Surgical treatment of secondary hyperparathyroidism in children with chronic kidney disease. Experience in 19 patients.

OBJECTIVES: Secondary hyperparathyroidism (sHPT) is an important contributor to bone disease and cardiovascular calcifications in children with chronic kidney disease (CKD). When conservative measures are ineffective, parathyroidectomy is indicated. The aim of our study was to evaluate the efficacy and safety of subtotal parathyroidectomy (sPTX) in pediatric and adolescent patients, and to provide a rationale for considering this aggressive treatment in CKD patients with uncontrolled sHPT. METHODS: We retrospectively analyzed the medical records of 19 pediatric CKD patients on dialysis with refractory sHPT who underwent sPTX at our institution between 2010 and 2020. All patients had clinical, radiological, and biochemical signs of renal osteodystrophy. RESULTS: One year after sPTX, parathyroid hormone (PTH) levels (median and interquartile range (IQR)) dropped from 2073 (1339-2484) to 164 (93-252) pg/mL (p=0.0001), alkaline phosphatase (ALP) levels from 1166 (764-2373) to 410 (126-421) IU/L (p=0.002), and the mean (±SDS) calcium-phosphate (Ca*P) product from 51±11 to 41±13 mg2/dL2 (p=0.07). Postoperatively, all patients presented with severe hungry bone syndrome (HBS) and required intravenous and oral calcium and calcitriol supplementation. None of them had other postoperative complication. Histological findings had a good correlation with preoperative parathyroid ultrasound imaging (n: 15) in 100 % and with technetium-99m (99mTc) sestamibi scintigraphy (n: 15) in 86.6 %. Clinical and radiological signs of bone disease improved in all patients. CONCLUSIONS: Pediatric sPTX is effective and safe to control sHPT and calcium-phosphate metabolism in children with CKD on dialysis and may mitigate irreversible bone deformities and progression of cardiovascular disease.

Clinical Utility of Definitive Diagnostic Tests for Choledocholithiasis in Pediatric Patients with Mild Gallstone Pancreatitis.

BACKGROUND: Gallstone pancreatitis was historically considered a risk factor for choledocholithiasis (CD). However, recent studies of adult patients evidenced a weak association between gallstone pancreatitis and CD. The aim of this study was to analyze this association in pediatric patients. METHODS: We conducted a retrospective study on patients with mild gallstone pancreatitis who underwent any definitive testing for CD (MRCP, ERCP, IOC), managed between March 2010 and September 2022. Patients were classified according to the presence or absence of risk factors for CD (total bilirubin ≥2 mg/dl; common bile duct >6 mm on ultrasound; and/or CD on ultrasound). We evaluated the diagnosis of CD on definitive testing in both groups and analyzed the predictive capacity of the presence of risk factors. RESULTS: Eighty-four patients were included in the final analysis. Seventy-nine percent were females. The median age was 13 (4-17) years. Forty-seven (55.9%) patients had one or more risk factors. The definitive testing confirmed 13 (15.5%) cases of CD, 12 (25.5%) in the group of patients with risk factors and 1 (2.7%) in those without risk factors. The sensitivity, specificity, positive predictive value and negative predictive value of the presence of associated risk factors were 92.3, 50.7, 25.5 and 97.3%, respectively. CONCLUSION: Pediatric patients with gallstone pancreatitis without associated risk factors have a very low incidence of CD. In these patients we suggest performing a laparoscopic cholecystectomy without intraoperative cholangiography or any other definitive test for CD. LEVEL OF EVIDENCE: Level II, retrospective study.

Development of a risk score for choledocholithiasis in pediatric patients.

PURPOSE: The prevalence of gallstones in children has increased over the last years. Choledocholithiasis (CD) is present in up to 30% of the cases. There is a scarcity of studies on the management of choledocholithiasis in children. The aim of this study was to develop a score that would allow predicting accurately the risk of CD in children with gallstones and reduce the number of non-therapeutic ERCP. MATERIALS AND METHODS: We conducted a retrospective study in children with gallstones and suspected CD seen between January 2010 and December 2019. The main outcome was the presence of CD confirmed by at least one of the following diagnostic tests: magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), and/or intraoperative cholangiography (IOC). We developed a risk score based on the presence or absence of the following risk factors: acute biliary pancreatitis, ascending cholangitis, elevated liver function tests (AST, ALT, total bilirubin [TB, ≥ 2 mg/dl], conjugated bilirubin, gamma-glutamyl transpeptidase, and alkaline phosphatase), CD on ultrasound (US; this was considered predictive but not confirmatory of CD), and dilation of the common bile duct (> 6 mm) by US. The score was divided into three different categories: low risk (no risk factors), intermediate risk (one risk factor present), high risk (≥ 2 risk factors present or ascending cholangitis). Given the main goal of reducing the number of diagnostic ERCPs, a very-high-risk subgroup (3 risk factors present or ascending cholangitis) was identified. RESULTS: We reviewed 133 patients with gallstones and suspected CD. In 56 (42.1%) patients, the presence of CD was confirmed by one or more of the definitive diagnostic tests (MRCP, ERCP, and IOC). The following variables were found to be the strongest predictors of CD: ascending cholangitis, TB ≥ 2 mg/dl, common bile duct > 6 mm, and the presence of CD by US. The positive predictive value for CD was 7.5% in the low-risk group (OR 0.06, P = < 0.001); 22.9% in the intermediate-risk group (OR 0.31, P = 0.007); 77.6% in the high-risk group (OR 20.14, P = < 0.001); and 95.7% in very-high-risk subgroup (OR 49.18, P = < 0.001). CONCLUSION: The risk score proposed in this study predicts accurately the presence of CD in children with gallstones. It can serve as a helpful tool to triage the need for costly and complex studies in the workup of CD, particularly in centers with limited resources. Finally, due to its high specificity and positive predictive value (PPV), the use of the very-high-risk criteria would allow for an important decrease in the number of non-therapeutic ERCP.

Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia.

PURPOSE: Biliary atresia is managed surgically by the Kasai portoenterostomy (KP). It has been reported by some groups that the outcomes of patients who have an early failed KP requiring a liver transplant (LTx) within the first year of life are worse than the outcomes of patients who undergo a primary LTx. The aim of this study was to identify preoperative parameters that could help predict what patients are at risk for the early failure of the procedure. MATERIALS AND METHODS: We conducted a retrospective chart review of all patients who underwent a KP between January 2008 and May 2018. The following preoperative parameters were analyzed: age at KP, anatomical variant of the biliary atresia, degree of liver fibrosis, CMV status, and PELD score. The main outcome of the study was the early failure of the KP (EF-K), which was defined as the need for LTx before 1 year of age, or BA-related death before 1 year of age. Second, we analyzed the risk factors associated with death without LTx within the first year of life. RESULTS: A total of 58 patients were included in the analysis. The native liver survival (NLS) was 56.5% and 48% at 1 and 5 years post KP, respectively. Overall survival (OS) was 79% and 76% at 1 and 5 years post KP, respectively. Early failure of KP occurred in 23 (39.7%) patients. OS in this group was 47% and 40% at 1 and 5 years, respectively. On the contrary, the OS of the remaining 35 (60.3%) patients was 100% at 1 and 5 years (P < 0.0001). When we compared all preoperative parameters, the only predictor of EF-K was the PELD score. When we analyzed the cases in the EF-K group who died without LTx, we found that the significant predictors were the cystic variant, a degree of liver fibrosis >4, and the PELD score. Nevertheless, on multivariate analysis, only PELD score was found as a statistically significant variable. CONCLUSION: Due to bad prognosis found in EF-K patients, we believe that it could be reasonable to offer them a primary LTx. PELD score was found to be the strongest preoperative parameter that allows predicting which patient will likely have an early failed KP. Further prospective and multicenter studies are needed to reinforce these results.

Respuesta al lenvatinib en una paciente pediátrica con insuficiencia respiratoria secundaria a carcinoma papilar de tiroides / Response to lenvatinib in a pediatric patient with respiratory failure associated with papillary thyroid carcinoma

El cáncer papilar de tiroides es el tumor tiroideo más común en la infancia. En estadios avanzados, puede presentarse con cuadro de insuficiencia respiratoria. El tratamiento de elección es la tiroidectomía total y iodo radiactivo. En tumores irresecables, se debería considerar terapia con inhibidores multicinasa.Niña de 10 años de edad derivada por insuficiencia respiratoria progresiva. Se realizó el diagnóstico de cáncer papilar de tiroides con metástasis pulmonares. Por presentar un tumor irresecable no pasible de cirugía, se indicó el uso compasivo de lenvatinib, que mostró una rápida y favorable respuesta clínica con resolución de la insuficiencia respiratoria al noveno día del tratamiento.El diagnóstico temprano de cáncer papilar de tiroides previene la grave morbilidad respiratoria ocasionada por diagnósticos tardíos. Podría considerarse el uso de lenvatinib como alternativa previa a las terapias de primera línea (cirugía e iodo radiactivo) en casos de enfermedad con gran compromiso local y a distancia.

Papillary thyroid cancer is the most common thyroid tumor in childhood. In advanced stages, it can present with respiratory failure. The treatment of choice is total thyroidectomy and radioactive iodine. In cases of unresectable tumors, therapy with multikinase inhibitors should be considered. A 10-year-old girl was referred for progressive respiratory failure. A diagnosis of papillary thyroid cancer with pulmonary metastases was made. Due to the presence of an unresectable tumor not subject to surgery, the compassionate use of lenvatinib was indicated, showing a rapid and favorable clinical response with resolution of respiratory failure on the ninth day.Early diagnosis of papillary thyroid cancer prevents severe respiratory morbidity caused by late diagnoses. The use of lenvatinib should be considered as a previous step towards first-line therapies (surgery and radioactive iodine) in cases with great local and distant involvement.

[Response to lenvatinib in a pediatric patient with respiratory failure associated with papillary thyroid carcinoma]. / Respuesta al lenvatinib en una paciente pediátrica con insuficiencia respiratoria secundaria a carcinoma papilar de tiroides.

Papillary thyroid cancer is the most common thyroid tumor in childhood. In advanced stages, it can present with respiratory failure. The treatment of choice is total thyroidectomy and radioactive iodine. In cases of unresectable tumors, therapy with multikinase inhibitors should be considered. A 10-year-old girl was referred for progressive respiratory failure. A diagnosis of papillary thyroid cancer with pulmonary metastases was made. Due to the presence of an unresectable tumor not subject to surgery, the compassionate use of lenvatinib was indicated, showing a rapid and favorable clinical response with resolution of respiratory failure on the ninth day. Early diagnosis of papillary thyroid cancer prevents severe respiratory morbidity caused by late diagnoses. The use of lenvatinib should be considered as a previous step towards first-line therapies (surgery and radioactive iodine) in cases with great local and distant involvement.

El cáncer papilar de tiroides es el tumor tiroideo más común en la infancia. En estadios avanzados, puede presentarse con cuadro de insuficiencia respiratoria. El tratamiento de elección es la tiroidectomía total y iodo radiactivo. En tumores irresecables, se debería considerar terapia con inhibidores multicinasa. Niña de 10 años de edad derivada por insuficiencia respiratoria progresiva. Se realizó el diagnóstico de cáncer papilar de tiroides con metástasis pulmonares. Por presentar un tumor irresecable no pasible de cirugía, se indicó el uso compasivo de lenvatinib, que mostró una rápida y favorable respuesta clínica con resolución de la insuficiencia respiratoria al noveno día del tratamiento. El diagnóstico temprano de cáncer papilar de tiroides previene la grave morbilidad respiratoria ocasionada por diagnósticos tardíos. Podría considerarse el uso de lenvatinib como alternativa previa a las terapias de primera línea (cirugía e iodo radiactivo) en casos de enfermedad con gran compromiso local y a distancia.

Childhood liver tumors in Argentina: Incidence trend and survival by treatment center. A report from the national pediatric cancer registry, ROHA network 2000-2015.

BACKGROUND: Information on the epidemiology of pediatric liver tumors in Latin America is limited. PURPOSE: To describe the incidence of liver tumors in a pediatric registry in Argentina according to geographic region, national trends over 16 years, and survival related to stage, age, sex, and care center. METHODS: Newly diagnosed liver tumors cases are registered in the Argentine Pediatric Oncology Hospital Registry (ROHA) with an estimated coverage of 91% of national cases. Age-standardized incidence rate per millon (ASR) was calculated based on the National Vital Statistics Reports. Five-year overall survival (OS) was estimated using the Kaplan-Meier method. The log-rank test was used to compare subgroup survival. RESULTS: Two hundred seven cases of hepatoblastoma (HB) and 73 of hepatocellular carcinoma (HCC) were identified. ASR of liver tumors was 1.8/million (95% confidence Interval [CI], 1.6-2.0) per year. ASR was 1.4 (1.2-1.6) for HB and 0.4 (0.3-0.5) for HCC. For HB, the highest incidence was found in the northwest region including the Altiplano. OS was 60.4% (53.4-66.8) for HB and 36.1% (25.2-47.2) for HCC. Five-year survival rate of children with metastatic HB treated at liver transplant hospitals (LTH) was 54.2% (30.3-73.0) compared to 13.3% (2.2-34.6) for those seen at other hospitals (OH) (P = .02), while for HCC this rate was 46.3% (30.7-60.6) at LTH compared to 17.5% (3.1-41.9) at OH (P = .01). CONCLUSIONS: The incidence rate of pediatric liver tumors was stable over the 16-year study period. Patients may benefit if at treatment initiation they are evaluated jointly with LTH specialists to define treatment strategies.

[Evolution of children one year post liver transplant]. / Evolución de niños post-trasplante hepático luego del primer año de sobrevida.

Orthotopic liver transplantation is the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the necessity to prevent long-term complications. The aim of this study was to analyze the evolution of transplanted patients with more than one year of follow up. Between November 1992 and November 2001, 238 patients underwent 264 liver transplantations. A total of 143 patients with more than one year of follow up were included. The median age of patients +/- SD was 5.41 years +/- 5.26 (r: 0.58-21.7 years). All children received primary immunosuppression with cyclosporine. The indications for liver replacement were: fulminant hepatic failure (n: 50), biliary atresia (n: 38), cirrhosis (n: 37), chronic cholestasis (n: 13) and miscellaneous (n: 5). The indications for liver re-transplantation were: biliary cirrhosis (n: 7), hepatic artery thrombosis (n: 4) and chronic rejection (n: 3). Reduced-size liver allografts were used in 73/157 liver transplants, 14 of them were from living-related donors and 11 were split-livers. Patient and graft survival rates were 93% and 86% respectively. Death risk was statistically higher in retransplanted and reduced-size grafted patients. Growth retardation and low bone density were recovered before the first 3 years post-transplant. The incidence of lymphoproliferative disease was 7.69%. De novo hepatitis B was diagnosed in 7 patients (4.8%). Social risk did not affect the outcome of our population. The prevention, detection and early treatment of complications in the long-term follow up contributed to improve the outcome.

Evolución de niños post-trasplante hepático luego del primer año de sobrevida. / [Evolution of children one year post liver transplant]

Orthotopic liver transplantation is the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the necessity to prevent long-term complications. The aim of this study was to analyze the evolution of transplanted patients with more than one year of follow up. Between November 1992 and November 2001, 238 patients underwent 264 liver transplantations. A total of 143 patients with more than one year of follow up were included. The median age of patients +/- SD was 5.41 years +/- 5.26 (r: 0.58-21.7 years). All children received primary immunosuppression with cyclosporine. The indications for liver replacement were: fulminant hepatic failure (n: 50), biliary atresia (n: 38), cirrhosis (n: 37), chronic cholestasis (n: 13) and miscellaneous (n: 5). The indications for liver re-transplantation were: biliary cirrhosis (n: 7), hepatic artery thrombosis (n: 4) and chronic rejection (n: 3). Reduced-size liver allografts were used in 73/157 liver transplants, 14 of them were from living-related donors and 11 were split-livers. Patient and graft survival rates were 93

and 86

respectively. Death risk was statistically higher in retransplanted and reduced-size grafted patients. Growth retardation and low bone density were recovered before the first 3 years post-transplant. The incidence of lymphoproliferative disease was 7.69

. De novo hepatitis B was diagnosed in 7 patients (4.8

). Social risk did not affect the outcome of our population. The prevention, detection and early treatment of complications in the long-term follow up contributed to improve the outcome.

Variantes anatómicas de la arteria hepática: la importancia de su reconocimiento en la cirugía digestiva / Anatomic variants of the hepatic artery: importance for the digestive surgery

Objetivo: describir las variantes anatómicas de la arteria hepática, resaltar su importancia en la cirugía digestiva y sugerir pautas para su reconocimiento tanto pre como intraoperatorio. Lugar de aplicación: dos hospitales públicos de alta complejidad. Diseño: longitudinal, retrospectivo, descriptivo. Población: 414 hígados para ser implantados en receptores de Trasplante Hepático, 374 fueron obtenidos de donante cadavéricos y 40 segmentados laterales izquierdo de donante vivo relacionado. Método: se disecaron y clasificaron de acuerdo a su disposición anatómica (según la clasificación propuesta por Michels y modificada por Hiatt) todas las arterias hepáticas de 374 hígados obtenidos de donante cadavérico y 40 de donante vivo relacionado. Resultados: encontramos un 70,7 por ciento de variantes tipo I (distribución normal), 10,6 por ciento tipo II (rama izquierda de la coronaria estomáquica), 7 por ciento tipo III (rama derecha de la Mesentérica superior), 4,8 por ciento tipo IV (triple arteria), 2 por ciento tipo V (hepática común de Mesentérica), 0,8 por ciento tipo VI (hepática común directa de aorta) y 4 por ciento otras variantes no clasificadas. Conclusión: las variantes anatómicas de la arteria hepática son más frecuentes de lo que uno puede esperar y rondan entre el 25 y 40 por ciento. Es importante su estudio preoperatorio y su reconocimiento intraquirúrgico para evitar lesiones de alguna de sus ramas que deriven en consecuencias graves e irreversibles tanto sobre la vía biliar como el hígado

Variantes anatómicas de la arteria hepática: la importancia de su reconocimiento en la cirugía digestiva / Anatomic variants of the hepatic artery: importance for the digestive surgery

Objetivo: describir las variantes anatómicas de la arteria hepática, resaltar su importancia en la cirugía digestiva y sugerir pautas para su reconocimiento tanto pre como intraoperatorio. Lugar de aplicación: dos hospitales públicos de alta complejidad. Diseño: longitudinal, retrospectivo, descriptivo. Población: 414 hígados para ser implantados en receptores de Trasplante Hepático, 374 fueron obtenidos de donante cadavéricos y 40 segmentados laterales izquierdo de donante vivo relacionado. Método: se disecaron y clasificaron de acuerdo a su disposición anatómica (según la clasificación propuesta por Michels y modificada por Hiatt) todas las arterias hepáticas de 374 hígados obtenidos de donante cadavérico y 40 de donante vivo relacionado. Resultados: encontramos un 70,7 por ciento de variantes tipo I (distribución normal), 10,6 por ciento tipo II (rama izquierda de la coronaria estomáquica), 7 por ciento tipo III (rama derecha de la Mesentérica superior), 4,8 por ciento tipo IV (triple arteria), 2 por ciento tipo V (hepática común de Mesentérica), 0,8 por ciento tipo VI (hepática común directa de aorta) y 4 por ciento otras variantes no clasificadas. Conclusión: las variantes anatómicas de la arteria hepática son más frecuentes de lo que uno puede esperar y rondan entre el 25 y 40 por ciento. Es importante su estudio preoperatorio y su reconocimiento intraquirúrgico para evitar lesiones de alguna de sus ramas que deriven en consecuencias graves e irreversibles tanto sobre la vía biliar como el hígado (AU)