Multisystemic Inflammatory Syndrome and Thrombotic Microangiopathy as Complications of COVID-19 in a Child: A Case Report.

Clinical presentations of the novel coronavirus (SARS-CoV-2) infection are quite varied, ranging from asymptomatic conditions to potentially fatal disease. The kidney is one of the affected targets of coronavirus disease (COVID-19) complications, and renal dysfunction is a significant prognostic factor for mortality. This report describes a series of clinical complications in a previously healthy child who developed nephritic syndrome with a concomitant SARS-CoV-2 infection. These complications include acute kidney injury that progressed to chronicity, multisystemic inflammatory syndrome, Kawasaki-like syndrome, and thrombotic microangiopathy.

Portal vein embolisation in a patient with situs inversus.

Portal vein embolisation (PVE) is a well-established technique used for patients who require major hepatic resections without sufficient volume of future remnant liver (FRL). We describe a case of PVE in a patient with situs inversus. Computed Tomography (CT) 4 weeks after the procedure demonstrated significant hypertrophy of the FRL. However, the surgical procedure was aborted due to signs of extrahepatic progression.

Portal vein embolization with n-butyl-cyanoacrylate through an ipsilateral approach before major hepatectomy: single center analysis of 50 consecutive patients.

PURPOSE: To evaluate the efficacy of portal vein embolization (PVE) with n-Butyl-cyanoacrylate (NBCA) through an ipsilateral approach before major hepatectomy. Secondary end-points were PVE safety, liver resection and patient outcome. METHODS: Over a 5-year period 50 non-cirrhotic consecutive patients were included with primary or secondary liver cancer treatable by hepatectomy with a liver remnant (FLR) volume less than 25% or less than 40% in diseased livers. RESULTS: There were 37 men and 13 women with a mean age of 57 years. Colorectal liver metastases were the most frequent tumor and patients were previously exposed to chemotherapy. FLR increased from 422 ml to 629 ml (P < 0.001) after PVE, corresponding to anincrease of 52%. The FLR ratio increased from 29.6% to 42.3% (P < 0.001). Kinetic growth rate was 2.98%/week. A negative association was observed between increase in the FLR and FLR ratio and FLR volume before PVE (P = 0.002). In 31 patients hepatectomy was accomplished and only one patient presented with liver insufficiency within 30 days after surgery. CONCLUSIONS: PVE with NBCA through an ipsilateral puncture is effective before major hepatectomy. Meticulous attention is needed especially near the end of the embolization procedure to avoid complications. TRIAL REGISTRATION: Clinical Study ISRCTN registration number: ISRCTN39855523 . Registered March 13th 2017.

Imaging of tuberous sclerosis complex: a pictorial review.

Tuberous sclerosis complex (TSC) is a genetically determined hamartomatous neurocutaneous disease with high phenotypic variability. TSC is characterized by widespread hamartomas and benign, or rarely malignant, neoplasms distributed in several organs throughout the body, especially in the brain, skin, retina, kidney, heart, and lung. Common manifestations include cortical tubers, subependymal nodules, white matter abnormalities, retinal abnormalities, cardiac rhabdomyoma, lymphangioleiomyomatosis, renal angiomyolipoma, and skin lesions. The wide range of organs affected by the disease implies that TSC1 and TSC2 genes play important roles in the regulation of cell proliferation and differentiation. Although recent advances in treatment have improved morbidity, the prognosis remains quite poor and nearly 40% of patients die by the age of 35 years. Imaging is important in the evaluation of TSC because of its role not only in presumptive diagnosis, but also in defining the full extent of involvement. This information allows a better understanding of the behavioural phenotype, as related to lesion location. Imaging also contributes to treatment planning. This pictorial review describes common and uncommon imaging manifestations of TSC.

Complexo esclerose tuberosa (CET) é uma síndrome hamartomatosa neurocutânea geneticamente determinada, com elevada variabilidade fenotípica. O CET é caracterizado por hamartomas generalizados e pela presença de neoplasias benignas, ou raramente malignas, acometendo vários órgãos, especialmente cérebro, pele, retina, rins, coração e pulmões. As manifestações mais comuns incluem tubérculos corticais, nódulos subependimais, anormalidades na substância branca, anormalidades da retina, rabdomioma cardíaco, linfangioleiomiomatose, angiomiolipoma renal e lesões de pele. A ampla gama de órgãos afetados sugere que genes TSC1 e TSC2 desempenham papel importante na regulação da proliferação e diferenciação celular. Apesar de os recentes avanços no tratamento terem melhorado a morbidade, o prognóstico permanece bastante ruim e cerca de 40% dos pacientes morrem próximo aos 35 anos de idade. Estudos por imagem são importantes na avaliação do CET, em razão do seu papel não só no diagnóstico presuntivo, mas também na definição da extensão total da doença. Estas informações permitem uma melhor compreensão do fenótipo comportamental, relacionado com a localização das lesões. A imagem auxilia, também, no planejamento do tratamento.. Este ensaio pictórico descreve as manifestações de imagem comuns e incomuns do CET.

Imaging of tuberous sclerosis complex: a pictorial review / Aspectos de imagem do complexo esclerose tuberosa: ensaio iconográfico

Abstract Tuberous sclerosis complex (TSC) is a genetically determined hamartomatous neurocutaneous disease with high phenotypic variability. TSC is characterized by widespread hamartomas and benign, or rarely malignant, neoplasms distributed in several organs throughout the body, especially in the brain, skin, retina, kidney, heart, and lung. Common manifestations include cortical tubers, subependymal nodules, white matter abnormalities, retinal abnormalities, cardiac rhabdomyoma, lymphangioleiomyomatosis, renal angiomyolipoma, and skin lesions. The wide range of organs affected by the disease implies that TSC1 and TSC2 genes play important roles in the regulation of cell proliferation and differentiation. Although recent advances in treatment have improved morbidity, the prognosis remains quite poor and nearly 40% of patients die by the age of 35 years. Imaging is important in the evaluation of TSC because of its role not only in presumptive diagnosis, but also in defining the full extent of involvement. This information allows a better understanding of the behavioural phenotype, as related to lesion location. Imaging also contributes to treatment planning. This pictorial review describes common and uncommon imaging manifestations of TSC.

Resumo Complexo esclerose tuberosa (CET) é uma síndrome hamartomatosa neurocutânea geneticamente determinada, com elevada variabilidade fenotípica. O CET é caracterizado por hamartomas generalizados e pela presença de neoplasias benignas, ou raramente malignas, acometendo vários órgãos, especialmente cérebro, pele, retina, rins, coração e pulmões. As manifestações mais comuns incluem tubérculos corticais, nódulos subependimais, anormalidades na substância branca, anormalidades da retina, rabdomioma cardíaco, linfangioleiomiomatose, angiomiolipoma renal e lesões de pele. A ampla gama de órgãos afetados sugere que genes TSC1 e TSC2 desempenham papel importante na regulação da proliferação e diferenciação celular. Apesar de os recentes avanços no tratamento terem melhorado a morbidade, o prognóstico permanece bastante ruim e cerca de 40% dos pacientes morrem próximo aos 35 anos de idade. Estudos por imagem são importantes na avaliação do CET, em razão do seu papel não só no diagnóstico presuntivo, mas também na definição da extensão total da doença. Estas informações permitem uma melhor compreensão do fenótipo comportamental, relacionado com a localização das lesões. A imagem auxilia, também, no planejamento do tratamento.. Este ensaio pictórico descreve as manifestações de imagem comuns e incomuns do CET.

Information of patients with life-threatening diseases: A survey of the attitude of Portuguese family practitioners.

HIGHLIGHTS: The attitudes of family physicians regarding breaking bad news are heterogeneous.Younger doctors seem to see the delivery of bad news more positively.This trend suggests that there will be a more open communication in the future. BACKGROUND: Family practice is the specialty with the highest number of doctors and covers all of Portugal, but, as far as we know, no studies have been carried out on the attitudes and practices of Portuguese family practice doctors about breaking bad news. However, the attitude of these doctors may have a high impact on patients. OBJECTIVE: To study the practice of family physicians on breaking bad news. METHODS: A questionnaire, specifically developed for this survey, was given to 196 doctors about 10% of the family physicians of Northern Portugal. RESULTS: One hundred fifty-nine (81%) of them participated in this study. The median age was 43 (26-64) and 108 (68%) of them were female. One hundred and seven (67%) doctors disclosed on principle the diagnosis and that rate rose to 81% when patients requested the disclosure. One hundred and two (64%) proactively questioned patients about their wish to know the diagnosis and then decided whether to convey it or not. Forty-seven 47 (30%) doctors disclosed the prognosis on principle and that rate rose to 48% when patients requested the disclosure. Seventy-three (46%) often questioned patients proactively about their wish to know the prognosis and then decided whether to convey it or not. One hundred and two (64%) doctors frequently include patients in treatment decisions. Physicians think that the disclosure may affect hope but may also give patients more control of the situation. CONCLUSION: Family practitioners disclose the diagnosis of a chronic life-threatening disease often, especially at patients' request. General practitioners do not disclose the prognosis of a life-threatening disease often, even at patients' request.

Family physicians' opinions on and difficulties with breaking bad news.

HIGHLIGHTS: Breaking bad news is still deemed a difficult task by family physicians.Family physicians feel they need training in breaking bad news.The family physicians' attitude to this issue is different from what they would wish if they themselves had a life-threatening disease. BACKGROUND: Family practice is the specialty with the highest number of doctors and covers all of Portugal. Therefore, the attitude of these doctors may have a high impact on patients. OBJECTIVE: To explore the opinion and difficulties of Portuguese family doctors on dealing with communication with patients with life threatening diseases. METHODS: A questionnaire was sent to about 10% of family doctors of Northern Portugal. The questionnaire included questions about the disclosure of information, if they feel they need training courses and what they would want if they had a life-threatening disease. RESULTS: A questionnaire was given to 196 doctors and 159 (81%) participated in this study. The median age was 43 years (26-64) and 108 (68%) were females. One hundred thirty-five (85%) consider that breaking bad news is a difficult task. One hundred twenty-four (78%) feel they need training in breaking bad news. For many doctors, the disclosure of diagnoses and prognoses has a detrimental psychological effect and affects patients' hope, but gives patients' control of the situation. Given a situation where the doctors themselves had a life-threatening disease, the vast majority would want to know the diagnosis and the prognosis and to participate in treatment decisions. CONCLUSIONS: Breaking bad news is still a difficult task. Their attitude to this duty is different from what they would wish if they themselves had a life-threatening disease. One important conclusion is the need of specific training in communication for family physicians that should begin in the training phase of their specialty.

Partial splenic embolization to permit continuation of systemic chemotherapy.

Systemic chemotherapy treatments, commonly those that comprise oxaliplatin, have been linked to the appearance of distinctive liver lesions that evolves to portal hypertension, spleen enlargement, platelets sequestration, and thrombocytopenia. This outcome can interrupt treatment or force dosage reduction, decreasing efficiency of cancer therapy. We conducted a prospective phase II study for the evaluation of partial splenic embolization in patients with thrombocytopenia that impeded systemic chemotherapy continuation. From August 2014 through July 2015, 33 patients underwent partial splenic embolization to increase platelets count and allow their return to treatment. Primary endpoint was the accomplishment of a thrombocyte level superior to 130 × 109 /L and the secondary endpoints were the return to chemotherapy and toxicity. Partial splenic embolization was done 36 times in 33 patients. All patients presented gastrointestinal cancer and colorectal malignancy was the commonest primary site. An average of 6.4 cycles of chemotherapy was done before splenic embolization and the most common regimen was Folfox. Mean platelet count prior to embolization was 69 × 109 /L. A total of 94% of patients achieved primary endpoint. All patients in need reinitiated treatment and median time to chemotherapy return was 14 days. No grade 3 or above adverse events were identified. Aiming for a 50% to 70% infarction area may be sufficient to achieve success without the complications associated with more extensive infarction. Combined with the better safety profile, partial splenic embolization is an excellent option in the management of thrombocytopenia, enabling the resumption of systemic chemotherapy with minimal procedure-related morbidity.

Organizing pneumonia: chest HRCT findings.

OBJECTIVE: To determine the frequency of HRCT findings and their distribution in the lung parenchyma of patients with organizing pneumonia. METHODS: This was a retrospective review of the HRCT scans of 36 adult patients (26 females and 10 males) with biopsy-proven organizing pneumonia. The patients were between 19 and 82 years of age (mean age, 56.2 years). The HRCT images were evaluated by two independent observers, discordant interpretations being resolved by consensus. RESULTS: The most common HRCT finding was that of ground-glass opacities, which were seen in 88.9% of the cases. The second most common finding was consolidation (in 83.3% of cases), followed by peribronchovascular opacities (in 52.8%), reticulation (in 38.9%), bronchiectasis (in 33.3%), interstitial nodules (in 27.8%), interlobular septal thickening (in 27.8%), perilobular pattern (in 22.2%), the reversed halo sign (in 16.7%), airspace nodules (in 11.1%), and the halo sign (in 8.3%). The lesions were predominantly bilateral, the middle and lower lung fields being the areas most commonly affected. CONCLUSIONS: Ground-glass opacities and consolidation were the most common findings, with a predominantly random distribution, although they were more common in the middle and lower thirds of the lungs.

Organizing pneumonia: chest HRCT findings / Pneumonia em organização: achados da TCAR de tórax

OBJECTIVE: To determine the frequency of HRCT findings and their distribution in the lung parenchyma of patients with organizing pneumonia. METHODS: This was a retrospective review of the HRCT scans of 36 adult patients (26 females and 10 males) with biopsy-proven organizing pneumonia. The patients were between 19 and 82 years of age (mean age, 56.2 years). The HRCT images were evaluated by two independent observers, discordant interpretations being resolved by consensus. RESULTS: The most common HRCT finding was that of ground-glass opacities, which were seen in 88.9% of the cases. The second most common finding was consolidation (in 83.3% of cases), followed by peribronchovascular opacities (in 52.8%), reticulation (in 38.9%), bronchiectasis (in 33.3%), interstitial nodules (in 27.8%), interlobular septal thickening (in 27.8%), perilobular pattern (in 22.2%), the reversed halo sign (in 16.7%), airspace nodules (in 11.1%), and the halo sign (in 8.3%). The lesions were predominantly bilateral, the middle and lower lung fields being the areas most commonly affected. CONCLUSIONS: Ground-glass opacities and consolidation were the most common findings, with a predominantly random distribution, although they were more common in the middle and lower thirds of the lungs. .

OBJETIVO: Determinar a frequência dos achados tomográficos e sua distribuição no parênquima pulmonar de pacientes com pneumonia em organização. MÉTODOS: Estudo retrospectivo das imagens de TCAR de 36 pacientes adultos (26 mulheres e 10 homens) com pneumonia em organização confirmada por exame histopatológico. A faixa etária dos pacientes incluídos foi de 19 a 82 anos (média: 56,2 anos). As imagens foram avaliadas por dois observadores, de forma independente, e os casos discordantes foram resolvidos por consenso. RESULTADOS: O achado tomográfico mais comum foi o de opacidades em vidro fosco, presentes em 88,9% dos casos. O segundo achado mais comum foi o de consolidação (em 83,3% dos casos), seguido de opacidades peribroncovasculares (em 52,8%), reticulação (em 38,9%), bronquiectasias (em 33,3%), nódulos intersticiais (em 27,8%), espessamento de septos interlobulares (em 27,8%), padrão perilobular (em 22,2%), sinal do halo invertido (em 16,7%), nódulos do espaço aéreo (em 11,1%) e sinal do halo (em 8,3%). As lesões foram predominantemente bilaterais, e os terços médios e inferiores dos pulmões foram as regiões mais afetadas. CONCLUSÕES: Os achados mais frequentes foram opacidades em vidro fosco e consolidações, com distribuição predominantemente aleatória, embora tenham sido mais comuns nos terços médios e inferiores dos pulmões. .