who were discharged alive from hospital with follow-up data, 24 (5%) had died, 129 (30%) had neurological sequelae, and 279 (65%) had completely recovered. INTERPRETATION: In southeast Asia, most causes of childhood encephalitis are either preventable or treatable, with Japanese encephalitis virus being the most common cause. We provide crucial information that could guide public health policy to improve diagnostic, vaccination, and early therapeutic guidelines on childhood encephalitis in the Greater Mekong region. FUNDING: Institut Pasteur, Institut Pasteur International Network, Fondation Merieux, Aviesan Sud, INSERM, Wellcome Trust, Institut de Recherche pour le Développement (IRD), and Fondation Total.

Integrated Management of Neonatal and Childhood Illness Training in Lao PDR: A Pilot Study of an Adaptable Approach to Training and Supervision.

Integrated Management of Neonatal and Childhood Illness (IMNCI) has been part of the national strategy for child health in Lao Peoples Democratic Republic since 2003. The program, while running for an extended period, has faced multiple challenges including maintaining the teaching quality for the implementation of the IMNCI guidelines and a structure to enable and support healthcare workers trained to apply the training in their workplace. A revised training model that focused on building skills for teaching according to adult learning principles in a pool of facilitators, a practical and hands-on training workshop for healthcare workers, and the establishment of a program of health center supervision was developed and implemented in three provinces. Participants in the revised model reported increased confidence in implementing IMNCI guidelines, they demonstrated competence in the steps of IMNCI and on follow-up assessment at a supervision visit were found to have improved patient care through the measurement of pediatric case management scores. This study highlights the importance of a focus on education to ensure the translation of guidelines into practice and thereby lead to improvements in the quality of pediatric care. The IMNCI training approach is acceptable and valued by healthcare worker participants.

The first picture archiving and communication system in Lao People's Democratic Republic: Changes in the utilization rate of imaging tests in the first year after implementation.

BACKGROUND: Implementation of the Picture Archiving and Communication System (PACS) is more challenging in developing countries than in developed countries. Given that the first PACS in Lao People's Democratic Republic (PDR) was successfully installed at the Children's Hospital of Lao PDR, we aimed to investigate whether the utilization rate of imaging tests increased after PACS implementation. METHODS: PACS was implemented at the Children's Hospital of Lao PDR in December 2014. We compared the utilization rates of imaging tests including X-ray and ultrasound examinations between the pre-PACS period (from December 2013 to November 2014) and the post-PACS period (from December 2014 to November 2015). The utilization rate was defined as the number of imaging tests divided by the number of patients per month. RESULTS: The average number of total imaging tests was 225.8/month (standard deviation [SD], 37.7) during the 1-year pre-PACS period and was 269.4/month (SD, 38.5) during the 1-year post-PACS period (P=0.0103). The utilization rate of total imaging tests significantly increased after PACS implementation (pre-PACS, 2.47%/month; post-PACS, 4.23%/month; P<0.0001). Increased utilization rates were observed for both X-rays (pre-PACS, 1.65%/month; post-PACS, 2.38%/month; P=0.0004) and ultrasound examinations (pre-PACS, 0.82%/month; post-PACS, 1.85%/month; P=0.0001). CONCLUSIONS: The implementation of PACS at the Children's Hospital of Lao PDR resulted in a significant increase in the utilization rate of imaging tests, suggesting the indirect benefit of improved quality of care. Our findings showed that the benefits of PACS can be realized even in a resource-limited country such as Lao PDR.