Peripheral circulation disturbances in two consecutive children with spinal muscular atrophy and literature review.

Spinal muscular atrophy is a progressive and severe hereditary (autosomal recessive) neuromuscular disease characterized by lower motor neuron degeneration in the spinal cord and brainstem causing a clinical picture of progressive muscle atrophy and weakness of skeletal and respiratory muscles. There is an ongoing discussion on the extent to which other tissues might be affected in patients with SMA. Several animal models and some case reports or small case series report involvement of other organ systems, such as peripheral nerve, brain, muscle, heart, vascular system, and pancreas.

Acute Disseminated Encephalomyelitis followed by Optic Neuritis: A Rare Syndrome of Uncertain Treatment and Prognosis.

Aim: Acute Disseminated Encephalomyelitis followed by optic neuritis (ADEM-ON), first described in 2013, is a rare demyelinating syndrome, typical of the pediatric age. We conducted a mini review of the existing literature, focusing on clinical, laboratory, radiological, therapeutic, and prognostic aspects in order to improve the identification of new cases.

Methods: We searched PubMed and Cochrane Library for studies on ADEM-ON between 2013 and 2018.

The Italian version of the 92-item Prodromal Questionnaire: Concurrent validity with the SIPS and factor analysis in a sample of 258 outpatients aged 11-36years.

Background: Current early screeners for psychosis-risk states have still to prove ability in identifying at-risk individuals. Among screeners, the 92-item Prodromal Questionnaire (PQ-92) is often used. We aimed to assess the validity of its Italian translation in a large Italian adolescent and young adult help-seeking sample.

Endometrial preparation with estradiol plus dienogest (Qlaira) for office hysteroscopic polypectomy: randomized pilot study.

Study Objective: To estimate the effectiveness of Qlaira for endometrial preparation in women undergoing hysteroscopic polypectomy in the office setting.

Design: Randomized clinical pilot study (Canadian Task Force classification II-2).

Setting: Academic research environment.

Correspondence between hysteroscopic and histologic findings in women with chronic endometritis.

Objective: To estimate if findings in women with chronic endometritis (CE) at fluid hysteroscopy correlate with the severity of inflammation at histology and to assess the possibility to score the severity of inflammation by fluid hysteroscopy.

Design: Controlled clinical study.

Setting: University hospital.

Detection of bacterial contamination in apheresis platelet products: American Red Cross experience, 2004.

Background: Routine quality control (QC) testing for bacterial contamination in apheresis platelet (PLT) products was implemented in all 36 regional blood centers of the American Red Cross in March 2004.

Study Design And Methods: PLT samples were cultured under aerobic conditions until the end of the product shelf life or when a positive reaction was indicated. To confirm the initial positive reaction, a new sample was taken from the unit for reculturing.

Transmission of HIV and hepatitis C virus from a nursing home patient to a health care worker.

Background: We report a case of simultaneous HIV and hepatitis C virus (HCV) transmission from a nursing home patient to a health care worker (HCW) whose HIV and HCV infections were diagnosed during routine blood donor screening.

Methods: Detailed information about the HCW, possible occupational and nonoccupational blood and body fluid exposures, and possible source patient was collected. Blood samples were drawn from the HCW and patient, and HIV and HCV laboratory testing was performed at the Centers for Disease Control and Prevention.

Comparison of argon and CO2 laser stapedotomy in primary otosclerosis surgery.

Purpose: The primary use for the laser in otosclerosis surgery is to create a stapes footplate fenestration that obviates the need for mechanical footplate removal. Experimental studies that evaluate the potential safety of visible (argon and potassium-titanyl-phosphate [KTP]) and invisible (CO2) light laser systems in stapes surgery report conflicting results. The purpose of this study is to compare the clinical safety and efficacy of the CO2 and argon laser systems when used for primary laser stapedotomy.

Acoustic tumor growth: implications for treatment choices.

Background: Knowledge of acoustic neuroma (AN) growth is essential for treatment planning.

Methods: A retrospective analysis of 119 patients with AN (mean age, 65 years; range, 37-84 years) followed with interval magnetic resonance imagings (MRIs) was performed. Change in maximum tumor dimension as a function of follow-up period was analyzed.

Cochlear implants in the geriatric population: benefits outweigh risks.

Cochlear implantation has become widely accepted as an effective means of hearing rehabilitation in severely and profoundly deaf individuals. In the elderly, cochlear implantation involves a number of unique issues that can affect patient outcomes. These factors include age-related changes in the auditory system, prolonged durations of deafness, diminished communication abilities, and coexisting medical and psychosocial problems.

Intraoperative corticosteroids in acoustic tumor surgery.

Background: Corticosteroids are frequently used for the prevention and treatment of neural edema. Although perioperative steroid therapy has been used in patients undergoing acoustic neuroma removal, the efficacy of such therapy has not been previously documented.

Methods: A retrospective review of 169 patients who underwent acoustic neuroma surgery with (n = 75) or without (n = 94) a single dose of intraoperative corticosteroids was performed.

Trypanosoma cruzi in a low- to moderate-risk blood donor population: seroprevalence and possible congenital transmission.

Background: Several recent studies documented the seroprevalence of Trypanosoma cruzi in blood donors at high risk for infection, but little information is available regarding donors with lower levels of risk. Thus, the present study was designed to measure the seroprevalence of T. cruzi in a donor population with a low to moderate risk for infection.

Beta-2 transferrin assay in the identification of perilymph.

Hypothesis: Western blot assay for beta-2 transferrin protein is a clinically useful method for the detection of human perilymph and should be used for the diagnosis of perilymph fistulas (PLFs).

Background: Considerable controversy exists regarding the diagnosis of PLF. Recent studies suggest that the detection of beta-2 transferrin protein may be useful in the identification of perilymph.

Prosthesis size in stapedectomy.

Primary stapedectomies were performed on 60 patients with bilateral otosclerosis. Every patient had a 4 mm long Robinson prosthesis with a 0.4 mm wide shaft placed in one ear and a 4 mm long Robinson prosthesis with 0.

p53 mutations and chromosome instability in basal cell carcinomas developed at an early or late age.

Tumor DNA from 45 primary basal cell carcinoma (BCC) biopsies was screened for p53 gene mutations, chromosome 9 allele loss, and microsatellite instability. p53 mutation frequency increased significantly as a function of the age at BCC onset ranging from 6% (1/16) in early BCC (before age 40 years) to 35% (10/29) in late BCC. All p53 mutations found implicated sunlight as the mutagen.

Stapedectomy in the elderly.

Clinical records were reviewed to examine the effectiveness of stapedectomy in patients 70 years and older. A total of 154 patients was studied, including 11 with profound hearing loss with long-standing otosclerosis. Ages at the time of surgery ranged from 70 to 92 years (mean, 76.

Prosthesis on a mobilized stapes footplate.

Managing a mobilized footplate in stapedectomy surgery can be challenging. Between 1963 and 1992, 145 footplates were inadvertently mobilized during otosclerosis surgery. After a vein graft, a 4.

Acoustic reflexes, auditory brainstem response, and MRI in the evaluation of acoustic neuromas.

Patient records were reviewed to determine whether persons with absent acoustic reflexes have a higher incidence of abnormal auditory brainstem response (ABR) results in the absence of a cerebellopontine angle (CPA) tumor than those with normal acoustic reflexes. Results showed patients with absent reflexes to have borderline or abnormal ABR results in 45.2% of the cases.

Interobserver variability on the histopathologic diagnosis of cutaneous melanoma and other pigmented skin lesions.

Purpose: To assess the interobserver agreement on the diagnosis and classification of cutaneous melanoma.

Materials And Methods: A set of 140 slides of cutaneous melanoma, including a small subset of benign pigmented skin lesions, were circulated to four experienced histopathologists. The kappa statistic for multiple ratings per subject was calculated using the method described by Fleiss.

Prognostic value of anesthesia type for patients treated for cutaneous melanoma.

Background: General anesthesia has been reported to compromise several cell-mediated immune functions. Hence it is plausible that anesthesia could affect the postoperative outcome of cancer patients.

Objective: To study the influence of the type of anesthesia on survival of 459 patients with localized cutaneous melanoma observed between 1980 and 1991.

Vestibular nerve section.

Introduction: Vestibular nerve section is considered to be the most effective surgical procedure for control of intractable symptoms secondary to labyrinthine and eighth nerve function. This study was developed to retrospectively evaluate the efficacy of vestibular nerve section in patients treated for disabling labyrinthine dysfunction.

Methods: A retrospective review of hospital and office records was carried out on 39 patients who underwent vestibular nerve section.