overview of the most common enzyme defect, glucose-6-phosphate-dehydrogenase deficiency

Background: Glucose-6-Phosphate-Dehydrogenase [G6PD] deficiency is the most common enzyme deficiency globally and is more remarkable in certain parts of the world which had high malaria prevalence in the past. It is an X-linked genetically inherited disorder, where the first presentation can be neonatal jaundice. There are over 300 variants of this disorder based upon the genetics. Although the morbidity and mortality is not very high, the deficiency can be more appropriately managed by proper preventive methods which include screening and avoidance of trigger. Also prompt management of acute hemolysis can save a child from many complications

Methodology: we conducted this review using a comprehensive search of MEDLINE, PubMed, and EMBASE from January 1987 to March 2017. The following search terms were used: G6PD deficiency, prevalence of G6PD, genetics of G6PD deficiency, management and diagnosis of G6PD

Aim of the work: this study aimed to understand about the etiology, pathophysiology and study various lines of prevention and management of G6PD deficiency in pediatric age group

Conclusion: proper preventive and treatment methods can avoid negative effects on the child's quality of life and reduce morbidity and mortality, therefore the child's care takers must be well informed

Basic considerations in cleft lip reconstruction

Reconstruction of a congenital lip is a simple procedure, but not as simple as "freshen the edges and approximate the wound". To have a good cosmetic and functional results a background knowledge of embryology and precise recognition of anatomical structures and land marks of the lip and its surrounding structures are necessary

Preoperative evaluation of eustachian tube function in reconstructive middle ear surgery

A correlation between preoperative eustachian tube functional results with postoperative stability and function of the ears undergoing reconstructive surgery i.e. [Tympano-plasties] is presented from our Institute. The study was conducted on 24 patients with a variety of Chronic middle ear diseases. The ear pathology included both tubo-tympanic and attico-antral diseases with or without cholesteatoma. Preoperatively, patients were divided into three main groups on the basis of tubal functions. The same patients were then followed up postoperatively for a period of three to six months and their ears were evaluated with regards to anatomical and functional stability. It was confirmed that a physiologically functioning eustachian tube in a chronic suppurative Otitis Media results in a successful tympano plasty both from anatomical and functional aspects