Activity-restriction only as treatment yields positive outcomes in pediatric spinal compression fractures: a prospective study of 47 patients at medium-term follow-up.

BACKGROUND AND PURPOSE: We aimed to evaluate the clinical outcomes, radiographic results, and health-related quality of life in pediatric AO type A1 spinal compression fractures treated with activity-restriction only. PATIENTS AND METHODS: All children between 2014 and 2020 with an AO type A1 spinal compression fracture treated with activity-restriction only identified from an institutional fracture registry were invited to a prospective outcomes study. Clinical findings and spinal radiographs were assessed at median 3 years, interquartile range (IQR) 1-4 follow-up from injury. Oswestry Disability Index, Pediatric Quality of Life Inventory Generic Core Scale (PedsQL), and PedsQL Pediatric Pain Questionnaire were compared with reference values. 63 children were identified, of whom 47 agreed to participate. 8 were polytrauma patients. RESULTS: Age at injury was median 11 (IQR 9-14) years. The number of injured vertebrae was median 2 (IQR 1-3). 82% (94 of 115) were thoracic vertebrae fractures and 70% (33 of 47) of the patients had thoracic vertebrae fractures only. At follow-up all but 2 fractures showed radiographic remodeling. There was no difference from the published reference values in the patient-reported outcome measures. A lower PedsQL physical functioning score was associated with higher patient-reported pain (P = 0.03). At follow-up 12 patients had hyperkyphosis (median difference from the reference values 4°, IQR 3-6, 95% confidence interval [CI] 3-6) and 5 hypolordosis (median difference from reference 8°, IQR 4-11, CI 4-14). None of the patients had surgery for deformity during follow-up. CONCLUSION: Clinical, radiographic, and health-related quality of life outcomes were good after activity-restriction treatment in pediatric A1 spinal compression fractures.

Characteristics, management and outcomes of choledochal malformations in Finnish adult patients.

CONCLUSIONS: Nearly half of operated patients developed long-term postoperative complications. A novel association between CMs and IBD was observed. Although no hepatobiliary malignancies regardless of treatment modality were encountered, the number of patients and length of follow-up remained limited.

Cutoffs and Characteristics of Abnormal Bowel Dilatation in Pediatric Short Bowel Syndrome.

OBJECTIVES: Although excessive intestinal dilatation associates with worsened outcomes in pediatric short bowel syndrome (SBS), little is known about the natural history and definition of pathological dilatation. We addressed fore-, mid-, and hind-gut dilatation in children with SBS, who had not undergone autologous intestinal reconstructive (AIR) surgery, in relation to controls. METHODS: SBS children without history of AIR surgery (n = 59) and age-matched controls without any disclosed intestinal pathology (n = 140) were included. Maximum diameter of duodenum, small bowel (SB), and colon were measured in each intestinal contrast series during 2002 to 2020 and expressed as diameter ratio (DR) to L5 vertebrae height. Predictive ability of DR for weaning off parenteral nutrition (PN) was analyzed with Cox proportional hazards regression models using multiple cutoffs. RESULTS: Duodenum (DDR), SB (SBDR), and colon (CDR) DR were 53%, 183%, and 23% higher in SBS patients compared to controls ( P < 0.01 for all). The maximal DDR and SBDR measured during follow-up is associated with current PN dependence and young age. DDR correlated with SBDR ( r = 0.586, P < 0.01). Patients with maximal DDR less than 1.5, which was also the 99th percentile for controls, were 2.5-fold more likely to wean off PN ( P = 0.005), whereas SBDR and CDR were not predictive for PN duration. CONCLUSIONS: All segments of remaining bowel, especially SB, dilate above normal levels in children with SBS. In SBS children without AIR surgery, PN dependence and young age is associated with duodenal and small intestinal dilatation, while duodenal dilatation also predicted prolonged PN.

Early disc degeneration in radiotherapy-treated childhood brain tumor survivors.

BACKGROUND: Childhood brain tumor (BT) survivors have an increased risk of treatment-related late effects, which can reduce health-related quality of life and increase morbidity. This study aimed to investigate lumbar disc degeneration in magnetic resonance imaging (MRI) in adult survivors of radiotherapy-treated childhood BT compared to age and sex-matched population controls. METHODS: In this cross-sectional comparative study, 127 survivors were identified from hospital registries. After a mean follow-up of 20.7 years (range 5-33.1), 67 survivors (mean age 28.4, range 16.2-43.5) were investigated with MRI and compared to 75 sex-matched population-based controls. Evaluated MRI phenotypes included Pfirrmann grading, , intervertebral disc protrusions, extrusions, and high-intensity-zone-lesions (HIZ). Groups were also compared for known risk factors of lumbar intervertebral disc (IVD) degeneration. RESULTS: Childhood BT survivors had higher Pfirrmann grades than controls at all lumbar levels (all p < 0.001). Lumbar disc protrusions at L4-5 (p = 0.02) and extrusions at L3-4 (p = 0.04), L4-5 (p = 0.004), and L5-S1 (p = 0.01) were significantly more common in the BT group compared to the control. The survivor cohort also had significantly more HIZ-lesons than the controls (n=13 and n=1, p=0.003). Age at diagnosis was associated with lower degree of IVD degeneration (p < 0.01). Blood pressure correlated with IVD degeneration (P < 0.05). CONCLUSIONS: Signs of early disc degeneration related to tumor treatment can be seen in the IVDs of survivors. Disc degeneration was more severe in children treated in adolescence.

Liver pathology and biochemistry in patients with mutations in TRIM37 gene (Mulibrey nanism).

BACKGROUND AND AIMS: Mulibrey nanism (MUL) is a multiorgan disease caused by recessive mutations in the TRIM37 gene. Chronic heart failure and hepatopathy are major determinants of prognosis in MUL patients, which prompted us to study liver biochemistry and pathology in a national cohort of MUL patients. METHODS: Clinical, laboratory and imaging data were collected in a cross-sectional survey and retrospectively from hospital records. Liver histology and immunohistochemistry for 10 biomarkers were assessed. RESULTS: Twenty-one MUL patients (age 1-51 years) with tumour suspicion showed moderate congestion, steatosis and fibrosis in liver biopsies and marginally elevated levels of serum GGT, AST, ALT and AST to platelet ratio index (APRI) in 20%-66%. Similarly, GGT, AST, ALT and APRI levels were moderately elevated in 12%-69% of 17 MUL patients prior to pericardiectomy. In a cross-sectional evaluation of 36 MUL outpatients, GGT, total bilirubin and galactose half-life (Gal½) correlated with age (r = 0.45, p = .017; r = 0.512, p = .007; r = 0.44, p = .03 respectively). The frequency of clearly abnormal serum values of 15 parameters analysed, however, was low even in patients with signs of restrictive cardiomyopathy. Transient elastography (TE) of the liver revealed elevated levels in 50% of patients with signs of heart failure and TE levels correlated with several biochemistry parameters. Biomarkers of fibrosis, sinusoidal capillarization and hepatocyte metaplasia showed increased expression in autopsy liver samples from 15 MUL patients. CONCLUSION: Liver disease in MUL patients was characterized by sinusoidal dilatation, steatosis and fibrosis with individual progression to cirrhosis and moderate association of histology with cardiac function, liver biochemistry and elastography.

Long-term Morbidity of Choledochal Malformations in Children.

OBJECTIVE: The aim of the study was to assess long-term morbidity in children operated for choledochal malformation (CM) by relating clinical complications to liver histopathology, follow-up imaging, liver stiffness, and biochemistry. METHODS: A single-center retrospective follow-up study including all CM patients (n = 55, 71% girls) treated during 1976 to 2018 was performed. Mann-Whitney U test and Spearman rank correlation were used for statistical analyses. RESULTS: During median follow-up of 5.8 (interquartile range, 2.5-12) years, 1 patient was lost to follow-up whereas all survived. Intraoperative liver biopsies showed fibrosis in 32%, and patients with Metavir stage ≥2 were younger at surgery (0.36 [0.11-1.9] vs 3.8 [0.72-10.5] years, P = 0.024) than those without fibrosis. Overall, 21% had long-term complications including cholangitis in 9 (>2 episodes in 5) patients, anastomotic stricture in 2 referred patients and adhesive volvulus or hepatocellular carcinoma in 1 each. Anastomotic strictures were successfully managed nonoperatively and hepatocellular carcinoma with thermoablation. In postoperative magnetic resonance cholangiography (MRCP) performed 6.4 (3.6-16) years after hepaticojejunostomy, diameters of both main intrahepatic ducts had decreased significantly to 3.0 (2.5-3.5) mm (P = 0.0001) but a distal cyst stump was remaining in 30% with a length of 6.0 (4.0-20) mm that associated with operation age (r = 0.71, P = 0.015) and fusiform CM type. Follow-up ultrasound revealed mild dilation of intrahepatic bile ducts in 6.3% and mildly to moderately elevated liver biochemistry in 23%, and liver stiffness (>7 kPa) in 22%. CONCLUSIONS: Whilst cholangitis was the most common postoperative problem, individual patients experienced other more significant complications and one quarter of patients showed evidence of underlying liver dysfunction.

Recessive MYH3 variants cause "Contractures, pterygia, and variable skeletal fusions syndrome 1B" mimicking Escobar variant multiple pterygium syndrome.

The multiple pterygium syndromes (MPS) are rare disorders with disease severity ranging from lethal to milder forms. The nonlethal Escobar variant MPS (EVMPS) is characterized by multiple pterygia and arthrogryposis, as well as various additional features including congenital anomalies. The genetic etiology of EVMPS is heterogeneous and the diagnosis has been based either on the detection of pathogenic CHRNG variants (~23% of patients), or suggestive clinical features. We describe four patients with a clinical suspicion of EVMPS who manifested with multiple pterygia, mild flexion contractures of several joints, and vertebral anomalies. We revealed recessively inherited MYH3 variants as the underlying cause in all patients: two novel variants, c.1053C>G, p.(Tyr351Ter) and c.3102+5G>C, as compound heterozygous with the hypomorphic MYH3 variant c.-9+1G>A. Recessive MYH3 variants have been previously associated with spondylocarpotarsal synostosis syndrome. Our findings now highlight multiple pterygia as an important feature in patients with recessive MYH3 variants. Based on all patients with recessive MYH3 variants reported up to date, we consider that this disease entity should be designated as "Contractures, pterygia, and variable skeletal fusions syndrome 1B," as recently suggested by OMIM. Our findings underline the importance of analyzing MYH3 in the differential diagnosis of EVMPS, particularly as the hypomorphic MYH3 variant might remain undetected by routine exome sequencing.

Biliary Atresia-associated Cholangitis: The Central Role and Effective Management of Bile Lakes.

OBJECTIVES: Cholangitis and bile lakes are incompletely understood complications after portoenterostomy (PE). We investigated relationships between recurrent cholangitis, bile lakes, and clinical outcomes as well as surgical management of bile lakes. METHODS: In this retrospective observational single institution study medical records and imaging studies of all patients who had undergone PE for biliary atresia during 1987 to 2016 (N = 61) were reviewed. We related occurrence of cholangitis episodes with the presence of intrahepatic bile lakes, patient characteristics, and PE outcomes. Risk factors for recurrent cholangitis and bile lakes, and management of bile lakes were analyzed. RESULTS: Despite routine antibiotic prophylaxis median of 3.0 cholangitis episodes (0.75 episodes/year) occurred in 48 (79%) patients. Intrahepatic bile lakes were discovered in 8 (13%) patients by 16 months after PE. Overall, 54% had survived with their native liver at median age of 7.3 years and 28 (46%) patients had ≥1 cholangitis episodes/year. Number and frequency of cholangitis episodes were >5 times higher among patients with bile lakes (P < 0.001). Six patients underwent Roux-en-Y bile lake-jejunostomy, resulting in regression/disappearance of bile lakes and normalization of serum bilirubin in 5 with reduction of median yearly cholangitis rate from 8.8 to 1.1 (P = 0.028) and native liver survival of 6.3 (range, 1.3-17) years after the operation. CONCLUSIONS: Bile lakes are a significant risk factor for recurrent cholangitis after PE and efficiently treated by operative intestinal drainage providing prolonged jaundice-free native liver survival. Bile lakes should be actively screened among patients presenting with recurrent cholangitis after PE.

Reduced differential renal function in scintigraphy predicted high-grade vesicoureteral reflux in children with antenatal hydronephrosiss.

AIM: A top-down approach is widely used for detecting vesicoureteral reflux (VUR) in children with febrile urinary tract infections. We evaluated the diagnostic value of renal scintigraphy in predicting VUR in children with antenatal hydronephrosis (AHN). METHODS: The voiding cystourethrogram (VCUG) and renal scintigraphy results of 125 AHN patients (76% male) admitted to the Children's Hospital of Helsinki University, Finland, from 2003 to 2013 were analysed. Of those, 94 had nonrefluxing hydronephrosis, nine had low-grade VUR and 22 had high-grade VUR. RESULTS: Scintigraphy was performed at a median age of 1.4 (0.8-15.6) months. In patients with high-grade VUR, the differential renal function (DRF) of the worse kidney was significantly lower than in patients without VUR, with a median of 35% and interquartile range (IQR) of 20-45 versus 47% (IQR: 44-49), (p < 0.001). There was no difference between patients with low-grade VUR and patients without VUR (p = 0.181). DRFs below 44% showed a sensitivity of 73% and specificity of 79% and predicted significantly high-grade VUR (odds ratio 9.82, 95% confidence interval 3.44-28.05, p < 0.001) in the univariate analysis. CONCLUSION: A DRF below 44% predicted high-grade VUR in patients with AHN and supported the decision to perform VCUG.

Correction to: Postnatal imaging of prenatally detected hydronephrosis-when is voiding cystourethrogram necessary?

The original publication contained an erroneous version of Table 3 with incorrect data regarding ureteral visibility. The authors apologize for any inconvenience caused by their mistake and are pleased to present the corrected table here.

Postnatal imaging of prenatally detected hydronephrosis-when is voiding cystourethrogram necessary?

OBJECTIVE: To evaluate whether grade 4-5 vesicoureteral reflux (VUR) can be predicted from renal ultrasound (RUS) findings and perform voiding cystourethrograms (VCUGs) only on high-risk patients. METHODS: The RUS and VCUG images of infants with prenatally detected hydronephrosis admitted to our institution between 2003 and 2013 were re-evaluated. The UTI episodes were collected retrospectively from patient journals. Patients with complex urinary tract anomalies were excluded. RESULTS: One hundred eighty, 44 female and 136 male, patients (352 renal units (RU)), 23 (30 RU) of them having grade 4-5 VUR, were included. The median age of the patients at the time of the RUS was 1.3 (0.1-3.0) months and the median follow-up time was 2.0 (0.1-11.2) years. In multivariate analysis, a visible ureter (OR 12.72; CI 5.33-32.04, p < 0.001) and shorter renal length (OR 2.67; CR 1.50-4.86, p < 0.001) in RUS predicted grade 4-5 VUR while a visible ureter predicted UTIs (OR 5.75; CI 2.59-12.66, p < 0.001). A three-grade risk score for high-grade VUR was developed based on the RUS findings and the patients were categorized into low-, intermediate-, and high-risk groups. The incidence of grade 4-5 VUR was 2.9% in the low-risk, 12.2% in the intermediate-risk, and 52.2% in the high-risk group. The sensitivity and specificity for detecting grade 4-5 VUR were 79 and 82%, respectively. CONCLUSIONS: In patients with antenatally detected hydronephrosis, a visible ureter and reduced renal length in RUS are significant risk factors for high-grade VUR. A RUS-based risk scoring would probably reduce the proportion of unnecessary VCUGs.

Small Bowel Dilatation Predicts Prolonged Parenteral Nutrition and Decreased Survival in Pediatric Short Bowel Syndrome.

OBJECTIVE: To analyze risk factors and prognostic significance of small bowel (SB) dilatation in children with short bowel syndrome (SBS). BACKGROUND: In SBS, the remaining SB may dilate as part of intestinal adaptation. The impact of dilatation on parenteral nutrition (PN) dependence and survival has not been studied systematically. METHODS: SB diameter of SBS children (n = 61) was measured in contrast SB series (n = 169, median age 0.94, range 0.32-2.7 years) during 2002 to 2015, and expressed as millimeters (SB width) and as ratio to L5 vertebra height (SB diameter ratio). Linear regression was used to examine risk factors for dilatation. PN weaning and survival were analyzed with Cox proportional hazards regression. RESULTS: Maximal SB diameter ratio during follow-up was predicted by PN dependence and SB atresia, while maximal absolute SB width by birth weight, age, PN duration, and remaining bowel length. Weaning off PN was 14-fold more likely in patients with maximal SB diameter ratio <2.00 compared with >3.00 (P = 0.005), and 5.4-fold more likely when maximal SB width was <20 mm compared with >30 mm (P = 0.023). After adjustment for age, remaining SB length, and the presence of ileocecal valve, both estimates of maximal SB dilatation remained significant independent predictors for weaning off PN. When all measurements were included, the cumulative survival was worse if SB diameter ratio exceeded 2.00 (P = 0.002-0.042). CONCLUSIONS: SB dilatation predicts prolonged PN duration and decreased survival in SBS children. Measurement of maximal SB diameter standardized to L5 vertebra height may be a valuable objective tool for patient follow-up and assessment of prognosis.

31Phosphorus magnetic resonance spectroscopy of the liver for evaluating inflammation and fibrosis in autoimmune hepatitis.

BACKGROUND: Liver biopsy is the gold standard in evaluating inflammation and fibrosis in autoimmune hepatitis. AIMS: In search of non-invasive follow-up tools in autoimmune hepatitis, we evaluated 31phosphorus magnetic resonance spectroscopy (31P MRS). METHODS: Twelve consecutive AIH patients (mean age 42.8 years, 10 women) underwent liver biopsy, routine laboratory liver function tests, which were compared to findings in 31P MRS and transient elastography (TE). RESULTS: Phosphoenolpuryvate (PEP) correlated with the grade of inflammation (r = 0.746, p = .005) and thromboplastin time (r = 0.592, p = .043). It also differentiated patients with active inflammation from patients without (t = 3.781, p = .009). There was no correlation between PEP and aminotransferase or immunoglobulin G levels. The phosphoethanolamine (PE)/phosphocholine (PC) ratio, PE/glyserophosphoethanolamine (GPE) ratio and PC/[total phosphomonoester (PME) + phosphodiester (PDE)] ratios correlated with immunoglobulin G (r = 0.764, p = .006; r = 0.618, p = .043; and r= -0.636, p = .035, respectively). PME/PDE and PE/GPE correlated with fibrosis (r = 0.668, p = .018 and r = 0.604, p = .037). PE/GPE also differentiated F3 from F0-2 patients (t = 3.810, p = .003). Phosphorus metabolites did not correlate with TE results and TE did not correlate with liver histology or laboratory parameters. CONCLUSIONS: 31P MRS seems to detect active inflammation and advanced fibrosis in AIH patients. TE was ineffective in fibrosis quantification.

Outcome after pin fixation of supracondylar humerus fractures in children: postoperative radiographic examinations are unnecessary.

Background and purpose - The quality of pin fixation of displaced supracondylar humerus fractures in children has not been assessed, and the clinical value of radiographic examinations after pin fixation is unclear. We evaluated pin configuration, quality of osteosynthesis, and outcome in 264 supracondylar fractures. The clinical significance of postoperative radiographs was analyzed. Patients and methods - 252 Gartland-III and 12 flexion-type supracondylar humerus fractures were pin-fixed in the periods 2002-2006 and 2012-2014. During 2012-2014, staff were intructed that postoperative radiographs should not be taken. Quality of reduction was assessed by measuring Baumann and lateral capitellohumeral angles (LCHA) and also by recording the crossing point of the anterior humeral line (AHL) with bony capitellum. Rotatory alignment was registered as normal or abnormal. Pin configuration and quality of osteosynthesis were evaluated. The clinical significance of postoperative radiographs was analyzed. Results - Postoperatively, Baumann angle was normal in 66% of the fractures, AHL crossed the capitellum in 84%, and no malrotation was evident in 85% of the fractures. Crossed pins were used in 89% of the cases. 2 or more pins fixed both fracture fragments in 66%. Radiographic examinations were inadequate for assessment of LCHA in 13%, of Bauman angle in 8%, of AHL in 2%, of rotation in 1%, and of pin fixation in 2% of the cases. Postoperative radiographs did not give useful information except in 1 patient who had corrective osteotomy. All 94 patients with follow-up (97%) who were treated during 2012-2014 were satisfied with the outcome. Interpretation - Despite pin fixation being deemed unsatisfactory in one-third of the cases, significant malunion was rare. Postoperative radiography did not alter management or outcome.

Transient elastography and aspartate aminotransferase to platelet ratio predict liver injury in paediatric intestinal failure.

BACKGROUND & AIMS: We aimed to evaluate the value of AST to platelet ratio (APRI) and transient elastography (TE) as predictors of liver histopathology in children with intestinal failure (IF). METHODS: Altogether 93 liver biopsies from 57 children with parenteral nutrition (PN) duration ≥3 months were analysed. APRI measurement and TE (n = 46) were performed at the time of biopsy. RESULTS: IF was caused by short bowel syndrome in 75% of patients. At the time of liver biopsy, PN dependent patients (n = 42) were younger with longer PN duration compared to those weaned off PN (n = 51) (2.2 vs. 7.6 years, P < 0.001; 26 vs. 10.5 months, P = 0.043). Elevated transaminase or bilirubin levels were found in 51%, splenomegaly in 26%, and oesophageal varices in 3.5%. Histological fibrosis was present in 61% (Metavir stage F1; 27%, F2; 26%, F3-4; 9%), cholestasis in 25% and steatosis in 22% of biopsy specimens. TE was superior to APRI in prediction of any liver histopathology (fibrosis, cholestasis or steatosis) with areas under the receiving operating curve (AUROC) of 0.86 (95% CI 0.74-0.97) and 0.67 (95% CI 0.58-0.78) respectively. For prediction of ≥F1 and ≥F2 fibrosis, AUROC values for TE were 0.78 (95% CI 0.64-0.93) and 0.73 (95% CI 0.59-0.88), whereas APRI did not correlate with fibrosis stages. For detection of histological cholestasis, the AUROC for APRI was 0.77 (95% CI 0.64-0.89). CONCLUSIONS: Both TE and APRI are promising noninvasive methods for monitoring the development of IF-related liver histopathology. TE values reflected the degree of fibrosis better while APRI detected histological cholestasis more accurately.

A Prospective Comparison of Noninvasive Methods in the Assessment of Liver Fibrosis and Esophageal Varices in Pediatric Chronic Liver Diseases.

GOALS AND BACKGROUND: We compared liver stiffness (LS), the aspartate aminotransferase-to-platelet ratio index (APRi), and the platelet-to-spleen size z score ratio (P/SZC) in the prediction of liver fibrosis and esophageal varices in children. STUDY: LS, APRi, SZC, and P/SZC were prospectively determined in 99 unselected consecutive children, who underwent liver biopsy for the follow-up of chronic liver disorders. LS was assessed by transient elastography. The spleen size was evaluated as the SD from age-specific and gender-specific normative values. Varices were assessed endoscopically (n=64). Biopsies were staged according to Metavir. RESULTS: The median patient age was 6.0 (interquartile range, 1.8 to 12.9) years. Underlying diagnoses included intestinal failure (n=31), biliary atresia (n=24), and others (n=44). LS showed the strongest correlation with the fibrosis stage (r=0.639, P<0.001) compared with P/SZC (r=-0.427, P=0.003), APRi (r=0.419, P=0.001), or SZC (r=0.396, P=0.004). LS clearly performed the best in predicting fibrosis with area under the receiver operator curve (AUROC) values of 0.789 [95% confidence interval (CI), 0.698-0.879; P<0.001] for any (Metavir≥1), and 0.831 (95% CI, 0.745-0.918; P<0.001) for significant (Metavir≥2) fibrosis. For the prediction of the presence of esophageal varices, APRi had a higher AUROC of 0.832 (95% CI, 0.730-0.934; P<0.001), when compared with LS, SZC, or P/SZC with AUROCs of 0.818 (95% CI, 0.706-0.930; P<0.001), 0.795 (95% CI, 0.683-0.904; P=0.001), and 0.760 (95% CI, 0.610-0.909; P=0.004), respectively. CONCLUSIONS: LS performed the best in predicting liver fibrosis, whereas APRi had the highest predictive accuracy for esophageal varices. An LS value over 7.7 kPa identified significant liver fibrosis with high accuracy, whereas low APRi ascertained the absence of esophageal varices.

Stratifying Antenatal Hydronephrosis: Predicting High-Grade VUR Using Ultrasound and Scintigraphy.

(1) Background: Antenatal hydronephrosis (AHN), detected in approximately one percent of prenatal ultrasounds, is caused by vesicoureteral reflux (VUR) in 15-21% of cases, a condition with significant risks such as urinary tract infections and renal scarring. Our study addresses the diagnostic challenges of VUR in AHN. Utilizing renal ultrasonography and scintigraphy, we developed a novel scoring system that accurately predicts high-grade VUR, optimizing diagnostic precision while minimizing the need for more invasive methods like voiding cystourethrogram (VCUG); (2) Methods: This retrospective study re-analyzed renal ultrasonography, scintigraphy, and VCUG images from infants admitted between 2003 and 2013, excluding cases with complex urinary anomalies; (3) Results: Our analysis included 124 patients (75% male), of whom 11% had high-grade VUR. The multivariate analysis identified visible ureter, reduced renal length, and decreased differential renal function (DRF) as primary predictors. Consequently, we established a three-tier risk score, classifying patients into low, intermediate, and high-risk groups for high-grade VUR, with corresponding prevalences of 2.3%, 22.2%, and 75.0%. The scoring system demonstrated 86% sensitivity and 79% specificity; (4) Conclusions: Our scoring system, focusing on objective parameters of the visible ureter, renal length, and DRF, effectively identifies high-grade VUR in AHN patients. This method enhances diagnostics in ANH by reducing reliance on VCUG and facilitating more tailored and less invasive patient care.

Risk of premature physeal closure in fractures of distal tibia.

The reported incidence of premature physeal closure (PPC) in fractures of the distal tibia has varied between 5 and 36%, but there is no consensus on the cause. We wanted to determine incidence and predictors of PPC in distal tibia physeal fractures in a population-based patient cohort. Two hundred forty-one patients (195 Peterson type I-V fractures and 46 transitional fractures) treated for a physeal fracture of the distal tibia during a 5-year period in two tertiary-level teaching hospitals. Odds ratios (OR) for developing PPC for different parameters (Peterson fracture type, associated fibula fracture, primary and postreduction displacement, number of reductions and the method of treatment) were calculated by binary logistic regression analysis. In 195 children with Peterson type I-V fractures PPC was diagnosed in 21 children (11%), of which 11 (6%) had surgery at mean 14 months from the fracture to correct either angular deformity or leg length discrepancy. The incidence of distal tibia PPC is at least 0.05/1000 children. More than one reduction attempt was the most significant risk factor (OR 7.0) for PPC. Peterson fracture type, associated fibula fracture, initial or post-reduction displacement or type of treatment did not correlate with PPC. The incidence of distal tibia PPC is at least 0.05/1000 children. The number of reductions correlates positively with the risk of PPC.

Compromised duodenal mucosal integrity in children with short bowel syndrome after adaptation to enteral autonomy.

BACKGROUND: Intestinal adaptation has been extensively studied experimentally, but very limited data is available on human subjects. In this study we assessed intestinal adaption in humans with short bowel syndrome (SBS). METHODS: We comparatively evaluated mucosal hyperplasia, inflammation, barrier function and nutrient transport using histology, immunohistochemistry and qPCR for selected 52 key genes in duodenal biopsies obtained from children with SBS after weaning off parenteral nutrition (n = 33), and matched controls without intestinal pathology (n = 12). Small bowel dilatation was assessed from contrast small bowel series. RESULTS: Duodenal mucosa of SBS children showed increased histologic inflammation of lamina propria (p = 0.033) and mucosal mRNA expression of tumor necrosis factor (p = 0.027), transforming growth factor (TGF)-ß2 (p = 0.006) and caveolin-1 (CAV1; p = 0.001). Villus height, crypt depth, enterocyte proliferation, apoptosis and expression of proliferation and nutrient transport genes remained unchanged. Pathologic small bowel dilatation reduced crypt depth (p = 0.045) and downregulated mRNA expression of interleukin (IL)-6 by three-fold (p = 0.008), while correlating negatively with IL6 (r = -0.609, p = 0.004). Loss of ileocecal valve (ICV) upregulated mRNA expression of toll-like receptor 4 (TLR4), TGF-ß1, CAV1, several apoptosis regulating genes, and mRNA expression of zonulin (p < 0.05 for all). CONCLUSIONS: Despite successful adaptation to enteral autonomy, duodenal mucosa of SBS children displayed histologic and molecular signs of abnormal inflammation and regulation of epithelial permeability, whereas no structural or molecular signs of adaptive hyperplasia or enhanced nutrient transport were observed. Excessive dilatation of the remaining small bowel paralleled impaired duodenal crypt homeostasis, while absence of ICV modified regulation of mucosal inflammation, regeneration and permeability. LEVEL OF EVIDENCE: II.

Segmental cystic kidney tumours in children.

OBJECTIVE: Segmental cystic tumours in the kidney are extremely rare in children. This study reports our experience of those tumours. MATERIAL AND METHODS: The operative database from 1993 to 2008 of the Hospital for Children and Adolescents, University of Helsinki, was evaluated for segmental cystic renal tumours without any solid component. Patient records, imaging studies and pathology specimens were reviewed. RESULTS: Four cases (three girls and one boy) were found; all were less than 3 years old at the time of diagnosis. All patients underwent kidney-preserving surgery with resection of the cystic tumour. Two patients had neoplasias: one cystic nephroma and one cystic partially differentiated nephroblastoma. Both of these patients had associated or predisposing disease (type I cystic pleuropulmonary blastoma of the lung and mulibrey nanism, respectively). Two patients had a non-neoplastic tumour; localized cystic disease of the kidney and segmental adult type autosomal dominant polycystic kidney disease. Neoplastic cystic tumours had a fibrous capsule in preoperative magnetic resonance imaging and also in operation, unlike non-neoplastic lesions. CONCLUSIONS: Preoperative and perioperative diagnosis is difficult in cases of segmental cystic kidney tumours in a child. If a neoplastic tumour is suspected complete resection of the tumour is mandatory.