Lack of awareness of neurogastroenterology and motility within medical education: Time to fill the gap.

Disorders of gut-brain interaction (DGBI), previously referred to as functional gastrointestinal disorders, affect 40.3% of adults in the general population and are diagnosed in 34.9% of new adult referrals to secondary care gastroenterology services. Despite their high prevalence, studies published in this issue of Neurogastroenterology and Motility by investigators based in Germany, the UK, and the USA demonstrate a mismatch between the clinical burden of DGBI and their representation in medical school and postgraduate curricula. This review outlines the salient findings of these studies and explores why and how negative perceptions toward DGBI exist, including factors related to misinformation and internalized stigma. The authors propose a selection of strategies to ameliorate physicians' attitudes toward and knowledge of neurogastroenterology and motility including linking trainees with dedicated clinician mentors with an interest in motility, exposing trainees to expert patients who can enhance empathy, extending Balint groups into gastroenterology training, and offering motility apprenticeships in specialist units. Urgent improvements to medical school and postgraduate curricula are required to ensure the longevity of this subspecialty field in gastroenterology, and to ensure the needs of a sizeable proportion of gastroenterology patients are appropriately met.

Review article: Emerging and current management of acute-on-chronic liver failure.

BACKGROUND: Acute-on-chronic liver failure (ACLF) is a clinically and pathophysiologically distinct condition from acutely decompensated cirrhosis and is characterised by systemic inflammation, extrahepatic organ failure, and high short-term mortality. AIMS: To provide a narrative review of the diagnostic criteria, prognosis, epidemiology, and general management principles of ACLF. Four specific interventions that are explored in detail are intravenous albumin, extracorporeal liver assist devices, granulocyte-colony stimulating factor, and liver transplantation. METHODS: We searched PubMed and Cochrane databases for articles published up to July 2023. RESULTS: Approximately 35% of hospital inpatients with decompensated cirrhosis have ACLF. There is significant heterogeneity in the criteria used to diagnose ACLF; different definitions identify different phenotypes with varying mortality. Criteria established by the European Association for the Study of the Liver were developed in prospective patient cohorts and are, to-date, the most well validated internationally. Systemic haemodynamic instability, renal dysfunction, coagulopathy, neurological dysfunction, and respiratory failure are key considerations when managing ACLF in the intensive care unit. Apart from liver transplantation, there are no accepted evidence-based treatments for ACLF, but several different approaches are under investigation. CONCLUSION: The recognition of ACLF as a distinct entity from acutely decompensated cirrhosis has allowed for better patient stratification in clinical settings, facilitating earlier engagement with the intensive care unit and liver transplantation teams. Research priorities over the next decade should focus on exploring novel treatment strategies with a particular focus on which, when, and how patients with ACLF should be treated.

Clinical Presentations of Acquired Hypothyroidism in Children: Experience in a Tertiary Care Hospital in Bangladesh.

A cross sectional study was conducted in Paediatric Endocrine Outpatient Department of BIRDEM General Hospital, a tertiary care centre in Dhaka, Bangladesh among patients diagnosed with acquired hypothyroidism during the period of January 2012 to December 2016. The study was done to find out the clinical presentations and associated disorders of all patients diagnosed with acquired hypothyroidism during the study period. Data were obtained by reviewing the medical records of the patients. Total 277 children were diagnosed of having thyroid disorders. Among them 145(52.3%) had acquired hypothyroidism. The commonest clinical presentations of children with acquired hypothyroidism were short stature (35.0%), excessive weight gain (31.5%), goiter (23.1%) and poor school performance (14.0%). Autoimmune hypothyroidism was found in 34.4% of children, sub-clinical hypothyroidism in 27.5% children and positive family history was found in 15.2% children with acquired hypothyroidism. The common associated diseases were diabetes and impaired glucose tolerance (4.9%), Down syndrome (3.5%), congenital heart disease (2.1%) and primary adrenal insufficiency (1.4%).

Clinical Presentation of Congenital Adrenal Hyperplasia in Children: Experience in a Tertiary Care Hospital of Bangladesh.

This cross sectional study was conducted in Paediatric Endocrine Outpatient Department of BIRDEM General Hospital, a tertiary care centre in Dhaka, Bangladesh among patients diagnosed with congenital adrenal hyperplasia (CAH) from January 2005 to December 2018. The study was aimed to find out the clinical and laboratory profile of all patients at presentation diagnosed with CAH during the study period. Data were obtained by reviewing the medical records of the patients. Total 102 children with CAH were diagnosed during the study period. Among them 68 were female and 34 were male (female to male ratio of 2:1). Median age was 3.5 month (range 0.2-158 month) and 5.5 month (range 1-108 month) in female and male respectively (p=0.42). Family history was available in 93 patients. Consanguinity was present in 16(17.2%), history of sib death in 12(12.9%), other family members were affected in 8(8.6%). Sixty patient (58.8%) had salt-wasting (SW), 39(38.2%) had simple virilizing (SV) and 3(2.9%) had non- classic form of CAH. Median age of presentation was 2 month (range 0.2-70 month) and 42 month (range 0.8-158 month) in SW and SV group respectively (p=0.001) and 119 month (range 108-152 month) in non- classic group. Common presentations were: genital ambiguity (64.7%), vomiting (46.5%), failure to thrive (41.6%), features of early puberty (precocious pseudopuberty) (24.5%), diarrhea (12.0%). Hyperpigmentation was noted in 49.0% of patients. Among the salt-wasting type in male failure to thrive (FTT) was the most common presentation (83.3%), followed by vomiting (75.0%). In female genital ambiguity was the commonest presentation (97.2%), followed by vomiting (77.1%). Among the simple virilizing type in male early puberty was the commonest presentation (100%) and genital ambiguity was the presenting feature in all the female (100.0%).

Approaches to building rapport with patients.

In this article, I detail my rapport-building trial and error exercise and outline why addressing a patient's occupation at the beginning of a consultation worked during my history taking. Context permitting, addressing occupation can be useful when setting the tone and rhythm of a conversation, helping to focus explanations on the back of a patient's everyday experiences, and allowing a history-taker to address a patient's ideas, concerns and expectations more comfortably. Broaching occupation can also be a useful way to extrapolate information about disease risk factors and baseline understanding from the start. Whatever the rapport-building technique used, self-awareness seems to be a critical skill in this exercise, and an approach used with one patient may not necessarily be useful with another.

Tension hydrothorax in a patient with SARS-CoV-2 pneumonitis and pleural Mycobacterium tuberculosis.

Unilateral pleural effusions are uncommonly reported in patients with SARS-CoV-2 pneumonitis. Herein, we report a case of a 42-year-old woman who presented to hospital with worsening dyspnoea on a background of a 2-week history of typical SARS-CoV-2 symptoms. On admission to the emergency department, the patient was severely hypoxic and hypotensive. A chest radiograph demonstrated a large left-sided pleural effusion with associated contralateral mediastinal shift (tension hydrothorax) and typical SARS-CoV-2 changes within the right lung. She was treated with thoracocentesis in which 2 L of serosanguinous, lymphocyte-rich fluid was drained from the left lung pleura. Following incubation, the pleural aspirate sample tested positive for Mycobacterium tuberculosis This case demonstrates the need to exclude non-SARS-CoV-2-related causes of pleural effusions, particularly when patients present in an atypical manner, that is, with tension hydrothorax. Given the non-specific symptomatology of SARS-CoV-2 pneumonitis, this case illustrates the importance of excluding other causes of respiratory distress.

Superior ophthalmic vein thrombosis secondary to COVID-19: an index case.

Superior ophthalmic vein thrombosis is a very rare condition, known to have a profound negative impact on vision and eye movement function and is usually associated with orbital infections, inflammation, tumors, or carotid cavernous fistulae. There is an increased risk of arterial and venous thrombosis associated with COVID-19, the presence of which is related to a significantly increased risk of mortality. We report an index case of superior ophthalmic vein thrombosis in a 61-year-old male patient who was diagnosed with COVID-19 pneumonitis and a concomitant saddle pulmonary embolus. He was swiftly treated with low molecular weight heparin which led to the resolution of the thrombosis within 3 weeks. This case highlights the importance of considering this entity in the context of COVID-19 as well as providing prompt treatment to reduce the risk of complications.

The relationship between seasonality, latitude and tuberculosis notifications in Pakistan.

BACKGROUND: Pakistan ranks amongst the top 20 highest burden tuberculosis (TB) countries in the world. Approximately 369,548 cases of TB (all forms) were notified in 2018, with an estimated incidence of 265 per 100,000 people per year. In other settings, TB has been shown to demonstrate seasonal variation, with higher incidence in the spring/summer months and lower incidence in the autumn/winter; the amplitude of seasonal variation has also been reported to be higher with increasing distance from the equator. METHODS: Notifications of newly-diagnosed pulmonary and extrapulmonary TB cases were obtained for 139 districts in Pakistan from 2011 to 2017. Data were provided by the Pakistan National TB Control Programme, Islamabad, Pakistan. Statistical analyses were performed to determine whether there was seasonal variation in TB notifications in Pakistan; whether the amplitude of seasonal variation in TB notifications varied according to latitude; whether the amplitude of seasonal variation of TB in Pakistan differed between extrapulmonary TB vs. pulmonary TB. To assess the quarterly seasonality of TB, we used the X-13-ARIMA-SEATS seasonal adjustment programme from the United States Census Bureau. The mean difference and corresponding 95% confidence intervals of seasonal amplitudes between different latitudes and clinical phenotype of TB were estimated using linear regression. RESULTS: TB notifications were highest in quarter 2, and lowest in quarter 4. The mean amplitude of seasonal variation was 25.5% (95% CI 25.0 to 25.9%). The mean seasonal amplitude of TB notifications from latitude 24.5°N- < 26.5°N was 29.5% (95% CI 29.3 to 29.7%) whilst the mean seasonal amplitude of TB notifications from latitude 34.5°N - < 36.5°N was 21.7% (95% CI 19.6 to 23.9%). The mean seasonal amplitude of TB notifications across Pakistan between latitudes 24.5°N to 36.5°N reached statistically significant difference (p < 0.001). The amplitude of seasonal variation was greater for extrapulmonary TB (mean seasonal amplitude: 32.6, 95% CI 21.4 to 21.8%) vs. smear positive pulmonary TB mean seasonal amplitude: 21.6, 95% CI 32.1 to 33.1%), p < 0.001. CONCLUSION: TB notifications in Pakistan exhibit seasonal variation with a peak in quarter 2 (April-June) and trough in quarter 4 (October-December). The amplitude of seasonality decreases with increasing latitude, and is more pronounced for extrapulmonary than for pulmonary TB.

The anatomical basis for transcutaneous auricular vagus nerve stimulation.

The array of end organ innervations of the vagus nerve, coupled with increased basic science evidence, has led to vagus nerve stimulation (VNS) being explored as a management option in a number of clinical disorders, such as heart failure, migraine and inflammatory bowel disease. Both invasive (surgically implanted) and non-invasive (transcutaneous) techniques of VNS exist. Transcutaneous VNS (tVNS) delivery systems rely on the cutaneous distribution of vagal afferents, either at the external ear (auricular branch of the vagus nerve) or at the neck (cervical branch of the vagus nerve), thus obviating the need for surgical implantation of a VNS delivery device and facilitating further investigations across a wide range of uses. The concept of electrically stimulating the auricular branch of the vagus nerve (ABVN), which provides somatosensory innervation to several aspects of the external ear, is relatively more recent compared with cervical VNS; thus, there is a relative paucity of literature surrounding its operation and functionality. Despite the increasing body of research exploring the therapeutic uses of auricular transcutaneous VNS (tVNS), a comprehensive review of the cutaneous, intracranial and central distribution of ABVN fibres has not been conducted to date. A review of the literature exploring the neuroanatomical basis of this neuromodulatory therapy is therefore timely. Our review article explores the neuroanatomy of the ABVN with reference to (1) clinical surveys examining Arnold's reflex, (2) cadaveric studies, (3) fMRI studies, (4) electrophysiological studies, (5) acupuncture studies, (6) retrograde tracing studies and (7) studies measuring changes in autonomic (cardiovascular) parameters in response to auricular tVNS. We also provide an overview of the fibre composition of the ABVN and the effects of auricular tVNS on the central nervous system. Cadaveric studies, of which a limited number exist in the literature, would be the 'gold-standard' approach to studying the cutaneous map of the ABVN; thus, there is a need for more such studies to be conducted. Functional magnetic resonance imaging (fMRI) represents a useful surrogate modality for discerning the auricular sites most likely innervated by the ABVN and the most promising locations for auricular tVNS. However, given the heterogeneity in the results of such investigations and the various limitations of using fMRI, the current literature lacks a clear consensus on the auricular sites that are most densely innervated by the ABVN and whether the brain regions secondarily activated by electrical auricular tVNS depend on specific parameters. At present, it is reasonable to surmise that the concha and inner tragus are suitable locations for vagal modulation. Given the therapeutic potential of auricular tVNS, there remains a need for the cutaneous map of the ABVN to be further refined and the effects of various stimulation parameters and stimulation sites to be determined.

Health utility scores from EQ-5D and health-related quality of life in patients with esophageal cancer: a real-world cross-sectional study.

Esophageal cancer and its treatment can cause serious morbidity/toxicity. These effects on health-related quality of life (HRQOL) can be measured using disease-specific scales such as FACT-E, generic scales such as EQ-5D-3L, or through symptoms. In a two-year cross-sectional study, we compared HRQOL across esophageal cancer patients treated in an ambulatory clinic and across multiple disease states, among patients with all stages of esophageal cancer. Consenting patients completed FACT-E, EQ-5D, a visual analog scale, and patient reported (PR)-ECOG. Symptom complexes were constructed from FACT-E domains. Responses were categorized by disease state: pre-, during, and post-treatment, surveillance, progression, and palliative chemotherapy. Spearman correlation and multivariable linear regression characterized these associations. In total, 199 patients completed 317 questionnaires. Mean FACT-E and subscale scores dropped from baseline through treatment and recovered during post-treatment surveillance (P < 0.001); EQ-5D health utility scores (HUS) displayed a similar pattern but with smaller differences (P = 0.07), and with evidence of ceiling effect. Among patients with stage II/III esophageal cancer, mean EQ-5D HUS varied across disease states (P < 0.001), along with FACT-E and subscales (P < 0.001). Among patients with advanced disease, there was no significant difference between baseline and on-treatment total scores, but improved esophageal cancer-specific scales were noted (P = 0.003). Strong correlation was observed between EQ-5D and FACT-E (R = 0.73), along with physical and functional subscales. In addition, the association between FACT-E and EQ-5D HUS was maintained in a multivariable model (P < 0.001). We interpret these results to suggest that in a real-world clinic setting, FACT-E, EQ-5D HUS, and symptoms were strongly correlated. Most HRQOL and symptom parameters suggested that patients had worse HRQOL and symptoms during curative therapy, but recovered well afterwards. In contrast, palliative chemotherapy had a neutral to positive impact on HRQOL/symptoms when compared to their baseline pre-treatment state.

Use of autologous fat grafting in reconstruction following mastectomy and breast conserving surgery: An updated systematic review protocol.

INTRODUCTION: Use of autologous fat grafting (AFG) for breast reconstructive surgery is gaining acceptance, but concerns regarding its efficacy and safety remain. We present a protocol for a systematic review that aims to update the findings since our previous systematic review on a number of outcomes of AFG. METHODS: The systematic review has been registered a priori (UIN: reviewregistry308). All study designs, including randomised controlled trials, cohort studies, case-controlled studies and case reports/series, reporting original data, on women undergoing AFG for breast reconstruction following mastectomy or breast conserving surgery, will be included. Six categorical outcomes will be assessed: oncological; clinical; aesthetic/functional; patient-reported; process; and radiological.The search strategy will be devised to investigate 'fat grafting and breast reconstruction'. Electronic databases will be searched, 01 April 2014 to 21 August 2017: PubMed, MEDLINE®, EMBASE, SCOPUS, CINAHL, PsychINFO, SciELO, The Cochrane Library, including the Cochrane Central Register of Controlled Trials (CENTRAL), Database of Abstracts of Reviews of Effect (DARE), the Cochrane Methodology Register, Health Technology Assessment Database, the NHS Economic Evaluation Databases and Cochrane Groups, ClinicalTrials.gov, Current Controlled Trials Database, the World Health Organisation (WHO) International Clinical Trials Registry Platform, UpToDate.com, NHS Evidence and the York Centre for Reviews and Dissemination. Grey literature will be searched. Two trained, independent teams will screen all titles and abstracts, and relevant full texts, for eligibility. Data will be extracted under standardised extraction fields into a preformatted database. ETHICS AND DISSEMINATION: The systematic review will be published in a peer-reviewed journal and presented at national and international meetings within fields of plastic, reconstructive and aesthetic surgery, and surgical oncology. The work will be disseminated electronically and in print. Brief reports of the review and findings will be disseminated to interested parties through email and direct communication. The review aims to guide healthcare practice and policy.

Cataract in children and adolescents with fibrocalculous pancreatic diabetes.

Cataracts have been infrequently reported in Fibrocalculous pancreatic diabetes (FCPD) which is a form of diabetes secondary to chronic, non-alcoholic pancreatitis in tropical countries. A retrospective study was carried out to find out frequency of cataracts in children and adolescents with FCPD admitted in the Paediatric Unit at Bangladesh Institute of Research and Rehabilitation on Diabetes, Endocrine and Metabolic disorders (BIRDEM). A total of 83 patients with FCPD were admitted during the study period of 2000 to 2007. Among them twenty patients (24%) had bilateral cataracts who were enrolled in the study. Mean age at the onset of diabetes was 13.9±1.7 years. There was female preponderance. Ninety percent patients were girls. There was longer duration of symptoms in all patients. Mean duration of symptoms before diagnosis was 15.0±10.2 months. All patients had hyperglycaemia, mean HbA1c value was 18.4±5.1. Most of the patients had snowflake type cortical deposits and posterior subcapsular cataract.

Prevalence of impaired glucose tolerance among children and adolescents with obesity.

The study was undertaken to see the prevalence of impaired glucose tolerance among children and adolescents with obesity, attending the Paediatric Endocrine OPD, Bangladesh Institute of Research and Rehabilitation in Diabetes, Endocrine and Metabolic disorders (BIRDEM), Dhaka, Bangladesh. A cross sectional study from January 2006 to December 2008 was conducted among obese children and adolescents (6-18 years). Children with any other endocrine disorder, dysmorphism/syndrome were excluded. Obesity was defined as BMI ≥ 95th percentile for age and sex using CDC growth chart. Children underwent two hours oral glucose tolerance test with 1.75 gm/kg or 75 gm of glucose, anthropometric and blood pressure measurement. Fasting serum insulin and lipid profile were measured. Impaired glucose tolerance (IGT) was defined as fasting plasma glucose (FPG) <7 mmol/L and 2 hours post glucose load ≥ 7.8 mmol/L to <11.1 mmol/L. Diabetes mellitus (DM) was defined as FPG ≥ 7 mmol/L or 2 hours post glucose load ≥ 11.1 mmol/L. Homeostasis model assessment was used to estimate insulin resistance. A total of 161 children presented with obesity. Male to female ratio was 1.3:1. Mean age was 10.3 ± 2 .5 years. Mean BMI was 27.86 ± 4.1 kg/m². IGT was found in 16.9% of children and adolescents. In children aged 6-10 years IGT was detected in 16.1% and in adolescents aged 11-18 years IGT was detected in 20%. Diabetes mellitus was detected in 2.1% of subjects, all were adolescents. Serum fasting insulin and hip circumference were significantly higher among children and adolescents with IGT compared to that of normal glucose tolerance. The high rate of IGT among obese children and adolescents is of concern. Factors contributing towards obesity needs to be identified and strategies should be planned for prevention and management of this health problem.

Prevalence of obesity among affluent school children in Dhaka.

A cross sectional study was conducted in September 2006 in one of the private schools in Dhaka, Bangladesh to see the prevalence of obesity among affluent school children and adolescents. Informed consent was taken from school authority to take anthropometric measurement of all school children. Standing height was measured with a stadiometer and weight with a bathroom scale. Waist and hip circumference were measured with a measuring tape. The body mass index (BMI) was calculated using weight in kilogram/(height in meter)2 formula. Obesity was defined as BMI≥95th percentile for age and sex, over weight as BMI≥85th percentile for age and sex, normal weight as BMI between 5th and 84th percentile and underweight as BMI <5th percentile. Official centers for disease control (CDC) growth chart for boys and girls age 2-20 years was used. Children and adolescents were divided into group 1(3-5 years), Group 2(6-9years), group 3(10-13 years) and group 4(14-18 years). There were a total of 468 children and adolescents (male 266, female 202). In group 1 there were 110 children, in group 2 there were177 children, in group 3 and 4 there were 149 and 32 adolescents respectively. The prevalence of obesity was 17.9%, higher among males (19.9%), compared to females (15.3%). Obesity was highest (27.7%) in group 2, 14.5% in group 1, 10.7% in group 3 and 9.4% in group 4.

Oral rehabilitation in ectodermal dysplasia.

Ectodermal dysplasia (ED) is an inherited heterogenous group of disorders causing mal formation of all tissues originating from ectoderm, like defect in hair, teeth, nail and sweat gland function. Numerous types have been described. Hypohydrotic ectodermal dysplasia (HED) is one of the common type; characterized by hypohidrosis, hypotrichosis (abnormal hair), onychodysplasia (abnormal nail) and hypodontia or anodontia (abnormal dentition). The significance of this disease lies in severe hypodontia. Dental clinicians can be the first to diagnose such cases. The dental team should be aware of its sign and symptoms in order to provide the correct therapies for the functional and psychologic needs of these patients. Here we have discussed oral rehabilitation of a 10 year old boy with ectodermal dysplasia. The objective of this case report is to make aware the people about such rare genetic disease and different methods of oral Rehabilitation of these patients for better social living, self esteem oral function.

Cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is a disorder that affects mostly those bones which derived from endochrondal and intramembranous ossification, such as the cranium and the clavicles. CCD is present at a frequency of one in one million individuals. Over 500 cases have been reported. A 13 years old boy reported in the Paediatric Endocrinology outpatient department, Bangladesh Institute of Research and Rehabilitation on Diabetes, Endocrine and Metabolic Disorders (BIRDEM) on 6th April 2006 with complaints of not growing according to his age, a wide gap over the head since birth and delayed shedding of primary teeth and also eruption of secondary teeth. The boy had elongated facies with midfacial hypoplasia, depressed nasal bridge with hypertelorism. His anterior fontanelle was open about 3/1.5 cm. He had short clavicle and his shoulders could be easily apposed. Radiological findings showed anterior fontanellle and sutures were open and widened, all decidual teeth were intact and all secondary teeth were impacted beneath the primary teeth and hypoplastic clavicle. He was diagnosed as a case of Cleidocranial Dysplasia on the basis of history, clinical examination and investigation.

Vaginal bleeding with multicystic ovaries and a pituitary mass in a child with severe hypothyroidism.

A seven year and ten months old girl presented with cyclic vaginal bleeding and a huge abdominopelvic mass. She had clinical features of hypothyroidism. The investigation results were consistent with the diagnosis of primary hypothyroidism with precocious puberty. She also had bilaterally enlarged cystic ovaries on CT scan of abdomen and CT scan of brain showed pituitary macroadenoma. After starting treatment with thyroxine, patient became euthyroid and her general condition improved. Treatment with thyroxine alone halted the cyclic vaginal bleeding, led to rapid resolution of the ovarian cysts and regression of the pituitary mass.