Acute Myopericarditis as the First Manifestation of Familial Mediterranean Fever: A Case Report.

Familial Mediterranean fever (FMF) is an autoinflammatory disorder, characterized by recurrent episodes of fever and polyserositis, and usually presents during the first two decades of life. Acute pericarditis is a rare manifestation of FMF and typically presents with other symptoms of the inflammatory disorder. A 27-year-old Arabian male presented to our hospital with pleuritic chest pain and shortness of breath while lying flat. His electrocardiogram showed changes suggestive of pericarditis, and his inflammatory markers and troponin were raised. His echocardiogram revealed a moderate-sized pericardial effusion with septa and a normal left ventricular function. He had a strong family history of FMF and consanguinity of the parents. He was treated for acute myopericarditis with colchicine and ibuprofen, and his symptoms improved gradually along with his inflammatory markers and troponin. Six weeks after discharge, he had a cardiac MRI, which revealed a thickened pericardium with profound enhancement (features suggestive of pericarditis) and no signs of myocarditis. He was asymptomatic, and his markers and troponin were within the normal range. His colchicine medication was continued indefinitely, and he was referred to a tertiary care hospital with a specialized periodic fever clinic for follow-up and genotype testing.

Hepatic Sarcoid-Like Reaction Mimicking Liver Metastases in a 36-Year-Old Female With Rheumatoid Arthritis.

Hepatic and splenic sarcoidosis are still challenging issues for medical imaging, and in many cases, medical images can't exclude the most common mimic of sarcoidosis which is liver metastases; therefore, a liver biopsy is required. A young female patient who had rheumatoid arthritis presented to our hospital with abdominal pain, anorexia, and weight loss for the past three weeks. She was admitted to the acute medical ward and treated with intravenous fluid hydration for hypercalcemia. Her liver function tests were deranged (anicteric cholestasis picture), and her etanercept medication was stopped after being reviewed by the rheumatologist and gastroenterologist. She had a CT and MRI scan of the abdomen, an ultrasound (US) of the abdomen with enhanced contrast, and a positron emission tomography (PET) scan. The radiological findings could not exclude liver metastases, but an ultrasound-guided liver biopsy confirmed the finding of hepatic granulomatous changes of sarcoidosis. Her symptoms and hypercalcemia resolved, and her liver functions gradually normalized.

Breast metastasis from thyroid follicular carcinoma in a 90-year-old patient 12 years after thyroidectomy and radiotherapy.

INTRODUCTION: Secondary lesions in the breast from extramammary malignancies are rarely encountered in medical practice. Medical imaging of the breast (mammography and ultrasonography) may not differentiate between a primary breast mass and a secondary tumor from an extramammary malignancy. CASE DESCRIPTION: A 90-year-old woman with a history of thyroid cancer, skin metastasis, thyroidectomy, and radiotherapy 12 years prior presented with pain in the left breast and no palpable masses in either breast or axilla. Mammography and ultrasonography of the breast confirmed a suspicious lesion in the upper outer quadrant of the left breast. Histopathology of the core biopsy, including immune histochemistry staining of the lesion, confirmed secondary follicular carcinoma of the thyroid. CONCLUSION: Thyroid cancer is a rare extramammary malignancy that may metastasize to the breast; however, it may rarely occur a few years after thyroidectomy and radiotherapy.

Emphysematous pyelonephritis and septic shock in an elderly male patient caused by extended-spectrum beta-lactamase producing Klebsiella pneumoniae.

Emphysematous Pyelonephritis is a rare necrotizing infection with gas production and a high overall mortality rate. An elderly diabetic male patient presented to our Emergency Department with septic shock and was found to have left perinephric emphysematous pyelonephritis with a large collection. He was managed with the proper antibiotic therapy, and two trials of percutaneous drainage of the collection with no significant reduction in the size of the abscess. After operative drainage and debridement of multiple pockets, he improved and was discharged home. Extender spectrum beta-lactamase producing organism was an additional challenge in our patient's management.

COVID-19 pneumonia identified by CT of the abdomen: A report of three emergency patients presenting with abdominal pain.

Patients with COVID-19 infection may present to the Emergency Department (ED) with gastrointestinal complaints and no respiratory symptoms. We are presenting 3 patients who came to the ED with abdominal pain; and the computed tomography [CT] of the abdomen showed findings suggestive of COVID-19 pneumonia. A 65-year-old male patient presented with symptoms of urinary tract infection and left renal angle tenderness. A 42-year-old male patient presented with right flank pain postextracorporeal shock wave lithotripsy. A 71-year-old male known to have type 2 diabetes mellitus and who had had whipple surgery for a neoplasm of the head of the pancreas presented with a painful epigasteric swelling. The 3 patients had positive COVID-19 polymerase chain reaction (PCR) tests and mild-to-moderate illness, and were discharged home after 2 weeks with a good recovery. The first patient had a false negative early PCR test, which turned positive on 2 repetitions of the test. A systematic review of CT abdomen, including inspection of the lung bases using the lung window in all CT abdomen, is essential to detect findings suggestive of COVID-19 pneumonia in patients requiring a CT abdomen study. As proven in the literature, CT findings of COVID-19 pneumonia have a higher sensitivity than the PCR test.

Dengue Fever and COVID-19 Co-Infection at the Emergency Department of a Tertiary Care Hospital in Jeddah, Saudi Arabia.

INTRODUCTION: Co-infection with COVID-19 and other viral or bacterial infections has been described in many studies. Most cases of dengue fever in Saudi Arabia have been reported in Jeddah city and mainly during the summer season. METHOD AND RESULTS: We describe four patients who presented to the emergency department with febrile illness accompanied by symptoms and blood tests suggestive of dengue fever. They also had radiological findings suggestive of COVID-19 chest infection and positive PCR tests. Three of the patients required hospital admission, and all had a good outcome. CONCLUSION: In areas with a high rate of dengue fever, serological screening for dengue fever should be considered as part of COVID-19 investigation, especially if symptoms or a full blood count are suggestive. LEARNING POINTS: Dengue fever (DF) and COVID-19 co-infection has been reported in countries with a high rate of DF.Symptoms and /or full blood count changes suggestive of DF should draw the attention for a DF screening while reviewing patients with suspected COVID-19 infection.

An Abdominal Aortic Aneurysm, Intramural Thrombus and Moderate Leak in an Asian Man Presenting with Acute Gastroenteritis.

INTRODUCTION: Missing a leaking abdominal aortic aneurysm (AAA) is common in medical practice because few at-risk patients have a history of AAA and many have an unusual presentation. BACKGROUND: AAA is less common among Asians than white Caucasians of the same age. Our patient had no significant risk factors apart from age and sex and had an unusual presentation. PATIENT AND METHODS: A 67-year-old Asian man presented to the emergency room (ER) with a 1-day history of nausea, vomiting, diarrhoea, fever and abdominal pain. He was febrile, dehydrated. and had marked tenderness at the right iliac fossa. Laboratory findings suggested bacterial gastroenteritis but this did not explain the localized tenderness at the right iliac fossa. RESULT AND DISCUSSION: A CT scan of the abdomen revealed an AAA arising above the origins of the renal arteries, an intramural thrombus, a retroperitoneal haematoma and a leak extending to the right iliac fossa. The patient was transferred to another hospital and underwent exploratory laparotomy, surgical repair of the aneurysm, and aortobi-iliac grafting with removal of the thrombus. The patient was discharged in good shape 3 weeks after surgery. Without the CT scan of the abdomen, the AAA could have been missed and the patient treated for severe gastroenteritis. LEARNING POINTS: Although abdominal aortic aneurysm leak is a rare differential diagnosis for elderly Asian patients with abdominal pain, it should be still suspected and investigated accordingly.In our elderly patient, as localized tenderness on clinical examination could not be explained by the gastroenteritis, an imaging study was essential to rule out a surgical cause.

Prostatic Abscess after Stapled Hemorrhoidopexy Caused by ESBL Extended Spectrum Beta Lactamase Producing Klebsiella pneumoniae: An Additional Challenge to Postoperative Sepsis.

Postoperative septic complications of hemorrhoids surgical interventions are rare, but very serious with high mortality rate. Early diagnosis and prompt therapy are essential to save patient's life. There are a good number of articles and case reports about these septic complications. We are presenting a case report of a prostatic abscess caused by extended spectrum beta lactamase (ESBL) producing Klebsiella pneumoniae after hemorrhoidopexy. Our patient was a healthy middle aged Saudi male who has no significant medical history apart from morbid obesity and recurrent urinary tract infections. ESBL producing K. pneumoniae could be detected only after aspiration of the prostatic abscess, but proper antibiotic was introduced intravenously on admission before culture of aspirate of the abscess was available. Antibiotic was continued for 30 days and abscess resolved completely. In our electronic search, we could not find any case report of prostatic abscess after stapled hemorrhoidopexy caused by ESBL producing organism. This is an additional challenge for treating physicians as these organisms are sensitive only to one group of antibiotics (carbapenem group).

Multiple Renal Abscesses due to ESBL Extended-Spectrum Beta-Lactamase-Producing Escherichia coli Causing Acute Pyelonephritis and Bacteremia: A Case Report with a Good Outcome (No Drainage Required).

Extended-spectrum beta-lactamase-producing Enterobacteriaceae urinary tract infections are challenging infections with increased mortality, morbidity, and failure of therapy. A 44-year-old Saudi male diabetic patient was seen at the ER of IMC Hospital with features of acute pyelonephritis: fever, burning urine, and left flank pain for three days. He was treated for cystitis at the Endocrine Clinic two weeks prior to his ER visit with nitrofurantoin and levofloxacin orally according to urine culture and sensitivity result. The patient was admitted, received IV meropenem, and continued to be febrile for three days. His urine and blood culture at ER grew the same ESBL-producing E. coli as in his urine culture from the Endocrine Clinic. His abdomen CT scan showed two left renal abscesses at the upper and middle poles. His temperature resolved on the fourth day of IV therapy. Intravenous meropenem was continued for 4 weeks after inserting PICC line and the patient was followed up by home healthcare. He was feeling better with occasional left flank pain and repeated abdomen CT scan showed complete resolution of both renal abscesses.

Allogeneic Stem Cell Transplantation in Congenital Hemoglobinopathies Using a Tailored Busulfan-Based Conditioning Regimen: Single-Center Experience.

Hematopoietic stem cell transplantation (HSCT) is the only proven curative option for patients with hemoglobinopathies, both thalassemia and sickle cell anemia (SCA). A busulfan-based myeloablative conditioning regimen is the standard of care for HSCT in these patients, although increased treatment-related morbidity, including veno-occlusive disease (VOD), has been demonstrated. Thirty-eight pediatric patients, median age 8 years (range, 6 months to 22 years), suffering from hemoglobinopathy were treated at Rambam Medical Center in Haifa, Israel, between 1998 and 2011. Thirty-four patients had thalassemia major and 4 had SCA. The 38 patients underwent 40 HSCTs, 34 of which were first transplants and 6 second transplants. Most transplants (32/40) were from matched sibling donors. Sources of stem cells were peripheral blood in 30 transplants, bone marrow in 7 transplants, and cord blood in 3 transplants. All received different customized busulfan-based conditioning regimens tailored by pharmacokinetic analysis of busulfan levels. Primary engraftment occurred in 37 of 40 transplants. Neutrophil engraftment (>.5 × 10(9)/L) occurred at a median of 15.3 days post-transplantation (range, 10 to 45). Platelet transfusion independence (>20 × 10(9)/L) occurred at a median of 22.3 days (range, 11 to 60). The rate of 5-year overall survival for thalassemia patients after first transplantation was 90.5% ± 5.3%. The rate of 5-year thalassemia-free survival was 81.7% ± 6.8%. Cumulative incidence of acute graft-versus-host disease (GVHD) was 17.6%. Rate of grades III to IV GVHD was 8.8%. Cumulative incidence of chronic GVHD was 23.5%, with 11.8% incidence of extensive chronic GVHD. One patient developed VOD. Full donor chimerism occurred in 36.4% of patients with class 1 + 2 thalassemia, compared with 78.6% in class 3 thalassemia (P = .049). Overall survival above 90% in patients undergoing their first transplant was demonstrated using busulfan-based conditioning regimens. The low incidence of VOD was probably due to busulfan area under the curve measurements and dose adjustment.

Significance of post-resection tissue shrinkage on surgical margins of oral squamous cell carcinoma.

BACKGROUND: Resecting oral squamous cell carcinoma (SCC) with an appropriate margin of uninvolved tissue is critical in preventing local recurrence and in making decisions regarding postoperative radiation therapy. This task can be difficult due to the discrepancy between margins measured intraoperatively and those measured microscopically by the pathologist after specimen processing. MATERIAL AND METHODS: A total of 61 patients underwent resective surgery with curative intent for primary oral SCC were included in this study. All patients underwent resection of the tumor with a measured 1-cm margin. Specimens were then submitted for processing and reviewing, and histopathologic margins were measured. The closest histopathologic margin was compared with the in situ margin (1 cm) to determine the percentage discrepancy. RESULTS: The mean discrepancy between the in situ margins and the histopathological margins of all close and positive margins were 47.6% for the buccal mucosa (with a P value corresponding to 0.05 equaling 2.1), which is statistically significant, 4.8% for the floor of mouth, 9.5% for the mandibular alveolus, 4.8% for the retromolar trigon, and 33.3% for the tongue. CONCLUSION: There is a significant difference among resection margins based on tumor anatomical location. Margins shrinkage after resection and processing should be considered at the time of the initial resection. Tumors located in the buccal mucosa show significantly greater discrepancies than tumors at other sites. These findings suggest that it is critical to consider the oral site when outlining margins to ensure adequacy of resection. Buccal SCC is an aggressive disease, and should be considered as an aggressive subsite within the oral cavity, requiring a radical and aggressive resective approach.

Autoimmune complications after hematopoietic stem cell transplantation in children with nonmalignant disorders.

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) remains the only curative treatment for many nonmalignant disorders, such as autoimmune disorders, inborn metabolic disorders, hemoglobinopathies, and immunodeficiency disorders. Autoimmune complications (AICs) after HSCT, such as autoimmune cytopenias, autoimmune hepatitis, primary biliary cirrhosis, and autoimmune cutaneous manifestations, are still neither well defined nor characterized. PATIENTS: Between 2000 and 2012, 92 patients (47 males, 45 females) were treated with HSCT in our hospital, 51 with congenital hemoglobinopathies, 19 with primary immunodeficiency disease, 10 with metabolic disorders, five with Fanconi anemia, three with aplastic anemia, and four with familial hemophagocytic lymphohistiocytosis. RESULTS: Mean age at HSCT was 6.4 years (range, 0.2-32 years) and mean duration of followup after HSCT was 6.81 years (range, 1-11 years). Sixteen (17.4%) patients developed chronic GVHD and five (5.4%) showed sclerodermatous features. Five (5.4%) patients were diagnosed with scleroderma manifestations, six (6.5%) with vitiligo, six (6.5%) with autoimmune hemolytic anemia (AIHA), six (6.5%) with idiopathic thrombocytopenia, three (3.3%) with mild leucopenia, two (2.2%) with aplastic anemia, two (2.2%) (one boy, one girl) with autoimmune thyroid disease, and one (1.1%) with autoimmune hepatitis. CONCLUSIONS: It was concluded that AICs are clinically significant complications after HSCT that contribute to morbidity but not to mortality. AICs are more frequent after HSCT for metabolic disorders, and sclerodermatous GVHD is more significant in children who underwent allogeneic HSCT for hemoglobinopathies. The potential to identify risk factors for AICs could lead to less morbidity and mortality and to maintain the patient's quality of life.

Factors influencing outcome and incidence of late complications in children who underwent allogeneic hematopoietic stem cell transplantation for hemoglobinopathy.

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) remains the only potentially curative treatment for severe hemoglobinopathy (HGP). Late complications (LCs) are all events occurring beyond two years post-HSCT. We retrospectively analyzed prevalence, factors influencing occurrence, and prognosis of LCs post-HSCT for HGP. PATIENTS AND METHODS: Between 2000 and 2011, 47 patients (21 males, 26 females; 43 with beta thalassemia major, four with sickle cell disease) who had survived more than two years post-HSCT for HGP were retrospectively reviewed. Mean age at HSCT was 7.7 years (1.1-32 years); mean follow-up was 7.1 years (2-11.6 years); 11 patients were splenectomized; mean ferritin level was 3022 ng/mL (350-10900); and seven patients underwent a second HSCT. RESULTS: Endocrinological complications were observed with primary gonadal failure in 16/20 mature females and 4/11 mature males, in five patients with primary hypothyroidism and in four with insulin-dependent diabetes mellitus (DM). Skeletal complications were observed in 10 with secondary osteoporosis; 22 patients had elevated transaminase levels; two had hepatitis B reactivation. Neurological, cardiac and ocular manifestations were relatively rare. A higher incidence of LCs was observed in splenectomized than in nonsplenectomized patients: cGVHD -64% versus 13% (P = .003); endocrine abnormalities -91% versus 30.5%, (P = .001); elevated transaminase levels -73% versus 33% (P = .043); mortality -18% versus 2.7% (NS). CONCLUSIONS: LCs post-HSCT for HGP are common and heterogeneous. Etiology is multifactorial with iron overload (IO), class, splenectomy, age, chronic GVHD, and corticosteroid (CS) treatment. Our data may help build follow-up guidelines to limit, detect, and treat any LCs and improve quality of life.

Delayed spontaneous remission in a child with primary acquired chronic pure red cell aplasia.

The acquired form of pure red cell aplasia (PRCA) presents either as an acute self-limited disease, predominantly seen in children, or as a chronic illness more frequently seen in adults with rare spontaneous remissions. A 14-year-old boy presented with pallor, without hepatosplenomegaly, jaundice, lymphadenopathy, petechiae, or any other apparent abnormalities. Isolated anemia in the presence of normal white cell and platelet counts with a marrow of normal cellularity and absence of erythroblasts but normal myeloid cells and megakaryocytes revealed the diagnosis of PRCA. All possible investigations excluded secondary causes of PRCA. The patient required packed red cell transfusions every 2 to 3 weeks. He failed therapy with intravenous immunoglobulin, corticosteroids, cyclosporine A plus corticosteroids, antithymocyte globulin, anti-CD 20 (rituximab), and erythropoietin (EPO). He showed a severe, resistant, and transfusion-dependent PRCA. Spontaneous remission with normal hemoglobin and reticulocyte levels was dramatic 6.5 years after the diagnosis of PRCA and 3.6 years after his last treatment.

Multi-drug-resistant tuberculosis in a patient presenting with bilateral gluteal abscesses and right leg swelling with end-stage renal disease.

This is a 40-year-old woman with end-stage renal disease (ESRD). She received empirical treatment for presumed TB of the right knee joint. The arthritis and the constitutional symptoms resolved despite the patient's poor compliance. One year later, she presented with TB abscesses involving the upper end of right tibia and bilateral gluteus muscles, which proved to be resistant to isoniazid, rifampicin and ethambutol. The patient was successfully treated with pyrazinamide, ofloxacin and amikacin.

Correction of hemodialysis anemia is associated with significant increase in serum concentration of IGF-I in patients treated with erythropoietin: a randomized controlled study.

BACKGROUND: The effect of erythropoietin (EPO) therapy on the serum level of IGF-I among hemodialysis patients is debated. The aim of this study is to study the effect of EPO on the erythropoiesis and the change of serum level of IGF-I among adequately hemodialyzed patients. PATIENTS AND METHODS: Forty patients (25 males and 15 females) who had an adequate level of both hemodialysis and nutrition were randomly allocated into two equal groups. Besides parenteral iron, the first group of patients received a conventional EPO dose regimen of 2000 U subcutaneously (SC) thrice weekly, the second group of patients remained on parenteral iron and ranked as a control group. The patients were subjected to thorough laboratory investigations. IGF-I concentration was measured before and at the end of the study. RESULTS: Both groups were comparable in their demographic, laboratory, dialysis level, and nutritional status. There was no statistical differences in hemoglobin, hematocrit %, iron store indices and serum level of IGF-I at the study entry. We found a significant rise of both hemoglobin and hematocrit as well as IGF-I serum level in the EPO group at the end of the study in comparison to their values at the starting points in comparison to the control group (P< 0.001). CONCLUSION: Erythropoietin therapy enhances erythropoiesis and modulates the serum concentration of IGF-I.

Parenteral iron therapy in treatment of anemia in end-stage renal disease patients: a comparative study between iron saccharate and gluconate.

BACKGROUND: Anemia in hemodialysis patients is a complex syndrome. The impetus of this study was to assess the safety and efficacy of iron saccharate complex (ISC) and sodium ferric gluconate complex (SFGC) in treating anemia in hemodialysis patients. METHODS: Forty-eight adult anemic patients of both genders (33 males and 15 females) who had an adequate level of both hemodialysis and nutrition status and received neither EPO nor parenteral iron therapy during the preceding 6 months were randomized to 2 groups. The first group comprised 22 patients who were treated with parenteral ISC, 100 mg twice weekly for 2 months and once weekly thereafter. The second group included 26 patients who received SFGC, 62.5 mg twice weekly for 2 months and once weekly thereafter. The patients were followed up for 6 months. RESULTS: This head-to-head study showed that iron stores were adequately repleted by the use of both drugs. Repletion of iron stores was associated with a significant rise in both hemoglobin and hematocrit in both groups at the end of the follow-up period in comparison to their initial values at the start of the study (p < 0.001). Both parenteral iron therapy preparations were tolerated without a statistical difference between both groups. CONCLUSION: This head-to-head study confirmed that both parenteral iron preparations are effective for adequate repletion of iron stores and constituted a step forward in the management of anemic hemodialysis patients without noticeable adverse effects related to the administration of both iron preparations.

Paediatric cerebral sinus vein thrombosis. A multi-center, case-controlled study.

The etiology and pathophysiology of cerebral sinus venous thrombosis (CSVT) in the paediatric population is still poorly understood, and the role of thrombophilic risk factors remains to be elucidated. In our multi-center case-controlled study we studied 46 patients with CSVT diagnosed from April 1996 to December 2003, consecutively referred for thrombophilia work-up. The results of thrombophilia screen were compared to 112 healthy paediatric controls. Anticoagulant therapy was applied according to treating physicians' decisions, and all cases were prospectively followed for a median of 4.1 years. Of 46 children, 8 had CSVT diagnosed in the neonatal period and therefore were analyzed separately. The prevalence of single thrombophilia markers and combinations of thrombophilic risk factors were similar among cases and controls. Among children with CSVT co-morbid systemic illness was present in most patients at diagnosis. Seven out of 8 children with idiopathic CSVT had thrombophilic risk factors as compared to 31/38 patients with co-morbid conditions. Anticoagulation was initiated in most patients, 11/46 patients continued therapy for a total of one year or more post event. Neither clinical presentation nor initial treatment decisions were affected by presence of thrombophilic risk factors in our study group. Thrombophilia prevalence was not increased in children with CSVT as compared to controls, however thrombophilia was more frequent among children with idiopathic CSVT. Thus, those selected patients would benefit most from thrombophilia work-up, required for long-term therapy considerations.

Immunohistochemical expressions of uPA and its receptor uPAR and their prognostic significant in urinary bladder carcinoma.

BACKGROUND: The authors found previously that plasma levels of urokinase-type plasminogen activator (uPA) and its receptor (uPAR) were elevated in patients with bladder carcinoma and were associated with features of biologically aggressive disease. The present study has been performed to analyze the expressions of two antigens by immunohistochemical staining in bladder transitional cell carcinoma. PATIENTS AND METHODS: The tumors from 72 men and 28 women with a mean age 46.15 years (range 30-67 years) were examined. Paraffin sections of 5 microm thickness were prepared for immunohistochemical staining of uPA and uPAR antigens. Age, sex, tumor grade and stage, DNA ploidy, lymph node status, and metastases were evaluated in relation to outcome. Univariate and multivariate analysis of survival were performed. RESULTS: The overall 5-year survival was 66%. Thirty six and 46 cases were positive for uPA and uPAR expressions, respectively. In univariate analysis, tumor stage, lymph node status, metastases, uPA and uPAR have a significant impact on the survival for these patients. In a Cox proportional hazard model, uPAR sustained its significant impact on survival. CONCLUSIONS: These findings suggest that uPAR is an independent additional prognostic factor in patients with transitional cell carcinoma of the bladder.