Medical Journal of Malaysia最新文献

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital malformation of the female urogenital tract characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. It was formerly known as Herlyn Werner Wunderlich Syndrome (HWWS). The syndrome usually presents with cyclic pelvic pain following menarche. Endometriosis is a prevalent complication. Magnetic resonance imaging (MRI) helps in diagnosing OHVIRA syndrome and endometriosis due to its high contrast resolution and objectivity. We reported a 13- year-old girl who was evaluated for cyclic pelvic pain after her menarche at 12 years of age. Magnetic resonance imaging (MRI) revealed two separate uterine cavities, services and vaginae, indicating didelphys. The left uterine cavity is filled with fluid, and the left hemivagina is dilated and filled with hyperintense and hypointense fluid on T1 and T2, respectively, indicating blood products. Left hemivagina dilatation implicated the presence of an obstructing vaginal septum. A single left adnexal cyst lesion with blood products was suggestive of an endometriotic cyst. Additionally, the left kidney was absent. A uterine didelphys with left hemivagina obstruction, hematometra, hematocolpos, and the ipsilateral ovarian endometriotic cyst was diagnosed. A final diagnosis of OHVIRA syndrome or HWWS was made, considering that she had no left kidney.MRI is a suitable diagnostic tool for precise anatomical delineation of the uterus, cervix, and vagina in uterovaginal disorders such as OHVIRA syndrome. MRI can also properly evaluate endometriosis and adhesion.

Juvenile nasopharyngeal angiofibroma (JNA) is a rare paediatric tumour known for its local destructiveness and high recurrence rate. Surgery is the primary treatment modality for JNA, though other options, such as hormonal therapy, embolisation and radiotherapy, exist for inoperable cases. The location of the tumour makes surgical intervention challenging. A 14-year-old male presented with epistaxis and headaches as the chief complaints and was diagnosed with nasopharynx angiofibroma by computed tomography (CT) scan in 2018. Pre-operative embolisation was performed and followed by surgical removal of a 4 cm tumour in January 2019. Pathological examination revealed CD34 positivity, S100 negativity and Ki-67 positivity (5 to 10%), confirming angiofibroma. In October 2019, a 3.6 cm recurrent tumour was treated with embolisation and a second surgery. Pathological findings again confirmed JNA. The patient underwent four surgeries in total, but epistaxis persisted. In 2021, local radiotherapy was administered using intensity-modulated radiation therapy (IMRT) at a dose of 60 Gy in 25 fractions. Serial magnetic resonance imaging (MRI) post-radiotherapy showed a decreasing tumour size, with no further epistaxis and no observed radiation side effects 2 years post-treatment. Radiation therapy remains a strong alternative for managing recurrent JNA.

Introduction: Cholestasis is bile flow disruption that leads to bile accumulation, which could lead to liver fibrosis. Ursodeoxycholic acid (UDCA) has a hepatoprotective effect. Glutathione (GSH) is an endogenous antioxidant that plays a role in maintaining the function and structure of liver cells. This study aimed to examine the effect of UDCA-GSH combination therapy in multiple doses on liver function in the Sprague-Dawley rats' liver fibrosis model.

Materials and methods: This was a randomised post-testonly study. A total of 28 rats were assigned into four groups: Group 1 is control group (C), samples had bile duct ligation and UDCA monotherapy 20 mg; Group 2, bile duct ligation + UDCA 10 mg + glutathione 10 mg (P1); Group 3, bile duct ligation + UDCA 20 mg + glutathione 15 mg (P2); Group 4, bile duct ligation + UDCA 30 mg + glutathione 20 mg (P3). Serum AST, ALT, ALP activity, total, direct and indirect bilirubin were collected. Shapiro-Wilk test was used for the normality test. All groups' data were compared using Kruskall-Wallis and Mann-Whitney tests.

Results: There was a significant difference in the ALP level in all rats and between the C and P2 groups. ALP level of all groups decreased significantly compared to the control group. Combination therapy group showed lower bilirubin levels. ALT levels significantly differed between the C-P1, P1-P2, and P1-P3 groups.

Conclusion: UDCA-GSH therapy improves liver function in BDL rats' models compared to UDCA monotherapy.

Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive paediatric brain tumour and nowadays has not had satisfactory result, with most patients do not survive within 1 year of diagnosis. Due to its proximity to critical organs, surgery is avoided, and radiation is the mainstay of treatment. In this case report, we present a case of DIPG treated with radiation and concurrent temozolomide. A 7- year-old child was admitted with complaints of weakness in the eyelid, upper and lower limbs 2 months ago. Physical examination showed tetra paresis and bilateral cranial nerve palsy. Magnetic resonance imaging (MRI) scan showed intracranial tumour consistent with DIPG. Diagnosis was made based on imaging as surgery or biopsy can lead to further morbidity. The patient underwent radiotherapy with concurrent chemotherapy of temozolomide. Radiation was given by dose of 54 Gy/30 fractions (30 × 1.8 Gy) with volumetric arc therapy (VMAT). Due to technical issue after the first five irradiations resulting in 2 weeks delay, boosting of dose by 5 × 1.8 Gy was then given, hence, the total dose was 63 Gy. The booster only targeted the gross tumour volume. Following radiation, the patient felt clinical improvement. Eyelid and limb movement improved since the 15th fraction. At the last fraction, the patient's condition improved symptomatically, but experienced complaints related to post radiation oedema including dizziness and nausea. These complaints were improved upon steroids administration. The MRI evaluation will be done after 8 to 12 weeks of radiation, considering the effects of acute radiation could still occur at this period. In conclusion, a combination of radiotherapy and temozolomide could be an option for DIPG management, with tolerable acute toxicity and possible clinical improvements.

Introduction: Oesophageal atresia (EA) is a life-threatening congenital oesophageal deformity that causes considerable newborn morbidity and death. Many prognostic variables have been linked to the survival of infants with EA, although the results of the studies are still conflicting. Furthermore, studies on EA effects in developing countries still need to be included. Here, we aimed to determine the survival of children with EA and link it to prognostic variables in a particular developing country.

Materials and methods: A cross-sectional observational retrospective study was conducted using medical records of paediatric patients with EA at our institution from January 2014 to December 2020.

Results: A total of 53 children with EA were included in the study. Log-rank analysis showed that definitive surgery and thrombocytopenia were significantly associated with the survival of children with EA, with a p-value of 0.007 and 0.002, respectively, whereas, sex, EA type, pneumonia and sepsis were not (p = 0.898, 0.919, 0.255, and 0.499, respectively). Multivariate analysis revealed that thrombocytopenia and definitive surgery were strongly associated with the survival of children with EA with a pvalue of 0.014 (hazard ratio (HR) = 2.67 [95% confidence interval (CI) = 1.22-5.85]) and 0.022 (HR =0.39 [95% CI = 0.17- 0.87]), respectively.

Conclusion: Our study shows that thrombocytopenia might increase mortality, while definitive surgery might be beneficial for the survival of paediatric patients with EA. It implies that definitive surgery should be performed as early as necessary to prevent further morbidity and mortality. Our study comprehensively provides the survival of children with EA and links it to prognostic variables in a particular developing country. It serves as a potential research project that can be applied to the clinical setting to help clinicians manage EA better.

Introduction: Conjoined twins (CT) is a rare congenital disorder characterised by the presence of malformations associated with secondary abnormal conjoined organ changes and abnormal hemodynamic superimposed effects about 1 in every 200 identical twin pregnancies, between 1 in 50,000 to 1 in 100,000 live births. The aim of this study is to describe the characteristics of conjoined twins.

Materials and methods: This was a retrospective descriptive study. All medical records of conjoined twins who were admitted to Hasan Sadikin Bandung General Hospital from January 1st, 2015, to June 30th, 2023, were reviewed for gender, conjoined type, birth order, risk factor and treatment.

Results: Of the 28 conjoined twins, 21 were girls (75%), and 7 were boys (25%); 19 (67,85%) were of the thoracoomphalopagus type; 11 (39,28%) were born as first children; 18 (64,28%) were born at 37 weeks of gestational age; and 22 twins' (78,57%) parents were aged between 21 and 35 years. None of the mothers had used medication, 13 (46,42%) took folic acid on occasion, five (17,85%) used traditional herbs, nine (32,14%) smoked and none drank alcohol. Parents who live in industrial areas were 18 (64.28%). There was no history of conjoined twins in previous pregnancies or deliveries or in the parent's family. Liver separation had been done in four (14.28%). Emergency separation in one twin. Nine (21.42%) patients died before surgery due to a worsening condition.

Conclusion: The conjoined twins were more common in girls, predominantly of the thoracoomphalopagus type. Risk factors that were commonly found were the first child, a gestational age of less than 37 weeks, and living in an industrial area.

Introduction: Gastric perforation is a rare occurrence, particularly in neonates. This is an emergency case in this population. The incidence of spontaneous gastric perforation in neonates is 1:2900 live births, with high mortality and morbidity rates. The primary treatment is surgical debridement and repair of the perforation, which has a high incidence of anastomotic leakage. At present, there is a plethora of studies investigating the efficacy of human dried amniotic membrane (H-DAM) technology in promoting wound healing. Consequently, researchers sought to ascertain whether there were differences in the number of adhesion and abscess classifications for the macroscopic evaluation of gastric perforation repair with HDAM as a biomaterial in New Zealand white rabbits.

Material and methods: A total of 30 male New Zealand rabbits underwent laparotomy and gastric perforation. These animals were then divided into three groups, with each group comprising 10 rabbits. Group 1 underwent primary repair, group 2 underwent omental patch repair, and group 3 underwent H-DAM patch repair. The rabbits were euthanised on the 7th day and the adhesion score and abscess classification were evaluated.

Result: A total of 30 samples of rabbits were homogeneous. On macroscopic evaluation, it was found that the H-DAM had the lowest mean adhesion score and the lowest incidence of abscess formation compared to all other groups.

Conclusions: It can be concluded that the utilisation of HDAM as a biomaterial patch in the treatment of gastric perforation in the rabbit model did not result in any instances of leakage, adhesion or infection.

Introduction: The number of inflammatory bowel diseases cases has increased throughout the years. Since, the current therapeutic methods have their adverse effects, this is leading to the development of alternative therapy derived from natural products.

Materials and methods: In the present study, our objective was to explore the potential of Citrus aurantifolia peel extract (CAPE) on 2, 4, 6-trinitrobenzene sulfonic acid (TNBS) induced colitis in mice. Twenty-eight male Balb/c mice were divided into four groups: (1) normal group, (2) TNBS group, (3) 125 mg/kg CAPE group and (4) 250 mg/kg CAPE group. Colitis was induced through rectal administration of TNBS. The anti-inflammatory effects of CAPE against colitis were assessed by body weight, DAI score, colonic length, weight-to-length ratio, haematology profile and histopathological examinations.

Results: Our results showed that CAPE maintained the body weight of mice, repressed the increase of DAI score, maintained mice colonic length and weight, improved blood profile and suppressed the excessive production of TNF-α, IL-6 and IL-1β. Furthermore, CAPE improved the histopathological score of colitis mice.

Conclusion: All the findings of this study suggested that Citrus aurantifolia peel extract may be a potential natural agent for protecting mice against TNBS-induced colitis.

Introduction: Inflammation caused by diabetes can damage multiple organs, including the lungs. Vitamin D (VD) has been shown to potentially reduce inflammation and boost the immune system. VD might play a role in diabetes' inflammatory response. This study aims to elucidate the evidence regarding the lung as the target organ for DM and the possible role of VD in preventing pulmonary damage progression in the diabetes rat model.

Material and methods: Thirty Sprague Dawley rats (3-monthold, 200 to 300 gm) were randomly divided into six groups, namely control (C), 4 weeks diabetes mellitus (DM1), 8 weeks DM (DM2) and three DM1 groups (VD1, VD2, and VD3) who received Vitamin D doses of 0.125, 0.25 and 0.50 μg/kg BW, respectively. After 4 weeks, daily VD was administered intraperitoneally for 30 days. Lung tissues were taken for IL- 6, MCP-1, NFKB and CD68 mRNA expression analysis and paraffin embedding. Immunohistochemical staining against CD68 and MCP-1 was conducted. Data were analysed using one-way ANOVA. p < 0.05 was considered statistically significant.

Results: DM2 group represented significantly higher IL6, MCP1, NFKB and CD68 mRNA expression than Control group (p < 0.05). Meanwhile, VD2 and VD3 groups revealed significantly lower mRNA expression of IL-6, MCP1, NFKB and CD68 than DM2 (p < 0.05). Immunostaining revealed the spreading of MCP1 protein expression in lung tissue along with macrophage infiltration in the DM2 group, which was reduced in the VD2 and the VD3 groups.

Conclusion: VD shows a protective effect on diabetesinduced lung damage by regulating inflammation factors.

Introduction: Hirschsprung disease (HSCR) is a congenital disorder caused by the absence of ganglion cells, which leads to a functional obstruction in infants. HSCR is divided into short, long and total colon aganglionosis (TCA). However, post-operative outcome assessment of patients with long-segment and TCA is scarce. We determined the functional outcomes, Hirschsprung-associated enterocolitis (HAEC) and complications of long-segment and TCA HSCR's children following pull-through surgery.

Materials and methods: Descriptive analysis research was done for children with HSCR long-segment and TCA who underwent an operation at our institutionfrom 2013 to 2020. We assessed the functional outcome and HAEC by the Krickenbeck and the HAEC scoring, respectively.

Results: We ascertained 13 HSCR long-segment and six TCA. We performed the following surgical procedures: Duhamel (n=7), Martin (n=4), Kimura (n=1), transabdominal Yancey-Soave (n=3) and transanal endorectal pull-through (n=4). All long-segment patients revealed good functional outcomes, whereas two TCA children suffered soiling and failed to achieve voluntary bowel movement. HAEC was noted in three long-segment and four TCA patients. Furthermore, surgical site infection and diaper rash were noticed in 10 and two patients, respectively.

Conclusion: Long-segment patients might have better functional outcomes TCA group, whereas the frequency of HAEC is compatible among arms. Long-term follow-up is important and necessary to identify complications early and define the proper treatment. Our study comprehensively analyzes functional outcomes, HAEC and complications of children with HSCR long-segment and TCA after definitive surgery in a developing country.