A rare case of Crigler-Najjar syndrome type 2: A case report and literature review.

Key Clinical Message: Crigler-Najjar syndrome type 2 should be suspected in any young patient presenting with isolated indirect hyperbilirubinemia where all other common etiologies have been excluded. It is a relatively benign condition that responds to phenobarbitone. Abstract: Crigler-Najjar syndrome (CNS) type 2 is an inborn cause of isolated indirect hyperbilirubinemia characterized by a partial deficiency of the enzyme uridine 5'-diphosphate-glucuronosyltransferase (UGT) responsible for bilirubin conjugation. Typically, this condition is diagnosed based on clinical manifestations, supplemented by enzyme analysis if feasible, and exhibits a significant response to phenobarbitone, known for its enzyme-inducing properties. In this case, we present a young male patient who had experienced recurrent isolated indirect hyperbilirubinemia since early childhood, with negative results in the hemolytic workup. The patient exhibited a UGT1A1 gene defect and demonstrated a highly favorable response to phenobarbitone treatment. The purpose of this report is to raise awareness among physicians about this benign condition and underscore the importance of avoiding unnecessary investigations.

Management of Diabetic Ketoacidosis in a Patient With Chronic Kidney Disease Under Maintenance Hemodialysis: A Case Report.

Diabetic ketoacidosis (DKA) is a potentially fatal metabolic complication seen in individuals with type 1 diabetes mellitus (DM) or type 2 DM under stress, such as infections and non-compliance with treatment. DKA in chronic kidney disease (CKD) patients undergoing maintenance hemodialysis (HD) presents challenges due to the unique pathophysiology and the absence of specific management guidelines. This case report highlights the importance of tailoring the treatment of DKA based on the specific requirements of CKD patients on HD. The presented case involves a 47-year-old female with type 2 DM and CKD who developed DKA in the context of a urinary tract infection (UTI). Management included insulin infusion, cautious fluid replacement therapy, electrolyte monitoring, and identifying precipitating factors, such as an infection. The case highlights the complexity of DKA management in CKD patients and the necessity of individualized approaches. More studies and guidelines are needed to optimize the proper management of DKA in CKD patients.

Prevalence of pre-operative anxiety and associated risk factors among patients awaiting elective surgery in a tertiary care hospital.

Background: Patients undergoing surgery have a fear of anesthesia and surgical procedures that results in anxiety. The global incidence of pre-operative anxiety is estimated at 60-92%. Age, gender, education, marital status, type of family, type of anesthesia and surgery, and history of surgery are the contributing factors. High levels of anxiety during the pre-operative period has negative impacts on surgical outcomes. The main objective of this study was to find out the prevalence of pre-operative anxiety and associated risk factors in a hospital setting of a developing country. Methods: This was a single center, analytical, cross-sectional study conducted among the admitted patients scheduled for elective surgeries in a tertiary care hospital. Non-probability convenience sampling was adopted and a total of 205 cases were included. The researchers themselves collected the data on the day before surgery using questionnaires comprised of two parts: semi-structured questionnaires prepared via literature review and Amsterdam Pre-operative Anxiety and Information Scale (APAIS). Data were analyzed in SPSS version 23. Bivariate and multivariate analyses were performed appropriately. Results: The prevalence of pre-operative anxiety was 25.85%. The median anaesthesia related, surgery related, and total anxiety scores were 4.00, 5.00 and 9.00 respectively. Likewise, the median score of information desired component scale was 5.00. Different anxiety scores were positively correlated with the information desire component score. The patients living in a nuclear family (adjusted OR, 2.480; 95% CI, 1.272-4.837, p = 0.008) and those without past history of surgery (adjusted OR, 2.451; 95% CI, 1.107-5.424, p = 0.027) had approximately 2.5 times higher risk of having pre-operative anxiety compared to those from a joint family and those having past history of surgery respectively. Those receiving spinal anesthesia had approximately two times lower risk of anxiety (adjusted OR, 0.511; 95% CI, 0.265-0.985, p = 0.045). Conclusions: One fourth of the patients had pre-operative anxiety. Type of family, type of anesthesia and past history of surgery were found to be the independent predictors of anxiety.

Complicated pylephlebitis secondary to perforated appendicitis in a child- A rare case report.

Introduction and importance: Pylephlebitis is a rare and life threatening thrombophlebitis of the portal vein. It commonly occurs following intra abdominal infections like appendicitis.It is even rarer in the pediatric age group. The nonspecific presentation impedes the diagnosis. Timely use of appropriate antibiotics and control of infection is paramount in its treatment and this case report highlights the same. Case presentation: 11 year old female child from a rural area was referred from a local hospital for persistent fever and abdominal pain despite medical treatment. Workup revealed perforated appendicitis, pylephlebitis, and multiple liver abscess. She was successfully treated with appendicectomy followed by antibiotics and anticoagulants. Clinical discussion: Pylephlebitis secondary to appendicitis was frequently lethal in the pre-antibiotic era. Doppler ultrasonography and CT scan are the investigations of choice to establish the diagnosis by showing a thrombus in the portal vein. With use of antibiotics, early diagnosis by imaging and surgical control of the primary infection, appendicitis-associated-pylephlebitis now has improved outcomes. Larger scale studies are required to establish the role of anticoagulants. Conclusion: Early diagnosis and intervention of this fatal condition is life saving but numerous gaps exist in the literature regarding the treatment recommendation.