Confluent and reticulated papillomatosis (CRP) was first described in 1927 by Gougerot and further characterised by Carteud.1 It is relatively rare, and the exact pathophysiology was not well known. Over the years, multiple treatment modalities were proposed. We report our experience with three cases of CRP which showed complete clearance with tetracycline group of antibiotics

Pneumonia is primarily a disease that is usually managedmedically with antibiotics. However, in rare cases it mayprogress to necrotising pneumonia, which is an uncommonbut severe complication of bacterial pneumonia. This caseillustrates a typical case of necrotising pneumoniacomplicated with parenchymal and pleural complicationsuch as empyema, pneumothorax with possiblebronchopleural fistula. Early consultation with thoracicsurgeon can be life-saving.

Acrodermatitis enteropathica (AE) is a rare genetic autosomal recessive disorder characterised byperiorificial inflammatory rash, diarrhoea and hair loss. Hereby, we report 3 cases of acrodermatitisenteropathica.

INTRODUCTION: Pericardiocentesis is a potentially life-saving procedure. We compared two low-cost models-an agar-based model and a novel model, Centesys-in terms of ultrasound image quality and realism, effectiveness of the model, and learners' confidence and satisfaction after training. METHODS: In this pilot randomised 2x2 crossover trial stratified by physician seniority, participants were assigned to undergo pericardiocentesis training either with the agar-based or Centesys model first, followed by the other model. Participants were asked to rate their confidence in performing ultrasound-guided pericardiocentesis, clarity and realism of cardiac structures on ultrasound imaging, and satisfaction on a 7-point Likert scale before and after training with each model. RESULTS: Twenty participants with median postgraduate year of 4 (interquartile range [IQR] 3.75-6) years were recruited. Pre-training, participants rated themselves a median score of 2.5 (IQR 2-4) for level of confidence in performing pericardiocentesis, which improved to 5 (IQR 4-6) post-training with Centesys ( CONCLUSION Centesys achieved greater learner satisfaction as compared to the agar-based model, and was an effective tool for teaching ultrasound-guided pericardiocentesis and drain insertion.

No abstract available.
Chorea ; Creutzfeldt-Jakob Syndrome

OBJECTIVES: To study the early clinical efficacy of combined therapy of stage 4 neuroblastoma. METHODS: A retrospective analysis was performed on the medical data and follow-up data of 14 children with stage 4 neuroblastoma who were diagnosed in Hong Kong University-Shenzhen Hospital from January 2016 to June 2021. RESULTS: The median age of onset was 3 years and 7.5 months in these 14 children. Among these children, 9 had positive results of bone marrow biopsy, 4 had N-Myc gene amplification, 13 had an increase in neuron-specific enolase, and 7 had an increase in vanilmandelic acid in urine. Based on the results of pathological examination, differentiated type was observed in 6 children, undifferentiated type in one child, mixed type, in one child and poorly differentiated type in 6 children. Of all the children, 10 received chemotherapy with the N7 regimen (including 2 children receiving arsenic trioxide in addition) and 4 received chemotherapy with the Rapid COJEC regimen. Thirteen children underwent surgery, 14 received hematopoietic stem cell transplantation, and 10 received radiotherapy. A total of 8 children received Ch14.18/CHO immunotherapy, among whom 1 child discontinued due to anaphylactic shock during immunotherapy, and the other 7 children completed Ch14.18/CHO treatment without serious adverse events, among whom 1 child was treated with Lu177 Dotatate 3 times after recurrence and is still undergoing chemotherapy at present. The median follow-up time was 45 months for all the 14 children. Four children experienced recurrence within 2 years, and the 2-year overall survival rate was 100%; 4 children experienced recurrence within 3 years, and 7 achieved disease-free survival within 3 years. CONCLUSIONS Multidisciplinary combined therapy is recommended for children with stage 4 neuroblastoma and can help them achieve better survival and prognosis.
Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Child ; Child, Preschool ; Combined Modality Therapy ; Humans ; Infant ; Neuroblastoma/drug therapy* ; Positron-Emission Tomography ; Radionuclide Imaging ; Retrospective Studies ; Treatment Outcome

Peptides are increasingly important resources for biological and therapeutic development, however, their intrinsic susceptibility to proteolytic degradation represents a big hurdle. As a natural agonist for GLP-1R, glucagon-like peptide 1 (GLP-1) is of significant clinical interest for the treatment of type-2 diabetes mellitus, but its in vivo instability and short half-life have largely prevented its therapeutic application. Here, we describe the rational design of a series of α/sulfono-γ-AA peptide hybrid analogues of GLP-1 as the GLP-1R agonists. Certain GLP-1 hybrid analogues exhibited enhanced stability (t _1/2 > 14 days) compared to t _1/2 (<1 day) of GLP-1 in the blood plasma and in vivo. These newly developed peptide hybrids may be viable alternative of semaglutide for type-2 diabetes treatment. Additionally, our findings suggest that sulfono-γ-AA residues could be adopted to substitute canonical amino acids residues to improve the pharmacological activity of peptide-based drugs.