Rare bleeding disorders: spectrum of disease and clinical manifestations in the Pakistani population

Background@#Rare inherited coagulation factor deficiencies constitute an important group of bleeding disorders. A higher frequency of these disorders is seen in areas of high consanguinity.Our aim was to study the prevalence and spectrum of rare inherited bleeding disorders, characterize the severity of the deficiencies, identify different clinical manifestations, and evaluate different treatments provided. @*Methods@#This cross-sectional study was conducted in the Department of Haematology, Armed Forces Institute of Pathology Rawalpindi, between January 2014 and December 2018.A detailed history was taken, and an examination was performed. The signs and symptoms were noted, and the patients were diagnosed on the basis of a coagulation profile. The disease severity was assessed using factor assays. @*Results@#Among 2,516 patients with suspected coagulation disorders, 774 (30.8%) had an inherited bleeding disorder. Of the 774 patients, 165 (21.3%) had a rare bleeding disorder;91 (55.2%) of them were males, and 74 (44.9%) were females, with a male-to-female ratio of 1.2:1. The median patient age was 9 years 3 months. The most common disorder was factor VII deficiency (46 patients, 27.9%). The most common clinical presentation was bruising in 102 (61.8%) and gum bleeding in 91 (55.2%) patients. @*Conclusion@#The most common rare bleeding disorder in our population is factor VII deficiency. The prevalence of these bleeding disorders is high in our population due to a high number of consanguineous marriages.

New Oral Anticoagulants versus Warfarin for Cerebral Venous Thrombosis: A Multi-Center, Observational Study / 대한뇌졸중학회지

No abstract available.

Scientific findings of pakistan origin halophyte launeae nudicaulis [L.] hook.

In arid and semi-arid regions of the world salinity is the one of the major problem that causes tremendous loss in the crop yield. It is required to bring these areas under utilization for maximizing the crop production either by removing salinity or by using salt tolerant crops. These plants are growing on the soil as creepers and herbs such as Citrullus colocynthis, Cres erotica, Launeae nudicaulis [L.] Hook.f. etc. One of such kind of plants, L. nudicaulis [L.] Hook.f. has been selected for the current research study. L. nudicaulis [L.] Hook.f. is abundantly found in Pakistan, especially in Karachi, parts of Sindh province and Cholistan desert. The L. nudicaulis [L.] Hook.f. Compositae [Asteraceae] is considered as facultive type of Halophyte which can grow on saline as well as non saline soil. According to the literature survey, this creeper facultive halophyte has great medicinal significance. It is recommended in Folk medicine as bitter tonic, stomachic, wound healing accelerator etc. Furthermore, it is reported to possess antioxidant, antimicrobial, insecticidal and cytotoxic properties. Present study is based on standardization, phytochemical, urease inhibition and heavy metal analysis of three morphological parts of L nudicaulis [L.] Hook.f. i.e. [A]root, [B] stems, and [C] leaves. Due to significant medicinal properties, agronomy and easy accessibility of this ignored halophyte, preliminary phytochemical, physico-chemical, moisture content, total ash content and florescent analysis of all parts of plant were separately performed to establish the standard protocol for its identification that is crucial for medical use and detenuination of existing chemical components either primary or secondary metabolites in each part and their behaviors. Elemental analysis and urease inhibition activity were carried out to prove this plant as both nutritional and agronomic potential along with their toxic components present in each part of L. nudicaulis [L.] Hook.f?

Clinico-haematological features of paroxysmal nocturnal haemoglobinuria

The aim of this study was to determine the frequency of various clinico-haematological features in patients suffering from paroxysmal nocturnal haemoglobinuria [PNH]. It was an observational study carried out from October 2008 - January 2016. All the patients of PNH, diagnosed on the basis of clinical and laboratory findings and confirmed by CD55 and CD59 deficiency on red cells by means of flow cytometry, were included in the study. A total of 22 patients were diagnosed which included 18 [81.8%] males and 4 [18.1%] females. Median age was 27 years. Pallor, fever, fatigability and haemoglobinuria were the most common clinical features. Pancytopenia was seen in 13 [59.09%] and hypocellular marrow was found in 14 [63.6%] patients. One patient presented with Budd Chiari syndrome

Dual infection by burkholderia cepacia and pseudomonas putida in an infective endocarditis case

Infective endocarditis is rarely caused by Burkholderia cepacia

Pseudomonas putida has not been reported to cause infective endocarditis so far. This is the first case of infective endocarditis being reported, that is caused by Pseudomonas putida and Burkholderia cepacia in an immunocompetent host with no predisposing factors. Aortic valve replacement surgery was carried out and antibiotics were given, to which the patient responded well and recovered

Efficacy of platelet rich plasma application in comparison to conventional dressing therapy in partial thickness burn wound

To determine the efficacy of Platelet Rich Plasma application in comparison to conventional dressing therapy in partial thickness burn wound . Comparative analytical study. This study was carried out at Department of Burns, Civil Hospital Karachi from March 2011 to January 2013. A comparative analytical study was conducted to determine the efficacy of Platelet Rich Plasma application in comparison to conventional dressing therapy in partial thickness burn wound at Department of Burns, Civil Hospital Karachi. All the admitted patients of either sex having age between 20-40 years, victims of fire and scald burn, having partial thickness burn with 10-30% of TBSA involved. Patients were divided into two groups on random basis. In group [A], Platelet Rich Plasma [PRP] application was given with three day gap between two applications until full recovery of the wound. While in group [B] conventional dressing therapy was adopted till full recovery of wound. All 30 patients of group A, selected for PRP application, were recovered maximally within 18 days [6 therapies with a gap of 3 days]. Whereas in other 30 cases, of group B selected for conventional dressing therapy, it took minimum 21 days or more for complete recovery. Hence recovery was found slow in conventional dressing therapy as compared to PRP and it is statistically significant at P,0.05. Platelet-rich plasma application in non-healing deep partial and full thickness burn wound accelerate the wound healing as compared to conventional dressing therapy and is very effective in preparing healthy beds for grafting and provides 100% graft take.Now it is up to the Burns surgeon to select it for rapid results to save time and cost with availability of more beds in Burn Centre

To evaluate the presence and spread of environmental pathogens and their sensitability. an epidemiological survey of the microbiological monitoring at burns ward civil hospital Karachi

To evaluate the presence and spread of environmental pathogens and their sensitability at burns ward civil hospital Karachi. Descriptive observational study. This study was conducted at Burns Center, Civil Hospital, Karachi during 2012. Samples were collected of air and examined through air microbe index. Samples of water were collected from different sources. Similarly samples were also collected from clothes, nails, hairs and hands of all the Health care personnel working in the burns ward. These samples were sent for culturing to conclude for presence of bacteria. Staphylococcus Aureus was the major organism obtained from the samples in the burns ward. The organism was also detected from the nasal swabs taken from some staff members. Being the most common cause of nosocomial infection it is necessary to take appropriate steps to cut the probable route of transmission. E.coli and Pseudomonas strains were also detected from the environment of the burns ward. E.coli was also detected in the food trays of the patient a probable route of transmission. Many of these strains were resistant to antibiotics which made it further difficult to treat them. Strains of Enterobacteriaceae, Proteus Mirabilis, Klebsiella and Citrobacter were collected from our personnel and were responsible for some hospital acquired infections. Staphylococcus Aureus was the major organism found. The major routes of transmission of opportunistic organisms were tap water and our staff members. Proper hygiene measures and awareness programs for the staff members should be carried out to aware them of the importance of hygiene

Frequency of chemical burn and mortality rate in admitted patients at a tertiary care hospital of Karachi

Developing countries have a high incidence of burn injuries creating a fear or respect public health problem. Acid assault is one of the most savagely violent of crime which aims to punish the victim or to destroy the victim's social life. This violent usually occurs in third world countries like Pakistan, Bangladesh, and Iran etc. In Pakistan 7 to 17 burn admissions annually recorded during the study period. To assess the epidemiological factors in terms of incidence morbidity and mortality and the effect of social and cultural issues associated with the chemical burns. A cross sectional retrospective Place and Duration of Study: This study was conducted in burns ward of Civil Hospital Karachi from May 2004 to Oct2010. All the patients were identified the etiology and the extent of damage that was produced as a result of throwing corrosive chemicals. Overall 127 patients record were included in study. It was more commonly found in younger age group i.e. 21 -30 years. The incidence was much more in males [74.0 %] as well as stratified by age. Majority of the patients [90.6%] were burn with Accidental. Moreover, the patients who had 3RD degree burn [41.9%] that leads to more mortality in our circumstances. Septicemia was the leading cause of death. The burn injury was significantly associated with degree of burn, depth, extent and mode of injury. The most common incidence found was accidental followed by assault. Lack of information about the catastrophic outcome of this action plus the wide spread availability of strong destructive chemicals are the main reasons for the rising incidence of this crime. The majority of deep burns were on homicidal. So measure should be taken to generate awareness for protective measures, immediate first aid, proper professional treatment, restriction in availability of acid, implementation in the existing law regarding the bail of accused etc to prevent these accidents and ensure safety

Current Bacteriological profile in Fire-burn victims and their associated mortality at the Burns Centre, Karachi Pakistan

This study attempts to document the current bacteriological profile from pertinent specimens of fire burn victims in our setting with respect to the time related variations in the isolation of microbial species, extent of their burn injury and their effect on mortality rate. During a three year period [Jan 2008 to Dec 2010], a prospective study was conducted on 477 fire burn victims at the Burns Centre of Civil Hospital, Karachi, with the exclusions of electrical, chemical and other forms of burn injury. Blood, pus, urine and wound biopsy samples were collected and cultured according to standard laboratory protocols. The data was analyzed according to the age, gender, time related changes, percentage of burn surface and the mortality rate. Total 477 patients were included in our study. Multiple specimens were collected [n=732] of which 649 [88.7%] cultures were positive for different organisms. Staphylococcus aureus was the commonest organism isolated in fire burn patients. During 1[st] week S. aureus was predominant [31.6%] followed by Pseudomonas. After 1[st] week S. aureus [24.6%] and Pseudomonas [25.5%] isolation rate became similar. Mortality rate due to infections was 18.9% in the subjects. S.aureus was the commonest cause of infection in fire burn patients in our setting followed by Pseudomonas. These suggests that hygiene should strictly be maintained around burn patients to avoid opportunistic infections

Assessment of four mortality prediction models in intensive care unit patients with sepsis

Early recognition of sepsis and the rapid institution of therapy are absolutely essential for appropriate management of patients admitted to the hospital. Both score-generating clinical tools and clinical acumen are important for identifying the sick, while early intervention in acute deterioration is beneficial, before and after ICU admission. Several scoring systems have been devised which attempt to identify risk factors and predict outcome of different patient groups. The purpose of this pilot study was to assess the value of four mortality risk scoring systems, i.e. Mortality Probability Models [MPM] - admission, MPM -24 hours, Simplified Acute Physiology Score [SAPS] II and Acute Physiology and Chronic Health Evaluation [APACHE] II, in predicting outcome in patients admitted to the intensive care unit with sepsis. The expected outcome was calculated according to the specifications of each system and was compared to the actual outcome