AVASCULAR NECROSIS OF THE FEMORAL HEAD – NOT TO BE OVERLOOKED SEQUELA AFTER COVID 19 INFECTION

BackgroundCOVID 19 infection could lead to different sequelae in survivors, known as post-COVID or long COVID 19 syndromes. Some of them are thought to be due to the thrombophylic changes observed in COVID 19 infection, but some are thought to be caused by the administrated (especially high dose) corticosteroid treatment. Avascular necrosis of the femoral head (AVNFH) is a multifactorial disease which leads to compromised vascular supply, ischemia and finally necrosis of the femoral head. As corticosteroids usage and thrombophylic states are among the main known risk factors for the development AVNFH [1], it could be presumed that the frequency of this disease will increase with the COVID 19 pandemic. The exact corticosteroid dose needed for the development of AVNFH is not clear, but it has been stated that a higher daily dose and a larger total cumulative dose increase substantially the risk for the development of osteonecrosis [2].ObjectivesTo describe in detail the characteristics of AVNFH diagnosed in patients after COVID 19 infection.MethodsThe study was done in a tertiary university rheumatological clinic. Data was extracted from the records of patients who have been referred to the clinic because of hip pain between June and December 2022. Inclusion criteria were: - a new onset of uni-or bilateral hip pain that started after a documented COVID 19 infection;and an MRI scan of the hip joints showing osteonecrosis of one or both femoral heads. Exclusion criteria were the presence of hip pain prior to the COVID 19 infection, anamnesis of traumatic injuries of the hips or pelvis, personal history of hypercoagulable states.ResultsNine patients (4 women and 5 men) with an average age 59.1 years (range 38-72) were included in the study. Four patients had been diagnosed with bilateral and five – with unilateral AVNFH, thus 13 hip joints were analysed in total (8 left and 5 right sided). The mean time lap between the COVID 19 infection and the start of the hip pain was 26.2 weeks (range 10-48 weeks). All patients had limited and painful movement in their symptomatic hip(s), especially internal rotation and four of the patients had also elevated CRP levels (mean 11.7 mg/L). The stage of the AVNFH was evaluated according to the Ficat-Arlet classification (0-IV stage). In four hips the AVNFH was stage I, five hips were classified as stage II and the remaining four joints - as stage III. All symptomatic hip joints exhibited effusion/synovitis on both ultrasound examination and the corresponding MRI scan. It should be noted that the presence of hip effusion was found to be related with a worse prognosis in AVNFH [1]. In three patients the amount of the effusion required arthrocentesis and fluid aspiration. The analysis of the joint fluid was consistent with a degenerative disease (i.e., low WBC count with predominant lymphocytes and no crystals). All patients included in our study had received corticosteroids during their COVID19 infection, while 6 of the patients had also been hospitalized due to more severe disease. According to the patients' documentation, the mean cumulative dose of the received corticosteroids was 936.2 mg prednisolone equivalent per patient (range 187-2272 mg).ConclusionAVNFH must not be overlooked in a new onset hip pain after COVID 19 infection. Our results show that corticosteroids administrated during the infection and the presence of hip joint effusion on ultrasound are especially suggestive for the development of osteonecrosis, as they were registered in all of our patients. The presence of these two factors necessitates patient referral for an MRI scan of the hips, in order that AVNFH be detected timely.References[1]Petek D, Hannouche D, Suva D. Osteonecrosis of the femoral head: pathophysiology and current concepts of treatment. EFORT Open Rev. 2019 Mar 15;4(3):85-97.[2]Kerachian MA, Séguin C, Harvey EJ. Glucocorticoids in osteonecrosis of the femoral head: a new understanding of the mechanisms of action. J Steroid Biochem Mol Biol. 2009 Apr;114(3-5):121-8.Acknowledgements:NIL.Disclosur of InterestsPLAMEN TODOROV Speakers bureau: speaker at national level for AbbVie, Novartis and UCB, Lily Mekenyan: None declared, Anastas Batalov Speakers bureau: Speaker at national level for AbbVie, Novartis, Pfizer, Stada, Elly Lilly.

EBV-related lymphoproliferative disease in Wiskott-Aldrich Syndrome patient

Case: Wiskott-Aldrich Syndrome (WAS) is a rare X-linked inborn error of immunity caused by mutations in the WAS gene. It is classically characterized by immunodeficiency, eczema, and micro-thrombocytopenia. It has been known since the 1960s that patients with WAS have an increased risk of lymphoproliferative disease though the exact incidence remains unknown in the American population. Limited case reports have discussed EBV-related lymphoproliferative disease in patients with WAS. We present a case of a 9-year-old boy with known WAS complicated by eczematous rash, thrombocytopenia, recurrent ear infections, and monoclonal gammopathy who was found to have submandibular EBV-associated lymphoid hyperplasia with associated lung and retroperitoneal lymphadenopathy. Family had been offered treatment with hematopoietic stem cell transplant but declined multiple times in the past. Earlier in the year, he presented with possible MIS-C with negative SARS-CoV-2 PCR. He presented to our hospital with mastoiditis and lymphadenopathy. Physical examination showed severe eczema on hands and tender right mastoid. Laboratory evaluation showed thrombocytopenia, elevated IgG of 6290, IgA of 744, IgE of 827, low IgM of 41, and 14% response to pneumococcal titers. He was empirically treated with intravenous antibiotics. ENT performed right postauricular incision and drainage and the culture grew Hemophilus influenza. Throughout his hospital stay, his submandibular lymphadenopathy became more prominent despite treatment. Core needle biopsy of right submandibular lymph node was suggestive of EBV-associated lymphoid hyperplasia. EBV PCR and antibodies were both positive. CT chest, abdomen, and pelvis revealed multifocal pulmonary lymphadenopathy and a diffuse, bilateral nodularity as well as retroperitoneal and mesenteric lymphadenopathy. He was given four doses of weekly Rituximab, which successfully decreased EBV viremia below linear detectability. Immunoglobulin replacement therapy (IgRT) was initiated. Bronchoalveolar lavage and lung biopsy were performed and are results are currently pending. Discussion(s): We present a case of a 9-year-old boy with known WAS awaiting transplant who was found to have submandibular EBV-associated lymphoid hyperplasia with associated lung and retroperitoneal lymphadenopathy. While lymphoproliferative disease is a known complication of WAS, EBV-related lymphoproliferative disease in WAS patients has only been reported as case reports and remains a rare but known complication of patient with WAS.Copyright © 2023 Elsevier Inc.

Disseminated Mucormycosis in a patient on long term immunosuppression: Severe fungal infections as multi-system disorders

Introduction: Mucormycosis is a rare, severe fungal infection with an incidence of 0.005 to 0.17 per million.1 but incidence has risen recently, particularly in the Asian subcontinent, due to use of immunosuppression for Covid19.2 Presentations can vary and are classified into: rhino-orbito-cerebral, pulmonary, cutaneous, disseminated, renal and gastrointestinal. Risk factors include diabetes, immunosuppression, iron overload, malnutrition, and prematurity.1,3 Although mucormycosis has an extremely high mortality rate and disseminated infection is usually fatal, treatment options exist if diagnosed early and surgical debridement may be curative. Objective(s): We present a case of mucormycois in a female patient in her 40s who was immunosuppressed with methotrexate for rheumatoid disease. This case is discussed to increase awareness of critical illness caused by opportunistic invasive fungal infections in immunosuppressed patients and promote timely identification and management. Method(s): We detail the clinical context and management of a patient with mucormycosis and discuss relevant literature. Result(s): A female patient in her 40s who had been experiencing upper respiratory tract symptoms for several weeks, including cough and brown sputum, was admitted with a presumptive diagnosis of methotrexate toxicity after a full blood count performed by the general practitioner demonstrated pancytopenia. Initially, National Early Warning System 2 score (NEWS2) was 2 but became intensely hypertensive during blood transfusion and then profoundly shocked with an escalating NEWS2. Broad-spectrum antibiotics and fluconazole were commenced for neutropenic sepsis and the patient was referred to critical care in multiple organ failure. Computerised tomography (CT) scan of the chest, abdomen and pelvis showed "left upper lobe consolidation, which with neutropenia might represent an angioinvasive aspergillosis". She had multiple areas of skin discolouration and desquamation. Haematology and Infectious Diseases opinions were sought, and a bone marrow biopsy was performed which showed severe toxic effects consistent with sepsis/life threatening infection. Progressive proptosis was noted, and CT scan of her head was requested. Sadly, she was never stable enough for CT transfer. Beta D Glucan and aspergillus antigen serology was negative. Broncho-alveolar lavage demonstrated Candida albicans and then, later, Rhizopus arrhizus was isolated and anti-fungal treatment changed to voriconazole and then amphotericin B. Upon reviewing the notes in light of the positive culture for Rhizopus, the patient had likely been exhibiting symptomatic Mucormycosis sinus infection for some time prior to this admission with disseminated infection. The patient's condition continued to deteriorate and she sadly died. Conclusion(s): * The Early Warning Score significantly underestimated how unwell the patient was upon arrival in ED, a systems-based assessment would have demonstrated that the patient had multiple system dysfunction and significant potential to deteriorate suddenly despite having stable observations * The methotrexate level has no clinical value in diagnosing or refuting a diagnosis of methotrexate toxicity * A full examination of the immunosuppressed patient including ENT is a necessity when searching for a source of infection * Invasive fungal infections can cause multi-system symptoms and atypical presentations * As a greater proportion of patients have received systemic immunosuppression for Covid-19, vigilance for more unusual pathogens, including Mucormycosis by clinicians is advised.

Case Report on a Rare Malignancy: Anorectal Melanoma

Introduction: Melanoma of the rectum is an extremely rare disease. The median survival rate is 2-5 years. Current treatment for this aggressive cancer is resection if possible and consider adjuvant or neoadjuvant radiotherapy;immunotherapy in nonresectable cases. Given the rapid spread of disease due to its submucosal growth and metastasis pattern, there is low success rates with treatments. Case Description/Methods: An 84-year-old male presented to the emergency department with an acute COVID-19 infection. The patient was also found to have gram-negative septicemia on blood cultures, so a CT abdomen/pelvis was performed (Figure 1a). The CT showed rectal wall thickening. A flexible sigmoidoscopy was planned for a future outpatient visit after recovering from his acute infection. The patient, however, developed an acute onset of dyspnea and had a high probability V/Q scan while in the hospital. He was started on anticoagulation, and shortly after starting therapy the patient developed bright red rectal bleeding. Due to the new onset of rectal bleeding it was decided to expedite the sigmoidoscopy. The sigmoidoscopy was performed in the hospital showing an ulcerated partially black pigmented non- obstructing medium-sized mass that was partially circumferential involving one-third of the lumen (Figure 1b). A biopsy of the lesion was taken using cold-forceps. The pathology stained positive for S100 consistent with melanoma. The diagnosis of anorectal melanoma was made, and colorectal surgery was consulted. The patient was deemed not to be a surgical candidate secondary to age and active COVID-19 infection. Oncology was consulted, and it was decided to start the patient on radiation and immunotherapy with a PD-1 inhibitor. Discussion(s): The symptoms of anorectal melanoma can be subtle and in this case report completely asymptomatic. Symptoms to be aware of are rectal bleeding and tenesmus. Diagnosing melanoma on sigmoidoscopy can be challenging as most tumors are not pigmented. Biopsies should be taken and sent for immunohistochemical staining for S100, if positive the patient should have a PET scan. Treatment choices for the tumor are based on staging. In a resectable tumor sphincter-saving local excision with radiotherapy to the site of the tumor and the pericolic and inguinal lymphatics is recommended. For unresectable tumors or tumors with distant metastasis, immunotherapy with PD-1 inhibitors (nivolumab and ipilimumab) is an emerging treatment choice.

Rare Case of Immune-Mediated Necrotizing Myopathy With Isolated Dysphagia as Presenting Symptom

Introduction: Immune mediated necrotizing myopathy (IMNM) is a rare, but progressive disease that accounts for about 19% of all inflammatory myopathies. Dysphagia occurs in 20-30% of IMNM patients. It often follows proximal muscle weakness and ensues in the later stages of the disease. We report a rare case of IMNM, presenting with dysphagia as the initial symptom, followed by proximal muscle weakness. Case Description/Methods: A 74-year-old male with a past medical history of coronary artery disease, hypertension, and hyperlipidemia presented to the ED with 2-3 weeks of intractable nausea, vomiting, and dysphagia for solids and liquids. Vital signs were stable, and initial labs displayed an AST of 188 U/L and ALT of 64 U/L with a normal bilirubin. Computed tomogram of the chest, abdomen, and pelvis were negative. An esophagram showed moderate to severe tertiary contraction, no mass or stricture, and a 13 mm barium tablet passed without difficulty. Esophagogastroduodenoscopy exhibited a spastic lower esophageal sphincter. Botox injections provided no significant relief. He then developed symmetrical proximal motor weakness and repeat labs demonstrated an elevated creatine kinase (CK) level of 6,357 U/L and aldolase of 43.4 U/L. Serology revealed positive PL-7 autoxantibodies, but negative JO-1, PL-12, KU, MI-2, EJ, SRP, anti-smooth muscle, and anti-mitochondrial antibodies. Muscle biopsy did not unveil endomysial inflammation or MHC-1 sarcolemmal upregulation. The diagnosis of IMNM was suspected. A percutaneous endoscopic gastrostomy feeding tube was placed as a mean of an alternative route of nutrition. He was started on steroids and recommended to follow up with outpatient rheumatology. He expired a month later after complications from an unrelated COVID-19 infection. Discussion(s): The typical presentation of IMNM includes painful proximal muscle weakness, elevated CK, presence of myositis-associated autoantibodies, and necrotic muscle fibers without mononuclear cell infiltrates on histology. Dysphagia occurs due to immune-mediated inflammation occurring in the skeletal muscle of the esophagus, resulting in incoordination of swallowing. Immunotherapy and intravenous immunoglobulin are often the mainstay of treatment. Our patient was unique in presentation with dysphagia as an initial presenting symptom of IMNM, as well as elevated enzymes from muscle breakdown. It is critical as clinicians to have a high degree of suspicion for IMNM due to the aggressive nature of the disease and refractoriness to treatment.

Acute Pancreatitis Following a Lower Extremity Angioplasty

Introduction: Acute pancreatitis affects a significant population globally. Usual etiologies are gallstones, alcohol, hypertriglyceridemia, medications;less frequent are trauma, hypercalcemia, infections, toxins, ischemia, anatomic anomalies, vasculitis, and idiopathic. Pancreatitis post coronary intervention is an uncommon cause with only 19 published cases in the last two decades. Being cognizant of this etiology is important given the increasing number of patients undergoing angiography. Case Description/Methods: An 81-year-old female with hypertension, diabetes, peripheral arterial disease, prior cholecystectomy underwent left lower extremity angioplasty at an outside center. Within a few hours, she started having severe epigastric pain radiating to her back, nausea, vomiting and loose bloody stool. She presented to the emergency department 24 hours after symptom onset. Epigastric tenderness was present on exam. Labs revealed leukocytosis (24,450/muL), elevated lipase (1410 U/L), elevated creatinine (1.3 mg/dL), lactate (3.1 mmol/L), calcium 9.4 mg/dL and triglycerides 161 mg/dL. Incidentally, found to be positive for COVID-19. Normal common bile duct diameter seen on sonogram. CT angiogram of the abdomen/pelvis showed acute pancreatitis, duodenal and central small bowel enteritis (Figure). She was not on any medications known to cause pancreatitis and denied alcohol use. Patient improved with analgesics and intravenous fluids. She had no recurrence of bloody stools and hemoglobin remained stable. On day 4, she was able to tolerate a regular diet, and leukocyte count and creatinine normalized. Patient did not have any COVID respiratory symptoms, and was discharged. Discussion(s): Given the temporal association to angioplasty and no other identifiable cause, acute pancreatitis was presumed to be due to the contrast used during angioplasty. Other possibilities included cholesterol embolism but no peripheral signs of cholesterol embolism were seen. Patient was an asymptomatic COVID-19 case. Although, there are case series of pancreatitis due to COVID, those were found in very sick symptomatic patients. On review of literature, cholesterol embolism was identified as a definite cause only on autopsy or laparotomy (Table). Other possible mechanisms are: high viscosity of the contrast media leading to ischemia and necrosis, contrast causing NF-kB activation followed by epithelial damage, and vasospasm. Pancreatitis after coronary angiography is rare, nonetheless, an important differential especially if there is a temporal relationship.

Bupivacaine-Induced Hepatotoxicity in a Healthy Patient Without Chronic Liver Disease After Shoulder Arthroscopy

Introduction: Bupivacaine is a local anesthetic which has been increasingly used in the post-operative state for pain control. Hepatotoxicity is a rare complication, and few cases are reported in patients with chronic liver disease. We present a case of acute liver injury from bupivacaine use in a healthy patient without prior history of liver disease. Case Description/Methods: A 68-year-old female with a past medical history of primary hypertension and recent nontraumatic complete tear of the right rotator cuff, presents to the hospital with fatigue, loss of appetite, and nausea. She recently underwent an arthroscopy of the right shoulder with repair of the rotator cuff two weeks prior. Her surgery was uncomplicated, and patient was started on bupivacaine ONQ pump infusion at 5 ml/hr for three days for post-operative pain. Further history reveals patient is non-alcoholic without prior liver disease, including cirrhosis. Review of systems is concerning for associated generalized abdominal discomfort. Physical exam demonstrated jaundice with scleral icterus with mild periumbilical tenderness to palpation without hepatosplenomegaly or ascites. Labs demonstrated elevated total bilirubin of 10.2 mg/dL with Alkaline phosphatase, ALT, and AST being 924 U/L, 429 U/L, and 279 U/L, respectively. Imaging studies including CT abdomen and pelvis with contrast, abdominal ultrasound, MRCP, and portal vein doppler were negative. Additional work up for underlying liver disease including acetaminophen and ethanol levels, SARS-CoV2, Hepatitis panel, EBV antigen, and urine toxicology were negative. It was determined patient had bupivacaine induced hepatotoxicity. Patient's health improved with conservative management and she was discharged with instructions for close monitoring of her LFTs. Discussion(s): Bupivacaine is an amino-amide anesthetic which binds to the intracellular portion of voltage-gated sodium channels and prevents depolarization of pain signals. It is metabolized by the liver and thus reports of hepatotoxicity, although rare, occur in patients with underlying liver pathology. Our patient became symptomatic with acute rise in LFTs. An extensive workup for other etiologies of acute liver toxicity was negative. Rapid vascular uptake of the drug is the most common reason for bupivacaine toxicity;and this remains a possibility for the mechanism of toxicity in our patient. A prior case report of bupivacaine hepatotoxicity demonstrated a cholestatic pattern, which is consistent with our findings.

Herpes Gastritis: A Rare Cause of Gastric Outlet Obstruction

Introduction: Gastrointestinal tract involvement from herpes simplex virus is commonly associated with esophagitis. However, herpes simplex infection of the stomach is very rare with only a handful of cases being reported in immunocompromised patients. We present a case of herpes gastritis causing gastric outlet obstruction in an otherwise healthy, immunocompetent individual. Case Description/Methods: A 37-year-old male with a recent past medical history of COVID-19 infection, presented to the hospital with intractable nausea, vomiting, bloating, and early satiety for two days. Upon evaluation, CBC and CMP were remarkable for a WBC of 12.5 k/mm3 and ALT and AST of 124 U/L and 129 U/L, respectively. Lipase was 373 U/L. A CT abdomen/pelvis w/contrast showed circumferential wall thickening with edematous changes in the antrum consistent with localized inflammatory response. There was suspicion for gastric lymphoma and patient was admitted for further workup. An EGD was performed which showed exudative esophagitis and antral wall edema with luminal narrowing of gastric antrum. Endoscopic ultrasound (EUS) showed a 2.5 x 3 cm antral wall lesion worrisome for linitis plastica. Esophageal biopsies showed focal cytologic changes consistent with herpes esophagitis. The FNA of the gastric antral wall showed multinucleation of the basal cell layer with classic ground glass nuclei, consistent with herpes infection. No dysplasia or malignancy was seen. Both HSV1 and HSV2 IgG were elevated. HSV IgM was normal. A HSV PCR was ordered but never resulted. Patient was started on Valacyclovir 1 g PO BID for 10 days. He underwent a follow-up EGD 3 months later which showed complete resolution of the gastric antral changes (Figure). Discussion(s): Herpes gastritis is extremely rare. Literature review has revealed only 3 case reports of herpes gastritis;and all involved immunocompromised patients. To the best of our knowledge, this is the first case of herpes gastritis in an immunocompetent patient. Our patient presented with symptoms of gastric outlet obstruction which was caused by local inflammation from herpes simplex. It is unclear if having a COVID 19 infection altered patient's immunity and lead to herpes gastritis. This may need further investigation. No established guideline exists for treatment duration. Our patient received 10-day course of Valacyclovir, and his symptoms improved. Furthermore, patient had complete resolution of the herpes infection on follow-up EGD, indicating adequate treatment response.

Anca-Associated but Not Vasculitis

Background/Aims A 72-year-old lady presented in primary care with complaints of generalised body aches, bilateral leg weakness and constitutional symptoms following a first dose of COVID-19 vaccine. Blood tests showed slightly raised inflammatory markers. She was initially diagnosed with polymyalgia rheumatica and was started on 40mg prednisolone with minimal improvement. Methods The examination in the rheumatology clinic was unremarkable. Investigations revealed raised white cell count, consistent with high dose steroid treatment, and elevated monocytes. There was mild improvement in inflammatory markers. The working diagnosis was of self-limiting viral illness. Further testing discovered strongly positive MPO ANCA (115 IU/ml), and the patient received three pulses of 500mg methylprednisolone for suspected vasculitis arranged by the medical team. There was no evidence of renal involvement. The diagnosis made at this point was autoimmune inflammatory disorder with unclear aetiology. At the subsequent clinic visit she reported mild shortness of breath, but no other symptoms suggestive of either vasculitis or connective tissue disease. Repeat ANCA showed significant reduction in MPO titre following pulse steroid treatment. CT of chest, abdomen and pelvis demonstrated a localised lobular/ nodular deformity of the liver. Viral hepatitis screen was negative. CA19-9 was raised at 100 U/ml. Liver biopsy was reported as poorly differentiated carcinoma without specific localising immunohistochemical features. Results The patient underwent hemi-hepatectomy for histologically confirmed pT2pNXM0R0 liver cholangiocarcinoma in a tertiary centre followed by adjuvant chemotherapy with capecitabine. With treatment, her MPO ANCA and CA19-9 levels declined. An interval CT scan of chest, abdomen and pelvis performed ten months after the surgery, showed no recurrence of malignancy. Given the fact that the patient's MPO ANCA fell following the treatment of cholangiocarcinoma, it is likely that positive MPO ANCA is associated with underlying malignancy rather than an active vasculitis. Conclusion This unusual case describes an evolution of the diagnostic process guided by non-specific symptoms and ANCA positivity, arriving at an unexpected diagnosis of malignancy. Although ANCA is a sensitive and specific marker of vasculitides, it can be positive in other conditions particularly hepatitis B, inflammatory bowel disease and autoimmune liver disorders. Malignancy can also be associated with ANCA in the absence of vasculitis. In one study, of 118 ANCA positive patients without ANCA-associated vasculitis, four were found to have malignancy. In a study of 1024 patients who had ANCA tested, 61 patients were found to have malignancy, predominantly haematological and lung cancers. However, after adjustment for sex, age and time of blood draw, no association was found between ANCA status and incidence of cancer. Interestingly, paraneoplastic vasculitis such as polyarteritis nodosa (PAN) has been described in the context of underlying cholangiocarcinoma, and is associated with ANCA rise. Moreover, patients with raised ANCA and PAN also have raised CA 19- 9.

A Rare Cause of Enteritis in an Adult Male

Introduction: IgA vasculitis is more commonly seen in the pediatric population than in adults. Rarely IgA vasculitis is associated with malignancy, most commonly solid tumor malignancies, although there are case reports of association with hematologic malignancies. We report a case of large B-cell lymphoma mimicking IgA vasculitis in a 33-year-old immunosuppressed male with a prior history of IgA vasculitis. Case Description/Methods: A 33-year-old Caucasian male post renal transplant from reflux nephropathy on chronic immunosuppression was hospitalized for postprandial epigastric abdominal pain, nausea, vomiting and diarrhea. Two years prior, he was admitted for the same symptoms, palpable purpura of the lower extremities and elevated serum IgA. Enteroscopy had shown duodenal and jejunal ulceration with biopsies staining positive for IgA, confirming IgA vasculitis. He had complete resolution with a steroid taper. His current presentation had resulted in multiple hospital admissions, but empiric trial of steroids failed to alleviate symptoms. Vitals were normal and exam was notable for epigastric tenderness. Labs were notable for WBC 19.00 x103/cmm with normal differential, hemoglobin 9.2 gm/dL (prior 11.0 gm/dL), CRP 20.7 mg/L, serum creatinine 2.7 mg/dL (prior 1.5 mg/dL), and urinalysis with proteinuria, sterile pyuria, and hematuria. CTA abdomen/pelvis revealed thickening of the duodenum with shotty mesenteric lymph nodes without ischemia. Enteroscopy revealed an erythematous duodenum and jejunum (figure A). Jejunal biopsy (figure B) revealed CD20 positive cells consistent with DLCBL (figure C). He was seen by oncology and treated with R-CHOP but later unfortunately expired due to COVID-19 complications. Discussion(s): Non small cell lung cancer and renal cell carcinoma are most commonly associated with IgA vasculitis. It may also be seen in both Hodgkin and Non-Hodgkin lymphomas in adult patients. If IgA vasculitis occurs after a malignancy is diagnosed, it may indicate that metastasis has occurred. Malignancy associated IgA vasculitis is more likely to have an incomplete response to steroids and requires treatment of the underlying malignancy to achieve remission. Our case illustrates posterior probability error and premature closure cognitive biases. We should consider alternative diagnoses rather than anchor on prior diagnoses even when presentations are similar. Our case also highlights the importance of considering occult malignancy in adults with diagnosis of IgA vasculitis.

The role of mobile applications in the conservative management of stress urinary incontinence in women

Urinary incontinence, affecting over 300 million women worldwide, regardless of race and age, is considered one of the most important health issues in the 21st century. owing to the scale of the problem, the priority should be to provide therapy to as many patients as possible. Although effective conservative treatment measures for urinary incontinence are available, they may not cater for all individuals who seek help. Sometimes, a sense of embarrassment or a fear of stigmatization causes patients' reluctance to report urinary incontinence symptoms to their health care provider and to join therapy. That forces therapists to search for a new approach. in this field, the use of mHealth technologies seems very promising. They have become even more valuable during the CoVid-19 pandemic, when the interest in telemedicine, as a means of providing care while not being exposed to the risk of virus infection, further increased. The purpose of this work was a narrative review showing possibilities of employing conservative measures to manage stress urinary incontinence in women, with a particular emphasis on the use of mHealth technologies, as recent studies have shown that mobile applications seem to be an effective tool in terms of improving stress urinary incontinence symptoms, satisfaction, and adherence to therapy.Copyright © Wroclaw University of Health and Sport Sciences.

L-Ornithine-L-Aspartate and Intermittent Renal Replacement Therapy in Fulminant Hepatitis A: A Case Report

Hepatitis A is a common viral infection worldwide that is transmitted via the fecal-oral route. Since the introduction of an efficient vaccine, the incidence of infection has decreased but the number of cases has risen due to widespread community outbreaks among unimmunized individuals. Classic symptoms include fever, malaise, dark urine, and jaundice, and are more common in older children and adults. People are often most infectious 14 days prior to and 7 days following the onset of jaundice. We will discuss the case of a young male patient, diagnosed with acute hepatitis A, leading to fulminant hepatitis refractory to conventional therapy and the development of subsequent kidney injury. The medical treatment through the course of hospitalization was challenging and included the use of L-ornithine-L-aspartate and prolonged intermittent hemodialysis, leading to a remarkable outcome. Hepatitis A is usually self-limited and vaccine-preventable;supportive care is often sufficient for treatment, and chronic infection or chronic liver disease rarely develops. However, fulminant hepatitis, although rare, can be very challenging to manage as in the case of our patient.Copyright © 2023 The Author(s).

Sarcoidosis-Associated Immune Thrombocytopenic Purpura and Focal Segmental Glomerulosclerosis

Purpose of study A 32-years old male with known multi-system sarcoidosis in remission for 5 years off treatment presented to the emergency room with complaints of generalized weakness, hematemesis, epistaxis, and bruises. Physical examination was notable for petechiae, ecchymosis along with papules and plaques suggestive of active sarcoid skin lesions on his extremities. Laboratory workup was significant for thrombocytopenia 3000/uL, acute kidney injury with sub-nephrotic proteinuria. Peripheral blood smear did not show evidence of hemolysis and direct Coombs test was negative. Infectious workup including COVID-19, HIV, and hepatitis serologies were negative. Computed Tomography (CT) of chest, abdomen, and pelvis showed mild splenomegaly and an increased number of sub-centimeter hilar and mediastinal lymph nodes. The patient was treated with dexamethasone 40 mg daily for 4 days and intravenousimmunoglobulins (IVIG-2 gm/kg) for possible Immune Thrombocytopenic Purpura (ITP) with improvement in platelet count to 42000/uL by day 3. His workup for AKI and sub-nephrotic proteinuria was negative apart from a positive ANA (1: 160) with low complements. The anti-phospholipid antibody panel was negative. The ACE level was markedly elevated (>80U/L). The patient could not get a renal biopsy due to severe thrombocytopenia. He was discharged but was re-admitted in 15 days for severe thrombocytopenia of 1000/uL, epistaxis, and bruising. We continued high dose steroids along with IVIG 1 gm/kg for refractory ITP with minimal response and started anti-CD20 agent (Rituximab) 375 mg/m2 weekly with thrombopoietin-receptor agonist (Eltrombopag). His platelets count improved in response to treatment and subsequent renal biopsy showed focal and segmental glomerulosclerosis along with mild interstitial fibrosis, tubular atrophy thought to be from long standing sarcoidosis. There was also evidence of focal arteriosclerosis with no evidence of granulomas, immune complex, complement, or IgG4 deposition. Given skin lesions, thrombocytopenia, extensive lymphadenopathy, and renal involvement with markedly elevated ACE levels the overall picture was consistent with active multi-system sarcoidosis. His platelet count increased to 177,000/uL at the time of discharge. Currently, the patient is on slow steroid taper along with Eltrombopag 25 mg every other day without any recurrence of his symptoms so far. Methods used We described one case of sarcoidosis with hematologic and renal involvement. Summary of results Our patient developed hematologic and renal complications approximately 6 years after being diagnosed with sarcoidosis. Initially, he did not demonstrate sufficient clinical response to IVIG and high dose steroids. However, after a course of anti-CD20 agent (Rituximab) and with the addition of thrombopoietin-receptor agonist (Eltrombopag) he showed improvement of platelet count and stabilization of the renal function. Currently, the patient is receiving maintenance therapy with Prednisone 7.5 mg daily along with Eltrombopag 25 mg twice weekly with no recurrence of ITP and stable renal function. A further decision on whether the patient needs another cycle of Rituximab will be determined by the patient's clinical course. Conclusions Highly variable manifestations of Sarcoidosis can pose a significant diagnostic and therapeutic challenge as can be seen from our case. ITP is a rare hematological manifestation of sarcoidosis and addition of anti-CD20 agents should be considered in refractory cases.

One-year outcomes following rehabilitation for chronic hip-related groin pain: ancillary analysis of a pilot randomized clinical trial

Purpose: Rehabilitation to address modifiable factors associated with chronic hip-related groin pain (CHRGP) may lead to reduced pain and improved function, yet little is known about its effectiveness. We assessed the preliminary effects of two interventions that target two distinct mechanisms, sensory disturbances and abnormal movement patterns. Sensory disturbances such as peripheral and central sensitization may contribute to pain persistence long after initial injury. Joint mobilization (JtMob) may impart a neurophysiological response within the nervous system that results in pain reduction and improved mobility. Abnormal movement patterns may create altered mechanical stresses on hip joint structures, resulting in pain and activity limitations. Movement pattern training (MoveTrain) may improve movement patterns and thus patient function. Method(s): Patients with CHRGP, 18-40, were enrolled. Assessments included self-report questionnaires, clinical exam, and quantitative sensory testing. Outcomes included the Hip disability and Osteoarthritis Outcome Score (HOOS), a patient-reported outcome;frontal plane kinematics of hip, pelvis, and trunk during single leg squat;and pain pressure threshold (PPT) assessed at the anterior groin of the most bothersome hip and dominant thenar eminence (local and generalized pressure hypersensitivity, respectively). Patients were randomized to JtMob or MoveTrain in a 1:1 ratio stratified by sex and HOOS Symptoms. Treatment for both groups included 10 individualized visits over 12 weeks with a trained physical therapist (PT);assessment of patient goals and education which focused on patient-specific tasks reported by the patient to be symptom-producing;instruction in a home exercise program (HEP);and handouts that provided education, description and benefits of assigned treatment and instructions for HEP. The key element of JtMob was PT-provided manual techniques using specific criteria to determine the joint mobilization techniques and parameters used for each patient. The patient's symptom report to each technique was monitored and if indicated, the technique modified according to our outlined procedures. The HEP included flexibility exercises. The key element of MoveTrain was task-specific instruction to correct abnormal movement patterns displayed during daily and patient-specific tasks. For example, hip adduction was minimized during a step descent. The HEP included repeated practice of modified tasks. Task difficulty was progressed based on each patient's performance. Immediately after treatment completion, patients returned for follow up assessment. To assess treatment sustainability after the active treatment phase, we collected HOOS at 6 and 12 months (extended follow-up), and kinematics and PPT at 12 months. Data from patients who provided any data after baseline were analyzed with a repeated measures analysis of variance (RM-ANOVA) with baseline value as a covariate, patient as a random effect, and an autoregressive covariance structure. After adjusting for baseline, the between-group difference in change from post-treatment to each extended follow-up results from pre-planned statistical contrasts in a RM-ANOVA that includes main effects for treatment group, visit and the group by visit interaction. The within-group treatment effect at each extended follow-up was calculated by subtracting the earlier time point from the later follow-up within each treatment group. Dependent samples t-tests were used to assess the degree of within-group change. Result(s): Demographics and outcome data are provided in Tables 1 and 2, respectively. Thirty-three patients with CHRGP were randomized and 29 (88%) provided post-treatment data. Four patients did not complete treatment or post-treatment testing (3 due to COVID pandemic, 1 lost to follow up);6 patients did not complete 12 month laboratory testing (due to pandemic), but did complete 12 month questionnaires. Previously, we reported that both groups reported clinically important improvements in HOOS subscales and MoveTrain group improved hip and pelvis kinematics immediately after treatment compared to baseline. After adjusting for baseline, there were no between-group differences in change in outcomes between post-treatment and extended follow-up when comparing JtMob and MoveTrain, indicating that treatment effects immediately post-treatment were maintained at 12 months after treatment completion. Conclusion(s): Our preliminary findings suggest that 12 weeks of JtMob or MoveTrain, may result in improvements in patient-reported pain and function and these effects may persist 12 months after treatment completion. A future, larger trial to definitively assess the efficacy of JtMob and MoveTrain and identify factors associated with long-term outcomes will improve our ability to develop treatment strategies for people with CHRGP. [Formula presented] [Formula presented]Copyright © 2023

New Paradigm in Robotic Prostatectomy: Planned Same Day Discharge in All Patients

INTRODUCTION AND OBJECTIVE: In 2016 we began offering optional same-day discharge (SDD) to all robotic prostatectomy (RP) patients with increasing acceptance that accelerated during the COVID pandemic. Our resulting 98% SDD rate for RP after COVID facilitated initiation of an ambulatory surgery center (ASC) robotic urology program without overnight capability and planned SDD in all patients. We assessed our outcomes with planned outpatient RP in all patients in both the hospital and ASC settings. METHOD(S): We reviewed one year of consecutive RPs performed by a single surgeon at either a free-standing ASC or one of three hospitals between October 2021-October 2022. Pelvic lymphadenectomy was performed in all patients. Assignment to ASC versus hospital RP was based primarily on insurance eligibility. ASC policy for robotic or non-robotic procedures alike excluded history of severe cardiac disease, difficult airway, malignant hyperthermia, or BMI >45kg/m2 with no additional limitations applied to robotic surgery. All patients were instructed to expect same-day discharge (SDD) directly from the recovery room regardless of ASC or hospital location with overnight stay only for unexpected complications or side effects of anesthesia. RESULT(S): Among 359 RP cases (162 ASC and 197 hospital), 356 (99%) were successfully discharged the same day as surgery with 3 overnight stays in the hospital group and none in the ASC group. Patients in the ASC group were younger (61.4yrs vs 67.1yrs, p<001) with no statistically-significant difference in BMI (29.2 kg/m2 vs 29.3 kg/ m2, p=0.3), preoperative Gleason Score (p=0.1), operative time (131min vs 134min, p=0.2) or blood loss (87.5cc vs 84.8cc, p=0.71). Excluding the three overnight patients in the hospital group, the mean postoperative recovery room stay among SDD patients was shorter in the ASC group (1.7hrs vs 2.3hrs, p<0.0001). The 90-day readmission rate was 2.5% in both groups (4/162 and 5/197, p=0.93). No readmissions occurred within 24 hours of surgery and only one within the first week. CONCLUSION(S): Same day discharge as a routine following robotic prostatectomy is feasible and safe with readmission rates no higher than series with overnight stays. SDD may enable ASC RP when overnight stay capabilities are not available at an ASC with minimal risk of need for hospital transfer.

Biofeedback Treatment Can Improve Clinical Condition and Quality of Life in Patients with Pelvic Floor Dyssynergy with Irritable Bowel Syndrome: A Prospective Cohort Study

Background: Chronic constipation is a common health concern. Defecatory disorders are considered one of the mechanisms of chronic idiopathic constipation. This study aimed to evaluate the effect of concurrent irritable bowel syndrome (IBS) on the success rate and response to biofeedback therapy in patients with chronic constipation and pelvic floor dyssynergia (PFD). Method(s): This prospective cohort study was performed at the Imam Khomeini Hospital Complex in Tehran from October 2020 to July 2021. Patients aged 18-70 years with chronic constipation and PFD confirmed by clinical examination, anorectal manometry, balloon expulsion test, and/or defecography were included. All patients failed to respond to treatment with lifestyle modifications and laxative use. The diagnosis of IBS was based on the ROME IV criteria. Biofeedback was educated and recommended to all patients. We used three different metrics to assess the patient's response to biofeedback: 1) constipation score (questionnaire), 2) lifestyle score (questionnaire), and 3) manometry findings (gastroenterologist report). Result(s): Forty patients were included in the final analysis, of which 7 men (17.5%) and 21 (52.2%) had IBS. The mean age of the study population was 37.7 +/- 11.4. The average resting pressure decreased in response to treatment;however, this decrease was statistically significant only in non-IBS patients (P = 0.007). Patients with and without IBS showed an increase in the percentage of anal sphincter relaxation in response to treatment, but this difference was not statistically significant. Although the first sensation decreased in both groups, this decrease was not statistically significant. Overall, the clinical response was the same across IBS and non-IBS patients, but constipation and lifestyle scores decreased significantly in both groups of patients with and without IBS (P < 0.001). Conclusion(s): Biofeedback treatment appears to improve the clinical condition and quality of life of patients with PFD. Considering that a better effect of biofeedback in correcting some manometric parameters has been seen in patients with IBS, it seems that paying attention to the association between these two diseases can be helpful in deciding on treatment.Copyright © 2023 The Author(s).

Development of a low-cost model for laparoscopic pyeloplasty simulation

Aims: Simulation is increasingly important in the training of paediatric urology;training time lost due to the Covid-19 pandemic, patient safety concerns and the uptake of technology (i.e., robotics) means that trainees must develop new methods of attaining operative competency. Laparoscopic pyeloplasty can be technically challenging and involves a steep learning curve to be confident in performing this procedure independently. We therefore aimed to develop a low-cost, high-fidelity model which trainees could create using easily available materials. Method(s): Unique Party Balloon (Unique Party Store)-1200 Scarlet Red (pack of 10) [2.34] were filled with cotton wool pads (2 packs of 50 rounds 1-Aldi) and fashioned into a kidney shape. Pink 102 latex Balloons (PABBEU) [4.99] were filled with a 50 ml of water to create the renal pelvis and superglued (Loctite pack of 3, Ryman) [4.99] to the kidney. Then Long Pastel Twisting Balloons (pack of 50)-[4.29] were attached over the tied end of the renal pelvis creating the ureter. The model was then secured into the Laparoscopic box trainer using Velcro Hook and Loop Coins (Syntego, 105 pack) [2.95]. Result(s): The model was utilised in departmental skills sessions. Feedback demonstrated the model provided a realistic feel performing the anastomosis due to the latex, water-filled, balloons. Total cost per model was 1.10 (20 models). Conclusion(s): We demonstrate a low-cost model of laparoscopic pyeloplasty which trainees can create easily themselves. However, further work will be needed to improve the model for anatomical relations, particularly crossing vessels.

Centering Group Treatment for Women With Interstitial Cystitis/Bladder Pain Syndrome: A Prospective, Parallel-Group Cohort Study

Importance: Women with interstitial cystitis/bladder pain syndrome (ICBPS) face isolation and treatment challenges. Group medical visits using Centering models have successfully treated other conditions but have not been explored in ICBPS. Objective(s): This study aimed to describe ICBPS pain and symptom control comparing standard treatment alone versus standard treatment augmented with Centering visits. Study Design: This prospective cohort study recruited women with ICBPS receiving standard care (control) or standard care augmented with group Centering. We administered validated questionnaires at baseline and monthly for 12 months. The primary outcome was change in the pain numerical rating scale, with Patient-Reported Outcomes Measurement Information System Pain Interference Scale and Bladder Pain/Interstitial Cystitis Symptom Score change as secondary measures. Result(s): We enrolled 45 women (20 Centering, 25 controls). Centering had significantly better numerical rating scale pain scores at 1 month (mean difference [diff], -3.45) and 2 months (mean diff, -3.58), better Patient-Reported Outcomes Measurement Information System Pain Interference Scale scores at 1 month (mean diff, -10.62) and 2 months (mean diff, -9.63), and better Bladder Pain/Interstitial Cystitis Symptom Score scores at 2 months (mean diff, -13.19), and 3 months (mean diff, -12.3) compared with controls. In modeling, treatment group (Centering or control) and educational levels were both associated with all the outcomes of interest. Beyond 6 months, there were too few participants for meaningful analyses. Conclusion(s): Women with ICBPS participating in a Centering group have, in the short term, less pain, pain interference, and ICBPS-specific symptoms than patients with usual care alone. Larger studies with more follow-up are needed to determine if this treatment effect extends over time.Copyright © 2022 American Urogynecologic Society. All rights reserved.

ANTIBODIES TO ANGIOTENSIN-CONVERTING ENZYME 2 IN INFERTILE PATIENTS WITH A HISTORY OF COVID-19 AND IN FERTILE WOMEN.

Angiotensin-converting enzyme 2 (ACE2) is a key component of the renin-angiotensin system (RAS) that affects fertility in women. Antibodies against ACE2 have predictive value for COVID-19 and may contribute to RAS dysregulation and reproductive failure. Objective(s): To investigate the prevalence and levels of anti-ACE2 autoantibodies in infertile patients with a history of COVID-19 and in fertile women. Material(s) and Method(s): Serum anti-ACE2 autoantibodies (M, G) were determined by ELISA in infertile patients with a history of COVID-19 (group 1, n=121), without a history of COVID-19 (group 2, n=79), and in fertile women (group 3, n=80). The association between antibodies against ACE2, SARS-CoV-2, thyroid antigens, and hormones was investigated. Result(s): Patients in groups 1 and 2 had higher rates of inflammatory gynecologic diseases, pelvic surgery, spontaneous miscarriages, and thyroid pathology than those in group 3. Anti-ACE2 antibodies were detected more frequently (40.5% and 38.8 %) and had higher levels in infertile patients than in fertile women (20%). Women with a history of COVID-19 were more likely to have anti-ACE2 IgG. Antibodies against ACE2 were significantly correlated with those against FSH. Conclusion(s): Patients with infertility, irrespective of a history of COVID-19, have a higher prevalence and higher anti-ACE2 antibody levels than fertile women. Anti-ACE2 antibodies are associated with primary and secondary infertility, and may be involved in the pathophysiology of infertility.Copyright © A group of authors, 2023.

Polytrauma patient with lateral thoracic spondyloptosis: case report and literature review

Background: Spondyloptosis is caused by high force trauma. The vast majority of cases occur in the sagittal plane and at transition points where ridged sections meet more flexible regions. Lateral thoracic spondyloptosis is extremely rare and there is no current consensus on the optimal treatment plan. Case Description: Here we present a case of a previously physically healthy 24-year-old polytrauma patient after he was struck as a pedestrian by a motor vehicle. Of note the patient was found to have lateral spondyloptosis between T9-10 with complete spinal cord transection. The patient also sustained multi-ligamentous left knee injury, pelvic fractures, open comminuted left tibia and fibular fracture, lacerated liver, bilateral renal lacerations, ischemic bowel, and an aortic arch pseudoaneurysm. Conclusion(s): Lateral thoracic spondyloptosis is a devastating injury with an extreme rate of persistent neurologic deficits. There is no unanimously accepted treatment because of the rarity if the injury and the poor outcomes that patients face. Additionally, patients who experience high level trauma often develop severe psychiatric illness, and the importance of identifying risk factors and implementing care early may improve patient outcomes.Copyright © AME Medical Journal.