A 34-year-old woman presented with worsening generalized swelling and breathlessness for four months; physical examination showed pallor, diffuse anasarca, and bilateral crackles on respiratory auscultation. Laboratory investigations showed severe hypoproteinemia, fat malabsorption with fat-soluble vitamin deficiency, and significant protein loss in the stool. Imaging studies revealed pulmonary edema, ascites, bowel wall edema, and a duodenal polyp. Further evaluating the duodenal polyp, a grade two duodenal neuroendocrine tumor (NET) was identified. She was managed with subcutaneous octreotide and duodenal polypectomy, resulting in significant clinical improvement. This case highlights the importance of diagnosing and managing protein-losing enteropathy secondary to gastric neuroendocrine tumors.

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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.

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.

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.

Nephrotic syndrome in children is mostly idiopathic in origin. About 90% of patients respond to corticosteroids; 80-90% have at least one relapse and 3-10% become corticosteroid resistant after the initial response. A kidney biopsy is seldom indicated for diagnosis except in patients with atypical presentation or corticosteroid resistance. For those in remission, the risk of relapse is reduced by the administration of daily low dose corticosteroids for 5-7 days at the onset of an upper respiratory infection. Some patients may continue having relapses through adult life. Many country-specific practice guidelines have been published, which are very similar with clinically insignificant differences.

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.