A case report: Familial glucocorticoid deficiency associated with familial focal segmental glomerulosclerosis

Background: Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive disorder characterized by isolated glucocorticoid deficiency in the presence of normal plasma renin and aldosterone level. Focal segmental glomerulosclerosis (FSGS) is a form of glomerular disease associated with proteinuria and nephritic syndrome. This is the first case of familial glucocorticoid deficiency associated with familial focal segmental glomerulosclerosis. Case Presentation: An eight month old boy presented with increased genital pigmentation. Initial investigation revealed that he was glucocorticoid deficient and was started on hydrocortisone and fludrocortisone with a diagnosis of primary adrenal insufficiency. Later fludrocortisone was withdrawn and he was diagnosed to have isolated glucocorticoid deficiency. He later developed focal segmental glomerulosclerosis for which he underwent renal transplantation at the age of five years. Now at the age of twelve years, this boy is doing well on hydrocortisone treatment. His two siblings and a first degree cousin also had isolated glucocorticoid deficiency. One of the above two siblings died due to renal failure secondary to focal segmental glomerulosclerosis. Conclusion: Patients with familial glucocorticoid deficiency should be carefully followed for development of features of nephrotic syndrome

A HYBRID NUMERICAL METHOD WITH GREATER EFFICIENCY FOR SOLVING INITIAL VALUE PROBLEMS

The main focus of this paper is to develop hybrid numerical method with greater efficiency for getting the solution of initial value problems (IVPs) in ordinary differential equations (ODEs) by merging the slopes used in Modified Euler’s method (MEM), Improved Euler’s method and a 2nd stage 2nd order contra harmonic mean method. Developed method has tested and analyzed for the stability, consistency and accuracy and observed that the developed method is more stable, consistent and accurate as compared to modified Euler’s method (MEM), Improved Euler’s method(IEM), modified improved modified Euler’s method (MIME) and a 2nd stage 2nd order contra harmonic mean method (C0M). Keywords: A hybrid numerical method, Ordinary differential equations, Initial value problems, Stability and consistenc

Male hypogonadism at a tertiary care hospital in Karachi, Pakistan

Background: Male hypogonadism is defined as \u27inadequate gonadal function, manifested by deficiency in gametogenesis and/or secretion of gonadal hormones\u27. Signs and symptoms of hypogonadism depend primarily on the age of onset. It can be classified according to the site primarily involved: the gonads, the hypothalamus, or the pituitary gland. The objective this study was to determine the presentation and aetiology of male hypogonadism seen in a tertiary care hospital. Methods: This cross-sectional study was conducted at Endocrine Clinics, Aga Khan University Hospital Karachi. Data of male patients with hypogonadism who attended clinics during January 2009 to August 2011 were reviewed. All male patients with clinical and biochemical evidence of hypogonadism were included in the study. Patients with Diabetes Mellitus, Metabolic Syndrome, Andropause, AIDS, Chronic Renal Failure, and Cirrhosis were excluded. Mean +/- SD were computed for quantitative variables. Frequency and percentages were computed for qualitative variables. Aetiology of male hypogonadism was categorised as primary and secondary hypogonadism. Results: A total of 85 patients with male hypogonadism attended the endocrine clinic. Mean age of patients was 25 +/- 10 years. Clinical presentations were small genitalia (65%), absent secondary sexual characteristics (53%), not attained puberty (47%), infertility (53%), erectile dysfunction (41%) and loss of libido (29%). Seventy-three (86%) patients had hypogonadotrophic hypogonadism (secondary hypogonadism) and 12 (14%) patients had hypergonadotrophic hypogonadism (primary hypogonadism). Among the patients with hypogonadotrophic hypogonadism 38 had idiopathic hypogonadotrophic hypogonadsim, 7 had pituitary adenoma, 6 had empty sella syndrome, 3 had Kallman\u27s syndrome, and 1 patient had haemosiderosis due to thalassaemia major; 18 patients did not undergo brain imaging. Conclusion: Small genitalia, absent secondary sexual characteristics and infertility were the main presenting features of hypogonad men. Majority of patients had hypogonadotrophic hypogonadsim

Entity Resolution using Convolutional Neural Network

Entity resolution is an important application in field of data cleaning. Standard approaches like deterministic methods and probabilistic methods are generally used for this purpose. Many new approaches using single layer perceptron, crowdsourcing etc. are developed to improve the efficiency and also to reduce the time of entity resolution. The approaches used for this purpose also depend on the type of dataset, labeled or unlabeled. This paper presents a new method for labeled data which uses single layered convolutional neural network to perform entity resolution. It also describes how crowdsourcing can be used with the output of the convolutional neural network to further improve the accuracy of the approach while minimizing the cost of crowdsourcing. The paper also discusses the data pre-processing steps used for training the convolutional neural network. Finally it describes the airplane sensor dataset which is used for demonstration of this approach and then shows the experimental results achieved using convolutional neural network

Pituitary stalk interruption syndrome presenting as short stature: a case report

Introduction: Pituitary stalk interruption syndrome is a rare congenital abnormality of the pituitary that is responsible for anterior pituitary deficiency. It is characterized by a classic triad of interrupted pituitary stalk, absent or ectopic posterior pituitary, and anterior pituitary hypoplasia or aplasia. Clinical presentation varies according to age. In adults it presents as short stature and anterior pituitary deficiency. Without early diagnosis and treatment, mortality and morbidity in these patients is high. Early diagnosis and treatment of this rare disease can prevent permanent short statue of the patient. We report the first case of pituitary stalk interruption syndrome from Pakistan. CASE PRESENTATION: A 17-year-old Pakistani young man presented with short stature and underdeveloped secondary sexual characters. His siblings and parents were healthy, with normal height. An examination showed his blood pressure was 90/60 mmHg, and his height, weight, and body mass index were 142 cm, 34.5 kg, and 17.10 kg/m2, respectively. He had no hair growth on his face, axilla, or pubis. His testes were between 1 and 2 mL in size, with a 4 cm-at-stretch micropenis. His lab investigations showed that his thyroid stimulating hormone (TSH) was 8.58 uIU/mL (0.4 to 4.2), his free thyroid hormone level FT4 was 0.46 ng/dL (0.89 to 1.76), his prolactin was 21.1 ng/mL (3.0 to 14.7), and his baseline cortisol was 0.30 ug/dL (4.3 to 22.4). His cortisol level after 60 minutes of cosyntropin injection was 3.5 ug/dL (4.3 to 22.4), his insulin like growth factor IGF-1 was 31.56 ng/mL (247.3 to 481.7), his testosterone level was under 2.5 ng/dL (2 to 800), his follicle stimulating hormone FSH was 0.41 uIU/mL (0.0 to 10.0), and his leutinizing hormone LH was under 0.1 uIU/mL (1.2 to 7.8). His bone age was 10 years according to the Greulich and Pyle method, as shown by X-rays. The results from his pituitary magnetic resonance imaging scan were consistent with pituitary stalk interruption syndrome. CONCLUSIONS: We describe a young man who presented with short stature and was found to have pituitary stalk interruption syndrome. Despite the fact that this is a rare disorder, it should always be kept in the differential diagnosis of a patient presenting with short stature. Patients with this disease have an excellent opportunity to reach normal height if they present before the joining of epiphyses

The Role of Private Label Brands in Enhancing Service Satisfaction in the Hotel Industry: Comparing Luxury and Boutique Hotels

This research proposes private label branding as a strategy that can have a positive effect on guest satisfaction and loyalty in hotels that cater to travelers seeking a special hotel experience. We test this using the well-established SERVQUAL model of service quality, based on a sample of guests in two hotels: a five-star luxury hotel (N=225) and a boutique hotel (N=101). The findings show that in the luxury hotel but not the boutique hotel, perception of a private label had a direct impact on loyalty, and an indirect impact on loyalty through its effect on guest satisfaction. The theoretical implications and recommendations for hoteliers based on the findings are discussed

Anomalous spectral evolution with bulk sensitivity in BiPd

We investigate the electronic structure of a noncentrosymmetric superconductor, BiPd using photoemission spectroscopy with multiple photon energies ranging from ultraviolet to hard x-ray. Experimental data exhibit interesting difference in the surface and bulk electronic structures of this system. While the surface Bi core level peaks appear at lower binding energies, the surface valence band features are found at the higher binding energy side of the bulk valence band; valence band is primarily constituted by the Pd 4d states. These changes in the electronic structure cannot be explained by the change in ionicity of the constituent elements via charge transfer. Analysis of the experimental data indicates that the Bi-Pd hybridization physics plays the key role in deriving the anomalous spectral evolution and the electronic properties of this system.Comment: Proceedings of DAE SSPS 201

Forensic Evaluation of the patterns of fatal injuries among pedestrians in road traffic accidents in Hyderabad, Pakistan.

Introduction: Road traffic accidents (RTAs) preventable yet responsible for a large number fatalities among children and adults. It is estimated that RTA causes approximately 1.35 million deaths (27.5 deaths per 100,000 population) through out the world.Objective: To evaluate the patterns of fatal injuries among pedestrians due to road traffic accidents brought in emergency unit of Liaquat University, hospital, Hyderabad.Methodology: This cross-sectional study conducted between Jan 2019 to Dec 2019 and all victims of road traffic accidents brought in the emergency due to fatal injuries belongs to either sex or age-group were included. Data collected includes demographic details; type, site, impact, intensity of inju-ries and different types of vehicles involved in the accidents were recorded from history/ autopsy findings of medico-legal officer. Collected data was entered and analyzed in SPSS ver. 23.Results: Motorcars were more involved (31.0%) in road traffic accidents followed by trucks/loaders (29.0%). Males (87.5%) outnumber female. Head and neck region (26.0%) affected most, while abdomen/pelvis were the least(16.0%) affected. Contusions occurred in predominantly (51.0%) while most (60.5%) pedestrians were found dead when received in the emergen-cy unit. Most common cause of death was head injury induced complica-tions.Conclusion: Injuries to head, neck and facial regions are more common while contusions are the predominant type of injuries occur in the pedestri-ans. Skull bone is most commonly fractured bone among the victimsKeywords: Pattern of injuries, Pedestrians, Road traffic accidents

Bicornuate unicollis uterine Müllerian anomaly with unilateral haematometra in a ten year old: A case report

Acute abdominal pain is a common complaint in the paediatric age group. Gastroenteritis and appendicitis are the common non-surgical and surgical conditions respectively. Haematometra due to a non-communicating horn of a bicornuate uterus is a rare presentation for acute abdomen in this age group. This is a case of a ten year nine month old female who presented with an acute abdomen for a period of two days. Physical examination revealed right iliac fossa tenderness. Pelvic ultrasound showed a bicornuate unicollis uterine Müllerian anomaly with unilateral haematometra. Magnetic Resonance Imaging (MRI) confirmed the ultrasonographic findings. A successful hemi-hysterectomy was done with resolution of symptoms