Kaposi sarcoma in three pediatric liver transplantation recipients.

BACKGROUND: Kaposi sarcoma (KS) is an endothelial cell tumor, rare in children. It is 200 times more frequent after solid organ transplantation than in the general population. METHODS: We report three cases of pediatric patients who developed KS after liver transplantation (LT). RESULTS: Case 1, a 4-year-old boy undergoing LT due to familial intrahepatic cholestasis. Five months after LT, he presented with fever, dyspnea, and cough with enlarged lymph nodes and splenomegaly, anemia, thrombocytopenia, elevated liver enzymes, and positive EBV viral load. Lymph node biopsy diagnosed KS with an elevated HHV8 viral load. Case 2, a 4-year-old boy who underwent LT due to secondary biliary cirrhosis resulting from extrahepatic biliary atresia. Two years later, graft dysfunction was noticed with positive EBV viral load, thrombocytopenia, massive cervical lymph node enlargement, and splenomegaly. Lymph node biopsy diagnosed KS, Castleman's disease, and plasmablastic lymphoma related to HHV8 infection. Case 3, a 15-month-old girl, who received two LT due to biliary cirrhosis. Six months later, she presented with diarrhea, abdominal distension, anemia, thrombocytopenia, enlarged lymph nodes, splenomegaly, and positive CMV viral load. Axillary lymph node biopsy diagnosed KS and HHV8 infection was confirmed. In all three cases, tacrolimus was discontinued and, after diagnosis, sirolimus was started. All recovered without relapse and have a good graft function. CONCLUSIONS: Kaposi sarcoma is a rare disease post-LT in children. Recognizing keywords and early diagnosis is crucial for timely treatment and survival.

Effectiveness of Intra-Arterial Chemotherapy for the Treatment of Intraocular Retinoblastoma: Relevance of a Multidisciplinary Setting.

Purpose: We aim to report about effectiveness and safety in the context of our centers' setting in the management of retinoblastoma with intra-arterial chemotherapy (IAC) in a 5-year retrospective analysis of the Portuguese population. Patients and Methods: Retrospective analysis of consecutive cases of retinoblastoma selected to initiate IAC between 2015 and 2020, at the Portuguese National Reference Center. All included patients underwent complete ophthalmological evaluation under anesthesia with fundus photography. Diagnosis and classification of retinoblastoma was made according to the International Classification of Intraocular Retinoblastoma (ICRB). The patients were further divided into two groups: Group I for primary IAC and Group II for secondary IAC. Tumor recurrence or relapses, systemic metastasis and deaths were documented. Main efficacy outcome included ocular salvage and recurrence-free survival rates estimated using the Kaplan-Meier method. Results: Twenty-eight eyes (19 eyes included in Group I and 9 eyes included in Group II) were eligible and a total of 130 IAC procedures were performed, with a median number of sessions of 4 (range 1-8) for each treated eye, during a median follow-up of 21 months (range 4-64). Of the included eyes, 22 (78.6%) were preserved. An overall survival of 100% was achieved. Considering the preserved eyes, the overall median decimal visual acuity achieved at the last visit was 0.15 (range 0.02-0.8). Three patients had permanent adverse events related to IAC (cataract, vitreous hemorrhage and choroidal ischemia). Considering the survival analysis of recurrence, the mean survival without recurrence was 84.2% for Group I and 66.7% for Group II, and the mean survival without enucleation was 78.6% (no events in Group II). Conclusion: IAC has been shown to be an effective and safe treatment for children with intraocular retinoblastoma. This study demonstrates that IAC is effective even in moderate sample sizes, when a multidisciplinary approach is available.

Characterization of the Portuguese population diagnosed with retinoblastoma.

The purpose of this study is to characterize demographically and genetically the Portuguese population with retinoblastoma; to report the clinical stage at presentation and its impact on survival and ocular preservation rate and, finally, to assess the incidence of retinoblastoma in Portugal. Retrospective observational study including children consecutively diagnosed with retinoblastoma at the Portuguese National Referral Center of Intraocular Tumors, between October 2015 and October 2020. Twenty-eight children were diagnosed with retinoblastoma at our center, 15 hereditary from which 12 presented with bilateral retinoblastoma and 3 were unilateral. The overall mean age at diagnosis was 13.6 ± 11.1 months with hereditary retinoblastomas diagnosed slightly earlier at 9.6 ± 6.3 months. A familial history of retinoblastoma was found in only 4 (14.3%) of the cases. A pathogenic mutation in the RB1 gene was found in 13 (46.4%) of the children. The most frequent sign at referral was leukocoria in 71.4% of patients. Considering the ICRB classification of the tumors, 84.6% of non-hereditable hereditary retinoblastomas were referred to our center in advanced stages. In the group of hereditable retinoblastomas 86.7% presented with one of the eyes with advanced intraocular retinoblastoma. Fourteen children had one eye enucleated due to retinoblastoma. No deaths were registered during the study period. Considering the incidence analysis, we registered a year-of-birth controlled incidence analysis of 4.04 per 100.000 living births (IC 95% 1.59-6.49). This is the first characterization of the Portuguese Population diagnosed with Retinoblastoma in the National Reference Center.

Erdheim-Chester disease: a rare non-Langerhans histiocytosis.

A 3-year-old girl was admitted to our hospital with diabetes insipidus and a left eye proptosis. During investigation of diabetes insipidus, an extensive osteolytic mass, involving skull base and maxillo-facial bones, was revealed. Biopsy exhibited dense infiltrate of foamy histiocytes, which were positive for CD68 and CD163 and negative for CD1a and S100 confirming histopathological diagnosis of Erdheim-Chester disease. Treatment with dabrafenib was initiated, with good response and no side effects.

Treatment of Advanced Retinoblastoma in Children Evacuated from Low-Income Countries: Experience from a National Referral Center in Portugal.

OBJECTIVE: The aim of our study was to characterize the evacuated African patients diagnosed with retinoblastoma and referred to the Portuguese national referral center (Centro Hospital e Universitário de Coimbra, University of Coimbra), identifying inequalities in the stage of diagnosis and prognostic results. DESIGN: Retrospective observational study of evacuated African patients diagnosed with retinoblastoma and referred to the Portuguese National Referral Center (Centro Hospital e Universitário de Coimbra, University of Coimbra). RESULTS: The study included 15 patients between October 2015 and October 2020 from Angola, Cape Verde, Guinea-Bissau and São Tomé and Príncipe. Seven (46.7%) children presented bilateral retinoblastoma. The median age at the time of diagnosis was 20.9 (interquartile range, 16-41) months. The presenting symptoms were leukocoria (86.7%), strabism (53.3%) and buphthalmus (40%). In terms of tumor staging, five (33.3%) children presented with extraocular retinoblastoma and 10 (66.7%) children presented with intraocular retinoblastoma. At presentation, no pineal involvement was diagnosed but two (13.3%) children presented with central nervous system involvement at the time of the first observation. Children were treated with enucleation, exenteration, systemic chemotherapy, intra-arterial chemotherapy and/or supportive palliative care. During the follow-up period (mean 27.2 ± 18.2 months), the overall survival was 73.3%. CONCLUSION: A small proportion of African children are being referred to our center, when considering the expected incidence of retinoblastoma in these countries, and referred children arrive at advanced stages of the disease, compromising treatment outcomes. Considering retinoblastoma is now a curable disease, national and international interventions are required to attempt a better management of children born in low-income countries.

Current Practices on Diagnosis, Prevention and Treatment of Post-Transplant Lymphoproliferative Disorder in Pediatric Patients after Solid Organ Transplantation: Results of ERN TransplantChild Healthcare Working Group Survey.

(1) Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT). However, there is lack of consensus in PTLD management. Our aim was to establish a present benchmark for comparison between international centers and between various organ transplant systems and modalities; (2) Methods: A cross-sectional questionnaire of relevant PTLD practices in pediatric transplantation was sent to multidisciplinary teams from 17 European center members of ERN TransplantChild to evaluate the centers' approach strategies for diagnosis and treatment and how current practices impact a cross-sectional series of PTLD cases; (3) Results: A total of 34 SOT programs from 13 European centers participated. The decision to start preemptive treatment and its guidance was based on both EBV viremia monitoring plus additional laboratory methods and clinical assessment (61%). Among treatment modalities the most common initial practice at diagnosis was to reduce the immunosuppression (61%). A total of 126 PTLD cases were reported during the period 2012-2016. According to their histopathological classification, monomorphic lesions were the most frequent (46%). Graft rejection after PTLD remission was 33%. Of the total cases diagnosed with PTLD, 88% survived; (4) Conclusions: There is still no consensus on prevention and treatment of PTLD, which implies the need to generate evidence. This might successively allow the development of clinical guidelines.

Pharmacokinetic analysis of etoposide distribution after administration directly into the fourth ventricle in a piglet model.

We hypothesize that infusion of chemotherapeutic agents directly into the fourth ventricle potentially may play a role in treating malignant posterior fossa brain tumors. Accordingly, we used a piglet model developed in our laboratory to test the safety of etoposide infusions into the fourth ventricle and to study the pharmacokinetics associated with these infusions. In 5 piglets, closed-tip silicone catheters were inserted into the fourth ventricle and lumbar cistern. Five consecutive daily infusions of etoposide (0.5 mg) were administered via the fourth ventricle catheter. Serum and CSF from both catheters were sampled for measurement of etoposide level by reversed-phase high performance liquid chromatography (HPLC). For CSF samples, area under the concentration-time curve (AUC) was calculated. Piglets underwent daily neurological examinations, a 4.7 Tesla MRI scan, and then were sacrificed for post-mortem brain examination. No neurological deficits or signs of meningitis were caused by intraventricular chemotherapy infusions. MRI scans showed catheter placement within the fourth ventricle but no signal changes in the brain stem or cerebellum. In all piglets, the mean fourth ventricular CSF peak etoposide level exceeded the mean peak lumbar etoposide levels by greater than 10-fold. Statistically significant differences between fourth ventricle and lumbar AUC were noted at peaks (DeltaAUC = 3384196 ng h/ml with 95%CI: 1758625, 5009767, P = 0.0044) and at all collection time points (DeltaAUC = 1422977 ng h/ml with 95%CI: 732188, 2113766, P = 0.0046) but not at troughs (DeltaAUC = -29546 ng h/ml (95%CI: -147526, 88434.2, P = 0.5251). Serum etoposide was absent at two and four hours after intraventricular infusions in all animals. Pathological analysis demonstrated meningitis, choroid plexitis, and ependymitis in the fourth and occasionally lateral ventricles. Etoposide can be infused directly into the fourth ventricle without clinical or radiographic evidence of damage. Autopsy examination revealed ventriculitis and meningitis which did not have a clinical correlate. Etoposide does not distribute evenly throughout CSF spaces after administration into the fourth ventricle, and higher peak CSF levels are observed in the fourth ventricle than in the lumbar cistern.

Safety and pharmacokinetic analysis of methotrexate administered directly into the fourth ventricle in a piglet model.

We have developed a piglet model to assess chemotherapy administration directly into the fourth ventricle as a potential treatment for medulloblastoma and other malignant posterior fossa tumors. The objective of this study was to assess safety and pharmacokinetics after methotrexate infusions into the fourth ventricle. Catheters were inserted into the fourth ventricle and lumbar cistern in five piglets. Two milligrams of Methotrexate (MTX) was infused into the fourth ventricle on five consecutive days. Safety was assessed by neurological examination, 4.7 T MRI, and post-mortem pathological analysis. MTX levels in serum and cerebrospinal fluid (CSF) were measured, and area under the concentration-time curve (AUC) was calculated for CSF samples. No neurological deficits were caused by MTX infusions. One piglet died from complications of anesthesia induction for MRI scanning. MRI scans showed accurate catheter placement without signal changes in the brainstem or cerebellum. One piglet had asymptomatic ventriculomegaly. Pathological analysis demonstrated meningitis and choroid plexitis consisting predominantly of CD-3 positive T-lymphocytes in all piglets and a small focal area of subependymal necrosis in one. In all piglets, mean peak MTX level in fourth ventricular CSF exceeded that in lumbar CSF by greater than five-fold. Serum MTX levels were undetectable or negligible. Statistically significant differences between fourth ventricle and lumbar AUC were detected at peaks (P = 0.01) and at all collection time points (P = 0.01) but not at troughs (P = 0.36). MTX can be infused into the fourth ventricle without clinical or radiographic evidence of damage. An inflammatory response without clinical correlate is observed. Significantly higher peak MTX levels are observed in the fourth ventricle than in the lumbar cistern.

Protein tyrosine phosphatase receptor type O regulates development and function of the sensory nervous system.

The roles of protein tyrosine phosphatases (PTPs) in differentiation and axon targeting by dorsal root ganglion (DRG) neurons are essentially unknown. The type III transmembrane PTP, PTPRO, is expressed in DRG neurons, and is implicated in the guidance of motor and retinal axons. We examined the role of PTPRO in DRG development and function using PTPRO(-/-) mice. The number of peptidergic nociceptive neurons in the DRG of PTPRO(-/-) mice was significantly decreased, while the total number of sensory neurons appeared unchanged. In addition, spinal pathfinding by both peptidergic and proprioceptive neurons was abnormal in PTPRO(-/-) mice. Lastly, PTPRO(-/-) mice performed abnormally on tests of thermal pain and sensorimotor coordination, suggesting that both nociception and proprioception were perturbed. Our data indicate that PTPRO is required for peptidergic differentiation and process outgrowth of sensory neurons, as well as mature sensory function, and provide the first evidence that RPTPs regulate DRG development.

Acute changes in systemic hemodynamics and serum vasopressin after complete cervical spinal cord injury in piglets.

BACKGROUND: Spinal cord injury (SCI) produces acute hemodynamic alterations through disruption of sympathetic output of the autonomic nervous system and places individuals with SCI at high risk of secondary ischemic insult to the spinal cord as well as to other organs. The purpose of this study was to examine hemodynamics and serum vasopressin concentration in the acute period following complete cervical SCI in piglets. METHODS: We developed a new model of traumatic complete cervical SCI in piglets and measured acute hemodynamic variables and serum arginine vasopressin (AVP) concentrations at baseline and for 4 h after SCI under fentanyl anesthesia. RESULTS: Complete cervical SCI caused an immediate tachycardia which lasted for approximately 1 h, immediate hypotension which was sustained for the 4-h duration of the study, decreases in both systemic and pulmonary vascular resistance, and a compensatory increase in cardiac output, which resulted initially from an increase in heart rate (HR) but was later sustained after resolution of tachycardia by an increase in cardiac stroke volume. Serum AVP concentration increased significantly after SCI and did not change in the control group. Neurogenic shock did not occur due to the robust increase in cardiac output and cardiac stroke volume. CONCLUSIONS: Complete cervical SCI produces hemodynamic alterations consistent with the withdrawal of sympathetic tone. Although mean arterial pressure (MAP) decreased significantly after SCI, the increase in serum vasopressin may have played a role in maintaining blood pressure and preventing circulatory collapse, a complication which is encountered frequently in patients with cervical and upper thoracic SCI.

Pediatric spinal cord injury in infant piglets: description of a new large animal model and review of the literature.

OBJECTIVE: To develop a new, clinically relevant large animal model of pediatric spinal cord injury (SCI) and compare the clinical and experimental features of pediatric SCI. METHODS: Infant piglets (3-5 weeks old) underwent contusive SCI by controlled cortical impactor at T7. Severe complete SCI was induced in 6 piglets, defined as SCI with no spontaneous return of sensorimotor function. Eight piglets received incomplete SCI, which was followed by partial recovery. Somatosensory evoked potentials, magnetic resonance imaging, neurobehavioral function, and histopathology were measured during a 28-day survival period. RESULTS: Mean SCI volume (defined as volume of necrotic tissue) was larger after complete compared with incomplete SCI (387 +/- 29 vs 77 +/- 38 mm3, respectively, P < 0.001). No functional recovery occurred after complete SCI. After incomplete SCI, piglets initially had an absence of lower extremity sensorimotor function, urinary and stool retention, and little to no rectal tone. Sensory responses recovered first (1-2 days after injury), followed by spontaneous voiding, lower extremity motor responses, regular bowel movements, and repetitive flexion-extension of the lower extremities when crawling. No piglet recovered spontaneous walking, although 4 of 8 animals with incomplete injuries were able to bear weight by 28 days. In vivo magnetic resonance imaging was performed safely, yielded high-resolution images of tissue injury, and correlated closely with injury volume seen on histopathology, which included intramedullary hemorrhage, cellular inflammation, necrosis, and apoptosis. CONCLUSION: Piglets performed well as a reproducible model of traumatic pediatric SCI in a large animal with chronic survival and utilizing multiple outcome measures, including evoked potentials, magnetic resonance imaging, functional outcome scores, and histopathology.

Is cancer latency an outdated concept? Lessons from chronic myeloid leukemia.

Our concept of cancer latency, the interval from when a cancer starts until it is diagnosed, has changed dramatically. A prior widely-used definition was the interval between an exposure to a cancer-causing substance and cancer diagnosis. However, this definition does not accurately reflect current knowledge of how most cancers develop assuming, mostly incorrectly, one exposure is the sole cause of a cancer, ignoring the possibility the cancer being considered would have developed anyway but that the exposure accelerated cancer development and eliding the randomness in when a cancer is diagnosed. We show, using chronic myeloid leukaemia as a model, that defining cancer latency is not as simple as it once seemed. It is difficult or impossible to know at which event or mutation to start to clock to measure cancer latency. It is equally difficult to know when to stop the clock given the stochastic nature of when cancers are diagnosed. Importantly, even in genetically-identical twins with the same driver mutation intervals to develop cancer vary substantially. And we discuss other confonders. Clearly we need a new definition of cancer latency or we need to abandon the concept of cancer latency in the modern era of cancer biology.

Post traumatic brain perfusion SPECT analysis using reconstructed ROI maps of radioactive microsphere derived cerebral blood flow and statistical parametric mapping.

BACKGROUND: Assessment of cerebral blood flow (CBF) by SPECT could be important in the management of patients with severe traumatic brain injury (TBI) because changes in regional CBF can affect outcome by promoting edema formation and intracranial pressure elevation (with cerebral hyperemia), or by causing secondary ischemic injury including post-traumatic stroke. The purpose of this study was to establish an improved method for evaluating regional CBF changes after TBI in piglets. METHODS: The focal effects of moderate traumatic brain injury (TBI) on cerebral blood flow (CBF) by SPECT cerebral blood perfusion (CBP) imaging in an animal model were investigated by parallelized statistical techniques. Regional CBF was measured by radioactive microspheres and by SPECT 2 hours after injury in sham-operated piglets versus those receiving severe TBI by fluid-percussion injury to the left parietal lobe. Qualitative SPECT CBP accuracy was assessed against reference radioactive microsphere regional CBF measurements by map reconstruction, registration and smoothing. Cerebral hypoperfusion in the test group was identified at the voxel level using statistical parametric mapping (SPM). RESULTS: A significant area of hypoperfusion (P < 0.01) was found as a response to the TBI. Statistical mapping of the reference microsphere CBF data confirms a focal decrease found with SPECT and SPM. CONCLUSION: The suitability of SPM for application to the experimental model and ability to provide insight into CBF changes in response to traumatic injury was validated by the SPECT SPM result of a decrease in CBP at the left parietal region injury area of the test group. Further study and correlation of this characteristic lesion with long-term outcomes and auxiliary diagnostic modalities is critical to developing more effective critical care treatment guidelines and automated medical imaging processing techniques.

Fracturas supracondíleas en pacientes pediátricos tratados quirúrgicamente con Técnica de Cruzada. Hospital Central San Cristóbal / Supracondylar humerus fractures in childrens surgically treated by Crossed Wiring Technique. San Cristóbal Central Hospital

Las fracturas supracondíleas en niños se asocian con algunas complicaciones. Su tratamiento estándar es la reducción cerrada y la fijación con alambres en distintas configuraciones. Se realizó un estudio observacional, analítico, prospectivo y longitudinal con pacientes pediátricos que ingresaron con fracturas supracondíleas de húmero tratados quirúrgicamente con alambres de Kirschner con "Técnica Cruzada" en el Hospital Central San Cristóbal enero a junio de 2022. Se incluyeron 30 pacientes. La edad promedio fue 6,3±2,2(3-10) años. La causa más frecuente del traumatismo fue la precipitación de altura en 63,3% de los casos. La posición del codo al momento del accidente fue en extensión en 90%. Todas las fracturas fueron cerradas. El tiempo promedio desde el accidente hasta su atención en emergencia fue de 8,5±13,4(2-72) horas. Los signos clínicos más frecuentes fueron dolor en 100,0%, limitación funcional en 96,7%, aumento de volumen 73,3% y deformidad 50,0%. Según la clasificación AO la más frecuente fue del tipo 13-M/3. 1 III en el 50% de los casos y según Gartland, las tipo IIIA en 53,3%. A las 4 semanas, 100,0% de las fracturas consolidaron, 13,3% presentó valgo y 6,7% varo en la radiografía anteroposterior. Mientras que, en la lateral, 33,3% antecurvatum. La media del ángulo de Baumann fue de 20,27±1,39 grados. La tasa de complicaciones fue de 16,66%, 2(6,7%) casos presentaron neuropraxia y 3(10,0%) granuloma. En conclusión, la Técnica Cruzada es segura en términos de reducción, funcionalidad y tasas de complicaciones en el seguimiento a medio plazo(AU)

Supracondylar fractures in children are associated with some complications. Its standard treatment is closed reduction and fixation with wires in different configurations. An observational, analytical, prospective and longitudinal study was made, with pediatric patients admitted with supracondylar humeral fractures surgically treated with Kirschner wires with the "Cross Technique" at the Hospital Central San Cristóbal from January to June 2022. 30 patients were included. The mean age was 6,3±2,2(3-10) years. The most frequent cause of trauma was high altitude precipitation in 63,3% of the cases. The position of the elbow at the time of the accident was 90% extended. All fractures were closed. The mean time from the accident to emergency care was 8,5±13,4(2-72) hours. The most frequent clinical signs were pain in 100,0%, functional limitation in 96.7%, volume increase in 73,3%, and deformity in 50,0%. According to the AO classification, the most frequent was type 13-M/3. 1 III in 50% of cases and according to Gartland, type IIIA in 53,3%. At 4 weeks, 100,0% of the patients consolidated, 13,3% presented valgus and 6,7% varus on the anteroposterior radiograph. While, on the side, 33,3% antecurvatum. The mean Baumann angle was 20,27 ± 1.39 degrees. The rate of complications was 16,66%, 2 (6,7%) cases presented neuropraxia and 3 (10,0%) granuloma. In conclusion, the Crossover Technique is safe in terms of reduction, functionality, and complication rates in medium-term follow-up(AU)

Sacral Myeloid Sarcoma Manifesting as Radiculopathy in a Pediatric Patient: An Unusual Form of Myeloid Leukemia Relapse.

Myeloid sarcoma (MS), granulocytic sarcoma or chloroma, is defined as a localized extramedullary mass of blasts of granulocytic lineage with or without maturation, occurring outside the bone marrow. MS can be diagnosed concurrently with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). The authors report a case of sacral MS occurring as a relapse of myeloid leukemia in a 5-year-old girl who was taken to the emergency department with radiculopathy symptoms.

Aplicación clínica de la Impresión 3D en el manejo de fracturas abiertas de tibia: Nuevas fronteras en Traumatología. Estudio experimental / Clinical application of 3D Printing in open tibial fractures management: New frontiers of Orthopaedics. An experimental study

La Impresión 3D es una tecnología emergente utilizada cada vez más en medicina. En los países en vías de desarrollo, donde las fracturas por motocicletas y automóviles se encuentran en aumento, la disponibilidad de fijadores externos para el manejo de fracturas abiertas es un problema frecuente. La impresión 3D puede ser una alternativa económica e igualmente confiable a los dispositivos tradicionales elaborados con acero o titanio. El objetivo de este trabajo es mostrar la experiencia con el uso de Impresión 3D y su aplicación en el manejo clínico de fracturas abiertas diafisiarias de tibia. Se realizó un estudio pre-experimental y prospectivo. Se incluyeron 14 pacientes con fracturas de tibia AO/ASIF 42A, 42B y 42C tratados con un fijador externo con rótulas elaboradas con Impresión 3D como medida de Control de Daños en Ortopedia desde su ingreso hasta su resolución definitiva. Todos los pacientes fueron de sexo masculino, con un promedio de edad 23,16 años con 50% entre 20-23 años. Las fracturas fueron 42,85% tipo 42A, 37,71% 42B y 21,42% 42C. El 78,57% de las fracturas fueron ocasionadas por motocicletas: 57,14% grado II según Gustilo y Anderson, un 28,57% grado III y 14,28% grado I. El 37,71% eran politraumatizados. Ninguno de los pacientes presentó complicaciones como pérdida de la reducción, aflojamiento de las rótulas, ruptura o fatiga de las rótulas ni fatiga de la barra. La impresión 3D demostró ser una herramienta y alternativa útil en el manejo agudo de fracturas abiertas diafisiarias de tibia(AU)

3D Printing is an emerging technology used more and more in medicine. In developing countries, where motorcycle and automobile fractures are on the rise, the availability of external fixators for the management of open fractures is a frequent problem. 3D printing can be a cheap and equally reliable alternative to traditional devices made of steel or titanium. The objective of this work is to show the experience with the use of 3D Printing and its application in the clinical management of open diaphyseal fractures of the tibia. A pre-experimental and prospective study was made. 14 patients with AO/ASIF tibia fractures 42A, 42B and 42C treated with an external fixator with 3D-printed ball-caps as a Damage Control measure in Orthopedics from admission to final resolution were included. All patients were male, with an average age of 23,16 years, 50% between 20-23 years. The fractures were 42,85% type 42A, 37,71% 42B and 21,42% 42C. 78,57% of the fractures were caused by motorcycles: 57,14% grade II according to Gustilo and Anderson, 28,57% grade III and 14.28% grade I. 37,71% were polytraumatized. None of the patients had complications such as loss of reduction, loosening of the patellas, rupture or fatigue of the patellas, or rod fatigue. 3D printing proved to be a useful tool and alternative in the acute management of open diaphyseal fractures of the tibia(AU)

Medición radiológica del extremo proximal del fémur / Radiological measurement of the femoral proximal end

El objetivo de este trabajo es determinar las mediciones radiológicas del extremo proximal del fémur en pacientes adultos cuyos casos fueron presentados en las discusiones clínico-radiológicas del servicio de Traumatología y Ortopedia del Hospital Central de San Cristóbal entre 2015 y 2021. Se realizó un estudio prospectivo, descriptivo, transversal, que incluyó los estudios radiológicos de cadera sana en proyección anteroposterior de pelvis centrada en pubis con rotación interna de 15 %, de 126 pacientes entre 20 y 95 años. Se midieron: ángulo cervicodiafisario, lateralización femoral, longitud y ancho del cuello femoral y diámetro de la circunferencia cefálica, mediante el sotfware MicroDicom DICOM 4.0.0. La media de ángulo cervicodiafisario fue 130,8 ± 4,5 grados, en 57 individuos (45,2 %) estuvo entre 127,4 y 133,3 grados (p = 0,001); la circunferencia cefálica media fue 42,0 ± 2,4 mm, en 60 personas (47,6 %) estuvo entre 40,2 y 45,7 mm (p = 0,001); la longitud cervical media fue 78,6 ± 16,4 mm, en 54 individuos (42,9 %) estuvo entre 69,4 y 92 mm (p = 0,001); el ancho cervical medio fue 75,9 ± 12,1 mm, en 64 casos, (50,8 %) estuvo entre 62,9 y 78,7 mm (p = 0,001). En 60 individuos (47,6 %) la lateralización del fémur estuvo entre 92,6 - 117,7 mm. Las medidas son independientes del sexo; a medida que aumenta la edad, el ángulo cervicodiafisario es menor (p= 0,021). Se realizaron tablas percentiladas de las medidas radiológicas del extremo proximal del fémur, según edad y sexo, que pueden servir de referencia en pacientes futuros(AU)

The objective of this work is to determine the radiological measurements of the proximal end of the femur in adult patients whose cases were presented in the clinical-radiological discussions of the Traumatology and Orthopedics service of the Central Hospital of San Cristóbal between 2015 and 2021. A prospective study was carried out, descriptive, crosssectional, which included radiological studies of a healthy hip in anteroposterior projection of the pelvis centered on the pubis with internal rotation of 15%, of 126 patients between 20 and 95 years of age. The following were measured: cervicodiaphyseal angle, femoral lateralization, length and width of the femoral neck, and diameter of the head circumference, using the MicroDicom DICOM 4.0.0 software. The mean neck-diaphyseal angle was 130.8 ± 4.5 degrees, in 57 individuals (45.2%) it was between 127.4 and 133.3 degrees (p = 0.001); the mean head circumference was 42.0 ± 2.4 mm, in 60 people (47.6%) it was between 40.2 and 45.7 mm (p = 0.001); the mean cervical length was 78.6 ± 16.4 mm, in 54 individuals (42.9 %) it was between 69.4 and 92 mm (p = 0.001); the mean cervical width was 75.9 ± 12.1 mm, in 64 cases (50.8%) it was between 62.9 and 78.7 mm (p= 0.001). In 60 individuals (47.6%), the lateralization of the femur was between 92.6 - 117.7 mm. The measurements are independent of sex; as age increases, the cervical shaft angle is less (p= 0.021). Percentage tables of the radiological measurements of the proximal end of the femur were made, according to age and sex, which can serve as a reference in future patients(AU)

Differential activities in adhesion and neurite growth of fibronectin type III repeats in the PTP-delta extracellular domain.

The full-length extracellular domain (ECD) of protein tyrosine phosphatase delta (PTP-delta) functions as a ligand to promote cell adhesion and neurite outgrowth; this ECD contains three immunoglobulin (Ig) repeats and eight fibronectin type III (FN III) repeats. However, it is not known which regions of the ECD regulate its ligand functions. Therefore, we constructed and expressed a fusion protein of the PTP-delta ECD lacking FN III repeats 4-8, and tested this protein for neuronal adhesion and neurite-promoting ability. Compared to the full-length isoform, the truncated ECD was poorer at promoting adhesion, but a more potent promoter of neurite growth. The results suggest that distal FN III repeats of PTP-delta are important in adhesive functions, but dispensable for neurite outgrowth promotion. As the predominant isoform of PTP-delta during neural development (type D) also lacks distal FN III repeats, the functional properties we observe may be relevant to periods of axon extension, suggesting that splice variants of receptor PTPs play distinct roles in neural development.

[Hodgkin's Lymphoma and Autoimmunity: Is There a Relationship?]. / Linfoma de Hodgkin e Autoimunidade: Existirá uma Relação?

INTRODUCTION: The relationship between lymphomas and autoimmune diseases has been reported as bi-directional, however there is a few data in pediatric population. The aim of this work is to evaluate the prevalence of autoimmune diseases in children and adolescents with Hodgkin's lymphoma followed in a Pediatric Oncology Unit. MATERIAL AND METHODS: By reviewing Hodgkin's lymphomas data from the past 16 years (collected prospectively), an apparently large incidence of autoimmune diseases, mostly in female patients, was noted. We decided to do this retrospective study with an update on follow-up. Data analyzed: age, gender, autoimmune disease, temporal relation with lymphoma, lymphoma's stage, histologic subtype and treatment. RESULTS: Fifty-two cases were included, 7 (13.5%) of which, all female, had an autoimmune disease diagnosed previously, simultaneously or after lymphoma. Autoimmune diseases were: juvenile idiopathic arthritis, inflammatory bowel disease, Behçet's disease, autoimmune hepatitis, systemic erythematosus lupus, Hashimoto's thyroiditis and idiopathic thrombocytopenic purpura. The diagnosis was made after lymphoma in 4 patients, before in 2 and simultaneously in 1 patient. All cases, except the one with simultaneous diagnosis, are out of treatment and without oncologic disease relapse. No deaths were registered. DISCUSSION: There was an important prevalence of autoimmune diseases in girls with Hodgkin's lymphoma. We present data and discuss the possible causes based on a literature review. CONCLUSIONS: This relationship should be invoked, requiring more studies, especially in pediatric age.

Introdução: A relação entre linfomas e doenças autoimunes é descrita na literatura como bidirecional, existindo poucos dados em idade pediátrica. Este trabalho tem como objetivo avaliar a prevalência de doenças autoimunes em crianças e adolescentes com linfoma de Hodgkin seguidos num Serviço de Oncologia Pediátrica. Material e Métodos: Ao rever a casuística do Serviço de linfomas de Hodgkin nos últimos 16 anos (dados colhidos prospetivamente), constatou-se uma incidência aparentemente elevada de doenças autoimunes nas raparigas pelo que se realizou um estudo retrospetivo, com atualização do seguimento fora de tratamento, relativamente à existência de doenças autoimunes. Avaliaram-se: idade, sexo, tipo de doença autoimune, relação temporal com o linfoma, estádio e grupo histológico do linfoma e terapêutica efetuada. Resultados: Incluíram-se 52 casos de linfoma de Hodgkin, dos quais sete (13,5%), todos do sexo feminino, tiveram uma doença autoimune diagnosticada previamente, em simult'neo ou posteriormente ao linfoma. As doenças autoimunes encontradas foram: artrite idiopática juvenil, doença inflamatória intestinal, doença de Behçet, hepatite autoimune, lúpus eritematoso sistémico, tiroidite de Hashimoto e púrpura trombocitopénica idiopática. Em quatro doentes o diagnóstico foi posterior ao linfoma, em dois, prévio, e num simult'neo. Todos os casos, exceto o diagnóstico simult'neo, estão fora de tratamento e sem recidiva da doença oncológica. Não se verificaram óbitos. Discussão: Verificou-se uma importante prevalência de doenças autoimunes nas raparigas com linfoma de Hodgkin. Apresentamos os dados e discutimos possíveis causas desta relação com base numa revisão bibliográfica. Conclusões: Esta associação deve ser evocada, sendo necessário mais estudos, sobretudo em idade pediátrica.