Harefuah最新文献

Introduction:

Background: Dizziness is a common condition that accounts for an estimated 5% of primary care clinic visits. The differential diagnosis is extensive, which may involve the inner ear, central and peripheral nervous system and the cardiovascular system among others. While routine cases can be diagnosed and treated with primary (general practitioner) or secondary (otolaryngologists (medicine in the community, there are many cases in which it is advisable to refer patients to a tertiary dizziness clinic. These cases include: unclear diagnosis, lack of improvement after medication or physiotherapy, medical comorbidities in which the central compensation process has been affected, chronic dizziness, bilateral vestibular disorder and more. Therefore, we established a multidisciplinary clinic (MDC) in a tertiary referral center at the Rambam Healthcare Campus.

Objectives: To investigate the first year of the tertiary MDC neurotology clinic.

Methods: Retrospective cohort of all patients examined at the tertiary MDC.

Results: The first 123 consecutive patients (62% women) aged 56 years on average (19-85years, ±16) seen in the MDC from October 1, 2020 to October 31, 2021 are reported. Prior to assessment, 79 (64%) patients had been referred by an ENT doctor in the community with an unclear cause of dizziness; 49 (40%) patients experienced ongoing symptoms for years and 61 (50%) patients had dizziness on a daily basis. A total of 56 )46%) patients were found to have a peripheral vestibular cause for their dizziness and 67 (54%) patients had a central or other causes. More than one cause of dizziness and/or imbalance was noted in 20 (36%) of patients. Among the common peripheral vestibular causes were benign paroxysmal positional vertigo (BPPV) and Meniere's disease, and the central and functional disorders included vestibular migraine and persistent postural perceptual dizziness (PPPD). The frequency and intensity of symptoms were reduced in 29 patients of the 33 (88%) who underwent vestibular physiotherapy in the MDC as opposed to 8 (50%) patients in the community (p <0.05). Physiotherapy was found to be more effective in patients younger than 65 years old, and with less than six months of symptoms (p <0.05). However, patients with a medical history of neurological or cardiovascular problems are less likely to improve (p <0.05).

Conclusions: A tertiary neurotology clinic of a multidisciplinary team can provide proper care in persistent dizziness and/or unclear diagnosis. Vestibular physiotherapy plays a crucial role in the management of a dizzy patient, and hence it should be started as soon as possible.

Myopia, short-sightedness, is the most frequent cause of vision difficulty and its prevalence is expected to rise. Nowadays, the usage of atropine drops is effective for myopia progression control. Studies on the Asian population showed that atropine 0.01% is more efficient and safer than solutions that have a higher concentration. In this study, we report findings from a multicenter Spanish study that showed that atropine 0.01% decreases the progression of myopia in a pediatric population, with relatively few adverse effects. The results of this study may be relevant to doctors who treat children who have myopia, as well as to the children treated and their parents.

Introduction: The rise in women's age globally, as well as lifetime hard physical work and high parity rate in the third world countries, is a substantial cause for female genital organ prolapse of variable degrees of severity. This, in turn, has a negative effect on the function of the affected women. The definitive treatment for this condition is surgery of various types in accordance with the anatomic injury, although it is not easily available throughout the world. Hence the use of vaginal pessaries, which is quite prevalent among the affected women, mainly in third world countries. These contraptions enable fixation of the prolapsed female pelvic organs in the pelvis and prevent their descent to the vagina and beyond. The most prevalent vaginal ring is the pessary ring, although a large variety of vaginal rings are available. In this article the authors review some of the aspects regarding the use of vaginal rings for pelvic organ prolapse. Generally, the use of these rings was found safe and effective - both by physicians as well as users, however, reports of some negative complications related to the use of these devices have been published. Another clinical experimental aspect of the use of these rings is to try to prevent premature delivery in pregnant women. Currently, this treatment option is still regarded as being controversial in terms of its effectiveness for this purpose.

Introduction: This case involved a 67-year-old female who was admitted for general anesthesia for a mitral clip procedure. Following anesthesia induction, the patient underwent an uneventful orotracheal intubation. Shortly afterwards she developed an ongoing respiratory failure, accompanied by neck and chest subcutaneous emphysema. Upon workup, she was found to have a 6 cm long tracheal laceration on the posterior side. Emergency repair surgery was performed using an extracorporeal membrane oxygenator (ECMO). The patient passed away 11 days later from sepsis. The aim of this case report is to increase awareness of this rare intubation complication, and explore the best approach to prevent, diagnose and treat tracheal injuries during endotracheal intubation.

Introduction: Factor XI (FXI) deficiency is an autosomal bleeding disorder characterized by injury-related hemorrhage, mostly associated with surgical procedures at sites noted for high fibrinolytic activity. Severe FXI deficiency is defined when the FXI level is lower than 15-20 IU/dL. Perioperative prophylactic treatment for high-bleeding-risk surgery in patients with severe FXI deficiency is based on fresh frozen plasma (FFP) transfusions or FXI concentrate (where available). Exposure to FFP and to FXI concentrate may lead to the development of inhibitory antibodies against FXI. This phenomenon occurs mostly in patients with very severe FXI deficiency (baseline FXI <1IU/dL) and is associated with an increased risk of substantial perioperative bleeding, unresponsive to FXI replacement. Thus, in individuals with severe FXI deficiency, routine testing for the presence of inhibitory antibodies against FXI is recommended. We present a 17-year-old adolescent patient with very severe FXI deficiency, who developed an inhibitor to FXI following FFP exposure associated with neurosurgery for medulloblastoma. Prophylactic treatment for subsequent invasive procedures consisted of single low dose (10 mcg/kg) recombinant activated factor VII (rFVIIa) and tranexamic acid (Hexakapron). The procedures were performed uneventfully, with no hemorrhagic or thrombotic complications. In patients with very severe FXI deficiency, the development of inhibitory antibodies following plasma replacement therapy comprises a rare and challenging occurrence. The formulation of a safe and effective evidence-based protocol for hemostatic support in these patients requires multi-center collaboration.

Introduction: Although Hematopoietic Stem Cell Transplantation (HSCT) is the only curative option for children with certain non-malignant disorders, a proportion of these children are admitted to the Pediatric Intensive Care Unit (PICU) due to treatment related life-threatening complications.

Aims: To analyze risk factors for ICU hospitalizations, morbidity and mortality in children with genetic diseases who have undergone HSCT and were admitted to intensive care units.

Methods: This retrospective study is based on the collection and analysis of clinical and laboratory data from the medical records of patients from the departments of Bone Marrow Transplantations and Intensive Care, from 2 hospitals, Hadassah and Rambam Medical Centers.

Results: Over the course of 15 years (2005-2019), 463 HSCT were performed for pediatric patients with non-malignant diseases, 68 of them (15%) required hospitalization in Intensive Care Units (ICU), 41% of the patients survived. The PICU mortality rate has decreased over the last years. Factors found to have a significant negative impact on PICU survival were severe neutropenia at admission to ICU, mechanical ventilation, inotropic support, and Multi Organ Failure (MOF).

Conclusions: Our results showed low incidence of ICU admissions and relatively high survival rate for pediatric patients with non-malignant disorders post HSCT, comparing with literature data.

Introduction: Langerhans cell histiocytosis (LCH) is a histiocytic disorder which is characterized by a wide variety of clinical presentations and is prevalent mostly in children .This is a single center study reviewing experience in the treatment of LCH in a pediatric hemato-oncology ward over 25 years. We summarized the demographics, the locations of the disease, the treatments administered, the reactivations and the survival of the patients.

Methods: A retrospective analysis of files was performed from patients who were referred and treated at the Dana-Dwek Children's hospital in the Tel-Aviv Sourasky Medical Center between the years 1996-2020.

Results: One hundred and six patients with LCH were treated during the period 1996-2020 in the Pediatric Hemato-Oncology division. The diagnosis was confirmed by a biopsy from a lesion. The primary location of the disease was single system in 91% of patients (mostly bone lesions) and 9% multisystem disease. Forty-five patients (42.4%) were treated by upfront chemotherapy according to the Histiocyte Society guidelines. Twenty patients (19%) had reactivation of their disease. Ninety percent of the reactivations occurred in the first four years after diagnosis. There was no mortality in this cohort.

Conclusions: This is a single center study summarizing the experience of a Pediatric Hemato-Oncology division in the Tel-Aviv Medical Center in the treatment of 106 patients with LCH over 25 years and is the first review of a large cohort of patients in Israel. The cohort was characterized by abundance of patients with bone disease and paucity of patients with multisystem disease with risk organ involvement. There was overall good response to treatment and all patients survived.

Introduction: The treatment of children with oncological and hematological diseases and bone marrow transplantations is under the same roof in the departments and units in Israel. The dramatic improvement in recent decades is a result of the biological understanding of the diseases, new biological and immunological medications, technological progress, database registration, joining large international groups and clinical trials in medical treatment as well as improvements in radiation therapy, surgery and supportive care. In this booklet, we present review articles on the progress in some of the common diseases in hematology and oncology for children, emphasizing the molecular tests that can be used to determine the diagnosis, introducing precision medicine implementing innovative biological and immunotherapeutic drugs and in order to shed light on the diagnosis of congenital genetic disease exposing cancer development. We also update on the work being conducted in the various departments in these fields. As more than 84% of all children diagnosed with cancer will experience long-term survival or cure, the significant challenges remaining for the treatment teams today include treating the recurrence of disease, as well as reducing the long-term side effects in order to improve the quality of life of the survivors.

Introduction:

Aims: To characterize the clinical, demographic, laboratory, and molecular biologic findings in children with HLH diagnosed and treated in our center.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare immuno-hematologic disorder more common in children than adults. It is divided into two separate conditions which are not always easy to differentiate: familial/primary HLH (FHL) and acquired/secondary HLH associated with malignant, infectious, and other inflammatory conditions. FHL is a life-threatening disorder caused mainly by mutations in genes that code for proteins participating in perforin-dependent cell death.

Methods: We collected data from the records of all children who were diagnosed with HLH according to the accepted HLH criteria, and treated in the Schneider Children's Medical Center of Israel between the years 2004-2020. Demographic, clinical and laboratory data were summarized into a national database.

Results: A total of 36 children (18 boys,18 girls) who were diagnosed as having HLH according to the diagnostic criteria and received treatment according to the Histiocyte Society '94 or 2004 international protocols, entered the study. Eleven of the patients were Arab (30%), while 25 were Jewish (70%). The most frequent clinical signs were fever (89%) and hepatosplenomegaly (61%). Laboratory tests showed bipenia in 100% and increased levels of Interleukin-2 receptor (IL-2R) in 97%. Mutations in 3 out of the 4 common HLH genes were found in 15 (42%) children. The most common mutant gene was MUNC 13-4 comprising 40% of mutant genes. Mutations were quite common among Arab patients (81%) compared to the Jewish HLH population (20%) (p<0.001). Patients bi-allelic MUNC 13-4 mutations had a particularly poor prognosis with 66% succumbing to the disease.

Conclusions: HLH is a severe, multisystem disorder. High clinical suspicion leading to timely diagnosis and treatment are crucial for preventing poor outcome and death. Genetic studies are of upmost importance in our population due to the high percentage of mutations, especially in the Arab population.

Introduction: Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy, constituting approximately 25% of childhood cancers. In recent decades, survival rates have improved dramatically, from approximately 10% in the 1960's to over 90% today. This tremendous achievement has been accomplished through collaborative randomized clinical trials, with progressive evolution of highly efficient, risk-adapted multi-agent chemotherapeutic regimens, effective central nervous system prophylactic strategies and improved supportive care. Recently, our understanding of the genetic basis of ALL has been greatly enhanced, and precise methods for treatment response assessment with serial measurements of minimal residual disease have been developed. Certain patient subgroups have genetically targetable lesions, such as Philadelphia-positive ALL, whose outcomes have been dramatically improved by combined tyrosine kinase inhibitors and chemotherapy, or specific patient subsets of "Philadelphia-like" ALL. Despite the great progress in curing childhood ALL, significant challenges still remain. Acute adverse effects of chemotherapy may be life-threatening, and long-term side effects often impair survivors' quality of life. Survival rates in patients with relapsed or refractory ALL remain poor. This led to the introduction of novel immune-based therapies into the treatment of relapsed/refractory B-ALL: blinatumomab, a CD19 bi-specific T-cell engager; inotuzumab- a CD22-immunotoxin, and CD19-CAR (chimeric antigen receptor) T cells. These modalities have demonstrated improved remission rates with reduced toxicity compared to chemotherapy. The role of immunotherapy in the treatment of newly-diagnosed and relapsed patients will be more precisely defined in the near future.