Chronic intestinal pseudo-obstruction (CIPO) is a rare clinical syndrome of ineffective intestinal motility characterised by clinical and radiological evidence of intestinal obstruction with no identifiable mechanical lesion. CIPO can either be idiopathic or secondary to a systemic disease, like systemic lupus erythematosus (SLE). Fewer than 30 cases of CIPO secondary to SLE have been reported so far. Here we describe a case of SLE with the initial presentation of CIPO. In SLE-related CIPO, treatment includes a combination of high-dose intravenous corticosteroids, immunosuppressants and supportive care. With awareness of this condition, unnecessary surgical intervention and repeated invasive procedures could be avoided. Early initiation of treatment would avoid complications and bring about resolution of symptoms.

ObjectiveWe aimed to adapt, translate and validate the Chronic Liver Disease Questionnaire (CLDQ) in Malaysian patients with chronic liver diseases of various aetiologies.SettingTertiary level teaching institution in Malaysia.ParticipantsThe validation process involved 211 adult patients (English language n=101, Malay language n=110) with chronic liver disease. Characteristics of the study subjects were as follows: mean (SD) age was 56 (12.8) years, 58.3% were male and 41.7% female. The inclusion criteria were patients 18 years or older with chronic hepatitis and/or liver cirrhosis of any aetiology. The exclusion criteria were as follows: presence of hepatic encephalopathy, ongoing treatment with interferon and presence of other chronic conditions that have an impact on health-related quality of life (HRQOL).MethodsA cross-sectional study was conducted. Cultural adaptation of the English version of the CLDQ was performed, and a Malay version was developed following standard forward–backward translation by independent native speakers. Psychometric properties of both versions were determined by assessing their internal consistency, test–retest reliability and discriminant and convergent validity.ResultsCronbach’s alpha for internal consistency across the various domains of the CLDQ was 0.95 for the English version and 0.92 for the Malay version. Test–retest analysis showed excellent reliability with an intraclass correlation coefficient of 0.89 for the English version and 0.93 for the Malay version. The average scores of both the English and Malay versions of the CLDQ demonstrated adequate discriminant validity by differentiating between non-cirrhosis (English 6.3, Malay 6.1), compensated cirrhosis (English 5.6, Malay 6.0) and decompensated cirrhosis (English 5.1, Malay 4.9) (p<0.001). Convergent validity showed that correlation was fair between the English (ρ=0.59) and Malay (p=0.47) CLDQ versions with the EQ-5D, a generic HRQOL instrument.ConclusionThe English and Malay versions of the CLDQ are reliable and valid disease-specific instruments for assessing HRQOL in Malaysian patients with chronic liver disease.

Background: β-thalassemia is a genetic disease that causes abnormal production of red blood cells (ineffective erythropoiesis, IE). IE is a condition known to change bone marrow composition. Purpose: To evaluate the effect of IE on the marrow fat content and fat unsaturation levels in the proximal femur using 1 H-MRS. Study Type: Prospective. Subjects: Twenty-three subjects were included in this study, seven control and 16 β-thalassemia subjects. Field Strength/Sequence: 3.0T; stimulated echo acquisition Mode (STEAM); magnetic resonance spectroscopy (MRS) sequence. Assessment: Multiecho MRS scans were performed in four regions of the proximal left femur of each subject, that is, diaphysis, femoral neck, femoral head, and greater trochanter. The examined regions were grouped into red (diaphysis and femoral neck) and yellow marrow regions (femoral head and greater trochanter). Statistical Tests: The Jonckheere-Terpstra test was used to evaluate the impact of increasing disease severity on bone marrow fat fraction (BMFF), marrow conversion index, and fat unsaturation index (UI). Pairwise comparison analysis was performed when a significant trend (P < 0.05) was found. K-means clustering analysis was used to examine the clusters observed when BMFF in the red and yellow regions were studied (diaphysis against greater trochanter). Results: BMFF showed a significant decreasing trend with increasing disease severity in both red (T JT = 109.00, z =-4.414, P < 0.05) and yellow marrow regions (T JT = 108.00, z =-4.438, P < 0.05). The opposite trend was observed in UI in both bone marrow regions (red marrow: T JT = 180.5, z = 3.515, P < 0.05; yellow marrow: T JT = 155.0, z = 2.282, P = 0.05). Three distinct forms of marrow adipogenesis were found when plotting BMFF diaphysis against BMFF greater trochanter: 1) normal (centroid: 80.4%, 66.6%), 2) partial disruption (centroid: 51.1%, 16.6%), and 3) total disruption (centroid: 2.6%, 1.6%). Data Conclusion: β-thalassemia is associated with decreased marrow fat, and increased marrow fat unsaturation level. Level of Evidence: 2 Technical Efficacy Stage: 3

In this report, we focus on two types of severe cutaneous adverse drug reactions, i.e Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN).

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Multiple myeloma is the third most common hematologic malignancy in Malaysia. The introduction of novel agents over the past decades has improved patient outcome and survival substantially. The usage of novel agents can be financially taxing, and hence resources limit its use. This study aims to report on the real-world treatment outcome when resources are limited. This is a retrospective study on newly diagnosed multiple myeloma (NDMM) patients diagnosed between 1 January 2008 and 31 December 2018 in a single academic center. Patients demographic and type of treatment were included for analysis of progression free survival and overall survival. Ninety-eight NDMM patients with a median age of 63.5 (ranged from 38 to 87 years old) were included. Half of the total patients received bortezomib-containing regimens while 40.8% received thalidomide-containing regimens, and remaining 9.2% had other agents as induction. Forty-seven patients (48.0%) achieved very good partial response (VGPR) or complete remission (CR), while remaining 51 patients (52.0%) have achieved partial response (PR) at best during induction therapy. Bortezomib use was associated with significantly deeper (p=0.001) and more rapid response (p=0.005) compared to other agents. Five-year OS and PFS were 45.3% and 18.4%, respectively. Triplet regimen, best initial response and upfront ASCT were significantly associated with better PFS. In conclusion, deep response significantly affects PFS and OS in NDMM patients. Thus, one of the goals of treatment is to ensure earlier and deeper response by including bortezomib as part of triplet combination in upfront therapy, followed by ASCT for those who are fit. This is feasible in a resource limited country such as Malaysia, especially there is a cheaper generic formulation.