Unfortunately, we are all too familiar with the statement: “Necrotizing enterocolitis remains the leading cause of gastrointestinal surgical emergency in preterm neonates”.
It's been five decades ...since the first animal models of necrotizing enterocolitis (NEC) were described. There remains much investigative work to be done on identifying various aspects of NEC, ranging from the underlying mechanisms to treatment modalities. Experimental NEC is mainly focused on a rat, mouse, and piglet models. Our aim is to not only highlight the pros and cons of these three main models, but to also present some of the less-used animal models that have contributed to the body of knowledge about NEC.
Choosing an appropriate model is essential to conducting effective research and answering the questions asked. As such, this paper reviews some of the variations that come with each model.
BACKGROUND Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare genetic condition caused by an X-linked mutation of the OCRL1 gene, with an estimated prevalence in the general ...population of approximately 1 in 500 000. It is a multisystem disorder most commonly affecting the eyes, central nervous system, and kidneys. These commonly manifest as congenital cataracts, intellectual disability, and proximal renal dysfunction (Fanconi-type). Epidermal lesions are an uncommon manifestation of this condition, and the association is not completely understood. CASE REPORT Here we present a case of a 9-year-old boy with Lowe syndrome who presented with multiple cystic masses found in the perianal region. An excision was then performed to remove the masses and found that the lesions were epidermal cysts, which are infrequently found in Lowe syndrome. After excision, the patient recovered uneventfully without complications. CONCLUSIONS While epidermal cysts are an uncommon manifestation that have been documented, our case remains unique given the location and associated symptoms of the lesions. At presentation, the constellation of pain and perianal masses was concerning for a malignant etiology. However, after diagnostic imaging was performed, these lesions were found to be epidermal cysts, an infrequent manifestation of Lowe syndrome. Few previous case reports described cystic lesions in association with Lowe syndrome, and none, to our knowledge, have described multiple symptomatic perianal lesions. This case is important to consider because epidermal cystic lesions can be found with this presentation and should be considered on differential diagnoses for dermatologic findings in Lowe syndrome patients.
Neonatal gastric perforation: Case report Vacaru, Alexandra; Sharafeddin, Fransua; Maidan, Alexandra ...
Journal of pediatric surgery case reports,
August 2023, 2023-08-00, 2023-08-01, Letnik:
95
Journal Article
Recenzirano
Odprti dostop
Neonatal gastric perforation (NGP) is a life-threatening condition associated with high morbidity and mortality rates. Despite several proposed etiological theories, the causative principle is still ...unclear and controversial.
A male neonate was born at 26 weeks 3 days gestation. The patient showed symptoms of acute respiratory failure and hypoxia and was admitted to the neonatal intensive care unit. On day of life (DOL) 2, a chest and abdominal radiography showed a large amount of free intraperitoneal air, for which exploratory laparotomy was performed. A small antimesenteric perforation in the distal ileum was found and repaired primarily. On DOL 7, a chest and abdominal radiography demonstrated recurrent pneumoperitoneum for which a repeat exploratory laparotomy was performed. The patient had global intestinal ischemia and a perforation in the anterior wall of the stomach, which was repaired. On DOL 12, a planned upper gastrointestinal water-soluble contrast study showed spillage of contrast into the anterior abdomen, for which the patient underwent a repeat exploratory laparotomy. We found a dehiscence of the prior gastric repair and closed it primarily. The remainder of the hospital course was uneventful and the patient reached full feedings on DOL 41.
Premature neonates are at risk for gastric perforation. Gastric perforation should be in the differential diagnosis of a premature neonate who presents abdominal distension and pneumoperitoneum. Due to the fragility of the premature neonatal tissues, confirming the integrity of the gastric closure with a contrast study is recommended prior to initiating enteral feedings.
Trisomy 18 and necrotizing enterocolitis Maidan, Alexandra; Sacks, Marla A.; Raymond, Steven L. ...
Journal of pediatric surgery case reports,
October 2022, 2022-10-00, 2022-10-01, Letnik:
85
Journal Article
Recenzirano
Odprti dostop
Both Trisomy 18 (T18) and Necrotizing enterocolitis totalis (NEC-T) are major causes of morbidity and mortality in neonates. However, to our knowledge there has never been a case of a full-term ...infant born with both T18 and NEC-T published in the literature.
We present a case of NEC totalis in male neonate born at 39 weeks gestation with Trisomy 18. He was diagnosed with T18 in-utero, and after genetic counseling, the mother carried the pregnancy to term. After delivery, the newborn developed signs of respiratory distress, requiring use of continuous positive airway pressure support. On day of life (DOL) 8, multiple bloody bowel movements raised suspicion for NEC and after supplemental investigations prompted a series of exploratory laparotomies that revealed extensive necrotizing enterocolitis involving the entire gastrointestinal tract. On DOL 12, the family opted for palliative care and the patient subsequently expired.
To our knowledge, after searching through the English literature, no cases were found showing a neonate with a combination of T18 and NEC-T.
Background: Neonatal gastric perforation (NGP) is a life-threatening condition with a high mortality rate. It accounts for 7% of all gastrointestinal tract (GIT) perforations. The number of NGP cases ...has been increasing due to the rise in premature and low birth weight neonates. In this study, we present our experience with gastric perforation in neonates.
Methods: This retrospective study analyzed all cases of gastric perforation in neonates that were treated at Loma Linda University Medical Center's Neonatal Intensive Care Unit (NICU) between the years 2000 and 2023. The study looked at several variables including patient demographics, birth weight, age at admission and surgery, comorbidities, use of non-steroidal anti-inflammatory drugs (NSAIDs) and steroids, and mortality rate.
Results: We treated 15 patients with neonatal gastric perforation (NGP) during the study tenure. The median age at admission was 2 days, with 67% admitted within that timeframe. Surgery occurred at a median age of 5.5 days, and the median birth weight was 2.075 kg, with 26.67% below 1 kg. Males comprised 67%, and 60% had patent ductus arteriosus. Steroids were given to 46.66%, and 30-day mortality was 26.67%, with higher rates among males. Idiopathic cases were common, with notable etiologies including ischemia, necrosis, and congenital anomalies. Primary surgical repair was the main modality, and perforations occurred at various locations. Two cases had necrotizing enterocolitis. Six patients had favorable outcomes, while others experienced mild to moderate complications.
Conclusion: Our research supports the idea that males have a worse outcome in terms of both prevalence and survival rates in neonatal gastric perforation (NGP) patients. However, our findings did not confirm the notion that NGP mortality risk is higher in neonates with low birth weight. We also discovered that the median time between admission and surgery in our study group was 1.5 days, emphasizing the importance of early detection of NGP in neonates. Early diagnosis can lead to better decision-making regarding treatment options and surgical intervention.
Human bestrophin-1 protein (hBest1) is a transmembrane channel associated with the calcium-dependent transport of chloride ions in the retinal pigment epithelium as well as with the transport of ...glutamate and GABA in nerve cells. Interactions between hBest1, sphingomyelins, phosphatidylcholines and cholesterol are crucial for hBest1 association with cell membrane domains and its biological functions. As cholesterol plays a key role in the formation of lipid rafts, motional ordering of lipids and modeling/remodeling of the lateral membrane structure, we examined the effect of different cholesterol concentrations on the surface tension of hBest1/POPC (1-palmitoyl-2-oleoyl-sn-glycero-3-phosphocholine) and hBest1/SM Langmuir monolayers in the presence/absence of Ca
ions using surface pressure measurements and Brewster angle microscopy studies. Here, we report that cholesterol: (1) has negligible condensing effect on pure hBest1 monolayers detected mainly in the presence of Ca
ions, and; (2) induces a condensing effect on composite hBest1/POPC and hBest1/SM monolayers. These results offer evidence for the significance of intermolecular protein-lipid interactions for the conformational dynamics of hBest1 and its biological functions as multimeric ion channel.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Thin films of cerium dioxide were deposited on silicon (100) substrates using e‐beam evaporation. The influence of different technological parameters, such as the substrate temperature, the oxygen ...pressure and the beam power on the crystallographic orientation of the films was explored. By optimizing these parameters, CeO2 thin films with a strongly preferred (200) orientation could be deposited on Si(100) substrates.
XRD patterns of the CeO2 thin films deposited at various temperatures.
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