[Introduction of hematological and molecular algorithms for diagnosis of factor XIII deficiency in Iran laboratories]

Factor XIII deficiency [FXIIID] is an extremely rare hemorrhagic disorder with an approximately 12 higher incidence in comparison with reported global incidence of the disorder. A standard diagnostic algorithm was proposed by the International Society of Thrombosis and Hemostasis [ISTH] for FXIIID, however, due to the lack of investments all parts of this algorithm cannot be applied in Iran. Thus, this study presented a national algorithm for diagnosis of FXIIID in Iran. For presentation of a national algorithm, all previously published data about Iranian patients with FXIIID as well as practical methods for diagnosis of FXIIID were collected using Science Direct, Google Scholar and PubMed databases. With available facilities, an algorithm with regards to the laboratory assessment, clinical presentations as well as family history can be suitable for an on time and less expensive diagnosis of FXIIID in Iran. Since ISTH diagnostic algorithm is expensive and time consuming, an economical and more suitable national algorithm with regards to available equipment may reduce the rate of misdiagnosis and its life-threatening consequences

[Effect of moderate-carbohydrate and low-calorie diet on metabolic risk factors, liver enzymes and sonographic findings in patients with non-alcoholic fatty liver disease [NAFLD]]

At present, non-alcoholic fatty liver disease [NAFLD] as the hepatic manifestation of the metabolic syndrome is the most common chronic liver disease. Although there is no cure for it yet, it is considered a priority among approaches for lifestyle modifications. This study was aimed to investigate the effect of moderate-carbohydrate, low-calorie diet in patients with NAFLD. A total of 19 overweight or obese subjects with NAFLD were studied for 6 weeks in a randomized controlled clinical trial. A low-calorie moderate-carbohydrate diet [55% energy from carbohydrate] was designed based on dietary habits, age, gender, height and weight of patients. At beginning and end of the study, biochemical parameters including fasting glucose, liver enzymes, insulin, triglycerides, total cholesterol, high-density lipoprotein [HDL-C] and low-density lipoprotein [LDL-C] cholesterol were assessed and height, weight and blood pressure were measured. Data were analyzed by SPSS software. The average age was 38.94 +/- 8.56 years and body mass index [BMI] was 29.44 +/- 2.88 kg/m2. After six weeks of dietary intervention, weight, fasting glucose levels and enzyme aspartate amino transferase [AST] decreased significantly and HDL cholesterol increased. Hepatic ultrasound findings also showed a relative improvement in 6 patients, and one patient who had a complete remission, statistical significance borderline [p=0.055]. The findings of the present study suggest the role of carbohydrate in low-calorie diets, which significantly improved metabolic status of the liver in NAFLD patients, in more than one third of the subjects over a period of 6 weeks

Clinical presentation of typhoid fever

Enteric fever represents a spectrum of acute systemic febrile illness with a myriad of presentations and complications. This study was carried out to evaluate the demographic data such as age, sex, clinical features and outcome of enteric fever patients presenting in District headquarter hospital Muzuffar Garh. Over a period of one year all children presenting with clinical and/or laboratory diagnosis of Typhoid fever, who were admitted to the Social Security Hospital M. Garh, were evaluated. The diagnosis of enteric fever was based on a positive Salmonella typhi or paratyphi blood/stool culture and/or a Widal serodiagnosis > or = 1/160 for O agglutinin, in the presence of evocative symptoms. There were 28 [66.66%] male and 14 [33.33%] female patients, ranging from 1 to 12 years, with a mean age of 6 years. Predominant symptoms were fever, prostration and apathy; anorexia and abdominal pain while the predominant signs were fever, coated tongue, toxic look, hepatosplenomegaly and abdominal tenderness. Hepatomegaly was observed almost as frequently as splenomegaly. Common clinical signs of typhoid fever in adults such as relative bradycardia and rose spots were seldom documented. All patients survived from their severe illness completely. Although there is considerable diversity in the clinical spectrum of typhoid fever, yet a presumptive clinical diagnosis of typhoid fever may be made with a combination of clinical features so that appropriate therapy may be started at the earliest possible time to avoid the increasing drug resistance, morbidity and mortality with typhoid fever

Current awareness of mothers regarding the use of ORS: knowledge, attitude and practice

To study the current situation about the knowledge, attitude and practice of mothers regarding the use of ORS in children with diarrhea. Hospital based descriptive study. Diarrhea section of Pediatric Department, Jinnah Hospital, Lahore. Material and For a period of 2 months [April and May 2003] a single interviewer, according to a pre-designed questionnaire, interviewed mothers of all children aged 1-60 months admitted with diarrhea and signs of some dehydration. Children presenting with severe dehydration, shock, sepsis, or any other primary diagnosis along with accompanying diarrhea like hepatitis, pneumonia, meningitis etc. were excluded. Queries were made regarding mother's education and socioeconomic background, the knowledge of the mother about diarrhea, ORS, source of information regarding ORS use, method of preparation and use of ORS, type and quantity of ORS used. A total of 151 mothers/ caretakers were interviewed. Among the children, 93[61.5%] were males and 58[38.5%] were females, majority [68.9%] is below 12 months of age. Most [41.05%] were from a rural area, and also majority [56.9%] is uneducated. Out of the total, 128 [84.7%] had used ORS, and 23 [15.3%] had never used it. 117 [77.4%] mothers knew that ORS [nimkol] is a rehydration solution. 89 [69.5%] used the commercially available sachet pack, 80 [62.5%] knew the proper method of preparation of ORS sachet pack, while 48 [37.5%] were unable to tell the right way of preparing it. Out of the 23 mothers who had never used ORS, the common reason was lack of knowledge about the use and importance of ORS. 114 [75.8%] mothers came to know about ORS from a health personal, a doctor in majority of cases, but only 28.9% were assessed for the preparation of ORS, and only 45.6% mother were told about the exact quantity of ORS to be used. Conclusions: Majority of children were below I year of age. Majority of mothers, despite being illiterate and having a rural background, knew about ORS use and had used it, but still 47.01% were preparing it wrongly. Although most of them got information from the first medical attendant, but it was rather incomplete, as the quantity of ORS to be used was not told and the mother's knowledge to prepare ORS was not assessed. Sachet pack was the predominant type of ORS used

Neonatal mortality in a district hospital setup

To evaluate the causes and risk factors of neonatal mortality, in a non-tertiary district hospital nursery. Descriptive study. This study was carried out in the Neonatal Unit of Social Security Hospital Muzuffar Garh, Pakistan from 1st May 2004 to 30th April 2005. All newborns admitted in the nursery during study period were counted and the expired ones were evaluated in detail for the cause of death. A total of 410 neonates were admitted during study period, 53.65% were male and 46.35 females. 47.22% were admitted within 1st 24 hours of their life, and 61.66% within 72 hours of birth. 56.66% of the expired neonates were low birth weight. Neonatal sepsis being the commonest cause of death [44%], 35% being of EOS and 65% cases of LOS. 2"d commonest cause of neonatal death was birth asphyxia [22%] and the third commonest cause was prematurity [19%] with its complications. Other causes of neonatal mortality were neonatal jau ndice [2.77%], meconium aspiration syndrome [1.66%], hyaline membrane disease [6.66%], neural tube defects [1.11%] and congenital heart disease [1.66%]. Many babies had more than one of the above said causes. Neonatal sepsis, birth asphyxia and prematurity are the major killers in nurseries