A Prospective, Randomized, Interventional Study of Oral Iron Supplementation Comparing Daily Dose with Alternate Day Regimen Using Hepcidin as a Biomarker in Iron Deficiency Anemia.

AIM: To assess effect of daily vis-a-vis alternate day oral iron therapy in terms of hemoglobin, reticulocyte hemoglobin equivalent (RET-He) and GI side effects using hepcidin as a biomarker. METHODS: A hospital based randomized interventional two-arm analytical study was done among patients of IDA (20 in each group). The study population was divided into two groups by randomisation. Group 1 received oral iron supplements on alternate day and Group 2 received iron supplements daily. Hemoglobin, RET-He, Serum ferritin and Hepcidin level were assessed. RESULTS: On day 2nd, the rise in Hepcidin was not significant from base line in alternate day therapy group but was significantly increased in daily therapy group. On day 3, the rise in hepcidin was significant from base line in both the groups but the mean change in hepcidin was more in daily therapy group. RET-He began increasing on day 2nd in both the groups. In alternate day therapy group, the rise in RET-He was significant from base line from the day 2nd onwards while the rise in RET-He in daily therapy group was not significant even on day 3. In alternate day iron therapy group, the mean increase in hemoglobin on day 21th (1.58 ±0.53 gm/dl) was significantly more than mean increase among daily therapy (0.41 ± 0.25 gm/dl, P <0.05). CONCLUSION: Alternate day single tablet dosing schedule of oral iron therapy (60mg of elemental iron, ferrous sulfate) was more effective and better tolerated (gastrointestinal side effects) compared to daily supplementation in IDA.

Low Dose Prophylaxis vis-a-vis on-Demand Treatment Strategies for Hemophilia: A Cost Effective and Disability Attenuating Approach.

AIM: To assess effect of low dose prophylaxis in hemophilics in terms of bleeding, joint function, QoL and cost-effectiveness. METHODS: Analytic study done during one year among 70 adult hemophilics. In observation period (12 weeks), on-demand factor and during prophylaxis (12 weeks), low dose factor was given (Factor VIII 10 IU/KgBW biweekly for haemophilia A and Factor IX 20 IU/KgBW weekly for haemophilia B). Clinical joint assessment was done by Gilbert score and improvement by WFH definitions. RESULTS: Bleed reduced by 68.99% in moderate hemophilics (40 v/s 129) and 64.86% in severe hemophilics (26 v/s74) (p<0.05). During observation in moderate hemophilics, joint, soft tissue and mucosal bleeds occurred in frequency of 120, 1 and 8. This was reduced to 39 joint bleeds, 1 soft tissue bleed and no mucosal bleed during prophylaxis. In severe hemophilics, 70 joint, 2 soft tissue bleeds and 2 mucosal bleeds occurred during observation which reduced to 26 joint bleeds without soft tissue/mucosal bleed in prophylaxis. Bleeding episodes decreased by 65.79% in joints, 66.67% in soft tissues, 100% mucosal bleeds. After prophylaxis one joints (0.61 %) showed good improvement in joint function, thirty (18.18 %) joints showed moderate improvement and ninety two joints (55.76 %) showed mild improvement in joint function. Hospitalization reduced by 60.34% (163 v/s 411) and absenteeism by 53.73% (279 v/s 603). Factors consumption reduced by 12.33 % during prophylaxis period. CONCLUSION: The low dose prophylaxis strategy significantly decreased the subsequent episodes of total bleeds including joint bleeds and improved the joint function as well as quality of life.

Hemophagocytic Lymphohistiocytosis in a Patient of Scrub Typhus.

Hemophagocytic lymphohistiocytosis (HLH) is a rare but aggressive and potentially fatal condition characterized by excessive immune activation. It can occur as primary/ familial and secondary/sporadic/ acquired form. Infections can play a role as triggers in the secondary form of HLH. A case of Hemophagocytic lymphohistiocytosis (HLH) in a patient of Scrub typhus is being reported here. Such association of scrub typhus and HLH is rare.

Reticulocyte Hemoglobin vis-a-vis Serum Ferritin as a Marker of Bone Marrow Iron Store in Iron Deficiency Anemia.

AIM: To evaluate reticulocyte hemoglobin (RET-He) vis-a-vis serum ferritin as a marker of bone marrow iron store in iron deficiency anemia (IDA). MATERIAL AND METHODS: A hospital based analytic study was conducted among patients (age group 15-65 years) with newly diagnosed and untreated IDA admitted in medicine ward and not suffering from any inflammatory disorders (excluded by C-reactive protein). Patient having other forms of anemia/ hemoglobinopathies/ malignancy, MCV > 80 fL and pregnant female were excluded. All patients were subjected to automated CBC, RET-He, iron studies and iron staining of bone marrow aspirates. RESULTS: Total 142 patients were included. Of these, 42 patients were excluded due to aparticulate bone marrow aspirate. Remaining 102 patients were classified in to Group A (grade 0 and 1-depleted iron stores) and Group B (grade 2 and 3 - functional iron deficiency). There were significant difference in means of RET-He (Group A 17.84 ± 2.39 vs. Group B 25.08 ± 4.42; P< 0.0001) and serum ferritin (Group A 8.68 ± 2.80 vs. Group B 15.61 ± 4.68; P < 0.0001). We observed significant positive correlation of ferritin with RET-He in total patients (r = 0.7860, p 0.0000), Group A (r = 0.7089, p 0.00) and Group B (r = 0.4675, p < 0.05) patients. RET-He was the only significant predictor of bone marrow iron stores (at P < 0.05). On ROC curve analysis, the AUC for RET-He was found to be 0.894 (P value < 0.01) and best cut off value for predicting IDA was 22.4 pg (sensitivity 98.88%, specificity 84.21%). The AUC for serum ferritin was 0.891 (P value < 0.01) and best cut off value for predicting IDA was 11.6 ng/ml (sensitivity 86.75%, specificity 89.47 %). CONCLUSIONS: RET-He correlated significantly with serum ferritin and is also a better predictor of bone marrow iron stores than the latter.

Atrial Septal Aneurysm Presenting as Clubbing without Clinically Apparent Cyanosis.

Atrial septal aneurysm (ASA) is a localised "saccular" deformity which protrudes to the right or the left atrium or on both sides. It is a rare, but well recognised cardiac abnormality. It is usually an incidental finding or may presents as atrial arrhythmias or arterial embolism. Though it is an acyanotic congenital heart disease but it may result in significant right to left shunt and cyanosis. We describe a patient of ASA with atrial septal defect who presented with clubbing and right to left shunt without clinically apparent cyanosis.

Methaemoglobinaemia as a result of nitrite poisoning.

Methaemoglobinaemia due to nitrite poisoning is rare. Awareness of this condition in the cyanosed patient not responding to oxygenation and timely administration of methylene blue may be life saving. We report a case of methaemoglobinaemia as a result of sodium nitrite poisoning.

Anti-nucleosome antibodies in patients with systemic lupus erythematosus: potential utility as a diagnostic tool and disease activity marker and its comparison with anti-dsDNA antibody.

OBJECTIVE: To compare the utility of anti-nucleosome antibodies and anti-dsDNA antibodies in diagnosis of Systemic Lupus Erythematosus (SLE) and as a marker of disease activity. METHODS: This is a hospital based observational study among 40 (37 females and 3 males) selected cases of SLE (> or = 4 ACR criteria) and 80 control. 40 cases of other systemic autoimmune disease (SAD) [e g. 29 cases of Rheumatoid arthritis, 4 cases of Systemic sclerosis/scleroderma, 4 cases of Sjögren syndrome, 3 cases of MCTD and 40 Healthy blood were taken as control. From each patient venous blood samples were collected and submitted for anti-nucleosome and anti-dsDNA antibodies assay by enzyme linked immunosorbent assay (ELISA). RESULTS: Anti-nucleosome antibodies were positive in 19 (47.5%) SLE, 02 (05%) other SAD and none of the healthy persons. Anti dsDNA antibodies were positive in 15 (37.5%) SLE patients, 07 (17.5%) other SAD and 01(2.5%) healthy persons. For diagnosis of SLE, sensitivity of anti-ds DNA and anti-nucleosome antibody was found to be 37.5% and 47.50% respectively. The specificity of anti-nucleosome was 100% and that of anti-dsDNA was 97.50%. So, anti-nucleosome antibody test is more specific and more sensitive for diagnosis of SLE than anti-dsDNA. When SLE cases were compared with SAD, sensitivity of anti-dsDNA and anti-nucleosome antibody, for diagnosis of SLE, found to be 37.50% and 47.50% respectively but the specificity of anti-nucleosome was 95% and that of anti-dsDNA was 82.50%. Both antibodies show positive correlation with SLEDAI score .The correlation coefficient was stronger for anti-dsDNA antibodies (r = +0.550, P = < .001) than anti-nucleosome antibodies (r = +0.332, P = < .05) CONCLUSIONS: Anti-nucleosome antibodies show higher positivity than anti-dsDNA antibodies among SLE than other SAD and healthy population. Anti-nucleosome antibodies are more sensitive and specific for the diagnosis of SLE than anti-dsDNA antibodies. Anti-nucleosome and anti-dsDNA both show positive correlation with SLEDAI. But anti-dsDNA antibodies show stronger correlation with SLEDAI than anti-nucleosome. So, anti-nucleosome antibodies can be used as an additional marker for diagnosis of SLE and SLE disease activity.

Intractable hiccups: How can chest X-ray help?

We describe the case of a young male presenting with hiccups refractory to antacids. Due to high index of suspicion was evaluated with a chest X-ray which revealed pericardial effusion. The effusion was drained, and the hiccups resolved. It is a teaching moment, to consider simple imaging, i.e., chest X-ray in patients with refractory hiccups to rule out more serious underlying causes.

Rheumatological Manifestations in HIV-Positive Patients: A Single-Center Study.

INTRODUCTION: Rheumatological manifestations (RM) are very common in human immunodeficiency virus (HIV)-infected patients. The aim of this study was to determine the clinical spectrum of musculoskeletal involvement and relationship with the Centers for Disease Control and Prevention (CDC) stage and CD4+ cells and other factors. METHODS: A cross-sectional study was conducted involving 75 patients of over 18 years of either sex with confirmed HIV status attending a tertiary care hospital in north India in one calendar year. Baseline demographic details, relevant history including duration of combination antiretroviral therapy (cART), RM, joints involved, CD4 cell count, and biochemical parameters were evaluated. RESULTS: Of 75 patients, 54 were male and 21 were female (mean age 33.15 ± 5.00 years, range 21-48 years). Most common RM was arthralgia (26.67%), followed by myalgia (18.67%), and arthritis (13.33%). Keratoderma blennorrhagicum (1.33%), tendo-achilles tendinitis (2.67%), and plantar fasciitis (2.67%) were other manifestations. Spondyloarthritis (SpA) was seen in 8% patients (undifferentiated SpA 4%, reactive arthritis 2.67%, psoriatic arthritis 1.67%). HIV-associated arthritis was seen in 2.67% while septic arthritis, rheumatoid arthritis, vasculitis, and diffuse infiltrative lymphocytic syndrome were seen in one patient (1.33%) each. The mean duration of disease in patients with RM was significantly less than patients without RM (p < 0.01). The erythrocyte sedimentation rate in patients with RM was significantly higher than in patients without RM (p < 0.05). Mean CD4 + cells were also significantly lower in patients with RM as compared to patients without RM (p < 0.05). Significantly fewer patients on cART had RM in comparison to patients not on cART (p < 0.001). Of 35 patients with RM,  25 were in CDC stage IV. CONCLUSION: RM are common in HIV-infected patients. HIV arthralgia, myalgia, and undifferentiated SpA are the common manifestations. RM were associated with low CD4 counts. Most of the cases with RM were in CDC stage IV.