Zafar Nazir

We report peri- and post -operative management of haemostasis in a 11 -year old girl with Glanzmann Thrombasthenia (GT) who had feminizing genitoplasty for genital ambiguity due to Congenital Adrenal Hyperplasia (CAH-21 Hydroxylase deficiency). A blend of Glanzmann Thrombasthenia (GT) and DSD 46XX due to CAH is not reported in literature. Surgery particularly genitourinary reconstruction in patients with GT is challenging due to risk of intra and post -operative bleeding. Haemostasis can successfully be achieved with platelet transfusions, antifibrinolytic (Tranexamic acid) and judicious use of recombinant factor VIIa (rFVIIa) even in a resource limited setting.

In the last decade, renal stones are being diagnosed more frequently in children across the globe. Children with renal stone often present with non-specific and subtle symptoms. Diagnosis of urolithiasis in children therefore requires a high index of suspicion especially for those living in endemic regions and with a positive family history. Additionally, management requires judicious use of radiological imaging by treating physicians.

Introduction Tracheotomy in its earlier days was most commonly performed for acute airway infection in children. Its indications are now changing; it is now most commonly performed for congenital malformations (McMurray and Prescott in Practical pediatric otolaryngology. W.B. Saunders Company, Philadelphia, pp 575–592, 1996). This shift in indication has increased the rate of survival of such patients, and therefore the number of children going home after tracheostomy has also increased. Objective This study was conducted (1) to observe the pattern of indication and complications for tracheostomy, in our part of the world, (2) the rate at which tracheostomy can help wean patients off the ventilator, and (3) the feasibility of sending these children home with tracheostomy. Materials and methods A retrospective study was done on 127 patients. The indications, final outcome and the complications encountered in and outside the hospital were studied through review of charts. Results Based on the main indications, patients were grouped into: prolonged ventilation group (PV) 61%, followed by mechanical obstruction group (MO) 22%, and the last being adjunct to surgery group (AS) 17%. The in-hospital complication rate was 30% and that at home was 18.11%. The most common complications included upper respiratory tract infections, and blockage or displacement of tubes. The late complication rate was 4%. Hundred (78.8%) patients on the ventilator could be successfully weaned off, with a p value of 0.001; 81 were sent home with the tracheostomy tube (TT). Forty of these were successfully decannulated and the overall decannulation rate was 48.8%. Conclusion A large number of tracheostomies have been performed in the PV group to reduce the intensive care unit (ICU) stay and to prevent nosocomial infections. The need arises from the high cost of prolonged stay in an ICU setup, which is a cause of major economic burden, and lack of financial assistance for these patients worsens the scenario. Home care of the tracheostomy tubes remains a good option for patients requiring long periods of time to overcome their primary pathology.