Mid-aortic syndrome (MAS) is a rare clinical entity that is
characterized by coarctation involving the distal thoracic and/or
abdominal aorta and its major branches accounting for 0.5–2%
of all cases of coarctation of the aorta (1). Renovascular
hypertension can be a significant sequelae - it is the main
symptomatic presentation of this disease among children and
adolescents. We describe a 9-year-old girl who presents with
recurrent abdominal pain and symptomatic hypertension. Due to
significant left ventricular systolic dysfunction and uncontrolled
hypertension, percutaneous balloon angioplasty was performed
to treat the coarctation. To our knowledge, this is the first
reported case of MAS in Malaysia. This case report highlights
the clinical presentation, the role of computed tomography
angiogram (CTA) in the diagnosis and current options in the
management of MAS.
Congenital pneumonia is one of the common causes of respiratory distress at birth with significant morbidity and mortality in infants. Estimates show that neonatal pneumonia including congenital pneumonia contributes to between 750 000 and 1.2 million neonatal deaths every year which accounts for 10% global child mortality. Etiological agents are many and vary but atypical bacterial causes are few. The commonest cause for atypical bacteria is Ureaplasma urealyticum. Congenital pneumonia is often clinically difficult to diagnose owing to poor specificity of clinical signs, with similarities in radiologic presentation with other respiratory conditions of the newborn. Isolation of causative organism (s) by culture from nasopharyngeal aspirates or tracheal aspirates obtained within 8 hours of life is the gold standard of its diagnosis. However, this technique is elaborate and time consuming in identifying atypical bacteria. Development of a more sensitive modality such as polymerase chain reaction (PCR) has dramatically altered the microbiological diagnosis of congenital pneumonia.
The present study aims to determine the limitations of traditional Jones criteria during the first episode of acute rheu- matic fever (ARF) at the initial referral hospital, in a cohort of patients below 18 years old who had undergone mitral valve repair in National Heart Institute (IJN) from 2011 to 2016. Carditis followed by fever and joint involvement were the most frequent manifestations at first diagnosis. Of the 50 patients, only seven (14%) fulfilled the traditional Jones criteria for the diagnosis of the first episode of ARF. When compulsory evidence of a previous group A Beta he- molytic streptococcus (GABHS) was disregarded, this figure rose to 54%. Therefore, strict adherence to Jones criteria with absolute documentation of GABHS will lead to underdiagnoses of ARF. The application of echocardiographic diagnostic criteria of rheumatic heart disease (RHD) needs to be emphasized to allow early diagnosis and adminis- tration of secondary prophylaxis to prevent progression to severe valvular disease.