A Case of Coombs-Negative Primary Warm Autoimmune Hemolytic Anemia Due to IgA Antibody That Responded Well to Rituximab and Steroids.

Warm autoimmune hemolytic anemia (WAIHA) occurs due to antibodies active at body temperature that react with antigens on the surface of red blood cells, leading to hemolysis. Antibodies are typically IgG. WAIHA, associated exclusively with IgA antibodies, remains rare. Direct antiglobulin (Coombs) test may result negative in IgA antibody associated WAIHA. IgA-mediated WAIHA can present with severe hemolytic anemia. Further testing using an expanded direct antiglobulin test (DAT) panel is necessary to detect IgA antibodies if there is a high suspicion of autoimmune hemolytic anemia in cases that initially test negative for DAT. Steroids with or without rituximab are the mainstay of treatment. Early detection using an extended DAT panel with monospecific antisera helps avoid further investigations, unnecessary transfusions, and complications.

Catheter-associated Bloodstream Infection in Children with Tunneled Central Venous Catheters: A Single-center Experience.

Background: Tunnelled catheters improve venous access in haematological diseases and malignancies, but are associated with complications. We prospectively analysed the episodes of CABSI and its associated risk factors. Aims: To study the incidence and risk factors for CABSI in children with tunnelled central venous catheters (both Hickmans and Chemoports). Materials and Methods: This is a prospective observational study done at our institute. Children under the age of 18 who underwent insertion of a Hickman or Chemoport from March 2018 to Dec 2022 were enrolled. Episodes of CABSI were noted and its risk factors were analysed. Results: In total, 258 catheters were inserted in 250 children. Age ranged from 1 month to 18 years (median 67 months) with 60% being boys. A total of 152 Hickmans, 106 chemoports were inserted. Indications for insertions were for requirement of BMT and chemotherapy in majority of cases. CABSI were seen in 28.6% of catheters. Younger children (< 4years), Neutropenia (counts < 1000) and use of TPN were significantly associated with CABSI(p value<0.05). Infection was more with externalized catheters (Hickman) than implantable ports (Chemoports) but was not statistical significant(p value>0.05). Almost 30% of catheters with CABSI required removal because of florid sepsis. Others could be salvaged with aggressive antibiotics. Conclusions: CABSI is a serious complications related to tunnelled catheter. Smaller children (<4 years), neutropenia and usage of TPN is a risk factor for development of CABSI. It can be treated with appropriate antibiotics and required removal in almost a third of all the cases.

A Case of Prolonged Wernicke's Encephalopathy After Treatment With IV Thiamine Due to the Subsequent Development of Refeeding Syndrome.

Wernicke's encephalopathy (WE) is a rare, life-threatening condition in which thiamine deficiency causes dysfunction of the Kreb's cycle, accumulation of lactic acid in the brain tissues, and irreversible cognitive impairment. Prompt treatment with IV thiamine can reverse the process. The classic Wernicke's triad of ataxia, memory issues, and ocular abnormalities is not often present. Caine's criteria, which requires two of the following: dietary deficiencies, ocular abnormalities, altered cognition or mental status, and cerebellar dysfunction, is highly sensitive and specific for Wernicke's diagnosis, especially in patients with alcohol use disorder. Refeeding syndrome (RS) has similar risk factors to WE, including disease states that lead to malnutrition. Patients with RS develop WE due to thiamine depletion that occurs when oral nutrition is reinitiated after a period of poor oral intake. We present a patient with initially undetected WE who developed RS after the initiation of treatment with IV thiamine. RS prolonged the neurologic symptoms of WE and led to an extended hospital stay and significant physical debility. In our patient, WE preceded RS instead of occurring as a consequence of it. The case highlights that if one of these disorders is present, the other may not be far behind. When WE precedes RS, prolonged treatment with IV thiamine may be warranted until the symptoms of both disorders resolve.

Transplanting Livers in Young Children - Looking Back at 100 Cases.

Introduction: Despite advances in medical therapy, liver replacement continues to be the only definitive mode of therapy for children with end-stage liver disease (ESLD). However, its acceptance in India has been discouraging more due to financial and logistic reasons than the availability of expertise. This report outlines our journey and highlights issues pertinent to circumstances in an emerging economy like India. Aim: The aim is to review a single center's 100 case experience with liver transplantation (LT). Materials and Methods: A prospective analysis of all children who underwent LT since 2005 at our institute was done. The data were collated from a maintained structured database. Results: Hundred children underwent LT. Sixty-four were boys. Age ranged from 5 to 144 months, with a median of 17 months. The mean weight of the cohort was 7.5 kg (ranging from 3.7 to 31.5 kg), with 60% of our children weighing between 5 and 10 kg. Biliary atresia is the most common indication (57%); others include metabolic disorders, progressive familial intrahepatic cholestasis, and hepatoblastoma. Two patients were for acute liver failure. Ninety-one children underwent live donor LT (mothers being the majority of the donors). None of the donors had any major postoperative complications. Major intraoperative complications include sepsis (39%), vascular complications (17%), biliary leak (11%), and intestinal complications (11%). Early postoperative deaths occurred in 18% of patients with sepsis being most common cause. The mean follow-up was 8.5 years and the overall survival is 70%. The mean survival is time (months) = 176.1. All surviving patients were followed up and had achieved good catch up growth by 3rd and 5th years posttransplant. The long-term sequalae include recurrent intercurrent infections, graft rejection (9%), posttransplant lymphoproliferative disease (5%), and portal vein stenosis (5%). Conclusion: Our experience demonstrates the feasibility of LT in children with ESLD in India. With longer-living grafts, patients often struggle with other issues such as compliance with follow-up, financial issues, recurrent infections, and neurological problems. Close monitoring with regular follow-up of patients helps in early recognition and treatment of late-onset complications, thus helping the overall long-term outcomes.

Mental Health of Frontline Nurses in India During COVID-19: A Multisite Study.

Background  The COVID-19 pandemic has been difficult for all healthcare providers. Nurses in Indian hospitals are at risk for mental health consequences of COVID-19-related stress. The study aimed to evaluate the mental health responses of Indian nurses working during the COVID-19 pandemic.  Method The study was carried out during the COVID-19 pandemic from November 2020 to February 2021. Frontline nurses (n=387) working in both government and private sectors were recruited from four hospital centers across Mangalore, India. Nurses were selected based on specific inclusion criteria, including active duty within wards and intensive care units designated for COVID-19 care or suspected cases of SARS-CoV-2 infection. Recruitment and data collection were facilitated by medical residents using a mix of physical and electronic survey methods. Results Nurses within the private sector with low personal protective equipment (PPE) security experienced heightened anxiety. Somatic symptoms were incrementally related to mental health depending on the workplace setting; private sector staff reported greater depression symptoms compared to those in government-run hospitals. Self-efficacy buffered against depression outcomes only in nurses within the private sector working within non-COVID units. Conclusions This study's findings showed differential responses to the stress of COVID-19 based on the setting. Future studies should further explore the factors associated with such differences. Somatic symptoms can be indicators of mental health adversity. Early detection and supportive interventions need to be taken into account.

Redo Surgeries in Anorectal Malformations: A Single-center Experience.

Aims and Objectives: Complications following posterior sagittal anorectoplasty (PSARP) for ARM are well known. In this article, we present our experience of managing five patients who required major redo surgeries for complications resulting from previous attempts to correct ARM. Materials and Methods: We reviewed all patients who underwent major redo surgeries in our hospital for complications from previous repairs for ARM, from June 2013 to June 2019. Data was obtained from hospital records and analysed. Results: Five patients whose ages ranged from 5 months to 14 years were included in the study. Four were boys and 1 was a girl. All patients had undergone PSARP in other hospitals. The presentations were retained distal bowel causing urinary retention and constipation (n=1), pulled through proximal urethra and bladder neck presenting as passage of urine from neo-anus (n=1), retained common channel (of cloaca) causing a 'H' type configuration (n=1), mispositioned neo-anus (n=1) following a primary PSARP and lastly undivided recto-urethral fistula causing fecaluria (n=1). All of them underwent redo repairs by posterior sagittal approach with documented improvement in their symptoms. Two of them required total bowel management to remain clean. Conclusion: All the complications reported here have been described in literature nevertheless, this report will add to the body of experience. Posterior sagittal approach (PSA) has proved to be very successful technique in correcting these complications.

Sestamibi-guided thoracoscopic excision of functional mediastinal parathyroid adenoma in a child: A case report and review of literature.

Primary hyperparathyroidism secondary to ectopic parathyroid (in anterior mediastinum) is rare in children. We report the case of a 12-year-old girl with a history of multiple fractures, renal calculi and limb deformities. She was diagnosed with hyperparathyroidism secondary to an intrathymic parathyroid adenoma. Sestamibi scan showed a lesion in the anterior mediastinum. A biochemical evaluation revealed hypercalcaemia, elevated alkaline phosphatase and parathormone levels. The lesion was marked with radioisotope and confirmed intraoperatively using a gamma camera. The child underwent thoracoscopic left thymectomy with the adenoma. Immediate decrease in calcium and parathyroid hormone values were noted intraoperatively and serial monitoring showed a downward trend. On follow-up, the child is doing well. Ectopic parathyroid adenoma is very rare. CT with radioisotope scans is helpful in diagnosis. Thoracoscopic excision of ectopic adenoma is safe in children.

A Multi-armed Unfurling Actuator for Airway Lumen Measurement.

Objective measurement of the lumen area demands an intraoperative diagnostic tool to aid on-site decision-making. We present a compliant mechanism-based unfurling actuator assembly integrated with a shaft connected to a motorized encoder to translate torque from the user at the proximal end to the actuator at the distal end. The actuator assembly has flexible arms coiled inside a cylindrical casing that moves radially outward upon actuation. Leveraging 3D printing of flexible materials, the unfurling actuator's four-arm design enables patency measurements in circumferential tracheal stenosis of varying grades. The rotary encoder output is correlated with the radially outward movement of the unfurling arms to estimate the lumen diameter. The measurement stability is analyzed using process control charts; data distribution over ten iterations reveals nearly 100% of process data falls between ±3 sigma (Upper and Lower control limits). Comparing measurements from the tool with direct measurement (vernier caliper) and ImageJ analysis, one-way ANOVA for circular morphology yields no significant differences in diameter p = 0.974 and area measurements p = 0.975.Clinical Relevance- Central airway narrowing reduces the effective lumen area in the tracheal and bronchial segments. Grading the degree of narrowing is often based on a suspicion index. A quick but thorough assessment of the airway caliber is essential in emergent or planned intubation, whether congenital, iatrogenic, or idiopathic tracheal stenosis.

A Right-Sided Approach to Anterior Communicating Artery Aneurysms: A Case Review and Technical Report.

Anterior communicating artery (ACoA) aneurysms are the most frequently encountered type of intracranial aneurysm. ACoA aneurysms may require treatment depending on clinical presentation, size, risk of rupture, and ruptured status. In patients where treatment is indicated, options entail endovascular securement or clipping. Under the clipping umbrella, surgical approaches traditionally entail a pterional craniotomy and its modifications such as the lateral supraorbital approach. Sidedness of this craniotomy has been a topic of debate. To discuss this we present a case and technical report with nuances of the approach wherein a 48-year-old female presented with the worst headache of her life. The patient was found to have a ruptured wide-necked 7.2 x 8.1 x 5.8 mm ACoA aneurysm more eccentric to the left and fed from the left A1 intertwined with a frontopolar branch, numerous perforators and the recurrent artery of Heubner. The patient underwent a successful clipping from a right-sided approach. As such, with appropriate skull base drilling, exposure, optimization of brain relaxation, and a generous opening of the Sylvian fissure bilateral internal carotid arteries, anterior cerebral arteries with both A1 and A2 segments, middle cerebral arteries, the ACoA, and the relevant anatomy can be appropriately visualized from a right-sided approach. Therefore, an approach is described to optimize exposure to allow for nearly all anterior communicating aneurysms to be clipped from a right-sided pterional approach.

Laryngeal Cyst in Children : A Retrospective Analysis.

Aim: To study the presentation, management and outcome of laryngeal cysts in children. Materials and methods: This is a retrospective observational study of children with Laryngeal cysts who were managed in the Department of Paediatric Surgery between April 2015 to Jan 2022. Observations and Results: Eight children with laryngeal cysts were included, 5 were girls. Age ranged from 3 days to 10 years (median 5 months). Weight ranged from 3kg to 40kg (median 6.3 kg). All presented with stridor. Three children had co-morbid conditions, one each with Leucocyte adhesion deficiency type -1, atrial septal defect & PHACES syndrome. Two children had severe respiratory distress and required tracheostomy at presentation. All children underwent MLB. The cysts were 2 subglottic cysts (one inflammatory cyst) and 6 vallecular cyst. Five underwent endoscopic excision of cysts, 1 underwent excision with lateral cervical approach, 1 underwent endoscopic de-roofing of cyst and airway reconstruction with anterior costal cartilage graft (associated Sub glottis stenosis). Conclusion: Laryngeal cysts are a rare cause of stridor in children. MLB is the best diagnostic tool. Treatment with excision / deroofing of the cyst endoscopically, is safe and effective in paediatric age group. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03495-w.

Paraprofessional delivery of online narrative exposure therapy for firefighters.

Firefighters are at increased risk for developing posttraumatic stress disorder (PTSD) and face numerous barriers to accessing mental health care. Innovative ways to increase access to evidence-based interventions are needed. This study was a case series testing the acceptability, feasibility, and preliminary effectiveness of a paraprofessional-delivered, virtual narrative exposure therapy (eNET) intervention for PTSD. Participants were 21 firefighters who met the criteria for clinical or subclinical probable PTSD and completed 10-12 sessions of eNET via videoconference. Participants completed self-report measures pre- and postintervention and at 2- and 6-month follow-ups as well as a postintervention qualitative interview. Paired samples t tests evidenced statistically significant decreases in PTSD, anxiety, and depressive symptom severity and functional impairment from pre- to postintervention, ds = 1.08-1.33, and in PTSD and anxiety symptom severity and functional impairment from preintervention to 6-month follow-up, ds = 0.69-1.10. The average PTSD symptom severity score fell from above to below the clinical cutoff for probable PTSD at postintervention and follow-ups. Qualitative interviews indicated that paraprofessionals were considered central to participants' success and experience with the intervention. No adverse events or safety concerns were raised. This study is an important step in demonstrating that appropriately trained and supervised paraprofessionals can effectively deliver eNET to firefighters with PTSD.

Kasai Portoenterostomy at a Slightly Delayed Age and Native Liver Survival in Children With Biliary Atresia: Single Center Experience.

OBJECTIVES: We studied the modifiable prognostic factors that extend native liver survival at 2 years after Kasai portoenterostomy (KPE). METHODS: We reviewed hospital records of patients with neonatal cholestasis, with focus on infants diagnosed with biliary atresia in a tertiary care hospital between January, 2014 and May, 2021. We determined the association of outcome with clinical and laboratory variables. RESULTS: Infants who underwent KPE at a median (IQR) age of 76 (72-79) days had best outcomes, with minimal severe post-KPE complications and 2-year survival rate of 84.6%, compared to other infants (younger and older days at KPE). The median (IQR) weight at KPE in this group was 4.66 (4.2, 5.0) kg. CONCLUSION: In contrast to traditional recommendations, babies with median age at KPE of 76 days had superior native liver survival (84.6%) and reduced post-KPE complications, as compared to earlier KPE age. Nutritional status and weight of infant at KPE could be associated with this survival difference. This observation needs confirmation through multicentric prospective study.

Risk Factors Associated With Recurrence and Death in Patients With Tall Cell Papillary Thyroid Cancer: A Single-Institution Cohort Study With Predictive Nomogram.

Importance: Tall cell morphology (TCM) is a rare and aggressive variant of papillary thyroid carcinoma (PTC) that has been associated with poor outcomes; however, the risk factors for worse survival are not well characterized. Objective: To identify prognostic factors associated with cancer recurrence and death in patients with PTC-TCM. Design, Setting, and Participants: All patients treated for PTC-TCM at a single tertiary-level academic health care institution from January 1, 1997, through July 31, 2018, were included. Tall cell variant (TCV) was defined as PTC with TCM of 30% or more; and tall cell features (TCF) was defined as PTC with TCM of less than 30%. Patients with other coexisting histologic findings and/or nonsurgical management were excluded. Clinicopathologic features associated with worse outcomes were identified using Kaplan-Meier and Cox proportional-hazards model. Data were analyzed from March 1, 2018, to August 15, 2018. Main Outcomes and Measures: Locoregional recurrence-free survival (LRRFS), distant recurrence-free survival (DRFS), and overall survival (OS) after surgery. Results: A total of 365 patients (median [range] age, 51.8 [15.9-91.6] years; 242 [66.3%] female) with PTC-TCM (TCV, 32%; TCF, 68%) were evaluable. Total thyroidectomy was performed in 336 (92%) patients; 19 (5.2%) received radiotherapy; and 15 (4.1%) received radioactive iodine. Clinical features were pT3 or T4, 65%; node-positive, 53%; and positive surgical margins, 24%. LRRFS at 1-, 3-, 5-, and 10-year was 95%, 87%, 82%, and 73%, respectively. On multivariable analysis, male sex and age were not independent predictors of inferior 5-year LRRFS, whereas positive surgical margins (HR, 3.5; 95% CI, 2.0-6.3), positive lymph nodes (HR, 2.8; 95% CI, 1.4-5.8), and primary tumor size of 3 cm or more (HR, 3.3; 95% CI, 1.4-7.8) were strongly associated with worse LRRFS. Age 55 years or older (HR, 3.2; 95% CI, 1.5-7.0), male sex (HR 4.5; 95% CI, 2.1-10.0), positive surgical margins (HR, 2.7; 95% CI, 1.2-6.0), nodal positivity (HR, 3.1; 95% CI, 1.3-7.7), tumor diameter of 1.5 cm or more (HR, 20.6; 95% CI, 2.8-152.1), and TCV vs TCF (HR, 3.1; 95% CI, 1.5-6.7) were associated with worse DRFS. Male sex (HR, 3.1; 95% 1.4-6.8) and tumor diameter of 1.5 cm or more (HR, 2.8; 95% CI, 1.0-7.4) were associated with worse OS. A findings-based nomogram was constructed to predict 10-year LRRFS (C index, 0.8). Conclusions and Relevance: This retrospective cohort study found that in patients with PTC-TCM, positive surgical margins, node positive disease, and tumor size of 3 cm or more were risk factors for worse LRRFS. Intensified locoregional therapy, including adjuvant radiation, may be considered for treating these patients.

Chest CT Findings in Patients with HIV Presenting to the Emergency Department: A Single Institute Experience.

PURPOSE: The aim of this study was to analyze chest CT imaging findings and relevant clinical factors in patients with HIV presenting to the emergency department (ED). MATERIALS AND METHODS: A retrospective review was performed to identify patients with HIV who received chest CT imaging evaluation in the acute ED setting. Analyzed patients included adults with a known diagnosis of HIV who presented to the ED at a single tertiary care center between 2004 and 2020 and received chest CT imaging. Chest CT findings were assessed by 2 radiologist readers, and relevant clinical data were gathered. Statistical analysis was performed to determine if imaging and clinical factors demonstrate significant associations with CD4 count, viral load, and antiretroviral therapy status. RESULTS: A total of 113 patients with HIV were identified who presented to the ED and underwent chest CT imaging evaluation (mean age 47 ± 11 years). Frequently detected chest CT findings included infectious pneumonia (24%), malignancy (11%), pleural effusion (17%), pericardial effusion (13%), and pulmonary embolism (4%). CD4 count, viral load, and active retroviral therapy demonstrated statistically significant associations with a number of key imaging and clinical factors, including presence of pneumonia, malignancy, average length of hospital admission, and survival. CONCLUSION: Patients with HIV present with a wide range of imaging findings when presenting in the acute ED setting. CD4 count, viral load, and active retroviral therapy status demonstrate statistically significant associations with multiple key imaging findings and clinical factors. Chest CT plays an integral role in the clinical management of this unique patient population.

Evaluating the Downstream Revenues of a Self-Pay Bi-Parametric Prostate MRI Program.

OBJECTIVE: To quantify downstream healthcare utilization and revenue associated with a self-pay bi-parametric prostate MRI (bpMRI) program. METHODS: Medical records of 592 patients who underwent bpMRI between August 2017 and March 2020 were examined for follow-up clinical activities. These include prostate biopsy, radical prostatectomy, external beam radiation therapy, brachytherapy, androgen deprivation therapy, CT Chest, Abdomen and Pelvis, PET/CT, MRI Pelvis, and Nuclear Medicine Bone Scans. The charges for each clinical activity were derived from the Medicare Physician Fee Schedule to conservatively estimate revenues. This patient population was further divided into four groups: Group A, patients who demonstrated an MRI lesion and underwent prostatectomy; Group B, patients who did not demonstrate lesion but underwent prostatectomy; Group C, patients who demonstrated lesion but did not undergo prostatectomy; and Group D, patients who neither demonstrated lesion nor underwent prostatectomy. Revenues for each group were categorized by Urology, Radiation Oncology and Radiology subspecialties. RESULTS: Conservative estimates yielded $520 of downstream revenue per patient who underwent bpMRI. Group A patients yielded 47% of total revenue ($1974 per patient). Group B patients, the smallest group, yielded $1828 per patient. Group C patients made up the largest group and grossed $398 per patient. Group D demonstrated the lowest per patient revenue of $179. Groups A and B yielded most relative revenue for Urology. Group C yielded most relative revenue for Radiation Oncology, and Group D yielded most relative revenue for Radiology. CONCLUSION: A self-pay bpMRI program has the potential to improve patient access to prostate cancer screening while remaining financial sustainable.

Mid-facial soft tissue re-suspension following skeletal fixation after maxillofacial trauma-a prospective case series study.

PURPOSE: Open reduction and internal fixation (ORIF) is used for reduction and stabilization of facial fractures. Despite perfect anatomic skeletal reduction, subtle asymmetries are seen in facial appearance as mid-facial soft tissue often reattaches to the respective facial bones in an aberrant, mostly lower in position. Thus, the present study evaluates the effectiveness of mid-facial soft tissue re-suspension procedure to prevent changes in mid-facial soft tissue profile after ORIF of maxillo-facial fractures. METHODS: Fifteen patients with maxillo-facial fractures requiring ORIF were included. After ORIF, mid-facial soft tissue resuspension (STRP) was done by suspending the soft tissue and periosteum over cheek region from temporal fascia by polydioxanone suture material. The results were analyzed by patient satisfaction survey and doctor perspective form on photographs by three surgeons 6 months postoperatively. RESULTS: All patients were male in the age range of 18-60 years. STRP was done unilaterally in 7 cases and bilaterally in 8 cases, with average time ranging from 10.6 to 20.5 min. The patient satisfaction survey revealed that 86.6% were pleased with their appearance and 93% said that there was no abnormal change in the esthetics postoperatively. The doctor perception form revealed almost 80% agreement, that there was minimal change in the appearance of cheek mound, nasolabial fold, corner of the mouth, and lateral canthus postoperatively. CONCLUSION: STRP is a simple technique to perform with fewer complications, and helps in restoring facial esthetics, same as prior to facial trauma; as it prevents soft tissue sagging after degloving incisions for fracture reduction and fixation.

Concealed Penile Duplication - Presenting Late.

We report a case of concealed penile duplication without many symptoms and normally looking external genitalia in a 10-year-old boy. He was evaluated with appropriate imaging and had successful surgical reconstruction of his genitalia with good cosmesis and functional outcome.

Hair Tourniquet of the Uvula.

Hair tourniquet syndrome (HTS) is a condition, where a strand of hair encircles the base of an appendage. We report a rare case of HTS of the uvula and review relevant literature.