Incidence and risk factors of bacterial sepsis and invasive fungal infection in neonates and infants requiring major surgery: an Italian multicentre prospective study.

BACKGROUND: Limited data are currently available on the incidence rates and risk factors for bacterial sepsis and invasive fungal infections (IFIs) among neonates and infants undergoing major surgery. AIM: To assess the incidence of bacterial sepsis and IFI, fungal colonization, risk factors for sepsis, and mortality in neonates and infants aged <3 months undergoing major surgery. METHODS: A multicentre prospective study was conducted involving 13 level-3 neonatal intensive care units in Italy, enrolling all infants aged ≤3 months undergoing major surgery. FINDINGS: From 2018 to 2021, 541 patients were enrolled. During hospitalization, 248 patients had a bacterial infection, and 23 patients had a fungal infection. Eighty-four patients were colonized by fungal strains. Overall, in-hospital mortality was 2.8%, but this was higher in infected than in uninfected infants (P = 0.034). In multivariate analysis, antibiotic exposure before surgery, ultrasound-guided or surgical placement of vascular catheters, vascular catheterization duration, and gestational age ≤28 weeks were all associated with bacterial sepsis. The risk of IFI was markedly higher in colonized infants (odds ratio (OR): 8.20; P < 0.001) and was linearly associated with the duration of vascular catheterization. Fungal colonization in infants with abdominal surgery increased the probability of IFI 11-fold (OR: 11.1; P < 0.001). CONCLUSION: Preventive strategies such as early removal of vascular catheters and the fluconazole prophylaxis should be considered to prevent bacterial and fungal sepsis in infants undergoing abdominal surgery, and even more so in those with fungal colonization.

Failure to thrive: the importance of measuring the electrolytes.

Diabetes insipidus (DI) is characterized by hypoosmotic polyuria related to deficiency of arginine-vasopressin (AVP) secretion (centraldiabetesinsipidus, CDI) or renalinsensitivity to AVP (nephrogenicdiabetesinsipidus, NDI). We report a case of a child with congenital NDI.

Congenital renal anomalies imaging: a valuable tool for pediatricians.

The purpose of this article is to review the main congenital anomalies of kidneys and urinary tract that can be diagnosed prenatally and postnatally by imaging technique. The incidence of congenital anomalies of the kidney and urinary tract during the past decade has been estimated to be 0.4 to 4.0 cases per 1000 births. Congenital kidney disease can evolve in chronic disease in childhood and in adulthood. A diagnostic imaging of the various congenital renal and urological conditions allows pediatricians to make a correct diagnosis and treatment. Because of the concerns about long-term effects of ionizing radiation, the most commonly and first used imaging modality for evaluation of the urinary system is ultrasound.

Pediatric urolithiasis.

Urolithiasis is a well-known condition that can affect any part of the urinary tract. With a rate of 3-5% the incidence of upper urinary tract for long has been higher in adults (1-3), but recently it has increased among children reaching 3,3% . Indeed, more than 1% of all urinary stones are seen in patients aged less than 18 years (4). Pediatric urolithiasis is endemic in Turkey and Far East and it is probably due to malnutrition and racial factors (5). The spontaneous stone passage is more likely in children than in adults, indeed ureteral calculi spontaneously pass into 41-63% of children (1). Rate of stone passage depends on size and stone location in the urinary system. Stones sized less than 5 mm have a passage rate ranging from 40% to 98%, whilst stones > 5 mm have between 55% and 50% (6). In the last decade, the use of alpha blockers has proven well efficacious in helping spontaneous passage of distal ureteric stones in adults (7-9). The latest EAU guidelines support their use in adults while remain vague about their use in children because of unclear safety and efficacy (4). In search of evidence supporting or not the use of medical expulsive therapy in children we reviewed the literature dealing with the management of urolithiasis in pediatric patients. The primary aim of the present study was to evaluate the efficacy of medical expulsive therapy (MET), defined as stone expulsion rate, with a-blockers compared to a control group. The secondary aim was to assess the safety, defined as side effects rate, of MET compared to a control group.

Alport's syndrome.

Alport's syndrome (AS, OMIM 301050) is a hereditary disorder characterized by progressive renal failure, hearing impairment and ocular changes. It is clinically and genetically heterogeneous and in its natural history, renal disease progresses from microscopic haematuria to proteinuria, and finally to progressive renal insufficiency. AS is caused by an inherited defect in a type IV collagen, a structural material, expressed in many tissues that is essential for the normal function of different parts of the body. In most of cases, about the 85%, Alport's syndrome is X-linked and is originated by mutations in the COL4A5 gene. In the remaining cases, it may be inherited in either an autosomal recessive, or rarely in an autosomal dominant manner. Mostly, the condition is caused by mutations in the COL4A3 or COL4A4 genes. Coexisting mutations in COL4A3, COL4A4, COL4A5 or COL4A6 were found to cause an Alport's syndrome phenotype with digenic inheritance. Diagnosis of the condition is based on family history, clinical signs, and specific procedures such as a kidney biopsy. The diagnosis can be confirmed by genetic testing. Treatment may include use of a hearing aid, hemodialysis, and peritoneal dialysis to treat those with end-stage renal failure, and, as the last step, kidney transplantation. Firstly described by Arthur C. Alport's, in 1927, over the years it has become a pathology of high scientific interest. At the moment, thanks to advances in diagnostic techniques, it is possible to make an early diagnosis avoiding irreversible damages and life -threatening complications.

Monosymptomatic enuresis: the therapeutic weapons.

Nocturnal enuresis (NE) was defined by the World Health Organization (ICD-10) and the American Psychiatric Association (DSM-5) as bed-wetting in children aged >5 years. In cases of mental retardation, the developmental age may be equivalent to 5 years. In this review, we focus on the current knowledge about the etiology of enuresis and the most recent therapeutical options. Both non-pharmacological and pharmacological therapies are included, although the relative effectiveness of each remains uncertain. To date, motivational, alarm and drug therapies are the mainstay of treatment. Alarm therapy remains the first-line treatment modality for NE, while desmopressin is the most commonly used medical treatment.

Renal involvement in paediatric Fabry disease.

Anderson-Fabry Disease (AFD) is a rare, X-linked inborn error of glycosphingolipid catabolism caused by a deficient or absent activity of the lysosomal enzyme, α-galactosidase A, resulting in the progressive multisystem lysosomal accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3). Among the wide spectrum of clinical signs and symptoms and the life-threatening complications of Fabry disease, renal failure causes significant morbidity and mortality. Various evidence shows that the accumulation of Gb3 in different renal cells is present since the first years of life, many years and usually decades before manifest symptoms and signs of renal involvement. Early renal damage can be demonstrated by clinical signs as microalbuminuria and proteinuria, developing as early as in the second decade of life. A decline in GFR is uncommon at paediatric ages but may be seen as early as adolescence. Renal biopsy is rarely used in paediatric patients with Fabry disease although evidence shows that it may be considered a valid tool for the diagnosis of early and potentially reversible nephropathy, as well as for the evaluation of the effectiveness of enzyme replacement therapy (ERT). Although there is consensus in considering the early initiation of ERT as the only tool able to prevent the progression of nephropathy, the issue on the correct timing for the onset of ERT in pediatric age remains open in the management of this chronic and progressive disease.

Conservative management in congenital severe bilateral hydronephrosis related to uretero-pelvic junction obstruction.

We report our experience in conservative management of patients with prenatal and neonatal diagnosis of severe bilateral ureteropelvic junction obstruction (UPJO), focusing on the actual predictors of renal function impairment or spontaneous resolution. Between 1996 and 2006, 20 patients with bilateral severe hydronephrosis related to UPJO were included in the study. Indications for surgery were an increased hydronephrosis, decreased renal function, onset of symptoms. Conservatively treated patients were followed up for 3 months to 10 years with renal ultrasound, DTPA diuretic, urine culture. At first renal scan, 22 out of 40 renal units had a poor, 10 an intermediary and 8 a good drainage. Pyeloplasty was required in 10 of the 40 kidneys, while 30 out of 40 kidneys were followed conservatively. At the end of follow up, sieric normalized creatinine and estimated glomerular filtration rate were normal in all patients. Our data showed that bilateral severe hydronephrosis related to UPJO can be safely managed in a similar manner of a unilateral case. A poor drainage could be considered a negative predictive factor in the feasibility of a conservative management.

Ureterocystoplasty (bladder augmentation) in a 16 year-old boy with Goldenhar syndrome.

The use of the dilated ureter for bladder augmentation is universally accepted for its lower rate of complications compared to the use of gastrointestinal segments. We report the case of a 16 yearold boy affected by Goldenhar syndrome who presented with neurogenic bladder with small-capacity, 5° grade vescico-ureteral reflux (VUR) with megaureter and bilateral hydronephrosis. Bladder augmentation using the distal dilated ureter, transuretero-ureterostomy left to right and Mitrofanoff's appendicovescicostomy were performed. Six months after surgery voiding cystourethrogram (VCUG) revealed a compliant bladder with a functional capacity of 400 ml. Ureterocystoplasty is a safe and effective method of augmenting small capacity urinary bladder. We suggest using the ureter, when available, instead of using gastrointestinal segments.

Cardiac dysfunction in children with essential obesity: preliminary data.

Obesity in children has been recognized as a major underlying factor of the pathogenesis of several diseases and a reduced life expectancy. This study aims to verify if clinical parameters, such as waist circumference and/or body mass index and biohumoral and inflammatory parameters can help predict cardiac structural and functional alterations, through an echocardiogram test in obese children and adolescents. Children were prospectively enrolled at the AUOC outpatients' department of Emergency Paediatrics, University Hospital, Messina, from June to December 2017. Clinical, metabolic parameters and an inflammation marker (HMGB1) were evaluated and a transthoracic echocardiogram was carried out. Twenty-two obese subjects were prospectively enrolled.HMGB1 values were 12.6 ± 2ng/ml, significantly higher compared to a previously studied healthy control group. A significant positive correlation was found both between total cholesterol levels and HMGB1 values (r=0.846, p=0.000) and between LDL cholesterol and HMBG1 values (r=0.663, p=0.001). No correlation was found between clinical, biohumoral and echocardiograph parameters. In obese children cardiac parameters obtained from echocardiogram tests may be in the normal range. However, other parameters may be altered in the early phase, showing that infantile obesity can compromise myocardial functions, even in the absence of comorbidities. Furthermore, the evaluation of concentrations of HMBG1 could explain how an initial inflammation can trigger the condition of meta-inflammation.

Hemodialysis in children: how, when and why.

End-stage renal diseases requiring chronic dialysis are rare in childhood and adolescence, but they are associated with high mortality and impaired quality of life (1, 2). The most common disease that causes chronic kidney disease (CKD) is primary glomerular disease (GD), followed by congenital abnormalities of the kidney and urinary tract, cystic, hereditary or congenital disorders and, more rarely, secondary GD. However, patients with secondary GD, urologic disorders, and metabolic diseases have greater mortality risk than patients with primary GD (3). Here, we focused on the different options of treatment available, and specifically we compared peritoneal dialysis and hemodialysis, showing pros and cons between them.

Laparoscopic transposition of lower pole crossing vessels in children with extrinsic pelvi-ureteric junction obstruction: a worthy alternative to dismembered pyeloplasty.

Pelviureteric junction obstruction (PUJO) due to intrinsic or extrinsic causes is a common problem in childhood. Extrinsic compression by a lower pole-crossing blood vessel can present symptomatically in older children. In these cases, laparoscopies Vascular Hitch can represent a valid alternative to pyeloplasty dismembered. We analyzed the data of 4 children affected by extrinsic PUJO treated at our institution with the laparoscopic Vascular Hitch procedure modified by Chapman. Surgical indications included presence of clinical symptoms, worsening of intermittent hydronephrosis, signs of obstruction on the MAG-3 scan, clear or suspected images of polar crossing vessels on CT scan or Uro-MRI. All procedures were completed laparoscopically. No complications occurred. Mean follow-up was 13 months with resolution of symptoms and PUJ obstruction and significant improvement of hydronephrosis in all cases. When blood vessels crossing lower pole represent the pure mechanical cause of UPJ obstruction the laparoscopic Vascular Hitch procedure represents an excellent alternative to dismembered pyeloplasty. It is less technically demanding then pyeloplasty and is associated with a lower complication rate. The main challenge is to intraoperatively ascertain the absence of associated intrinsic stenosis.

Laparoscopic nephrectomy in children with Wilms tumor. Considerations after 10 years of experience.

Despite laparoscopy in children is considered safe and is routinely used for several procedures, even in neonates and in pediatric oncology, its role in the treatment of pediatric renal tumors is still controversial. This study analyzes the results of laparoscopic nephrectomy for Wilms Tumor (WT) in pediatric age compared with open nephrectomy after 10 years of experience in a single centre. From 1993 in our center of reference for pediatric oncology, 30 patients with WT have been treated. We performed 21 open nephrectomy and in the last 10 years 9 laparoscopic nephrectomy. In all patients treated laparoscopically, the same technique made by the same equip was used. Compared with patients treated by open surgery, we did not find a significant difference in terms of outcome and survival. In the open surgery group, two patients had lung relapse while in the other group there was one local relapse. These three children obtained and maintained a second complete remission with chemotherapy. Open surgery complications were a tumor rupture in two cases, and an episode of pancreatitis 10 days after surgery. In the laparoscopic group, there were two conversions to open surgery not considered as complications but a surgical choice for cystic areas present in the tumor. As far as complications and oncologic outcomes are concerned, both techniques showed similar results. In experienced hands, laparoscopy proves to be an attractive alternative to open surgery for pediatric renal tumors.

Post-rotavirus vaccine intussusception in identical twins: A case report.

The intussusception is one of the most frequent causes of occlusive syndrome in infants and in children. (1) The mesenteric lymphadenopathy, wich is very rare post rotavirus vaccination, can cause intussusception, (2-5) especially in genetically predisposed individuals. (6) There is an association between intussusception and some classes of genotype. (7-9) Two infants aged 3 months, vaccinated against rotavirus. After about a week, one of the 2 identical infants presented inconsolable crying, vomiting, loose stools mixed with blood, and was diagnosed with bowel obstruction with intussusception. He was operated in urgency. After a few hours, his brother presented vomiting, and was admitted to our Hospital for suspected intussusception. The controls carried out have confirmed the presence of intussusception that was treated early, before the onset of severe symptoms. The incidence of post rotavirus vaccine intussusception is very low. The determining factor hypothetically might be linked to the presence of a genotype that exposes infants to a greater risk of developing mesenteric lymphadenitis and intussusception. In our case, the diagnosis of intussusception occurred in a twin, which allowed us to recognize early symptoms which accused the brother and schedule the surgery with less urgency. Our experience may want to sensitize families and pediatricians to report cases of intussusception given a theoretical familiar association. The study of the genotype could be decisive for or not to exclude the presence of a risk of invagination, thus avoiding vaccination.

Isolated pancreatic metastasis from melanoma. Case report.

Pancreas is frequently site of isolated metastasis, approximately in the 40% of cases in patient with previous history of malignant neoplasia, more frequently from renal cell carcinoma. The melanoma metastasis can also interest the pancreas in case of disseminated disease (50% of the cases); more rarely the pancreas is site of isolated metastases from melanoma. The treatment of the pancreatic metastases from melanoma is controversial: the therapeutic choices are few and the role of surgery is not well defined. If the metastasis are confined to the pancreas, the surgical treatment can be useful for better long time survival. We report a rare case of melanoma with pancreatic isolated metastasi in a patient with a previous melanotic metastasis to the inguinal lymph nodes without evidence of primitive tumor.

Management of "open" syringocele based on urodynamic findings.

INTRODUCTION: Depending on the type and size of the syringocele and the age of the patient, syringocele is treated medically or surgically, with endoscopy or open surgery. We report our experience in 10 "open" consecutive cases of syringocele, propose a clinical classification and discuss the management. MATERIAL AND METHODS: In all patients (pts), diagnosis was achieved through voiding cystourethrography. All pts performed multichannel urodynamic studies. Pts with impaired compliance and/or detrusor instability (4 pts out of 10) underwent endoscopic unroofing. They were followed up until to 24 months after the endoscopic procedure. Pts with normal urodynamic findings were treated medically and followed with clinical examination. All pts performed an urinalysis every 2 weeks for the first three months and monthly for an year. RESULTS: In endoscopic treated pts, voiding cystourethrography showed a normal profile of the urethra at 3 months follow-up. Pts with UTI, but with normal urodynamic parameter were treated with antibiotic therapy. At serial follow-up, all pts were completely symptom-free. Urinalysises were normal, negative for infection or haematuria. DISCUSSION: In the literature to date, there isn't accordance if and when treated "open" syringoceles. In our opinion, it is useful to classify "open" syringocele based on urodynamic findings. Syringocele needs endoscopic surgical treatment if it is obstructed or a cause of dysfunctional alteration of the bladder, in order to avoid unnecessary surgery in syringocele without urodynamic abnormality.

The value of postmortem retrievals in the analysis of long-term, well-functioning cemented femoral stems.

Multiple sections from long-term, low-friction bilateral Charnley prostheses harvested from two patients who died 20 and 22 years after the primary implantation were studied. All sections showed generally sound mechanical integrity at both the cement-bone and cement-metal interfaces, in spite of localized evidence of cement fracture and debonding. The host tissues showed no clear evidence of an adverse biologic response toward the implant. These findings aided in the delineation of the sequence of biologic and mechanical events that lead to prosthesis failure. They suggest that if the apparently unavoidable mechanical deterioration of cemented implants can be limited by good fixation between the host and prosthesis, then disastrous biologic events, such as osteolysis, may be avoided, and long-term function, preserved.