Childhood Acquired Heart Diseases in Jos; North Central Nigeria

The patterns of childhood acquired heart diseases (AHD) vary in different parts of the world and may evolve over time. We aimed to compare the pattern of childhood AHD in our institution to the historical and contemporary patterns in other parts of the country; and to highlight possible regional differences and changes in trend. Materials and Methods: Pediatric echocardiography records spanning a period of 10 years were reviewed. Echocardiography records of children with echocardiographic or irrefutable clinical diagnoses of AHD were identified and relevant data extracted from their records. Results: One hundred and seventy five children were diagnosed with AHD during the period; including seven that had coexisting congenital heart disease (CHD). They were aged 4 weeks to 18 years (mean 9.84?4.5 years) and comprised 80 (45.7) males and 95 (54.3) females. Rheumatic heart disease (RHD) was the cause of the AHD in 101 (58.0) children; followed by dilated cardiomyopathy (33 cases; 18.9) which was the most frequent AHD in younger (under 5 years) children. Other AHD encountered were cor pulmonale in 16 (9.1); pericardial disease in 15 (8.6); infective endocarditis in 8 (4.6) and aortic aneurysms in 2 (1.1) children. Only one case each of endomyocardial fibrosis (EMF) and Kawasaki Disease were seen during the period. Conclusions: The majority of childhood acquired heart diseases in our environment are still of infectious aeitology; with RHD remaining the most frequent; particularly in older children. Community-based screening and multicenter collaborative studies will help to better describe the pattern of AHD in our country. More vigorous pursuit of the Millennium development goals will contribute to reducing the burden of childhood acquired heart diseases in the country

Libyan Boy with Autosomal Recessive Trait (p22-phox defect) of Chronic Granulomatous Disease

Chronic granulomatous disease (CGD) is a primary immune deficiency disorder of the phago-cytes. In this disorder, phagocytic cells (polymorphonuclear leukocytes and monocytes) can-not produce active oxygen metabolites and, therefore, cannot destroy the ingested intracellular bacteria. Clinically, patients with CGD usually have recurrent bacterial and fungal infections causing abscess and granuloma for-mation in the skin, lymph nodes and visceral organs.In this report, we present a boy from Libya with a rare autosomal recessive trait of CGD (defect of p22phox) who has chronic lun disease following multiple severe pneumonia attacks. The case we present suffered from bloody diarrhea since the third month of www.ljm.org.ly page163 Libyan Journal of Medicine, Volume 1, 2006ljmhis life. He also had recurrent episodes of fever, and later, developed persistent cervical lym- phadenitis and failure to gain weight. CGD is a very rare condi-tion worldwide. It is also not rec-ognized here in Libya, and usu-ally not in the list of differential diagnosis for chronic pulmonary infections. We advise that pediatricians and general practitioners who treat chronic cases of lung diseases (with or without chronic diarrhea) should consider primary immunodeficiency disorders in the hope that early diagnosis and treatment maprevent chronic complications especially of the respiratory tract. Furthermore, we state that, to the best of our knowledge, this is the first documented case of CGD from Libya.killing is usually caused by a defect in the nicotinamide adenine dinucleotide phosphate (NADPH) oxi-dase enzyme complex. NADPH is the enzyme that generates the microbicidal respiratory burst [2, 3]. The deficiencies of oxidative metabolism are usually detected with either the nitroblue tetrazolium (NBT) dye test or the dihydrorhodamine (DHR) oxidation test. These tests are based on the ability of products of oxidative metabolism to alter the oxidation states of reporter molecules so that they can be detected microscopically (in the case of NBT) or by flow cytometry (in the case of DHR) [4]. However, NBT test, al-though more conventionally used, is sometimes not sufficiently sen-sitive. Therefore, many investiga- tors have preferred the use of the DHR test [5]