Acta Medica Indonesiana – The Indonesian Journal of Internal Medicine
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Current Updates on Diagnosis and Management of Cholangiocarcinoma: from Surgery to Targeted Therapy
Cholangiocarcinoma is commonly described as any malignancy arising from the lining of the bile duct and is recognized as one of the most common biliary malignancies. We conducted a literature review of current available evidences and guidelines.Based on the anatomical location of the origin of the mass, cholangiocarcinoma can be divided into intrahepatic, perihilar, and distal cholangiocarcinoma. Each of these subtypes has their own risk factors, best treatment options, and prognosis. The most common risk factors for cholangiocarcinoma also differs based on geography and population backgrounds. Histopathological biopsy remained the gold standard for cholangiocarcinoma diagnosis, however various advances has been made in diagnostic procedure, including MRCP, EUS, ERCP, EBUS, and cholangioscopy. Surgical resection is still the best treatment modality for cholangiocarcinoma, but it can only be done in few patients considering most patients were diagnosed in the unresectable state. Other treatment options includes conventional chemotherapy, locoregional therapy, systemic targeted therapy, and palliative best supportive care. Cholangiocarcinoma has an abundance of molecular targets and advances in biomolecular technologies bring further hope for future curative treatment options. Treatment options should be chosen individually based on each patient’s condition and setting. Cholangiocarcinoma is still a major health problem in hepatobiliary malignancies. Multiple options are available for cholangiocarcinoma treatments
Gender Differences of Gly972Arg Polymorphism of the IRS-1 Gene Related to Cardiovascular Disease Risk Factors Among Indonesians
Background: Cardiovascular disease is driven by traditional risk factors, sex, and genetic differences. The Asian population, specifically Indonesians, has been known at high risk of insulin resistance and endothelial dysfunction. A possible genetic risk factor related to cardiovascular diseases is Gly972Arg polymorphism of insulin receptor substrate 1 (IRS-1) gene, as this impairs endothelial function. To date, whether there is a gender difference in Gly972Arg polymorphism of the IRS-1 gene in Indonesians is unknown. This study aimed to to define whether there is a gender difference in Gly972Arg polymorphism of the IRS-1 gene in Indonesians. Methods: We studied adults living in two areas (rural and urban) in Indonesia. We collected demographic and clinical data from the study subjects. Gly972Arg polymorphism of the IRS-1 gene (rs1801278) was detected using TaqMan real-time polymerase chain reaction. Results: A total of 378 subjects were recruited. The wild-type allele (CC) was found in 86 (22.8%) subjects, heterozygous mutant allele (CT) in 245 (64.8%), and homozygous mutant allele in 47 (12.4%). The proportion of subjects with T alleles was significantly higher among women than men (54.6% vs. 45.4%, odds ratio: 1.89; p = 0.01). Subjects with T allele more often have hypertension (odds ratio: 1.69, p = 0.058). Conclusion: There were a higher proportion of women than men carrying the T allele of Gly972Arg polymorphism among Indonesians. Individuals with the T allele appeared to show a greater prevalence of hypertension. These results may explain a possible mechanism of the high prevalence of metabolic syndrome in Indonesia, especially in women
Factors Affecting the Quality of Life of Patients After Kidney Transplantation: A Cross-Sectional Study
Background: Kidney transplantation is currently the best choice for renal replacement therapy, due to its effect in reducing mortality and improving the quality of life (QoL) of patients with end-stage renal disease. This study aimed to identify factors affecting QoL after kidney transplantation. Methods: We conducted a cross sectional study by recruiting patients who had kidney transplantation at Cipto Mangunkusumo General Hospital, Jakarta, Indonesia, from 2018 – 2020. QoL was assessed using the 36-item Short Form Health Survey (SF-36) questionnaire. We evaluated age, sex, body mass index, hemoglobin level, estimated glomerular filtration rate, duration of dialysis before transplantation, history of diabetes, depression, and performance status as factors associated with QoL score. Results: We involved 107 subjects in our study. Depression, hemoglobin level, ECOG performance status, and duration of dialysis were factors affecting the physical component score (R2=0.21). Depression and hemoglobin level were factors affecting the mental component score (R2=0.34 ). Depression, hemoglobin level, and ECOG performance status were factors contributed to the total QoL score (R2=0.41). Conclusion: Factors that contributed to QoL status were depression, ECOG performance status, and hemoglobin levels. This study supported the need for assessment of QoL on regular basis, psychological aspects including depression, as well as other factors that can affect QoL such as performance status and hemoglobin level in patients before and after kidney transplantations
Factors Associated with Sarcopenia in Maintenance Hemodialysis Patients: A Cross-Sectional Study
Background: Sarcopenia is associated with worse outcomes in maintenance hemodialysis (MHD) patients. Differences in criteria and methods used to diagnose sarcopenia, results in a wide range of prevalence. Factors associated with sarcopenia in MHD have not been well-studied. This study aimed to investigate the prevalence and factors associated with sarcopenia in the MHD population. Methods: Observational cross-sectional study was done with 96 MHD patients aged ≥18 years old, with dialysis vintage ≥120 days at Cipto Mangunkusumo Hospital March-May 2022. Descriptive, bivariate, and logistic regression analysis were done to find sarcopenia’s prevalence and association with Simplify Creatinine Index (SCI), type 2 diabetes (DM), Interleukin-6 (IL-6), nutritional status, physical activity, and phosphate serum level. Asian Working Group for Sarcopenia (AWGS) 2019 criteria used to diagnose sarcopenia, Hand Grip Strength (HGS) to identify muscle strength, Bioimpedance Spectroscopy (BIS) to calculate muscle mass, and 6-meter walk test to evaluate physical performance. Results: The prevalence of sarcopenia was 54.2%. Factors with a significant association in bivariate analysis were phosphate serum level (p=0.008), SCI (p=0.005) and low physical activity (International Physical Activity Questionnaire) (p-0.006). Logistic regression analysis found higher phosphate serum level and high physical activity protective of sarcopenia (OR 0.677;CI95% 0.493-0.93 and OR 0.313;CI95% 0.130-0.755 respectively). Conclusion: The prevalence of sarcopenia in the MHD population was 54.2%. Phosphate serum level, SCI, and physical activity were significantly correlated with sarcopenia. Both high phosphate level and high physical activity were protective against sarcopenia
The Comparison of Monocyte Human Leukocyte Antigen-D-Related (mHLA-DR) Expression Levels Between Corona Virus Disease 2019 (COVID-19) Patients and Healthy Subjects
Background: SARS-CoV-2 can trigger a dysfunctional immune response in COVID-19 patients and lead to immunosuppression. HLA-DR molecule expressed on the surface of monocytes, known as mHLA-DR, has been widely used as a reliable marker of immunosuppression. Downregulation of mHLA-DR reflects an immunosuppressed state. This study aimed to compare the expression level of mHLA-DR between COVID-19 patients and healthy subjects concerning immune system dysregulation that can be triggered by SARS-CoV-2 and lead to immunosuppression. Methods: This was an analytic observational study with a cross-sectional design that measured the mHLA-DR expression in EDTA blood samples from 34 COVID-19 patients and 15 healthy subjects using the BD FACSLyricTM Flow Cytometry System. The mHLA-DR examination results were expressed in AB/C (antibodies bound per cell) that were quantified using a standard curve constructed with Quantibrite phycoerythrin beads (BD Biosciences). Results: Expression of mHLA-DR in COVID-19 patients (n = 34) were 21,201 [2,646-92,384] AB/C, with 40,543.5 [9,797-92,384] AB/C mild cases (n = 22), 21,201 [9,831-31,930] AB/C moderate cases (n = 6), and 7,496 [2,646-13,674] AB/C severe to critical cases (n= 6). Expression of mHLA-DR in healthy subjects (n = 15) was 43,161 [25,147-89,846] AB/C. Based on the Mann-Whitney U test, the mHLA-DR expression in COVID-19 patients significantly differed from the mHLA-DR expression in healthy subjects (p = 0.010). Conclusion: The level of mHLA-DR expression in COVID-19 patients was lower and significantly different from healthy subjects. Moreover, immunosuppression could be indicated by the decrease of mHLA-DR expression, which was below the reference range found in severe to critically ill COVID-19 patients
Revisiting the Overlooked Infection: Rickettsioses
The prevalence of human Rickettsioses cases in Indonesia is unknown and could probably be underestimated. The high prevalence of seropositive Rickettsia sp. was reported in small mammals (as vectors) and humans. In Indonesia, a recent study in patients with acute fever revealed that the prevalence of Rickettsioses is 10%. Many cases of Rickettsioses were often misdiagnosed with dengue fever, enteric fever, or leptospirosis due to their overlapping clinical manifestation. The limitation of point of care testing in Indonesia hindered the adequacy of diagnosis confirmation. Appropriate empirical or definitive treatment with macrolide, mainly doxycycline, is preferable compared to other broad-spectrum antibiotics, such as cephalosporin or quinolones. Moreover, when left untreated, Rickettsioses may deteriorate progressively to fatal outcomes, such as meningitis, sepsis, and even death. The awareness of health care practitioners, the availability of confirmatory rapid diagnostic tests and adequate treatment choices are important in eradicating this disease
Primary Mediastinal Gray Zone Lymphoma in an Immunocompetent Young Adult
A 28 -year-old man presented to our hospital with a rapidly growing nodule in the left cervical and bilateral axilla with a history of loss of weight. He has been experiencing a nodule in her right cervical since two years ago and had done FNAC and diagnosed extrapulmonary tuberculosis (EPTB), on Examination at left cervical colli; mass size 9cm x 7 cm, mobile, Lymphadenopathy at pre auricular size 3cm x 3cm, lymphadenopathy supraclavicular size 1cm x 0,5 cm. bilateral lymphadenopathy Axila size 4cmx 4cmx 2xcm mobile. The other physical exam was normal. Laboratory test Hb 10,4 d/dL, WBC 14.250/ mm3, LED 78 mm/hours, D-dimer 1,81 ug/mL, Fibrinogen 452 mg/dL. HIV test non-reactive. HbsAg and HCV test negative. CT-Scan Thorax: Enlarged Anterior mediastinal Lymph node with a diameter 0f 2.9 cm, right paratracheal with a diameter of 1,2cm and 1,1 cm, and right perihilar with a diameter of 1,3 cm. and the left perihilar diameter 0,9. And hypodense lesion of the spleen measuring 2,3cm x 1,6 cm. The patient underwent a biopsy with pathology biopsy and immunohistochemistry (IHC), CD 20+. CD 3-, CD 30+ CD79a +, MUM1 +, Ki67 80-90% +, CD15-, BCL6+ and BCL 2+. For this patient, we started an R-CHOP regimen (Rituximab 375 mg/m2 (d1), Cyclophopamid 750 mg/m2 (d1), Doxorubicin 50 mg/m2 (d1), Vincristine1,2 mg/m2 (d1) and 1 Prednisone 100 mg (d1-d5). We presented the patient with PMGZL has achieved a complete response, especially with chemotherapy R-CHOP regimen
Scoring System for Predicting No Hearing Recovery in Unilateral Idiopathic Sudden Sensorineural Hearing Loss
Background: The varying degrees of hearing recovery in idiopathic sudden sensory neural hearing loss (ISSHL) patients indicate the need of model to predict no hearing recovery. We aimed to aid in the counseling of ISSHL patients about their recovery chances by developing a simple clinical scoring system to predict no hearing recovery using clinical information available at first visit. Methods: A retrospective cohort study, using medical records was conducted from January 2017-May 2019 in Cipto Mangunkusumo General Hospital and Proklamasi Ear, Nose, Throat, Head and Neck (ENT-HN) Surgery Specialized Hospital in Jakarta, Indonesia. The outcome measure is no hearing recovery and we built the prediction score developed based on multiple logistic regression analyses and tested for discriminative ability. There were 183 adults unilateral ISSHL patients included in the study. Results: The proportion of no hearing recovery was 56%. The independent predictors were older age 30-60 years and >60 years old (Odds Ratio 4.0; 95% CI 1.4-11.8; p=0.012 and OR 5.3; 95% CI 1.5-18.4; p=0.008, respectively) as compared with 18-<30 years old, later onset (onset 15-60 days and >60 days had OR 5.4; 95% CI 1.7-16.9; p=0.004 and OR 12.6; 95% CI 2.9-54.6; p=0.001, respectively, as compared with onset < 3 days), and presence of vertigo (OR 2.3; 95% CI 1.1-4.6; p=0.026). Prediction scores ranged from 3 to 12, with three categories for age, four for onset, and two for the presence of vertigo. The predictions showed adequate calibration and good discriminative ability (AUC 0.77). Conclusion:Using information of age, onset and presence of vertigo at first visit, ISSHL patient with increased risk of no hearing recovery can be identified with moderate accuracy. This prediction model could help clinician in predicting patients’ prognosis.
Primary Central Nervous System Lymphoma in an Immunocompetent Young Adult Patient: A Rare Case
Primary CNS Lymphoma (PCNSL) is a rare form aggressive extra nodal non-Hodgkin Lymphoma (NHL) that comprising 1-2% of the primary brain tumors that develops in the brain, spinal cord, eye or leptomeningeal area without evidence of systemic involvement. The overall incidence of PCNSL with immunocompetent patients is only 0,47/100.000 year in PCNSL. Approximately 10-20% of patients have ocular involvement and around one third have multifocal neurological disease. Overall long-term survival rate only 20-40%, this is because the management of PCNSL is limited to ability of the drug due to cross the blood brain barrier (BBB). We present a B-cell central nervous system lymphoma in an immunocompetent patient who treat responses with chemotherapy. A 35-year-old man presented to our hospital with suddenly unconscious 4 hours before admission. He was experiencing headache and blurred of vision withing 3 months and have episode seizure. On Examination, GCS E2 M3 Aphasia, Hemiparesis dextra, papil edema, VOD/VOS: NLP. The other physical exam was normal. Laboratory tests Hb 10,7 g/dl, LDH 446 U/L, and D-dimer 3,21ug/ml. Rubella IgG 76,9, CMV Ig G 245,6 and, HSV IgG and IgM negative, HIV test non-reactive, Toxoplasma IgG and Toxoplasma IgM negative, HbsAg and HCV test negative. Brain MRI and MRI Spectroscopy: Lobulated mass size 7,08 cm x 4,75 cm at caudates nucleus sinistra-periventricular lateralis sinistra, Cholin/NAA ratio: 5-9, Cholin/Creatin ration 6-11 suspect malignancy dd/Lymphoma. MRI whole spine: Bulging discus intervertebral C4-C5. Chest and Abdomen CT-Scan are normal. Bone Survey normal, EEG: Epileproform left temporal. Cerebrospinal Fluid: Gliosis reaction sup malignancy.The patient underwent craniotomy and biopsy Pathology Anatomy and IHC Basal Ganglia revealed a Diffuse Large B Cell Lymphoma (NHL) Non-Germinal Center, CD 20 +, Ki 67 95% (High Grade), CD 45 +, CD 3 -, BCL6 +, Mum 1+. The patient we give induction therapy with RMP Regimens (Rituximab 375 mg/m2, day 1, 15 and 29, High Dose Methotrexate (HDMTX) 3000mg/m2 day 2, 16 and 30, and Procarbazine 60mg/m2 day 3-12) because Procarbazine in not available in Palembang we change to Dacarbazine 375mg/m2 days 3,17 and 31), Dexamethasone 5mg/6 hours, and has finished Low Dose Whole Brain Radiotherapy for consolation therapy. PCNSL is rare form aggressive extra nodal NHL, especially in Immunocompetent patient. In this particular case of patients High Dose Methotrexate Chemotherapy has achieved high respond especially for this patient that showed GCS E4M5V6 and recovery neurological deficit after 2 cycle chemotherapy
Chronic Kidney Disease Care in Indonesia: Challenges and Opportunities
The burden of chronic kidney disease (CKD) is a significant global health concern. Previous study reported that the CKD incidence reached 200 cases per million per year in many countries with the prevalence 11.5% (4.8% in stages 1-2 and 6.7% in stages 3-5). Other study further reported that the estimate prevalence of CKD was 15% higher in low- and middle-income countries compared to the high-income countries. However, there are limited statistics available on the epidemiology of CKD in Indonesia. According to the Basic Health Research (Riset Kesehatan Dasar [Riskesdas], 2018], the prevalence of CKD in Indonesia increased from 0.2% in 2013 to 0.3% in 2018. These results may understate the true prevalence of CKD in our population. Despite the limited data on the CKD prevalence, the number of patients receiving kidney replacement treatment (KRT), primarily in the form of hemodialysis, is rapidly rising (i.e., more than 132.000 in 2018).A comprehensive nephrology referral system is also a challenge. We can argue this statement with evidence from the tertiary care, where it was reported that most kidney failure patients (83%) commenced dialysis with an urgent start, along with late referral to nephrologist (90%), started dialysis with temporary catheter (95.2%), and the median eGFR to start dialysis was 5.3 (range: 0.6 – 14.6) ml/minute/1.73 m2. However, individual awareness, as well as an effective screening and prevention program for high-risk group are also a significant hurdles. Since 2022, the Ministry of Health has initiated a health transformation program to improve the health system, to address health disparities, both within the country and between countries. One of the health transformation programs which specify in nephrology care is the implementation of the Uro-Nephrology Support Program (Program Pengampuan Uro-Nefrologi), with the aim to strengthen services, provide equal distribution of services and increase the latest technology for the diagnosis and treatment of urology/nephrology diseases in Indonesia. This program included secondary and tertiary care to improve the extent and quality of care to slowing the CKD progression, improving kidney replacement therapy (hemodialysis, peritoneal dialysis, and kidney transplant) access and treatment, as well as to provide dialysis training program for health care workers. Providing high-quality nephrology care that all Indonesians can access is challenging. Yet, steps have already been taken in the direction of service enhancement. Thus, there is hope for better kidney health in Indonesia. Governments, academic medical centres, nephrology societies, as well as the citizen will all need to work together and take consistent effort to make a sustainable and comprehensive kidney care