Clinical Characteristics and Outcome of Budd-Chiari Syndrome at a Tertiary Care Hospital in Pakistan.

OBJECTIVE: To determine the clinical characteristics of Budd-Chiari syndrome (BCS), its causes and outcome at a tertiary care hospital. STUDY DESIGN: An observational study .PLACE AND DURATION OF STUDY: The Aga Khan University Hospital,Karachi, from 2004 to 2014. METHODOLOGY: Aretrospective analysis of data was conducted. Apredesigned questionnaire was filled from medical records of patients with BCS. Clinical features, etiology, management and outcome was noted from 2004 to 2014. Descriptive statistics were determined. RESULTS: Forty-five patients\u27 charts were reviewed; 26 (57.8%) were male patients. The median (IQR) age at diagnosis was 26.0 (20.5 to 34.5) years. Primary BCS was seen in 27 (60.0%) patients. The most frequent clinical features included ascites (82.2%), abdominal pain (55.6%), and hepatomegaly (31.1%). Acombined hepatic vein/inferior vena cava block was found in 25 (55.6%) patients. Out of the 28 tested patients protein C and protein S deficiencies were detected in 22 (78.6%) and 17 (60.7%) patients, respectively. Antithrombin III deficiency was detected in 14 (58.3%) of those tested patients. Anticoagulants were used in 24 (53.3%) patients. TIPS was done in 11 (24.4%) patients. Mortality was 6.7% (n=3). CONCLUSION: Congenital thrombophilia was a major causal factor. Age, clinical features, biochemistry and management are important factors in survival

Interventions to reduce neonatal mortality from neonatal tetanus in low and middle income countries - a systematic review

Background In 1988, WHO estimated around 787,000 newborns deaths due to neonatal tetanus. Despite few success stories majority of the Low and Middle Income Countries (LMICs) are still struggling to reduce neonatal mortality due to neonatal tetanus. We conducted a systematic review to understand the interventions that have had a substantial effect on reducing neonatal mortality rate due to neonatal tetanus in LMICs and come up with feasible recommendations for decreasing neonatal tetanus in the Pakistani setting. Methods We systemically reviewed the published literature (Pubmed and Pubget databases) to identify appropriate interventions for reducing tetanus related neonatal mortality. A total of 26 out of 30 studies were shortlisted for preliminary screening after removing overlapping information. Key words used were “neonatal tetanus, neonatal mortality, tetanus toxoid women”. Of these twenty-six studies, 20 were excluded. The pre-defined exclusion criteria was (i) strategies and interventions to reduce mortality among neonates not described (ii) no abstract/author (4 studies) (iii) not freely accessible online (1 study) (iv) conducted in high income countries (2 studies) and (v) not directly related to neonatal tetanus mortality and tetanus toxoid immunization (5). Finally six studies which met the eligibility criteria were entered in the pre-designed data extraction form and five were selected for commentary as they were directly linked with neonatal tetanus reduction. Results Interventions that were identified to reduce neonatal mortality in LMICs were: a) vaccination of women of child bearing age (married and unmarried both) with tetanus toxoid b) community based interventions i.e. tetanus toxoid immunization for all mothers; clean and skilled care at delivery; newborn resuscitation; exclusive breastfeeding; umbilical cord care and management of infections in newborns c) supplementary immunization (in addition to regular EPI program) d) safer delivery practices. Conclusion The key intervention to reduce neonatal mortality from neonatal tetanus was found to be vaccination of pregnant women with tetanus toxoid. In the resource poor countries like Pakistan, this single intervention coupled with regular effective antenatal checkups, clean delivery practices and compliance with the “high- risk” approach can be effective in reducing neonatal tetanus

R990G Polymorphism of calcium sensing receptor gene Is associated with high Parathyroid Hormone levels in subjects with Vitamin D deficiency: a cross-sectional study

Single nucleotide polymorphisms (SNPs), R990G and A986S of the calcium sensing receptor (CaSR) gene, are shown to influence response of parathyroid hormone (PTH) in subjects with optimal vitamin D levels. This cross-sectional study was conducted in subjects with vitaminDdeficiency (VDD) to observe association’s betweenCaSR polymorphisms, plasma iPTH, and serumcalcium levels. Adult females ( = 140) with known VDD, intact parathyroid hormone (iPTH), and calcium levels were recruited for genotype analysis.The frequencies of the 986 alleles GG, GT, and TT were 68%, 25%, and 7%, respectively, whereas the frequencies of the 990 alleles AA, AG, and GG were 80%, 8.9%, and 11.1%, respectively.Thesubjects with GG genotype of R990G polymorphism had higher iPTH levels (148.65 versus 91.47 and 86.1 pg/mL for GG versus AA, AG, resp., = 0.008) and lower calcium levels (8.4 versus 9.04 and 9.07mg/dL for GG versus AA, AG, resp., = 0.002). No such association of A986S polymorphism with plasma iPTH or serum calcium levels was observed in the present study. Patients with VDD bearing the GG genotype of R990G SNPs are prone to have higher iPTH levels and lower calcium

Role of serum angiotensin converting enzyme in sarcoidosis

This study was conducted to determine the role of Serum Angiotensin Converting Enzyme (SACE) as a marker in the differential diagnosis of pulmonary diseases and prognosis of sarcoidosis, A retrospective analysis of 113 medical records of patients at The Aga Khan University Hospital, with laboratory investigation for SACE was performed. Among 113 patients, 51 cases were found to have sarcoidosis, 44 of them had SACE levels greater than 52 lU/L (mean ACE 104.44). SACE levels were also found elevated in other clinical conditions like tuberculosis (mean 58.64 lUlL), but the enzyme level were less (p0.04) than those found in sarcoidosis (mean (92-97 lUlL). SACE activity was found to be considerably lower in other chronic lung diseases such as, fibrosing alveolitis (mean 43.98 lUlL), interstitial lung disease (mean 42.11 lU/L) and chronic obstructive lung disease (mean 40.85 lUlL). Twenty patients of sarcoidosis, who received steroid tretalment subsequently showed a decline in the SACE levels. SACE is a useful marker in differential diagnosis as 37.2% cases of sarcoidosis compared to only 9.09% of tuberculosis had SACE levels greater than 100 lUlL. In addition, our data also suggest that serum ACE is useful for the diagnosis as well as monitoring prognosis in sarcoidosis (JPMA 48:131,1998)

A Logistics Approach to Improve Medication Reconciliation in the Outpatient Clinic Setting

Medication reconciliation (MR) is the process of determining the most accurate account of medication the patient is taking. Although MR seems simple, research has shown that 25% of medical errors are related to lack of medication reconciliation [1]. In the community clinic setting, the task of medication reconciliation falls on the Medical Assistants (MAs). A preliminary study of MR accuracy in an outpatient clinic found an average of 5.8 discrepancies in the chart medication list [2]. The most notable were incomplete directions, wrong directions, or wrong frequency of dosing. Unoptimized medication use is estimated to result in 275,869 deaths per year in the United States [3]. These are potentially avoidable deaths. A factor contributing to these poor outcomes is the 15-minute office visit and insufficient time to assess how patients are using their medications [4]. Improved MR would help optimization of medication use. Currently, a care team member dedicated to MR and medication management entirely does not exist due to lack of clear fiscal incentive. This study aimed to identify interventions to improve MR without using much more than existing resources. This was done by studying the current processes of MR in ambulatory care clinics, identifying gaps, and proposing interventions for potential improvement. These interventions were subjected to cost vs value analysis to determine which interventions were the most feasible given the 15 minute office visit the healthcare system currently operates in

Fibroblast growth factor 23 (FGF23) levels, phosphate Intake and its association with Indices of renal handling of phosphate in healthy volunteers

FGF23 is a novel phosphaturic hormone; we aimed to assess the FGF23 levels and its association with dietary phosphate intake and indices of renal handling of phosphate in this study. Prospective study was conducted in which dietary phosphate intake was assessed by food frequency questionnaire (FFQ) along with blood and spot urine samples were collected after overnight fast for determining serum phosphate, FGF23, fractional excretion of phosphate (FePO4 ) and tubular maximum for phosphate (TmP/GFR). FGF23 (C-Term) was measured by a sandwich ELISA. The mean dietary phosphate intake of eighty healthy adults (mean age of 29 ± 5 years) was 1220 ± 426 mg; median FGF23 was 49.9 RU/ml (IQR=33, 76) and mean FePO4 was 7 ± 4.7. Subjects were stratified into two groups according to serum phosphate levels. Significant difference was not found in dietary phosphate intake and FGF23 levels in the two groups. However, TmP/GFR and creatinine were significantly different in the two groups. FePO4 was high in both the groups. Overall a rising pattern of FGF23 levels was seen with increasing serum phosphate levels. Significant positive correlation was found between FGF23 and dietary phosphate (r=0.22, p\u3c0.05) and negative correlation was seen between FGF23 and FePO4 (r=-0.260, p\u3c0.05)