No abstract

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.

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.

No abstract

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.