Quebec platelet disorder

A case of Quebec platelet disorder is hereby reported. A 33 years old woman presented with history of epistaxis and gum bleeding since childhood and menorrhagia and bleeding per vaginum after puberty, also had history of excessive blood loss after birth of child. Her coagulation profile was normal but platelet function testing by platelet aggregation assay showed abnormal aggregation of platelet with epinephrine. This type of response is seen in Quebec platelet disorder which is a rare autosomal dominant disorder of platelet function characterized by increased bleeding after any injury or trauma

Growth of Manufacturing Employment in Pakistan: A Comparative Analysis of Punjab and Sindh (Preliminary Results)

Provincial economic disparities in Pakistan are often discussed in political circles. Recently, these disparities have also caught the attention of economic planners. I However, very few professional studies have analysed the issue due to paucity of inter-provincial data. Moreover, the existing studies base their analysis on cross-sectional data obtained from various sources and cannot be used to analyse disparities in economic growth and its sources.2 The purpose of present paper is to provide an initial contribution to the analysis of provincial economic changes in Pakistan. Employment growths in the manufacturing sectors of Punjab and Sindh are analysed for the period 1980-87. Regional differences in employment growth are usually attributed to differences in industrial structure and also to differential impacts of regional-specific influences (infra-structure, policies, political situation, etc.) that determine competitiveness of a region. The present study analyses these two components of growth for Sindh and Punjab in order to explain differential growth in these provinces. Such an analysis is useful for provincial planning as it may help plan for a balanced growth

Acetaminophen induced Steven Johnson syndrome-toxic epidermal necrolysis overlap

Steven Johnson Syndrome and Toxic Epidermal Necrolysis are rare but severe form of hypersensitivity inflammatory reactions to multiple offending agents including drugs. Acetaminophen is extensively used due to its analgesic and anti-pyretic properties. It is rendered to be relatively safe, with hepatotoxicity considered to be the major adverse effect. However, very few cases of Steven Johnson Syndrome and Toxic Epidermal Necrolysis have been reported with acetaminophen usage in the past. We present the case of a 40 years old lady who developed an overlap of the two condition after taking several doses of acetaminophen for fever. She presented with widespread maculopapular rash, stinging in the eyes, oral mucosal ulcerations and high grade fever. She was successfully treated with corticosteroid therapy along with the supportive treatment. This case addresses the fact, that severe hypersensitivity reactions can occur with acetaminophen which can be potentially life threatening

Non-diabetic renal disease in patients with type-2 diabetes mellitus

Diabetic nephropathy (DN) is the leading cause of end-stage renal disease in diabetics worldwide, yet most patients with type-2 diabetes mellitus are not formally evaluated with a renal biopsy. The diagnosis is almost always based on clinical grounds. A wide spectrum of non-diabetic renal disease (NDRD) is reported to occur in patients with type-2 diabetes. It has been estimated that up to one-third of all diabetic patients who present with proteinuria are suffering from NDRD. The aim of this analysis was to evaluate the prevalence and etiology of NDRD in patients with type-2 diabetes. We retrospectively reviewed the medical records of patients with type-2 diabetes who underwent kidney biopsy on clinical suspicion of NDRD (absence of diabetic retinopathy and/or neuropathy; short duration of diabetes, i.e. less than five years) from January 2003 through December 2007 at the Aga Khan University Hospital, Karachi. Based on the biopsy findings, patients were grouped as Group-I, isolated NDRD; Group-II, NDRD with underlying DN; and Group-III, isolated DN. Of 68 patients studied, 75% were males and the mean age was 56 years. The mean duration of diabetes was nine years. Group-I included 34 patients (52%), Group-II included 11 patients (17%) and Group-III included 23 patients (31%). Among the Group-I patients, the mean age was 56 years (41-77 years). The most common NDRDs were acute interstitial nephritis (32%), diffuse proliferative glomerulonephritis (17%); membranous nephropathy (12%) and crescentic glomerulonephritis (12%). Among Group-II, the mean age was 60 years (46-71 years), and the most common lesion was interstitial nephritis superimposed on underlying DN (63% cases). Among Group-III, the mean age was 53 years (42- 80 years). The mean proteinuria was 5, 6.3 and 7.3 g/24 h of urine collection in Groups I, II and III, respectively (P = NS). The mean duration of diabetes was 7.3, 11.7 and 10.7 years in Groups I, II and III, respectively. The duration of diabetes was significantly less in Group-I compared with Group-II and Group-III (P = 0.04). Our study suggests that the prevalence of NDRD (either isolated or superimposed on underlying DN) is high in appropriate clinical settings. Performing renal biopsy in diabetics with no extrarenal end organ damage other than nephropathy helps to diagnose and treat NDRD. This is the first report from Pakistan documenting the prevalence of NDRD in patients with type-2 diabetes

Clinically significant contrast induced acute kidney injury after non-emergent cardiac catheterization--risk factors and impact on length of hospital stay.

Objective: To evaluate the frequency and risk factors associated with clinically significant contrast-induced nephropathy(CIN) in patients undergoing non-emergent coronary angiography. Study Design: Descriptive study. Place and Duration of Study: The Aga Khan University Hospital, Karachi, from January 2005 to December 2007. Methodology: Case records of patients who underwent coronary angiography with a serum creatinine of ≥ 1.5 mg/dl atthe time of procedure were evaluated. Clinically significant contrast induced nephropathy (CSCIN) was defined as either doubling of serum creatinine from baseline value within a week following the procedure or need for emergency hemodialysis after the procedure.Results: One hundred and sixteen patients met the inclusion criteria. Mean age was 64.0 ± 11.5 years, 72% were males.Overall prevalence of CIN was 17% (rise of serum creatinine by ≥ 0.5 mg/dl) while that of clinically significant CIN (CSCIN) was 9.5% (11 patients). Patients with CSCIN had significantly lower left ventricular ejection fraction (p = 0.03, OR: 0.24;95% CI = 0.06 – 0.91) and higher prevalence of cerebrovascular disease (p \u3c 0.001, OR: 14.66; 95% CI = 3.30 – 65.08).Mean baseline serum creatinine was significantly higher, 3.0 ± 1.5 vs. 2.0 ± 1.1 mg/dl (p = 0.03, OR: 1.47; 95% CI =1.03 – 2.11) whereas mean GFR estimated by Cockcroft-Gault formula was significantly lower at 25 ± 7.4 vs. 41.0 ± 14.6ml/minute (p = 0.001, OR = 0.89, 95% CI = 0.84 – 0.95) at the time of procedure in patients with CSCIN. Mean length ofhospital stay was significantly higher in this group compared to those without CIN, 9.0 ± 5.1 vs. 3.0 ± 3.2 days (p = 0.001,OR = 1.31, 95% CI = 1.12 – 1.54). Multivariate analysis revealed low GFR (p = 0.001, OR = 0.88; 95% CI = 0.82 – 0.95)and low ejection fraction (p = 0.03, OR = 0.20; 95% CI = 0.04 – 0.91) to be independent factors associated withCSCIN. No significant differences were noted between the two groups in patients with hypertension, diabetes andheart failure. Conclusion: CSCIN is a significant concern in high risk groups despite prophylaxis. Patients with lower EF, cerebrovascular disease and low GFR at the time of procedure are more likely to have CIN

Giant cell tumour of the sacrum: Function-preserving surgery with extended curettage and ilio-lumbar fusion

Giant cell tumours of the sacrum pose a unique therapeutic challenge due to the inaccessibility of the tumour, significant intra-operative blood loss from extensive vascularity, high rate of local recurrence with conservative surgery, and loss of neurological function and mechanical instability with en-bloc excision. We present a case where successful outcome was achieved by tailoring treatment in consideration of the above issues. A 28 year old male diagnosed on biopsy to have giant cell tumour of the sacrum presented to us with low-back pain, left-sided S1 radiculopathy, ankle weakness and urinary incontinence. MRI showed a tumour involving the S1 and S2 vertebral segments, breaching the posterior cortex and compressing the neural elements. An angiographic tumour embolization was performed followed by surgery through a posterior approach whereby an extended curettage was done, carefully freeing the sacral nerve roots and abrading the bone using high-speed burr. An ilio-sacro-lumbar fusion was done employing iliolumbar instrumentation and bone grafting. Post-operatively, within a week the patient was ambulated with a lumbar corset. At 9 months follow-up, the patient was completely pain free, had no ankle weakness, and had normal continence. This treatment approach resulted in preservation of neurologic function and maintenance of spinal stability, thus the patient returned to full function