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Antiphospholipid syndrome (APLS) is an autoimmune condition which commonly manifests as an arterial or venous thrombosis affecting medium to large vessels, with the presence of antiphospholipid antibodies. APLS can be a primary disease by itself, or secondary to other autoimmune diseases, such as Systemic Lupus Erythematosus (SLE). Catastrophic APLS is a rare but a fatal sequelae of APLS, affecting up to three or more organs, and progresses rapidly with a high mortality rate. We report a case of catastrophic APLS in a young woman with underlying SLE who presented to us with multiple cranial nerve palsies due to bilateral pontine infarct, and eventually developed deep vein thrombosis and pulmonary embolism during the course of the illness. She was treated with high dose corticosteroids and intravenous cyclophosphamide with biochemical improvement. In this case report, we would like to highlight the fact that our patient had bilateral pontine infarcts as the initial presentation, with no inciting events and  antiphospholipid antibodies were negative during the acute illness.

Introduction: The alteration of the gut microbiome in the gut-kidney axis has been associated with a pro-inflammatory state and chronic kidney disease (CKD). A small-scaled Italian study has shown an association between the gut microbiome and Immunoglobulin A Nephropathy (IgAN). However, there is no data on gut microbiota in IgAN in the Asian population. This study compares the gut microbial abundance and diversity between healthy volunteers and Malaysian IgAN cohort., Methods: A comparative cross-sectional study was conducted involving biopsy-proven IgAN patients in clinical remission with matched controls in a Malaysian tertiary centre. Demographic data, routine blood and urine results were recorded. Stool samples were collected and their DNA was extracted by 16S rRNA gene sequencing to profile their gut microbiota., Results: Thirty-six IgAN patients (13 male; 23 female) with the mean age of 45.5 ± 13.4 years and median estimated glomerular filtration rate (eGFR) of 79.0 (62.1-92.2) mls/min/1.73m 2 with median remission of 7 years were analysed and compared with 12 healthy controls (4 male; 8 female) with the mean age of 46.5 ± 13.5 years and eGFR of 86.5 (74.2-93.7) mls/min/1.73m 2 . Other demographic and laboratory parameters such as gender, ethnicity, body mass index (BMI), haemoglobin, serum urea and serum albumin were comparable between the two groups. There were no significant differences seen in the Operational Taxonomic Unit (OTU) and alpha diversity (Shannon index) between IgAN and healthy controls. Alpha diversity increased with increasing CKD stage (p = 0.025). Firmicutes/Bacteroidetes (F/B) ratio was low in both IgAN and healthy cohort. Fusobacteria phylum was significantly increased (p = 0.005) whereas Euryarchaoeota phylum was reduced (p = 0.016) in the IgAN group as compared to the control cohort., Conclusion: Although we found no differences in OTU and alpha diversity between IgAN in remission and control cohort, there were some differences between the two groups at phylum level.

Thrombotic thrombocytopenic purpura is a rare but life threatening medical condition. Early recognition and treatment of thrombotic thrombocytopenic purpura is important especially in patients who do not present with the classic pentad to reduce the high mortality. Herein, we describe a case of a patient who does not fulfil the classic pentad features thrombotic thrombocytopenic purpura that was induced by dengue fever. The patients' initial full blood picture did not have all the typical features of microangiopathic haemolytic anaemia but there were fragmented red blood cells. However, even a small number of fragmented red blood cells in the peripheral blood should alert physicians of the possible diagnosis of thrombotic thrombocytopenic purpura together with other symptoms. Furthermore, signs and symptoms of thrombotic thrombocytopenic purpura and dengue fever can overlap such as fever, thrombocytopenia, neurological deficit mimicking dengue encephalopathy and dengue induced acute kidney injury.

Background: Malignancies are among the leading causes of death in Systemic Lupus Erythematosus (SLE) patients with studies reporting a higher prevalence of malignancy in SLE patients compared to the general population. We wanted to determine the frequency of cancer in a cohort of SLE patients and identify its associated risk factors. Methods: Cross-sectional study involving SLE patients attending the nephrology outpatient clinic, Universiti Kebangsaan Malaysia Medical Centre between January and June 2014. Results: We recruited 228 patients (207 female, 21 male), aged 40.48 ± 12.86 years with mean SLE duration of 11.65 ± 6.46 years. Majority (87%) had lupus nephritis and were in remission with a median SLEDAI score 2 (0, 14). Majority (89%) were on corticosteroid with either a steroid sparing agent like mycophenolate mofetil (15.4%), azathioprine (36.8%) or ciclosporin (15.4%). One hundred and sixty (70.2%) patients were either receiving or had received intravenous cyclophosphamide with median dose of 5,173.6 ± 3,242.4 mg. Seven female patients were diagnosed with cancer during the course of their SLE with 56 (34-78) years being median age at malignancy and SLE duration of 4 (0-12) years. Majority (5/7) had lupus nephritis and all patients a median dose of prednisolone 10 (2.5, 10) mg with 10 (4-24) years of steroids. Two patients had a family history of cancer with majority developing cancer after the diagnosis of SLE. Two patients received intravenous cyclophosphamide prior to the development of cancer for their SLE compared to overall cohort of 160. Three patients had colorectal cancer, 2 had cervical cancer, 1 had breast cancer, and one patient had germ cell tumour and one thyroid cancer. All patients had their cancer successful treated with no signs of recurrence. Conclusion: We found a lower occurrence of cancer in our SLE patients as compared with the reported literature., (Creative Commons Attribution License)

A 54 year old lady with underlying chronic lung disease on long term oxygen therapy and end stage renal disease of unknown aetiology on regular haemodialysis for two years started developing progressive shortness of breath during her routine haemodialysis. She was unable to tolerate her haemodialysis sessions which had to be terminated prematurely in view of her symptoms despite adjustment of her dry weight and treatment of anaemia. She was not in chronic fluid overload and her symptoms always worsened after initiation of haemodialysis and improved after termination of haemodialysis. She was admitted to hospital for further investigations and initially treated for a lung infection but her symptoms did not improve. A computed tomography pulmonary angiography did not reveal any evidence of pulmonary embolism, and was consistent with chronic fibrotic changes. Her hypoxemia was concluded to be due to her underlying chronic lung disease, worsened by alveolar hypoventilation during haemodialysis. Her symptoms improved slightly with supplemental oxygen during her routine haemodialysis but we had to shorten her haemodialysis duration to 3 hours.

Managing primary or even secondary glomerulonephritis remains a challenge to many nephrologists. In primary focal segmental glomerulosclerosis (FSGS) with heavy proteinuria, renin aldosterone system blockade and high dose of oral prednisolone is the mainstay of treatment. Other immunosuppressive medications like Cyclophosphamide, Cyclosporine A and Mycophenolate Mofetil (MMF) are warranted if a complete remission is not achieved.  We illustrate a case of 21 year old gentleman with primary FSGS that was difficult to achieve remission despite on high dose steroid and oral Cyclophosphamide. He was also not responsive to a combination of MMF and Cyclosporine A (CSA) and even throughout the therapy he developed significant steroid and CSA toxicity. He presented to our center with severe nephrotic syndrome and acute kidney injury requiring acute haemodialysis. Despite re-challenged him again on high dose prednisolone, total of 2.4g of intravenous Cyclophosphamide, and MMF, he failed to achieve remission. He was subsequently given intravenous Rituximab 500mg/weekly for 4 doses and able to attained remission for 1 year. He relapsed again and a second course of Rituximab 500mg/weekly for 6 doses were given to attain remission. This case demonstrates the difficulty in managing refractory steroid dependent FSGS and we found that Rituximab is proven beneficial in this case to induce remission.

Acinetobacter baumannii is an aerobic Gram-negative coccobacillus that is associated with hospital acquired pneumonia. There is increased reporting of emergent cases of community acquired multidrug resistance (MDR) acinetobacter associated with a higher mortality due to antibiotic resistance. Community acquired MDR acinetobacter pneumonia has not been reported in Malaysia. Here we report a case of a 19-year-old army officer who presented with fever and respiratory symptoms for 5 days. He had no known medical illness before and no history of hospitalization. Upon arrival, he was in septicaemic shock, requiring invasive ventilator support and renal replacement therapy in intensive care unit. Chest radiograph showed bilateral lung consolidations and bronchoscopy revealed haemoserous and greenish bronchiole secretion. He was treated with broad spectrum antibiotics and oseltamivir. Unfortunately he died on day 3 of hospital admission. His bronchial lavage culture came back positive for MDR Acinetobacter baumannii . This case illustrates that clinicians need to be aware that MDR Acinetobacter baumannii can cause severe community acquired pneumonia. We may need to consider this diagnosis in patients who do not respond to standard therapy.