bluuebunny/crossref_metadata_2025_split huggingface dataset:
PROPHYLACTIC PERIPHERAL LASER AND FLUORESCEIN ANGIOGRAPHY AFTER BEVACIZUMAB FOR RETINOPATHY OF PREMATURITY
Authors: Garcia Gonzalez Jose M. , Snyder Laura , Blair Michael , Rohr Ashley , Shapiro Michael , Greenwald Mark 
Published: March 2017

Purpose:
To report reactivation rate after bevacizumab treatment for retinopathy of prematurity (ROP) in eyes with classic ROP (CROP) versus aggressive posterior ROP (APROP) and to report peripheral fluorescein angiography findings in these eyes.

Methods:
Retrospective chart review was conducted on consecutive infants treated with bevacizumab for ROP, followed by fluorescein angiography and prophylactic laser to persistent avascular retina.

Results:
Sixty-four eyes of 33 patients were included. Mean gestational age was 25 weeks with mean birth weight of 674 g. Mean follow-up was 125 weeks post-menstrual age (PMA). Reactivation requiring treatment after initial bevacizumab was more common in eyes with APROP (8/16) than with CROP (2/48; P < 0.0001). At mean 73 weeks PMA, eyes with APROP had more avascular retina (mean 4.4 disk diameters vs. 2.6 disk diameters; P = 0.0004) and higher percentage of leakage (11/11 eyes vs. 22/38 eyes; P = 0.01) on fluorescein angiography than in eyes with CROP. Unfavorable outcome occurred in 1 of 16 eyes with APROP and in no eyes with CROP. No eye that underwent prophylactic laser after bevacizumab had a poor structural outcome.

Conclusion:
In our study, bevacizumab-treated eyes with APROP have a higher likelihood of recurrence and larger area of persistent nonperfusion than in eyes with CROP. Treatment of ROP with bevacizumab followed by prophylactic laser has a low rate of unfavorable structural outcome.
BLIND-SIDED BY COSMETIC VEIN SCLEROTHERAPY: A CASE OF OPHTHALMIC ARTERIAL OCCLUSION
Authors: Arunakirinathan Meena , Walker Robbie J. E. , Hassan Noor , Ameen Sally , Younis Saad 
Published: March 2017

Purpose:
Cosmetic vein sclerotherapy is increasingly used to treat varicose veins because of its effectiveness and adherence with British Pharmacopoeia specifications. We present the first documented case of ophthalmic artery occlusion resulting in panocular ischemia secondary to intravascular injection of sodium tetradecyl sulfate sclerosant in a young healthy women seeking treatment for prominent facial veins in her forehead.

Methods:
The patient presented with unilateral sudden loss of vision. Funduscopy demonstrated a pale retina, cherry-red spot, and sclerosant visualized directly at the macula. She underwent emergency treatment for central retinal artery occlusion followed by fundal photographs, fluorescein angiography, and optical coherence tomography.

Results:
Despite intervention, the vision remained no perception to light. Magnetic resonance imaging, echocardiography, and Doppler ultrasound were unremarkable. The patient later developed neovascular sequelae requiring laser pan-retinal photocoagulation.

Conclusion:
Widely regarded as safe, and approved by the U.S. Food and Drug Administration, the only published ocular side effects of foam sclerotherapy are transient visual disturbances or temporary scotomas. This case demonstrates irreversible loss of vision as a previously unreported complication. While undoubtedly rare, we believe physicians and surgeons using sclerosant in the orbital adnexa, face, nose, and sinuses should be aware of this sight-threatening complication of injection and counsel potential patients accordingly.
Diagnostic accuracy of laser-evoked potentials in diabetic neuropathy
Authors: Di Stefano Giulia , La Cesa Silvia , Leone Caterina , Pepe Alessia , Galosi Eleonora , Fiorelli Marco , Valeriani Massimiliano , Lacerenza Marco , Pergolini Mario , Biasiotta Antonella , Cruccu Giorgio , Truini Andrea 
Published: March 2017

Abstract
Although the most widely agreed neurophysiological tool for investigating small fiber damage is laser-evoked potential (LEP) recording, no study has documented its diagnostic accuracy. In this clinical, neurophysiological, and skin biopsy study, we collected age-corrected LEP normative ranges, verified the association of LEPs with pinprick sensory disturbances in the typical diabetic mixed fiber polyneuropathy, and assessed the sensitivity and specificity of LEPs in diabetic small fiber neuropathy. From 288 LEP recordings from the face, hand, and foot in 73 healthy subjects, we collected age-corrected normative ranges for LEPs. We then selected 100 patients with mixed-fiber diabetic neuropathy and 25 patients with possible small-fiber diabetic neuropathy. In the 100 patients with mixed fiber neuropathy, we verified how LEP abnormalities were associated with clinically evident pinprick sensory disturbances. In the 25 patients with possible pure small fiber neuropathy, using the skin biopsy for assessing the intraepidermal nerve fiber density as a reference standard, we calculated LEP sensitivity and specificity. In healthy participants, age strongly influenced normative ranges for all LEP variables. By applying age-corrected normative ranges for LEPs, we found that LEPs were strongly associated with pinprick sensory disturbances. In relation to the skin biopsy findings, LEPs yielded 78% sensitivity and 81% specificity in the diagnosis of diabetic small fiber neuropathy. Our study, providing age-corrected normative ranges for the main LEP data and their diagnostic accuracy, helps to make LEPs more reliable as a clinical diagnostic tool, and proposes this technique as a less invasive alternative to skin biopsy for diagnosing diabetic small fiber neuropathy.
Prevalence and incidence of chronic pain with or without neuropathic characteristics in patients with cancer
Authors: Bouhassira Didier , Luporsi Elisabeth , Krakowski Ivan 
Published: March 2017

Abstract
This prospective national multicenter study was carried out to estimate the prevalence and incidence of chronic pain with or without neuropathic characteristics in patients with cancer in France. All consecutive outpatients (n = 1885) seen over 2 weeks for cancer treatment in 12 oncology units were invited to participate in the study, and 1805 were included. Patients underwent a clinical examination during visit 1, and a questionnaire was completed to detect chronic pain (defined as daily pain for at least 3 months), and to characterize its intensity, location, and neuropathic characteristics (ie, DN4 score ≥4). The impact of pain on quality of life was assessed with the Brief Pain Inventory. Patients without pain at visit 1 were included in the incidence study and were seen at 3 and 6 months after visit 1. The overall prevalence of chronic pain was 28.2% (95% CI: 26.3-30.5), ranging from 22.5% to 35.4%, depending on the location of the primary tumor. Neuropathic characteristics were present in 20.9% of these patients, with a prevalence of 2.9% to 9.7%, depending on primary tumor location. Pain intensity and interference were higher in patients with neuropathic characteristics. In total, 1285 patients were included in the incidence study, 873 of whom were seen at least once, 3, or 6 months after the first visit. The incidence of chronic pain during the 6-month follow-up period ranged from 13% to 28%, depending on primary tumor location, and neuropathic characteristics were found in 19.9% of patients with chronic pain.
Natural History and Treatment Outcomes of Severe Autoimmune Hepatitis
Authors: Sonthalia Nikhil , Rathi Pravin M. , Jain Samit S. , Surude Ravindra G. , Mohite Ashok R. , Pawar Sunil V. , Contractor Qais 
Published: March 2017

Goals:
The aim of this study was to analyze the natural history and treatment outcomes of autoimmune hepatitis (AIH) variants presenting with severe-AIH.

Background:
Severe acute presentation is an uncommon manifestation of AIH, and it remains poorly characterized.

Materials and Methods:
We included 101 patients with AIH from January 2011 to December 2015. Patients were classified as seropositive-AIH and seronegative-AIH. Patients with acute liver failure, acute-on-chronic liver failure, and severe acute hepatitis were defined as severe-AIH patients. Patient characteristics and treatment outcomes with follow-up until 12 months were analyzed between the different groups.

Results:
Out of 101 cases, 24 (23.76%) had severe AIH. Of them 9 (37.5%) had severe acute hepatitis, 3 (12.5%) had acute liver failure, and 12 (50%) had acute-on-chronic liver failure. Seronegative-AIH patients presented with severe-AIH significantly more frequently compared with seropositive-AIH patients (50% vs. 20.27%, P=0.022). Severe-AIH had 50% complete responders, 25% partial responders, and 25% treatment failures. Jaundice (88.88% vs. 68.7%, P=0.048), encephalopathy (55.55% vs. 6.66%, P=0.014), and higher international normalized ratio values (2.17±0.60 vs. 1.82±0.14, P=0.038) were factors associated with nonresponse rather than the presence or absence of autoantibodies in severe-AIH. The hazard ratio for predicting remission in the non-severe AIH group as compared with the severe-AIH group was 1.502, which was statistically not significant (95% CI, 0.799-2.827; P=0.205).

Conclusion:
Approximately 24% of patients with AIH have severe-AIH. Conventional autoantibodies are often absent in severe-AIH; however, it does not alter the outcome. Immunosuppressants should be given expediently in patients with severe-AIH.
Cutaneous manifestations of systemic viral diseases in neonates: an update
Authors: Castillo Stephanie A. , Pham Anh Khoa , Dinulos James G. 
Published: March 2017

Purpose of review
Dermatologic findings may be the first signs of a neonatal viral infection. This review provides an update of the diagnostic features and therapies for selected viral illnesses [herpes simplex virus (HSV), varicella zoster virus, enterovirus, and Zika virus] that present with cutaneous manifestations in the neonate.

Recent findings
HSV DNA polymerase chain reaction of plasma and cerebrospinal fluid, routinely used in the diagnosis of neonatal HSV, may have expanded utility in assessing prognosis and acyclovir therapeutic efficacy. Maternal antiviral suppressive therapy may alter the clinical appearance of congenital HSV, resulting in delayed diagnosis and treatment. VariZIG, a varicella zoster immune globulin, is a US Food and Drug Administration approved form of prophylaxis for varicella. The Centers for Disease Control and Prevention has expanded the period of VariZIG eligibility for preterm infants, a group particularly susceptible to severe varicella infection. For severe neonatal enterovirus sepsis, the results of a randomized, double-blind, placebo-controlled trial of pleconaril, a viral capsid inhibitor, suggest that this compound is an effective therapy. Human Parechovirus type 3, a strain within a newly formed viral genus, has a similar, and potentially underestimated, clinical presentation to enterovirus sepsis. However, a distinctive erythematous palmoplantar rash may be specific to human Parechovirus type 3 infection. Perinatal Zika virus infection in the neonate may present with a nonspecific macular and papular rash. As this rash is not specific, obtaining a maternal travel history and, if appropriate, requesting additional diagnostic testing are critical for early diagnosis.

Summary
Neonatal rashes may be harmless and transient, whereas others may reflect the presence of a severe systemic illness. Recognizing key cutaneous features of viral-associated rashes may aid in the prompt and accurate diagnosis and treatment of neonatal viral illnesses.
Nephrogenic diabetes insipidus
Authors: Bockenhauer D. , Bichet Daniel G. 
Published: March 2017

Purpose of review
In nephrogenic diabetes insipidus (NDI), the kidney is unable to concentrate urine despite elevated concentrations of the antidiuretic hormone arginine-vasopressin. In congenital NDI, polyuria and polydipsia are present from birth and should be immediately recognized to avoid severe episodes of dehydration. Unfortunately, NDI is still often recognized late after a ‘diagnostic odyssey’ involving false leads and dangerous treatments.
Once diagnosed, appropriate treatment can be started. Moreover, laboratory studies have identified promising new compounds, which may help achieve urinary concentration independent of vasopressin.

Recent findings
MAGED2 mutations caused X-linked polyhydramnios with prematurity and a severe but transient form of antenatal Bartter's syndrome.
We distinguish two types of hereditary NDI: a ‘pure’ type with loss of water only and a complex type with loss of water and ions. Mutations in the AVPR2 or AQP2 genes, encoding the vasopressin V2 receptor and the water channel Aquaporin2, respectively, lead to a ‘pure’ NDI with loss of water but normal conservation of ions. Mutations in genes that encode membrane proteins involved in sodium chloride reabsorption in the thick ascending limb of Henle's loop lead to Bartter syndrome, a complex polyuric-polydipsic disorder often presenting with polyhydramnios. A new variant of this was recently identified: seven families were described with transient antenatal Bartter's syndrome, polyhydramnios and MAGED2 mutations.
Multiple compounds have been identified experimentally that may stimulate urinary concentration independently of the vasopressin V2 receptor. These compounds may provide new treatments for patients with X-linked NDI.

Summary
A plea for early consideration of the diagnosis of NDI, confirmation by phenotypic and/or genetic testing and appropriate adjustment of treatment in affected patients.
Improved Starch Digestion of Sucrase‐deficient Shrews Treated With Oral Glucoamylase Enzyme Supplements
Authors: Nichols Buford L. , Avery Stephen E. , Quezada‐Calvillo Roberto , Kilani Shadi B. , Lin Amy Hui‐Mei , Burrin Douglas G. , Hodges Benjamin E. , Chacko Shaji K. , Opekun Antone R. , Hindawy Marwa El , Hamaker Bruce R. , Oda Sen‐Ichi 
Published: March 2017

ABSTRACTBackground and Objective:Although named because of its sucrose hydrolytic activity, this mucosal enzyme plays a leading role in starch digestion because of its maltase and glucoamylase activities. Sucrase‐deficient mutant shrews, Suncus murinus, were used as a model to investigate starch digestion in patients with congenital sucrase‐isomaltase deficiency. Starch digestion is much more complex than sucrose digestion. Six enzyme activities, 2 α‐amylases (Amy), and 4 mucosal α‐glucosidases (maltases), including maltase‐glucoamylase (Mgam) and sucrase‐isomaltase (Si) subunit activities, are needed to digest starch to absorbable free glucose. Amy breaks down insoluble starch to soluble dextrins; mucosal Mgam and Si can either directly digest starch to glucose or convert the post‐α‐amylolytic dextrins to glucose. Starch digestion is reduced because of sucrase deficiency and oral glucoamylase enzyme supplement can correct the starch maldigestion. The aim of the present study was to measure glucogenesis in suc/suc shrews after feeding of starch and improvement of glucogenesis by oral glucoamylase supplements.Methods:Sucrase mutant (suc/suc) and heterozygous (+/suc) shrews were fed with 13C‐enriched starch diets. Glucogenesis derived from starch was measured as blood 13C‐glucose enrichment and oral recombinant C‐terminal Mgam glucoamylase (M20) was supplemented to improve starch digestion.Results:After feedings, suc/suc and +/suc shrews had different starch digestions as shown by blood glucose enrichment and the suc/suc had lower total glucose concentrations. Oral supplements of glucoamylase increased suc/suc total blood glucose and quantitative starch digestion to glucose.Conclusions:Sucrase deficiency, in this model of congenital sucrase‐isomaltase deficiency, reduces blood glucose response to starch feeding. Supplementing the diet with oral recombinant glucoamylase significantly improved starch digestion in the sucrase‐deficient shrew.
Outcomes in Pediatric Autoimmune Hepatitis and Significance of Azathioprine Metabolites
Authors: Sheiko Melissa A. , Sundaram Shikha S. , Capocelli Kelley E. , Pan Zhaoxing , McCoy Annette M. , Mack Cara L. 
Published: March 2017

ABSTRACTObjectives:Autoimmune hepatitis (AIH) is a common pediatric liver disease and long‐term remission is usually maintained with azathioprine (AZA). There is no consensus on the target range for AZA active metabolite 6‐thioguanine (6‐TGN) levels in pediatric AIH. The aim of the present study was to characterize the outcomes of pediatric patients with AIH and determine correlations between AZA dosing or 6‐TGN metabolite levels and biochemical remission.Methods:A retrospective chart review was performed and data on presentation, laboratories including AZA metabolite levels, medication use, and outcomes were collected.Results:Between 2002 and 2013, 66 children with AIH were identified (mean age at diagnosis 9.6 ± 5.1 years) with a mean follow‐up period of 2.9 ± 3.2 years. Common presenting symptoms included jaundice, fatigue, and abdominal pain. The majority of subjects received steroids for induction and AZA for maintenance of remission. Seventy‐nine percent achieved biochemical remission (mean time to remission 6.2 ± 9.2 months), 14% were in the induction phase of therapy, 6% required liver transplantation, and 18% were weaned off immunosuppression and remained in remission. 6‐TGN levels ranging from 50 to 250 pmol/8 × 108 red blood cell count were associated with biochemical remission (alanine aminotransferase levels of ⩽50 U/L).Conclusions:The vast majority of children with AIH maintain a sustained remission with AZA monotherapy. Biochemical remission was maintained with 6‐TGN levels much lower than that recommended for inflammatory bowel disease. These findings suggest that patients should be maintained at the lowest AZA dose possible that is associated with biochemical remission.
A Rare Cause of Neonatal Hemolytic Anemia: Glutathione Synthetase Deficiency
Authors: Soylu Ustkoyuncu Pembe , Mutlu Fatma Türkan , Kiraz Aslihan , Tag Balkis Zuhal , Yel Sibel 
Published: March 2017

Background:
Isolated hemolysis or hemolytic anemia and 5-oxoprolinuria are 2 distinct medical conditions in the clinical spectrum associated with glutathione synthetase deficiency.

Clinical Observation:
A 1-day-old female baby presented with anemia and respiratory distress. Her hemoglobin level was 9.5 g/dL and the total serum bilirubin level was 5.6 mg/dL. Metabolic acidosis was detected in her blood gas analysis. Metabolic acidosis recurred despite treatment and further investigation was required. Her 5-oxoproline level was 3815 mmol/mol creatinine in urine organic acid analysis, and a homozygous mutation [p.R125H (c.374G>A)] was found in the glutathione synthetase gene.

Conclusions:
GSD has been observed in very few patients and is rarely considered in the differential diagnosis of hemolytic anemia in newborns.
Sirolimus as an Effective Agent in the Treatment of Immune Thrombocytopenia (ITP) and Evans Syndrome (ES): A Single Institution’s Experience
Authors: Jasinski Sylwia , Weinblatt Mark E. , Glasser Chana L. 
Published: March 2017

Background:
Autoimmune cytopenias are characterized by immune-mediated destruction of hematopoietic cell lines with immune thrombocytopenia (ITP) affecting platelets and Evans syndrome (ES) affecting platelets and red blood cells. For patients with persistent disease, limited options for effective and well-tolerated therapies exist.

Objectives:
Our aim is to describe our institution’s experience with sirolimus as therapy for pediatric patients with persistent ITP and ES.

Design/Method:
A retrospective analysis was performed in patients with persistent ITP and ES treated with sirolimus. Responses were categorized as complete response (CR), partial response, modest response, or no response.

Results:
Of the 17 patients treated, 12 had ITP and 5 had ES. Seventy-three percent of ITP patients achieved a CR, 78% of them by 3 months. Only 2 patients did not achieve a durable response. Eighty percent of ES patients had a response, with 50% of them achieving CR and the other 50% an asymptomatic partial response. One patient with ES achieved modest response, but discontinued therapy due to an adverse effect. Of the patients that achieved CR, 90% remain off all therapy for a median of 2 years.

Conclusions:
Our data suggest that sirolimus is a safe and effective steroid-sparing agent in the treatment of persistent ITP and ES.
Gynecologic Malignancies in Pregnancy: Balancing Fetal Risks With Oncologic Safety
Authors: Cordeiro Christina N. , Gemignani Mary L. 
Published: March 2017

Importance
Cancer occurs in 0.05% to 0.1% of all pregnancies. Despite literature reporting good oncologic and fetal outcomes in women treated for cancer during pregnancy, as many as 44% of gynecologists would offer termination, and 37% would not administer chemotherapy or radiotherapy in pregnancy.

Objectives
The aims of this study were to summarize current recommendations for the treatment of cervical and ovarian cancers in pregnancy and to review updates on existing knowledge regarding the safety of surgical and chemotherapeutic treatments in pregnancy, including both oncologic and fetal outcomes.

Evidence Acquisition
A detailed literature review was performed on PubMed.

Results
The treatment of gynecologic malignancies during pregnancy mirrors that outside pregnancy, with a balance between maternal versus fetal health. Fertility-sparing surgery can be offered to stage IA2 and low-risk IB1 cervical, stage I epithelial ovarian, germ cell ovarian, or sex-cord stromal ovarian tumors. Delayed treatment can be offered for stage IB1 cervical cancer. Neoadjuvant and/or adjuvant chemotherapy can be given for advanced gynecologic cancers with good disease-free survival without significant adverse neonatal outcomes.

Conclusions
A multidisciplinary approach and improved education of providers regarding the surgical and chemotherapeutic treatments in pregnancy are needed in order to fully inform patients regarding treatment options. Further research in women who are pregnant is needed to determine the safety of diagnostic and therapeutic procedures used in the nonpregnant woman.

Relevance
This article reviews and supports treatment of gynecologic cancer during pregnancy, calls for additional study and long-term follow-up, and justifies improved education of patients and providers regarding treatment options.

Target Audience
Obstetricians and gynecologists, family physicians.

Learning Objectives
After completing this activity, the learner should be better able to (1) review general principles in the management and treatment of gynecologic cancers in pregnancy, (2) review the diagnosis and treatment of cervical cancer in pregnancy, and (3) review the diagnosis and treatment of ovarian cancer in pregnancy.
Is Mirabegron Equally as Effective When Used as First- or Second-Line Therapy in Women With Overactive Bladder?
Authors: Serati Maurizio , Roberti Maggiore Umberto Leone , Sorice Paola , Cantaluppi Simona , Agrò Enrico Finazzi , Ghezzi Fabio 
Published: March 2017

(Abstracted from Int Urogynecol J 2016; DOI 10.1007/s00192-016-3219-x)
Oral antimuscarinic agents have been the criterion standard of pharmacotherapy for the treatment of overactive bladder (OAB) symptoms for several decades. Mirabegron, a selective β3-adrenergic receptor agonist, represents a new class of drugs for OAB with proven efficacy, good safety, and tolerability.