Impella Versus Extracorporeal Membranous Oxygenation (ECMO) for Cardiogenic Shock: A Systematic Review and Meta-analysis.

The use of mechanical circulatory support (MCS) in cardiogenic shock (CS) is increasing. We conducted a systematic review and meta-analysis to compare the outcomes of Impella use with extracorporeal membranous oxygenation (ECMO) support in patients with CS. We searched the Medline, EMBASE, Cochrane, and Clinicaltrials.gov databases for observational studies comparing Impella to ECMO in patients with CS. Risk ratios (RRs) for categorical variables and standardized mean differences (SMDs) for continuous variables were calculated with 95% confidence intervals (CIs) using a random-effects model. Twelve retrospective studies and one prospective study (Impella n=6652, ECMO n=1232) were identified. Impella use was associated with lower incidence of in-hospital mortality (RR 0.88 [95% CI 0.80-0.94], P=0.0004), stroke (RR 0.30 [0.21-0.42], P<0.00001), access-site bleeding (RR 0.50 [0.37-0.69], P<0.0001), major bleeding (RR 0.56 [0.39-0.80], P=0.002), and limb ischemia (RR 0.42 [0.27-0.65], P=0.0001). Baseline lactate levels were significantly lower in the Impella group (SMD -0.52 [-0.73- -0.31], P<0.00001). There was no significant difference in mortality at 6-12 months, MCS duration, need for MCS escalation, bridge-to-LVAD or heart transplant, and renal replacement therapy use between Impella and ECMO groups. In patients with CS, Impella device use was associated with lower in-hospital mortality, stroke, and device-related complications than ECMO. However, patients in the ECMO group had higher baseline lactate levels.

A nonsense mutation in S-antigen (p.Glu306*) causes Oguchi disease.

PURPOSE: Genetic studies were performed to identify the causative mutation in a 15-year-old girl diagnosed with congenital stationary night blindness (CSNB) presenting Mizuo-Nakamura phenomenon, a typical Oguchi disease symptom. The patient also had dural sinus thrombosis (DST), thrombocytopenia, and systemic lupus erythematosus (SLE). METHODS: Mutation analysis was done by sequencing two candidate genes, S-antigen (SAG; arrestin 1), associated with Oguchi type 1, and rhodopsin kinase (GRK1), associated with Oguchi type 2. In addition, the C677T variation in the methylenetetrahydrofolate reductase (MTHFR) gene was also screened in the family, to determine its probable association with hyperhomocysteinemia in the patient. RESULTS: Sequencing of the SAG and GRK1 resulted in identifying a novel homozygous nonsense mutation (c.916G>T; p.Glu306*) in SAG, which in unaffected siblings either was present in a heterozygous state or absent. The C677T heterozygous allele in the MTHFR gene was found to be associated with hyperhomocysteinemia in the patient and other family members. CONCLUSIONS: This is the first report of Oguchi type 1 in a Pakistani patient due to a nonsense mutation (c.916G>T; p.Glu306*) in SAG. The neurologic and hematological abnormalities likely are not associated with the SAG variant.

Life-Threatening Thrombocytopenia Secondary to Trimethoprim/Sulfamethoxazole.

Thrombocytopenia is an uncommon side effect of trimethoprim/sulfamethoxazole (TMP/SMX) when given in the usual recommended adult dosage. We report a case of severe and possibly life-threatening thrombocytopenia associated with TMP/SMX therapy. A 92-year-old female presented after a mechanical fall and subsequent intractable bleeding from a laceration on her left leg. She had a history of cellulitis of the lower extremities treated with a 10-day course of TMP/SMX. Her last dose was two days before the visit. The physical examination was significant for a small laceration on her left shin, with persistent oozing of blood. Her blood work was notable for white blood cells (WBC) 9.4×10^9/L (9.4×10^3/mm^3), hemoglobin 125g/L (12.5 g/dL) and platelets 5×10^9/L (5×10^3/mm^3). A repeat platelet count was 4×10^9/L. Prothrombin time was 11 seconds and the international normalized ratio (INR) was one. The TMP/SMX was discontinued and one unit of platelets was transfused. Her platelet count subsequently increased to 108×10^9/L. Severe thrombocytopenia with a platelet count of ≤10×10^9/Lmay rarely result in the catastrophic spontaneous bleeding. Thus, low platelet counts associated with TMP/SMX carry potential life-threatening complications. The clinicians should be aware of this adverse effect of TMP/SMX, which appears to be dose/duration independent. We suggest careful monitoring of complete blood cell count, especially platelet count, before and during TMP/SMX therapy.

Facial Weakness, Diplopia, and Fever in a 31-Year-Old: An Atypical Case of Tuberculous Meningitis.

Tuberculous meningitis (TBM) is an infection of the central nervous system (CNS) meninges that carries high morbidity and mortality. It is important to recognize, as patients may present with atypical symptoms. We describe the case of a 31-year-old man with a history of diabetes who presented with a sub-acute onset of right-sided facial weakness and right gaze difficulty with diplopia. History revealed low-grade fever, right-sided headache, fatigue and moderate weight loss for the past several weeks. The patient did not report neck stiffness, rigidity, fever, chills or cough. The physical exam revealed sixth nerve palsy with a right Horner's syndrome. Magnetic resonance imaging (MRI) of the brain showed pachymeningeal enhancement. A spinal tap revealed elevated white blood cells (WBCs), glucose and protein; cerebrospinal fluid (CSF) culture showed Mycobacterium tuberculosis. The patient was diagnosed with TBM and treated with isoniazid, rifampin, pyrazinamide, ethambutol and vitamin B6 for 12 months. The timely diagnosis of TBM can be challenging due to a nonspecific clinical presentation. In patients with a sub-acute onset of headache, fever and meningeal signs, TBM should be considered in the differential. If suspected, treatment should be initiated immediately to prevent further neurological impairment and death.

Spinal Schwannoma with Intradural Intramedullary Hemorrhage.

Patients with spinal abnormalities infrequently present with intradural intramedullary bleeding. The more common causes include spinal trauma, arteriovenous malformations and saccular aneurysms of spinal arteries. On occasion, spinal cord tumors either primary or metastatic may cause intramedullary bleed with ependymoma of the conus medullaris. Spinal nerve sheath tumors such as schwannomas only rarely cause intradural intramedullary bleed, especially in the absence of spinal cord or nerve root symptoms. We report a case of spinal intradural schwannoma presenting with acute onset of quadriparesis. Cerebral angiography studies were negative but magnetic resonance imaging (MRI) of the spine revealed a large hemorrhagic tumor in the thoracolumbar junction. However, we suggest that the patients with intradural intramedullary bleed should be evaluated for underlying spine disease.

Serial Magnetic Resonance Imaging in Creutzfeldt-Jakob Disease: a Case Report and Literature Review.

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. CJD usually appears in later life and runs a rapid course. Typically, the onset of symptoms occurs about age 60 and about 90% of individuals die within one year. We report a case of 67-year-old male presented with progressive aphasia, confusion, dysphagia and inability to carry out activities of daily life (ADLs) over a period of three to four weeks. The patient had past medical history of chronic atrial fibrillation and hypertension. Prior to admission, the patient was treated for ischemic stroke of left basal ganglia but continued to have worsening encephalopathy. The spinal tap revealed a 14-3-3 protein level of thirteen times the upper limit of normal; electroencephalogram (EEG) showed a diffuse slowing of the background and periodic sharp waves with greater involvement of the left hemisphere. Diffusion-weighted imaging (DWI) magnetic resonance imaging (MRI) at the time of admission showed extensive signal abnormality in the basal ganglia bilaterally and in the cerebral cortex bilaterally, particularly over the left cerebral hemisphere. The persistence of the MRI findings over several weeks was concerning for spongiform encephalopathy. The probable diagnosis of Creutzfeldt-Jakob disease was made based on these imaging findings taken together with the patient's clinical signs and symptoms of a rapidly progressive encephalopathy. The patient was able to have some quality time with his family as the diagnosis was made earlier than perhaps otherwise and expired peacefully after comfort care measures were chosen. Serial MRI may serve as a clue to the early diagnosis of CJD and potentially provide a better quality of life for the patients.

Cardiovascular Dysfunction Presenting as Autonomic Dysreflexia in a Patient with Spinal Cord Injury.

Autonomic dysreflexia (AD) is a medical emergency that is characterized by hypertension as an autonomic response to noxious stimuli in patients with a history of spinal cord injury at the level of T6 or above. We present the case of a 31-year-old Caucasian male with a history of spinal cord injury at the level of C3-C4, with symptoms described as recurring episodes of hypertension with flushing and sweating above the level of the lesion for the past five to six years. His symptoms are triggered by bowel distention, excitement, a bumpy car ride, or a simple turning of the neck to the left. Physical examination and laboratory studies ruled out other possible differentials (e.g., migraines, pheochromocytoma). As a result, AD was diagnosed.

Breast Cancer Metastases to the Gastrointestinal Tract Presenting with Anemia and Intra-abdominal Bleed.

Signet ring adenocarcinoma of the breast with synchronous metastasis to the gastrointestinal (GI) tract is a rare occurrence, typically presenting with abdominal pain, dyspepsia, or GI bleed. We report a case of metastatic breast cancer presenting with a complaint of anemia. A further diagnostic evaluation revealed generalized lymphadenopathy, nodular thickening of the urinary bladder wall, bone lesions, and enlarged pancreas. Biopsies from the lymph nodes, pancreatic biopsy, and bladder nodule all revealed a signet cell carcinoma. An upper and lower GI endoscopy revealed multiple ulcerated gastric mucosal nodules and polypoid folds in the cecum and proximal ascending colon; the biopsies from these lesions were also positive for signet ring cell adenocarcinoma.

Diagonal Earlobe Crease as a Significant Marker for Coronary Artery Disease: A Case-control Study.

OBJECTIVES: To investigate the association between diagonal earlobe crease (DELC) and coronary artery disease (CAD). Limited data exists in South Asia and no prior studies have been performed in Pakistan to assess this relationship. METHODS: In this case-control study, 200 participants from December 2015 to March 2016 at Shifa International Hospital, Islamabad, Pakistan were enrolled. Consecutive non-probability sampling was used to recruit patients. Cases were enrolled from cardiac care unit (CCU) of the hospital with angiography-proven CAD. Controls were selected from surgical, medical and neurology units of the hospital if they had no previously established evidence or symptoms of CAD. Patients were evaluated in terms of age and any history of hypertension, diabetes and/or smoking. Cases and controls were examined separately by two investigators for the unilateral or bilateral presence of DELC of the lobular portion of either auricle. Patients with ear piercings were excluded from the study. The data was analyzed in statistical product and service solutions (SPSS) (IBM, Delaware, Chicago), and an online statistical software. RESULTS: Out of the 200 patients, 126 (63%) were males and 74 (37%) were females. In the 100 cases, 76 had DELC and 24 had no crease whereas, among the 100 controls, 36 had DELC and 64 had no DELC (p <0.001, OR = 5.63, CI = 2.91-10.93). The prevalence of diseases such as hypertension, diabetes, smoking among the cases and controls were 66%, 53%, 27% and 27%, 18%, 25% respectively. The effect of hypertension and diabetes on the presence of DELC was statistically significant (p <0.05) but the impact of smoking on DELC presence was insignificant (p >0.05). CONCLUSION: There is a significant association between DELC and CAD. This is the first case-control study from South Asia disclosing this important correlation. Our study also reports a high frequency of DELC in patients suffering from hypertension and diabetes mellitus. No association between smoking and DELC was found.

Anti-n-Methyl-d-Aspartate-Receptor (NMDAR) Encephalitis in Association with Ovarian Teratoma.

Anti-N-methyl-D-aspartate-Receptor (NMDAR) encephalitis is an autoimmune disorder with a multifaceted presentation that involves memory deficits, psychiatric symptoms, and autonomic instability. This case report describes the classic presentation of Anti-NMDAR encephalitis and highlights its association with ovarian teratomas. We present a 26 -year-old female who came in with new onset seizures and altered mentation who subsequently developed automatism. Electroencephalograms (EEG) showed left frontal spikes and right temporal delta activity. Magnetic resonance imaging (MRI) revealed right temporal hyper-intensity. The diagnosis was established with positive anti-NMDAR antibodies in the cerebrospinal fluid (CSF). The patient was initially treated with steroids and valproic acid, however, her condition progressively worsened. A five-day course of intravenous immunoglobulins (IVIG) was started followed by rituximab. The clinical course was complicated with the patient developing neutropenic fever and cerebrospinal fluid cultures (CSF) growing methicillin-sensitive Staphylococcus aureus (MSSA). She underwent pelvic imaging which showed a right ovarian teratoma. Evidence suggests that removal of ovarian tumor leads to better clinical and mortality outcomes in patients with Anti-NMDAR encephalitis. It is important for the internist to consider paraneoplastic syndromes in patients with Anti-NMDAR encephalitis.

Efficacy of spinal cord stimulation as an adjunct therapy for chronic refractory angina pectoris.

INTRODUCTION: Patients with chronic refractory angina whose symptoms are not controlled with conventional therapies have a poor quality of life. Adjunctive therapies, such as spinal cord stimulation (SCS) may be considered in these cases. We sought to examine whether SCS is associated with changes in exercise capacity and angina severity in these patients. METHODS: We searched Pubmed, Medline and other databases until December 2015. Two reviewers independently extracted data and assessed risk of bias. Exercise capacity included exercise duration and rate pressure product, determined via an exercise test. Angina severity included daily angina frequency and nitrate consumption. RESULTS: A total of 518 participants (1048.25 person-years of follow-up), from 14 studies met our inclusion criteria. The mean age was 66.8years and 68.5% were men. SCS implant duration ranged from 3weeks to 5years (median: 6months). Using random effects meta-analysis, we found that SCS was associated with a higher exercise duration (1.90min, 95% CI 1.71, 2.06) and lower angina severity, 1.55 less daily angina episodes, (95% CI -1.75, -1.33), 1.54 less daily nitrates consumed, (95% CI -1.81, -1.26), and a 22 points higher SF-36 angina frequency score (95% CI 10.76, 32.81; p<0.0001) on follow-up. The change in rate pressure product was not significant. CONCLUSION: This meta-analysis suggests that SCS, as an adjunct therapy to medical management, may be associated with a longer exercise duration and lower angina frequency and nitrate consumption in patients with chronic refractory angina pectoris who are not candidates for percutaneous intervention or revascularization.

Pleuropericarditis complicated by a pericardial cyst.

We report the case of a 36-year-old woman with a recent upper respiratory illness who presented with chest pressure, lasting for five days, which improved with leaning forward. Physical examination and laboratory studies were unremarkable. Chest computed tomography scan revealed a 6.9 cm × 4 cm × 2.5 cm pericardial cyst. The patient was diagnosed with pleuropericarditis complicated by a pericardial cyst. Pericardial cysts are rare mediastinal lesions that are often congenital, but may be inflammatory. If symptomatic, surgical resection or percutaneous aspiration may be considered. In our patient, treatment with ibuprofen and colchicine resulted in a decrease in cyst size, suggesting an inflammatory component. We describe an uncommon case of reduction of a pericardial cyst shortly after treatment of pericarditis due to a probable viral infection. Non-operative management of pericardial cysts may be appropriate in these select cases. .

Choroidal analysis in healthy eyes using swept-source optical coherence tomography compared to spectral domain optical coherence tomography.

PURPOSE: To compare analyses of choroidal thickness and volume in healthy eyes measured concurrently with prototype long-wavelength swept-source optical coherence tomography (OCT) and commercially available spectral-domain optical coherence tomography (OCT) with and without enhanced depth imaging (EDI). DESIGN: Prospective cross sectional study. METHODS: The study included 19 healthy subjects (19 eyes), who were prospectively recruited to undergo 2 consecutive imaging sessions on the same randomly selected eye using spectral domain OCT and a prototype long-wavelength swept-source OCT. On spectral domain OCT, 2 line scans, 1 with and 1 without EDI, and 1 volumetric scan were obtained. On swept-source OCT, 1 line scan and 1 volumetric scan were obtained. Scan patterns on swept-source OCT were created to simulate those available on Cirrus HD-OCT to keep the time of image acquisition constant. Swept-source OCT volumetric scans were motion corrected using a novel registration algorithm. Choroidal thickness and volume were analyzed. RESULTS: The choroidoscleral interface was clearly visualized in 19/19 (100%) of eyes imaged by swept-source OCT, compared to 14/19 (73.6%) and 13/19 (68.4%) eyes imaged by spectral domain OCT, with and without EDI, respectively. There was no significant difference in choroidal thickness measurements on the line scans obtained on either system (P = 0.10). Choroidal volume could not be assessed on volumetric scans from spectral domain OCT. Mean choroidal volume from swept-source OCT volumetric scans was 11.77 ± 3.13 mm(3) (6.43 mm(3)-17.15 mm(3)). CONCLUSION: This is the first study that compares simultaneously a prototype long-wavelength swept-source OCT to a commercially available spectral domain OCT for a detailed analysis of choroid in healthy eyes. Swept-source OCT shows potential for better choroidal analysis. Studies using swept-source OCT in diseased eyes will further define this new technology's utility in chorioretinal diseases.

Enhanced visualization of the choroido-scleral interface using swept-source OCT.

An accurate analysis of the thickness and volume of choroid using optical coherence tomography (OCT) requires precise visualization of the choroido-scleral interface. Results of studies that use spectral-domain OCT (SD-OCT) to look at the percent visualization of the choroido-scleral interface for accurate analysis of choroidal thickness show varying success rates. This study assessed the visualization of choroido-scleral interface in 19 healthy participants (19 eyes) prospectively recruited for consecutive high-definition raster scanning with an SD-OCT system with and without enhanced depth imaging (EDI) and a prototype long-wavelength swept-source OCT (SS-OCT) system. Choroido-scleral interface was visualized in all eyes imaged on SS-OCT, compared with 13 of 19 (68.4%) and 14 of 19 (73.6%) eyes imaged on SD-OCT without EDI (P = .009) and with EDI (P = .02), respectively. The prototype long-wavelength SS-OCT system, with its higher acquisition speed and deeper tissue penetration, may have better clinical utility in determining the involvement of choroid in various chorioretinal diseases.