St John's Medical Research Articles

Pyruvate kinase deficiency: A case series of congenital non-spherocytic hemolytic anemia

BackgroundPyruvate kinase deficiency (PKD) is a common cause of congenital non-spherocytic hemolytic anemia (CNSHA). The heterogeneity of clinical presentation complicates the diagnosis and management of this disease. The objective was to describe the clinical, laboratory, and genetic profile of pediatric patients with PKD. Material and methodsIt was a retrospective, observational study. Children and their family members presenting to the Department of Pediatric Hematology and Oncology, St John's Medical College Hospital Bangalore, India, and diagnosed with PKD during 2011–2023 were enrolled. ResultsEight patients were identified through retrospective chart reviews. Four cases were clustered in one family and affected twins in the second family. All patients had a history of neonatal jaundice; however, they varied in their severity and the requirement for transfusion support. Some patients underwent splenectomy and one had hematopoietic stem cell transplant. ConclusionPKD is a known cause of congenital non-spherocytic hemolytic anemia with heterogeneous presentation. A high index of suspicion, enzyme assay, and genetic studies are required for diagnosis.

Posttreatment symptoms in hypothyroidism and correlations with thyroid-stimulating hormone and free triiodothyronine and free thyroxine: A ThyPRO-based follow-up study

ABSTRACT Background: Treatment of primary hypothyroidism with levothyroxine does not fully alleviate its symptoms. Though there have studies to correlate levels of tri-iodothyronine with symptomatology, its role still remains controversial. Aims: To study symptoms of hypothyroidism before and after levo-thyroxine therapy using ThyPRO 39 scores and to correlate these with thyroid hormone levels. Materials and Methods: This quasi-experimental study was conducted at the in and outpatients' departments of Internal Medicine and Endocrinology of St John's Medical College Hospital. Patients were enrolled if they were aged over 18 years and were diagnosed with hypothyroidism (TSH>/= 10 IU/l). Pregnant ladies and patients who had undergone radioiodine therapy or were diagnosed with thyroid cancers were excluded. Results: A total of 46 (F=28) patients were enrolled into the study. The mean age of the study group was 41.63 +/-SD 12.078 years.Among hypothyroid symptoms at diagnosis, Tiredness and Emotional Susceptibility had the highest effect size. Tiredness, Cognitive complaints and Depressive symptoms had significant negative correlation with fT3.While Goitre, Tiredness, and Cognitive complaints showed significant association with TSH, no correlations were noted with fT4 and many scores. There was a statistically significant negative correlation between fT3 and post therapy domain scores of Goitre, Tiredness, Impaired Daily Life and Impaired Social Life. There was a significant difference in the QoL domain between those with and without co-morbidities, the latter group having lower scores signifying better QoL. Conclusion: Symptoms of hypothyroidism may have a correlation with fT3 levels. Larger well-designed studies with supplementation of T3(triiodothyronine) and correlation with symptoms and tissue levels of fT3 will give conclusive answers.

Simulation Training in Hemodynamic Monitoring and Mechanical Ventilation: An Assessment of Physician's Performance.

ABSTRACTBackgroundSimulation is to imitate or replicate real-life scenarios in order to improve cognitive, diagnostic and therapeutic skills. An ideal model should be good enough to output realistic clinical scenarios and respond to interventions done by trainees in real time. Use of simulation-based training has been tried in various fields of medicine. The aim of our study was to prospectively evaluate the effectiveness of simulation model “CRITICA”™ (MEDUPLAY systems) in training critical care physicians.Materials and methodsThe advanced intensive care unit (ICU) simulator “CRITICA”™ (MEDUPLAY systems) was developed as a joint collaboration between the Indian Institute of Science, Bengaluru and St John’s Medical College, Bengaluru. Two-day workshop was conducted. Intensive didactic and case-based scenarios were simulated to formally teach principles of advanced ICU scenarios. The physicians were tested on clinical scenarios in hemodynamic monitoring and mechanical ventilation displayed on the simulator. Assessment of the analytical thinking and pattern recognition ability was carried out before and after the display of the scenarios. Pre- and posttest scores were collected.ResultsThe postsimulation test scores were higher than pretest scores and were statistically significant in hemodynamic monitoring and mechanical ventilation module. [Hemodynamic monitoring pre- and posttest scores 4.41 (2.06) vs 5.23 (2.22) p < 0.001] [Mechanical ventilation pre- and posttest scores 4 (2–5.5) vs 7.5 (6.5–8.5) p < 0.001]. A greater increase in posttest scores was seen in the mechanical ventilation module as compared to hemodynamic module. There was no effect of specialty or designation of a trainee on difference in pre- and posttest scores.ConclusionSimulator-based training in hemodynamic monitoring and mechanical ventilation was effective. Comparison of routine classroom teaching and simulator-based training needs to be evaluated prospectively.How to cite this articleHavaldar AA, Krishna B, Sampath S, Paramasivam SK. Simulation Training in Hemodynamic Monitoring and Mechanical Ventilation: An Assessment of Physician’s Performance. Indian J Crit Care Med 2020;24(6):423–428.

The Eternal Grove - A garden of remembrance.

The Health and Humanities division, St John's Medical College, together with the student-led environment body Ecologics, initiated the plan to have a garden space dedicated to the remembrance of those who have donated their bodies to medical education.

Time Required to perform Selected Nursing Care Activities with A View to determine the Nursing Manpower Requirement in PICU

The lack of unequivocal decision on the nurse patient ratio at the global level raises the issue to measure the required nurse patient ratio at all levels. Adequate nurse staffing levels are a fundamental prerequisite for satisfactory pediatric care. So, the aims of the study were 1. To analyze the time required to perform selected nursing care activities 2. To determine the nursing manpower requirement in PICU 3. To compare the time taken to perform selected nursing care among ventilated and non-ventilated patients. A descriptive, quantitative study was done in the PICU of St John's Medical College Hospital, Bangalore.9 staff nurses those who met the inclusion criteria were enrolled in to the study by purposive sampling technique. 39 activities which were routinely carried out in PICU were observed. The observation was performed in all three different shifts. The previous 6 months census were recorded for calculating the average number of patients in a day. The primary outcome was to calculate the time required to perform the nursing care activities with a view to calculate the nursing manpower requirement. The collected data were analyzed using descriptive and inferential statistics. The study shows that the total time required to perform nursing care activities for a ventilated patient is 16.18 hours and for a non-ventilated patient is 12.16 hours. The nursing manpower calculated by using Arndt Huckabay formula revealed that nursing manpower required in PICU is 26, whereas currently there are 22 staff nurses working for providing holistic care to the patients. Also, the comparison between the time taken to perform nursing care activities in ventilated and non-ventilated patients revealed that ventilated patients require more time for nursing care activities than non-ventilated patients.

The effect of inhaled steroids on the intraocular pressure.

Steroids are known to play a role in the pathogenesis of glaucoma, but little is known about the effect of inhaled corticosteroids (ICS). The purpose of this study was to investigate whether there is a clinically significant association between inhalational steroids and intraocular pressure (IOP). This was a cross-sectional, case-control study performed at St John's Medical College, Bengaluru, India, from October 2013 to July 2015 of 200 patients using 800 mcg of budesonide or its equivalent dose of ICS and 200 healthy controls not using any form of steroids. Patients using ICS for a period of at least 6 months with no usage of oral or topical steroids within the last 3 months were included as cases. Age- and sex-matched controls were recruited from among the general patient population of the ophthalmological department. IOP and central corneal thickness (CCT) were analyzed. Cases were divided into two subgroups. Group 1 had IOP of <21 mm Hg and cup-to-disc ratio of <0.5. Group 2 had IOP of ≥21 mm Hg or cup-to-disc ratio of ≥0.5 or cup-disc asymmetry ≥0.2. These two subgroups were analyzed to determine whether there was an increased risk of developing ocular hypertension or glaucoma with extended use of ICS. A total of 400 subjects participated, with 200 in each group. The mean IOP of cases was 15.31 ± 3.27 mm Hg, statistically significantly higher than the mean of 13.39 mm Hg ± 1.95 in controls (P < 0.001). The mean CCT in cases was 522.02 ± 30.47 μm, lower than the mean of 528.73 ± 29.09 μm of the control group (P > 0.001). Of the 200 cases, 11 (5.5%) had ocular hypertension and 2 (1%) had open-angle glaucoma. There was no statistically significant correlation between duration of inhaled steroids usage and increase in IOP (P = 0.62). There was no development of ocular hypertension or glaucoma among the controls. Our findings suggest a probable association between ICS and IOP and that it may be advisable to measure baseline IOPs and CCT and to follow patients on ICS at regular intervals.

Introducing a molecular basis to physiology in undergraduate medical education.

Medical professionals in India need to understand the concepts of molecular genetics to stay up-to-date with clinical care as well as to strengthen basic research in the biomedical sciences. We introduced a modular course on 'Fundamentals in molecular and cellular biology' for first-year medical undergraduate students in the second semester of the first MBBS year in 2015, as part of 'Innovations in teaching physiology' at St John's Medical College. This was a voluntary, add-on course with didactic lectures, hands-on practical classes and a research paper presentation. Evaluation of students' performance in the course took into account their performance in the research paper presentation-based group activity and their attendance for the hands-on practical sessions. Feedback on the content, delivery, evaluation and future improvements of the course was sought from the students. About 65% of students regularly attended the 10-hour long course. Of the students evaluated for the course, 43% achieved the highest, and 11% achieved the lowest grade assigned. Nearly 72% of the students provided feedback on the course. This first outing of the modular course on 'Fundamentals in molecular and cellular biology' generated excitement among the students and achieved some learning for them. It also brought to light hidden challenges in conducting such a voluntary course for medical students in India. Our experience with the course will help in designing a better-integrated course for exposure of first-year medical students to advances in molecular biology and its applied aspects as they progress through the MBBS course.

On the integration of Ethics into the Physiology curriculum.

A one-day state-level workshop was organised in Karnataka to share the experience of a programme implemented earlier, in 2015-16, at St John's Medical College, Bengaluru that integrated the teaching of ethics into the physiology curriculum. The aim was to develop the programme further, list the challenges likely to be faced while scaling it up, and identify other colleges which could participate in the scaling up. Twenty-eight participants, representing 13 medical colleges, and five resource persons attended the workshop. There was a consensus that the integration of ethics into the physiology course was relevant and desirable, although the participants identified several critical challenges which might arise. These included the lack of institutional support, a possible lack of student "buy-in" since it was beyond the requirements of the examinations, and time constraints. Specific areas of integration were identified. Three medical colleges, including the host institution, opted to implement the programme and refine it further.

Unravelling thyroid dysfunction in rheumatoid arthritis: History matters.

Autoimmune thyroid disease (AITD) frequently coexists with other systemic autoimmune conditions such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Due to the overlapping and nonspecific nature of symptoms, it is difficult to clinically uncover thyroidal illnesses in RA patients. This study was conducted to estimate the prevalence of thyroid dysfunction including the presence of anti-thyroid peroxidase (antiTPO) autoantibodies in patients with RA and to analyze symptomatology of thyroid dysfunction in patients diagnosed with RA. This cross-sectional, prospective study was conducted on 100 patients with RA, attending the Rheumatology Outpatient Department at St John's Medical College and Hospital, Bangalore, India. Twenty-two patients had biochemical evidence of thyroid dysfunction, hypothyroidism being the commonest (15/22 patients). Although fatigue and hair loss were the most common symptoms, only weight gain and cold intolerance were found to be statistically significant (P < 0.05) predictors of hypothyroidism and 32 patients were antiTPO positive. It was observed that equal numbers of patients developed hypothyroidism after diagnosis of RA and vice versa. History taking at the bedside to elicit symptoms, especially weight gain and cold intolerance, is quintessential to ensure timely diagnosis of hypothyroidism.

"I take up more responsibilities for my family's wellbeing" - A qualitative approach to the cultural aspects of resilience seen among young adults in Bengaluru, India.

Resilience refers to the pursuit of positive in the face of adversity. The present study using qualitative methods aimed to identify culture-specific factors linked to resilience. Participants, (N=31, aged 16-24 years) whose parents were being treated for chronic medical conditions or psychiatric disorders at St John's Medical College Hospital, Bengaluru, were studied using Focused group discussion and In-depth interviews. Using the 'Grounded theory' approach, two major themes were identified as important factors influencing resilience: (1) Family and socially defined roles/responsibilities and in particular adherence to traditionally defined social responsibilities. (2) Participation in rituals and experiencing spirituality. Application and relevance of these themes in promoting resilience among young vulnerable adults are discussed.

The mysteries of type 2 diabetes in developing countries.

More research is needed in low- and middle-income countries where the epidemic of type 2 diabetes is on the rise. KM Venkat Narayan talks to Fiona Fleck. [ILLUSTRATION OMITTED] KM Venkat Narayan is one of the world's leading researchers on type 2 diabetes. He directs the Emory Global Diabetes Research Center. Prior to joining Emory University In 2006, he spent 10 years at the United States Centers for Disease Control and Prevention, leading the science efforts in his role as Chief of the Diabetes Epidemiology Section and later the Epidemiology and Statistics Branch. Narayan worked on the first diet-exercise intervention study as part of the Pima Indian Study of diabetes at the National Institute of Diabetes and Digestive and Kidney Diseases from 1992 to 1996, where he helped to develop the Diabetes Prevention Program (DPP). Before that, he worked in India, the United Arab Emirates and the United Kingdom of Great Britain and Northern Ireland as a tenured public health physician. He graduated in medicine in 1980 from St John's Medical College, Bangalore, India and subsequently qualified in geriatric medicine, public health and management. He is a member of several international and national committees on type 2 diabetes and other noncommunicable diseases. In 2015 he won the American Diabetes Association's Kelly West Award for outstanding achievement in epidemiology and is the Danish Diabetes Academy visiting professor at the University of Copenhagen. Q; How did you become interested in type 2 diabetes research? A: When I was a physician and public health person working largely on cardiovascular diseases (CVD) in the early 1990s, a leading cause of death in Scotland at the time, I was invited to join a study of people with type 2 diabetes. This study included a work group to develop standards of care and I got to attend the Cambridge Diabetes Epidemiology Seminar. I met Peter Bennett, who was leading the Pima Indian Study at the National Institutes for Health in the United States of America. He invited me to join his team for two years. It was a difficult decision. My wife and I had to leave tenured jobs and move to Phoenix, Arizona. But those two years have extended to 24 years. Spending time with the Pima Indians and studying the disease and the suffering it was causing was a defining experience for me. I sensed that what was happening in the Pima community could happen to the rest of the world. Q: And it did happen. As a cause of death, diabetes jumped from number 15 in 1990 to number nine in 2010. Which countries now have a high burden of type 2 diabetes and which are tackling this disease in the most effective way? A: Countries in the Middle East have a very high prevalence. The countries with the largest number of people with type 2 diabetes are China, India and the United States of America (USA). A major concern today is the increasing numbers of people with type 2 diabetes--not just in China and India--but also in other middle-income and low-income countries. The greatest success in the last 10 to 15 years has been in reducing deaths and complications in people with diabetes, such as heart attacks, stroke, amputations and kidney failure, particularly in Finland, the USA and other high-income countries. High-income countries now have effective tools for the control of blood glucose, blood pressure, lipids and screening for early complications and have done useful research to find ways to implement good quality of care. Q: So more people are living with type 2 diabetes than two decades ago? A: Yes, more people are surviving longer with the disease, but that means there are more and more people with costly complications. For example, diabetes is the leading contributor to the rise in inflation-adjusted health-care costs in the United States. Q: If it's so expensive to manage the disease and its complications, why isn't more done to prevent it? …

Galactomannan Assay and Invasive Pulmonary Aspergillosis - Comparison of the Test Performance at an in-house and the Kit Cut-off.

Invasive Pulmonary Aspergillosis (IPA) is an important opportunistic infection with a high degree of mortality and morbidity. Galactomannan assay (GM assay) is found to be useful for diagnosis of IPA in patients with neutropenia. However the utility of this assay has not been evaluated in a mixed patient population with other co-morbid conditions. Though a kit cut-off of 0.5 has been recommended for the diagnosis of IPA, studies have reported a higher sensitivity with cut-offs more than 0.5. To establish an in-house cut-off and compare its utility with the kit cut-off to diagnose and categorize IPA as proven, probable and possible in patients with varied underlying risk factors. This observational study was done in St John's Medical College, Bangalore, Karnataka, India from January 2013-December 2014. GM assay was performed on 25 each of healthy controls and clinically diagnosed cases of IPA. The in-house cut-off was calculated by plotting the Receiver Operating Characteristic Curve (ROC). The in-house cut-off was calculated to be 0.52. Using this and the kit cut-off (0.5), the Sensitivity, Specificity, Positive Predictive Value (PPV) and the Negative Predictive Value (NPV) were found to be 75%, 79%, 76%, 82% and 79%, 71%, 77%, 82% respectively. Diabetes mellitus was found to be associated with more than 50% of the patients. The established in house cut-off using healthy controls and patients with clinical diagnosis of IPA was not significantly different from that of the kit cut-off. Using either of these cut-offs, we could re-categorize two of the possible IPA cases in the probable group. This study helped to understand the clinical utility of this assay even in a mixed patient population with multiple co-morbidities.

Analysis of Fetal Palate to Assist Pre-natal Ultrasound.

Cleft palate is one of the major facial congenital malformation in newborns. Pre-natal detection of this malformation is limited to detection of clefting of hard palate but isolated soft palate clefting still remains challenge for sonologists. As Indian literature is limited present study was attempted to provide dimensions and position of fetal palate by digitized images. To study dimensions, position and differences in parameters between second and third trimester fetuses. Median sagittal section of 32 formalin fixed fetuses was selected from the Department of Anatomy, St John's Medical College, Bangalore, Karnataka, India. Anatomical landmarks-The Nasion (N), Sellaturcica (S), Anterior Nasal Spine (ANS), Posterior Nasal Spine (PNS), tip of Uvula (U) were marked on sections. Length of hard palate (from ANS to PNS), Length of soft palate (from PNS to U), Hard palate/soft palate angle was defined. The anterior position of soft palate and its posterior position in relation to anterior cranial base were marked as N-S-PNS and N-S-U angle, respectively. The measurements were acquired directly from the digitized images using ImageJ software. Statistical analysis was done using SPSS 16. The mean values of ANS-PNS and PNS-U were 23.59±3.69mm and 14.39±2.70mm, respectively. The mean values of hard palate/soft palate angle, N-S-PNS and N-S-U angle were 144.720±11.11,51.150±9.09 and 93.370±9.58, respectively. Significant difference was noted between trimesters for length of hard and soft palate but not for palatal angles. During Pre-natal assessment of cleft palate, it is important for sonologist to keep in mind that the dimensions of palate proportionately increased in last two trimesters while the position remains constant.

Salim Yusuf: global leader in cardiovascular disease research

Salim Yusuf: global leader in cardiovascular disease research

Use of pleural fluid ceruloplasmin in the differentiation of exudative and transudative pleural effusion

Background:Differentiating into transudate or exudate is the first step in the evaluation of effusions. Light's criteria is the standard but a significant number of transudates may not be differentiated based on these criteria. Acute phase proteins (APP) are present in plasma, which increase or decrease by about 25% during an acute inflammatory response. Ceruloplasmin (CP) is a positive APP. Hence, this study was done to know the diagnostic value of pleural fluid (pf) CP and pf to serum ceruloplasmin ratio (CPr) to differentiate the pleural effusion (PE) into exudate and transudate as compared to Light's criteria.Materials and Methods:Setting: Medical wards of St John's Medical College Hospital, Bangalore.Design:Cross-sectional descriptive study. Patients with PE were divided into exudate and transudate by definitive diagnosis. pfCP, CPr and Light's criteria were compared with definitive diagnosis for the differentiation of pf into exudate and transudate.Results:The mean value of the pfCP and CPr was found to be significantly different between exudates and transudates. Sensitivity and specificity of pfCP at ≥ 13.34 mg/dl is 89.7% and 83.3%, CPr at ≥ 0.37 is 91.4% and 83.3%, Light's criteria 94.82% and 83.3%, respectively. Light's criteria, pfCP and CPr have similar PPV (98%) with Light's criteria having higher NPV (62.5%) than pfCP (45%) and CPr (50%), respectively. CPr has higher NPV than pfCP.Conclusions:pfCP and CPr can differentiate pf into exudate and transudate with comparable PPV to Light's criteria.

Dispatches

Dispatches

Morphometric Analysis of Superior Articular Facets of Atlas Vertebra and Its Clinical Applications in Ergonomics of Atlanto-Occipital Joints

The classic variants in superior articular facets of atlas such as, variability in shape, presence of notch, constrictions, partial or complete separation of facets, and presence of pressure facets, have been reported to a limited extent in relation to ergonomics of cranio-vertebral joint. The objectives of the present study were to report the analyzed morphological features and metrical values of superior articular facets of the atlas. The morphological features which were included in this study were variability in shapes, constrictions and partial or complete separation of facets. The metrical measure included the surface area measurement of the facets. This study was carried out on 50 (100 sides) dry atlas vertebrae from the Department of Anatomy, St John's Medical College, Bangalore, India. The superior articular facets were studied for the shape and symmetry, constrictions and partial or complete separation of facets. The surface area of the facets were measured by using Image J (Image processing and analysis in JAVA) tool, that was primarily designed to measure and analyze different metrics. The mean surface area on the right side was 157.3 ± 29.02, and on the left side, it was 158.5 ± 24.85. Oval, irregular, kidney and figure of eight shapes were observed either on both sides or on one side. Bilateral constrictions of facets were seen in 58 % of vertebrae. Bilateral, complete separations of facets were seen in 30% of vertebrae. The observations of the present study are important to understand the ergonomics of the cranio-vertebral joint and they are helpful for academicians, neuro-surgeons, clinicians and radiologists in day to day clinical practice.

Derivatives and Its Formation from the Meiotic Segregation in the Carrier Parents with Reciprocal Translocation

Chromosome derivatives are one of the outcomes in gametes, from the parents with reciprocal translocation. Individuals with derivatives have chromosomal imbalance, resulting in multiple congenital abnormalities and high mortality . The present investigation reports the observed derivatives in 19 probands; the sex ratio, parental origin and its association with meiotic segregation and the gain or loss in the chromosomes. From 1977 to 2007, 19 probands, who were referred for karyotyping and counseling to the Division of Human Genetics, St John's Medical College, Bengaluru, had cytogenetically confirmed derivatives. Their ages ranged from neonates to 14 years. 20 derivatives were observed in 13 males and 7 females; since one male showed 2 derivatives. The sex ratio was 1.7:1. 11 males and 7 females had 46 chromosomes and 1 male and 2 females had 47 chromosomes. 7 males and 2 females had derivatives of paternal origin and 5 males and 5 females had derivatives of maternal origin. 2:2 adjacent type 1 meiotic segregation was paternal in 9 and maternal in 7 probands. The subtype AB CB was paternal in 6 and maternal in 4; AD CD paternal in 4 and maternal in 2. The 3: 1 mode of maternal meiotic segregation in 3 showed AB CD CB or AD CD CB or AB AD CD sub types. The gain in partial trisomy was paternal in 9 and maternal in 15 and the loss in partial monosomy was paternal in 9 and maternal in 7. The article on the derivatives and its various aspects is reported for the first time in India.

A surgical audit of pediatric renal transplants at St John's Medical College Hospital

A surgical audit of pediatric renal transplants at St John's Medical College Hospital

Supratrochlear foramen of the humerus

The presence of the supratrochlear foramen was studied in 150 dry adult humeri, of unknown sex from the Department of Anatomy, St John's Medical College, Bangalore: The dimensions of the foramen were measured. In bones where the foramen was absent, opacity or translucency of the septum was observed. A supratrochlear foramen was found in 28% of the total humerii. It was almost equal in number on both the sides (right, 27.9%; left, 27.8%). Most of the foramina were oval in shape, their transverse and vertical diameters being, on average, 6.92 mm and 4.64 mm, respectively. A translucent septum was seen in 66% of the bones, in 51.51% of right and in 48.48% of the left. Only nine bones had opaque septae (6%). The study was done on bones belonging to South Indians and the findings were compared with the data available on other races. The paper discusses the evolutionary aspects of the foramen in addition to its surgical and orthopedic significance.