The limit of sensitivity of the chemiluminescent assay for detection of bacteria by hemeprotein catalysis of luminol oxidation was determined, both experimentally and theoretically, to be no lower than 105 to 106 viable bacteria per ml.
Summary Phaeochromocytoma, a rare neuroendocrine tumour of chromaffin cell origin, is characterised by catecholamine excess. Clinical presentation ranges from asymptomatic disease to life-threatening multiorgan dysfunction. Catecholamine-induced cardiomyopathy is a dreaded complication with high lethality. While there is lack of evidence-based guidelines for use of veno-arterial extracorporeal membrane oxygenation (V-A ECMO) in the management of this condition, limited to case reports and small case series, V-A ECMO has been reported as ‘bridge to recovery’ therapy, providing circulatory support in the initial period of stabilisation prior to surgery. We report on two patients presenting with catecholamine-induced cardiomyopathy and circulatory collapse who were successfully treated with V-A ECMO for 5 and 6 days, respectively, providing initial haemodynamic support. After stabilisation and introduction of alpha-blockade, both cases had favourable outcomes, with successful laparoscopic adrenalectomies on days 62 and 83 of admission, respectively. Our case reports provide further support for the use of V-A ECMO in the treatment of such gravely ill patients. Learning points Phaeochromocytoma should be considered in the diagnosis of patients presenting with acute cardiomyopathy. Management of catecholamine-induced cardiomyopathy is complex and requires multidisciplinary specialist input. Pre-operative management of phaeochromocytoma involves alpha-blockade; however, haemodynamic instability in the setting of cardiogenic shock can preclude alpha-blockade use. Veno-arterial extracorporeal membrane oxygenation is a life-saving intervention which may be considered in cases of acute catecholamine-induced cardiomyopathy and cardiogenic shock in order to provide the required haemodynamic support in the initial phase of treatment, enabling the administration of traditional pharmacological agents, including alpha-blockade.
Background: APECED is a rare monogenic autosomal recessive syndrome caused by mutation in the AIRE gene located on chromosome 21q22.3. Phenotypic expression is variable but classically includes chronic mucocutaneous candidiasis, chronic hypoparathyroidism and hypoadrenalism. Studies demonstrate prevalence of autoimmune hepatitis (AIH) is 12%, and hyposplenism is approximately 20%. (Peterson P, Pitkänen J, Sillanpää N,2004; Capalbo D, De Martino L, Giardino G, 2012) Clinical Case: A 29 year old man was diagnosed with autoimmune hepatitis (AIH) at 6 years. He presented with symptomatic hypocalcaemia secondary to autoimmune hypoparathyroidism at 11 years. At 12 years he developed primary adrenal insufficiency. He also had nail dystrophy and mucocutaneous candidiasis. Genetic analysis revealed he was homozygous for for a 13bp deletion in Exon 8 of the AIRE gene consistent with diagnosis of APECED. His sister, who presented with hypoparathyroidism, was also AIRE gene positive. He transitioned from paediatric to adult care age at 16 years and subsequently defaulted from care. Following re-engagement with adult services at age 19 years, he was diagnosed with cirrhosis and portal hypertension and has had an oesophageal variceal bleed. Regular dental review has not revealed an oral mucocutaneous malignancy. At age 24, during an admission with decompensated liver disease, an elevated LDH 384 I.U. /l (120 - 220) and bilirubin (total) 121 μmol/l (5-24) were noted. A blood film demonstrated Howell Jolly bodies, target cells, polychromasia, target cells and anisopoikilocytosis consistent with hyposplenism. In 2011 a CT abdomen reported the spleen as severely shrunken. On review of previous abdominal ultrasounds reports in dating back to 2012 the spleen was undetectable. He was therefore diagnosed with functional hyposplenism. Hyposplenism was complicated by streptococcal sepsis. He was treated as per guidelines with pneumococcus, haemophilus, meningitis C, influenza vaccinations and penicillin prophylaxis. The pathogenesis underlying functional hyposplenism in APECED is unknown however it is hypothesized that it occurs secondary to local AIRE gene dysfunction in the spleen.(Starzyk J, Kumorowicz-Kopiec M, Kowalczyk M, 2001). Another possible contributory factor in this gentleman is his liver cirrhosis. Discussion: This case highlights the multifaceted nature of this rare disease as well as the associated morbidity and need for a multidisciplinary approach to a complex multi-organ condition. It is important to be cognisant of its many associations in order to avoid missing life threatening diagnoses. Patients with APECED should be evaluated for hyposplenia and appropriate vaccination schedule initiated.
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