Publications

We present a case of a patient with undiagnosed myasthenia gravis presenting in myasthenic crisis. In this case, the patient presented primarily with myocardial infarction with nonobstructive coronary arteries and respiratory failure and was later diagnosed to be in myasthenic crisis. The myasthenic crisis was treated with intravenous immunoglobulin (IVIg) and corticosteroids. Given the inadequate response to IVIg and glucocorticoids, the patient was subsequently administered an anti-CD20 monoclonal antibody in the form of rituximab. The patient responded well to rituximab, and her cardiac function subsequently improved.

Autoimmune hepatitis (AIH) is a long-lasting liver ailment. It causes hepatocellular necrosis and inflammation, leading to fibrosis. It can develop into cirrhosis and liver failure. The disease predominantly affects young to middle-aged women more than men. AIH flares up during gestation and is linked with a high rate of embryonic and maternal problems. With maternal and antenatal care becoming advanced, this disorder should be identified and managed for successful maternal and embryonic outcomes. We present a case report of a primigravida diagnosed with AIH at 14 weeks antenatally. Our main aim in reporting this case is to create general awareness for healthcare professionals and thereby for patients and caregivers about this condition in pregnancy.

An immediate need exists for a safe, quick, and effective reversal agent in patients who present with anticoagulant-induced major bleed. Dabigatran, a newer oral anticoagulant and a direct thrombin inhibitor (DTI), can also induce bleeding risk. Idarucizumab is a specific dabigatran reversal drug that the United States Food and Drug Administration (US FDA) has approved in the event of a significant bleeding caused by this drug. In this particular drug-related bleeding, it can be challenging to precisely dose alternative reversal agents like prothrombin complex concentrates (PCCs) and activated PCCs (aPCCs) depending on coagulation characteristics. Additionally, they may result in thromboembolic problems. Despite these drawbacks, the inability to get idarucizumab may necessitate the use of these medications in cases of life-threatening bleeding. We describe the case of a 65-year-old male who reported to the hospital with coagulopathy, anemia, and fresh bleeding per rectum (Hb: 5.8 gm/dL, PT 20.02 seconds, INR: 1.55). He was on dabigatran for the past 1 month. Even after stopping dabigatran, injection of vitamin K, 4 units of blood transfusion, and 8 units of fresh frozen plasma (FFP), he was still bleeding, with fall in hemoglobin level. Following the administration of PCC, he significantly improved, and no additional transfusion products were needed. He could be sent home after 4 days. After 1 month, he returned for follow-up with no further complications.

BACKGROUND: Drowning is the third leading cause of unintentional injury and death worldwide, accounting for 7% of all injury-related deaths. People who are at higher risk include those who have free accessibility to water and younger children lacking supervision. The common complications of drowning are mainly due to hypoxia. It mainly affects the respiratory, cardiac, and central nervous system. Rarely, drowning may cause acute kidney injury (AKI) secondary to rhabdomyolysis. Here, we present a case of near drowning with AKI, rhabdomyolysis, and elevated intracranial tension (ICT) with a false localizing sign (abducent nerve palsy).

CASE DESCRIPTION: A 21-year-old female patient was brought with a history of alleged drowning for about 3-5 minutes. The patient presented with loss of consciousness; she was gasping and had diffuse subcutaneous emphysema. On examination, the patient had bilateral coarse crepitations. She was started on mechanical ventilation. After 2 days, the patient developed elevated renal parameters and had an episode of ventricular tachycardia. After 10 days, the patient developed diplopia and blurring of vision. Ophthalmological examination revealed bilateral abducent nerve palsy with established papilledema and hemorrhagic retinopathy. The patient was treated with IV antibiotics, IV fluids, DC shock, inotropes, hemodialysis, and acetazolamide. After 15 days, the patient recovered from bilateral lateral rectus palsy and had no blurring of vision.

CONCLUSION: Drowning is a leading cause of accidental death. This patient had diffuse subcutaneous emphysema, rhabdomyolysis with AKI, ventricular tachycardia, and raised ICT. In drowning, raised ICT often has a poor outcome. However, with constant monitoring and timely intervention this patient survived.

Multiple myeloma (MM) is a clonal plasma cell proliferative disorder that accounts for 1% of all cancers (Rajkumar). It is a disease of older adults, presenting with anemia, bone pain, and end-organ damage in the form of CRAB features. With increasing awareness regarding this entity and the availability of more-sensitive diagnostic modalities, involvement of younger age-groups with atypical presentations is becoming more common. Newer case series report that nearly 50% of MM cases present initially with renal failure of unknown etiology (Shankar et al.), highlighting the unique role of the nephrologist in the diagnosis of this condition. In these two case reports, we wish to highlight two cases presenting with acute renal failure of unknown etiology, who were subsequently diagnosed to have occult MM with light chain deposition disease (LCDD).

A high risk of thrombosis is linked to myeloproliferative neoplasm (MPN) and antiphospholipid syndrome (APS). The systemic autoimmune disorder known as APS is characterized by persistently positive antiphospholipid antibodies [anticardiolipin (aCL), lupus anticoagulant, and antibeta 2 glycoprotein 1 IgG and IgM antibodies] in conjunction with obstetrical complications or thrombosis (Chayoua et al.). Polycythemia vera (PV) is a MPN that causes too many red blood cells (RBCs) in the blood and proinflammatory cytokines. In this report, we present a case of a 25-year-old lady with a history of second-trimester abortion who presented with abdominal pain and hepatosplenomegaly. Owing to erythrocytosis, thrombocytosis, and moderate hepatosplenomegaly, workup for MPN was done along with prothrombotic workup including APS, and she turned out to be positive for both. Coexistence of MPN and APS is rare in the literature. The optimal management of patients with coexistent APS and MPN has not been defined so far. Immediate anticoagulation with specific treatment for MPN is essential to prevent further thromboembolic episodes and progression to catastrophic APS.

Takotsubo cardiomyopathy (TCM) is a type of disorder of cardiomyocytes in which there is apical akinesia and ballooning, whereas the base is hyperkinetic. Reverse Takotsubo cardiomyopathy (rTCM) is a rare variant of TCM in which the base of the heart is akinetic and ballooned out rather than the apex, which is hyperkinetic. Takotsubo cardiomyopathy is usually seen in postmenopausal women, but a rising number of cases of the reverse variant are being reported in peripartum women. We present a case of a 24-year-old primigravida at 37 weeks of gestation, who presented with an acute onset of breathlessness just after cesarean delivery. A 2D echocardiogram revealed changes of rTCM with an overall ejection fraction of 40%. She was treated for the same, and a 2D echocardiogram repeated after 1 week showed improvement in the ejection fraction to >60%, which supported our diagnosis of peripartum rTCM. Another important objective of this study is to differentiate TCM occurring in the peripartum period from peripartum cardiomyopathy (PPCM), both of which are clinically indistinguishable but have different etiopathogenesis, treatment, and prognosis. While rTCM treatment mostly includes the management of heart failure, such as oxygen supplementation, diuretics, and noninvasive mechanical ventilation, the management of PPCM also includes bromocriptine, along with treating heart failure. The outcome of rTCM is excellent, with recovery of left ventricle function in almost all cases, while a few patients of PPCM have irreversible heart failure, making it imperative to differentiate between the two clinical entities.

Amyloidosis is an extremely rare condition with a variety of symptoms that, in extreme situations, can be fatal. Basically a protein misfolding disorder, it is characterized by deposition of insoluble polymeric protein fibrils in tissues and organs. There are different types of amyloidosis; here, we see a case of secondary amyloidosis, which is usually a consequence of a chronic disease. The case is unique, as there are many research projects showing the link of tuberculosis (TB) and secondary amyloidosis, but it is rarely documented because of extrapulmonary TB, which here is tuberculous lymphadenitis, and also here the patient is younger than the mean age. The clinical manifestations of this disease are seen mainly in the renal system, gastrointestinal system, and reticuloendothelial system. For which the management is mainly supportive, along with treatment of the underlying cause.

INTRODUCTION: Acute kidney injury (AKI) is a well-known serious complication of cardiopulmonary bypass (CPB) surgery and one of the significant risk factors for mortality, prolonged hospital stay, and additional cost. Patients having preexisting kidney dysfunction are more likely to develop AKI in the perioperative period. The complexity of CPB surgery often leads to AKI. Mechanisms of AKI include kidney hypoperfusion due to low-pressure blood flow. The nonpulsatile perfusion of the kidney, hypothermia, and inflammatory milieu, which causes afferent arteriolar constriction, contribute to AKI. The early postoperative period is characterized by a low cardiac output state, which gradually surpasses kidney compensatory mechanisms and filtration reserve. Various indigenous and infused vasopressors cause markedly elevated afferent arteriolar resistance, leading to a drop in glomerular filtration rate (GFR). Several studies have assessed the value of risk factors and their association with AKI after cardiac surgery. The evidence was mixed, with some showing a positive association. With an aim to clarify this relationship further, especially in the Indian population, we tried to study the incidence and clinical profile of AKI and its correlation with functional and clinical outcomes. We also tried to look for any diagnostic markers of AKI in the setting of cardiac surgery.

METHODOLOGY: The study was conducted among patients attending the Department of General Medicine and Cardiology at a tertiary care hospital in Delhi. It was a prospective longitudinal observational study conducted between March 2022 and February 2024. Around 200 patients underwent cardiac surgery using a cardiopulmonary bypass machine at the study center during the study period. History, including comorbidities such as transient ischemic attacks, previous stroke, coronary artery disease, diabetes mellitus, hypertension, chronic obstructive pulmonary disease (COPD), and complete physical examination, were recorded. Patients were followed up preoperatively and postoperatively up to day 28. Preoperative details such as hemoglobin, serum creatinine, blood transfusion, and urine output were recorded. Intraoperative details such as duration of surgery, ACC (aortic cross-clamp) duration, hypotension, vasopressor use, and re-exploration were recorded. Postoperative findings such as urine output and serial kidney function tests on day 3, day 7, and day 28 were documented.

RESULTS: Among 200 subjects, 99 patients had hypertension, and 70 patients developed AKI. Older age (>60 years) was significantly associated with AKI (p-value 0.04367). Comorbid conditions such as T2DM, hypertension, dyslipidemia, and COPD were significantly associated with AKI as compared to those without comorbidities (Chi-squared test, p-value < 0.0001). In the study, there was no association between the type of surgery and the development of AKI (Chi-squared test, p-value 0.07). There was no relationship between AKI severity and cardiopulmonary bypass (CPB) duration. Similarly, there was no association between the severity of AKI and ACC duration. Intraoperative hypotension was significantly associated with AKI. About 53% of hypotensive patients developed AKI during surgery as compared to 19.44% of normotensive patients (p-value < 0.0001, Chi-squared test). AKI was linked with a significantly prolonged hospital stay. A prolonged stay of >3 weeks was seen in 8.5% (6 out of 70) of patients who developed AKI as compared to 2.3% (3 out of 130) of patients without AKI. Most patients with AKI (57%) recovered within 1 week, and 24.28% recovered between 1 and 4 weeks. In the study, 8 patients (11.2%) developed acute kidney disease (AKD), and 5 patients (7%) died.

CONCLUSION: This prospective study concluded that AKI is a common complication in the perioperative period of cardiopulmonary bypass surgery. Older age, comorbid conditions, and intraoperative hypotension were significantly associated with AKI. AKI was linked with extended hospital stay and longer recovery times. Severe grades of AKI were associated with progression to AKD, need for dialysis, and higher mortality. It is imperative to focus on interventions to minimize and address the risk factors to reduce morbidity and mortality associated with AKI in CPB surgery.

Woolly hair with palmoplantar keratoderma (WH-PPK) is a group of four autosomal recessive syndromes. Type 4 WH-PPK is usually associated with KANK2 mutation and does not have cardiac morbidity among its features. Here we report a 25-year-old woman with woolly hair, palmoplantar keratoderma without any cardiac morbidity. However, she had sensorineural hearing loss and maculopathy. Thus, we present a patient with type 4 WH-PPK with a novel phenotype to highlight the rare WH-PPK syndromes. The association of woolly hair and palmoplantar keratoderma without cardiomyopathy, and with macular deposits and sensorineural hearing loss, has not been reported before.