Crohn's disease and Takayasu's arteritis: An uncommon association

Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both diseases could share some common etiologic origin.We describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory markers.Clinical and histological features from colonic specimens were consistent with CD.Treatment with steroids and azathioprine was started,however disease flared every time steroids were tapered.One year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and weakness.At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic aneurysm.TA associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo follow-up.We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.

Appendicitis in children less than five years old:A challenge for the general practitioner

Acute appendicitis is one of the most common indications for abdominal surgery in pediatrics with peak incidence in the second decade of life. Acute appendicitis in the first years of life is an uncommon event. The clinical presentation is often varied and the diagnosis may be overshadowed by other medical conditions.Gastroenteritis is the most common misdiagnosis, with a history of diarrhea present in 33% to 41% of patients. Pain is the most common presenting symptom in children less than 5 years old, followed by vomiting, fever, anorexia and diarrhea. The most common physical sign is focal tenderness(61% of the patients) followed by guarding(55%), diffuse tenderness(39%), rebound(32%), and mass(6%). Neonatal appendicitis is a very rare disease with high mortality; presenting symptoms are nonspecific with abdominal distension representing the main clinical presentation. The younger the patient, the earlier perforation occurs: 70% of patients less than 3 years develop a perforation within 48 h of onset of symptoms. A timely diagnosis reduces the risk of complications. We highlight the epidemiology, pathophysiology, clinical signs and laboratory clues of appendicitis in young children and suggest an algorithm for early diagnosis.

Role of tristetraprolin phosphorylation in paediatric patients with inflammatory bowel disease

BACKGROUND Intestinal inflammation and epithelial injury are the leading actors of inflammatory bowel disease(IBD),causing an excessive pro-inflammatory cytokines expression.Tristetraprolin(TTP),an mRNA binding protein,plays a role in regulating the inflammatory factors,recognizing specific sequences on the 3’untranslated region of cytokine mRNAs.TTP activity depends on its phosphorylation state:the unphosphorylated TTP degrades pro-inflammatory cytokine mRNAs;on the contrary,the phosphorylated TTP fails to destabilize mRNAs furthering their expression.The phospho-TTP forms a complex with the chaperone protein 14-3-3.This binding could be one of the factors that promote intestinal inflammation as a cause of disease progression.AIM To assess if TTP phosphorylation has a role in paediatric IBD.METHODS The study was carried out on a cohort of paediatric IBD patients.For each patient enrolled,a specimen of inflamed and non-inflamed colonic mucosa was collected.Furthermore,the experiments were conducted on macrophages differentiated from blood samples of the same patients.Macrophages from healthy donors’blood were used as controls.Co-immunoprecipitation assay and immunoblotting analyses were performed to observe the formation of the phospho-TTP/14-3-3 complex.In the same samples TNF-αexpression was also evaluated as major factor of the pro-inflammatory activity.RESULTS In this work we studied indirectly the phosphorylation of TTP through the binding with the chaperone protein 14-3-3.In inflamed and non-inflamed colon mucosa of IBD paediatric patients immunoblot assay demonstrated a higher expression of the TTP in inflamed samples respect to the non-inflamed;the coimmunoprecipitated 14-3-3 protein showed the same trend of expression.In the TNF-αgene expression analysis higher levels of the cytokine in inflamed tissues compared to controls were evident.The same experiments were conducted on macrophages from IBD paediatric patients and healthy controls.The immunoblot results demonstrated a high expression of both TTP and co-immunoprecipitated 14-4-3 protein in IBD-derived macrophages in comparison to healthy donors.TNF-αprotein levels from macrophages lysates showed the same trend of expression in favour of IBD paediatric patients compared to healthy controls.CONCLUSION In this work,for the first time,we describe a relation between phospho-TTP/14-3-3 complex and IBD.Indeed,a higher expression of TTP/14-3-3 was recorded in IBD samples in comparison to controls.

Acute lobar nephritis in children: Not so easy to recognize and manage

Acute lobar nephritis(ALN) is a localized non-liquefactive inflammatory renal bacterial infection, which typically involves one or more lobes. ALN is considered to be a midpoint in the spectrum of upper urinary tract infection, a spectrum ranging from uncomplicated pyelonephritis to intrarenal abscess. This condition may be difficult to recognize due to the lack of specific symptoms and laboratory findings. Therefore the disease is probably underdiagnosed. Computed tomography scanning represents the diagnostic gold standard for ALN, but magnetic resonance imagine could be considered in order to limit irradiation. The diagnosis is relevant since initial intravenous antibiotic therapy and overall length of treatment should not be shorter than 3 wk. We review the literature and analyze the ALN clinical presentation starting from four cases with the aim to give to the clinicians the elements to suspect and recognize the ALN in children.

Sedation and analgesia in children with cerebral palsy:a narrative review

Background Patients with cognitive impairment due to cerebral palsy experience pain more often than healthy peers and frequently require diagnostic and therapeutic painful procedures.Analgesia and procedural sedation outside the operating room are often required,but they may not adequately be provided because of the inability to accurately recognize and classify the state of pain and for the perceived higher risk of complications.Data sources We reviewed the available literature to highlight the specific risk factors and area of criticism,that should be further improved.We searched the Cochrane Library,Medline,Pubmed from 1987 to September 2018 using key words such as'cerebral palsy and children and pain'or'sedation and cerebral palsy and children'.Results While different pain scales are useful in recognizing pain expressions,anxiety scales are not available.Moreover,studies on non-pharmacological techniques do not always have comparable results.Several risk factors,from anatomic abnormalities to liver and kidney functioning,should be kept in mind before proceeding with sedation.Conclusions Large trials are needed to assess the impact of non-pharmacological techniques and to evaluate which pain control strategy(pharmacological and non-pharmacological)should be used in different settings.

Neonatal acute scrotum:do not forget Amyand hernia

A 15-day old full-term newborn was admitted to the emergency department for right scrotal swelling,irritability and poor feeding that started 12 hours earlier,without fever or vomiting.Parents reported that his last defecation occurred 3 days before admis-sion.Physical examination was remarkable for a swollen,erythematous and tender right hemiscrotum(figure 1).