Clinicopathological study of lepromatous leprosy and histoid leprosy in a tertiary care institute

Background: Lepromatous leprosy is a clinical course seen in patients with inefficient cellular immunity against Mycobacterium leprae, resulting in anergy. A highly bacilliferrous type of lepromatous leprosy is histoid leprosy. Histoid leprosy was initially reported to manifest after the failure of long-term dapsone monotherapy, irregular therapy, or inadequate therapy. However, it is now well known that histoid leprosy develops de-novo as well. This study was undertaken to know the incidence and clinicopathological characteristics of lepromatous leprosy and histoid leprosy.Materials and Methods: This was a combined (both retrospective and prospective) study of lepromatous and histoid Leprosy, undertaken in the Department of Pathology, Karnataka Institute of Medical Sciences, Hubballi, over 42 months from January 2013 to June 2016.Results: Out of the 16 clinically diagnosed lepromatous leprosy cases, only 5 were histologically proven to be the same. Of the seven clinically diagnosed histoid leprosy cases, 4 were histoid leprosy on histopathology and the remaining 3 were lepromatous leprosy. All 4 patients of histoid leprosy were cases of De- Novo histoid leprosy. The commonest skin lesion encountered were erythematous patches (9/16) in lepromatous leprosy and nodules (5/7) in histoid leprosy.Conclusions: Lepromatous leprosy when diagnosed in an early stage, interrupts the epidemiological chain and avoids the associated disabilities and psychological effects for the patient and family. Histoid leprosy is a rare entity that can also present with no history of prior anti-leprosy treatment, the diagnosis of “de-novo histoid leprosy” should be kept in mind.

Present situation in hemophilia therapy: current standards and future expectations

Гемофилии — заболевания, вызванные единичными мутациями в генах и проявляющиеся дефицитом белков, участвующих в каскадах свертывания крови. Характеризуются приобретённой тенденцией к кровотечениям на протяжении всей жизни и представляют собой потенциально жизнеугрожающее состояние. Важно вовремя диагностировать данную патологию крови и своевременно начать профилактику, чтобы избежать возможных осложнений и эпизодов сильных кровотечений. Стандартом на сегодняшний день является заместительная терапия недостающих факторов свертывания, исследуются способы увеличения периодов полураспада и меры предупреждения развития аутоантител — внедряются подкожные формы введения препаратов. Однако сегодня профилактика и лечение гемофилии — это не только заместительная терапия недостающих факторов свертывания. В разработках и на разных стадиях клинических исследований находятся потенциальные мишени для опосредованного восполнения недостающих факторов свертывания. Основная цель в лечении гемофилий — профилактика кровотечений, но при неадекватной терапии, потере ее эффективности или отсутствии своевременной диагностики развившееся кровотечение представляет собой угрозу для жизни пациентов и требует специфического подхода. Hemophilia is a disease caused by single gene mutation and is manifested by a defi ciency of proteins involved in blood coagulation cascades. This disease is characterized by an acquired tendency to bleed throughout life and represents a potentially life-threatening condition. It is important to recognize this condition early and start the prevention in time to avoid possible complications and episodes of severe bleeding. The standard therapy today is replacement for coagulation factors; methods of increasing the half-life and prevention of the development of autoantibodies are being studied — subcutaneous forms of drug administration are being introduced. However, today the prevention and treatment of hemophilia is not only replacement therapy of coagulation factors. Potential targets for mediated replacement of missing coagulation factors are in development and at various stages of clinical research. The main goal in the treatment of hemophilia is to prevent bleeding, but with inadequate therapy, loss of its effectiveness or lack of timely diagnosis, severe bleeding is a threat to the lives of patients, and requires a specific approach.

Mortality After Delay of Adequate Empiric Antimicrobial Treatment of Bloodstream Infection

Background: Timely empiric antimicrobial therapy is one of the cornerstones of the management of suspected bloodstream infection (BSI). However, studies about the effects of empiric therapy on mortality have reported inconsistent results. The objective of this study was to estimate the effect of delay of appropriate empiric therapy on early mortality in patients with BSI. Methods: Data for the propensity score matching (PSM) study were obtained from a cohort of patients with BSI. Inadequate empiric treatment was defined as in vitro resistance to the antimicrobial regimen administered <6 h after blood cultures were taken. The primary outcome measure was 14-day mortality. Thirty-day mortality and median length of stay (LOS) were secondary outcomes. PSM was applied to control for confounding. Results: Of a total of 893 included patients with BSI, 35.7% received inadequate initial empiric treatment. In the PSM cohort (n = 334), 14-day mortality was 9.6% for inadequate antibiotic treatment, compared to. 10.2% in adequate empiric treatment (p = 0.85). No prolonged median LOS was observed in patients who initially received inadequate therapy (10.5 vs. 10.7 days, p = 0.89). Conclusions: In this study, we found no clear effect of inadequate empirical treatment on mortality in a low-risk BSI population. The importance of early empiric therapy compared to other determinants, may be limited. This may not apply for specific subpopulations, e.g., patients with sepsis.

Spinal extradural meningioma en plaque with nerve root attachment and extracanal (intrathoracic) extension

Meningiomas are relatively common primary spinal tumours, being the second most common intraspinal tumours probably after vertebral haemangioma.It constitutes about 25% of all intraspinal tumours; however, in the presence of extradural spinal lesions, the diagnosis of meningioma is uncommon and often not among the first two considerations. Purely extradural spinal meningioma, especially of the “En plaque” variety, usually simulate malignant disease (metastatic diseases and lymphoma) and may result in inadequate therapy, however, the presence of nerve root attachment is even rarer.Our case report is that of an entirely cervicothoracic extradural en?plaque meningioma (WHO grade 1) with a nerve root attachment (right C7) and intrathoracic extension. We highlighted the issues in diagnosis, operative intervention and long-term follow-up.

Challenges in the Contemporary Management of Syphilis among Pregnant Women in New Orleans, LA

Objective. The aim of this retrospective review is to evaluate trends in the management of maternal and congenital syphilis (CS) in a tertiary care center in New Orleans, LA. Study Design. All cases of maternal and neonatal syphilis over a five year period at Touro Infirmary, New Orleans, LA, were identified using ICD-9/10 codes. Charts were reviewed for demographic and obstetrical variables, stage of syphilis at diagnosis, lab values, and treatment regimen. Newborn treatment and other outcomes were recorded. Results. During the study period 106 infected mother-baby pairs were identified. Of these, 73 charts are available for review. 41% (n = 30) of women received inadequate therapy according to their stage of disease. 9% of newborns (n = 6) were found to be symptomatic for CS; however, only 83.3% of these were admitted to the neonatal intensive care unit. Only 20% (n = 6) of infants were adequately treated with an extended penicillin regimen if the mother was not adequately treated. Furthermore, only 63.0% of newborns had a nontreponemal titer performed. Conclusion. With rising rates of CS, strict adherence to the 2015 CDC guidelines for treatment of syphilis must be maintained.

Juvenile myoclonic epilepsy: neurophysiological aspects

The review presents an update on the electroencephalographic characteristics and neurophysiology of juvenile myoclonic epilepsy (JME) in Russia and foreign countries.Materials and methods.The authors have performed a literary search for the full-text publications on JME electroencephalography and neurophysiology worldwide and in the Russian Federation, which are available in Russian and foreign databases.Results and discussion. Analysis of the literature suggests that JME patients have some electroencephalographic pattern features that include the atypical morphology of spike-and-slow-wave complexes, as well as amplitude asymmetry, focal discharges, focal onset of paroxysms, focal paroxysmal shift, and generalized paroxysmal fast rhythm.The analysis of the literature also indicates that primary care neurologists, clinical neurophysiologists, and epileptologists should be informed about the electroencephalographic characteristics of JME to timely diagnose the disease and to rule out the use of inadequate therapy.

A Systematic Review and Meta-Analysis of Epidemiology and Clinical Manifestations of Human Brucellosis in China

Background. Brucellosis has a wide spectrum of clinical manifestations and it may last several days or even several years; however, it is often misdiagnosed and therefore may cause inadequate therapy and prolonged illness. Previous studies about meta-analysis of manifestations of brucellosis reported in English lacked the data published in Chinese, which did not provide details about the contact history, laboratory tests, and misdiagnosis. We undertake a meta-analysis of clinical manifestations of human brucellosis in China to identify those gaps in the literature. We have searched published articles in electronic databases up to December 2016 identified as relating to clinical features of human brucellosis in China. 68 studies were included in the analysis. The main clinical manifestations were fever, fatigue, arthralgia, and muscle pain (87%, 63%, 62%, and 56%, resp.). There are significant differences between adults and children. Rash, respiratory and cardiac complications, and orchitis/epididymitis were more prevalent in children patients. The common complications of brucellosis were hepatitis, followed by osteoarthritis, respiratory diseases, cardiovascular diseases, central nervous system dysfunction, hemophagocytic syndrome, and orchitis/epididymitis in male. In the nonpastoral areas, brucellosis has a high ratio of misdiagnosis. Our analysis provides further evidence for the accurate diagnosis, particularly in assessing severe, debilitating sequelae of this infection.

Invalidization in patients with myasthenia, factors affecting disability

Myasthenia gravis, as a disease associated with impaired neuromuscular transmission and manifested by muscle weakness and fatigue, necessarily leads to the disability of patients. There were analyzed terms and the factors influencing upon disability in 52 patients with myasthenia, 67 per cent of whom had a disability group. The duration of the disease from the beginning to the referral to the examination in the Bureau of the Medical and Social Expertise was revealed to amount in average of 2.68 ± 0.44 years. Out of factors influencing upon more earlier disability there were noted primary or early generalization of the process, severe crisis course and pronounced severity according to MGFA, poor compensation on anticholinesterase drugs, inadequate therapy. There was noted the positive effect of thymectomy on the consequent course of the disease, only 2 out of the 18 operated patients showed its further progression.