Triple trouble--macrophage activation syndrome in a case of severe leptospirosis and scrub typhus co-infection.

Macrophage activation syndrome is a potentially life threatening phenomenon characterised by aggressive proliferation of macrophages and T lymphocytes leading to haemophagocytosis of other blood cells and multi organ failure. Here we present a very unusual combination of leptospirosis and scrub typhus infection leading to macrophage activation syndrome. Scrub typhus associated with macrophage activation syndrome has rarely been reported in India. A 40 year old female presented with high grade fever, seizures, bodyache, arthralgia and severe breathlessness. Investigations revealed persistent thrombocytopenia, impaired liver function tests, renal dysfunction, leptospiral IgM ELISA positive and a positive Weil Felix test. There was evidence of haemophagocytosis in bone marrow. Macrophage activation syndrome if left untreated has been associated with rapidly fatal outcome and early treatment can help us save that one precious thing..called life..!

Association of plasma proteins at multiple stages of glycation and antioxidant status with erythrocyte oxidative stress in patients with type 2 diabetes.

This study examines the individual stages of plasma protein glycation, antioxidant status and their association with erythrocyte oxidative stress in patients with type 2 diabetes mellitus (T2DM). Study was carried out on blood from 70 patients with T2DM and 40 healthy age- and gender-matched volunteers. Biomarkers of plasma protein glycation (fructosamine, protein carbonyls, advanced glycation end products [AGEs], amyloid), antioxidant status (thiols, total antioxidant capacity and erythrocyte oxidative parameters), osmotic fragility, lipid peroxidation (LPO), reduced glutathione (GSH) and catalase were determined. Plasma glycation markers were higher in T2DM patients than in healthy volunteers: fructosamine 578 vs. 525 micromol/mL; carbonyl 21.23 vs. 18.84 nmol/mg protein (P < or = 0.01); AGEs 213.94 vs. 178.27 AU/mg protein (P < or = 0. 05); and amyloid 0.53 vs. 0.40 A530 nm (P < 0.01). Plasma antioxidant status was significantly reduced in patients with diabetes compared to the healthy volunteers, with lower plasma protein thiols (1.16 vs. 1.36 nmol/mg protein; P < 0.01) and total antioxidant capacity (26 vs. 34 micromol; P < 0.01). Erythrocytes from the patient group were found to show greater oxidative damage, with elevated numbers of fragile cells and increased LPO, and reduced GSH level. Among the glycation markers, positive correlations were evident between fructosamine and amyloid (r = 0.350, P < 0.001) and AGEs and amyloid (r = 0.070). Plasma glycation markers showed negative correlation with plasma antioxidant status while positive correlation was demonstrated between erythrocytes fragility and AGEs and amyloid. Erythrocyte LPO levels correlated positively with amyloid. These data suggest that increased levels of multiple plasma protein glycation products in T2DM patients play a key role in reduced plasma antioxidant status and amplified erythrocyte oxidative damage.

Hypermobility syndrome.

Three cases of Hypermobility Syndrome (HS) are discussed. The first case was a young female, aged 24, with musculoskeletal symptoms since 5 years. Second was an 18 year old male with similar symptoms since 3 years. The third was an elderly female who presented with knee joint osteoarthritis (OA) and apparent but reducible deformities of hands mimicking rheumatoid arthritis (RA). All three patients had hypermobility of joints in absence of demonstrable systemic rheumatic disease. The prevalence, clinical features and management of the entity is discussed. An increased awareness of this condition among physicians is warranted as some patients may be erroneously diagnosed as RA/SLE and may be put on DMARDs and steroids.

CARASIL.

We herewith report a case of 46 year old male with clinical features suggestive of CARASIL (Cerebral Autosomal Recessive Arteriopathy with Sub cortical Infarcts and Leucoencephalopathy). He had recurrent strokes in early age before reaching 35 years. He was normotensive and had severe degenerative changes in lumbar spine and knee joint on radiographs and had diffuse alopecia. Neuroimaging revealed diffuse hyperintense lesions in cerebral white matter and basal ganglia on MR images.

Evan's syndrome revisited.

A female aged 43 years presented with acute per vaginal bleeding since six days, severe thrombocytopenia and anaemia, she responded partially to platelets and blood transfusion initially. Four days later she started bleeding from nose, intravenous access sites, developed right sided hemiparesis and subsequently died. Her investigations were suggestive of Idiopathic Thrombocytopenia Purpura (ITP) and Autoimmune Haemolytic Anaemia (AIHA). So a diagnosis of Evan's syndrome was made.

Lemierre's syndrome--the syndrome quite forgotten.

A 17 year old male presented with seizures, headache, and fever and left chronic suppurative otitis media. A 35 year old male presented with headache, giddiness, vomiting, pain in eyes, diplopia and right chronic suppurative otitis media. Brain imaging in both revealed thrombosis of lateral and sigmoid sinus and also of internal jugular vein on the left and right side respectively. A diagnosis of Lemierre's syndrome was made in both. They were treated with antibiotics and anticoagulants, and they responded to treatment. We want to report this case as we feel, that with the advent of the antibiotic era, this syndrome has become rare; and so "quite forgotten" or overlooked, by many physicians.

Barrett's metaplasia in Indian obese male patients with gastro-oesophageal reflux disease.

Although 10% to 15% of gastro-oesophageal reflex disease (GERD) patients have Barrett's oesophagus, neither the frequency nor the severity of symptoms correlates with the presence of Barrett's epithelium. Age, male sex, white race, and obesity have been implicated in some studies. The studies in Indian population reflect low prevalence rates; however, there is no study of obesity as independent risk factor for Barrett's disease in Indian population. The present study showed Barrett's oesophagus in 9.5% patients who were obese as compared to non-obese patients having Barrett's 6.7% suggesting obesity might not predispose to Barrett's oesophagus, in patients of GERD taken criterion for obesity as BMI more than 30.

Gastric hemangioma: an unusual cause of upper gastrointestinal bleed.

We report a 36-year-old lady who presented with hematemesis. Emergency endoscopy showed a polypoidal lesion in the gastric fundus that appeared like a varix. Celiac angiogram confirmed this to be a hemangioma located in the fundus. This was managed by arterial embolization; the patient is symptom-free 6 months later.

Intra-arterial thrombolysis in acute brainstem infarct.

A young male presented with vertebrobasilar ischaemic stroke. He was given intra-arterial thrombolysis, following which he showed gradual partial recovery.

MR venography in cerebral venous sinus thrombosis.

A case of left transverse and sigmoid sinus thrombosis in a 60 years female is reported. The diagnosis was confirmed on MRI with MR venography.

Cholelithiasis in thalassaemia major.

The incidence of gall stones in thalassaemia is less than that in sickle cell anaemia or hereditary spherocytosis. With adequate blood transfusions, the incidence is as low as 2%. There are not many reports on cholelithiasis in thalassaemia. A case of 24-year-old female with thalassaemia major and gall stone is reported here.

Ossification of posterior longitudinal ligament causing cervical cord compression.

Ossification of the posterior longitudinal ligament (OPLL) is an uncommon cause of compressive myelopathy outside Japan. A case of cervical cord compression in a female whose MRI showed OPLL is reported. T2 weighted MRI images are the most effective to evaluate both spinal cord compression due to ossification and abnormal signal intensity of the cord. OPLL should be included in the differential diagnosis of cervical radiculomyelopathy.