The enpp4 ectonucleotidase regulates kidney patterning signalling networks in Xenopus embryos

The enpp ectonucleotidases regulate lipidic and purinergic signalling pathways by controlling the extracellular concentrations of purines and bioactive lipids. Although both pathways are key regulators of kidney physiology and linked to human renal pathologies, their roles during nephrogenesis remain poorly understood. We previously showed that the pronephros was a major site of enpp expression and now demonstrate an unsuspected role for the conserved vertebrate enpp4 protein during kidney formation in Xenopus. Enpp4 over-expression results in ectopic renal tissues and, on rare occasion, complete mini-duplication of the entire kidney. Enpp4 is required and sufficient for pronephric markers expression and regulates the expression of RA, Notch and Wnt pathway members. Enpp4 is a membrane protein that binds, without hydrolyzing, phosphatidylserine and its effects are mediated by the receptor s1pr5, although not via the generation of S1P. Finally, we propose a novel and non-catalytic mechanism by which lipidic signalling regulates nephrogenesis.

Vascular anatomical considerations in preparing colonic flaps to replace the oesophagus

Background The colon is among the best options to substitute the oesophagus; it is well known for its durability and good function that makes it most suitable for paediatric patients. The steps of the procedure, postoperative complications, and outcome assessment were thoroughly discussed in previous reports. However, in this report, we have tried to focus on one basic and essential step of the operation, which is fashioning of the colonic flap used to substitute the oesophagus. Results The study included 50 consecutive paediatric cases who underwent colonic replacement of the oesophagus during the period 2010 through 2020. The indication for oesophageal replacement was either oesophageal atresia (27 cases) or corrosive strictures (23 cases). Our standard technique was using a middle segment of the colon (transverse colon) based on the left colic vessels (vascular pedicle) after ligation of middle colic vessels. Variations of the middle colic vessels were encountered that included single, double, or absent vessels. In a single case (2%), the middle colic vessels were multiple, short, and non-branching with interrupted continuity of marginal vessels at that point. In the latter situation, we had to use a different technique by fashioning a right colonic flap based on the middle colic vessels. Conclusion In colonic replacement of the oesophagus, preparing a pedicled flap from the transverse colon based on the left colic vessels was almost always feasible owing to the stable collateral marginal vessels. On a rare occasion, the marginal vessels were interrupted by disturbed anatomy of the middle colic vessels when we had to shift to another technique using a right colonic flap.

Primary Small-Cell Carcinoma of the Hard Palate: An Extremely Rare Occasion

: Most primary small cells carcinoma develop from the lung, and extra-pulmonary small cell carcinoma accounts for less than 5% of all small cell carcinoma cases. In the head and neck region, the most common sites are the larynx and salivary glands; however primary involvement of the palate is extremely rare. The extra-pulmonary small cell carcinoma is similar to its pulmonary counterpart regarding morphology, immunohistochemistry, and electron microscopy features and derives from pluripotential stem cells that develop neuroendocrine features. There is growing evidence regarding the ability of extra-pulmonary small cell carcinoma to arise from pluripotent basilar cells capable of divergent differentiation with the consequence of arising neuroendocrine phenotype as a trans-differentiation phenomenon in the progression of an organ-specific carcinoma. The differentiation between primary and metastatic extra-pulmonary small cell carcinoma is challenging. There is also no standard guideline for treatment as it is a rare occasion, and there is no consensus between radiation oncologists and cancer surgeons about the best treatment strategy. In this study, a very rare case of hard palate small cell carcinoma with neuroendocrine pathology features is presented. To our best of knowledge, this is the third case report of extra-pulmonary small cell carcinoma involving the palate in the literature review. Knowing the clinical presentation and pathology characteristics of such rare tumor in addition to follow-up outcome can be highly useful to establish a reliable guideline for hard palate small cell carcinoma management.

A rare case of compression atelectasis due to spontaneous diaphragmatic hernia: a case report

Type IV diaphragmatic hernia is a rare case which has various clinical manifestations. The clinical manifestations as a result of protruded organ towards thoracic cavity in diaphragmatic hernia are varied from gastrointestinal symptoms such as epigastric pain, dysphagia and acid reflux. However, in a rare occasion, dyspnea may present as a result of compression atelectasis due to excessive increased in intrathoracic pressure. Here, we report a 57-year-old male patient who had spontaneous diaphragmatic hernia admitted with dyspnea as a chief complaint. This report might add another perspective to the physicians in facing the patient with atelectasis as the result of a high level of lung compression secondary to type IV diaphragmatic hernia.

Pharmacological treatment of the patients with croup

Background: Croup is a respiratory illness usually caused by acute viral infection of the larynx, trachea, and bronchi, and characterized by the abrupt onset of a barking cough, inspiratory stridor, hoarseness, and respiratory distress due to upper airway obstruction. Croup commonly affects children younger than 6 years of age, with peak incidence between 7 and 36 months. Although the disease is usually self-limited, it may occasionally become life threatening, and can, on rare occasion, lead to respiratory failure.Current Concepts: Treatment of viral croup depends on the severity of symptoms as denoted by Westley croup score (i.e., mild, moderate, or severe). A single dose of oral or intramuscular dexamethasone (0.15-0.6 mg/kg) is the mainstay of treatment for viral croup, irrespective of severity. A single dose of nebulized budesonide (2 mg) is equally effective as systemically administered dexamethasone, and is considered when a patient is unable to take a medicine orally. Nebulized L-epinephrine (1:1,000, 3-5 mL) causes vasoconstriction in the mucosa, rapidly reducing airway edema. Addition of nebulized L-epinephrine is indicated in the patients with croup of at least moderate severity, displaying chest retraction and signs of labored breathing.Discussion and Conclusion: The most effective pharmacological treatments for patients with viral croup are oral or intramuscular dexamethasone, and nebulized L-epinephrine. Especially, corticosteroids can significantly decrease the intensity of croup symptoms and reduce hospital admissions, return visits to emergency department and length of stay in the hospital.

Two decades of experience on ablation in children with Ebstein’s anomaly

Introduction: Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein’s anomaly. Methods and results: During the study period, of 89 patients diagnosed with Ebstein’s anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully. Conclusions: Ablation in patients with Ebstein’s anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

“Sack of marble” appearance in mature cystic teratoma: an unusual finding

Mature cystic teratoma (Dermoid) of ovary is a benign germ cell tumor consisting predominantly of ectodermal derivatives. In rare occasion sebaceous material may get aggregated to form marble like appearance because of continuous mobility of Dermoid and its content. Such a case in a menopausal woman has been presented in this report.

The enpp4 ectonucleotidase regulates kidney patterning signalling networks in Xenopus embryos

The enpp ectonucleotidases regulate lipidic and purinergic signalling pathways by controlling the extracellular concentrations of purines and bioactive lipids. Although both pathways are key regulators of kidney physiology and linked to human renal pathologies, their roles during nephrogenesis remain poorly understood. We previously showed that the pronephros was a major site of enpp expression and now demonstrate an unsuspected role for the conserved vertebrate enpp4 protein during kidney formation in Xenopus. Enpp4 over-expression results in ectopic renal tissues and, on rare occasion, complete mini-duplication of the entire kidney. Enpp4 is required and sufficient for expression of the pronephric markers and regulates the expression of RA, Notch and Wnt pathway members. Enpp4 is a membrane protein that binds, without hydrolyzing, phosphatidylserine and its effects are mediated by the receptor s1pr5, although not via the generation of S1P. Finally, we propose a novel and surprising non-catalytic mechanism by which lipidic signalling regulates nephrogenesis.

Lactoferrin amyloid presenting as a mural nodule in a pancreatic cystic lesion prompting pancreatoduodenectomy: a case report

Background Amyloid deposition in pancreas is rare. Lactoferrin amyloid deposition has not been reported in pancreas, till date. Presence of enhancing mural nodule in a cyst on imaging is a worrisome feature for malignancy, and warrants surgical resection in a surgically fit candidate, as per Fukuoka guidelines for management of cystic lesions in pancreas. Case report We report a case of localized amyloidosis presenting as a mural nodule in a 1.6 cm cyst located in the head of pancreas, which led to pancreatoduodenectomy in a 69 year old woman. Histological evaluation revealed a simple mucinous cyst with localized lactoferrin amyloid deposition corresponding to the mural nodule identified on imaging. Conclusions We report the first case of localized lactoferrin amyloid deposition in pancreas that presented as a mural nodule in a cystic lesion and prompted pancreatoduodenectomy. This unique case illustrates that on rare occasion mural nodule in a cyst can be benign. It adds amyloid deposition to the differential diagnosis of mural nodules in pancreatic cystic lesions seen on imaging.

MYOCARDIAL INFARCTION AT YOUNG ADULTS – RISK FACTORS AND MANAGEMENT

Introduction: Generally, the myocardial infarction is far more common among the population which is over 45 years. However, on rare occasion it can be seen amongst the younger population such as the above discussed case. Objective: The report from the case of this overview shows an acute myocardial infarction in a young 28-year-old man, due to thrombotic occlusion that has nothing to do with coagulation problems. Methods: The Coronary Angiography is of paramount importance when it comes to reaching a correct diagnosis, and by extension, treatments for such cases. Moreover, the detailed clinical case history further aided in determining the key risk factors. Results: When it ensues in younger age, the myocardial infarction is associated with significant morbidity, psychological effects and financial limits to the individual and his or her family. Conclusions: In the examined case we described the contemporary management of a myocardial infraction event, wherein the patient was advised to stop smoking, form a consistent regular exercising physical activity regime, put more conscious effort into having a healthy diet, and reduce stress levels as much as possible.