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Skin lesions are frequently observed in children with rheumatic diseases, particularly in conditions such as IgA vasculitis (IgAV) and Kawasaki disease (KD). In paediatric vasculitis, the presence of skin lesions serves as an early indicator, emphasising the importance of timely diagnosis to prevent complications, such as cardiac or renal involvement. Conversely, autoinflammatory disorders like juvenile systemic lupus erythematosus (SLE) and juvenile dermatomyositis (DM) may manifest with cutaneous manifestations either at the onset of disease or during its progression. Identifying these skin lesions prior to the appearance of systemic symptoms offers an opportunity for early diagnosis and treatment, which has a positive influence on the outcomes. Additionally, it is noteworthy that specific rheumatological conditions, such as acute rheumatic fever (ARF) or oligoarticular or polyarticular forms of juvenile idiopathic arthritis (JIA), may exhibit occasional, but significant skin involvement, which is strongly correlated with an unfavourable prognosis. The assessment of skin is important in the holist approach to assessing patients for potentially systemic/multisystem disorder and helps distinguish discrete conditions.

This study, conducted by searching keywords such as "maternal lupus", "neonatal lupus", and "congenital heart block" in databases including PubMed and Scopus, provides a detailed narrative review on fetal and neonatal lupus. Autoantibodies like anti-Ro/SSA and anti-La/SSB may cross the placenta and cause complications in neonates, such as congenital heart block (CHB). Management options involve hydroxychloroquine, which is able to counteract some of the adverse events, although the drug needs to be used carefully because of its impact on the QTc interval. Advanced pacing strategies for neonates with CHB, especially in severe forms like hydrops, are also assessed. This review emphasizes the need for interdisciplinary care by rheumatologists, obstetricians, and pediatricians in order to achieve the best maternal and neonatal health in lupus pregnancies. This multidisciplinary approach seeks to improve the outcomes and management of the disease, decreasing the burden on mothers and their infants., Competing Interests: The authors declare no conflict of interest. No part of the review, including its graphics, are copied or published elsewhere in whole or in part.

Background: Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) syndrome is a rare pediatric disorder consisting of a sudden onset of obsessive-compulsive disorder (OCD) and/or tics after a group A Streptococcus (GAS) infection., Methods: In the period between 2013 and 2023, 61 children presented to our Pediatric Rheumatology unit with a suspicion of PANDAS syndrome. Among these, a retrospective analysis was conducted, and 19 fulfilled the current classification criteria and were included in this study., Results: The male-to-female ratio was 14:5, the median age at onset was 7.0 (2.0-9.5) years, and the median age at diagnosis was 8.0 (3.0-10.4) years. The median follow-up period was 16.0 (6.0-72.0) months. Family and personal history were relevant in 7/19 and 6/19 patients. Tics were present in all patients. Details for motor tics were retrospectively available in 18/19 patients, with the eyes (11/18) and neck/head (10/18) being most often involved. Vocal tics were documented in 8/19, behavioral changes in 10/19, and OCD in 2/19. Regarding the therapeutic response, all patients responded to amoxicillin, 12/13 to benzathine benzylpenicillin, and 7/9 to azithromycin., Conclusions: Our findings partially overlap with previous reports. Larger prospective studies are needed to improve treatment strategies and classification criteria.

Background: Hereditary spastic paraplegia (HSP) encompass a variety of neurodegenerative disorders that are characterized by progressive deterioration of walking ability and a high risk for long-term disability. The management of problems associated with HSP, such as stiffness, deformity, muscle contractures, and cramping, requires strict adherence to recommended physiotherapy activity regimes. The aim of this paper is to conduct a critical narrative review of the available evidence focusing exclusively to the therapeutic advantages associated with various forms of physical therapy (PT) in the context of HSP, emphasizing the specific benefit of every distinct approach in relation to muscle relaxation, muscle strength, spasticity reduction, improvement of weakness, enhancement of balance, posture, walking ability, and overall quality of life., Methods: To conduct a literature review, the databases PubMed, Scopus, and DOAJ (last access in June 2023) were searched., Results: The PubMed search returned a total of 230 articles, Scopus returned 218, and DOAJ returned no results. After screening, the final list included 7 papers on PT treatment for HSP patients., Conclusion: Electrostimulation, magnetotherapy, hydrotherapy, PT, robot-assisted gait training, and balance rehabilitation have the potential to increase lower extremity strength and decrease spasticity in HSP patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Di Ludovico, Ciarelli, La Bella, Scorrano, Chiarelli and Farello.)

Drug-Induced Enterocolitis Syndrome (DIES) is a drug-induced hypersensitivity reaction non-IgE mediated involving the gastrointestinal system that occurs 2 to 4 h after drug administration. Antibiotics, specifically amoxicillin or amoxicillin/clavulanate, represent the most frequent drugs involved. Symptoms include nausea, vomiting, abdominal pain, diarrhea, pallor, lethargy, and dehydration, which can be severe and result in hypovolemic shock. The main laboratory finding is neutrophilic leukocytosis. To the best of our knowledge, 12 cases of DIES (9 children-onset and 3 adult-onset cases) were described in the literature. DIES is a rare clinically well-described allergic disease; however, the pathogenetic mechanism is still unclear. It requires to be recognized early and correctly treated by physicians.

A Nasal Provocation Test allows the differentiation of allergic and non-allergic rhinitis, but it is difficult and expensive. Therefore, nasal cytology is taking hold as an alternative. We carried out a cross-sectional study, including 29 patients with persistent rhinitis according to ARIA definition and negative skin prick tests. Nasal symptoms were scored from 0 to 5 using a visual analogue scale, and patients underwent blood tests to investigate blood cell count (particularly eosinophilia and basophilia), to analyze serum total and specific IgE and eosinophil cationic protein (ECP), and to perform nasal cytology. We performed a univariate logistical analysis to evaluate the association between total serum IgE, serum eosinophilia, basophils, and ECP and the presence of eosinophils in the nasal mucosa, and a multivariate logistic model in order to weight the single variable on the presence of eosinophils to level of the nasal mucosa. A statistically significant association between serum total IgE levels and the severity of nasal eosinophilic inflammation was found (confidence interval C.I. 1.08-4.65, odds ratio OR 2.24, p value 0.03). For this reason, we imagine a therapeutic trial with nasal steroids and oral antihistamines in patients with suspected LAR and increased total IgE levels, reserving nasal cytology and NPT to non-responders to the first-line therapy.

The clinical, functional, and structural pattern of chronic lung disease of prematurity has changed enormously in last years, mirroring a better perinatal management and an increasing lung immaturity with the survival of increasingly premature infants. Respiratory symptoms and lung function impairment related to prematurity seem to improve over time, but premature birth increases the likelihood of lung function impairment in late childhood, predisposing to chronic obstructive pulmonary disease (COPD). It is mandatory to identify those individuals born premature who are at risk for developing long-term lung disease through a better awareness of physicians, the use of standardized CT imaging scores, and a more comprehensive periodic lung function evaluation. The aim of this narrative review was to provide a systematic approach to lifelong respiratory symptoms, lung function impairment, and lung structural anomalies in order to better understand the specific role of prematurity on lung health.

Hepatic cysts have become increasingly frequent findings thanks to the improvement in diagnostic investigations. Distinction has to be made between congenital hepatic cysts (like liver cyst, PLCD) and acquired forms (such as a parasitic cyst and a cyst occurring as part of a neoplastic process) (1). When a simple hepatic cyst becomes symptomatic, when its size is > 4 cm or when there is some radiological suspicion of malignancy (thick wall, peripheral enhancement on CT/MRI) surgical management is indicated and relies on a variety of techniques (2). Presently the two most common techniques are percutaneous aspiration with sclerotherapy and laparoscopic fenestration. The use of laparoscopic approach has achieved, in the last years, some great results, for it shortens hospital stay, involves minimal invasiveness and offers low recurrence rate. We report three cases of symptomatic hepatic cysts successfully treated by using laparoscopic procedure. KEY WORDS: Laparoscopic deroofing, Sclerotherapy, Simple hepatic cysts.

Cystic intestinal pneumatosis (CIP) is the presence of gas bubbles inside the wall of the intestine. In literature it has been reported to be associated with a variety of clinical conditions such as: superior mesenteric ischemia, intestinal perforation, bowel necrosis, infections which can cause mucosal alterations and therefore increasing its permeability, necrotizing enterocolitis in babies, trauma, intestinal obstruction, autoimmune and pulmonary pathologies. Its presence is usually documented by radiological techniques such as abdominal X-ray, CT scan and endoscopy and are usually characterized by the typical pattern of gas bubbles in the wall of the intestine. Majority of cases may be managed conservatively but intramural gas resulting from bowel necrosis represents a surgical emergency. We admitted an 86- year old man who presented with mild to moderate and persistent non-specific abdominal pain recurring for the last three weeks. No history of previous surgery was recorded. On examination, bowel sounds were dull and rare, general tenderness on the left of the abdomen but no particular resistance was noted, breath sounds were reduced and right hemi-thorax had reduced expansion as well. The patient was dehydrated and no central or peripheral neurological deficits were observed. Chest X-ray was performed and showed the presence of free air beneath the diaphragm. A complete abdominal CT scan was then done and documented the presence of pneumoperitoneum and multiple cystic like structures containing air in the right peri-splenic and para-renal regions. Laparotomy was then done for suspected perforation of the gut. The small gut was entangled with adhesions and presence of cystic structures with bubbles of air along the wall of the gut. The affected part of the small gut was then resected. The histology result confirms the presence of cystic pneumatosis of the small gut without any sign of intestinal wall perforation., Key Words: Cystic pneumatosis, Pneumoperitoneum, Radiological investigations, Surgical approach.

Morgagni-Larrey hernia is uncommon congenital disease in the adults and presents with mild to severe clinical symptoms. In literature, about 80% of Morgagni -Larrey hernia are found on the right side of the chest cavity and have usually a peritoneal sack. Only 2-3% of patients at birth are symptomatic and therefore eligible for urgent surgery. Less severe forms of the disease are characterized by persistent epigastric and subcostal pains sometimes associated with vomiting and are frequently mistaken for dyspeptic disturbances. We present two case reports; the first one is a patient of 74 years who presented with persistent epigastric pain, vomiting, slight to moderate dyspnea on exertion associated with tachycardia, tachypnea and dyspeptic symptoms. Such symptoms have been going on for the last 6 months. The second patient is a 90 year old woman who was admitted in our ward for abdominal pain and distension associated with vomiting. In both cases a Morgagni-Larrey diaphragmatic hernia was discovered by using esophagogastroduodenoscopy, gastrographyn swallow and CT scan in the first case and only CT scan in the second one., Key Words: Morgagni-Larrey hernia, Radiological investigations, Symptoms in adults, Surgical approach.

Background: Although associated with certain advantages, laparoscopic repair of inguinal hernias implies longer operative times, increased intra-abdominal injuries, and a higher rate of urinary retention. To address these issues, we developed the transabdominal rivet technique (TART) for laparoscopic inguinal hernia repair., Methods: Three patients underwent bilateral inguinal hernia repair: two underwent TART only, and one underwent a standard transabdominal preperitoneal technique on one side and TART on the other for comparison. TART was performed using a round ring of mesh connected to two non absorbable polyethylene straps; the ring closed the gap in the abdominal wall, and the straps were passed through the defect and secured externally., Results: The average operative time was 12 minutes per side, and the average hospital stay was 22.3 hours. No patients required analgesics. Urinary retention occurred in one patient with preexisting prostatitis and was not thought to have been the result of the procedure. Good mesh positioning was observed by computed tomography at 2 months, and no recurrence was seen 6 months after the operation., Conclusions: Based on the results of this preliminary study, TART is a safe, rapid, feasible procedure for treatment of inguinal hernia repair. This study serves as a foundation for further research using a larger sample size.