Neurofibromatosis type 1 first presented as hypertensive crisis.

Neurofibromatosis type 1 (NF1) is known to cause vascular complications including stenotic and aneurysmal disease. Here, we report a case of a woman in her early 20s, who presented with unilateral facial nerve palsy and hypertensive crisis, and was later found to have multiple bilateral intrarenal microaneurysms along with classic cutaneous manifestations of NF1. A causal relationship between the pathophysiology of NF1 and the development of renal artery microaneurysm and hypertension is proposed in this case report.

Psychosocial factors in patients with kidney failure and role for social worker: A secondary data audit.

BACKGROUND: People with kidney failure face a multitude of psychosocial stressors that affect disease trajectory and health outcomes. OBJECTIVES: To investigate psychosocial factors affecting people with kidney failure before or at start of kidney replacement therapy (KRT) and kidney supportive and palliative care (KSPC) phases of illness and to explore role of social worker during the illness trajectory. METHODS: We conducted a secondary data audit of patients either before or at start of KRT (Phase 1) and at the KSPC (Phase 2) of illness and had psychosocial assessments between March 2012 and March 2020 in an Australian setting. RESULTS: Seventy-nine individuals, aged 70 ± 12 years, had at least two psychosocial assessments, one in each of the two phases of illness. The median time between social worker evaluations in Phase 1 and Phase 2 was 522 (116-943) days. Adjustment to illness and treatment (90%) was the most prevalent psychosocial issue identified in Phase 1, which declined to 39% in Phase 2. Need for aged care assistance (7.6%-63%; p < 0.001) and carer support (7.6%-42%; p < 0.001) increased significantly from Phase 1 to Phase 2. There was a significant increase in psychosocial interventions by the social worker in Phase 2, including supportive counselling (53%-73%; p < 0.05), provision of education and information (43%-65%; p < 0.01), and referrals (28%-62%; p < 0.01). CONCLUSION: Adults nearing or at the start of KRT experience immense psychosocial burden and adaptive demands that recognisably change during the course of illness. The positive role played by the nephrology social worker warrants further investigation.

Psychosocial factors affecting patients with end-stage kidney disease and the impact of the social worker.

BACKGROUND: End-stage kidney disease (ESKD) incidence has been increasing over time, contributing significantly to morbidity and mortality. However, there is limited data examining the psychosocial factors affecting people with ESKD and how the social worker fits within the multidisciplinary CKD care. This integrative systematic review aims to summarise the existing evidence on psychosocial determinants of outcomes in ESKD and the role of the social worker in nephrology care. METHOD: The literature search was conducted using PubMed and MEDLINE, targeting articles published from database inception until May 2021. This systematic review was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. The Joanna Briggs Institute tools were employed to assess the quality of included studies. RESULTS: Of the 397 citations, 13 studies applicable to 1465 patients met the inclusion criteria. The studies were of cross-sectional, experimental, and exploratory qualitative design in nature. The findings of the studies were summarised into three major themes-psychosocial factors, role of the renal social worker, and impact of the renal social worker. The studies demonstrated that concerns related to adjustment, death and dying, family and social functioning, and loss were common amongst participants of the included studies indicating the need for a social worker. Three studies explored the impact of social workers in ESKD, revealing that people who received support from social workers had an improved quality of life, lower depression scores, and reduced hospitalisations and emergency room visits. CONCLUSION: This review summarizes the multitude of physical and psychological stressors that patients with ESRD face, and highlights the positive role social workers can play in improving the psychosocial stressors in this patient group, and the need for large-scale randomised trials to understand the role of social workers as part of a multidisciplinary nephrology care.

Coronary artery vasculitis: a review of current literature.

Cardiac vasculitis is recognized as a heterogeneous disease process with a wide spectrum of manifestations including pericarditis, myocarditis, valvular heart disease and less frequently, coronary artery vasculitis (CAV). CAV encompasses an emerging field of diseases which differ from conventional atherosclerotic disease and have a proclivity for the younger population groups. CAV portends multiple complications including the development of coronary artery aneurysms, coronary stenotic lesions, and thrombosis, all which may result in acute coronary syndromes. There are several aetiologies for CAV; with Kawasaki's disease, Takayasu's arteritis, Polyarteritis Nodosa, and Giant-Cell Arteritis more frequently described clinically, and in literature. There is a growing role for multi-modality imaging in assisting the diagnostic process; including transthoracic echocardiography, cardiac magnetic resonance imaging, computed tomography coronary angiography, fluorodeoxyglucose-positron emission tomography and conventional coronary angiogram with intravascular ultrasound. Whilst the treatment paradigms fundamentally vary between different aetiologies, there are overlaps with pharmacological regimes in immunosuppressive agents and anti-platelet therapies. Interventional and surgical management are is a consideration in select populations groups, within a multi-disciplinary context. Further large-scale studies are required to better appropriately outline management protocols in this niche population.

Danaparoid use for haemodialysis in a morbidly obese patient with heparin-induced thrombocytopenia - Need for a higher than recommended weight-based dosing.

WHAT IS KNOWN AND OBJECTIVE: Heparin is widely used to prevent clotting of the extracorporeal circuit during haemodialysis (HD). Heparin-induced thrombocytopenia (HIT) is a potentially devastating immune mediated adverse drug reaction caused by the emergence of antibodies that activate platelets in the presence of heparin, leading to a pro-thrombotic state. Danaparoid is an alternative anticoagulant used in patients on HD with HIT but its dosing recommendations in obese patients on HD are relatively scarce. CASE SUMMARY: We report a case of a 48-year-old morbidly obese patient who received weight-based dosing of danaparoid for HD with monitoring of anti-Xa activity. However, despite the patient's anti-Xa level being within the therapeutic range at various time points, the circuit lines kept clotting during HD. WHAT IS NEW AND CONCLUSION: The report provides evidence that the manufacturer's recommendations on dosing danaparoid based on body weight may lead to sub-optimal therapeutic benefit and highlight the need for higher than recommended weight-based dosing in obese individuals on dialysis.

Prominent subcutaneous oedema as a masquerading symptom of an underlying inflammatory myopathy.

The inflammatory myopathies are a group of immune-mediated inflammatory muscle disorders that typically present with marked proximal muscle weakness. We report four cases of inflammatory myopathies with marked subcutaneous oedema as their main complaint. Three of the four patients had normal or low levels of creatine kinase, an enzyme often markedly elevated in these disorders. Magnetic resonance imaging of the muscles, followed by a muscle biopsy were used to make a definitive diagnosis.

A case of valproate induced hyperammonemic encephalopathy.

A 36-years-old man on phenytoin, levetiracetam, and sodium valproate presented with acute confusion. Routine investigations including serum valproate and phenytoin concentration were normal. His serum ammonia concentration was raised. His valproate was held and 2 days later he recovered with concordant normalisation of serum ammonia concentration. Urea acid cycle disorder was ruled out, and a diagnosis of valproate induced hyperammonemic encephalopathy (VHE) was made. Asymptomatic hyperammonemia occurs in 15-50% of valproate-treated patients, and while the true incidence of VHE is not known, it is a recognized complication of sodium valproate treatment. VHE typically presents acutely with impaired consciousness, lethargy, and vomiting. Valproate concentrations may be in the therapeutic range, and liver function tests are typically "normal." Treatment for VHE consists of ceasing valproate and providing supportive care. Some have advocated carnitine replacement.