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ABSTRACTS FROM THE 2020 LOUISIANA ANNUAL ACP MEETING
CONTENTS
JOURNAL EDITORIAL STAFF
EDITOR D. Luke Glancy, MD
ASSOCIATE EDITOR L.W. Johnson, MD
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A CASE OF THYROTOXICOSIS INDUCED PERIODIC PARALYSIS
CHIEF EXECUTIVE OFFICER JeffWilliams
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A CASE OF REGORAFENIB INDUCED PANCREATITIS: A RARE ADVERSE EVENT OF TYROSINE KINASE INHIBITORS
JOURNAL EDITORIAL BOARD Vice Chair, K. Barton Farris, MD Secretary/Treasurer, Richard Paddock, MD
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RESOLVING HEMI’S, INDUCING STEMI’S
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HEMORRHAGIC BULLAE AS A PRESENTATION OF PHLEGMASIA CERULEA DOLENS WITH MAY-THURNER SYNDROME IT’S NOT ALWAYS TUBERCULOSIS! TREE-IN-BUD OPACITIES LEADING TO A DIAGNOSIS OF PULMONARY SARCOID
Anthony Blalock, MD D. Luke Glancy, MD L.W. Johnson, MD Fred A. Lopez, MD
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9 HEPATIC SCHISTOSOMIASIS FOUND IN EXPLANTED LIVER POST-TRANSPLANT 10 ACCUSTOMED TO INFECTION 11 UNUSUAL PANCREATIC MALIGNANCY 12 RECURRENT RHABDOMYOLYSIS 13 I DON’T WANT TO SEE A DOCTOR; THEY FREAK OUT LOOKING AT MY LABS: A CASE OF MAY-HEGGLIN SYNDROME 14 A NEW HAND FINDING THAT COULD POINT TOWARD LIVER DISEASE 15 SOCIAL MEDIA AND A CASE OF ANAPHYLAXIS 16 A CASE OF PURTSCHER’S RETINOPATHY SECONDARY TO ACUTE ALCOHOL INDUCED PANCREATITIS 17 A RARE PRESENTATION OF DEVIC’S DISEASE 18 ACUTE CORONARY SYNDROME FOLLOWING THERAPEUTIC EPINEPHRINE FOR ANAPHYLAXIS. 19 THE ABDOMINAL COCOON SYNDROME: A CASE REPORT 20 HONEY, I SHRUNK THE LUNGS 21 PSEUDOANEURYSM OF THE MITRAL-AORTIC INTERVALVULAR FIBROSA DUE TO MITRAL ANNULAR CALCIFICATION 22 WOBBLE, WOBBLE – A PARKINSON’S DISEASE MIMICKER 23 DELAYED REACTION AND ACUTE INTERSTITIAL NEPHRITIS INDUCED BY PIPERACILLIN: A CASE REPORT 24 HEPATOCELLULAR CARCINOMA (HCC) OUTCOMES IN A PUBLIC HOSPITAL SETTING WITH INCREASED ACCESSIBILITY TO PROCEDURAL BASED THERAPY 25 A CASE OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN THE SETTING OF CLASSIC HODGKIN LYMPHOMA, HIV, AND EBV 26 ENOXAPARIN INDUCED PITUITARY APOPLEXY –AN UNRECOGNIZED RISK! 27 PROFOUND NEUTROPENIA AS A SEQUELA OF PRIMARY EBV INFECTION 28 STEROID-INDUCED ACUTE PORPHYRIA: A RARE CASE OF UNEXPLAINED ABDOMINAL PAIN
LSMS 2021 BOARD OF GOVERNORS OFFICERS President, William Freeman, MD Past President, Katherine Williams, MD President-Elect, John Noble, Jr., MD Vice President, George Ellis, Jr., MD Speaker of the House, T. Steen Trawick, MD Vice Speaker, R. Reece Newsome, MD Secretary/Treasurer, Richard Paddock, MD Chair, COL, David Broussard, MD
COUNCILORS District 1 Member, Vacant
District 1 Alternate, Maurice Sholas, MD District 2 Member, Robert Chugden, MD District 3 Member, Allen Vander, MD District 4 Member, Richard “Rick”Michael, MD District 5 Member, Gwenn Jackson, MD District 6 Member, Michael Roppolo, MD District 7 Member, Brian Gamborg, MD District 8 Member, Lance Templeton, MD District 9 Member, Andy Blalock, MD District 10 Member, Nicholas Viviano, MD District 10 Alternate, James Connolly, MD SECTION REPRESENTATIVES Senior Physician Member, Marcus Pittman, III, MD Senior Physician Alternate, Donnie Batie, MD Young Physician Member, Amberly Nunez, MD Resident/Fellow Member, Blake Denley, MD Medical Student Member, Brittany Wagner Employed Physician Member, Matthew Giglia, MD Private Practice Physician Member, Vicki Steen, MD
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Each year medical students from the four medical schools and residents from the eight Internal Medicine training programs in Louisiana are invited to submit abstracts for the Annual American College of Physicians (ACP) Meeting of the Louisiana Chapter. The content of these abstracts includes clinical case vignettes or research activities. The abstracts have all identifying features removed (i.e., names, institutional affiliations, etc.) before being sent to physician judges. This year a total of 132 abstracts were submitted for the meeting. Each judge scores each abstract independently and then the scores from all judges are averaged and ranked. This year we are excited
to be able to publish the 3 most highly ranked abstracts presented at this year’s competition that were selected for oral presentations. An additional 22 abstracts presented as posters were selected
by judges for publication. All abstracts (3 oral and 22 poster) were presented at the Associates Meeting held at The Louisiana Cancer Research Center in New Orleans on March 6, 2020. We would like to thank the Journal of the Louisiana State Medical Society and appreciate its efforts to publicize the hard work of these trainees.
Shane Sanne, DO, FACP Chair, Louisiana Associates Liaison Committee
Lee S. Engel, MD, PhD, FACP Past Governor, Louisiana Chapter ACP
Angela Johnson, MD, FACP Governor, Louisiana Chapter ACP
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A CASE OF THYROTOXICOSIS INDUCED PERIODIC PARALYSIS R. Poudel MD, N. Sahami Department of Internal Medicine, Leonard J. Chabert Medical Center, Houma, LA
INTRODUCTION: Thyrotoxicosis brings to mind many different symptomatic complications. As thyroid hormones essentially effects every cell in the body, it’s important to consider some of the potentially fatal complications.
CASE: A 28 year old man with hyperthyroidism and atrial fibrillation presented with acute onset paralysis and palpitations one hour prior to arrival. He had been evaluated due to malaise, palpitations, cough, and congestion and treated with dexamethasone 80mg IM and IVF. He later awoke from sleep and was unable to move his extremities secondary to diffuse weakness. He reported noncompliance with his home medications that included levothyroxine, methimazole and atenolol. His examwas positive for a diffuse, non-nodular goiter, tachycardia with an irregularly irregular rhythm and 2/5 strength in all extremities. Relevant lab studies showed K<2.0, Mg 1.4, TSH 0.015, T4 >5.00. EKG revealed atrial fibrillation, incomplete right bundle branch block and a QTc of 706 ms. Initial management included prophylthiouracil hydrocortisone , Lugol’s solution, cholestyramine, and magnesium supplementation which resolved the EKG changes, electrolyte disturbances and his symptoms by day two. He was discharged with counselling on triggering factors for periodic paralysis secondary to hyperthyroidism. DISCUSSION: Thyrotoxicosis and thyroid storm are dangerous complications of hyperthyroid states which can be life threatening. With the overall prevalence of hyperthyroidism in the U.S. at around 1.3%, it’s important to recognize and educate patients on the complications of ineffective treatment leading to acquired hypokalemic periodic paralysis. Specific signs of this acquired condition include sudden generalized weakness with preserved consciousness. The mechanism is currently not well understood but it’s proposed that thyroid hormone stimulates beta-adrenergic receptors which increase NA/K ATPase activity on skeletal muscles. This shifts potassium into cells causing hypokalemia resulting in hyperpolarization of the muscle membrane and decreased “excitability” of the muscle fibers leading to paralysis. Moreover, a hyperthyroid state also potentiates the hypokalemic action of epinephrine and insulin which also activates Na/K ATPase pump contributing further to hypokalemia. Keys to decreasing morbidity and mortality lies in the early detection of hyperthyroid induced hypokalemia with mindful correction of potassium as these patients are susceptible to rapid potassium shifts which may precipitate severe hyperkalemia.
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A CASE OF REGORAFENIB INDUCED PANCREATITIS: A RARE ADVERSE EVENT OF TYROSINE KINASE INHIBITORS P. M. S. Justiniano, MD Department of Internal Medicine, Leonard J. Chabert Medical Center, Houma, LA INTRODUCTION: Regorafenib is a multi-target tyrosine kinase inhibitor that may be indicated for treatment of patients with metastatic colorectal cancer, gastrointestinal stromal tumor or hepatocellular carcinoma. Acute pancreatitis is a rare adverse event of tyrosine kinase inhibitors and has been reported in <1.6% of patients during clinical trials for treatment of hepatocellular carcinoma with regorafenib. CASE: A 56 year old woman with metastatic colorectal cancer with prior partial colectomy and resection of recurrence, presented with abdominal pain four days after beginning therapy with regorafenib. Abdominal pain was described as sharp, epigastric, 9/10 in intensity, radiating to the back, and worsened with meals. She had decreased appetite and oral intake due to nausea and vomiting. She reported multiple episodes of non-bloody, non-bilious emesis. Additionally, she had loose stools without melena or hematochezia. She denied prior history of similar episodes. She did not have a history of gallstones, abdominal trauma, sick contacts or family history of pancreatitis. Denied, smoking, alcohol use or illicit drug use. Physical examination was pertinent for epigastric tenderness, without rebound, guarding, murphy’s sign or organomegaly. Laboratory studies were pertinent for elevated lipase >1000 U/L. Triglycerides, calcium and liver function tests were within normal limits. CT scan of the abdomen and pelvis with contrast showed pancreatic and peripancreatic edema. Treatment for acute pancreatitis was initiated with Lactated Ringers, anti-emetics and diet was advanced as tolerated. Regorafenib was discontinued and her symptoms improved on the third day of hospitalization, the patient was discharged in stable condition. DISCUSSION: Acute pancreatitis is most commonly precipitated by gallstones, alcohol use or other causes such as hypertriglyceridemia, hypercalcemia, or specific medications, which were ruled out in this patient. Regorafenib was considered the cause of this patient’s acute pancreatitis, given the close temporal relationship between the initiation of therapy while other causative factors were excluded. Per literature review, pancreatitis as a suspected adverse reaction of regorafenib in a patient with metastatic colorectal adenocarcinoma has not been previously reported. Acute pancreatitis is a potentially rare severe adverse event that may occur in patients starting therapy with tyrosine kinase inhibitors such as regorafenib.
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RESOLVING HEMI’S, INDUCING STEMI’S G. Devany MD, R. Eschete II MS3, J. Godke MD Department of Medicine, Baton Rouge General Medical Center, Baton Rouge, LA INTRODUCTION: Post thrombolysis acute myocardial infarction (AMI) is a rare phenomenon but requires early recognition. Concern for an AMI should not delay or alter the management of acute ischemic stroke. CASE: A 78-year-old man with hypertension, hyperlipidemia, aortic valve replacement, right-sided carotid endarterectomy, and single vessel coronary artery bypass presented with a two-hour history of sudden onset left-sided weakness and left- sided facial droop. Physical exam revealed normal vital signs, a left-sided facial droop, left arm strength of 1/5 and left leg strength of 0/5. The patient’s National Institute of Health Stroke Scale score was 10. The initial Computed Tomography (CT) of the head was normal. The consulting neurologist diagnosed a small vessel distribution infarction causing pure motor deficits. Three hours post-onset, thrombolysis with tissue plasminogen activator (tPA) was initiated. Then the patient complained of chest tightness with substernal pain and he was noted to be diaphoretic and in obvious distress. An EKG showed ST elevations in leads II, III, aVF with ST depressions in leads I and aVL suggestive of posterior/inferior myocardial infarction. The consulting cardiologist confirmed the STEMI diagnosis and advised against any further anticoagulation or percutaneous coronary intervention due to tPA contraindications. The STEMI was medically treated. On the following day, a head-CT revealed a subacute infarction of the right thalamus and posterior limb of the internal capsule. DISCUSSION: Lysis of a thrombus overlying cholesterol containing atherosclerotic plaque may destabilize the plaque. This has been postulated to cause distal vessel cholesterol crystal embolization, not dissolved by thrombolytics. Patients with cerebrovascular and coronary artery disease might experience unintended plaque destabilization in the coronary vascular system following thrombolysis. Another explanation is that thrombolytics cause a disruption of an intracardiac thrombus and subsequent embolization to the distal coronary arteries. One report identified intracardiac thrombus in 26%of patients experiencing transient ischemic attacks by transesophageal echocardiography (TEE). Another study found a cardiac thrombus present in 2.7% of patients who had received tPA for an acute ischemic stroke. In both reports, the presence of intracardiac thrombus was associated with poor prognosis.
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HEMORRHAGIC BULLAE AS A PRESENTATION OF PHLEGMASIA CERULEA DOLENS WITH MAY-THURNER SYNDROME H. Bernstein BS, M. La MD, J. Tien MD, H. Shi MD, S. J. Cossich MD Department of Medicine, Tulane Health Sciences Center, New Orleans, LA INTRODUCTION: May Thurner syndrome is a severe variant of venous thromboembolism (VTE) where the iliac vein is extrinsically compressed by the arterial system, vertebrae, or other bony structures. Symptoms include extremity pain, swelling, and skin discoloration and ulceration due to venous insufficiency. Phlegmasia Cerulea Dolens (PCD) is a severe complication of VTE where compartment syndrome can occur due to the swelling of the extremity. CASE: A 54-year old man with dementia and chronic kidney disease presented with bilateral lower extremity swelling and blistering. Examination revealed multiple tense fluid filled bullae in medial lower leg to the foot on his right and left lower extremities. They were primarily located in the distribution of saphenous veins. Femoral pulses were palpable, dorsalis pedis and posterior tibial pulses were only observed by Doppler ultrasound. A venous Doppler ultrasound study showed extensive occlusive thrombus in the left common femoral, superficial femoral, greater saphenous, and popliteal veins. A heparin dripwas initiated. Findings froma CT of the lower extremitywere concerning for phlegmasia dolens given extensive iliofemoral thrombosis and May-Thurner syndrome. Vascular surgery performed urgent mechanical thrombectomy and stenting of the left common iliac, external iliac, common femoral, and popliteal veins. Intravascular ultrasound (IVUS) confirmed aortic bifurcation compression on caval bifurcation as well as left common iliac vein compression between right common iliac and spine. He was discharged on apixiban. DISCUSSION: Skin findings from extensive VTE and PCD are often nonspecific. Marked swelling and cyanosis of the limb can occur. More often, techniques such as ultrasonography are performed to evaluate venous status. We demonstrate one of the few cases where acute on chronic VTE, including blockage of a large proximal vein, led to formation of large bullae and blistering. We hypothesized that these findings resulted from a change in venous return due to interstitial fluid hydrostatic pressure which lead to separation of epidermal cells. The treatment strategy initially involves anticoagulation, then subsequent angioplasty and stenting if applicable. Quick relief of the obstruction is key to avoiding future limb amputation.
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IT’S NOT ALWAYS TUBERCULOSIS! TREE-IN-BUD OPACITIES LEADING TO A DIAGNOSIS OF PULMONARY SARCOID J. Hong BA, J. Spraglin MD, H. Shi MD Department of Medicine, Tulane Health Sciences Center, New Orleans, LA INTRODUCTION: The “tree-in-bud” sign can be commonly caused by respiratory infections including that of mycobacterial, bacterial, and viral causes. The pattern of the tree correlates to an intralobular inflammatory bronchiole and the bud correlates to inflammatory filling in alveolar ducts. Pulmonary sarcoid can show nodules in a perilymphatic distribution, but not in this particular appearance. Thus, a high degree of suspicion is required to proceed to invasive testing such as bronchoscopy, especially in a mycobacterial tuberculosis rule-out. CASE: A 27-year-old homeless man with Brugada syndrome and implanted cardioverter defibrillator initially presented for acute onset of chest pain and cough for three days. EKG showed normal sinus rhythm with no signs of ischemia. His ICD was interrogated with no events evident. Labs showed troponin <0.015 (normal 0.015- 0.045), WBC 7.2 (4.5- 11.0), lactate 1.1 (0.9- 1.7 mmol/L), NT-Pro-B Natriuretic Peptide BNP 7 (0 - 450 pg/mL). He was about to be discharged when the results of his chest X-ray showed multifocal patchy airspace opacities. The patient endorsed a history of alcohol abuse, intranasal cocaine use, and vaping. He previously had been incarcerated and was a machinist at a shipyard. CT chest scan showed centrilobular tree-in-bud opacities and mediastinal lymphadenopathy. With concern for MTB, a bronchoscopy was performed with bronchoalveolar lavage (BAL) and endobronchial biopsy was performed. AFB, fungal, bacterial cultures were negative. T-spot, Mycobacterium tuberculosis (MTB) PCR from sputum and BAL were negative. Finally, pathology from the biopsy showed fibrosing non-necrotizing granulomatous pneumonitis consistent with sarcoid. Angiotensin converting enzyme (ACE) level was elevated at 127 (14- 82 U/L). DISCUSSION: Pulmonary sarcoidosis is often very difficult to diagnose, especially when a patient presents with nonspecific respiratory symptoms. In this patient, there was a broad differential on admission given his cardiac history of Brugada and nonspecific symptoms of cough. Ultimately, he fulfilled sarcoid criteria with tissue biopsy and exclusion of MTB. He will need to undergo further testing to rule out extrapulmonary sarcoid manifestations, such as cardiac sarcoid, as he is high risk given his genetic Brugada.
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HEPATIC SCHISTOSOMIASIS FOUND IN EXPLANTED LIVER POST-TRANSPLANT H. Alrowais MD 1 , O. Nnedu 2 1 Department of Medicine, Ochsner Clinic Medical Center, New Orleans, LA 2 Department of Medicine, Section of Infectious Disease, Ochsner Medical Center, New Orleans, LA INTRODUCTION: About 779 million individuals are at risk for Schistosomiasis. Due to travel and immigration, about 400,000 or more people with Schistosoma infection may live in the United States. Schistosomiasis can present acutely with nocturnal fever, myalgia, headache, and abdominal pain. Chronic infection develops over time, from an initial granulomatous inflammatory response to Schistosome eggs progressing to fibrosis. The initial host response is a delayed hypersensitivity with modest Th1 response, which is followed by a dominant Th2 mediated response producing inflammatory cytokines leading to granuloma formation. CASE: A 57-year-old Saudi Arabian man with ESRD secondary to membranous nephropathy (unsuccessfully treated with tacrolimus) was referred for a combined kidney and liver transplantation. Shortly after starting dialysis, he was diagnosed with NASH cirrhosis and referred for infectious disease pre-transplant consultation. He had anasarca, abdominal distension, and crackles at the lung bases. Serology was positive for CMV IgG, Strongyloides and Schistosoma IgG and seronegative for HAV, HBV, HCV andHIV. Hewas prescribed praziquantel for Schistosoma and ivermectin for Strongyloides before transplant. Pathologic evaluation of the explanted liver showed helminthes and ova consistent with the adult Schistosomiasis). The patient underwent a successful liver and kidney transplant but he passed away due to post-operative complications. DISCUSSION: The impact of tacrolimus on host immune response to parasite eggs is unclear. Tacrolimus has been shown to inhibit differentiation of T-cells to Th1 and Th2 subsets. In theory, use of tacrolimus would decrease host immune responses to the Schistosoma eggs thereby decreasing pathology. Early studies showed that adult Schistosoma parasite possess a Tacrolimus binding protein. Subsequent studies however showed that Tacrolimus had no in vivo or in vitro effect on adult parasite growth suggesting that Tacrolimus binding protein in Schistosoma species does not play a significant role in parasite survival. There is limited published data on solid organ transplantation and Schistosomiasis. Two observational studies showed no survival difference between transplant recipients with Schistosomiasis and those without. There have been three published cases of recurrent hepatic Schistosomiasis following liver transplantation but this didn’t lead to graft loss. Using organs harvested from individuals with Schistosomiasis did not affect transplant survival in these cases.
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ACCUSTOMED TO INFECTION J. Hanawi-Heintze MD, N. R. Sells MD Department of Medicine, University Hospital and Clinics, LSU Health, Lafayette, LA
INTRODUCTION: Coccidioides species are known to be endemic to the southwestern United States. Men in endemic areas are disproportionately affected, especially if there is exposure to dust.
CASE: A 68-year-oldmanwith COPD and chronic osteomyelitis of the sacrumpresented after being told of an abnormal lab results. Findings showed an elevated CRP of 20 and a white cell count of 17,000. The patient did not have any major complaints and review of systems was unremarkable other than baseline shortness of breath and dyspnea on exertion. He was found to be hypoxic and noted to have a complicated sacral abscess. The patient was started on broad spectrum antibiotics for his acute infection as well as oxygen for his hypoxia. A CT Thorax revealed increased fibrosis, a 3cm lucent area in the right lung apex consistent with a cavitary lesion, bibasilar consolidation and hilar lymphadenopathy. A full interstitial lung disease workup, gram stain, sputum culture, fungal culture, and acid-fast bacillus testing was unremarkable. Antibiotics were deescalated prior to discharge. One month later, fungal cultures grew Coccidioides immitis. He was in clinic where serology was drawn. The patient did admit to spending time experiencing transient homelessness in Arizona several years prior. Coccidioides IgG and IgM were positive and he was started on a 6 month course of Fluconazole. DISCUSSION: Manifestations of coccidioidomycosis can range from asymptomatic to very mild infections in approximately 50% of cases to clinical illness in 40-50% of cases. The incubation period is usually 1-3 weeks. In this case, it is difficult to determine whether the extent of his disease is secondary only to coccidioidomycosis or to a combination of coccidioides infection and another disease process. Coccidioides infection can manifest similarly to interstitial lung disease, pulmonary fibrosis, TB, or COPD exacerbations. In non-endemic areas, it is easily excluded from the differential. Our patient case most likely a more complicated and chronic course of infection rather than an acute process.
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UNUSUAL PANCREATIC MALIGNANCY J. Coe MD, A. Mahmood MD, F. Shaikh MD, R. Lee DO, R. Randive DO, S. Aggarwal MD, N. Salagundla MD Department of Medicine, University Hospital and Clinics, LSU Health, Lafayette, LA
INTRODUCTION: Typically, pancreatic malignancies arise from the exocrine portion of the pancreas. However, a subset of pancreatic cancers are derived from neuroendocrine cells, leading to the development of small cell cancers. These cells are present mainly in the gastrointestinal tract and lung.
CASE: A 58 year old man with hypertension and alcohol abuse presented endorsing severe lower back pain for 3 weeks. This pain radiated towards the abdomen and was described as a sharp that worsened with ambulation. CT abdomen and pelvis 1 week prior to presentation had shown a mass at the junction of the head and body of the pancreas. This CT also showed hepatic and pulmonary lesions concerning for possible metastases. A EUS was performed with biopsy of the pancreatic and a liver lesion CT thorax demonstrated multiple nodules consistent with metastases and necrotic hilar and mediastinal lymph nodes. MRI brain revealed supratentorial and intratentorial enhancing foci. The pathology results from both masses revealed poorly differentiated neuroendocrine carcinoma, small cell variant. He was discharged with pain control and urgent follow up in oncology clinic. DISCUSSION: While pancreatic neuroendocrine neoplasms are very rare (1-2% of pancreatic malignancies), their incidence in the U.S. has been increasing. These malignancies can present similar to small cell carcinoma of the lung. These cancers are graded based on mitotic count and Ki-67 index with higher levels indicating a higher grade and the more poorly differentiated the tumor. The mainstay of treatment is systemic chemotherapy. The prognosis for these patients tends to be poor with median survival time from diagnosis at less than 1 year. Malignant cells which are hormonally active (producing glucagon, insulin, or gastrin) tend to be discovered earlier, improving survival rates. Pancreatic small cell cancer tends to metastasize in a pattern similar to small cell lung cancer. As a result, patients with pancreatic small cell should have brain imaging as well as lung imaging to screen for metastatic lesions.
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RECURRENT RHABDOMYOLYSIS J. K. Khera, DO, J. Schouest MD, G. Ardoin MD Department of Medicine, University Hospital and Clinics, LSU Health, Lafayette, LA
INTRODUCTION: Rhabdomyolysis entails the destruction of skeletal muscles whichmay be asymptomatic or set the stage for fulminant renal failure. The common causes of rhabdomyolysis are extensive yet some patients possess a low threshold for supervening recurrent rhabdomyolysis. Such patients with recurrent episodes require testing to rule out acquired myopathies such as dermatomyositis and polymyositis, muscular dystrophy, and metabolic myopathies such as disorders of fat and carbohydrate metabolism, and mitochondrial function. CASE: A 30 year old man presented with body aches, dark urine, and malaise with a CK of 84,000. Five days prior to admission, he was tested positive for Influenza B. Remarkably this was the patient’s fourth episode in the past four years with the first case being a complicated course secondary to influenza B infection and the two subsequent cases being vague in nature. His employment during these times involved minimal exertion in colder temperatures. Given his predisposition, he received a muscle biopsy two years ago showing muscle strands consistent with typical rhabdomyolysis but the cause could not be established. Thyroid stimulating hormone, HIV, urine drug screens, anti-nuclear antibody, and myositis panels were non- diagnostic. He was evaluated by Nephrology, Endocrine, Rheumatology, and Neurology. Post discharge, he was scheduled for follow-up with rheumatology and blood samples were sent to the Lantern Project to test for neuromuscular diseases. DISCUSSION: Rhabdomyolysis stemming from influenza is surely a known cause but in this case, we are suspect an underlying muscle disorder as the root of illness because two of four episodes were not secondary to a viral illness. Furthermore, episodes that ensued from the influenza infection were out of proportion to the degree of viral illness. This case is perplexing because of the low threshold for recurrent muscle breakdown that has been twice unveiled by Influenza B, yet in the other two instances the true etiology remains a mystery despite thorough investigations. We felt that a referral to a myopathy specialist was warranted because an underlyingmetabolic or muscle storage disorder seems most likely to be encouraging recurrent rhabdomyolysis.
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I DON’T WANT TO SEE A DOCTOR; THEY FREAK OUT LOOKING AT MY LABS: A CASE OF MAY-HEGGLIN SYNDROME S. Jiwani MD, S. Guntupalli MD, R. P. Mansour MD Department of Internal Medicine, LSU-Health Sciences Center, Shreveport, LA. INTRODUCTION: May-Hegglin syndrome is a rare hereditary macro-thrombocytopenia caused by mutation of the MYH-9 gene encoding the heavy chain A of non-muscle myosin class II. We present a case that emphasizes the importance of awareness of rare disorders that could potentially avoid over-investigation in these benign conditions. CASE: 72-year-oldwomanwithhypertension, chronic kidney disease Stage 3, hiatal hernia, tobacco use presented for preoperative evaluation for cataract extraction. She was noted to have significant thrombocytopenia of 3K/µL along with elevated BUN and creatinine. She was emergently admitted for further evaluation. She denied any symptoms except for a prolonged history of easy bruising. Physical exam was unremarkable. She mentioned that her platelet count has always been low (<50K/µL) and refused to see physicians due to their anxiety about her abnormal labs. She was previously investigated for thrombocytopenia and had multiple diagnostic as well as therapeutic interventions including bone marrow biopsies, steroids, intravenous immunoglobulins without improvement. Her family history was consistent with low platelet counts for at least 3 generations. Repeat laboratory testing revealed an improved platelet count of 43 K/µL with elevated mean platelet volume (MPV). Peripheral blood smear showed large platelets, normal red and white blood cells without inclusion bodies. After carefully reviewing her history and laboratory findings, it was suspected that the patient may have a familial thrombocytopenia. Further genetic testing revealed an inherited MYH-9 mutation that is autosomal dominant. DISCUSSION: MYH-9 related disorders are characterized by macro-thrombocytopenia (due to premature release of platelets), often associated with glomerulonephritis, sensorineural deafness, cataracts and cytoplasmic inclusion bodies within leukocytes. Management is mainly conservative and directed towards prevention of iron deficiency anemia in young females. Use of desmopressin, in combination with tranexamic acid, is recommended in perioperative setting. Our case emphasizes the importance of history-taking skills that could potentially minimize further diagnostic or therapeutic interventions in this benign genetic disorder.
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A NEW HAND FINDING THAT COULD POINT TOWARD LIVER DISEASE A. Phelps MD, S. Gillet BS, C. Carlos BS, C. Talley MD, M. Maylin, MD Department of Internal Medicine, Tulane Health Sciences Center, New Orleans, LA
INTRODUCTION: Any patient presenting with an arterial thromboembolism (ATE) without history of recent trauma or intervention to the presenting limb should have a thorough work up. This case illustrates the importance of exhausting all possibilities to obtain the diagnosis
CASE: A 66-year-old man with recently treated hepatitis C, hypertension, and a 120-pack year smoking history presented with three days of progressively worsening gangrene of the digits without a history of trauma. He was cachectic with dusky skin and digits two through four on the right hand were cold bilaterally, with black discoloration noted distally on the second digit. Radial pulse was 2+ bilaterally. TSH, A1C, ABI andWBI were within normal limits. Autoimmune workup, including ANA, anticardiolipin, and beta2-glycoprotein I antibodies, returned negative. EKG and telemetry monitoring did not reveal any arrhythmias. Transesophageal echocardiogram had no evidence of valvular disease or vegetations. Pathology report from a skin biopsy of the right index finger noted thromboembolism with medium-sized vessel vasculitis. Malignancy workup began with a CT chest, which incidentally revealed three large hypoechoic liver masses, the largest measuring 9.6 x 7.5 cm, in addition to mediastinal and hilar lymphadenopathy. A diagnosis of hepatocellular carcinoma was made by IR guided liver biopsy. DISCUSSION: A thorough cardiac workup for a patient with ATE should be obtained as the majority of arterial embolisms that travel to the extremities originate in the heart. EKG and telemetry monitoring to assess for atrial fibrillation, echocardiogram to assess for valvular disease, endocarditis, myxoma, and left ventricular dysfunction, and risk stratification with hemoglobin A1C, lipid panel, and TSH should all be performed initially. If no source is identified, further workup should focus on causes of hyper coagulable states. Antiphospholipid syndrome should be excluded with testing for anticardiolipin and beta2- glycoprotein I antibodies. Malignancy should be considered as a possible etiology with age appropriate cancer screening as early stage cancer carries a significantly increased risk of ATE. Recognition of malignancy as a potential cause arterial thromboembolism could lead to earlier diagnosis and treatment.
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SOCIAL MEDIA AND A CASE OF ANAPHYLAXIS Dexter Matrana DO 1 , Gordon Myers MD 2 , Elizabeth Smith MD 2 , Sanjay Kamboj MD 2 Department of Medicine, 1 Internal Medicine Residency Program, 2 Section of Allergy/Immunology, LSU Health Sciences Center, New Orleans, LA INTRODUCTION: Alpha Gal Syndrome (Tick Induced Meat Allergy) is a rare cause of delayed anaphylaxis that develops weeks tomonths after a tick bite following ingestion of mammalian meats. CASE: A 28-year-old woman without significant past medical history presented to the Allergy/Immunology clinic with concerns of having alpha gal syndrome. Three months prior she was bitten by a tick while hiking in Northwest Georgia. She reports about four hours after ingestion of a pepperoni pizza, she woke up experiencing wheezing, shortness of breath, and hives all over her body. She took Benadryl and by the morning her symptoms had completely resolved. A few weeks later, she ate a cheeseburger, and approximately four hours later, developed similar symptom. Coincidentally, she heard a podcast about meat allergy and the symptoms described matched her symptoms, prompting her to seek medical evaluation. As her history and presentation were consistent with Alpha Gal syndrome, a specific IgE level to Alpha-Gal was ordered and found to be elevated at 46.3 IU/mL. A diagnosis of Alpha Gal syndrome was confirmed, and she was instructed to avoid beef, pork, lamb and other mammalian meats. DISCUSSION: The diagnosis of alpha gal was initially discovered in 2006. Approximately three months after a Lone Star tick bite, there is a immunoglobulin class switch that is thought to occur in the skin that leads to the development of an IgE antibody to alpha-1,3-galactose, a carbohydrate found on mammalian meats. Through the use of public awareness regarding tick bites, various news outlets and social media platforms have increased awareness of this rare disease. When produced correctly and factually accurate, social media can be a powerful tool to help raise awareness to rare diseases.
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A CASE OF PURTSCHER’S RETINOPATHY SECONDARY TO ACUTE ALCOHOL INDUCED PANCREATITIS Ryley McPeters MD, Tyler Thorne MD, Tejas Joshi MD, Mathoula Bilalis MS-4, Lance Turkish MD, John Amoss, MD, Stephen Landreneau, MD Department of Medicine, LSU Health Sciences Center, New Orleans, LA INTRODUCTION: Purtscher’s retinopathy is a rare condition first described following intracranial trauma. This occlusive microvasculopathy is characterized by sudden visual loss with multiple areas of retinal whitening in the posterior pole of the eye. Similar findings have been seen in atraumatic patients termed Purtscher-like retinopathy (PuR). PuR has been described with acute pancreatitis and connective tissue diseases. PuR, associated with systemic disease, develops through the activation of the complement cascade which leads to leukoembolization of the retinal precapillary arterioles and cause the clinical appearance of Purtscher’s retinopathy. CASE: A 28 year-old woman with a history of alcohol abuse and multiple of admissions for acute pancreatitis presented with chief complaint of emesis and abdominal pain. Physical exam, initial blood tests and abdominal ultrasound were consistent with acute pancreatitis. The patient received appropriate fluid resuscitation and adequate pain control. During her hospital stay, the patient awoke from a nap, noted blurry vision, and saw “little dots floating around,” which almost completely resolved in the evening. The following morning, she had worsening blurry vision and floaters. Exam by Ophthalmology revealed cotton wool spots, macular edema and neurosensory detachment of the macula bilaterally secondary to PUR. Visual field testing showed loss of central field bilaterally with preserved peripheral vision. Visual acuity was 20/200 bilaterally with no improvement with pinhole. The patient received intraocular steroid injections with weekly follow up. Examination at 8 weeks demonstrated improvement of visual acuity to 20/40 bilaterally. DISCUSSION: The development of PuR is independent on the severity of pancreatitis and presents with a wide range of manifestations. The diagnosis is made clinically with sudden loss of visual acuity associated with optic nerve edema and visual field loss, along with retinal findings such as cotton-wool spots, retinal hemorrhage, artery attenuation, venous dilation, and Purtscher flecken. The treatment for the ocular complications of PuR have not been proven and prognosis depends on the areas of the retina that are affected.
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A RARE PRESENTATION OF DEVIC’S DISEASE A. Gaddam MD, S. Varakantam MD, A. Williams MD, B. Hubble, DO R. Kaval MD, S. Zuckerman MD, A. Dewitt MD, V. Tati MD. Department of Medicine, Baton Rouge General Medical Center, Baton Rouge, LA. INTRODUCTION: Neuromyelitis Optica (NMO, also known as Devic’s disease) and Neuromyelitis Optica Spectrum Disorder (NMOSD) are rare inflammatory disorders of the central nervous system characterized by severe, immune-mediated demyelination and axonal damage predominantly targeting optic nerves and spinal cord. The hallmark features include acute attacks of optic neuritis and acute myelitis. CASE: A 58-year-old women with hypertension and hypothyroidism presented with a two-day history of severe and rapidly progressive nausea and vomiting and associatedmid-thoracic upper back pain. Initial examandworkupwere unremarkable aside from an elevated protein gap. Serum protein electrophoresis revealed a polyclonal increase in gamma globulin with no evidence of monoclonal protein and HIV/hepatitis panel were negative. She experienced numbness and tingling in her right lower extremity, progressing to weakness of bilateral lower extremities, and urinary retention. Magnetic resonance imaging (MRI) of the head revealed increased signal in a periventricular distribution bilaterally and in the deep white matter of both cerebral hemispheres. MRI of the spine revealed extensive abnormal signal and diffuse enhancement of multiple spinal cord segments involving the lower cervical and upper thoracic cord extending to the T10 level, most significantly T2 to T5 levels. NMO was highly suspected, so high dose steroids were started with minimal response and ultimately required plasmapheresis. NMO-IgG (Aquaporin-4) antibody later confirmed the diagnosis. Neurologic symptoms persisted prompting initiation of Rituximab as the next guideline-directed therapy with gradual improvement. She was placed in a rehabilitation facility and at her nine month follow up, she remained stable with only minimal residual lower extremity weakness and no relapse. DISCUSSION: The real challenge is the recognition of NMO in settings unaccompanied by optic neuritis and acute myelitis. Diagnoses was confirmed by seropositivity for AQP4-IgG and presence of Area Postrema syndrome which is one of the six core clinical characteristics apart from Optic neuritis and Acute Myelitis. Longitudinally extensive spinal cord lesions are characteristic of NMO and differentiate it from multiple sclerosis. Early diagnosis and prompt initiation of therapy with intravenous glucocorticoids, plasmapheresis and Rituximab is essential for severe and unresponsive disease to prevent disabling effects of this rapidly progressive disorder. Long term immunosuppression with Rituximab should be tailored to the severity of attacks, disability, CD20 levels and to prevent relapses. Data is lacking regarding the duration of therapy to achieve remission, as well as in the maintenance of long term remission in those treated with Rituximab.
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ACUTE CORONARY SYNDROME FOLLOWING THERAPEUTIC EPINEPHRINE FOR ANAPHYLAXIS. M. Okoronkwo MD, R. Jupiter. MD, S. Lim. MD Department of Medicine, LSU Health Sciences Center, New Orleans, Louisiana
INTRODUCTION: Vasospastic angina is caused by focal or diffuse spasm of an epicardial coronary artery and myocardial infarction may develop in some if the spasm is persistent. Here we present a case associated with treatment of anaphylaxis causing a myocardial infarction.
CASE: A 35-year old woman with a significant history of daily tobacco use presented to an urgent care clinic with an urticarial rash that developed 30 minutes after taking amoxicillin/clavulanic acid. She subsequently developed weakness and shortness of breath. Her blood pressure was 90/63, pulse was 57 beats/min, respiratory rate was 28 breaths per min and oxygen saturation was 98% on room air. She received an intramuscular injection of epinephrine and intravenous methylprednisolone, diphenhydramine, famotidine, and a 1500 cc bolus of normal saline. While awaiting transfer to an emergency department, she complained of chest pain. An emergent EKG revealed ST elevations through the anterior lateral leads with reciprocal depression in the inferior leads. Patient was transferred to a facility of higher level care for acute coronary syndrome. DISCUSSION: Two mechanisms have been elucidated to describe myocardial injury in the setting of anaphylaxis. Kounis Syndrome is defined as acute myocardial injury due to the anaphylactic response from mast cell and basophil mediated histamine release resulting in coronary vasospasm. Alpha receptor mediated vasospasm from therapeutic intermuscular epinephrine is suspected to result in myocardial injury as well. Our patient, having an allergic cutaneous manifestation along with respiratory and cardiovascular compromise, satisfied anaphylaxis criteria for epinephrine use. Case studies describe the temporal relationship of epinephrine treatment and chest pain with reported in as little as tenminutes after administration. Our patient developed chest pain shortly after epinephrine administration: This temporal relationship favors epinephrine induced vasospasmas the presumedmechanismof myocardial insult. Identification of patientsmost prone to this particular adverse effect of intramuscular epinephrine is a needed area of research and further exploration.
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THE ABDOMINAL COCOON SYNDROME: A CASE REPORT O. Abana MD, S. Rashid MD, B Blancher, K Sulaiman MD Department of Internal Medicine, LSUHSC Shreveport, Louisiana
INTRODUCTION: Encapsulating peritoneal sclerosis (EPS) is themost devastating complication of peritoneal dialysis. While recurrent bacterial peritonitis has been strongly linked, EPS secondary to fungal peritonitis is a lot less common.
CASE: A 19 year-old woman with Type 1 DM, Nephropathic Cystinosis and ESRD was admitted with a two-week history of progressive abdominal distension associatedwithnausea and vomiting. She hadbeenonperitoneal dialysis (PD) for 14 years but was switched to hemodialysis (HD) 3 months prior to current presentation due to complications of fungal peritonitis (cultures positive for C. parapsilosis). She had been discharged on oral antifungal agents but stopped taking them after just 2 weeks. Physical exam was unremarkable except for a soft, distended abdomen with normoactive bowel sounds. Laboratory tests showed WBC elevated at 17,000 and Hb decreased at 7.2g/dl. KUB showed diffuse peritoneal calcifications and contrasted CT abdomen showed abdominal cocooning consistent with encapsulating peritoneal sclerosis. Blood and urine cultures showed no antimicrobial growth but ascitic fluid grew Candida parapsilosis. She received a prolonged course of intravenous micafungin based on sensitivities with plan for possible surgical and/or immunosuppressive therapy for EPS following treatment of fungal peritonitis. DISCUSSION: EPS is a devastating but rare complication of long-term PD. Extensive thickening and fibrosis of the peritoneum results in the formation of a fibrous cocoon encapsulating the bowel leading to intestinal obstruction, strangulation and perforation. Since the duration of time on PD (particularly after five or more years) is a major risk factor, some have advocated a time limit for PD. However, the vast majority of patients on PD do not develop EPS, and the potential benefit of pre-emptively stopping peritoneal dialysis is offset by the risks associated with HD. EPS has also been shown to present after withdrawal from PD in most patients and EPS can develop up to 5 years after cessation of PD. Other risk factors include multiple episodes of severe peritonitis, younger age at initiation of PD, higher concentration of dialysate fluids and certain drugs such as beta blockers.
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HONEY, I SHRUNK THE LUNGS S Rashid MD, V. Nguyen MD Department of Internal Medicine, LSUHSC Shreveport, Louisiana
INTRODUCTION: Systemic Lupus Erythematosus (SLE) is an autoimmune inflammatory disease that can affect many organ systems including dermatologic, rheumatologic, renal, pulmonary, hematologic and central nervous system. The clinical course varies among patients and comprises of periods of relapses and remissions. We present a case of a patient with shrinking lung syndrome as a pulmonary complication secondary to lupus. CASE: Our patient is a 33-year old woman with history of stage V lupus nephritis diagnosed at age 19, hypertension, seizure disorder, smoking history who was admitted for a 7-month history of dyspnea on exertion and hypoxic respiratory failure. Physical exam was significant for decreased breath sounds bilaterally. Chest radiograph showed reduced lung volumes and prominent pulmonary artery. Chest computed tomography (CT) revealed linear atelectasis in bilateral lungs, however no evidence of interstitial lung disease. Pulmonary function test (PFT) revealed reduced lung volumes, increased FEV1/ FVC ratio, consistent with restrictive pattern of lung disease. Forced vital capacity was 29% of predicted, down from 34% 6-months prior. Sniff test was performed to evaluate for diaphragmatic paralysis was normal. DISCUSSION: This case highlights the importance of maintaining a high index of suspicion for various pulmonary manifestations of SLE. Some of the commonly presented complications include pleuritis, acute lupus pneumonitis, interstitial lung disease and pulmonary hypertension. Shrinking lung syndrome is a rare phenomenon defined by progressive dyspnea on exertion and pleuritic chest pain. The exact pathogenesis remains unclear; however, it is proposed that myositis affecting the diaphragms results in diaphragmatic elevation and decreased function. Diagnosis is based on progressive worsening in lung volumes and restrictive pattern on PFTs and lack of interstitial lung or pleural disease on chest CT. Treatment involves glucocorticoids and immunosuppressive therapy that may improve pulmonary symptoms and function. In our patient, PFT’s demonstrated a restrictive pattern and worsening in lung volumes over time without evidence of interstitial lung or pleural disease on chest CT, confirming shrinking lung syndrome. Our patient was started on corticosteroids for treatment.
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PSEUDOANEURYSM OF THE MITRAL- AORTIC INTERVALVULAR FIBROSA DUE TO MITRAL ANNULAR CALCIFICATION R. B. Lee DO 1 , M. Bondugula, MD 1 , C. M. Langley RN 2 , P. S. Fail MD 2 , K. Nagendran MD 2 1. Department of Medicine, University Hospital and Clinics, LSU Health, Lafayette, LA. 2. Cardiovascular Institute of the South, Lafayette, LA INTRODUCTION: The mitral-aortic intervalvular fibrosa (MAIF) is part of the cardiac cytoskeleton providing structural integrity to the mitral and aortic valves. This structure is proximal to the anterior leaflet of themitral valve (MV) and gives rise to themitral annulus. Damage to the MAIF due to calcification, endocarditis, ablation, or valve replacement may result in pseudoaneurysm. CASE: An 83-year-old woman with paroxysmal atrial fibrillation, hypertension, and hyperlipidemia presented with dyspnea on minimal exertion. Transthoracic echocardiogram was significant for severe MR with eccentric jet. Transesophageal echocardiogram (TEE) confirmed severe MR but was notable for a billowing structure near the anterior MV leaflet. Computed tomography angiography of the coronaries was performed with a possible aneurysm noted at the base of the MV. A cardiac magnetic resonance imaging (MRI) identified a 2.7 x 2.5 cm pseudoaneurysm of the MAIF. At that time, she was experiencing New York Heart Association Class III symptoms. The decision was made to proceed with possible MV clipping. Via right femoral vein access, a transeptal puncture was performed and the pseudoaneurysm ostium was wired but could not be advanced into the left ventricle. As a result, right femoral artery access was obtained and a wire was passed into the left ventricle. The pseudoaneurysmwas snared from the left atrium and a 10mm vascular plug was inserted for occlusion. After occlusion, a significant reduction in MR was noted intra-operatively and the day post procedure. The patient was noted to have complete resolution of symptoms two weeks later. DISCUSSION: MAIF pseudoaneurysm is a rare and sometimes a fatal complication which can manifest with MR. In this case, the MAIF pseudoaneurysmwas likely due to mitral annular calcification. Multiple imaging studies were required to fully evaluate this anomaly and cardiac MRI ultimately identified it. The pseudoaneurysm was occluded leading to resolution of MR.
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WOBBLE, WOBBLE – A PARKINSON’S DISEASE MIMICKER A. Reine MD, R. Thibodaux MD Department of Medicine, LSU Health Sciences Center, Baton Rouge, LA
INTRODUCTION: Paraneoplastic neurologic syndromes (PNSs) are rare, usually autoimmune disorders associated with malignancy and not caused by direct tumor invasion, metastasis or consequences of therapy. PNSs typically precede the diagnosis of the malignancy making the diagnosis quite difficult. Almost 30 autoantibodies have been identified and are usually directed against intracellular neuronal proteins or neuronal cell surface proteins. PNSs can affect the central, peripheral, and autonomic nervous systems. Neuroimaging is often unrevealing, and the diagnosis is made by demonstrating a specific paraneoplastic autoantibody. CASE: A 61-year-old woman with hypertension, hypothyroidism, type 2 diabetes, hyperlipidemia, and endometrial carcinoma (Stage IIIc with metastatic intraabdominal nodes) presented for right hand tremors. She had undergone hysterectomy, chemotherapy and radiation 1 year prior with significant improvement in her lymphadenopathy and stable disease noted on most recent abdominal imaging three months prior. She presented 2 months later with worsening tremor affecting writing, “wobbly” gait, and recurrent falls. MRI of the brain revealed no acute intracranial abnormality and toxic/metabolic evaluation was unrevealing. She was diagnosed and treated for Parkinson disease however, her symptoms continued to progress. A paraneoplastic antibody panel was obtained and was markedly positive (1:491,520 titer) for PCA-1(purkinje cell cytoplasmic antibody type 1 or Anti-Yo) which is associatedwith paraneoplastic cerebellar degeneration (PCD). Subsequent surveillance CT scans revealed worsening intraabdominal lymphadenopathy as well as bilateral pleural effusions for which a thoracentesis was performed revealing metastatic endometrial carcinoma. Systemic chemotherapy was unsuccessful due to intolerable side effects and she was discharged to home hospice. DISCUSSION: PCA-1 is a paraneoplastic autoantibody associated with PCD and occurs primarily in patients with breast or gynecological malignancies. The target for these antibodies are the cerebellar degeneration related proteins expressed by purkinje, breast cancer, and gynecological cancer cells. PCA-1 associated PNS occurs in less than 1% of associated malignancies and usually presents with symptoms of acute to subacute truncal and/or limb ataxia, dizziness, diplopia, and dysarthria. Severe disability is common with greater than 90% of affected patients. Brain imaging at the time of presentation of symptoms does not commonly reveal cerebellar atrophy. Treatment of the tumor is critical for any neurologic recovery, although response rates are typically poor. Nevertheless, PNSs are important to recognize as they may allowmore prompt diagnosis and treatment of an otherwise occult malignancy.
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