Systemic capillary leak syndrome (SCLS) is a rare entity that is frequently idiopathic or, rarely, associated with infections, autoimmune diseases, drugs, surgery, and cancer. Several cancers can directly cause SCLS, although it is very uncommon as the inaugural presentation of a non-Hodgkin lymphoma. We report a case of SCLS as a paraneoplastic syndrome which revealed a large B-cell lymphoma, a non-Hodgkin lymphoma of B-cell origin.

UNASSIGNED: Skin breakdown is common in the intensive care unit (ICU). This pilot evaluation aimed to determine whether a nurse-constructed urinary catheter securement device using a silicone adhesive could reduce the complications of blistering and other skin breakdowns in a high-risk ICU population with Foley catheters.
UNASSIGNED: A prospective, non-randomised performance improvement study using a convenience sample was carried out.
UNASSIGNED: The study sample consisted of 29 patients with urethral Foley catheters and any degree of thigh oedema in a surgical ICU at an academic quarternary medical center.
UNASSIGNED: Patients were fitted with a standard acrylic-adhesive catheter securement device on one thigh and a nurse-constructed device on the contralateral thigh. At the beginning of each 12-hour shift, the nurse moved the Foley catheter from one securement device to the other; the nurse recorded the assessment findings at the end of the shift.
UNASSIGNED: The average age of the 29 patients was 61±16 (range 20-87) years. Visible skin compromise occurred in 21% of the time with the standard acrylic securement device; an equal percentage of men and women developed skin breakdown. Oedema status was a significant factor related to skin breakdown. There was no visible damage to the skin associated with the nurse-constructed silicone-adhesive device.
UNASSIGNED: A silicone adhesive urinary catheter securement device causes less skin damage than one with acrylic adhesive. One-step application, pain-free and atraumatic removal, and reliable securement are essential considerations in product development.

TAFRO syndrome is a rare and aggressive inflammatory entity characterized by thrombocytopenia, anasarca, fever, renal failure, reticulin fibrosis, and organomegaly. This entity supposes a diagnostic and therapeutic challenge due to its significant overlap with Castleman\'s disease. However, distinct clinical and histological features warrant its classification as a separate subtype of idiopathic multicentric Castleman\'s disease (iMCD). While recent modifications have been made to the diagnostic criteria for iMCD, these criteria lack specificity for this particular condition, further complicating diagnosis. Due to its inflammatory nature, several complex molecular signaling pathways are involved, including the JAK-STAT pathway, NF-kB, and signal amplifiers such as IL-6 and VEGF. Understanding the involvement of immune dysfunction, some infectious agents, genetic mutations, and specific molecular and signaling pathways could improve the knowledge and management of the condition, leading to effective treatment strategies. The current therapeutic approaches include corticosteroids, anti-IL6 drugs, rituximab, and chemotherapy, among others, but response rates vary, highlighting the need for personalized strategies. The prognosis is uncertain due to diagnostic difficulties, emphasizing the importance of early intervention and appropriate targeted treatment. This comprehensive review examines the evolving landscape of TAFRO syndrome, including the pathophysiology, diagnostic criteria, treatment strategies, prognosis, and future perspectives.

We present a life-threatening case of postpartum acute necrotizing pancreatitis. The patient is a 37-year-old female with no past medical history who delivered a healthy baby boy via cesarean section. Twenty days later, she presented to the emergency department with acute onset of nausea, non-bloody vomiting, abdominal bloating, and epigastric pain radiating to the back. Less than 24 hours later, she progressed into septic shock despite aggressive resuscitation, requiring vasopressor support in the ICU. Initial CT imaging showed multiple patchy hypodensities throughout the pancreas consistent with severe necrotizing pancreatitis. Her hospitalization was further complicated by difficulty obtaining source control of her infection, Clostridium difficile, and nutritional deficiencies that resulted in gross anasarca. She was discharged from the hospital on day 59 after undergoing multiple percutaneous drain placements, IV antibiotics, and endoscopic gastrocystostomy with four pancreatic necrosectomies. Since discharge, the patient has required readmission twice for complications from her pancreatitis.

Galactosialidosis (GS) is a rare lysosomal storage disorder. We reported here, the case of a 29-day-old boy who had increased body swelling, difficulty breathing, and petechiae on the trunk since birth. The antenatal history was unremarkable. Clinical laboratory findings included coarse facies, hepatosplenomegaly, gross ascites, thrombocytopenia, nephrotic range proteinuria, and bilateral hydronephrosis. The diagnostic challenge was resolved after genetic testing, which revealed GS with a novel homozygous c.1158dupA mutation.

BACKGROUND: Castleman disease (CD), also known as angiofollicular lymph node hyperplasia or large lymph node hyperplasia, is a rare medical condition. Despite its rarity, it exhibits diverse clinical presentations and outcomes, which pose challenges for comprehensive understanding and management. This study aims to shed light on the demographics, associations, and outcomes of CD by conducting a retrospective analysis.
METHODS: The National Inpatient Sample (US) was used to identify patients with the diagnosis of Castleman disease using ICD-10 diagnosis code D47.Z2, during the years 2016-2019. Data was collected on demographics, associated diagnoses, treatments and outcomes. Data analysis was performed using STATA Version 17, College Station, TX: Stata Corp LLC.
RESULTS: Our study identified 791 hospitalizations involving adult CD patients. The mean age of these patients was 52.4 years, with a male predominance (56.1%). Whites comprised the largest racial group affected (50.1%). Most patients were covered by Medicare (39.6%). The majority received treatment in urban teaching hospitals (84.0%) and large-bed size facilities (62.5%). In-hospital mortality was low at 2.8%, with an average length of stay of 7.5 days and average total charges of $109,308. Common associations included acute kidney injury (27.0%), congestive heart failure (17.1%), sepsis (16.4%), and acute respiratory failure (12.6%). Hematological and lymphatic associations featured anemia (47.5%), thrombocytopenia (12.2%), and other conditions. Red blood cell transfusions were administered to 11.1% of patients.
CONCLUSIONS: This study contributes valuable insights into CD, a rare and clinically heterogeneous disease. It underscores the importance of recognizing its associations and complications. Additionally, it highlights the need for further research and improved diagnostic and treatment guidelines to address the complexity of this condition.

UNASSIGNED: Physicians must be alert for the exocrine pancreatic insufficiency diagnosis through the follow-up of postgastrectomy patients, regardless the severity and lag time. Urgent albumin and pancreatic enzyme replacement should be considered when diagnosed.
UNASSIGNED: It is documented that exocrine pancreatic insufficiency (EPI) can develop after gastrectomy. Steatorrhea, malnutrition, and weight loss are common symptoms of the disease; however, it is usually mild to moderate postgastrectomy. This article reports a case of EPI manifested by hypoalbuminemia leading to dyspnea and anasarca, which are not typical symptoms of postgastrectomy EPI. A 61-year-old man with a history of gastric adenocarcinoma treated by total gastrectomy and chemoradiotherapy was admitted to the hospital with dyspnea and anasarca. Despite being diagnosed as a case of malignancy recurrence in another hospital, based on the symptoms described, no evidence of malignancy was found. His ascites and pleural effusion were determined to be caused by hypoalbuminemia. In addition, he claimed steatorrhea, and his stool elastase was lower than expected. EPI was diagnosed based on his medical history, paraclinical tests, and examinations. He remained asymptomatic for 1 year after being treated with albumin and pancreatic enzymes. Postgastrectomy EPI may be severe enough to cause steatorrhea or hypoalbuminemia. Hence, regardless of the severity of the presentation, physicians must be alert for this diagnosis throughout the follow-up of patients with a history of gastrectomy. Urgent albumin and pancreatic enzyme replacement should be considered when diagnosed.

OBJECTIVE: Central lymphatic obstructions are associated with anasarca and high mortality. We hypothesized that opening dilated cutaneous lymphatic channels by creating a lymphocutaneous fistula (LCF) would decompress the lymphatic circulation and improve anasarca.
METHODS: We reviewed all patients that had at least one LCF created between 9/2019 and 12/2022. LCF efficacy was determined by changes in weight, urine/diuresis, ventilation, and clinical status.
RESULTS: We created eleven LCFs in four infants. LCFs initially drained 108 cc/kg/d (IQR68-265 cc/kg/d). Weights significantly decreased after LCF creation (6.9 [IQR6.1-8.1] kg vs. 6.1 [IQR 4.9-7.6] kg, P = 0.042). Ventilatory support decreased significantly in all patients after at least one LCF was created, and 3/4 patients (75%) had significantly lower peak inspiratory pressures (28 [IQR 25-31] cmH2O vs. 22 [IQR 22-24] cmH2O, P = 0.005; 36 [IQR36-38] cmH2O vs. 33 [IQR 33-35] cmH2O, P = 0.002; 36 [IQR 34-47] cmH2O vs. 28 [28-31] cmH2O, P = 0.002). LCFs remained patent for 29d (IQR 16-49d). LCFs contracted over time, and 6/11 (54.5%) were eventually revised. There were no complications. Two patients died from overwhelming disease, one died from unrelated causes, and one remains alive 29 months after their initial LCF.
CONCLUSIONS: LCFs provide safe and effective temporary lymphatic decompression in patients with central lymphatic obstruction. While LCFs are not a cure, they can serve as a bridge to more definitive therapies or spontaneous lymphatic remodeling.
METHODS: IV.

The definitive role of viral infections, such as rotavirus, in causing Kawasaki disease (KD) remains uncertain. However, the intriguing observation of concomitant rotavirus infection and KD suggests a potential association. This study aimed to investigate this relationship. We reported a case of concomitant KD and rotavirus infection complicated by hyponatraemia and anasarca. For the systematic review, we used three large databases, namely PubMed, Ovid, and Scopus, to search articles with the terms \"Kawasaki\" and \"rotavirus\". We also used Google Scholar as our secondary source. We included articles that fulfilled the following criteria: (i) articles reporting on children aged 18 and below; (ii) articles reporting on patients infected with rotavirus prior to or concomitant with KD; and (iii) articles written in English only. Three articles were included and analysed in combination with our reported patient. All patients exhibited gastrointestinal symptoms, including diarrhoea and vomiting, in addition to non-resolving fevers, which eventually manifested more signs and symptoms to support the diagnosis of KD. Stool samples from all patients revealed positive rotavirus antigens. Two patients (n = 2) were noted to have hyponatraemia and hypoalbuminaemia. Three (n = 3) manifested coronary artery abnormalities (CAA). Even though the relationship is not fully understood yet, it is known that the combination of these two pathologies can cause catastrophic immune responses and complications.

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