Challenges in diagnosis and management of acquired factor V inhibitors

Inhibitors to factor V is a rare phenomenon with varied clinical presentation ranging from asymptomatic states to life-threatening bleeds. They are known to be associated with exposure to bovine thrombin, drugs, autoimmune diseases and malignancies. Establishing the diagnosis of FV inhibitors is challenging and the presence of lupus-like properties of the inhibitor can further complicate the diagnosis. Here we document an unusual case of an asymptomatic elderly female posted for pacemaker implantation and incidentally, the laboratory workup revealed a disproportionately abnormal coagulation screen. The intricacies in the diagnosis and management are discussed along with a brief review of the literature. An awareness of the diverse manifestations of this underrecognized disorder and difficulties in management is essential for medical practitioners, particularly in patients with idiopathic severe bleeding diathesis.

Magnetic resonance imaging of perianal fistulas in clinically symptomatic patients and the correlation among plain, contrast, and post jelly MRI fistulography

Abstract Objectives To evaluate different types of perianal fistulas and their complications on magnetic resonance imaging (MRI) and to compare plain, contrast, and jelly magnetic resonance fistulography findings. Materials and Methods This prospective study was performed in 30 patients who presented with perianal pus discharge or external fistulous opening. Magnetic resonance imaging of the perianal region before and after giving intravenous contrast and after injecting jelly through a percutaneous opening was performed on a 3T scanner and the results were correlated. Results The mean age of the patients was 40.13±13.88 years (range 19-75 years). The male to female ratio was 14:1. The most common type of fistula was St. James classification type I, which was seen in 13 patients (43%), followed by type IV in 30%, type III in 16%, type II in 6.66%, and type V in 3.33% of the patients. Using agreement analysis, we compared the number of primary and secondary tracts, internal openings, and horseshoe tracts and found a significant agreement between plain and post Jelly MRI fistulography (kappa statistic close to 1).When comparing plain and contrast MRI, there was significant agreement in the primary and secondary tracts, while statistically insignificant results were obtained (p>0.05) for the horseshoe tract and internal openings. Contrast injection was helpful in 7 subjects (23.3%) as peripheral enhancement of abscesses were better delineated. Conclusion Magnetic resonance imaging is the one stop diagnostic modality for perianal fistulas. Acquisition of axial (Ax) T2, axial T2 FS, coronal T2 and coronal T2 FS sequences without administering intravenous contrast or jelly is usually sufficient for the diagnosis of fistulas and their complications.

A case of paraneoplastic hyperleukocytosis closely mimicking chronic neutrophilic leukemia

Leukemoid reaction and myeloproliferative syndrome are close mimickers and frequently pose a diagnostic dilemma, particularly when the leukocyte count is very high. Leukocyte alkaline phosphatase score frequently aids in diagnosis but may or may not be contributory, especially in differentiating chronic neutrophilic leukemia. Herein, we document a case of leukemoid reaction with extensive hyperleukocytosis in a 46-year-old female with poorly differentiated carcinoma. The tumor itself as well as the associated leukocytosis portends a poor prognosis

De novo Philadelphia chromosome positive myelodysplastic syndrome: Report of two cases with brief literature review

Myelodysplastic syndromes (MDSs) are characteristically defined by the presence of specific karyotypic abnormalities, based on which they have been prognosticated. Translocation t(9;22)(q34;q11.2) (Philadelphia positive [Ph +ve]) and corresponding BCR-ABL fusion transcript is the defining parameter of chronic myeloid leukemia. It is also seen in a fair proportion of adult acute lymphoblastic leukemia. Occurrence of a Ph +ve MDS is very uncommon, and that too is seen mostly on progression to higher stage/acute leukemia. Even rarer is the de novo presence of Ph positivity in an MDS. A literature search through PubMed has shown only about forty cases of Ph +ve MDS among which less than half had shown Ph positivity at the time of initial diagnosis. Due to its rarity, this entity has not yet found its space in current WHO 2008 classification and is still under “yet to be validated phase” in current practice of hematological malignancies. The benefit of using a tyrosine kinase inhibitor in such a situation is also debatable. We report here two such cases of de novo Ph +ve MDS, diagnosed in last 1½ year at our institute along with brief literature review

Microfilariae in bone marrow aspirate of a case of myelofibrosis: A cause or coincidence?

Filariasis is among the common parasitic infestations found in India, with Wuchereria bancrofti being the most common causative organism. Presentation ranges from clinically asymptomatic to profound elephantiasis. It is also detected incidentally in diagnostic samples such as body fluids, fine needle aspirates, peripheral blood smears, and other cytological smears. Its detection in bone marrow aspirates with an associated hematolymphoid neoplasm is rare, with only a few case reports. We report one such case of young male who presented with leukocytosis of 253 × 109/L with basophilia and massive splenomegaly. Bone marrow aspirate smears showed the presence of microfilariae along with other features of a myeloproliferative neoplasm (MPN). The present case is probably the first case of finding a microfilaria in a case of MPN

Clinicopathological spectrum of hairy-cell leukemia: A single-center study with brief review of Indian literature

Objective: The presence of specific chemotherapeutic protocols for hairy cell leukemia (HCL) makes it essential to discriminate this entity from other lymphoproliferative disorders. Hence, awareness of the variations in clinical presentations and immunophenotypic aberrancies is requisite to ensure diagnostic accuracy. Materials and Methods: A retrospective study was carried out to analyze the clinical-pathological profile of patients with HCL diagnosed over a period of 81 months (2010–September 2017) in our institute. Flow cytometry was performed in all the patients, and further, BRAFV600E mutation analysis was performed by real-time polymerase chain reaction in a limited number of samples. Result: A total of 353 lymphoproliferative disorders were assessed during the period, of which 16 (4.5%) were diagnosed as HCL, which included 15 cases of classical HCL and single case of HCL-v. Striking male predominance was noted with a median age of 52 (range 22–90 years). 47% patients presented with pancytopenia, while 20% cases had leukocytosis. Three patients presented with bleeding diathesis in the form of melena and purpuric spots. The absence of splenomegaly was observed in 20% patients (4/15) while 2 (13.3%) cases had lymphadenopathy. Hypocellular marrow was observed in 13% cases. Bright expression of CD20/CD22 along with CD25/CD103/CD123/CD11c was noted in all the patients of classical HCL. Aberrant expression of CD23 and CD5 was seen in 33% ( n =5) and 6.7% ( n =1) cases respectively. CD200 was positive in all the 5/15 cases tested. The case of HCL–v presented with very high leukocyte count and exhibited a CD103/CD11c+ and CD123/CD25- profile. BRAFV600E, mutation was present in all the four patients tested who included patients with a hypocellular marrow and absent splenomegaly. Conclusion: HCL has characteristic profiles, yet it may exhibit unusual clinical and immunophenotypic presentations. Perspicacious use of flow cytometry and BRAFV600E mutation analysis will aid in the diagnosis in unprecedented cases

Caesarean section audit in a tertiary hospital of North India using Robson’s classification

Background: In view of upsurging Caesarean section (CS) rate worldwide WHO conducted two multicountry surveys to diagnose the driving determinants. In two WHO surveys increased overall CS rate was observed from 26.4% to 31.2% worldwide except Japan. Both WHO 2014 and FIGO 2016 recommend Robson ten- group classification for monitoring caesarean rate over time because of its clarity, tenacity, resilience and pliability. Our Aim is to classify women delivered in our Hospital as per Robson ten –group classification and access the factor driving caesarean rate in each group.Methods: This is a retrospective study 1671 caesarean section conducted in tertiary hospital over 6 months (July- December) 2018. All the delivering women were classified according to Robson ten-group classification and data was analyzed using Microsoft excel and SPSS 23 software.Results: During the study period there were 5917 deliveries. Of these 1671 deliveries were CS accounting for CS rate of 28.24% . The major contributor to CS rate were women in group 5 followed by primigravida’s in group 1 and 2. Increasing  CS rate was observed in group 1 ,2, 3 and 5. Most common indication for caesarean section was fetal distress , failed induction , previous caesarean , breech and Antepartum hemorrhage.Conclusions: Increasing trend in CS rate is observed in group 1,2 ,3 and 5. In order to reduce CS rate among group 2 better patient selection is required for induction of labour based on Bishop score. In order to reduce CS rate in group 5 promotion of  VBAC deliveries should be encouraged. By classifying women according to Robson group 10 classification  helps in identification of women likely to deliver by caesarean  and to identify effective strategies to optimize the CS rate.

A comparative study of sublingual versus oral misoprostol following oral mifepristone for second trimester termination of pregnancy

Background: The objective of this study is to assess the effectualness and safety of sublingual versus oral misoprostol following oral mifepristone for second trimester termination of pregnancy.Methods: This institution based contingent study was conducted on 220 women requesting for mid-trimester termination of pregnancy between 12-20 weeks with legal indication as per Govt. MTP act. After excluding the women as per exclusion criteria, they were randomly allocated into two groups (Group A Sublingual, Group B Oral), the women received 200 mg oral mifepristone followed by sublingual or oral misoprostol 400µg three hourly for a maximum of 5 doses 48 hours later. The course of misoprostol was reiterated if women failed to abort in 24 hours.Results: The mean induction-abortion interval of Group-A and Group -B was 4.02±1.39 hours and 6.44±1.79 hours respectively. The mean dose of misoprostol in Group–A and Group-B was 680±220.4µg and 1003.6±274.9µg. Hence mean Induction-abortion interval and dose were shortened in  Group-A as compare to Group-B (p <0.05). There was 100% success rate noticed via both routes. Evacuation was done in 4 (3.64%) women in Group-B as compare to only 1 (0.91%) in Group-A . The acceptability was significantly more in Group-B (100%) as compare Group-A (52.73%), probably because of unpleasant taste of sublingual misoprostol. All side effects (Nausea, pain, headache, and diarrhea) were common in both the Groups, only fever was significantly more common in sublingual group as compare to oral group (p<0.05).Conclusions: From present study authors conclude that, sublingual misoprostol when combined with mifepristone is effective for medical abortion in second trimester in terms of effectualness, endurability and success rate than oral route.

​Dental Management Of Children With Special Health Care Needs

The management of children with health care needs creates hesitation and anxiety among health professionals includingdentists. There has been general agreement that disabled population has a higher prevalence of dental caries, poor oralhygiene, and compromised gingival and periodontal health than healthy population. Oral healthcare professionals requirespecialized knowledge acquired through special training and increased awareness. The purpose of this article is to describethe characteristics of some common developmental disabilities and medically compromised states and the challenges of theseissues present to the oral healthcare professionals.

Efficacy of Topical Diluted Autologous Serum in Patients of Dry Eye in Bundelkhand Region

Background: The dry eye is a symptom complex occurring as sequelae to deficiency or abnormalities of the tear filmwhich can lead to the persistent and progressive damage of the ocular surface. Conventional artificial tear therapies are limited in supplying the neurotrophic factors, vitamins, and immunoglobulins necessary for the health of the ocular surface. Serum has been shown to contain essential tears components in comparable concentrations to natural tears.Present study has been done to evaluate the effectiveness of diluted autologous serum eyedrops in relieving patients’ symptoms and improvement in corneal phenotype in patients of dry eye. Methods: The study included 36 eyes of 25 patients with dry eye disorder from Bundelkhand region only, who were prescribed 20% diluted autologous serum eye drops along with artificial tears. Patients were asked to apply this serum 8-10 times per day in affected eye and they were examined on end of 1st, 2nd, 4th, 6th and 8th week to determine the efficacy of the treatment. Evaluation of efficacy of autologous serum treatment for dry eye, was determined by comparing subjective and objective score calculated by a scoring system suggested by “National Eye Institute: Industry workshop on clinical trials”from day 1 to every subsequent visit for follow up. Results: We noticed that after 8 weeks’ treatment of topical diluted autologous serum, 35 cases of dry eyes showed decrease in subjective as well as objective score and only 1 case of eye did not show improvement after 8-week treatment. Conclusion: We concluded that the use of 20% diluted autologous serum tears appears to be a safe and good therapy in cases of dry eye disorder as it was effective in improvement of symptoms and objective signs even in severe dry eye disorder.

Endodontic Retreatment by Removal of Metallic Post: A Case Report.

Endodontic retreatment is a considered as a comprehensive field, divided into various categories such as coronal disassembly, removing obturation materials, locating previously missed canals, etc. This field of endodontics has its own technologies and techniques that are required to achieve clinical success. Because the field of non-surgical retreatment is comprehensive, this case report will limit its focus to the removal of the post. Removal of posts from endodontically treated teeth is a major obstacle in the retreatment of teeth having recurrent pathology, which often leads to the extraction of a tooth that might have been saved with endodontic retreatment. This case report shows the non-surgical endodontic retreatment of incompletely obturated maxillary central incisors by removal of metallic posts.

Endodontic and post-endodontic management of a fused molar.

Treatment of fused teeth needs special care and attention to the bizarre anatomy. This paper describes root canal treatment of a fused carious tooth presenting with apical periodontitis. It is a rare case of fusion of the mandibular second molar with a paramolar. There is no literature regarding placement of crown over endodontically treated fused teeth. In this case, the fused teeth were endodontically treated and restored by a porcelain fused to metal crown.

Protein p 16INK4A expression in cervical intraepithelial neoplasia and invasive squamous cell carcinoma of uterine cervix.

The association of human papilloma virus (HPV) infection and cervical intraepithelial neoplasia (CIN) is well recognized. Interaction of HPV oncogenic proteins with cellular regulatory proteins leads to up regulation of p16 INK4A , a CDK inhibitor, which is a biomarker for HPV infection. We investigated p16 expression in CIN and invasive squamous cell carcinoma (SCC) which has not been reported in the Indian population previously. Materials and Methods: Retrospective analysis of 100 cases with 20 cases each of histologically normal cervical epithelium, CIN1, 2, 3 and invasive SCC for p16 expression was performed by immunohistochemistry using commercially available mouse monoclonal antibody to p16 (clone 6H12). Statistical Analysis: For differences in expression among groups, statistical analysis was carried out using ANOVA and post hoc test of Scheffe. Results: p16 immunoreactivity was found to be both nuclear and/or cytoplasmic. The normal cervical epithelium was predominantly negative for p16 (18/20). There was a progressive increase of p16 expression with the grade of CIN. In CIN 1, two cases (20%) showed nuclear and nucleocytoplasmic positivity respectively. In contrast, diffuse strong nuclear or nucleocytoplasmic expression was observed in 45 and 55% cases of CIN 2 and CIN 3 respectively. All except one squamous cell carcinoma stained strongly positive for p16. The difference in expression between CIN 2/3 and SCC versus normal cervix was found highly significant (p is equal to 0.008 and p less than 0.001). Conclusions: p16 expression correlates excellently with the grade of CIN and is a sensitive marker of cervical intraepithelial neoplasia.

Diffuse panbronchiolitis: Report of a rare disease from India.

Initially described by a group of Japanese clinicians and pulmonary pathologists to distinguish it from other chronic obstructive lung diseases, diffuse panbronchiolitis (DPB) is an uncommon disorder which has been reported largely from the eastern world. It is imperative to recognise this condition because of its potentially treatable nature. Recently, long-term macrolide therapy has revolutionised its management. Herein, we describe a 65-year-old male who was being managed as a case of chronic bronchitis before this diagnosis was suspected and proved.