Idiopathic pulmonary haemosiderosis misdiagnosed as haemolytic anaemia: a case report.

Idiopathic pulmonary haemosiderosis is a rare disease primarily affecting children. The condition is characterized by widespread bleeding from alveolar capillaries, resulting in symptoms such as haemoptysis, shortness of breath and iron deficiency anaemia. However, it is not a specific disease and sometimes can manifest solely as anaemia, which may be easily overlooked and misdiagnosed. The purpose of this case report was to describe a 1-year-old boy who exhibited haemolytic anaemia as the only symptom of idiopathic pulmonary haemosiderosis, with the intention of offering clinical insights into the precise diagnosis and subsequent management of this rare and easily misdiagnosed disease. Clinicians should keep idiopathic pulmonary haemosiderosis in mind when evaluating children with haemolytic anaemia and promptly initiate testing and treatment to prevent misdiagnosis and improve outcomes.

Idiopathic Pulmonary Haemosiderosis: An Unusual Case Of Anaemia With Pulmonary Involvement.

Idiopathic pulmonary haemosiderosis is a rare disorder, with recurrent life-threatening alveolar haemorrhages and chronic lung parenchymal changes. It is associated with a triad of haemoptysis, iron deficiency anaemia, and diffuse pulmonary infiltrates. Although most cases are idiopathic, secondary haemosiderosis linked to known diseases has also been observed. Most of the cases remain undiagnosed because the disease is very low on the list of differentials. There is no specified age for the disease. The present study reports on an adolescent female patient who presented with microcytic anaemia and bilateral lung infiltrates to the National Institute of Child Health (NICH), Karachi, a tertiary care hospital. She was diagnosed with Idiopathic pulmonary haemosiderosis after ruling out other possibilities.

Diffuse alveolar haemorrhage in children hospitalised in a tertiary­level hospital: A retrospective descriptive study.

Background: Diffuse alveolar haemorrhage (DAH) is considered a rare condition in children. There is no consensus on the management of DAH syndromes in Africa or other low- and middle-income countries. In this brief report, the clinical characteristics, management and outcomes of children treated for DAH in the Chris Hani Baragwanath Academic Hospital paediatric pulmonology unit in Johannesburg, South Africa are described. Fifteen children were included in this case series, of whom 11 (73.3%) presented with severe microcytic anaemia. Of the 11 children who had bronchoalveolar lavage, 9 (81.8%; 60.0% of the total) had haemosiderin-laden macrophages on microscopy. Only 5 children had a lung biopsy, of whom 3 (60.0%) had capillaritis. All the children were started on oral prednisone at presentation, and 11 (73.3%) received additional complementary treatment. Nine children (60.0%) had normal haemoglobin levels 1 year after initiation of treatment. Our series supports previous reports that DAH is uncommon in children. A large proportion of our patients responded well to treatment despite some resource limitations. What the study adds: The study provides additional data on children presenting with diffuse alveolar haemorrhage in a South African tertiary hospital. What are the implications of the findings: There is a need for South African pulmonologists to come together and conduct a national audit of these patients in different hospitals to determine the incidence in our country, as well as to inform a management plan in the presence or absence of specialised tests.

Why SWI? The sensitivity of susceptibility weighted imaging in aneurysmal subarachnoid haemorrhage in the chronic phase.

Background: Incidentally detected unruptured intracranial aneurysms have a prevalence of 3% with some predisposed to rupture and others remaining static. Diagnostic knowledge of previous aneurysmal subarachnoid haemorrhage (ASAH) in the chronic phase could identify patients requiring treatment. Objectives: To assess the sensitivity of susceptibility weighted imaging (SWI) in the detection of ASAH at 3 months post ictus and determine any influencing effects. Method: A retrospective chart analysis of 46 patients with ASAH who underwent post-embolisation SWI imaging at 3 months. The SWI and available initial CT brain scans or CT reports were evaluated and correlated with patient demographics and clinical severity. Results: Susceptibility weighted imaging indicated a sensitivity of 95.7% in the detection of ASAH at 3 months. Increased number of haemosiderin zones on SWI correlated with older patient age (p = 0.0003). Clinical severity (World Federation Neurosurgical Societies Score) showed a tendency towards a statistically relevant relationship (p = 0.07). No statistically significant relationship was identified between the number of haemosiderin zones and initial CT modified Fisher score (p = 0.34) or the causative aneurysm location (p = 0.37). Conclusion: Susceptibility weighted imaging is sensitive in the detection of ASAH at 3 months, increasing in sensitivity with patient age and higher initial clinical severity. Contribution: In patients presenting in the subacute to chronic phase with a clinically suspicious history of previous aneurysm rupture but without convincing CT or spectrophotometry evidence, SWI can detect previous rupture. This can identify patients who could benefit from endovascular treatment and those who can safely undergo follow-up imaging.

Magnetic resonance imaging T2* of the pancreas value using an online software tool and correlate with T2* value of myocardium and liver among patients with transfusion-dependent thalassemia major.

Objective: Patients with thalassemia major do require lifetime blood transfusions that eventually result in iron accumulation in different organs. We described the usefulness of using magnetic resonance imaging (MRI) T2*imaging values for the evaluation of pancreatic iron load in these patients, and we correlated it with MRI T2* haemosiderosis of the myocardium and liver that has been recognized as a non-invasive assessment of iron overload among patients with thalassemia major. Materials and methods: We conducted a cross-sectional study on 39 patients with thalassemia major in one of the tertiary university hospitals for a 1-year period. Demographic data were collected from the patient's history. MRI T2* of the pancreas, liver, and heart were executed on all patients in the same setting. Objective values of iron overload in these organs were obtained using the MRI post-processing software from online software. Results: A total of 32 (82.1%) patients had pancreatic iron overload including 2 patients (5.1%) with severe iron overload and 15 patients (38.5%) with moderate and mild iron overload, respectively. Nine patients (23.1%) had myocardial iron overload, which included 3 patients (7.7%) who had severe cardiac haemosiderosis. Notably, 37 patients (94.9%) had liver iron overload, which included 15 patients (38.5%) who had severe liver haemosiderosis. There was a moderate positive correlation between the relaxation time of the pancreas and heart haemosiderosis (r = 0.504, P < 0.001). No significant correlation was found between the relaxation time of the pancreas with the liver and the heart with the liver. Conclusion: Pancreatic haemosiderosis precedes cardiac haemosiderosis, which establishes a basis for initiating earlier iron chelation therapy to patients with thalassemia major.

Pulmonary Haemosiderosis Secondary to Hereditary Haemochromatosis; a Case Report.

Introduction: Hereditary haemochromatosis (HH) is an autosomal recessive disease of increased intestinal absorption of iron, leading to accumulation in tissues which may progress to organ damage, most commonly in the liver. Iron deposition in the liver can lead to cirrhosis and hepatocellular carcinoma. Other common manifestations of haemochromatosis include diabetes, bronzing of the skin, arthropathy and cardiomyopathy. Here, we describe a case of pulmonary haemosiderosis secondary to HH. Case Description: A 49-year-old male with no medical history or family history of iron overload presented with fatigue, shortness of breath and chest pain after a recent finding of elevated ferritin. The patient was found to have biallelic C282Y mutations of the human homeostatic iron regulator protein (HFE) protein and after further workup with laboratory tests and imaging was diagnosed with HH with secondary pulmonary haemosiderosis. The patient is receiving twice weekly phlebotomies and has had an overall improvement in his symptoms. Practical Implications: The presentation of haemochromatosis can vary widely depending on the severity of iron overload and the presence of conditions that predispose organ dysfunction. Pulmonary haemosiderosis is a very rare manifestation of HH. This report illustrates the various manifestations of this disease and provides insight into this rare presentation to improve the diagnosis of this disease.

Diffuse alveolar haemorrhage associated with subsequent development of ANCA positivity and emphysema in three young adults.

BACKGROUND: Diffuse alveolar haemorrhage (DAH) is characterized by the diffuse accumulation of red blood cells within the alveoli, presence of ground glass opacities and/or consolidation on computed tomography (CT). Aside from identifiable non-immune causes, DAH is classically subdivided into idiopathic (idiopathic pulmonary haemosiderosis, IPH) and autoimmune DAH. Here we describe three cases presenting with recurrent pulmonary haemorrhage, initially classified as IPH, who, several years after first presentation, develop anti myeloperoxidase antibodies (MPO) positivity, emphysema on CT and, in one case, renal involvement. CASE PRESENTATION: Patient 1 was diagnosed with IPH aged 14. Her disease remained poorly controlled despite immunosuppression, although ANCA remained negative over the years. Nineteen years from initial presentation, she developed MPO-ANCA positive antibodies and mild renal impairment. She was treated with Rituximab with good response. From first presentation, the chest CT was consistently characterized by diffuse ground-glass opacities and interlobular septal thickening. Ten years later, cystic opacities consistent with emphysema, with a striking peribronchovascular distribution, developed. Patient 2 was diagnosed with IPH aged 32. He was treated with corticosteroids and methotrexate, with fluctuating response. At 11 years from initial presentation, MPO-ANCA positivity was identified, and emphysema with a peribronchovascular distribution was observed on CT, with subsequent significant increase in extent. Patient 3 was diagnosed with IPH at the age of seven, and had recurrent episodes of haemoptysis of varying degree of severity, treated with intermittent courses of corticosteroids until age 11, when he was intubated due to severe DAH. Eight years after the diagnosis emphysematous changes were noted on CT and MPO-ANCA positivity developed for the first time 11 years after initial diagnosis. CONCLUSIONS: We believe these three cases highlight: 1) the possibility of development of ANCA positivity several years down the line from first DAH presentation 2) the possibility that DAH may lead to cystic/emphysematous changes with peribronchovascular distribution on CT. Moreover, the need for ongoing immunosuppressive treatment and the development of emphysema, emphasize a possible role played by autoimmune phenomena, even when DAH is initially diagnosed as "idiopathic". Further studies are required to better understand the relationship between DAH, ANCA positivity and development of emphysema.

Risk of iron overload with chronic indiscriminate use of intravenous iron products in ESRD and IBD populations.

The routine use of recombinant erythropoiesis-stimulating agents (ESA) over the past three decades has enabled the partial correction of anaemia in most patients with end-stage renal disease (ESRD). Since ESA use frequently leads to iron deficiency, almost all ESA-treated haemodialysis patients worldwide receive intravenous iron (IV) to ensure sufficient available iron during ESA therapy. Patients with inflammatory bowel disease (IBD) are also often treated with IV iron preparations, as anaemia is common in IBD. Over the past few years, liver magnetic resonance imaging (MRI) has become the gold standard method for non-invasive diagnosis and follow-up of iron overload diseases. Studies using MRI to quantify liver iron concentration in ESRD have shown a link between high infused iron dose and risk of haemosiderosis in dialysis patients. In September 2017, the Pharmacovigilance Committee (PRAC) of the European Medicines Agency (EMA) considered convergent publications over the last few years on iatrogenic haemosiderosis in dialysis patients and requested that companies holding marketing authorization for iron products should investigate the risk of iron overload, particularly in patients with end-stage renal disease on dialysis and, by analogy, patients with IBD. We present a narrative review of data supporting the views and decision of the EMA, and then give our expert opinion on this controversial field of anaemia therapeutics.

Superficial siderosis due to filum terminale tumor: An uncommon cause of cranial nerve dysfunction.

If superficial siderosis is suspected based on clinical presentation, susceptibility weighted imaging should be undertaken in addition to standard MRI sequences as it is more sensitive than T2 weighted imaging. Once diagnosed, imaging of the entire brain and spine must be undertaken to assess for an underlying cause.

Dual-energy CT for liver iron quantification in patients with haematological disorders.

OBJECTIVES: To retrospectively quantify liver iron content in haematological patients suspected of transfusional haemosiderosis using dual-energy CT (DECT) and correlate with serum ferritin levels and estimated quantity of transfused iron. METHODS: One hundred forty-seven consecutive dual-source dual-energy non-contrast chest-CTs in 110 haematologic patients intended primarily for exclusion of pulmonary infection between September 2016 and June 2017 were retrospectively evaluated. Image data was post-processed with a software prototype. After material decomposition, an iron enhancement map was created and freehand ROIs were drawn including most of the partially examined liver. The virtual iron content (VIC) was calculated and expressed in milligram/millilitre. VIC was correlated with serum ferritin and estimated amount of transfused iron. Scans of patients who had not received blood products were considered controls. RESULTS: Forty-eight (32.7%) cases (controls) had not received any blood transfusions whereas 67.3% had received one transfusion or more. Median serum ferritin and VIC were 138.0 µg/dl (range, 6.0-2628.0 µg/dl) and 1.33 mg/ml (range, - 0.94-7.56 mg/ml) in the post-transfusional group and 27.0 µg/dl (range, 1.0-248.0 µg/dl) and 0.61 mg/ml (range, - 2.1-2.4) in the control group. Correlation between serum ferritin and VIC was strong (r = 0.623; p < 0.001) as well as that between serum ferritin and estimated quantity of transfused iron (r = 0.681; p < 0.001). CONCLUSIONS: Hepatic VIC obtained via dual-energy chest-CT examinational protocol strongly correlates with serum ferritin levels and estimated amount of transfused iron and could therefore be used in the routine diagnosis for complementary evaluation of transfusional haemosiderosis. KEY POINTS: • Virtual liver iron content was measured in routine chest-CTs of haematological patients suspected of having iron overload. Chest-CTs were primarily intended for exclusion of pulmonary infection. • Measurements correlate strongly with the most widely used blood marker of iron overload serum ferritin (after exclusion of infection) and the amount of transfused iron. • Liver VIC could be used for supplemental evaluation of transfusional haemosiderosis in haematological patients.

Diffuse pulmonary small nodular and patchy infiltrates on chest X-ray with hemoptysis: TB or not TB?-a call for scale up of respiratory medicine services in African TB high burden countries: a case of idiopathic pulmonary hemosiderosis.

Tuberculosis is still one of the most common respiratory diseases in Africa and worldwide and miliary tuberculosis is a regular manifestation of it. Idiopathic pulmonary hemosiderosis is a rare disease entity, presenting in children as well as adults. It is characterized by the triad of recurrent episodes of alveolar hemorrhage, presenting as hemoptysis, iron deficiency anemia and bilateral pulmonary infiltrates seen on chest X-ray. These symptoms and signs can easily be confused for other diseases i.e. miliary tuberculosis, delaying appropriate management. The etiology of idiopathic pulmonary hemosiderosis remains unclear. Diagnosis is established by lung biopsy, revealing hemosiderin laden macrophages in the alveoli. Treatment during an acute episode includes corticosteroids and/or immunosuppressive therapy, as well as supportive measures. Long-term follow-up is essential.

Early diagnosis of idiopathic pulmonary haemosiderosis: increased haemosiderin-laden macrophages in repeat bronchoscopy.

Idiopathic pulmonary haemosiderosis (IPH) is a diagnosis of exclusion, which is characterized by persistent or recurrent episodes of alveolar haemorrhage. Early diagnosis of IPH, especially in the case of first-time manifestation, is challenging because previous episodes of alveolar haemorrhage are often difficult to prove. Repeated episodes of alveolar haemorrhage can result in chronic iron-deficient anaemia and irreversible interstitial fibrosis; thus, early recognition and intervention are desirable in terms of clinical outcome. We report a case of IPH that was diagnosed early by confirming the presence of an increased number of haemosiderin-laden macrophages with alveolar haemorrhage in repeat bronchoscopy. We wanted to highlight that decreased but sustained attenuation of ground-glass opacities on high-resolution computed tomography does not always correlate with successful remission in patients with IPH. Repeat bronchoscopy can be useful in the early recognition of IPH, especially in the case of sustained opacities a few months after alveolar haemorrhage.

Non-invasive measurement of liver iron concentration using 3-Tesla magnetic resonance imaging: validation against biopsy.

OBJECTIVES: To evaluate the performance and limitations of the R2* and signal intensity ratio (SIR) methods for quantifying liver iron concentration (LIC) at 3 T. METHODS: A total of 105 patients who underwent a liver biopsy with biochemical LIC (LICb) were included prospectively. All patients underwent a 3-T MRI scan with a breath-hold multiple-echo gradient-echo sequence (mGRE). LIC calculated by 3-T SIR algorithm (LICSIR) and by R2* (LICR2*) were correlated with LICb. Sensitivity and specificity were calculated. The comparison of methods was analysed for successive classes. RESULTS: LICb was strongly correlated with R2* (r = 0.95, p < 0.001) and LICSIR (r = 0.92, p < 0.001). In comparison to LICb, LICR2* and LICSIR detect liver iron overload with a sensitivity/specificity of 0.96/0.93 and 0.92/0.95, respectively, and a bias ± SD of 7.6 ± 73.4 and 14.8 ± 37.6 µmol/g, respectively. LICR2* presented the lowest differences for patients with LICb values under 130 µmol/g. Above this value, LICSIR has the lowest differences. CONCLUSIONS: At 3 T, R2* provides precise LIC quantification for lower overload but the SIR method is recommended to overcome R2* limitations in higher overload. Our software, available at www.mrquantif.org , uses both methods jointly and selects the best one. KEY POINTS: • Liver iron can be accurately quantified by MRI at 3 T • At 3 T, R2* provides precise quantification of slight liver iron overload • At 3 T, SIR method is recommended in case of high iron overload • Slight liver iron overload present in metabolic syndrome can be depicted • Treatment can be monitored with great confidence.

Spontaneous convexity subarachnoid haemorrhage: Clinical series of 3 patients with associated cerebral amyloid angiopathy. / Hemorragia subaracnoidea espontánea de la convexidad cerebral: una serie clínica de 3 pacientes asociada con angiopatía amiloide cerebral.

INTRODUCTION: Convexity subarachnoid haemorrhage (cSAH) is a rare type of spontaneous, non-traumatic, and nonaneurysmal SAH characterised by blood collections in one or more cortical sulci in the convexity of the brain; the aetiology varies. We report a clinical case series of 3 patients with cSAH associated with probable cerebral amyloid angiopathy (CAA) who presented with focal sensory seizures and responded well to corticosteroid treatment. PATIENTS: Case 1 was a 67-year-old man reporting right-sided paroxysmal sensory episodes with Jacksonian progression, cheiro-oral symptoms, and motor dysphasia. Case 2 was a 79-year-old man reporting left-sided paroxysmal episodes with cheiro-oral signs and dysarthria. Case 3 was a 71-year-old woman also reporting recurrent left cheiro-oral signs and dysarthria. None of the patients had headache or clinical dementia. Aneurysms were ruled out using MR angiography. RESULTS: Brain CT scan detected an isolated hyperintensity in a sulcus of the frontal convexity; brain gradient echo T2-weighted MRI sequences showed meningeal haemosiderosis and microbleeds. However, no atrophy was identified in medial temporal lobes including the hippocampal formation. All patients had low levels of beta-amyloid in CSF, low values on the Hulstaert index and high levels of phosphorylated tau protein. Patients were initially treated with prednisone and levetiracetam, but symptoms recurred in 2 patients after prednisone was discontinued. CONCLUSIONS: We present a series of 3 patients with cSAH associated with CAA, characterised by a stereotypical syndrome responding well to corticoid treatment; there were no cases of headache or clinical dementia.

Idiopathic pulmonary haemosiderosis in paediatric patients: how to make an early diagnosis.

BACKGROUND: Idiopathic pulmonary haemosiderosis (IPH) is a rare but potentially lethal condition in paediatric patients. This condition is considered an immune-mediated disorder, but its pathogenesis is still unknown. Idiopathic pulmonary haemosiderosis is characterized by the classical triad of haemoptysis, iron-deficiency anaemia, and diffuse parenchymal consolidation on chest radiology. Unfortunately, this triad of signs is not frequent in children at the onset of this disease, resulting in a delay in diagnosis and a negative outcome. CASE PRESENTATION: This case report describes a 4-year-old girl who was admitted for an acute episode of lower respiratory tract infection associated with severe dyspnoea, polypnoea, and severe anaemia (haemoglobin levels, 5.9 g/dL). She had a history of previous similar episodes, with anaemia treated unsuccessfully with iron supplementation and managed through repeated blood transfusions in the acute phase. She did not experience haemoptysis. A computed tomography (CT) scan of the thorax showed ground-glass opacity suggestive of pulmonary haemorrhage. After other causes of intra-alveolar haemorrhage were excluded, IPH was confirmed by the presence of siderophages in bronchoalveolar lavage. Immunosuppressive corticosteroid treatment was immediately started with a good clinical response. CONCLUSION: This case highlights the fact that IPH should be suspected in children with recurrent lower respiratory tract infections who have a history of iron-deficiency anaemia who shows no signs of improvement with iron supplementation and may require repeated blood transfusions. The absence of haemoptysis does not exclude the diagnosis of IPH in children. An early and prompt diagnosis is recommended in order to start adequate immunosuppressive treatment.

Long-term clinical course of idiopathic pulmonary haemosiderosis with rheumatoid arthritis.

Idiopathic pulmonary haemosiderosis (IPH) is a rare cause of diffuse alveolar haemorrhage during childhood, and its precise pathophysiology and long-term clinical course remain unclear. A 31-year-old man was diagnosed with IPH at four years of age and had recurrent episodes of haemoptysis. The patient's symptoms responded well to steroids. However, pulmonary fibrosis and the cystic region in the lung progressively worsened. At age 27, the patient developed polyarthritis with positive anti-cyclic citrullinated peptide antibodies. The patient also developed hand synovitis, which was diagnosed with ultrasonography. These results indicate complications from rheumatoid arthritis. The patient's dyspnoea gradually worsened, and at the age of 31, he developed pneumothorax and an acute exacerbation of IPH. The clinical course from ages 4 to 31 included progressive chronic respiratory failure because of pulmonary fibrosis, acute exacerbations, complications with rheumatoid arthritis, and deliberation regarding lung transplantation. The development of rheumatoid arthritis after the onset of IPH supports the theory of an autoimmune mechanism of IPH.

Use of Corticosteroids in the management of Idiopathic Pulmonary Haemosiderosis: Do we have enough evidence.

Idiopathic Pulmonary Haemosiderosis (IPH) is a rare disease commonly affecting the paediatric population with approximately 500 globally reported cases in the literature. The disease usually presentswith a symptom triad consisting of ferropenic anaemia, cough with haemoptysis and diffuse bilateral alveolar infiltrates. Therapeutic options for this disorder are not only limited but also not fully effective. Moreover, corticosteroids remain the mainstay of IPH treatment. This communication reviews the available evidence in support of corticosteriod usage in the treatment of IPH. We conclude that the use of corticosteroid in IPH treatment is unfathomed and demands further investigation.

Iron overload secondary to cirrhosis: a mimic of hereditary haemochromatosis?

AIMS: Hepatic iron deposition unrelated to hereditary haemochromatosis is common in cirrhosis. The aim of this study was to determine whether hepatic haemosiderosis secondary to cirrhosis is associated with iron deposition in extrahepatic organs. METHODS AND RESULTS: Records of consecutive adult patients with cirrhosis who underwent autopsy were reviewed. Storage iron was assessed by histochemical staining of sections of liver, heart, pancreas and spleen. HFE genotyping was performed on subjects with significant liver, cardiac and/or pancreatic iron. The 104 individuals were predominantly male (63%), with a mean age of 55 years. About half (46%) had stainable hepatocyte iron, 2+ or less in most cases. In six subjects, there was heavy iron deposition (4+) in hepatocytes and biliary epithelium. All six of these cases had pancreatic iron and five also had cardiac iron. None of these subjects had an explanatory HFE genotype. CONCLUSIONS: In this series, heavy hepatocyte iron deposition secondary to cirrhosis was commonly associated with pancreatic and cardiac iron. Although this phenomenon appears to be relatively uncommon, the resulting pattern of iron deposition is similar to haemochromatosis. Patients with marked hepatic haemosiderosis secondary to cirrhosis may be at risk of developing extrahepatic complications of iron overload.

Multiparametric magnetic resonance for the non-invasive diagnosis of liver disease.

BACKGROUND & AIMS: With the increasing prevalence of liver disease worldwide, there is an urgent clinical need for reliable methods to diagnose and stage liver pathology. Liver biopsy, the current gold standard, is invasive and limited by sampling and observer dependent variability. In this study, we aimed to assess the diagnostic accuracy of a novel magnetic resonance protocol for liver tissue characterisation. METHODS: We conducted a prospective study comparing our magnetic resonance technique against liver biopsy. The individual components of the scanning protocol were T1 mapping, proton spectroscopy and T2* mapping, which quantified liver fibrosis, steatosis and haemosiderosis, respectively. Unselected adult patients referred for liver biopsy as part of their routine care were recruited. Scans performed prior to liver biopsy were analysed by physicians blinded to the histology results. The associations between magnetic resonance and histology variables were assessed. Receiver-operating characteristic analyses were also carried out. RESULTS: Paired magnetic resonance and biopsy data were obtained in 79 patients. Magnetic resonance measures correlated strongly with histology (r(s)=0.68 p<0.0001 for fibrosis; r(s)=0.89 p<0.001 for steatosis; r(s)=-0.69 p<0.0001 for haemosiderosis). The area under the receiver operating characteristic curve was 0.94, 0.93, and 0.94 for the diagnosis of any degree of fibrosis, steatosis and haemosiderosis respectively. CONCLUSION: The novel scanning method described here provides high diagnostic accuracy for the assessment of liver fibrosis, steatosis and haemosiderosis and could potentially replace liver biopsy for many indications. This is the first demonstration of a non-invasive test to differentiate early stages of fibrosis from normal liver.

Severe heart failure, dilated cardiomyopathy and pulmonary haemosiderosis in coeliac disease: report of two cases.

Coeliac disease (CD) is a chronic inflammatory, multi-system disorder with protean manifestations which has been linked to various auto-immune-mediated disorders. Dilated cardiomyopathy (DCM) is a rare extra-intestinal manifestation that is being recognised increasingly in patients with CD. Two cases of CD are described, an 18-year-old boy and a 13-year-old girl, both of whom presented with rapid onset of congestive heart failure and severe left ventricular systolic dysfunction. Upper limb venous thrombosis and recurrent haemoptysis secondary to pulmonary haemosiderosis in the second case were the other unusual features. The importance of CD screening of patients with DCM and pulmonary haemosiderosis is emphasised.