Renal manifestations in adult-onset Still's disease: a systematic review.

OBJECTIVE: We aimed to review the literature on the clinical presentation, renal pathology, treatment, and outcome of renal manifestations in adult-onset Still's disease (AOSD). METHODS: We used PRISMA guidelines for our systematic review and included all English-language original articles from inception till September 15, 2023, on AOSD and kidney involvement in any form. Data on patient demographics, diagnostic criteria, clinical presentation, renal pathology, treatment employed including dialysis, outcome, cause of death were collected and analyzed. RESULTS: The median age at the diagnosis of renal issues was 37, with a higher prevalence among females (58.1%). Among the cases, 28 experienced renal problems after being diagnosed with AOSD, 12 had simultaneous diagnoses of renal issues and AOSD, and in 4 cases, renal problems appeared before AOSD diagnosis. Out of the 44 cases, 36 underwent renal biopsy, revealing various pathology findings including AA amyloidosis (25%), collapsing glomerulopathy (11.4%), thrombotic microangiopathy (TMA) (11.4%), IgA nephropathy (9.1%), minimal change disease (6.8%), and others. Some cases were clinically diagnosed with TMA, proximal tubular dysfunction, or macrophage activation syndrome-related acute kidney injury. Treatment approaches varied, but glucocorticoids were commonly used. Renal involvement was associated with increased mortality and morbidity, with 6 out of 44 patients passing away, 4 progressing to end-stage renal disease (ESRD), and data on 2 cases' outcomes not available. CONCLUSION: Renal manifestations in AOSD are diverse but rarely studied owing to the rarity of the disease. Studies with larger data would be essential to study further on the pathogenesis and implications.

Inflammation in sleep apnea: an update.

Obstructive sleep apnea (OSA) is a common disorder associated with cardiovascular disease (CVD). One theory to explain this relationship proposes that OSA can induce systemic inflammation, thereby inducing CVD. This theory is based on the premise that obesity is a pro-inflammatory state, and that physiological derangements during sleep in subjects with OSA further aggravate inflammation. In support of this theory, some clinical studies have shown elevated inflammatory biomarkers in OSA subjects, or improvement in these markers following treatment of OSA. However, the data are inconsistent and often confounded by the effects of comorbid obesity. Animal models of OSA have been developed, which involve exposure of rodents or cells to intermittent hypoxia, a hallmark feature of OSA. Several of these experiments demonstrate that intermittent hypoxia can stimulate inflammatory pathways and lead to cardiovascular or metabolic pathology. In this review, we review relationships between OSA and inflammation, with particular attention to studies published within the last year.

Multiorgan Dysfunction in a Patient With Adult-Onset Still's Disease Flare: A Case Report.

Adult-onset Still's disease (AOSD) is a rare multisystem inflammatory disorder. A 71-year-old lady who was on treatment for AOSD presented with clinical evidence of heart failure and was subsequently found to have impaired renal and hepatic function. Following extensive workup including a liver biopsy, the cause of liver dysfunction was determined to be congestive hepatopathy, while renal dysfunction was presumed to stem from the low output state. The etiology of myocardial dysfunction, driving liver and kidney injury, was considered to be myocarditis from AOSD or global myocardial dysfunction from a systemic inflammatory state. Management involved pulse-dose glucocorticoids followed by taper and anakinra for AOSD, alongside goal-directed medical therapy for cardiac failure. At follow-up after a month, hepatic and renal function had fully recovered, whereas cardiac function remained compromised, evidenced by persistently depressed ejection fraction and global hypokinesia on a repeat echocardiogram. This report delineates a systematic approach to multiorgan dysfunction in a patient with a rare condition such as AOSD and reviews the reported causes of hepatic and cardiac involvement in AOSD.

A pragmatic calibration of the ROX index to predict outcome of nasal high-flow therapy in India.

PURPOSE: Identifying thresholds at which the ROX index would satisfactorily predict HFNC failure across heterogenous resourced contexts is necessary for clinical use. METHODS: Patients ≥18 years admitted to 30 diverse ICUs across 10 states in India who required HFNC for respiratory support were retrospectively included in this study. Patient data and hourly ROX indices were then analyzed and contextualized to clinical outcomes as well as with ROX index thresholds in other regions of the world. MEASUREMENTS AND MAIN RESULTS: Among the 614 patients included, 276 (44.9%) required respiratory escalation. Pneumonia was the most common diagnosis on admission. Receiver operating characteristic curve analysis revealed that a baseline ROX score of 7.86 was similar to 4.88 in other populations which was confirmed by Kaplan-Mier curves (hazard ratio,3.58 (2.72-4.69, p < 0.001)). ROX scores at 11.84 or 5.89 had roles in screening and confirming HFNC failure. The index performed poorly in a subset of patients who died without respiratory escalation. The ROX index was most predictive in the initial hours of ICU admission and a longer duration of HFNC was associated with more severe outcomes. CONCLUSIONS: When optimally calibrated this index can using a method that can reliably predict the risk of HFNC failure among ICU patients from diverse settings.

AIMS65, Glasgow-Blatchford bleeding score and modified Glasgow-Blatchford bleeding score in predicting outcomes of upper gastrointestinal bleeding: An accuracy and calibration study.

BACKGROUND: Albumin, international normalized ratio (INR), mental status, systolic blood pressure, age >65 years (AIMS65), Glasgow-Blatchford bleeding score (GBS) and modified GBS (mGBS) are three pre-endoscopy scoring systems used in the risk stratification of upper gastrointestinal bleeding (UGIB). The utility of such scoring systems in a population is estimated by their accuracy and calibration in the population. We aimed at validating and comparing the accuracy of the three scoring systems in predicting clinical outcomes including in-hospital mortality, need for blood transfusion, endoscopic treatment and rebleeding risk. METHOD: We conducted a single-center, retrospective cohort study on patients with UGIB at a tertiary care center in India over 12 months. Clinical and laboratory data was collected from all patients admitted with UGIB. All patients were risk stratified using AIMS65, GBS and mGBS. The clinical outcome examined were: in-hospital mortality, requirement of blood transfusion, need for endoscopic treatment and rebleeding during hospital stay. The area under receiver-operating curve (AUROC) was calculated to assess the performance and calibration curves (Hosmer-Lemeshow goodness of fit curve) were plotted to examine how accurately the model describes the data of all three scoring systems. RESULTS: Total 260 patients were included in the study, of which 236 (90.8%) were males. As many as 144 (55.4%) patients required blood transfusion and 64 (30.8%) required endoscopic treatment. While the incidence of rebleeding was 7.7%, in hospital mortality was 15.4%. Of 208 who underwent endoscopy, the most common causes identified were varices (49%) and gastritis (18.2%), followed by ulcer (11%), Mallory-Weiss tear (8.1%), portal hypertensive gastropathy (6.7%), malignancy (4.8%) and esophageal candidiasis (1.9%). The median AIMS65 score was 1, GBS 7 and mGBS 6. The area under curve (AUROC) for AIMS65, GBS and mGBS was (0.77, 0.73,0.70), (0.75, 0.82,0.83), (0.56, 0.58,0.83), (0.81, 0.94,0.53) for in-hospital mortality, blood transfusion requirement, endoscopic treatment and rebleeding prediction, respectively. CONCLUSION: GBS and mGBS are superior to AIMS65 in predicting the requirement of blood transfusion and rebleeding risk, whereas in-hospital mortality was better predicted by AIMS 65. Both scores performed poorly in predicting the need of endoscopic treatment. An AIMS65 of 0,1 and a GBS of ≤ 1 are not associated with significant adverse events. A poor calibration of the scores in our population points to the lack of generalizability of these scoring systems.

Pituitary apoplexy induced by gonadotropin-releasing hormone (GnRH) agonist administration for treatment of prostate cancer: a systematic review.

OBJECTIVE: We aimed to review of literature on the clinical presentation, management and outcomes of pituitary apoplexy following gonadotrophic release hormone (GnRH) agonist administration for the treatment of prostate cancer. METHODS: We used PRISMA guidelines for our systematic review and included all English language original articles on pituitary apoplexy following GnRH agonist administration among prostate cancer patients from Jan 1, 1995 to Dec 31, 2020. Data on patient demographics, prostate cancer type, Gleason score at diagnosis, history of pituitary adenoma, clinical presentation, GnRH agonist, interval to pituitary apoplexy, laboratory evaluation at admission, radiologic findings, treatment of pituitary apoplexy, time to surgery if performed, pathology findings, and clinical/hormonal outcomes were collected and analyzed. RESULTS: Twenty-one patients with pituitary apoplexy met our inclusion criteria. The mean age of patients was 70 (60-83) years. Leuprolide was the most common used GnRH agonist, used in 61.9% of patients. Median duration to symptom onset was 5 h (few minutes to 6 months). Headache was reported by all patients followed by ophthalmoplegia (85.7%) and nausea/vomiting (71.4%). Three patients had blindness at presentation. Only 8 cases reported complete anterior pituitary hormone evaluation on presentation and the most common endocrine abnormality was FSH elevation. Tumor size was described only in 15 cases and the mean tumor size was 26.26 mm (18-48 mm). Suprasellar extension was the most common imaging finding seen in 7 patients. 71.4% of patients underwent pituitary surgery, while 23.8% were managed conservatively. Interval between symptoms onset to pituitary surgery was 7 days (1-90 days). Gonadotroph adenoma was most common histopathologic finding. Clinical resolution was comparable, while endocrine outcomes were variable among patients with conservative vs surgical management. CONCLUSION: Although the use of GnRH agonists is relatively safe, it can rarely lead to pituitary apoplexy especially in patients with pre-existing pituitary adenoma. Physicians should be aware of this complication as it can be life threatening. A multidisciplinary team approach is recommended in treating individuals with pituitary apoplexy.

Use of home telemedicine for critical illness rehabilitation: an Indian success story.

One-fifth of healthcare beneficiaries in developed nations get discharged from hospitals to physician supervised skilled nursing care facilities. In low-income and middle-income countries like India, postdischarge skilled nursing facilities are at a very nascent stage and largely underequipped in terms of infrastructure, skilled nursing and physician staff to manage complicated patients. Hence the responsibility of management of such patients lies largely with their families. We present a case where a 26-year-old man with Duchenne Muscular Dystrophy who became ventilator dependent following major surgeries was weaned off his ventilator and rehabilitated back to his prehospital state. This was done at his home with visiting nurses and rehabilitation services under telemedicine supervision by a critical care specialist. Use of telemedicine services could be a viable and cost-effective option to ensure adherence to evidence-based medicine and standardisation of care in resource limited countries such as India.

Case of autoimmune polyglandular syndrome type 2: how we uncovered the diagnosis.

A 24-year-old man with no significant medical history presented to the medical clinic with vomiting and giddiness for 2 days, loss of appetite for 1 month and progressive fatigability for the preceding 4 months. On examination, he was found to be hypotensive and was admitted to the hospital for work-up. Considering his abnormal labs and physical findings, he was worked up and was diagnosed with primary adrenal insufficiency. On further work-up for the aetiology of his Addison's disease, he was found to have concurrent autoimmune thyroiditis and vitiligo. A final diagnosis of autoimmune polyglandular syndrome type 2 was made. The patient was started on hormone replacement therapy and reported improvement of symptoms on 3-month follow-up visit.

Antibiotic Prescription Practices for Surgical Prophylaxis in India: An Observational Study.

Perioperative prophylactic antibiotics following surgeries have been shown to reduce surgical site infections, and their administration is common practice. Despite clear guidelines regarding this being set forth by the Infectious Disease Society of America (IDSA), adherence to them is yet to translate to common practice in many parts of the world. A retrospective chart review of 409 patients who underwent elective surgeries over a period of 7 months at three different hospitals in India was performed. In-hospital antibiotic prescriptions of these patients were examined for any apparent inappropriateness (use of antibiotics with coverage broader than the target microbes and use of antibiotics with overlapping spectrum of target microbes). Four hundred ninety-five (48.9%) of the 1,012 patient intensive care unit (ICU) days had apparent inappropriateness in the choice of their prophylactic antibiotics and only 3.2% of the antibiotic units used were in accordance with the present IDSA guidelines. Injudicious use of broad-spectrum antibiotics for surgical prophylaxis is prevalent in low- and middle-income countries such as India. This poses the risk of emergence of resistant microorganisms in these areas and their potential spread across the borders. There is an acute need for diligent antibiotic stewardship programs in these areas to curb such practices.

Periaortic Abscess following DeBakey Type-1 Aortic Dissection Repair with Dacron Graft-Early Diagnosis and Management.

A 71-year-old male with history of DeBakey type-1 aortic dissection and repair with dacron graft three months prior to presentation was brought to the emergency room with complaints of high-grade fevers, chills, and shortness of breath. Chest X-ray revealed right lower lobe infiltrates and widened superior mediastinum. A follow-up CT chest with contrast showed fluid collection around the aortic graft. He was started on intravenous broad-spectrum antibiotics, and a TEE was done for further evaluation of periaortic fluid collection which showed findings to suggest periaortic abscess. The patient underwent surgical drainage of the abscess and was found to have an abscess around the surgical aortic graft which was drained followed by two weeks of antibiotic treatment. The patient was discharged to a rehabilitation facility and remained asymptomatic at three-month follow-up appointment. Type-1 aortic dissection is a medical emergency requiring acute surgical intervention, and despite significant advancements in diagnosis and management, the immediate and long-term complications remain high leading to increased risk of mortality. Our patient developed spontaneous periaortic abscess three months postoperatively requiring intensive antibiotic therapy along with surgical drainage. Our case emphasizes the importance of early diagnosis and management of late complications of periaortic abscess in patients with aortic dissection repair.

A Rare Case of Gastric Lipoma Presenting with Gastric Outlet Obstruction Treated Endoscopically.

An 85-year-old male referred to the Gastroenterology (GI) clinic with three-month history of failure to thrive and three-week history of nausea, vomiting, and melanotic stools. Ulcerative mass obstructing gastric outlet was found on endoscopy and on follow-up CT abdomen a homogeneous submucosal mass in the gastric antrum was identified. Radiological diagnosis of giant gastric lipoma was established and patient was evaluated for surgery and, however, was rendered unfit for surgery due to his comorbid conditions. Patient was taken for endoscopic resection of the mass. On endoscopy, only partial resection was achieved due to the size of the mass, but endoloops were deployed at the stalk at the end of the procedure in hope of limiting blood supply to the lesion. On six-week follow-up endoscopy, patient's mass had completely disappeared with limited scar tissue at the site.

Effect of Transcatheter Aortic Valve Implantation on Renal Function in Patients With Previous Renal Dysfunction.

The study aims to investigate the incidence of immediate renal function improvement in renal dysfunction patients who had transcatheter aortic valve implantation (TAVI). TAVI patients with ≥ moderate reduced renal function [estimated GFR ≤ 60 ml/min/1.73 m2] at baseline were identified from a prospectively maintained database. Patients were divided into 3 groups based on percent change [(discharge eGFR - baseline eGFR/baseline GFR) × 100] in eGFR post-TAVR. Improvement ≥ 10%, no change, Decline ≥ 10%. Multivariable logistic regression was performed to identify factors that predicted improvement/decline in GFR postprocedure. Out of 677 patients, 359 (53%) had eGFR ≤ 60 ml/min/1.73 m2. Of these, 188 (52%) had an improvement in eGFR ≥ 10%, 125 (34%) had no change and 48 (14%) observed decline ≥ 10%. All groups had similar proportions of females and age was comparable in patient groups. Patients in whom a decline in eGFR was observed had significantly higher Society of thoracic Surgeons scores (10.7 vs 8.2 vs 8.2; p = 0.007) and incidence of liver disease (6% vs, 0% vs 2%; p = 0.014) than the no-change or improved groups respectively. On multivariable analysis, independent predictors of decline/improvement in eGFR were being female, low left ventricular ejection fraction and baseline liver dysfunction. In conclusion, over half of patients with compromised renal function who underwent TAVI experience an immediate improvement in kidney function post-TAVI. Being female, baseline liver dysfunction and a low left ventricular ejection fraction is associated with an immediate decline in eGFR.

A case of tricuspid valve non-bacterial thrombotic endocarditis presenting as pulmonary embolism in a patient with antiphospholipid antibody syndrome.

A 47-year-old woman with a medical history of Raynaud's phenomenon presented with fever, cough and shortness of breath. She was found to have left lower lobe consolidation and pleural effusion and was treated as a case of pneumonia. During the hospital course, her respiratory status worsened, and she was intubated on the third hospital day. To investigate the high A-a gradient, a Computerized Tomographic Pulmonary Embolism (CTPE) study was done which identified a large left lower pulmonary artery embolism. She was also found to have a new murmur, and an echocardiogram demonstrated a large lesion on tricuspid valve. However, multiple sets of her blood cultures came back consistently negative. Alternative diagnoses for culture-negative endocarditis were considered, and a full set of rheumatological workup was done. Laboratory tests were suggestive of antiphospholipid syndrome, hence the diagnosis of tricuspid valve Libman-Sacks endocarditis was made.

Case of acute paraspinal pyomyositis in an elderly diabetic secondary to spread from urinary tract infection.

A 73-year-old woman with medical history of diabetes and hypertension presented with right-sided back pain and night sweats of 1 month duration. On physical examination, there was costovertebral angle tenderness and her urinalysis was indicative for urinary tract infection. She was admitted and managed with intravenous antibiotics. On initial imaging, there was a right-sided retroperitoneal mass adjacent to right kidney. This was investigated further with MRI, which showed diffuse inflammation of right paraspinal muscles with two loculated abscesses. The abscesses were aspirated and the culture sent grew Streptococcus agalactiae (Group B Streptococcus) and the patient was treated with 6-week course of intravenous antibiotics.

Fever of Unknown Origin as a Sole Presentation of Subacute Thyroiditis in an Elderly Patient: A Case Report with Literature Review.

An 80-year-old Caucasian male presented with fever of 3-week duration. Outpatient workup for infectious etiologies was negative and due to persistent fever, he was hospitalized for further evaluation of fever of unknown origin (FUO). Physical examination and laboratory studies remained unremarkable; however a follow-up CT scan of chest, abdomen, and pelvis with contrast done to rule out malignancy as an underlying cause of FUO revealed heterogeneous thyroid gland with surrounding hazy changes suggestive of thyroiditis. Thyroid function tests confirmed the diagnosis of subacute thyroiditis. The patient was started on prednisone with good response in his symptoms and was eventually discharged to home. The importance of our case lies in the fact that diagnosing subacute thyroiditis in the absence of classical symptoms of neck pain can be challenging and a physician should have a very high index of suspicion especially in an elderly patient where FUO can be the sole presentation.

STEMI in a Young Male after Use of Synephrine-Containing Dietary Supplement.

A twenty-two-year-old male with no significant past medical history who presented with chest pain was found to have ST-segment elevation in leads II, III, aVF, and V4-V6. On subsequent EKGs, patient had new ST-segment elevations in anterolateral leads with dynamic changes. Cardiac catheterization showed acute dissection with thrombosis of the distal left main coronary artery leading into the ostial left anterior descending artery. The patient had no cardiac risk factors including hypertension, hyperlipidemia, diabetes, or family history of early cardiac disease. On further inquiry, the patient was found to be on two separate performance-enhancing supplements which contained synephrine, a sympathomimetic chemical which was later attributed as the cause of his acute coronary syndrome. Synephrine acts on alpha-1 adrenergic receptors causing peripheral and coronary vasoconstriction, hypertension, and hyperglycemia. Increased hemodynamic stress on the coronary arteries can lead to fatal dissections. Ours is an atypical case of synephrine-induced nonatherosclerotic spontaneous coronary artery dissection which helps caution the physicians about the importance of dietary supplement use in the history and possible side effects of such performance-enhancing additives.

Adipose HIF-1α causes obesity by suppressing brown adipose tissue thermogenesis.

Hypoxia-inducible factor-1α (HIF-1α) in adipose tissue is known to promote obesity. We hypothesized that HIF-1α interferes with brown fat thermogenesis, thus decreasing energy expenditure. To test this hypothesis, we compared transgenic mice constitutively expressing HIF-1α in adipose tissues (HIF-1α++) at usual temperature (22 °C), where brown fat is somewhat active, or at thermoneutrality (30 °C), where brown fat is minimally active. HIF-1α++ mice or control litter mates were separated into room temperature (22 °C) or thermoneutrality (30 °C) groups. We assessed weight gain, food intake, calorimetry, activity, and oxygen consumption and transcriptional changes in isolated white and brown adipocytes. At 22 °C, HIF-1α++ mice exhibited accelerated weight gain, cold and glucose intolerance, hyperglycemia, and decreased energy expenditure without changes in food intake or activity. These changes were absent or minimal at thermoneutrality. In brown adipocytes of HIF-1α++ mice, oxygen consumption decreased ~50 % in association with reduced mitochondrial content, uncoupling protein 2, and peroxisome proliferator-activated receptor gamma coactivator 1 (PGC-1α). In conclusion, adipose HIF-1α overexpression inhibits thermogenesis and cellular respiration in brown adipose tissue, promoting obesity in the setting of reduced ambient temperature. KEY MESSAGE: Constitutive HIF-1α activation in adipose tissue promotes weight gain in mice. The weight gain is associated with reduced brown adipose tissue function and oxygen consumption. Reduced oxygen consumption may be mediated by reductions in mitochondria.

Silent myocardial infarction secondary to cardiac autonomic neuropathy in a patient with rheumatoid arthritis.

An 83-year-old female patient with rheumatoid arthritis and hypertension presented to the emergency department with fever and chills of 1 day duration. On examination, temperature was 100.9 F, heart rate 111/min and she had orthostatic hypotension. Laboratory tests showed elevated blood urea nitrogen and white cell count. The patient underwent treatment for symptomatic urinary tract infection and while her fever and leucocytosis resolved, tachycardia persisted. An EKG done showed T inversions in leads II, III, arteriovenous fistula, V2 and V3. Troponin-I was elevated. Nuclear stress test revealed apical wall motion abnormality confirming myocardial infarction. Ewing's tests were carried out at bedside and these diagnosed severe autonomic neuropathy. Rheumatoid arthritis can cause cardiac autonomic neuropathy from chronic inflammation. This case entails the importance of assessing and detecting cardiac autonomic neuropathy in chronic inflammatory conditions, and the need to be cautious of acute coronary events in these patients, even for minimal or no symptoms.

Effect of Acute Intermittent CPAP Depressurization during Sleep in Obese Patients.

BACKGROUND: Obstructive Sleep Apnea (OSA) describes intermittent collapse of the airway during sleep, for which continuous positive airway pressure (CPAP) is often prescribed for treatment. Prior studies suggest that discontinuation of CPAP leads to a gradual, rather than immediate return of baseline severity of OSA. The objective of this study was to determine the extent of OSA recurrence during short intervals of CPAP depressurization during sleep. METHODS: Nine obese (BMI = 40.4 ± 3.5) subjects with severe OSA (AHI = 88.9 ± 6.8) adherent to CPAP were studied during one night in the sleep laboratory. Nasal CPAP was delivered at therapeutic (11.1 ± 0.6 cm H20) or atmospheric pressure, in alternating fashion for 1-hour periods during the night. We compared sleep architecture and metrics of OSA during CPAP-on and CPAP-off periods. RESULTS: 8/9 subjects tolerated CPAP withdrawal. The average AHI during CPAP-on and CPAP-off periods was 3.6 ± 0.6 and 15.8 ± 3.6 respectively (p<0.05). The average 3% ODI during CPAP-on and CPAP-off was 4.7 ± 2 and 20.4 ± 4.7 respectively (p<0.05). CPAP depressurization also induced more awake (p<0.05) and stage N1 (p<0.01) sleep, and less stage REM (p<0.05) with a trend towards decreased stage N3 (p = 0.064). CONCLUSION: Acute intermittent depressurization of CPAP during sleep led to deterioration of sleep architecture but only partial re-emergence of OSA. These observations suggest carryover effects of CPAP.