Saranya Buppajarntham

Spontaneous coronary artery dissection following a preeclampsia pregnancy

1. IntroductionSpontaneous coronary artery dissection (SCAD) is an uncommoncause of acute myocardial infarction. The dissection of tunica intima ormedia,formingofhematomaunderneatharterialwallandcompressingthe true coronary lumen leads to myocardial infarction of distal cardiactissue. Thehigh suspiciousindexshouldmakein young female withoutany cardiovascular risk, especially in peripartum period. Emergent cor-onaryangiogramfollowedbyintravascularultrasoundisrecommendedfor diagnosis; however optimal treatment is still inconclusive [1].

Extensive loculated ascites in hepatic amyloidosis

Context: Amyloidosis is a disease of extracellular deposition of misfolded proteinaceous subunits, which could be systemic or localized disease. Though hepatic amyloidosis was not uncommon in autopsy series, most cases of hepatic amyloidosis were asymptomatic. Ascites, jaundice, portal hypertension, and gastrointestinal bleeding from esophageal varices were reported in literature. Case report: A 42-year-old man with end-stage renal disease on hemodialysis and recent small bowel obstruction presented with chronic abdominal pain. Computed tomography of abdomen and pelvis showed extensive loculated ascites and multiple small bowel loops tethered to adhesions and hepatomegaly. Finally, hepatic venography and liver biopsy confirmed hepatic amyloidosis with portal hypertension. The patient was waiting for liver transplant for definite treatment. Conclusion: We report a rare case of hepatic amyloidosis with prior small bowel obstruction presented with extensive loculated ascites and multiple small bowel loops tethered to adhesions.

Sigmoid volvulus: is it related to Marfan syndrome?

To the Editor, We report a case of a 65-year-old male, who presented with abdominal distention for 1 day, with past medical history of diabetes, hypertension, atrial fibrillation, cerebrovascular accident with residual right hemiparesis, and mild expressive aphasia. He reported of having diffuse abdominal distention, feeling nauseated with multiple episodes of non-bilious vomiting. He was afebrile with normal blood pressure. Abdominal exams showed distended abdomen with tympanic note on percussion. Rectal examination revealed emptied rectal vault. Abdominal X-ray showed marked dilatation of sigmoid loop, extending from the pelvis into the right upper quadrant, with coffee-bean appearance. Due to a high suspicionofsigmoidvolvulus,immediaterigidsigmoidoscopywas

Concomitant typhlitis and Clostridium difficile colitis developed after first R-CHOP chemotherapy in a non-Hodgkin lymphoma patient

Typhlitis or neutropenic enterocolitis (NEC) is a life-threatening condition that occurs in neutropenic patients. Early recognition is crucial owing to high death rate. We present a case of a 54-year-old man, diagnosed with non-Hodgkin lymphoma who received a first cycle of rituximab, cyclophosphamide, hydroxydaunorubicin (doxorubicin), oncovin (vincristine), prednisolone (R-CHOP) chemotherapy 10u2005days prior presenting. He developed fever, mucositis, watery diarrhoea and right lower quadrant pain with rebound tenderness. He also had neutropenia, with an absolute neutrophil count of zero. CT abdomen confirmed the diagnosis of typhlitis, demonstrating characteristic terminal ileum, caecal and right-sided colon involvement. Moreover, stool PCR was also positive for toxigenic Clostridium difficile. Therefore, the patient was diagnosed with concomitant typhlitis and C difficile-associated diarrhoea (CDAD). He was empirically treated with intravenous cefepime, intravenous metronidazole and oral vancomycin. His symptoms resolved in 10u2005days. This case illustrated a successful medical treatment of typhlitis in concomitance with CDAD.

Warburg effect associated with transformed lymphoplasmacytic lymphoma to diffuse large B-cell lymphoma

Warburg effect is a rare metabolic complication in hematologic malignancies, commonly presented with lactic acidosis and hypoglycemia. Mechanism explained by abnormality of mitochondrial oxidative phosphorylation in cancer cells and energy production is mostly dependent on anaerobic respiration or glycolysis pathway to meet large tumor demand. We present a case with history of lymphoplasmacytic lymphoma and Waldenstörm macroglobulinemia, partial response to chemotherapy. Lymphoplasmacytic lymphoma transformed to diffuse large B-cell lymphoma, which is aggressive and rapid progression, leading to Warburg effect. Patient developed more than 10-cm retroperitoneal mass less than 1 year, and his symptoms were progressively worsening within 3 weeks. Warburg effect represents poor prognosis no matter with or without hypoglycemia. Treatment of choice is cytoreduction with early chemotherapy. Our patient died 2 days after Warburg effect occurred.

Tumor-like pyogenic liver abscess caused by Klebsiella pneumoniae in diabetes

We have read a very interesting case reported by Williams et al. [1] ‘‘Occult pyogenic liver abscess in an adolescent with type 2 diabetes’’ which described a case of Klebsiella liver abscess (KLA) in a poorly controlled diabetic with an initial presentation of fever of unknown origin. In this letter, we would like to highlight another atypical presentation of KLA in a diabetic patient that presented as a tumor-like liver mass. A 63-year-old Vietnamese male with diabetes had a kidney biopsy 1 month prior to admission for evaluation of nephrotic syndrome that was complicated by the development of a kidney hematoma. Computed tomography (CT) of abdomen was performed to reassess the kidney hematoma when an ill-defined multicystic liver mass was incidentally discovered (Fig. 1, panel a–c). The patient had stable vital signs and was afebrile without any evidence of abdominal pain or leukocytosis. Since his blood cultures remained negative, a necrotizing tumor mass was the most likely diagnosis at that point. Additionally, the pathologic report of the kidney biopsy showed nodular diabetic glomerulosclerosis. The patient was discharged home to be followed up with tumor marker tests and further evaluation as an outpatient. A month after discharge, the patient returned with significant fatigue and anemia (hemoglobin drop from 7 to 5 g/dl) accompanied by mild right-sided upper abdominal pain. On exam, his vital signs were stable, he was afebrile, and well-oriented but looked pale and chronically ill. An abdominal exam revealed hepatomegaly with mild tenderness localized in the right upper quadrant. Tumor marker levels from the previous admission were within normal range (alpha fetoprotein level: 1.4 ng/ml and CA19-9: 32 U/ml). Laboratory results were as follows: white blood cell count 10 9 10/mm (N 80 %, L 9 %), alkaline phosphatase 169 IU/l, total bilirubin 0.2 mg/dl, direct bilirubin 0.2 mg/dl, albumin 1.4 g/dl, globulin 4.7 g/dl, aspartate transaminase 27 IU/l, alanine transaminase 16 IU/l, international normalized ratio 1.1, blood glucose 130 mg/dl, and serum creatinine 2.6 mg/dl. Due to chronic kidney disease, a CT scan of the abdomen was performed without use of intravenous contrast that demonstrated an increase in the size of the liver mass (Fig. 1 panel d–f). The liver mass was growing gradually for over a month without any signs and symptoms of infection, which was again attributed to likely being a necrotizing tumor mass. There was a serious concern for chronic bleeding from the site of the tumor and the patient received a right-sided hepatic lobectomy for bleeding control and tissue diagnosis. Surprisingly, the pathology report revealed a liver abscess with chronic inflammation and the tissue cultures grew pansensitive Klebsiella pneumoniae. KLA is an emerging disease in North America, frequently found in the Asian population, diabetics, and patients with chronic kidney disease [2]. K. pneumonia was isolated in 7–27 % of pyogenic liver abscess in USA but only a few cases of metastatic infection have been reported such as endophthalmitis, lung abscess, and meningitis. The incidence is higher in Taiwan, Japan, and south-east Asia. Common presentations are fever, abdominal pain, and leukocytosis which were initially absent in our case and misled us to pursue for liver malignancy. The findings suggestive of KLA on CT or ultrasonography are the formation of gas within the abscesses, pneumatosis in the biliary tree, an irregular abscess border, and an overall solid appearance with internal necrotic debris. Since several hepatic neoplasms or metastatic liver diseases S. Buppajarntham (&) M. Shah P. Junpaparp Internal Medicine Department, Albert Einstein Medical Center, 5501 Old York Road, Philadelphia, PA 19141, USA e-mail: BuppajaS@einstein.edu

A rare complication of a common stress test

In 2008, regadenoson, a selective adenosine2A (A2A) receptor agonist, was approved by the US Federal and Drug Administration for use as a pharmacologic stress agent in myocardial perfusion studies. By stimulating A2A receptors in coronary smooth muscle, it can increase coronary blood flow by 2.5-fold or greater. Previous data showed non-inferiority of regadenoson in detecting reversible myocardial ischemia, compared to adenosine. Given less serious adverse effects, being better tolerated and easily administered, regadenoson has been widely used for myocardial perfusion imaging. As adenosine receptors have many sub-types and are located in multi-organ systems, regadenoson can cause various adverse effects, including bronchospasm, atrioventricular block, or hypotension. However, adverse effects on the central nervous system are rarely reported. As adenosine receptors (A1 and A2A receptors) play a major role in neuron-glial cells interaction, regadenoson can provoke seizure through A2A receptor activation. We hereby report a case of regadenoson associated-seizure and review seizure mechanism. This may raise more concern for a rare serious adverse effect of regadenoson which should be taken into consideration when selecting cardiac stress modalities. <Learning objective: Regadenoson can provoke seizure through central A2A receptor activation. This should be taken into consideration when selecting cardiac stress test modalities, particularly in patients with known seizure disorder or history of organic brain disease.>.

THE MAGNITUDE OF CARDIOVASCULAR CHANGE IN CIRRHOTIC CARDIOMYOPATHY IN PREDICTING OF HEPATORENAL SYNDROME

Cirrhotic cardiomyopathy describes a constellation of cardiovascular abnormalities that can be present in cirrhotic patients. There remains a scarcity of data studying the relationship between cirrhotic cardiomyopathy and hepatorenal syndrome (HRS). We sought to identify cardiovascular abnormalities

A Report of Congestive Myelopathy as a Result of Chronic Chylous Reflux Syndrome

Dear Editor, n nWe would like to share an interesting case of congestive myelopathy involving a 26-year-old female with chronic gynecological chylous reflux disease who presented with bilateral lower extremities weakness, decreased sensation, and urinary incontinence of 2 months duration. A medical review revealed chronic vaginal discharge and lower-extremity swelling due to chronic chylous reflux disease. Upon examination, the patient had normal muscle strength in the upper extremities, motor power grades of 4 in the left lower extremity (LLE) and 1 in the right lower extremity (RLE), and loss of rectal tone. Sensory examination revealed decreased touch and vibratory sensation in both lower extremities. All cranial nerve and cerebellar functions were intact. Spinal MRI revealed T2-weighted hyperintense signals and atrophy of the thoracic cord with enhancing curvilinear T2-weighted hypointense signals within the thecal sac and flow voids anterior and posterior to the conus medullaris (Fig. 1). The findings were suggestive of a dural arteriovenous malformation (AVM). Surprisingly, subsequent spinal angiography did not reveal any evidence of either a dural AVM or a fistula. We therefore diagnosed this patient with congestive myelopathy secondary to venous drainage dysfunction as a result of chylous reflux syndrome. n n n nFig. 1 n nThoracic spine T2-weighted MRI. Sagittal view (A) and cross-sectional view (B) showing hyperintense signal and atrophy of the thoracic spinal cord (arrows). n n n nPrimary chylous reflux syndrome is a rare lymphatic disorder that is characterized by retrograde flow of chyle from its normal route. Hypothesized pathophysiologies include an incompetent lymphatic valvular system, lymphatic hypoplasia, and dilated incompetent megalymphatics. However, the definite pathophysiology remains unclear.1,2 Approximately 20 cases of primary chylous reflux syndrome have been reported during the past century. The common manifestations are lymphedema, chyluria, and chylous genital discharge. Some patients may experience complications such as chylothorax, chyloperitoneum, and chylopericardium.3 However, neurological deficits have never been reported in association with this condition. n nThis patient had been diagnosed with primary chylous reflux syndrome as a young child, and was conservatively managed with dietary control. At the age of 26 years she gradually developed venous congestive myelopathy or Foix-Alajouanine syndrome. Given that the most common cause of venous congestive myelopathy is spinal dural arteriovenous fistula, extensive investigations including MRI/MRA and spinal angiography were conducted.4 The results were negative for concurrent vascular malformations. To date, only 14 cases of venous congestive myelopathy without concurrent vascular malformation have been reported,5 none of which had underlying chylous reflux syndrome. Chyle is normally absorbed through the lacteals, drains into the thoracic duct, and empties into the venous system. In patients with chylous reflux disorder, the retrograde flow of lymph may contribute to retention of lymph within the pelvic cavity, with efflux into the vagina, causing vaginal discharge. These can ultimately lead to the development of aberrant vascular channels and can increase the lymph absorption through the venous system, as evidenced in the present patient by increased lower-extremity swelling. This can result in chylous reflux disorder eventually involving the spinal venous plexus or Batson venous plexus and causing chronic venostasis, contributing to venous congestive myelopathy. n nThe treatment of choice for primary chylous reflux disorder remains lymphangiography- or lymphoscintigraphy-guided surgery. Removal of the pelvic megalymphatics can theoretically redirect the chylous lymphatic flow toward the thoracic duct.6 However, there is no recommendation regarding the treatment of venous congestive myelopathy resulting from chylous reflux syndrome. Our patient has been refusing surgery since she was young, and at this admission again selected conservative treatment with rehabilitation. The paraplegia was slightly improved in approximately 8 weeks (motor power grades: 5/5 in LLE and 2/5 in RLE) and the patient could finally ambulate with crutches.

STEMI complicated with serum sodium of 113 mmol/L from syndrome of inappropriate antidiuretic hormone secretion: How worse could it be?

Hyponatremia commonly occurs in acute coronary syndrome and has been recognized as a worse prognostic indicator in patients with ST-segment elevation myocardial infarction (STEMI). However STEMI with preexisting hyponatremia from syndrome of inappropriate antidiuretic hormone secretion (SIADH) has never been described in the literature. We describe a case of 59-year-old woman who presented with STEMI and received emergent percutaneous coronary intervention who also had SIADH with the lowest serum sodium measurement of 113xa0mmol/L. Initially, she was treated with hypertonic saline to reduce central nervous system complications. Then, vasopressin receptor 2 antagonist and demeclocycline were started as well as fluid restriction and salt tablet. Her sodium level and clinical symptoms improved. Subsequently, we found cavitary right upper lung mass and a biopsy report revealed small cell lung cancer as a cause of SIADH. Severe hyponatremia from SIADH complicated with STEMI could potentially have reduced adverse outcomes by normalizing sodium level through vasopressin receptor 2 antagonist or demeclocycline. .