Systemic Illness Research Articles

Characteristics and therapeutic outcomes of subcutaneous panniculitis-like T-cell lymphoma with and without germline HAVCR2 mutations in Thai children and adolescents.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin lymphoma associated with hemophagocytic lymphohistiocytosis (HLH)/HLH-like systemic illnesses and germline HAVCR2 mutations. Although previous studies suggested successful treatment of SPTCL with immunosuppressive therapy (IST) without chemotherapy, IST data in pediatric SPTCL remain scarce. To explore characteristics and therapeutic outcomes, comparing IST-based and chemotherapy-based regimens in pediatric SPTCL, in this retrospective cohort study, patients with SPTCL diagnosed at age ≤20 years during 2007-2023 were enrolled from 6 hematology/oncology centers in Thailand. HAVCR2 exon 2 sequencing was performed using DNA extracted from peripheral blood or bone marrow. Presence of HLH/HLH-like systemic illnesses, treatment outcomes, and adverse events (AEs) were reviewed and analyzed. Of 22 patients with SPTCL (median age at diagnosis, 11.5 years [range, 6.0-19.0]; 63.6% males), 86.4% harbored germline HAVCR2 mutation, either homozygous (77.3%) or heterozygous (9.1%) p.Y82C variant, while 68.2% developed HLH/HLH-like systemic illnesses. Overall, 36.4% received IST as first-line treatment. Durable complete remission (CR) was achieved in 71.4% and 50.0% after first-line chemotherapy and IST, respectively (P=0.45); however, chemotherapy tended to increase any AEs compared to IST (57.1% vs. 12.5%; P=0.07). The most common AEs were hypertension (27.3%), febrile neutropenia (18.2%), and fungal infection (13.6%). Among the relapsed cases, 71.4% could reach CR after subsequent-line therapy. Fatality (4.5%) only occurred in the chemotherapy group. Pediatric SPTCL in Thailand frequently involves germline HAVCR2 mutations and/or HLH/HLH-like systemic illnesses. With comparable response and modest therapy-related toxicity, IST-based regimens may alternatively be considered as first-line treatment for pediatric SPTCL.

Chronic Abdominal Pain in Patients with Inflammatory Bowel Disease in Remission: A Continuing Challenge for Clinicians.

Inflammatory bowel disease (IBD) is a chronic condition that includes ulcerative colitis and Crohn's disease. It is characterized by a relapsing and remitting pattern that negatively impacts quality of life (QoL). Current goals of treatment involve symptomatic, biochemical, and endoscopic remission in a treat-to-target approach. Despite effective treatment and remission of IBD, many patients report frequent and isolated abdominal pain. A wide range of etiologies exist, including surgery-related, infections, pelvic conditions, immune-related, and systemic illnesses. Disorders of the gut-brain interaction (DGBI), frequently characterized by abdominal pain, are increasingly recognized in IBD patients, including those with quiescent disease. Various mechanisms are involved and numerous non-pharmacologic and pharmacologic therapies have been proposed. Hereby, we outline the pertinent findings of the literature on management of chronic abdominal pain, focusing on quiescent IBD.

HPB SO49 - EUS guided biliary drainage prior to pancreaticoduodenectomy - The Leeds experience

Abstract Background Biliary obstruction is a common manifestation of biliary tract cancer. Decompression is indicated if patients develop systemic illness due to the obstruction or to facilitate chemotherapy. Stent insertion via ERCP is the current first line decompressive technique, although in certain situations such as duodenal/ampullary obstruction, is not always feasible. When ERCP has failed, our centre has implemented using lumen apposing metallic stents (LAMS) delivered by the HOT AXIOS™ system to create a draining choledochoduodenostomy. Our aim is to report on our experience of carrying out pancreaticoduodenectomy after biliary drainage has been achieved with this technique. Method All patients who underwent biliary drainage with a LAMS and then surgical resection of a biliary tract cancer were identified from a prospective database. Both procedures were carried out at St James’s Hospital, Leeds, between June 2018 and May 2024 by expert gastroenterology and pancreatic surgical consultants. Procedural and inpatient notes were retrospectively reviewed. The primary outcomes were the assessment of technical feasibility of pancreaticoduodenectomy and of perioperative complications, classified by the Clavien-Dindo (CD) system. Secondary outcomes were the safe and clinically effective (defined as >50% reduction of serum bilirubin within 7 days) application of LAMS. Results Thirteen patients were identified with a median age of 69 (51-81), twelve requiring drainage due to jaundice and one due to cholangitis. Duration to resection was median 68 days (12-377). Surgical resection was successful in all thirteen patients, in seven cases the surgeons commented on fibrosis due to the stent, with one qualifying that this was no more than a conventional biliary stent. Postoperatively five patients had no complications, one CD grade 1, five CD grade 2 and one CD grade 5. There were no significant post endoscopic complications and 10 patients demonstrated clinical effectiveness of the LAMS. Conclusion ERCP is the current first line method of drainage in malignant biliary obstruction. Technical success is reliant on unimpeded access to the bile duct. The HOT AXIOSÔ system circumvents this reliance by delivering a LAMS in a single stage forming a draining choledochoduodenostomy. Our centre’s experience suggests that it is a safe and effective method of biliary drainage which does not impede surgical resection. Additionally, it is not associated with the risk of post procedural pancreatitis. Our experience justifies further large-scale studies into this technique with consideration for its use not only when ERCP is not possible.

Impacts of microplastics on ecosystem services and their microbial degradation: a systematic review of the recent state of the art and future prospects.

Microplastics are tiny plastic particles with a usual diameter ranging from ~ 1 μ to 5µm. Recently, microplastic pollution has raised the attention of the worldwide environmental and human concerns. In human beings, digestive system illness, respiratory system disorders, sleep disturbances, obesity, diabetes, and even cancer have been reported after microplastic exposure either through food, air, or skin. Similarly, microplastics are also having negative impacts on the plant health, soil microorganisms, aquatic lives, and other animals. Policies and initiatives have already been in the pipeline to address this problem to deal with microplastic pollution. However, many obstacles are also being observed such as lack of knowledge, lack of research, and also absence of regulatory frameworks. This article has covered the distribution of microplastics in water, soil, food and air. Application of multimodel strategies including fewer plastic item consumption, developing low-cost novel technologies using microorganisms, biofilm, and genetic modified microorganisms has been used to reduce microplastics from the environment. Researchers, academician, policy-makers, and environmentalists should work jointly to cope up with microplastic contamination and their effect on the ecosystem as a whole which can be reduced in the coming years and also to make earth clean.

Coronary Artery Vasculitis and Encasement: Multimodality Imaging Findings and Mimics.

Coronary artery vasculitis (CAV) and coronary artery encasement are rarely diagnosed conditions that are important diagnostic considerations, particularly in patients with acute coronary syndrome without traditional cardiovascular risk factors or systemic illness. Vasculitis refers to inflammation of the blood vessel walls, which can be primary or secondary. This process should be distinguished from neoplastic involvement of the coronary arteries, termed coronary artery encasement. Prospective diagnosis of these diseases is challenging, often requiring multidisciplinary workup with careful attention to clinical presentation and multiorgan findings. While CAV and coronary artery encasement can be indistinguishable at coronary CT angiography, certain imaging features help order the differential diagnosis. CAV should be considered when there is smooth wall thickening that is circumferential and/or continuous. A diagnosis of coronary artery encasement is favored when there is irregular or nodular wall thickening that is eccentric to the vessel lumen. Epicardial fat stranding may also appear more extensive compared with CAV. Potential mimics of CAV include atherosclerosis, acute plaque rupture, coronary artery aneurysm, and spontaneous coronary artery dissection. Detection and diagnosis of CAV may help avoid complications related to accelerated atherosclerosis and infarction. Radiologists should be familiar with the range of pathologic conditions that can affect the coronary arteries beyond atherosclerosis as they may be the first to raise such diagnostic possibilities, guiding next steps in patient workup and management. ©RSNA, 2024 Supplemental material is available for this article.

Gastrointestinal Issues in Depression, Anxiety, and Neurodegenerative Diseases: A Systematic Review on Pathways and Clinical Targets Implications.

Multiple illnesses commonly involve both the Central Nervous System (CNS) and the Gastrointestinal Tract (GI) simultaneously. Consistent evidence suggests that neurological disorders impair GI tract function and worsen the symptomatology and pathophysiology of digestive disorders. On the other hand, it has been proposed that early functional changes in the GI tract contribute to the genesis of several CNS illnesses. Additionally, the role played by the gut in these diseases can be seen as a paradigm for how the gut and the brain interact. We mentioned significant GI symptoms and discussed how the GI tract affects central nervous system illnesses, including depression, anxiety, Alzheimer's disease, and Parkinson's disease in this study. We also explored potential pathophysiological underpinnings and novel targets for the creation of future therapies targeted at gut-brain connections. In this situation, modulating the gut microbiota through the administration of fecal microbiota transplants or probiotics may represent a new therapeutic option for this population, not only to treat GI problems but also behavioral problems, given the role that dysbiosis and leaky gut play in many neurological disorders. Accurate diagnosis and treatment of co-existing illnesses also require coordination between psychiatrists, neurologists, gastroenterologists, and other specialties, as well as a thorough history and thorough physical examination.

Brucella canis and Chlamydia spp.: insights into canine infections, diagnostics, and potential tick-borne transmission

Brucella and Chlamydia spp. are zoonotic pathogens with significant implications for both human and animal health. Brucella species, which is responsible for brucellosis, manifest in a range of clinical symptoms and present substantial zoonotic risks. Though less commonly reported, canine infections with Brucella raise concerns about the potential for transmission to humans and highlight the need for continued vigilance in veterinary settings. Chlamydia spp. is notable for its ability to cause diverse diseases in animals, from mild infections to severe systemic illnesses. In dogs, Chlamydia infections can lead to symptoms such as conjunctivitis, respiratory disorders, and reproductive issues, including infertility and abortion. The zoonotic potential of Chlamydia, particularly Chlamydia felis, emphasises the need for thorough monitoring and control measures in both domestic animals and humans. Ticks play a significant role in the transmission of these pathogens. Research has identified Chlamydia spp. in ticks; however, the exact epidemiological implications remain unclear. Similarly, Brucella has been detected in ticks, but conclusive evidence of tick-borne transmission to humans or between animals is still lacking. Improved diagnostic tools and control strategies are essential for managing the risks associated with Brucella and Chlamydia infections in both animals and humans, with a particular focus on the role of ticks as potential vectors.

The short-term prognosis of salvianolate use during hospitalization in patients with acute myocardial infarction

Abstract Background Salvianolate, derived from Salvia miltiorrhiza Bunge, is a traditional Chinese medicine known for its potential to promote blood circulation and eliminate blood stasis, often prescribed for cardiovascular system illnesses. This study aims to explore the impact of salvianolate treatment on the prognosis of patients with myocardial infarction during hospitalization. Methods This study included an acute myocardial infarction cohort from 71 secondary or tertiary hospitals between January 1, 2010 and June 30, 2023. Patients were divided into NSTEMI and STEMI groups. Clinical outcomes included cardiac death and major adverse cardiovascular and cerebrovascular events (MACCE, composite of cardiac death, myocardial infarction, ischemic stroke) within 30 days. Cox multivariable regression analyzed the association between salvianolate use and outcomes in STEMI/NSTEMI patients. Propensity score matching (PSM) adjusted for demographic and clinical differences between salvianolate and non-salvianolate groups. The matching factors including sex, age, Killip class, previous diseases (diabetes, hypertension, stroke, and renal insufficiency), previous percutaneous coronary intervention (PCI), in-hospital aspirin, clopidogrel, ticagrelor, lipid-lowering agents, nitrates, PCI, and thrombolytic therapy. Results A total of 76,157 STEMI patients and 74,373 NSTEMI patients were assessed. Among the STEMI group, 7,636 patients used salvianolate and 68,521 did not. Multivariate Cox regression analysis revealed that salvianolate use during hospitalization reduced cardiac death (aHR = 0.707, 95%CI = 0.623-0.803, P < 0.001) and MACCE (aHR = 0.730, 95%CI = 0.651-0.820, P < 0.001) at 30 days. These findings remained consistent in the matched population (n = 15,264) (cardiac death: aHR = 0.669, 95%CI = 0.569-0.786, P < 0.001; MACCE: aHR = 0.679, 95%CI = 0.586-0.787, P < 0.001). In the NSTEMI group, 6,461 patients used salvianolate, while67,912 did not. After matching, salvianolate use during hospitalization showed no significant difference in cardiac death and MACCE compared to not use. Conclusions Patients with STEMI treated with salvianolate exhibited improved short-term cardiovascular and cerebrovascular outcomes, while NSTEMI patients did not experience significant short-term prognosis enhancement.

33 Use of CHU Ste. Justine online resources prior to visiting the Emergency Department for less-urgent, acute febrile illness

Abstract Background Over the past 20 years, visits to paediatric emergency departments (EDs) across Canada have steadily increased, with a higher proportion of presentations for lower acuity symptoms. Many paediatric institutions’ websites now provide information and recommendations for caregivers on common illnesses and reasons for seeing healthcare providers or going to the ED. Increasing the use of internet resources may improve health literacy and hopefully decrease ED use for lower acuity presentations like uncomplicated viral illness. Objectives The objective of this study was to assess whether caregivers presenting with children with lower acuity at triage were aware of and using the online resources provided by our paediatric institution to help with at-home management of febrile illnesses and to guide their decision to present to the ED. Design/Methods This was a cross-sectional study in a tertiary care university-affiliated paediatric ED in an urban setting. Participants were a convenience sample of caregivers of children 6 months to 17 years old with a fever 72 hours or less and triaged low acuity (Canadian Triage and Acuity Scale levels 3 to 5). Caregivers of children with underlying significant systemic illness were excluded. Consenting participants completed two questionnaires. The first questionnaire included demographic data, information on baseline internet utilisation, use of general online health information and our institutions’ website specifically, and whether use of those resources influenced their decision to present to the ED. After browsing dedicated web materials developed by our institution (“Before going to the emergency room” and “Fever”), questionnaire 2 asked for feedback on the website. Results From April to July 2023, 100 caregivers participated in the study (response rates questionnaires 1 and 2: 80% and 70%, respectively) (Table 1). Half (49%) of caregivers researched their child’s symptoms prior to coming to the ED (Table 2). Approximately one third (39%) of respondents were aware that our institution provided online information for caregivers about common symptoms and when to present to the ED. Half (51%) of caregivers who completed questionnaire 2 said they would not have come to the ED if they had read these resources prior (Table 3). Many (66%) respondents reported they would be “likely/very likely” to look up their child’s symptoms on the institutional website before going to the ED in the future. Conclusion Half of caregivers presenting to the ED for their child’s lower-acuity febrile illness conducted research on their child’s symptoms prior to visiting the ED and most were influenced in their decision to come to ED by what they found. Most caregivers were not aware that our institution had online resources available. Promotion of the institutional resources available to parents may empower parents to manage common symptoms at home and prevent ED visits for lower-acuity illness.

HIV testing services and HIV self-testing programming within emergency care in Kenya: a qualitative study of healthcare personnel to inform enhanced service delivery approaches

ABSTRACT In Kenya, persons insufficiently engaged in HIV Testing Services (HTS) are often treated in emergency departments (ED). There are limited data from healthcare workers on ED-HTS. A qualitative study was completed to understand challenges and facilitators for ED-HTS and HIV self-testing (HIVST). Data were collected via six focus groups of healthcare workers. Data were inductively analyzed and mapped to the Capability–Opportunity–Motivation Behavioral Model. Focus groups were completed with 49 healthcare workers: 18 nurses, 15 HIV counselors, 10 physicians and 6 administrators. HTS challenges included staff burdens, resources access, deficiencies in systems integration and illness severity. HTS facilitators included education of healthcare workers and patients, services coordination, and specific follow-up processes. HIVST challenges included accuracy concerns, follow-up barriers and psychosocial risks. HIVST facilitators were patient autonomy and confidentiality, resource utilization and ability to reach higher-risk persons. Mapping to the Capability–Opportunity–Motivation Behavioral Model interventions within the domains of knowledge, decision processes, environmental aspects, social influences and professional identities could support enhanced ED-HTS with integrated HIVST delivery. This study provided insights into challenges and facilitators on ED-HTS and identifies pragmatic approaches to improve healthcare workers’ behaviors and abilities to provide services to persons already in contact with healthcare.

Exceptional Rarity Unveiled: Isolated Fungal Osteomyelitis of Great Toe by Fusarium Solani-Unprecedented Presentation and Exquisite Management Insights

Fungal osteomyelitis is a rare but significant condition, often associated with immunocompromised states or severe systemic illnesses. This condition involves fungal pathogens, primarily Aspergillus, Candida, and Mucor species. Among these, Aspergillus is the most commonly implicated, especially in cases of vertebral osteomyelitis, although fungal infections involving bones like the distal phalanx of the toe are rare. In particular, Fusarium solani, though less frequently reported, has gained recognition for its role in causing localized osteomyelitis in both immunocompetent and immunocompromised patients. A case involving a 57-year-old male with no significant history of immunosuppression is presented. The patient developed localized pain and swelling in the left toe following a minor traumatic injury. Initial radiographic imaging revealed bone erosion, leading to a diagnosis of osteomyelitis. Cultures from bone biopsy identified Fusarium solani as the causative pathogen. This is noteworthy as the fungus, though commonly found in soil, water, and plants, rarely infects bone structures. The patient was treated with a combination of antifungal therapy, including voriconazole and surgical debridement. The rarity of Fusarium solani osteomyelitis and its unusual presentation in an immunocompetent host adds to the complexity of diagnosing fungal infections in osteomyelitis. Treatment typically involves a multidisciplinary approach that combines both medical and surgical interventions. The case highlights the importance of considering fungal pathogens, even in unusual clinical settings, especially when initial treatments fail to provide resolution. Early diagnosis and targeted therapy can improve outcomes, particularly in cases involving uncommon fungal pathogens such as Fusarium solani. This report emphasizes the need for awareness of fungal osteomyelitis in immunocompetent individuals and underscores the critical role of fungal cultures and imaging studies in guiding management.

Functional Design Of Peroral Delivery Systems Based On Polymethylsesquoxane Hydrogels For The Therapy Of Iron Deficiency Anemia

Anemia is a prevalent circulatory system illness that is severely harmful to patients. The development of novel oral delivery systems for iron compounds with enhanced biopharmaceutical properties is vital considering the severe side effects associated with oral medication use. We believe incorporating iron compounds to polymethylsilsesquioxane hydrogels is a promising approach. According to previously published materials, such a system should have great biocompatibility and a capacity for iron compounds, and it may be able to release contents into the intestine. This study investigated polymethysilsesquioxane hydrogels with varying silicate unit concentrations. Potential iron-containing medicines were iron(III) chloride (FeCl3∙6H2O)) and iron(II) D-gluconate. All hydrogels were found to have nearly 100% sorption activity for a saturated solution of FeCl3∙6H2O (0.27 M) during the experiment, but only around 30% sorption capacity was found for a saturated solution of D-gluconate (0.24 M). A specific field of study was the distribution of iron atoms within hydrogels. It has been established that the largest regions devoid of iron atoms are observed in a hydrogel with a maximum quantity of inorganic units. The outcomes provide opportunities for the precise engineering of polymer matrix structures for iron compound delivery.

Physical health challenges faced by elders with severe mental illness: population-based retrospective cohort study.

Severe mental illness (SMI), which includes schizophrenia, schizoaffective disorder and bipolar disorder, has profound health impacts, even in the elderly. To evaluate relative risk of hospital admission and length of hospital stay for physical illness in elders with SMI. To construct a population-based retrospective cohort observed from April 2007 to March 2016, data from a case registry with full but de-identified electronic health records were retrieved for patients of the South London and Maudsley NHS Foundation Trust, the single secondary mental healthcare service provider in south-east London. We compared participants with SMI aged >60 years old with the general population of the same age and residing in the same areas through data linkage by age-, sex- and fiscal-year-standardised admission ratios (SARs) for primary diagnoses at hospital discharge. Furthermore, we compared the duration of hospital stay with an age-, sex- and cause-of-admission-matched random group by linear regression for major causes of admission. In total, records for 4175 older people with SMI were obtained, relating to 10 342 admission episodes, showing an overall SAR for all physical illnesses of 5.15 (95% CI: 5.05, 5.25). Among the top causes of admission, SARs ranged from 3.87 for circulatory system disorders (ICD-10 codes: I00-I99) to 6.99 for genitourinary system or urinary conditions (N00-N39). Specifically, the diagnostic group of 'symptoms, signs and findings, not elsewhere classified' (R00-R99) had an elevated SAR of 6.56 (95% CI: 6.22, 6.90). Elders with SMI also had significantly longer hospital stays than their counterparts in the general population, especially for digestive system illnesses (K00-K93), after adjusting for confounding. Poorer overall physical health and specific patterns were identified in elders with SMI.

Urticarial vasculitis

Purpose of review Urticarial vasculitis is a rare condition manifesting with a variety of clinical presentations ranging from skin limited lesions to life-threatening systemic illnesses. This review aims to highlight the recent findings on the etiology, diagnostic modalities, and therapeutic strategies and course of urticarial vasculitis. Recent findings In addition to well established triggers, urticarial vasculitis (UV) cases associated with severe acute respiratory syndrome coronavirus 2 (SARS-Cov2) disease and COVID-19 vaccines, vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome, and adenosine deaminase (ADA) deficiency have been reported. A clinical-dermoscopic model for differentiating urticarial vasculitis has been developed with purpuric patches and globules favoring UV diagnosis and thus diminishing the need for histopathology. The efficacy of treatment modalities has been reviewed, and antihistamines, systemic corticosteroids, omalizumab, cyclophosphamide, tocilizumab, anti-interleukin (IL)-1 agents, and rituximab were shown to have the highest success rates. Regarding the durability of remission, rituximab, dapsone, and MMF were related to long-lasting treatment free responses. The course of hypocomplementemic urticarial vasculitis was investigated in an epidemiological study, revealing 5- and 10-year survival rates of 92% and 83%, respectively. Chronic obstructive pulmonary disease, septicemia, and end-stage renal disease were identified as causes of mortality. Summary With the aid of dermoscopy, a noninvasive tool, differentiation from chronic spontaneous urticaria can be made, and the need for histopathological examination can be diminished. Although clear definitions and consensus criteria for performing disease severity are lacking, careful screening is needed to tailor the treatment on an individual basis. Emerging infections like SARS-CoV 2, vaccines, and autoinflammatory disorders like VEXAS syndrome and ADA deficiency are new associations. The optimal use of well established agents like systemic corticosteroids and immunomodulators are mainstay treatment modalities, whereas IL-1 inhibitors, omalizumab, rituximab and Janus Kinase inhibitors may represent viable alternatives in selected cases.

Sterile pyogranuloma/granuloma syndrome in two dogs presenting with extensive ulceration and systemic illness

AbstractThis report describes two cases of canine sterile pyogranuloma/granuloma syndrome presenting with fulminant extensive ulcerations, pyrexia and systemic malaise; one case with concurrent unilateral uveitis. This presentation contrasts to typically reported cases, and due to the severity of signs, urgent distinction from aggressive infectious causes was required. Both cases required extended immunosuppressive treatments for resolution.

Dental Decay Dilemma: Addressing Caries in Pakistan's Population

Maintaining good oral health is essential for general wellbeing since it affects a person's comfort level when speaking, eating, and interacting with others. It affects systemic health as well as the condition of the teeth, gums, and oral tissues. Maintaining good oral hygiene reduces the risk of systemic illnesses like diabetes and cardiovascular disease in addition to common dental problems like cavities and gum disease.

6664 A Case of Undistinguished Congenital Adrenal Hyperplasiain a Patient with Hypertensive Emergency and Hypokalemia

Abstract Disclosure: V.L. Del Signore: None. S.T. Kaufman: None. CAH is a group of autosomal recessive disorders resulting in alterations of adrenal steroid biosynthesis. With 95% of CAH cases due to 21-hydroxylase deficiency; other causes are rare. The metabolic, phenotypic, and physiologic derangements are specific to the altered enzymatic pathways, most of which are life threatening. Identifying the enzymatic defect is crucial to treat the patient. We present a case of undifferentiated CAH in an adult resulting in significant systemic illness. A 26-year-old well virilized male presented with chief complaint of shortness of breath, lower extremity swelling, and abdominal distension for 3 weeks. He was found to have acute decompensated heart failure with LVEF 20-25%, NSTEMI, atrial fibrillation, hypertensive emergency with BP of 224/102, and potassium (K+) of 2.7 (3.5-5mmol/L). PMH included hypertension and CAH diagnosed at birth. Patient was unsure of his CAH defect. Pediatric endocrinology prescribed glucocorticoids until age 17 at which point he was told steroids were no longer required. He had been lost to follow up for 9 years. Pediatric records were unattainable. Patient remained hypokalemic and hypertensive despite aggressive K+ repletion and multiple IV antihypertensives. Endocrine work up revealed renin 0.19 (0.25- 5.82ng/mL/hr), aldosterone 4 (< or = 21ng/dL), DHEAs 97 (74-617ug/dL), FSH <0.3 (1.4 - 18.1 m[IU]/mL), LH <0.3 (1.5 - 9.3 m[IU]/mL), total testosterone 306 (250-1,100 ng/dL), SHBG 32 (10-50nmol/L), progesterone 7 (0.2-1.4 ng/mL), ACTH 345 (6-50 pg/mL), AM cortisol 4.4 (5-23 ug/dL), 17 hydroxyprogesterone 311 (32-307 ng/dL), androstenedione 1,926 (50-220 ng/dL), 11-deoxycortisol >10,000 (< or = 119 ng/dL). Cosyntropin stimulation test: 30-minute cortisol 11.1 (>18 ug/dL) and 60-minute cortisol 10.4 (>18 ug/dL). Based on clinical and lab findings a diagnosis of 11-beta hydroxylase deficiency was made. Spironolactone was started to counteract the excessive mineralocorticoid effects of deoxycorticosterone and hydrocortisone to decrease ACTH production. With this treatment, patient’s potassium and blood pressure gradually improved. IV antihypertensives were weaned as he transitioned to oral agents. This case demonstrates the importance of understanding and identifying the underlying pathophysiology of CAH to treat patients appropriately. This patient had a mineralocorticoid excess state, but adequate cortisol precursors to prevent adrenal crisis. He requires glucocorticoid therapy to prevent excessive ACTH from driving their BP and metabolic derangements. With appropriate diagnosis of 11-beta hydroxylase deficiency, patient’s excess risk of morbidity and mortality due to the downstream effects of uncontrolled CAH can be minimized. Presentation: 6/3/2024

Relation between Urine Cytological Findings and Renal Function in Patients with Kidney Stones in Taif, Saudi Arabia

Background and Objectives: Urine serves as a vital diagnostic fluid, and urine cytology analysis plays a crucial role in identifying urinary system illnesses such as bladder cancer and kidney stones. The Paris System for Reporting Urinary Cytology establishes a uniform method for diagnosing urinary tract cancer. This study aimed to provide valuable insights that can inform diagnostic strategies related to kidney stones and ultimately improve patient outcomes via the early detection of the cellular changes associated with kidney stones and their relation to kidney function tests. Materials and Methods: A comparative study was conducted and comprised two groups: group 1, consisting of 50 patients diagnosed with kidney stones, and group 2, comprising 50 patients diagnosed with other kidney diseases. Renal function tests and urinalysis (via the PAP staining of urine cellular deposits to detect nuclear changes) were performed, and the results were analyzed. Results: There was a statistically significant increase in urinary red blood cells, white blood cells, and nuclear reactive atypical changes in urinary sediments of kidney stone patients compared to the patients without stones, while there was a decrease in the estimated glomerular filtration rate (eGFR). eGFR showed a 96.7% specificity in detecting cases with nuclear reactive atypia. Conclusions: eGFR emerges as a reliable diagnostic marker for the comprehensive assessment of kidney stones, particularly when associated with nuclear atypia. The significant correlation between the indicators of chronic kidney disease, such as decreased eGFR, and the presence of kidney stones emphasizes the urgent need for efficient diagnostic practices.

Feto-Maternal Complications of Anticoagulant Use Before and After Childbirth

Anticoagulants prevent blood clotting, but their use in pregnancy poses challenges due to bleeding risks, particularly during delivery and postpartum. Objectives: To evaluate the maternal and fetal complications associated with anticoagulant use before and after childbirth, considering the safety and effectiveness of low molecular weight heparin and Warfarin. Methods: A cohort study was conducted at the Tertiary Care Hospital of Sindh, from August 2022 to July 2023. 266 pregnant women were chosen via consecutive sampling. Pregnant women with a gestational age of > 12 weeks, aged 18-35 years probably taking anti-coagulation therapy during pregnancy and puerperium were included in the study. While women suffering from any major systemic illness and women taking any other potential teratogenic drugs were excluded. Results: Maternal complications occurred in 53% of participants, with postpartum hemorrhage being the most frequent (19.9%), significantly higher in the low molecular weight heparin group (p<0.05). Pre-eclampsia affected 10.5% of participants, predominantly in low molecular weight heparin users (p=0.028). Fetal complications were reported in 59.3% of cases, with intrauterine growth restriction (13.5%) and premature birth (18.0%) being the most common. Stillbirths were more frequent among Warfarin users. Neonatal intensive care admission was required for 11.3% of infants, with low molecular weight heparin exposure showing the highest incidence. Conclusions: It was concluded that low molecular weight heparin was with poor maternal outcomes such as postpartum hemorrhage, wound hematoma, deep vein thrombosis, and pre-eclampsia, while fetal complications included intrauterine growth restriction, stillbirth, premature birth and low birth weight.

Wrist fusion with ulnar translocation for aggressive giant cell tumour of distal end radius: Short-term functional and clinico-radiological outcomes

BackgroundThe distal end radius (DER) is the third most common site of a giant cell tumour (GCT) in bone. GCT is a locally aggressive benign tumour with metastatic potential. The main goals in the management of GCT of DER are the complete removal of the tumours, the prevention of recurrence, and the restoration of the functional wrist. PurposeThis case series reports the clinical and functional outcome of en bloc tumour resection, ulnar translocation and wrist arthrodesis in patients with high-grade GCT of DER. MethodsSixteen patients with Campanacci grade II & III GCT of distal end radius who underwent the procedure between 2009 and 2018 and had a follow-up of 2 years were included. Patients with distant metastasis, chronic severe systemic illness, operated previously, and follow-up dropouts were excluded from the study. The patient's demographic profile and tumour characteristics were collected at the initial visit. Grip strength, modified MAYO wrist score, and VAS score for wrist pain were collected preoperatively and at 1-year and 2-year follow-ups. ResultsThe study included predominantly females (13 vs 3). The mean age was 31.5 ± 8 years with a symptom duration of 6.5 ± 3.1 months (range 3–14). The mean resection length was 8.31 ± 1.5 cm (range 6–12). The mean Ulnoradial and ulnocarpal union time was 22.7 ± 8.0 and 17.5 ± 2.3 weeks, respectively. The mean modified Mayo Wrist score was 20.63 ± 9.4, 48.7 ± 5.6 and 60.6 ± 4.0 at preop, 1 year and 2 years follow-up. The mean MSTS score was 22.68 ± 1.8 (range 19–26). Two patients had ulnoradial nonunion. ConclusionWrist arthrodesis by ulnar translocation and plate fixation is a viable option in the management of GCT of distal end radius. The short-term clinical and functional outcomes are favourable for restoring adequate wrist function.