Goal-Directed Rehabilitation Versus Standard Care for Individuals with Hereditary Cerebellar Ataxia: A Multicenter, Single-Blind, Randomized Controlled Superiority Trial.

Objective: Rehabilitation is thought to reduce ataxia severity in individuals with hereditary cerebellar ataxia (HCA). This multicenter, randomized controlled superiority trial aimed to examine the efficacy of a 30-week goal-directed rehabilitation program compared with 30 weeks of standard care on function, ataxia, health-related quality of life, and balance in individuals with an HCA.

Methods: Individuals with an autosomal dominant or recessive ataxia (aged ≥15 years) were enrolled at 5 sites in Australia.

Interrater Reliability of the Scale for the Assessment and Rating of Ataxia, Berg Balance Scale, and Functional Independence Measure Motor Domain in Individuals With Hereditary Cerebellar Ataxia.

Objective: To determine the interrater reliability of the Scale for the Assessment and Rating of Ataxia (SARA), Berg Balance Scale (BBS), and motor domain of the FIM (m-FIM) administered by physiotherapists in individuals with a hereditary cerebellar ataxia (HCA).

Design: Participants were assessed by 1 of 4 physiotherapists. Assessments were video-recorded and the remaining 3 physiotherapists scored the scales for each participant.

In-line sample trap columns with diatomite for large-volume injection in CZE-IM-MS.

The analysis of low-abundant compounds with capillary zone electrophoresis-drift-tube ion mobility spectrometry-mass spectrometry (CZE-DTIMS-MS) is compromised due to the low injectable sample volumes in CZE and low duty cycle in DTIMS. Fritless packed in-line trap columns, using solid-phase extraction sorbent particles, have been used to increase injection volumes in CZE, but these columns are difficult to prepare and exhibit rapidly increasing back pressures. To provide smooth and complete filling of trap columns as well as to ensure higher and sustained flow rates though the columns, blends of cation and anion exchange particles with diatomite were used.

Rehabilitation for ataxia study: protocol for a randomised controlled trial of an outpatient and supported home-based physiotherapy programme for people with hereditary cerebellar ataxia.

Introduction: Emerging evidence indicates that rehabilitation can improve ataxia, mobility and independence in everyday activities in individuals with hereditary cerebellar ataxia. However, with the rarity of the genetic ataxias and known recruitment challenges in rehabilitation trials, most studies have been underpowered, non-randomised or non-controlled. This study will be the first, appropriately powered randomised controlled trial to examine the efficacy of an outpatient and home-based rehabilitation programme on improving motor function for individuals with hereditary cerebellar ataxia.

Postpartum amenorrhoea-galactorrhoea associated with hyperprolactinaemia and pituitary enlargement in primary hypothyroidism.

We report a 36-year-old woman with primary hypothyroidism revealed by postpartum amenorrhoea-galactorrhoea associated with hyperprolactinaemia and suprasellar pituitary enlargement on magnetic resonance imaging (MRI). On thyroid hormone replacement therapy all clinical, biochemical, radiological and endocrine abnormalities disappeared. Hyperplasia of pituitary thyrotrophs and/or lactotrophs seems to be responsible for the pituitary enlargement seen on MRI.

Endotoxin-induced liver damage in rats is minimized by beta 2-adrenoceptor stimulation.

Objective And Design: To investigate the effects of beta(2)-adrenoceptor (beta(2)-AR) stimulation on endotoxin-induced liver damage and systemic cytokine levels in rats.

Subjects: Standard male Wistar rats.

Treatment: A disease-model of lipopolysaccharide (LPS)-induced acute systemic inflammation was used.

High volume continuous venovenous haemofiltration (HV-CVVH) in an equine endotoxaemic shock model.

Equine acute abdominal disease is often associated with shock. Important aspects in the onset of this complication include hypovolaemia, the translocation of endotoxins from the gut and the subsequent activation of the cytokine network. The clinical efficacy of high volume continuous venovenous haemofiltration (HV-CVVH) and the clearance of cytokines were therefore investigated in an equine endotoxaemic model.

[Recurrent attacks of angioedema ascribed to the use of estrogen preparations and a pregnancy (hereditary angioedema type 3)].

A woman experienced recurrent attacks of angioedema from the age of 17 to 21 years and these appeared to be associated with the use of oestrogens. After stopping the medication her complaints disappeared, but they returned during her first pregnancy. Angioedema is a serious condition, which can lead to acute abdominal symptoms, oedema of the upper respiratory tract and death by asphyxiation.

Oedema disease is associated with metabolic acidosis and small intestinal acidosis.

Limited information is available about the pathogenesis and pathophysiology of oedema disease (OD). Oedema disease is caused by specific enterotoxemic Escherichia coli (SLTIIv-toxin producing) strains; however, the same strains are also found in non-afflicted pigs. Furthermore, it is unclear how the 80 kDa SLTIIv-toxin can pass the intestinal barrier.

Asystole during combination chemotherapy for non-Hodgkin's lymphoma: the acute tumor lysis syndrome.

The acute tumor lysis syndrome is a rare condition that has most frequently been documented in patients with rapidly dividing myeloproliferative and lymphoproliferative malignancies. It is characterized by the development of hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, acute renal failure and metabolic acidosis, as a result of massive tumor cell destruction, usually secondary to effective cytotoxic treatment. We want to present the case history of a 62-year-old woman who died from cardiorespiratory arrest during combination chemotherapy for non-Hodgkin's lymphoma due to acute tumor lysis syndrome.

Chronic tophaceous gout in the elderly.

Gout in the elderly seems to represent a special subgroup. The presentation is often atypical and drug treatment poses special difficulties. We present two elderly patients with large subcutaneous tophi leading to grotesque deformities.

Pneumomediastinum: an unusual complication of bronchial asthma in a young man.

We report a patient with bronchial asthma who presented with pneumomediastinum, pneumopericardium, pneumoretroperitoneum, pneumorrhachis and extensive subcutaneous emphysema, after a period of coughing. Pathogenesis, diagnostic procedures and treatment of pneumomediastinum and its complications are discussed.

[Meningo-encephalitis and hydrocephalus caused by Epstein-Barr virus].

In a 35-year-old woman who presented with acute somnolence, confusion and slow irregular breathing, Epstein-Barr virus (EBV) meningoencephalitis was diagnosed after serological testing and a polymerase chain reaction of the cerebrospinal fluid. She developed papilloedema and bilateral nervus abducens paresis. A CT scan showed generalized oedema of the brain and triventricular hydrocephalus.

High volume hemofiltration improves hemodynamics and survival of pigs exposed to gut ischemia and reperfusion.

This study assesses the influence of high volume continuous hemofiltration on hemodynamics of pigs subjected to bowel ischemia/reperfusion. Twelve anesthetized and ventilated pigs were studied for 60 min during clamping of the superior mesenteric artery (SMA) and subsequently for 90 min after release of the clamp, while measuring global hemodynamics, SMA flow, and jejunal pCO2. They were randomly divided into two groups: pigs in "control" group were subjected to SMA clamping only.

The potential role of hemofiltration in the treatment of patients with septic shock and multiple organ dysfunction syndrome.

Hemofiltration may improve the hemodynamics of septic shock and the survival of multiple organ dysfunction syndrome patients. The pros and cons of this statement are reviewed and the practical implications are discussed.

Infusion of ultrafiltrate from endotoxemic pigs depresses myocardial performance in normal pigs.

We previously showed a beneficial effect of hemofiltration on hemodynamics of endotoxic shock pigs. To test the hypothesis that this effect of hemofiltration is caused by convective removal of factors that adversely affect hemodynamics during endotoxemia, we infused ultrafiltrate from endotoxic shock pigs into healthy pigs. Their hemodynamics were compared with those of pigs who were infused with ultrafiltrate from healthy pigs.

The role of hemofiltration in the critically-ill intensive care unit patient: present and future.

The development of acute renal failure (ARF) in intensive care unit (ICU) patients carries a poor prognosis. Today, most cases are no longer an isolated organ dysfunction but are part of a multiple system organ failure syndrome. Recently developed renal replacement therapies allow adequate metabolic control, even in highly catabolic and hemodynamically unstable patients.

High volume hemofiltration improves right ventricular function in endotoxin-induced shock in the pig.

This study assessed the influence of continuous high volume hemofiltration on right ventricular function of pigs with endotoxin induced shock. Eighteen anesthetized and ventilated pigs were studied for 240 min after the start of infusion of 0.5 mg/kg endotoxin over 30 min.

Hemodynamic aspects of multiple organ failure.

Multiple organ failure (MOF) secondary to sepsis is associated with a high mortality. A large body of evidence suggests that the disturbed relationship between oxygen supply and oxygen uptake plays an important role in the pathogenesis of MOF. The relationship between oxygen-supply dependency and MOF and the practical implications of the relationship are reviewed.

Albumin abuse in intensive care medicine.

Albumin is a much abused and expensive drug in intensive care units. One of the motivations for its use is the prevention of pulmonary edema by enhancing the colloid osmotic pressure (COP). Fear of pulmonary edema has led to the formation of a magic (arbitrary) albumin value varying from one intensive care unit to another.