Hospitalization Events Among Adolescents and Adults With Sickle Cell Disease in a Tertiary Care Center in Central India.

BACKGROUND: Sickle cell disease (SCD) is an inherited red blood cell disorder, wherein mutation causes the substitution of glutamic acid to valine at the sixth position of the ß-globin chain. These include sickle cell anemia (homozygous sickle mutation), sickle-beta thalassemia, and hemoglobin SCD. The clinical manifestations of SCD are protean. Individuals with SCD suffer from both acute and chronic complications, which include recurring episodes of pain commonly called vaso-occlusive crisis (VOC) - acute chest syndrome (ACS); aseptic necrosis of the bone; micro-infarction of the spleen, brain, and kidney; infections; stroke; and organ damage affecting every part of the body. SCD necessitates frequent hospitalizations because of severe complications, which pose a significant burden on caregivers and economic strain on healthcare systems. The pattern of hospital admission with SCD varies in different parts of the world. OBJECTIVE: This study aimed to determine the causes of hospitalization among adolescent and adult patients with SCD and to determine factors associated with their hospital stay. METHODS: The study was a hospital-based prospective observational study comprising adolescent and adult patients diagnosed with SCD, aged 15-45 years, who were hospitalized in the Department of General Medicine at All India Institute of Medical Sciences in Raipur from August 2021 to August 2022. RESULT: According to our study, the primary reason for hospitalization was a painful crisis, accounting for 63% of cases, followed by infection (17%), ACS (11%), and acute hemolytic crisis (9%). Notably, we did not observe any significant differences between genders and causes of admission (p > 0.05). Joint pain (p = 0.005), back pain (p = 0.001), and chest pain (p = 0.001) were more frequently reported by adults over the age of 19. In addition, our analysis of the duration of hospital stays and various factors revealed that patients admitted for infections had a significantly longer mean hospital stay duration (p = 0.040). CONCLUSION: Acute painful crises were the primary cause of hospital admission among individuals with SCD; many patients also encountered infections and ACS. Furthermore, patients who experienced infections and VOC had a lengthier duration of hospital stay. Therefore, it is essential to provide them with comprehensive instructions on various preventive measures against infections and the factors that trigger painful crises.

Electrocardiographic Changes and Their Association With Disease Severity in Adults With Sickle Cell Anemia at a Tertiary Care Center: A Cross-Sectional Study.

Introduction Sickle cell anemia (SCA), a severe hematological disorder, is characterized by the presence of sickle-shaped erythrocytes that obstruct capillaries and restrict blood flow. This pathophysiology not only promotes systemic complications but may also influence cardiac function. Cardiac complications are a leading cause of mortality in SCA patients, yet the specific electrocardiographic (ECG) changes associated with disease severity are not thoroughly understood. This cross-sectional study aimed to explore ECG abnormalities in adults with SCA and correlate these findings with disease severity. Methods An observational cross-sectional study was conducted over 18 months, from January 2022 to June 2023, among 140 SCA patients at the Sickle Cell OPD of All India Institute of Medical Sciences, Raipur, Raipur, India. Steady-state SCA (HbS >50%) patients screened by high-performance liquid chromatography were enrolled. A history, physical examination, complete blood count, and ECG were done for all cases. The disease severity score was calculated using the Adegoke and Kuti severity scores, and their association with various ECG changes was studied. The chi-square test (Fisher's exact test, wherever applicable) was used for comparing the proportion. The correlation was done using the Pearson correlation coefficient or Spearman's rho. Results Out of 140 patients, the mean age of the study participants was 26 ± 6 years. More than half of the cases (80; 57%) fall under the 18-27 age group, with a male-to-female ratio of 4:3. A total of 99 (70.7%) of the participants had mild disease, and 41 (29.3%) had moderate disease. The QT interval was significantly higher among patients with mild disease compared to those with moderate disease (p-value: <0.01). QTc dispersion and prolonged QTc interval were significantly higher among patients with moderate disease compared to mild disease (p-value <0.01, 0.04, respectively). Sinus tachycardia and right ventricular hypertrophy with p-pulmonale were significantly higher in moderate severity (p < 0.01). A significant positive correlation was observed between QTc dispersion, P-wave dispersion, and severity (r: 0.19, 0.17; p-value: 0.02, 0.04, respectively). Conclusion As the disease severity progressed, the ECG changes studied had a higher distribution and significance. ECG is a readily and widely accessible investigation that can be used to screen all SCA patients for early recognition of various underlying cardiac complications.

Analysis of Drugs Prescribed to Elderly Patients in a Tertiary Health Care Center in Raipur, Central India: An Observational Study.

Background Most elderly patients suffer from multiple diseases and are on multiple drugs for treatment. Polypharmacy in the elderly, physiological changes with old age, changes in the pharmacokinetics and pharmacodynamic effects of many drugs, and newer drug prescription trends for diseases like diabetes and cardiovascular disease make drug prescribing in the elderly more difficult. There are many chances of drug-drug interactions with easily available over-the-counter (OTC) medications. To prevent the irrational use of drugs in the elderly, there is a need for prescription analysis studies. Prescription analysis studies will help in finding errors in prescriptions and also change trends in the use of medication among the elderly. Methodology This cross-sectional observation study was conducted on 234 elderly patients to investigate medicine use patterns among the geriatric patients attending the Medicine Outpatient Department in a tertiary care teaching hospital. Drug data were collected from the study participants after obtaining written informed consent and analysed, including demographic details, personal history, disease history, and details of the drug, including the generic name of the drug, dose and duration of therapy, and prescription pattern. The proportions of drugs prescribed for different diseases were analysed. Also, the drugs were analysed as per their pharmacological profiles. Results and interpretation A total of 1298 drug prescriptions were analysed in this study. Of the total participants, 60.26% were male, 35% were unemployed, 53% were retired, and 27% were taking OTC herbal medications. Most of the patients included in this study were suffering from diabetes, hypertension, and other comorbidities. Telmisartan and telmisartan in combination with other drugs were the most commonly encountered prescription drugs, i.e., 24% among the cardiovascular drugs. Aspirin and statins alone or in combination were the most commonly encountered prescriptions, i.e., 27.88% of the drugs used for prophylaxis of cardiovascular diseases. Conclusion This study showed a prescription pattern for the elderly and highlighted precautions to be taken with some of the prescribed drugs. As polypharmacy is observed with elderly prescriptions, possible drug interactions must be taken into account. Regular prescription analysis of drugs prescribed to the elderly will help in the appropriate and rational use of drugs.

Ten-Year Cardiovascular Risk as Predicted by the QRISK®3 Calculator in Diabetic Patients Attending a Tertiary Care Teaching Hospital in Central India and Its Application to Stratify Statin Over-Users and Under-Users.

BACKGROUND: Cardiovascular disease (CVD) is an important cause of morbidity and mortality in diabetic patients. As such, risk stratification is essential to identify the risk factors of CVD and provide early intervention. The QRISK®3 tool, recommended by the National Institute for Health and Care Excellence (NICE) guidelines, has the option to choose the patient's ethnicity, which is not available in other tools. However, there is a paucity of data regarding the use of this tool in the Indian population. Therefore, this study was planned to predict 10-year CVD risk using the QRISK®3 tool and to determine statin eligibility in diabetic patients. METHODS: We enrolled diabetic patients visiting our general medicine outpatient department and diabetic clinic in the study. We collected data from clinical and prescription records, as well as through patient interviews. We analyzed the data to determine the 10-year CVD risk using the QRISK®3 risk tool, which is available online. A cut-off QRISK score of 10%, as recommended by the NICE guidelines (2014), was used to stratify patients as "over-users" and "under-users." We also analyzed the data to determine any correlation between other risk factors and QRISK scores. RESULTS: Of the 134 diabetic patients recruited in this study, 43 (32.09%) had a CVD risk score of <10%, of which 16 (37.21%) were categorized as "over-users." Of the patients, 91 had a CVD risk score of ≥10%, of which 17 (18.68%) were categorized as "under-users." Risk factors showing a positive correlation with QRISK score included duration of diabetes, age, blood pressure treatment, waist circumference, and non-high-density lipoprotein cholesterol level. CONCLUSION: QRISK score can be useful to predict 10-year CVD risk in the Indian population and to stratify patients as statin over-users and under-users. This tool can be used in the Indian set-up to identify potential candidates for statin initiation.

Altered T-cell profile in sickle cell disease.

Background: Impaired immune status due to altered T-cell response in sickle cell disease (SCD) might provide substantial insight into immune activity in SCD patients. Materials & methods: A total of 30 healthy control, 20 SCD patients in a crisis state and 38 SCD patients in a steady state were evaluated for T-cell subsets. Results: A significant decrease in CD8+ (p = 0.012) and CD8+45RA-197+ (p = 0.015) T-cells were observed among SCD patients. Naive T-cells (45RA+197+; p < 0.01) were elevated and effector (RA-197-) and central memory (RA-197+) T-cells were grossly reduced in the crisis state. Negative regression of naive T-cells with CD8+57+ affirmed immune inactivation. The predictor score reflected 100% sensitivity for predicting the crisis state (area under the curve = 0.851; p < 0.001). Conclusion: Monitoring naive T-cells with predictive scores can help assess the early shift from a steady state to a crisis state.

The sickle-shaped hemoglobin in sickle cell disease (SCD) patients are known to cause frequent episodes of blockage in small vessels. Repeated episodes of blockage result in tissue injury and create a state of chronic inflammation. In response, a series of inflammatory reactions initiate such that the immune response in these patients is quite altered. To understand these changes, this study was conducted to observe alterations in T-cell subtypes and gain substantial insight into immune activity in SCD patients. A total of 30 healthy control, 20 SCD patients in a crisis state and 38 SCD patients in a steady state were evaluated for T-cell subsets. The SCD patient experienced a gross decrease in T-cells with killing ability and memorizing ability for immune responses. The SCD patients in crisis state reported a significant increase in inactivated T-cells but the levels of activated T-cells that can defend and memorize the immune response were quite low. The finding suggested that this group of SCD patients had compromised immune activation that hindered the activation and differentiation of inactivated T-cells to their effector and memory cells. An equation was derived considering all the parameters that were significantly altered to derive a predictive score that showed 100% sensitivity for predicting a crisis state. Hence, it is proposed that monitoring the inactivated T-cell population or predictive score might help clinicians to assess clinical severity at an early stage and initiate appropriate preventive measures.

Steroid and sphingomonas.

Sphingomonas paucimobilis is a Gram-negative bacteria identified as a rare infectious agent, more commonly seen among immune-compromised hosts. Infections include bacteremia, septicemia leading to septic shock, bone and soft-tissue infections such as septic arthritis, osteomyelitis, infection of shunts, and implantable devices. Although the organism has been reported to have low pathogenicity, infections in the immune compromised can be devastating leading to even death.

Résumé Sphingomonas paucimobilis est une bactérie Gram-négative identifiée comme un agent infectieux rare, plus fréquemment observé chez les hôtes immunodéprimés. Les infections comprenaient la bactériémie, la septicémie entraînant un choc septique, les infections des os et des tissus mous telles que l'arthrite septique, l'ostéomyélite, l'infection des shunts et des dispositifs implantables. Bien que l'organisme ait été signalé comme ayant une faible pathogénicité, les infections chez les personnes immunodéprimées peuvent être dévastatrices et même entraîner la mort. Mots clés: Sphingomonas, stéroïdes, antibiotiques.

Evaluation of an in-house PCR for diagnosing scrub typhus along with a preliminary study of genotypic characterization of Orientia tsutshugamushi circulating in Chhattisgarh: A prospective clinico-microbiological study.

PURPOSE: Scrub typhus, caused by Orientia tsutsugamushi (O. tsutsugamushi) present nonspecific clinical features during manifestation of acute undifferentiated febrile illness (AUFI) to render its early diagnosis difficult. Accordingly, this study was undertaken to assess an in-house groEL PCR versus IgM ELISA for the diagnosis of scrub typhus and to genotypically characterise the randomly selected scrub typhus positive cases. METHODS: Blood samples, collected from two hundred twenty one (221) AUFI cases were subjected to groEL PCR and IgM ELISA for diagnosis of scrub typhus. Eleven randomly selected PCR positive cases were processed for DNA sequencing to determine the genetic diversity of O. tsutsugamushi in Chhattisgarh. RESULTS: Scrub typhus prevalence of 35.2% were detected among AUFI cases using both in-house groEL PCR and IgM ELISA. PCR alone showed sensitivity, specificity, positive and negative predictive values of 66.6% (CI: 55.08-76.94), 100% (CI: 90 to 100),100% (CI: 93.15 to 100) and 57.37% (CI: 44.05 to 69.96) while for IgM ELISA, these parameters were 62.8% (CI: 51.13-73.50), 100% (CI: 90 to 100), 100% (CI: 92.75 to 100) and 54.68% (CI: 41.75 to 67.18) respectively. PCR and ELISA could detect scrub typhus in 37.2% and 33.3% cases, when tested alone. groEL PCR detected the O. tsutsugamushi throughout the course of infection. Phylogenetic analysis depicted 5 of 11 positive cases belonged to Kuroki, Japan strain of O. tsutsugamushi, followed by Gilliam and Karp strain in 4 and 2 cases respectively. CONCLUSION: Scrub typhus should be considered in differential diagnosis of AUFI. groEL PCR may aid on to IgM ELISA test for optimum laboratory diagnosis of scrub typhus by its implementation especially in seronegative cases. Predominance of Kuroki-like strain followed by Gillian and Karp strains of O. tsutsugamushi in Chhattisgarh confirm variable geographical distribution of O. tsutsugamushi and provide the baseline epidemiological data which will eventually be used to help the researchers for developing better diagnostic tests and vaccine covering the predominant genotypes.

Diagnosing Joubert Syndrome in Two Adult Siblings: A Very Rare Case Report.

Joubert syndrome (JS) is a rare genetic disorder usually diagnosed during childhood. Adult Joubert syndrome is rare, and that too in siblings from a non-consanguineous marriage in their adulthood is extremely rare, with very few cases reported worldwide. The need for expensive imaging modality to aid diagnosis has also been cited as a drawback in diagnosing the condition in resource-poor areas. We describe the case of two adult siblings who came for other diseases and were diagnosed with Joubert syndrome.

Usefulness of STOPP/START criteria and Beers criteria for prescribing in geriatric patients in a tertiary health care center, Raipur, Central India.

Background: Polypharmacy and inappropriate prescribing are risk factors for adverse clinical outcomes in older people. Screening tools can identify potential medicine-related patient safety incidents for the elderly on multiple medicines and with chronic diseases. Methods: In this prospective observational study, details of demography, diagnosis, history of constipation/peptic ulcer disease, over-the-counter medications, and clinical and laboratory findings were noted. Information obtained was reviewed and analyzed with the help of STOPP/START and Beers 2019 criteria. At 1 month follow-up, improvement was assessed with the help of a structured questionnaire. Results: As per the criteria, modification in drugs was recommended for 213 drugs; it was actually performed for 27.73% and 48.71% drugs as per Beers and STOPP/START criteria, respectively. Glimepiride was replaced with short-acting sulfonylureas because of hypoglycemia, and angiotensin receptor blockers were stopped because of hyperkalemia as per Beers criteria. Statins were started in 19 patients by START criteria. Overall general health improvement was observed at 1 month, but an increase in anxiety, tension, worry, depressed feel, and insomnia was observed in initial days of the coronavirus disease 2019 pandemic. Conclusions: In view of polypharmacy in the prescriptions, the combination of prescribing criteria need to be considered while prescribing medications to the elderly to get optimum therapeutic benefits and improvement in the quality of life. The quality of primary care of the elderly can also be improved by use of screening tools such as STOPP/START and Beers criteria by a primary/family physician. Prescription evaluation by a trained pharmacologist/physician for possible drug/food/disease interactions and for therapy modification can be incorporated for routine geriatric care at a tertiary care center. Clinical trial registry of India registration number: CTRI/2020/01/022852.

Wilson's Disease: Diagnosis in Novel Way.

Wilson's disease first described by Kinnier Wilson in 1912, is a rare autosomal recessive genetic disorder involving a defect in copper metabolism. This disease affects between one in 30,000 to one in 100,000 individuals and has a carrier frequency of one in every 90. It is characterized by hepatic and neurological symptoms. The usual age of presentation is 4 to 40 years but this disorder has been detected in children as young as three years and adults as old as 70 years with males and females being equally affected. Diagnosing Wilson's disease at the earliest is crucial as it is not only progressive and fatal if untreated, but also responds promptly to medication. Here we are going to present a novel way to diagnose a case of Wilson disease in a resource-limited setting. The diagnosis was possible with detailed present and past history raising strong clinical suspicion of environmental or genetically related disease. The diagnosis was done in a novel way by first diagnosing in daughter thereafter confirming the same diagnosis in patient.

Gaucher's Disease in an Adult Female: A Rare Entity.

Gaucher's disease is a rare inborn error of metabolism with an autosomal recessive pattern of inheritance. With over 26 million births occurring per annum, extrapolation of this figure would give us an estimated burden of 17,000 babies born with lysosomal storage disorder (LSD). Given the large population of India and the high rates of consanguineous marriage that takes place in the subcontinent, LSD might not be as rare as we perceive it to be. We report a rare occurrence of type-1 Gaucher's disease in an adult female patient born of a non-consanguineous marriage, belonging to the tropical area of Chhattisgarh, India where there is a predominance of malaria, thalassemia, and sickling. The diagnosis was challenging in this case since we needed to work out all the differential diagnoses of pancytopenia with hepatomegaly and massive splenomegaly. The key part was her medical history where there was documentation of her elder brother's death due to some mental illness of undiagnosed etiology. Being a difficult time due to coronavirus disease 2019 ( COVID-19) , we were able to diagnose the patient with a bone marrow biopsy followed by glucocerebrosidase enzyme level suggestive of Gaucher's disease.

Study of clinical profile of falciparum malaria in a tertiary referral centre in Central India.

BACKGROUND AND OBJECTIVE: Malaria, a disease with protean manifestations is endemic in India with an estimated 70-100 million cases each year. Of these 45-50% are plasmodium falciparum. The present study is aimed at to study clinical features, complications, response to treatment and outcome in a tertiary care hospital. METHODOLOGY: This hospital based cross sectional study was done on 80 confirmed cases of falciparum malaria (either by peripheral smear or rapid diagnostic test) more than 12 years of age admitted in NKPSIMS and LMH from December 2009 to December 2010.A case sheet proforma was prepared and data (demographic profile, clinical feature, investigation, treatment, and complication) from all indoor patients was collected and analyzed. RESULT: Out of 80 patients, 60 (75%) were males and 20 (25%) were females. Most of the patients were between the age group 21-40 years with the highest incidence between the age group of 21-30. The numbers of admissions due to malaria increased from June onward with maximum number of cases were found in the month of September. Fever was the most common symptom followed by impaired consciousness. Anemia was present in 52 (65%) patients, out of which 5 (6.25%) patients had severe anemia. Thrombocytopenia was present in 46 (57.5%) patients. Abnormal liver function tests were observed in 28 (35%) subjects while abnormal kidney function tests were observed in 26 (32.5%) patients. As per WHO definition of severe falciparum malaria, 37 patients suffered from severe falciparum malaria (46.25%) in the form of impaired consciousness or unarousable coma, clinical jaundice plus evidence of other organ dysfunction, severe renal impairment, severe anemia, circulatory collapse and ARDS. Maximum number, 62 (77%) patients received the combination of artesunate and clindamycin. This also showed that the combination of artesunate and clindamycin in severe Plasmodium falciparum malaria is a very good therapeutic option. Apart from being effective in seriously ill patients it is quite safe also. Mortality rate was 6.25%.Cause of death was acute renal failure with metabolic acidosis, aspiration pneumonia secondary to seizure, cerebral malaria and circulatory shock. CONCLUSION: Early diagnosis, anticipation of complications, close monitoring of vital parameters and combination therapy to overcome drug resistance perhaps helped to curtail the extent of mortality in this study.

Renal Doppler indices in sickle cell disease: early radiologic predictors of renovascular changes.

OBJECTIVE: The purpose of our study was to detect changes in renovascular resistance through renal Doppler indexes in young sickle cell disease patients with normal routine urine laboratory tests. CONCLUSION: Renal Doppler sonography resistive index and pulsatility index values can serve as early radiologic predictors of renovascular changes in sickle cell disease. Thereby, these findings can guide clinicians in the use of more intensive monitoring of laboratory values and initiating adequate treatment at an early stage.