Systemic Capillary Leak Syndrome as a Paraneoplastic Syndrome.

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.

Skin integrity preservation using a nurse-constructed silicone adhesive Foley catheter.

AIMS: 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. DESIGN: A prospective, non-randomised performance improvement study using a convenience sample was carried out. SUBJECTS AND SETTING: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Unraveling TAFRO Syndrome: An In-Depth Look at the Pathophysiology, Management, and Future Perspectives.

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.

Hydrops fetalis caused by a complex congenital heart defect with concurrent hypoplasia of pulmonary blood vessels and lungs visualized by micro-CT in a French Bulldog.

BACKGROUND: Hydrops fetalis (HF) is fluid accumulation in fetus body cavities and subcutaneous tissue. The condition has been described in various farm and companion animal species, including dogs. Most of cases result from a heart defect. Exact nature of this defect is rarely clarified. CASE PRESENTATION: A newborn, male French bulldog puppy with severe HF underwent a full anatomopathological examination to diagnose the primary cause of HF. Based on the anatomopathological examination, fetal ultrasound, and micro-computed tomography, transposition of the great arteries with hypoplasia of the ascending aorta, aortic arch interruption, ostium secundum atrial septal defect, severe tricuspid valve dysplasia, as well as hypoplasia of pulmonary vessels and lungs were diagnosed. CONCLUSIONS: This is the first report of HF caused by severe, complex congenital heart defects with concurrent pulmonary vessel and lung hypoplasia.

A Case of Life-Threatening Postpartum Necrotizing Pancreatitis Requiring Gastrocystostomy With Serial Pancreatic Necrosectomies.

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.

Checkpoint Inhibitor-Related Capillary Leak Syndrome (CLS).

Immune checkpoint inhibitors (ICIs) are now being widely used for the treatment of various malignancies, but they have a distinctive set of side effects due to the overactivation of the immune system, which is important to recognize. Capillary leak syndrome (CLS) is a rare but potentially life-threatening side effect of ICIs that causes a significant increase in the permeability of capillaries, leading to the leakage of plasma-containing proteins from these small vessels. This condition results in several clinical features, including edema, hypotension, hypoalbuminemia, and hemoconcentration. Timely recognition and discontinuation of the offending immunotherapy can optimize outcomes. Treatment is focused on supportive care and prompt initiation of immunosuppressants, such as steroids.

Castleman disease- demographics, associations, and outcomes: an analysis of adult 791 cases.

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. CONCLUSION: 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.

Protein-losing enteropathy secondary to graft-versus-host disease

Protein-losing enteropathy is a gastrointestinal complication of Graft versus host disease. The clinical presentation can be similar to that of multiple pathologies and represents a diagnostic challenge for clinicians. We report a 23-year-old man with a history of acute lymphoid leukemia that required allogeneic hematopoietic stem cell transplantation that came to evaluation due to anasarca. We report a 23-year-old man with a history of acute lymphoid leukemia who required allogeneic hematopoietic stem cell transplantation and came to evaluation due to anasarca. (AU)

A life-threatening presentation of postgastrectomy exocrine pancreatic insufficiency: A case report.

Key Clinical Message: 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. Abstract: 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.

Surgical creation of lymphocutaneous fistulas for the management of infants with central lymphatic obstruction.

PURPOSE: 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. CONCLUSION: 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. LEVEL OF EVIDENCE: IV.

Concomitant Kawasaki Disease and Rotavirus Infection-More Than Just a Coincidence: A Case Report.

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.

Case report: Rehabilitation course in thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly syndrome complicated by cerebral infarction in the left parabolic coronary region.

Although thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (TAFRO) syndrome was first reported in 2010, its pathogenesis and prognosis are still unknown. Moreover, reports on rehabilitation in patients with TAFRO are limited. In severe cases, dyspnea and muscle weakness could impede improvements in activities of daily living (ADL). However, reports on exercise intensity showed no worsening of TAFRO within the load of 11-13 on the Borg scale. Herein, we describe the rehabilitation and progress in a 61-year-old woman with TAFRO syndrome complicated by cerebral infarction from early onset to discharge. After cerebral infarction onset in the perforating artery, she was admitted to the intensive care unit due to decreased blood pressure and underwent continuous hemodiafiltration. Two weeks following transfer to a general ward, the patient started gait training using a brace due to low blood pressure, respiration, and tachycardia. After initiating gait training, increasing the amount of training was difficult due to a high Borg scale of 15-19, elevated respiratory rate, and worsening tachycardia. Furthermore, there was little improvement in muscle strength on the healthy side after continuous training, owing to long-term steroid administration. On day 100 after transfer, the patient was discharged home with a T-cane gait at a monitored level. The patient had severe hemiplegia due to complications with severe TAFRO syndrome delaying early bed release and gait training; tachycardia; and respiratory distress. Additionally, delayed recovery from muscle weakness on the non-paralyzed side made it difficult for the patient to walk and perform ADLs. Despite these issues, low-frequency rehabilitation was useful. However, low-frequency rehabilitation with gait training, using a Borg scale 15-19 orthosis, did not adversely affect the course of TAFRO syndrome.

Anasarca Revealing Severe Cardiac Involvement Due to Behçet's Disease (BD): A Case Report.

Behçet's disease (BD) is a systemic vasculitis which is most often manifested by recurrent oral aphthosis, genital aphthosis, and ocular involvement with sometimes visceral damage, in particular neurological, digestive, vascular, or renal. We report the case of a 21-year-old man admitted for anasarca who revealed severe cardiac involvement associating endomyocardial fibrosis, intracardiac thrombi and involvement of the tricuspid valve in the context of BD diagnosed a posteriori. Cardiac involvement is exceptional during BD, especially as a mode of entry into the disease. It can be particularly severe, hence the need for early diagnosis, rapid and sometimes aggressive management. Close monitoring is also necessary in order to watch for the occurrence of visceral manifestations, particularly in young patients.

Anasarca as a manifestation of childhood volvulus: Diagnostic and management challenges in an 11-year old Nigerian boy.

Background: Small intestine volvulus occurs more commonly among younger children. It often poses diagnostic challenges when it occurs in older children and adults. For good clinical outcomes, it is essential to have prompt presentation, diagnosis and early intervention. Anasarca is not a common clinical manifestation of small intestine volvulus. Case Presentation: We report this unusual presentation of small intestine volvulus in an 11-year old Nigerian boy who first presented only with anasarca. While being investigated for the cause of the anasarca, he developed features of acute abdomen thought to be spontaneous bacteria peritonitis initially. He had surgery where the diagnosis of small intestine volvulus was made. Conclusion: The diagnosis and management of both anasarca and small intestine volvulus could be fraught with challenges. It is possible that anasarca can be the first manifestation of small intestine volvulus.

Insulin edema: An untypical etiology of anasarca in a 19-year old female presenting to a pediatric emergency department.

Pulmonary edema and anasarca are both common findings in patients presenting to emergency departments (ED). The differential diagnosis for these conditions is very wide and requires an initially broad approach that considers multiple organ systems. Insulin edema has been previously described in multiple case reports as a likely cause of acutely developing edema in mostly type I diabetics either initiating or increasing the intensity of their insulin regimens. This case report describes a 19-year-old female with history of type I diabetes mellitus recently admitted to the hospital for diabetic ketoacidosis (DKA) presenting to a pediatric emergency department (ED) with dyspnea and weight gain. The patient had been reportedly poorly compliant on her insulin therapy before her last admission for DKA and had strictly begun to adhere to her insulin therapy in the interim. Her clinical presentation was notable for hypoxia requiring supplemental oxygen, bilateral lower extremity pitting edema, weight gain of 30 kg since discharge 9 days ago, a chest Xray displaying bilateral pulmonary edema and a work-up otherwise unrevealing for cardiac, renal, or liver etiologies. She was then admitted to the Pediatric Intensive Care Unit (PICU) on supplemental oxygen where through further evaluation she was determined to have insulin edema. This case details an unlikely etiology of anasarca and pulmonary edema, however diagnosing this condition highlights the broad diagnostic process that must be considered for any patient without known significant cardiac, renal, or liver history presenting with respiratory distress and anasarca especially on initial presentation to an emergency department.

Infantile Galactosialidosis with Novel Mutation: An Early Presentation.

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.

Síndrome Edematoso Generalizado por citomegalovirus

El síndrome edematoso generalizado o anasarca está presente secundario a un desequilibrio de la homeostasis hídrica, electrolítica y osmolar del organismo, En el adulto el estado de anasarca es más frecuente secundario a insuficiencia cardiaca, en pediatría, el edema que se generaliza está asociado con más frecuencia a bajo aporte proteico, baja síntesis de proteínas o debido a perdida de proteínas de origen gastrointestinal o renal. La disminución de la presión oncótica plasmática genera fuga de líquidos a compartimientos intersticiales de forma generalizada y produce edema. Existen múltiples patologías y mecanismos para la producción del edema generalizado; el conocimiento de la fisiopatología de su desarrollo permite un análisis clínico, de laboratorio y de gabinete que orientan al diagnostico. La infección por citomegalovirus es una causa poco frecuente de edema generalizado, reconocer esta entidad y llegar a un adecuado diagnóstico diferencial es el objetivo de esta revisión.

The generalized edematous syndrome or anasarca is present secondary to an imbalance in the body's water, electrolyte and osmolar homeostasis. In adults, the state of anasarca is more frequent secondary to heart failure; in pediatrics, generalized edema is associated with more frequency due to low protein intake, low protein synthesis or due to protein loss of gastrointestinal or renal origin. The decrease in plasma oncotic pressure generates generalized fluid leakage into interstitial compartments and produces edema. There are multiple pathologies and mechanisms for the production of generalized edema; knowledge of the pathophysiology of its development allows a clinical, laboratory and office analysis that guides the diagnosis. Cytomegalovirus infection is a rare cause of generalized edema; recognizing this entity and reaching an appropriate differential diagnosis is the objective of this review.

Congenital malformations in brachycephalic dogs: A retrospective study.

The popularity of brachycephalic dogs has increased in recent years due to their docile temperament and peculiar features. The historical inbreeding and consequent lack of genetic diversity involved in the development of these breeds led to an increase in the manifestation of deleterious genes that may lead to malformations. In addition, there are serious health issues intrinsic to the conformation, mainly attributed to these extreme characteristics. Therefore, this retrospective study aimed to observe the frequency of malformations in brachycephalic dogs compared to the pure and mixed breeds (MB). The medical records of pregnant bitches admitted at the Service of Obstetrics and Animal Reproduction (SORA) from January 2017 to December 2021 were retrieved from the hospital's computer system and analyzed one by one. Seven hundred sixty-eight neonates born from 168 litters were included in this study. Of these litters, 72.6% (122/168) were brachycephalic. Malformations were found in 52 puppies, with an incidence of 6.77% (52/768). Of the 32 litters that produced malformed puppies, 28 were brachycephalic (87.5%). In total, 23 types of malformations were registered, the most common being cleft palate (1.30%) and anasarca (1.17%). Ten of the puppies (10/52; 19.23%) presented two or more associated malformations. Bitches above 7 years were more prone to present malformed puppies in their litters. Brachycephalic breeds were 3.03 times more likely to present malformed neonates when compared to other breeds; the odds ratio increased to 5.07 when modern brachycephalic was compared to ancestral brachycephalic. Regarding the mode of delivery, elective cesarean sections accounted for 66.6% of births while 19.64% were eutocic vaginal deliveries, and 13.69% were dystocic. The presence of malformed puppies in a litter causes suffering for the owner, the bitch and for the puppy itself, therefore, the veterinarian plays a key role in this scenario. Knowledge about congenital abnormalities, their causes, diagnosis, and approach is essential to reduce the incidence of malformations and improve the quality of life of these animals.

TAFRO syndrome: A severe manifestation of Sjogren's syndrome? A systematic review.

BACKGROUND: Sjögren's syndrome (SjS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the salivary and lacrimal glands associated with sicca syndrome. TAFRO syndrome is a systemic inflammatory disease of unknown cause, characterized by Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis, Renal dysfunction and Organomegaly, first reported in 2010 in Japanese patients. Despite their rarity, both conditions have been concurrently reported in several patients during the recent years, hence questioning the existence of shared or related features. METHODS: A systematic review of the literature regarding SjS associated with TAFRO syndrome (SjS-TAFRO) was performed. The 2019 updated Masaki diagnostic criteria were used for TAFRO syndrome and SjS was considered when the diagnosis was mentioned by the authors, necessarily with either anti-Sjogren's Syndrome A (SSA) ± anti-Sjogren's Syndrome B (SSB) antibodies and/or histological evidence of focal lymphocytic sialadenitis. RESULTS: Ten cases of SjS-TAFRO have been reported in the literature to date. Compared to SjS patients without TAFRO syndrome, these 10 SjS-TAFRO had a lower female predominance (2.3:1 vs 9:1 women to man ratio) and a higher frequency of anti-SSA antibodies (90% vs 70%). All fulfilled the three major Masaki criteria i.e., anasarca, thrombocytopenia, and systemic inflammation. Seven of them (70%) had megakaryocyte hyperplasia or reticulin fibrosis in the bone marrow. Lymph node biopsy was performed in 8 out of 10 cases (80%) and results were consistent with Castleman disease in 6 (75%). Eight of them had developed renal failure (80%) within six months. Nine of them (90%) had organomegaly, with hepatosplenomegaly in 8 cases and splenomegaly alone in 1. CONCLUSION: This review brings new insights regarding TAFRO syndrome and suggests it could be a severe manifestation of SjS. The identification of shared abnormal signaling pathways could help in the therapeutic management of both diseases, which face an unmet therapeutic need.