Pyridoxine Refractory Sideroblastic Anemia: Diagnosis and Misdiagnosis

Muhammad Matloob Alam,Abdulrhman Alathaibi,Ruwayd Adel Attar,Muhammad Kashif,Hamdan Saeed Al-Ghamdi,Sultan Abdulaziz Alharthi,Abdulmohsen Bokhary,Muteb Althomali 대한소아혈액종양학회 2022 Clinical Pediatric Hematology-Oncology Vol.29 No.2

We report the case of a 7-year-old girl who was originally diagnosed at the age of 6 months with transfusion-dependent red cell aplasia based on a combination of se-vere anemia, reticulocytopenia and bone marrow findings. Since early infancy due to severe microcytic/hypochromic anemia she received multiple packed RBCs transfusions. She subsequently developed hepatomegaly, hypothyroidism, diabetes, liver cirrhosis and latterly, a severe cardiomyopathy due to significant iron overload refractory to regular chelating agents. Genetic study was offered, confirmed the pres-ence of SLC25A38 gene mutation and her diagnosis was revised to pyridoxine re-fractory sideroblastic anemia (PRSA). It is a non-syndromic, autosomal recessive dis-order, characterized by severe microcytic anemia since infancy and increased serum ferritin, which is not responsive to pyridoxine. Since the clinical course of this dis-order is very similar to that of thalassemia major and other red cell aplasia. Prompt recognition and initiation of appropriate treatment are important to reduce the de-velopment of secondary disease complications due to iron overload. Given the poten-tial for misdiagnosis and delay in the recognition of sideroblastic anemia, a careful bone marrow examination and genetic study should be included while investigating children with unexplained anemia.

Implementation of co-teaching approach in an inclusive classroom: overview of the challenges, readiness, and role of special education teacher

Abdul Rahim Hamdan,Muhammad Khairul Anuar,Aqeel Khan 서울대학교 교육연구소 2016 Asia Pacific Education Review Vol.17 No.2

The objective of this study was to determine the relationship aspect of the challenge, readiness, and the role of special education teacher (SET) in implementing common approaches in inclusive classrooms. Experiences as a moderator were used to see the effect of the co-teaching component. This study used a sampling method that involved 240 respondent’s representatives of the population of SET in Malaysia. The findings of this study showed that there was a high positive tendency in choosing the approach that is likely to progress than the existing ones. In this area, there were challenges and readiness of positive selection than teacher experience. Overall, this study could impact the implementation of co-teaching approach than the implementation of the existing programme.

Activity of Pralsetinib Versus Selpercatinib in Patients with RET Fusionpositive Non-Small Cell Lung Cancer (NSCLC)

( Faiza Ahmed ),( Israr Khan ),( Nazma Hanif ),( Alaa Hamdan ),( Mohammad Ali Zaidi ),( Zeynep Yukselen ),( Umer Salman ),( Sakshi Mishra ),( Jack Michel ),( Muhammad Haris Khan ),( Andre Thompson ),( 대한결핵 및 호흡기학회 2021 대한결핵 및 호흡기학회 추계학술대회 초록집 Vol.129 No.-

Background Pralsetinib and selpercatinib are anticancer drugs targeting RET fusions (oncogenic drivers) in non-small cell lung cancer (NSCLC). The US FDA approved both drugs in 2020 for the treatment of RET-fusion positive NSCLC. Methods We assessed the efficacy & safety of pralsetinib versus selpercatinib in RET fusion-positive NSCLC in two clinical trials: phase1/2 ARROW trial (n=354) and phase 1/2 LIBRETTO-001 trial (n=144). Results In patients who had previously received platinum-based therapy (n=105), Drilon et al., 2020 showed an overall response rate (ORR) of 64% (95% CI, 54-73) and a median duration of response (mDOR) of 17.5 months (95% CI, 12.0-NE) with selpercatinib in phase 1/2 LIBRETTO-001 trial. Furthermore, a complete response (CR) of 2%, partial response (PR) of 62%, stable disease (SD) of 29%, and median progression-free survival (mPFS) of 16.5 months ( 95% CI, 13.7 - NE) were reported. Treatment naive patients (n=39) demonstrated an ORR of 85% (95% CI, 70-94) and PR of 85%. The responses were determined by the independent review committee. Lee et al., 2019 (n=111) demonstrated a rapid lowering of RET circulating tumor DNA (ctDNA) in 81% of NSCLC patients with pralsetinib therapy. A phase 1/2 ARROW trial (n= 116) exhibited a median follow-up of 8.8 months in patients who received pralsetinib 400 mg daily. Additionally, ORR was 65%, CR 6%, disease control rate 93 (95% CI, 87 - 97), and resulted in overall tumor size reduction in 96% of patients. Refer to (Table 1) for further details. Conclusion Pralsetinib and Selpercatinib have shown promising anti-tumor activity and an acceptable safety profile in RET fusion-positive NSCLC. However, we cannot draw a firm conclusion regarding which one is better due to insufficient data. Clinical trials, including AcceleRET, are still ongoing, and data is pending. Further comparative studies are needed to derive meaningful survival outcomes.

Correlation Between Neutrophil-to-Lymphocyte Ratio and Motoric Deterioration in Patients With Guillain-Barre Syndrome

Felisitas Farica Sutantoyo,Fadil Mudjiani Basuki,Fidiana Muhammad Hamdan 대한신경과학회 2022 Journal of Clinical Neurology Vol.18 No.6

Background and Purpose Guillain-Barre syndrome (GBS) is a common cause of inflammation-related acute flaccid paralysis, and is characterized by acute onset, rapid progression, and symmetrical weakness. GBS is an emergency with high morbidity and long-term disability rates. It is important to determine the prognostic factors for GBS in order to improve the disease outcomes. This study aimed to identify the correlation between the neutrophil-to-lymphocyte ratio (NLR) on day 1 of hospitalization (D1) and motor deterioration in GBS patients. Methods This observational analytical study applied a cross-sectional analysis to the medical records of GBS patients who were hospitalized at Dr. Soetomo General Hospital Surabaya from January 2018 to March 2020. The analysis used the chi-square bivariate test, multivariate analysis with logistic regression, and correlation analysis with the Spearman test. Results The study included 61 subjects. Statistical tests showed that there was no correlation between NLR and changes in the Medical Research Council sum scores (ΔMRC sum scores) during D1–D3, D1–D7, D1–D14, and D1 to the day of discharge (p>0.05). There was a significant correlation between NLR and the Erasmus GBS outcome score (EGOS) (p=0.006). NLR values differed significantly within each treatment group (p=0.001). Therefore, a subanalysis within each treatment group was conducted, which revealed a significant negative correlation (p<0.05) between NLR and the ΔMRC sum score during D1–D14 in the group treated without immunotherapy. Conclusions There was no correlation between NLR and motor deterioration in patients with GBS during hospitalization. However, NLR was significantly correlated with EGOS, and there was a negative correlation between NLR and motor deterioration during D1–D14 in GBS patients treated without immunotherapy.

Delayed Cerebral Ischemia after Embolization in Ruptured Spinal Arteriovenous Fistula with Subarachnoid Hemorrhage : A Case Report

Achmad Firdaus Sani,Dedy Kurniawan,Muhammad Hamdan,Jovian Philip Swatan 대한신경외과학회 2023 Journal of Korean neurosurgical society Vol.66 No.2

Delayed cerebral ischemia (DCI) remains a devastating complication in subarachnoid hemorrhage (SAH), however, there were no present reports that is associated with a ruptured spinal arteriovenous fistula (sAVF). We would like to present a rare case of DCI following embolization of a ruptured perimedullary sAVF. Initially, the patient clinical symptoms mimic a SAH caused by a ruptured intracranial aneurysm. Further evaluation revealed that the SAH was caused by a ruptured perimedullary sAVF and the patient’s condition improved following the embolization procedure. Three days later, the patient developed an acute left-sided facial and motor weakness, which persisted until the patient was discharged on the day-15 onset. A magnetic resonance imaging and angiography is performed 1.5 years after discharge and revealed no signs of cerebral infarction and hemorrhage. In this paper, we reported DCI after embolization in a ruptured sAVF with SAH, supported by evidence from the current literature. We would like to also stress the importance of complete spinal and cerebral vessel imaging to reveal the underlying abnormalities and determine the most appropriate intervention.

Biochemical screening of Lactobacilli in midgut of wild Aedes aegypti mosquitoes. A step toward paratransgenesis candidate

Alhaji Hamisu Maimusa,Abu Hassan AHMAD,Nur Faeza Abu Kassim,Hamdan Ahmad,Hamady Dieng,Junaid RAHIM,Muhammad Ahmad DAHIRU 한국곤충학회 2019 Entomological Research Vol.49 No.2

This study was conducted to isolate and identify lactobacilli from larval and adult midgut of wild Aedes aegypti (Ae. aegypti) to find candidate bacteria for paratransgenic control. Characterization of the bacterial symbionts was done using Gram staining, motility test, catalase test, and biochemical tests, among others, and the morphological features were confirmed using a standard scheme that simplifies the identification of lactic acid bacteria. A total of 174 strains were isolated and identified, 135 strains from larval midgut and 39 strains from adult midgut (mean ± SE, 4.00 ± 0.72; P = 0.00). The isolated species were confirmed to be Lactobacillus fermentum, L. casei, L. acidophilus, L. viridescens, L. brevis and L. gasseri. It can be concluded that Ae. aegypti has the potential of harboring the cultivable bacterial symbionts. In conclusion, the isolated species were nominated for paratransgenic control, particularly L. fermentum, being found in large numbers from both larval and adulxt midgut.

The Safety and Efficacy of Anakinra, an Interleukin-1 Antagonist in Severe Cases of COVID-19: A Systematic Review and Meta-Analysis

Manoj Kumar Reddy Somagutta,Lourdes Pormento Maria Kezia,Pousette Hamid,Alaa Hamdan,Muhammad Adnan Khan,Rockeven Desir,Rupalakshmi Vijayan,Saloni Shirke,Rishan Jeyakumar,Zeryab Dogar,Sarabjot Singh Ma 대한감염학회 2021 Infection and Chemotherapy Vol.53 No.2

This study aims to assess anakinra's safety and efficacy for treating severe coronavirus disease 2019 (COVID-19). Numerous electronic databases were searched and finally 15 studies with a total of 3,530 patients, 757 in the anakinra arm, 1,685 in the control arm were included. The pooled adjusted odds ratio (OR) for mortality in the treatment arm was 0.34 (95% confidence interval [CI], 0.21 - 0.54, I2 = 48%), indicating a significant association between anakinra and mortality. A significant association was found regarding mechanical ventilation requirements in anakinra group compared to the control group OR, 0.68 (95% CI, 0.49 - 0.95, I2 = 50%). For the safety of anakinra, we evaluated thromboembolism risk and liver transaminases elevation. Thromboembolism risk was OR, 1.59 (95% CI, 0.65 - 3.91, I2 = 0%) and elevation in liver transaminases with OR was 1.35 (95% CI, 0.61 - 3.03, I2 = 76%). Both were not statistically significant over the control group. Anakinra is beneficial in lowering mortality in COVID-19 patients. However, these non-significant differences in the safety profile between the anakinra and control groups may have been the result of baseline characteristics of the intervention group, and further studies are essential in evaluating anakinra's safety profile.

The Efficacy of vitamin C, thiamine, and corticosteroid therapy in adult sepsis patients: a systematic review and meta-analysis

Manoj Kumar Reddy Somagutta,Maria Kezia Lourdes Pormento,Muhammad Adnan Khan,Alaa Hamdan,Namrata Hange,Manish KC,Sukrut Pagad,Molly Sanjay Jain,Sivasthikka Lingarajah,Vishal Sharma,Jaspreet Kaur,Berna 대한중환자의학회 2021 Acute and Critical Care Vol.36 No.3

Background: Previous studies have suggested favorable outcomes of hydrocortisone, ascorbic acid (vitamin C), and thiamine (HAT) therapy in patients with sepsis. However, similar results have not been duplicated in sequential studies. This meta-analysis aimed to reevaluate the value of HAT treatment in patients with sepsis. Methods: Electronic databases were searched up until October 2020 for any studies that compared the effect of HAT versus non-HAT use in patients with sepsis. Results: Data from 15 studies (eight randomized controlled trials [RCTs] and seven cohort studies) involving 67,349 patients were included. The results from the RCTs show no significant benefit of triple therapy on hospital mortality (risk ratio [RR], 0.99; P=0.92; I2=0%); intensive care unit (ICU) mortality (RR, 0.77; P=0.20; I2=58%); ICU length of stay (weighted mean difference [WMD], 0.11; P=0.86; I2=37%) or hospital length of stay (WMD: 0.57; P=0.49; I2=17%), and renal replacement therapy (RR, 0.64; P=0.44; I2=39%). The delta Sequential Organ Failure Assessment (SOFA) score favored treatment after a sensitivity analysis (WMD, –0.72; P=0.01; I2=32%). However, a significant effect was noted for the duration of vasopressor use (WMD, –25.49; P<0.001; I2=46%). The results from cohort studies have also shown no significant benefit of HAT therapy on hospital mortality, ICU mortality, ICU length of stay, length of hospital stay, the delta SOFA score, the use of renal replacement therapy, or vasopressor duration. Conclusions: HAT therapy significantly reduced the duration of vasopressor use and improved the SOFA score but appeared not to have significant benefits in other outcomes for patients with sepsis. Further RCTs can help understand its benefit exclusively.

Intravenous Leiomyomatosis Extending from the Uterus to the Heart

Mohd Yusof Mubarak,Awang Ramli Kama Azira,Yahaya Zainab,Ghapar Abd Kahar,Mohamed Dani Noordini,Ismail Hamidah,Muhammad Nor Mohamad Arif,Leman Hamdan,Mohammed Yusri 아시아심장혈관영상의학회 2019 Cardiovascular Imaging Asia Vol.3 No.2

We report a case of intravenous leiomyomatosis with right atrium extension through the left gonadal vein, left renal vein and inferior vena cava. The tumor moved in and out of the right ventricle via the tricuspid valve, which could lead to valve obstruction and sudden death. We illustrate the importance of multimodality cardiac imaging to diagnose this condition by performing echocardiography, cardiac magnetic resonance imaging and computed tomography.

Efficacy and Safety of Tepotinib and Capmatinib in the Treatment of Metastatic Non-Small Cell Lung Cancer (NSCLC) Harboring Mesenchymal- Epithelial Transition (MET) Exon 14 Skipping Alterations

( Faiza Ahmed ),( Israr Khan ),( Alejandra Castro-varela ),( Alanna Barrios-ruiz ),( Zarlakhta Zamani ),( Alaa Hamdan ),( Sakshi Mishra ),( Muhammad Khan ),( Umer Salman ),( Zeynep Yukselen ),( Kim An 대한결핵 및 호흡기학회 2021 대한결핵 및 호흡기학회 추계학술대회 초록집 Vol.129 No.0

Background The United States Food and Drug Administration (FDA) approved tepotinib and capmatinib for the treatment of mesenchymal-epithelial transition (MET) exon-14 mutated non-small cell lung cancer (NSCLC) in February 2021 and May 2020, but no comparative study has been done so far. Here we compare Results of two-phase 2 trials evaluating the efficacy and safety of these new drugs. Methods Studies demonstrating the role of tepotinib and capmatinib in NSCLC patients were identified using PubMed, Scopus, and Google Scholar databases from inception till February 2021. Results Participants received tepotinib (n=152) and capmatinib (n=97) in two distinct studies. The main efficacy measures in the combined-biopsy group were overall response rate (ORR) of 46% and 56% with tepotinib therapy in VISION trial as per independent review committee and investigator assessment. In contrast, ORR of 41% was noted in previously treated patients vs. 68% in treatment naive patients with capmatinib monotherapy in GEOMETRY mono-1 study. Patients in the tepotinib group showed a median duration of response (mDOR) and a median progression free survival (mPFS) of 11.1 months and 8.5 months, respectively. While with capmatinib study, the mDOR varied from 9.7 months with a mPFS of 5.4 months in those who received prior therapy to mDOR of 12.6 months with a mPFS of 12.4 months in capmatinib monotherapy. The most commonly reported toxicity was grade 3-4 peripheral edema in both arms (tepotinib: 7% vs. Capmatinib: 11%/14%). Conclusions Both drugs exhibited promising activity in the treatment of NSCLC carrying MET exon 14 skipping mutation. However, capmatinib showed an improved ORR, mDOR, and mPFS in treatment naive patients compared to patients treated with tepotinib. Further large and long-term direct comparative clinical trials are warranted to elaborate on beneficial outcomes.