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1.
Diagnostics (Basel) ; 14(4)2024 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-38396423

RESUMO

(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.

2.
Acta Orthop ; 95: 8-13, 2024 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-38240180

RESUMO

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.


Assuntos
Fraturas por Compressão , Fraturas da Coluna Vertebral , Humanos , Criança , Adolescente , Estudos Prospectivos , Seguimentos , Fraturas por Compressão/diagnóstico por imagem , Fraturas por Compressão/cirurgia , Qualidade de Vida , Fraturas da Coluna Vertebral/diagnóstico por imagem , Fraturas da Coluna Vertebral/cirurgia , Dor
3.
J Pediatr Gastroenterol Nutr ; 77(6): 720-725, 2023 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-37679875

RESUMO

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.


Assuntos
Enteropatias , Síndrome do Intestino Curto , Humanos , Criança , Síndrome do Intestino Curto/complicações , Dilatação , Estudos Retrospectivos , Intestino Delgado/cirurgia , Intestino Delgado/patologia , Intestinos/patologia , Enteropatias/complicações
4.
BMC Musculoskelet Disord ; 24(1): 441, 2023 May 31.
Artigo em Inglês | MEDLINE | ID: mdl-37259117

RESUMO

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.


Assuntos
Neoplasias Encefálicas , Degeneração do Disco Intervertebral , Deslocamento do Disco Intervertebral , Disco Intervertebral , Criança , Humanos , Degeneração do Disco Intervertebral/patologia , Vértebras Lombares/diagnóstico por imagem , Vértebras Lombares/patologia , Estudos Transversais , Qualidade de Vida , Deslocamento do Disco Intervertebral/complicações , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/complicações , Imageamento por Ressonância Magnética/métodos , Disco Intervertebral/patologia
5.
Scand J Gastroenterol ; 58(9): 1038-1043, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37070861

RESUMO

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.


Assuntos
Cisto do Colédoco , Humanos , Adulto , Cisto do Colédoco/cirurgia , Cisto do Colédoco/complicações , Finlândia/epidemiologia , Ducto Colédoco , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia
6.
Liver Int ; 42(6): 1369-1378, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35220664

RESUMO

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.


Assuntos
Técnicas de Imagem por Elasticidade , Nanismo de Mulibrey , Proteínas com Motivo Tripartido , Ubiquitina-Proteína Ligases , Adolescente , Adulto , Biomarcadores , Criança , Pré-Escolar , Estudos Transversais , Humanos , Lactente , Pessoa de Meia-Idade , Nanismo de Mulibrey/genética , Nanismo de Mulibrey/patologia , Mutação , Estudos Retrospectivos , Proteínas com Motivo Tripartido/genética , Ubiquitina-Proteína Ligases/genética , Adulto Jovem
7.
J Pediatr Gastroenterol Nutr ; 72(6): 820-825, 2021 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-33470752

RESUMO

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.


Assuntos
Cisto do Colédoco , Ductos Biliares Intra-Hepáticos , Criança , Cisto do Colédoco/diagnóstico por imagem , Cisto do Colédoco/epidemiologia , Feminino , Seguimentos , Humanos , Masculino , Morbidade , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos
8.
J Pediatr Orthop B ; 30(1): 25-31, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32558778

RESUMO

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.


Assuntos
Fraturas do Tornozelo , Fraturas da Tíbia , Criança , Lâmina de Crescimento , Humanos , Estudos Retrospectivos , Tíbia/diagnóstico por imagem , Tíbia/cirurgia , Fraturas da Tíbia/diagnóstico por imagem , Fraturas da Tíbia/epidemiologia , Fraturas da Tíbia/cirurgia
9.
J Pediatr Surg ; 56(5): 966-974, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33131778

RESUMO

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.


Assuntos
Síndrome do Intestino Curto , Adaptação Fisiológica , Animais , Criança , Modelos Animais de Doenças , Humanos , Mucosa Intestinal , Intestino Delgado , Ratos , Ratos Sprague-Dawley
10.
Am J Med Genet A ; 182(11): 2605-2610, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32902138

RESUMO

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.


Assuntos
Anormalidades Múltiplas/genética , Proteínas do Citoesqueleto/genética , Genes Recessivos , Variação Genética , Hipertermia Maligna/genética , Anormalidades da Pele/genética , Criança , Pré-Escolar , Contratura/genética , Feminino , Deleção de Genes , Heterozigoto , Humanos , Lordose/genética , Masculino , Mutação , Linhagem , Fenótipo , Escoliose/genética , Análise de Sequência de DNA , Irmãos , Sequenciamento do Exoma
11.
Hepatol Commun ; 3(3): 382-391, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30859150

RESUMO

We investigated noninvasive follow-up markers for histologic liver fibrosis and portal hypertension (PH) in patients with biliary atresia after successful portoenterostomy (PE). Among children with bilirubin <20 µmol/L after PE (n = 39), Metavir fibrosis stage was evaluated at PE and in follow-up protocol liver biopsies (n = 83). PH was defined as endoscopically confirmed esophageal varices or thrombocytopenia associated with splenomegaly. The accuracy of liver biochemistry and stiffness in detecting liver fibrosis and PH was analyzed by the area under the receiving operating characteristic curve (AUROC) and multiple regression models. During a median native liver survival of 8.3 years (interquartile range 2.5-10.8 years), cirrhosis (Metavir F4) had developed in 51% of patients and PH in 54% of patients. Cirrhosis was equally common in all age tertiles of 1.2-2.1 years (n = 10/27), 3.9-5.8 years (n = 12/28), and 9.0-14 years (n = 12/28). In the two oldest age tertiles, histologic liver fibrosis had progressed further in patients with PH than without PH (P < 0.001). PH was accurately predicted by the aspartate aminotransferase-to-platelet ratio index (APRI) (cutoff, 0.70; AUROC, 0.92), bile acids (cutoff, 49 µmol/L; AUROC, 0.91), and liver stiffness (cutoff, 16.9 kPa; AUROC, 0.89; P < 0.001 each) across all age tertiles. Liver stiffness was the most accurate predictor of cirrhosis overall (AUROC, 0.82; P < 0.001), whereas bilirubin was >11 µmol/L in the youngest tertile (AUROC, 0.91; P < 0.001), bile acids was >80 µmol/L in the middle tertile (AUROC, 0.81; P = 0.009), and liver stiffness was >24 kPa in the oldest age tertile (AUROC, 0.96; P = 0.002). Conclusion: After successful PE, development of PH associates with progression of liver fibrosis and can be accurately detected by APRI and stiffness. Liver stiffness most accurately identified cirrhosis in older children, whereas biochemical markers of cholestasis closely reflected histologic cirrhosis in younger children.

12.
J Pediatr Gastroenterol Nutr ; 68(4): 488-494, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30628982

RESUMO

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.


Assuntos
Bile , Atresia Biliar/cirurgia , Colangite/cirurgia , Cistos/cirurgia , Portoenterostomia Hepática/efeitos adversos , Pré-Escolar , Colangite/etiologia , Colangite/mortalidade , Cistos/etiologia , Cistos/mortalidade , Drenagem , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos
13.
Acta Paediatr ; 108(4): 751-756, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30113092

RESUMO

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.


Assuntos
Hidronefrose/complicações , Refluxo Vesicoureteral/diagnóstico por imagem , Refluxo Vesicoureteral/etiologia , Feminino , Humanos , Hidronefrose/diagnóstico por imagem , Lactente , Recém-Nascido , Testes de Função Renal/métodos , Masculino , Valor Preditivo dos Testes , Cintilografia , Estudos Retrospectivos , Índice de Gravidade de Doença , Ultrassonografia Pré-Natal
14.
Pediatr Nephrol ; 33(10): 1805, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29948311

RESUMO

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.

15.
Pediatr Nephrol ; 33(10): 1751-1757, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29626243

RESUMO

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.


Assuntos
Cistografia/métodos , Hidronefrose/diagnóstico por imagem , Cuidado Pós-Natal/métodos , Infecções Urinárias/epidemiologia , Urografia/métodos , Refluxo Vesicoureteral/diagnóstico por imagem , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Hidronefrose/complicações , Incidência , Lactente , Recém-Nascido , Masculino , Seleção de Pacientes , Gravidez , Diagnóstico Pré-Natal , Medição de Risco/métodos , Ultrassonografia Pré-Natal , Ureter/diagnóstico por imagem , Bexiga Urinária/diagnóstico por imagem , Infecções Urinárias/etiologia , Infecções Urinárias/microbiologia , Micção , Refluxo Vesicoureteral/etiologia
16.
Eur J Radiol ; 90: 205-211, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28583636

RESUMO

PURPOSE: To study liver 31P MRS, histology, transient elastography, and liver function tests in patients with virus C hepatitis (HCV) or autoimmune hepatitis (AIH) to test the hypothesis that 31P MR metabolic profile of these diseases differ. MATERIALS AND METHODS: 25 patients with HCV (n=12) or AIH (n=13) underwent proton decoupled 31P MRS spectroscopy performed on a 3.0T MR imager. Intensities of phosphomonoesters (PME) of phosphoethanolamine (PE) and phosphocholine (PC), phosphodiesters (PDE) of glycerophosphoethanolamine (GPE) and glycerophosphocholine (GPC), and γ, α and ß resonances of adenosine triphosphate (ATP), and nicotinamide adenine dinucleotide phosphate (NADPH) were determined. Liver stiffness was measured by transient elastography. Inflammation and fibrosis were staged according to METAVIR from biopsy samples. Activities of alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALT) and thromboplastin time (TT) were determined from serum samples. RESULTS: PME had a stronger correlation with AST (z=1.73, p=0.04) and ALT (z=1.77, p=0.04) in HCV than in AIH patients. PME, PME/PDE, PE/GPE correlated positively and PDE negatively with inflammatory activity. PE, PC and PME correlated positively with liver function tests. CONCLUSION: 31P-MRS suggests a more serious liver damage in HCV than in AIH with similar histopathological findings. 31P-MRS is more sensitive in detecting inflammation than fibrosis in the liver.


Assuntos
Hepatite C/metabolismo , Hepatite C/patologia , Hepatite Autoimune/metabolismo , Hepatite Autoimune/patologia , Espectroscopia de Ressonância Magnética/métodos , Trifosfato de Adenosina/metabolismo , Aspartato Aminotransferases/metabolismo , Etanolaminas/metabolismo , Feminino , Hepatite C/diagnóstico por imagem , Hepatite Autoimune/diagnóstico por imagem , Humanos , Fígado/diagnóstico por imagem , Fígado/metabolismo , Fígado/patologia , Masculino , Metaboloma , Pessoa de Meia-Idade , Fosfatidiletanolaminas/metabolismo , Fósforo , Prótons
17.
Scand J Gastroenterol ; 52(8): 886-892, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28415898

RESUMO

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.


Assuntos
Hepatite Autoimune/complicações , Cirrose Hepática/diagnóstico por imagem , Cirrose Hepática/patologia , Fígado/patologia , Fósforo/análise , Adulto , Idoso , Biópsia , Técnicas de Imagem por Elasticidade , Feminino , Finlândia , Humanos , Espectroscopia de Ressonância Magnética , Pessoa de Meia-Idade , Fosfoenolpiruvato/sangue , Adulto Jovem
18.
J Pediatr Surg ; 52(7): 1121-1127, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28185632

RESUMO

BACKGROUND: In remains unclear why in some short bowel syndrome (SBS) patients, the remaining small bowel (SB) dilates excessively leading to requirement of tapering surgery. METHODS: Among SBS children, we retrospectively analyzed risk factors for tapering surgery with logistic regression and compared the outcome of operated patients (n=16) to those managed conservatively (n=44) with Cox proportional hazards regression. RESULTS: SBS was caused by necrotizing enterocolitis (NEC) (n=31), SB atresia (SBA) (n=13), midgut volvulus (n=12), or gastroschisis (n=4). Patients with spontaneous symptomatic SB dilatation unable to wean parenteral nutrition (PN) underwent tapering surgery at median age of 1.04 (interquartile range 0.70-3.27) years. Missing ICV was related to an 8-fold (p=0.003) increased risk while SBA diagnosis was related to a 13-fold risk of tapering surgery (p<0.001). Increasing SB length and NEC diagnosis were protective of tapering (p=0.027-0.004). Of operated patients, 75% reached enteral autonomy during follow-up and their postoperative adjusted PN weaning rate was similar to nonoperated children (p=0.842). CONCLUSION: SBS children with short remaining SB, missing ICV, and SBA etiology are more likely while NEC patients are less likely than others to necessitate tapering surgery. Postoperative PN weaning rates were comparable to patients who initially had more favorable intestinal anatomy and adapted without surgery.


Assuntos
Intestino Delgado/cirurgia , Síndrome do Intestino Curto/cirurgia , Adolescente , Criança , Pré-Escolar , Tratamento Conservador , Dilatação Patológica/etiologia , Dilatação Patológica/cirurgia , Feminino , Seguimentos , Humanos , Lactente , Intestino Delgado/patologia , Modelos Logísticos , Masculino , Estudos Retrospectivos , Fatores de Risco , Síndrome do Intestino Curto/patologia , Síndrome do Intestino Curto/terapia , Resultado do Tratamento
19.
Acta Orthop ; 88(1): 109-115, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27774833

RESUMO

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.


Assuntos
Pinos Ortopédicos , Fixação Interna de Fraturas/métodos , Fraturas do Úmero/cirurgia , Radiografia , Procedimentos Desnecessários , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Fraturas do Úmero/diagnóstico , Lactente , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
20.
Ann Surg ; 266(2): 369-375, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-27433892

RESUMO

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.


Assuntos
Intestino Delgado/patologia , Nutrição Parenteral , Síndrome do Intestino Curto/patologia , Síndrome do Intestino Curto/terapia , Pré-Escolar , Feminino , Humanos , Lactente , Modelos Lineares , Masculino , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Síndrome do Intestino Curto/mortalidade , Fatores de Tempo
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