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.

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.

Clinical and Epidemiological Study of Poisoning Cases Presenting to the Emergency Department of a Tertiary Care Center in Central India.

Objective To analyze the clinical and epidemiological characteristics of acute cases of poisoning and the pre-hospital measures that the patient receives before seeking care in an emergency department at a tertiary care center in Central India. Methods An observational prospective study was carried out over 18 months, and the relevant findings were documented using a predesigned data collection form. All patients who presented to the emergency department and were 18 years of age or older were recruited, and consent was sought. Data analysis was performed using the SPSS software. Results A total of 102 patients diagnosed with poisoning were taken for this study, and data were collected and analyzed. The mean age was 32.8 ± 13.75 years. Of the study population, 63 (61.8%) patients were males. In our study, the most common cause of poisoning was impulsive intake of poison (n = 22, 21.5%) and suicidal ingestion in patients with depression (n = 18, 17.6%). In the emergency department, 61 patients (59.8%) received gastric lavage, and 37 patients (36.3%) received an antidote. The most common agent of poisoning was pesticide ingestion, accounting for 45 (44%) of the total cases. Prescribed drugs were the second-largest group (n = 19, 18.6%). Other common poisoning agents were rodenticides (n = 12, 11.7%), corrosives (n = 8, 7.8%), and aluminum phosphide (n = 3, 2.9%). Out of 102 patients, 82 patients survived, 15 patients died, and five patients left against medical advice (LAMA). One patient had residual comorbidity and was discharged with jejunostomy. The maximum mortality (22.5%) was due to organophosphorus compounds. Conclusions While accidental encounters are also common, intentional self-harm accounts for the majority of poisonings; homicidal motives are less likely. Pesticides were the most often used poisoning agents, followed by prescribed and over-the-counter drugs, rodenticides, corrosive agents, and aluminum phosphide. Of the poisoned cases, 69.6% had a full recovery, 22.54% of them died, and eight (7.84%) among them left against medical advice (LAMA). Organophosphorus chemicals were the cause of the highest mortality (22.5%).

Factors Influencing Corrected Count Increment After Platelet Transfusion in Thrombocytopenic Patients.

Background Platelet transfusion is a life-saving procedure for thrombocytopenic patients. Platelet transfusions can be either prophylactic or therapeutic. Prophylactic platelet transfusion reduces the risk of bleeding before any specific procedure, whereas therapeutic platelet transfusion helps to control active bleeding. Evaluation of the response to platelet transfusion by calculating the corrected count increment (CCI) is important to determine the success of platelet transfusion and to plan subsequent patient management. Methods We conducted a prospective observational study in patients who received at least one unit of platelet concentrate (random donor platelet (RDP) or single donor platelet (SDP)) admitted under or seen by General Medicine. Patients over 60 years, multiparous females, and chemotherapy patients were excluded. The patient's pre-transfusion and post-transfusion platelet counts at one hour and 24 hours were taken, and CCI was calculated. Results We studied 60 patients during the study period, out of which 35 were males (58.33%) and 25 were females (41.67%). The mean age was 36.2 years, and the age range was from 15 to 60 years. The majority of patients were O-Rh positive (41.67%), followed by B-Rh positive (40%), A-Rh positive (11.67%), and least by AB-Rh positive (5%) and only one patient with O-Rh negative (1.67%). The logistic regression analysis showed that sepsis (p=0.025), splenomegaly (p=0.004), COVID-19 (p=0.016), dengue (p=0.028), systemic lupus erythematosus (SLE) (p=0.045), immune thrombocytopenic purpura (ITP) (p=0.003) and fever (p=0.007) significantly contributed to unsuccessful CCI. However, acute leukemia (p=0.238), active bleeding (p=0.147), disseminated intravascular coagulopathy (DIC) (p=0.952), aplastic anemia (AA) (p=0.114), and sickle cell disease (SCD) (p=0.739) did not show any statistical significance. Conclusion Unsuccessful CCI at 24 hours is attributed to non-immunological clinical factors like sepsis, splenomegaly, COVID-19, and fever, whereas immunological clinical factors like SLE, dengue, and ITP resulted in unsuccessful CCI at one hour, as evident by this study.

Serum uric acid level in chronic liver disease and its correlation with Child-Pugh score in a tertiary care hospital from South India.

Background: Chronic liver disease (CLD) is one of the important causes of morbidity and mortality in our country, and since the damage to the liver is irreversible, we have to look for many severity markers or predictors for the prognosis of the patient. In this study, we have tried to correlate the level of serum uric acid (UA) with the severity of CLD presented as a Child-Pugh score. Methods: A cross-sectional observational study was conducted at Vijayanagar Institute of Medical Science (VIMS), Ballari, Karnataka, from October 2015 to June 2017 in the Department of General Medicine. Fifty patients diagnosed with CLD, aged between 18 and 65 years, of either gender, were enrolled in the study. Serum UA levels were measured, and liver function and coagulation parameters were assessed. A statistical analysis was performed to evaluate the association between serum UA levels, liver function test, and coagulation parameters. Results: In our study, the mean serum UA level was 6.52 mg/dl and was raised in patients with CLD in correlation to its severity. Alcoholic liver disease (ALD) was the most common etiology for CLD (80%) followed by hepatitis B (Hep B) virus infection (12%) and hepatitis C (Hep C) virus infection (6%). Serum UA levels increased as the Child-Turcotte-Pugh (CTP) score increased. The mean UA level in CTP class C was 8.29 mg/dl. Various parameters such as serum aspartate aminotransferase (AST), alanine transaminase (ALT), alkaline phosphatase, total bilirubin, international normalized ratio (INR), calcium, and albumin were significantly associated with serum UA levels in CLD patients. Conclusion: The correlation between rising blood UA levels and the Child-Pugh score shows that UA estimate may be a valid and affordable indicator for assessing the extent of liver cirrhosis in CLD.

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.

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.