Starting from scratch: The primary hyperacidity theory of the cause of pyloric stenosis of infancy

The early observations of those who first described babies with pyloric stenosis (PS) are used as a backdrop for the development of the Inherited Primary Hyperacidity theory of cause. Those early truths, uncomplicated by modern technology, have acted as a springboard for pathogenesis. Hyperacidity, male predominance, family history, self-cure, enhanced appetite, and time-sensitive presentation were all well known to the early pioneers. Any system of pathogenesis must explain all these clinical features. The Inherited Primary Hyperacidity theory does this and is a credible explanation for all the clinical features established by these early pioneers. The evidence which supports a delay in maturation of the negative feedback between gastrin and gastric acidity is presented. Such a phenomenon further supports the Primary Hyperacidity theory.

Assessment of Biochemical Changes Among Covid-19 Cases in a Tertiary Hospital of Bangladesh During Early Pandemic

Background: Corona virus disease 2019 (COVID-19) involves various organs of the body causing several biochemical changes which plays an essential role in estimating the patients’ condition and prognosis, directing treatment, and even evaluating the curative effects. The present study aimed to assess the biochemical changes among the COVID-19 patients during early pandemic. Methods: This cross sectional study was conducted at Combined Military Hospital (CMH) Dhaka among purposively selected 237 confirm COVID-19 cases. Data were collected through face to face interview and review of medical records using a pre-tested semi-structured questionnaire. The study was conducted in the Combined Military Hospital Dhaka from 15 April 2020 to 31 May 2020 Results: Highest number of the respondents were in the age group of 31-40 years (37.1%) with male predominance (83.1%). About 95.6% were Muslim and 58.6% were educated up to secondary level. About 87.8% had contact with a confirmed case and having 47.7%, 37.2%, 15.1% cardiovascular, endocrine and respiratory comorbidities respectively. Fever (34.6%) was the most common presenting symptoms followed by cough (22.9%), sore throat (10.6%). Neutrophilia observed in 26.16%, lymphopenia in 20.7%, thrombocytopenia in 14.3% cases, 30.0% with positive D-dimer test, 22.4% either sepsis or systemic infection in procalcitonin estimation, 28.3% with increased ferritin, 28.7% with positive C reactive protein, 21.1% with increased LDH. Chi-square analysis revealed a statistically significant association between cardiovascular and endocrine diseases with several biochemical changes (p<0.05). Conclusion: Early identification of various biochemical changes would help the physician for appropriate assessment and management. JOPSOM 2021; 40(1): 34-42

Clinicopathologic Features of Lymphoproliferative Neoplasms Involving the Liver

Background and Objectives: Primary hepatic lymphoproliferative neoplasms (PHL) are uncommon. This retrospective study is aimed to present the clinicopathological characteristics of PHL and compare to secondary hepatic lymphoproliferative neoplasms (SHL). Materials and Methods: Patients who were diagnosed with lymphoproliferative neoplasms involving the liver between January 2004 and December 2018 at a tertiary medical center in central Taiwan were included. The demographic and clinical data, radiological results and histopathological findings were reviewed and summarized. Results: We analyzed 36 patients comprising 6 PHL patients and 30 SHL patients. The median age at diagnosis tended to be younger in PHL than in SHL (59 vs. 63 years old, p = 0.349). Both entities had a small male predominance. The PHL patients tended to have higher levels of aspartate aminotransferase, alanine transaminase and serum albumin and lower levels of alkaline phosphatase, total bilirubin, γ-glutamyl transferase and lactate dehydrogenase compared with SHL, but there was no significant difference. Multiple mass lesions were the most common radiological finding in both groups. Diffuse large B-cell lymphoma was the predominant subtype in both groups (67% in PHL and 40% in SHL). The PHL patients had a longer median survival than the SHL patients (not reached vs. 3 months, p = 0.003). Conclusions: Although there was no significant difference between PHL and SHL in clinical, laboratory and radiological features, the SHL patients had very poor outcomes with a median survival time of 3 months. Effective therapies are urgently required for these patients.

Use of Suture-Mediated Closure Devices for Closure of Punctures in Prosthetic Patches or Grafts is Associated With High Rates of Technical Success and Low Complication Rates

Objectives Despite the increasing use of endovascular techniques in the management of peripheral vascular disease, there is little data on the safety of percutaneous closure devices in punctures of synthetic vascular material. Our paper sought to address this paucity in the literature by reviewing the incidence of complications occurring in patients in whom the ProGlide device was utilised to achieve haemostasis post-percutaneous puncture of vascular patches and graft materials. Methods A retrospective review of patient records was conducted at a tertiary referral centre. Patients who had undergone percutaneous punctures of prosthetic bypass grafts or patch angioplasties between January 2011 and December 2020 were identified from a prospectively collected database. Medical records and post-procedural imaging were reviewed to assess the occurrence of post-procedural complications such as pseudoaneurysms, puncture site stenosis and further interventions for access complications. Results A total of 73 punctures of prosthetic material were performed in 42 patients, of which 39 utilised ProGlides. Median age of included patients was 72 years. There was male predominance in the cohort (69.8%), and most punctures (87.3%) were through polyurethane patches. Device success rate was 95%, and no patients required open repair. There was a low incidence of complications, with no patients developing pseudoaneurysms, arteriovenous fistulas, ischaemic limbs or > 50% stenosis when either manual pressure or the ProGlide device was used to achieve haemostasis. Furthermore, there were no returns to theatre or further interventions performed for access site complications. Conclusion The use of the ProGlide closure device has a low incidence of complications and its safety appears to be equivalent to manual compression when used to achieve haemostasis in percutaneous punctures of synthetic vascular material in select patients. To our knowledge, this is the only article to date to assess the safety of the ProGlide in this setting.

Clinical features of anaphylaxis in children

Background: Despite the considerable increase in anaphylaxis frequency, there are limited studies on clinical features of anaphylaxis in children in developing countries. Objective: We aimed to analyze the demographic and clinical features of anaphylaxis in children in Turkey by comparing different age groups and triggers. Methods: Medical records of 147 children, ages 0‐18 years, diagnosed with anaphylaxis between 2010 and 2019 were retrospectively analyzed. Results: The mean ± standard deviation age at first anaphylaxis episode was 5.9 ± 5.2 years, with a male predominance (63.9%); 25.2% were infants and 52.4% were < 6 years of age at their first anaphylaxis episode; 78.2% were atopic, with the highest frequency in children with food-induced anaphylaxis (FIA). The home (51.7%) was the most frequent setting. The overall leading cause of anaphylaxis was food (44.2%), which was more frequent at < 6 years of age, followed by drugs (28.6%) and bee venom (22.4%), both were more frequent among older children (>6 years). The patients with venom allergy had the highest rate of rapid onset of symptoms (p < 0.001). Gastrointestinal symptoms were observed significantly more in infants (48.6%) and in children with FIA (38.5%); cardiovascular symptoms were more frequently observed in children > 6 years of age (48.6%) and in children with drug-induced anaphylaxis (64.3%). Although recurrent anaphylaxis was reported for 23.1% of the patients, it was highest in the patients with FIA (35.9%). Overall, only 47.6% of the patients received epinephrine in the emergency department (ED) and 27.3% were referred to an allergy specialist, with the patients with FIA having the lowest rate for both, 32.3% and 10.8%, respectively. Children with drug-induced anaphylaxis had the highest rate of severe anaphylaxis (57.1%). Conclusion: There is a need to improve anaphylaxis recognition and management in all children regardless of age and trigger. Inadequate treatment was most evident in infants and patients with FIA.

Factors affecting clinical manifestation of chromosomal imbalance in carriers of segmental autosomal mosaicism: differential impact of gender

Mosaicism for unbalanced chromosomal rearrangements segmental mosaicism (SM) is rare, both in patients referred for cytogenetic testing and in prenatal diagnoses. In contrast, in preimplantation embryos SM is a frequent finding and, therefore, is even more challenging. However, there is no consistency among results of published studies on the clinical outcomes of embryos with SM, primarily due to the small number of reported cases. Moreover, there is the problem of predicting the potential for the optimal development of a mosaic embryo to a healthy individual. Therefore, we suggested comparing factors predisposing to favorable and poor prognoses, identified in postnatal and prenatal cohorts of SM carriers, with those obtained from studies on preimplantation embryos. We analyzed 580 published cases of SM including (i) postnatally diagnosed affected carriers, (ii) clinically asymptomatic carriers, (iii) prenatally diagnosed carriers, and (iv) miscarriages. We observed a concordance with preimplantation diagnoses regarding the clinical significance of the extent of mosaicism as well as a predominance of deletions over other types of rearrangements. However, there is no concordance regarding excessive involvement of chromosomes 1, 5, and 9 in unbalanced rearrangements and a preferential involvement of larger chromosomes compared to short ones. Paternal age was not found to be associated with SM in postnatally disease-defined individuals. We have identified maternal age and preferential involvement of chromosome 18 in rearrangements associated with clinical manifestations. Male predominance was found among normal pregnancy outcomes and among disease-defined carriers of rearrangements resulting in a gain of genomic material. Female predominance was found among abnormal pregnancy outcomes, among disease-defined carriers of loss and gain/loss rearrangements, and among transmitting carriers of gonadal SM, both affected and asymptomatic. According to data obtained from “post-embryo” studies, clinical manifestations of chromosomal imbalance are associated with a high proportion of abnormal cells, female gender, the type of rearrangement and involved chromosome(s), and maternal age. We believe these data are instructive in the challenging medical genetic counseling of parents faced with no option other than transfer of an embryo with segmental mosaicism.

Evaluation and management of voice disorders: Our experiences

Background: Change in voice is one of the most common complains among patients visiting to ENT outpatient. The causes are numerous and need to be evaluated before approaching to curative intent of treatment. Aims and Objectives: The current study was designed with an aim to analyze the spectrum of voice disorders and their management option. Materials and Methods: Prospective study conducted between June 25, 2020, and November 30, 2021. Clinical, demographic profiles were recorded. Fiber-optic laryngoscopy was performed in all the cases. Radiology examination computed tomography/magnetic resonance imaging was supplemented only in required cases. Treatment was executed based of etiological profile analysis. Minimum 3 months follow-up was collected post-therapy. Statistical analysis was performed using Statistical Package for the Social Sciences version 24. Pearson Chi-square test was used for see the association between parameters. P-value was considered significant while being <0.05. Results: Out of 218 patients, the most patients (approx. 70%) occupied in the age group of 30–50 years. There was male predominance (76.6%). Voice change secondary to laryngopharyngeal reflux was seen in 56.4% of cases. Benign vocal fold lesions (nodule/cyst/polyp) were noticed in 26.5% of cases. Malignant lesions were seen in 1.8% of cases. Benign vocal fold lesions (polyp and cyst) were treated by microlaryngeal surgery (MLS). Pre-malignant lesion (leukoplakia) was treated with MLS stripping. Out of four malignant lesions, one was in early stage and underwent supraglottic laryngectomy while others were in advanced stage (T4) and treated by total laryngectomy. Conclusions: Voice disorders comprise wide etiological profile from reflux to malignant lesion. Timely proper evaluation followed by definitive management achieves good treatment outcomes.

EXPERIENCE OF SEVERE ACUTE RESPIRATORY SYNDROME CORONAVIRUS-2 (SARS-COV-2) - COVID-19 AT A TERTIARY CARE HOSPITAL IN QUETTA, BALUCHISTAN

Objective: To share the experience of Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2)-COVID-19 at a tertiary care hospital in Quetta, Baluchistan. Study Design: Cross-sectional study. Place and Duration of Study: Department of Pathology, Combined Military Hospital Quetta, from Mar to Dec 2020. Methodology: This study included 14,741 study participants presenting to CMH Quetta with signs and symptoms of Coronavirus Disease-19 (COVID-19) and others undergoing COVID-19 PCR for screening purpose. Nasopharyngeal swab collected from these study participants were tested for COVID-19 viral RNA by real-time Reverse Transcription Polymerase Chain reaction (RT-PCR) assay. Results: Out of these 14,741 study participants, 1886 (12.7%) were found to be SARS-CoV-2 PCR positive. Among 1886 study participants, 1503 (80%) were males while 383 (20%) were females. Mean age of the study participants was 36 ± 14 years. Most frequent clinical presentations were body aches (96.5%), fever (94.1%), cough (66.8%) and loss of appetite (68.2%). Around 67 (3.5%) positive study participants were asymptomatic. Conclusion: In this study, we observed male predominance but severity of signs and symptoms among female study participants. SARS-COVID-19 caused disease with wide range of clinical spectrum and disease can be fatal as well.

Histopathological Spectrum of Non-neoplastic and Neoplastic Lesions of Urinary Bladder

Introduction: Urinary bladder is a site of various non-neoplastic and neoplastic lesions, both of which can present with hematuria. Cystoscopy allows for direct visualization of the bladder mucosa and also obtaining tissue for histopathologic evaluation. The most common non-neoplastic and neoplastic lesion of the urinary bladder is non-specific cystitis and urothelial carcinoma respectively. Materials and methods: This study is a 4-year retrospective study conducted in the Department of Pathology at Patan Hospital, Nepal. All cases from the urinary bladder i.e., both cystoscopic and cystectomy samples were included in the study. Hematoxylin and eosin-stained slides were re-evaluated whenever required. Results: A total of 145 cases were included which consisted of 17 cystectomy specimens and 128 cystoscopic biopsies. There was a male predominance. The non-neoplastic and neoplastic cases consisted of 32% and 68% respectively. Chronic non-specific cystitis and high-grade infiltrating urothelial carcinoma were the most common non-neoplastic and neoplastic lesions respectively. 56.1% of cystoscopic biopsies had the presence of detrusor muscle with 21.7% showing its invasion. Conclusions: Urinary bladder lesions have a wide spectrum ranging from non-neoplastic to neoplastic conditions. The presence of detrusor muscle in a cystoscopic biopsy, and its evaluation for invasion helps in diagnosis and further planning of patient management.

EVALUATION OF THE VALIDITY OF HEMOCUE HB 201+ FOR PREDONATION ASSESSMENT OF LOW AND HIGH HAEMOGLOBIN LEVELS IN BLOOD DONORS: ONE YEAR PROSPECTIVE STUDY IN BLOOD BANK OF GMC DODA

Introduction: Predonation assessment of haemoglobin in blood donors is important to avoid unnecessary rejection of potential blood donors. Compared to the conventional screening methods for haemoglobin assessment the method to be used in blood banks needs to be rapid, sensitive and accurate for both low and high haemoglobin values. Materials and methods: This was a prospective study conducted in blood bank of GMC Doda w.e.f 1st April 2020 to 31st March 2021. Finger prick samples were taken from 1214 potential blood donors and hemoglobin was estimated using HemoCue Hb 201+. Also venous samples of potential blood donors were obtained and run on automated haematologyanalysers. The values were compared. Male donors with haemoglobin in the range of 12.5g/dl -18.5g/dl and females with hemoglobin in the range of 12.5g/dl -16.5g/dl were considered fit for donation. Results: We analyzed that haemoglobin levels measured by HemoCue Hb 201+ were higher than those measured by automated haematologyanalyser. We found a significant correlation between the standard method and HemoCue Hb 201+ for haemoglobin levels less than 12.5 g/dL. However the correlation was found to be less for high haemoglobin levels >18.5g/dl. Sixty percent of donors, who had haemoglobin levels higher than upper limit were unnecessarily rejected accounting for high deferral rate. Conclusion: We concluded that HemoCue Hb 201+ was suitable for screening for low haemoglobin in blood donors. Male predominance among blood donors and topography of this particular area accounts for donors with haemoglobin values near upper limit which were falsely deferred on the basis of HemoCue Hb 201+. Also testing with this method was found to be expensive, so it was recommended that qualitative methods should be used for primary screening among blood donors and accurate quantitative methods to be used only when qualitative methods fail.