Reporting Practices of Serum Protein Electrophoresis in Pakistan - a Multicenter Survey.

BACKGROUND: Serum Protein Electrophoresis (SPE) is crucial for the diagnosis and follow-up of monoclonal gammopathy (MG), as it helps to separate and identify these paraproteins. Currently, Pakistan lacks standardized guidelines for SPE reporting and analytical performance. This survey aims to analyze reporting variations from Consultant Chemical Pathologists in Pakistani laboratories. METHODS: This cross-sectional survey was conducted by the section of Chemical Pathology, Department of Pathology and Laboratory Medicine, at Aga Khan University Hospital, Karachi. A previously validated and published tool was used with some modifications to assess analytical techniques, reporting patterns, and interpretations provided with SPE by different laboratories. Frequency and percentages were calculated for each response and descriptive results were also evaluated. Differences between laboratories were also assessed qualitatively. RESULTS: Out of the eight laboratories contacted, seven participated in the survey, yielding a response rate of 87.5%. Immunofixation Electrophoresis (IFE) was used by all labs for serum immunotyping. All labs reported a new small abnormal band in patients with no known monoclonal gammopathy or with a known M-protein. Variations were found in terminologies used to label paraprotein, terminologies used to report normal and pathological SPE patterns, electrophoretic technique, methods for quantifying paraprotein in the gamma region on SPE and for albumin quantification. Similarly, the number of decimal places reported, reporting of multiple monoclonal proteins and small paraprotein in the beta region or monoclonal proteins less than 1 g/L, approach for screening, number of fractions reported in gamma region and reporting of interferences were also not standardized and var-iations were noticed. CONCLUSIONS: Our survey highlighted variations in practices of SPE reporting. These differences in laboratory practices could result in inconsistent test results, which could adversely affect patient care.

The Frequency of Immunofluorescence Antinuclear Antibody Patterns and Extractable Nuclear Antigen: Experience From a Large Laboratory in Pakistan.

Background Autoimmune disorders have shown an increasing incidence in the last few years. The systemic response to the disorder is characterized by the expression of antinuclear antibody (ANA), which serves as the serological hallmark of autoimmunity. Its presence may indicate either a systemic autoimmune disease such as systemic lupus erythematosus (SLE), scleroderma, and polymyositis/dermatomyositis or an organ-specific condition such as autoimmune thyroiditis and hepatitis. The systemic response may vary from one individual to another in each population. Several specific autoantibodies are also found to be associated with specific rheumatic diseases. Aim We aim to report the frequency of ANA positivity, ANA immunofluorescence patterns, and the presence of extractable nuclear antigen (ENA) among the general Pakistani population from one of the largest laboratories in Pakistan. Material and methods A total of 1,966 blood samples from a random Pakistani population were included, who were referred by their physicians with suspicion of autoimmune disease. These blood samples were subjected to ANA testing by indirect immunofluorescence method, and subsequently, positive samples were further analyzed for ENA detection in the Section of Chemical Pathology, Department of Pathology at Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan. An ANA titer of ≥1:80 was taken as positive. ANA was divided into subgroups based on titer: negative, weakly positive (titer of 1:80 or 1:160), moderately positive (titer of 1:320 or 1:640), and strongly positive (titer of ≥1:1280). Further, the frequency of ANA in male and female participants was studied in different age groups (2 to <10, 10 to <20, 20 to <30, 30 to <40, 40 to <50, 50 to <60, 60 to <70, 70 to <80, and 80+ years). Results This study included 1,966 participants, out of which 1,100 (55%) were ANA-positive at a titer of ≥1:80. Out of these ANA positives, the proportion of weakly positive (titer of 1:80 or 1:160), moderately positive (titer of 1:320 or 1:640), and strongly positive (titer of ≥1:1280) were 48.7%, 2.6%, and 4.2%, respectively. The ages ranged from two to 91 years, with a mean age of 43.64 ± 17.4 years. Females (75.5%) showed predominance over males (24.5%) in all age groups, with a ratio of 3:1. The age group in which most ANA positivity was found was 30 to <40 years. Among 1,100 ANA-positive sera, 383 (34.8%) participants tested positive for at least one out of 15 ENA. The most frequent autoantibodies noticed were anti-recombinant Ro52 (Ro52) (19.8%), anti-Sjogren's syndrome type A (SSA) (17.2%), and anti-ribonucleoprotein (RNP) (13.3%). The most prevalent ANA patterns were nuclear homogeneous (27.7%), followed by nuclear speckled (26.5%). Conclusion The frequency of ANA positivity is high in the Pakistani population and differs in different sex and age groups.

Metabolic risk factors in pediatric stone formers: a report from an emerging economy.

The goal of this study was to investigate metabolic risk factors in pediatric stone formers in an emerging economy. A prospective, data collection enrolled 250 children age <1-15 years at our center. Risk factors were evaluated by gender and in age groups <1-5, 6-10 and 11-15 years. Patients were evaluated for demographics, blood and 24 h urine for calcium, magnesium, phosphate, uric acid, electrolytes and additional protein, citrate, ammonia and oxalate in urine. All reported values were two sided and statistical significance was considered at p value ≤0.05. The mean age at diagnosis was 7.50 ± 3.56 years with a male to female ratio of 1.84:1. A family history of urolithiasis was found in 41 (16.4 %), urinary tract infection in 18 (7 %) and chronic diarrhea in 75 (30 %). Hypercalcemia was seen in 37 (14.8 %), hyperuricemia in 23 (9.2 %) and hyperphosphatemia in 6 (2.4 %). Urinary metabolic abnormalities were identified in 248 (98 %) of the cases. Hypocitraturia was found in 207 (82.8 %), hyperoxaluria in 62 (26.4 %), hyperuricosuria in 82 (32.8 %), hypercalciuria in 51 (20.4 %), hyperphosphaturia in 46 (18.4 %), hyperammonuria in 10 (4 %), hypocalciuria in 82 (32.8 %), and hypovolemia in 73 (29.2 %). Risk factors were similar between genders except higher rates of hyponatriuria, hypophosphaturia, and hypocalciuria in females. Hyperuricosuria, hyponatriuria, and hypovolemia were highest in 1-5 years (52, 49, 49 %) as compared to (18, 21, 12 %) those in 11-15 years (p < 0.001), respectively. This study shows that careful metabolic analysis can identify risk factors in 98 % of the children where appropriate metaphylaxis can be undertaken both for treatment and prevention of recurrence.

Chemical Composition Of Stones In Paediatric Urolithiasis.

BACKGROUND: Chemical composition of stones is one of the important diagnostic criteria for aetiology of stone formation and treatment to prevent recurrence. This paper reports composition of stones in children at a tertiary hospital by Fourier Transformation Infrared Spectroscopy (FTIR). METHODS: Between January-June 2015, 412 urinary stones from children were analysed by FTIR. Chi-square tests were used for the comparison of categorical measurements between groups. All reported values were 2-sided and statistical significance was considered at p-value ≤0.05. RESULTS: Of the 412 stones, 263 (63.8%) were renal, 101(24.5%) bladder and 48 (11.7%) ureteric. The mean age of children was 7.15±4.13 years with a M:F ratio 2.4:1. Of the 412 stones, 144(34.9%) were pure stones composed of one compound and 268(65.1%) were mixtures. Frequency of compound in stones was Ammonium Acid Urate (AAU) (65%), Calcium Oxalate (CaOx) (76.9%), Uric Acid (5%), Calcium Phosphate Apatite (7%), Whitlockite (8.4%), Struvite (4%), Cystine (0.72%) and Xanthine (2.11%). Frequency of compounds analysed in three ages groups 0-5, 6-10 and 11-15 years showed high frequency of AAU (73%) in 0-5 years as compared to (60%) in 11-15 years (p<0.018). CaOx (90%) in 11-15 as compared to (62.5%) in 0-5 years (p<0.001). Bladder stones were more prevalent in children 0-5 years (32%) vs 19% in 11-15 years (p<0.004) while renal were 75% in 11-15 years and 54% in 0-5 years (p<0.04). CONCLUSIONS: AAU stones known to be associated with malnutrition and chronic diarrhoea are highly prevalent in paediatric stones formers in our population in the kidney, bladder and ureter.