Adherence and Cost Effectivity of Home-Based Prophylaxis Over Institutionalized Prophylaxis in Patients with Hemophilia

Purpose: Home-based prophylaxis in hemophilia facilitates the treatment of patients with hemophilia (PwH) at home resulting in an improved quality of life, experiencing less pain and greater flexibility in daily activities. This literature studies the cost effectivity and adherence to prophylaxis treatment after the implementation of home-based prophylaxis therapy in PwH registered under the Hemophilia Treatment Centre (HTC) of Assam Medical College and Hospital. Materials and Methods:?PwH and their parents were advised for self/home infusion after being trained by a medical professional for 6 months. Data were collected on the skip in prophylaxis treatment by PwH and their traveling cost to access the prophylaxis treatment before and after the implementation of home infusion, through questionnaire and telephonic interview. Results:?The mean number of days of skip in prophylaxis was significantly reduced from 25 (±11) to 4 (±2) days after implementation of home infusion. The mean transportation cost was also found to be significantly decreased from Rs. 3297 (±2251) to 440 (±279). Before home/self-infusion, 77% of the registered PwH were found to skip prophylaxis doses more than 12 times a year but after home infusion, no PwH were found to skip more than 12 doses a year. Conclusion:?Home therapy facilitates the PwH to strictly adhere to the prophylaxis regime significantly reducing the skipping of doses to be administered to the PwH. The risks of regular traveling and the burden of transportation expenditure to avail the prophylaxis treatment was also found to be reduced significantly.

Study of Aetiological and Endoscopic Profile of Patients with Upper Gastrointestinal Bleed in North East India - A Hospital Based Cross Sectional Study

Upper Gastro-Intestinal Bleeding (UGIB) is one of the common complaints with which patients present to casualty. It is associated with significant morbidity and mortality. The aetiological spectrum of UGIB is variable in different geographical regions. Our study aimed to analyse the aetiology, endoscopic profile, mortality, Rockall score and predictors of mortality in patients with UGIB, in North East India. METHODSThis cross-sectional study was conducted at Assam Medical College and Hospital in North East India. We enrolled patients with age 12 years and above, who were admitted between July 2019 and January 2020 with a history suggestive of UGIB. Demographic data of the patients was collected, after which they underwent clinical examination, and upper GI endoscopy. Mean ± standard deviation was used to express continuous variables. Frequency and percentage were used to express categorical variables. Test of significance for qualitative data was assessed by Chi-square test (for 2 x 2 tables). P value less than 0.05 was taken as statistically significant. RESULTSWe analysed 117 patients diagnosed with UGIB [80.34 % male, 19.60 % female], ratio of male to female of [4.08:1] was seen. The most common symptom was melena 87 patients (74.15 %), endoscopy finding showed that 48.71 % had oesophageal and / or gastric varices, 26.49 % had peptic ulcers, 17.94 % had gastric erosions / duodenal erosions / erosive gastritis, 1.7 % had Mallory-Weiss tear, 1.7 % had gastric malignancy, 1.7 % had GJ stoma bleed, 1.7 % had both oesophageal varices and peptic ulcer disease. Partial gastric outlet obstruction was observed in peptic ulcer disease in 2 patients (6.45 % of total peptic ulcer disease patients). 73.75 % patients had Rockall score < 5 and 26.49 % patients had Rockall score > 6. H. pylori infection (assessed by RUT) was an independent predictor of upper GI bleed in both variceal and non-variceal bleed [p < 0.001]. The mortality in our study was 7.69 %. Predictors of mortality in the study population were, patients with variceal bleed [p = < 0.001], Rockall score > 6 [p = 0.013], and chronic liver disease [p < 0.001]. The average duration of hospital admission of the study population is about 4.6 + / - 0.4 days. CONCLUSIONSThe study reported oesophageal varices was the most common cause of UGIB, followed by peptic ulcer in North East India. H. pylori was an independent predictor of both variceal and non-variceal bleed. Partial gastric outlet obstruction (GOO) was one of the common benign complication of peptic ulcer disease. Variceal bleed, Rockall score > 6, chronic liver disease were predictors of mortality.

Unilateral Lung Hypoplasia - A Rare Presentation in Adulthood

Lung hypoplasia is one of the developmental anomalies of the lung parenchyma, in which there is a decrease in the number and size of airways, alveoli and vessels. Here is a case of 36-year-old female with a history of fever, cough with expectoration, dyspnoea, with past history of similar complaints suggestive of recurrent respiratory infections. Chest x ray revealed homogenous opacity in left chest. HRCT thorax set the diagnosis of left pulmonary hypoplasia with compensatory hyperinflation of right lung. Patient was treated conservatively and was advised follow up. Lung hypoplasia majority of the times diagnosed immediately after birth, it is associated with severe respiratory failure and high mortality rate. There are less severe, unilateral forms in which they usually survive with compensatory hyperinflation of the opposite lung and may not be diagnosed till adulthood. Patients usually does not have any symptoms, but patients may present with symptoms suggestive of chronic bronchitis and recurrent lower respiratory tract infections. HRCT thorax helps to differentiate it from other congenital or acquired conditions. Treatment will be conservative, surgical resection is indicated in cases with severe cystic changes and severe symptoms.Lung hypoplasia comes under the spectrum of developmental anomalies of lung, which causes incomplete development of lung tissue with decrease in the total number of lung cells, airways and alveoli, which finally leads to reduction in lung size and weight. It may be unilateral or bilateral and can lead to severe respiratory failure and death immediately after birth, which is one of the common causes of perinatal mortality.1The less severe forms of lung hypoplasia can survive and may not be diagnosed till adulthood, many of the times in asymptomatic patients it is detected as an incidental finding on radiological imaging, or while investigating for recurrent respiratory tract infections. HRCT thorax is the diagnostic tool of choice.2 Here we present a case report of a 36-year-old female patient, with unilateral pulmonary hypoplasia and history of recurrent respiratory tract infections since last 10 years.

An Undiagnosed Case of Epilepsy for 13 Years Presenting in Status Epilepticus.

Epilepsy, one of the most common neurological diseases globally is a chronic non-communicable disease that affects people of all ages. Around 50 million people worldwide are suffering from epilepsy at present. The risk of premature death is up to three times higher than that of general population. Up to 70% of the people living with epilepsy could live seizure- free if properly diagnosed and treated.[1] General examination is more often missed in an epilepsy patient or seizure disorder patient, but unfortunately it plays an important role when diagnosis is concerned. Here we mention a case report of 14 year old girl suffering from epilepsy, who remains undiagnosed for 13 years with characteristics clinical and radiological features of Tuberous sclerosis.Tuberous sclerosis is a type of true phakomatoses (congenital neuroectodermoses). As the name suggests it mainly affects the CNS and cutaneous system. It is characterised by development of benign tumours affecting brain, skin, retina, visceras and cutaneous changes. It is an autosomal dominant disorder but with variable penetrance in which a variety of lesions in the skin, kidney, heart, nervous system and other organs. Bourneville's articles (between 1880- 1900), presented the first systematic features of the disease and related the cerebral lesions to those of skin of the face. It is equally frequent in all the races and in both the sexes.[2] The incidence is estimated to be 1 case per 6000 live birth, with a prevalence of 1 in 10,000 births.[3] The mutation occurs either in one of the two sites-the long arm of chromosome known as TSC 1 (Hamartin), or in the short arm of chromosome 16 known as TSC 2 (tuberin). Because of mosaicism approximately 15% of sporadic cases show no identifiable mutation and tend to have milder manifestations. In a study conducted on 325 TSC individuals, 17% of the mutations were found in the TSC1 gene, 50% in the TSC2 gene, 4% unclassified variants, and 29% with no identifiable mutations.[4] The disease is basically a triad of epilepsy, developmental delay and adenoma sebaceum known as ″EPILOIA″.[2] Here we present a case report of 14 year old female patient with clinical and radiological features of TSC