Massive Pulmonary Arteriovenous Malformation as a Cause of Fetal Heart Failure.

Pulmonary arteriovenous malformations (AVMs) are abnormal connections between the pulmonary arteries and veins that can result in rapid-onset heart failure. We present a case of a fetus with pulmonary AVMs diagnosed at 22 weeks gestation. Fetal echocardiography showed cardiomegaly and dilated pulmonary arteries and veins reflecting the hemodynamic significance of the shunt. Inverted flow through the ductus arteriosus was also present. Fetal autopsy following medical termination of the pregnancy confirmed the morphological findings, including displacement of arteries and veins in proximity to the pleural surface. The genetic study was negative. This report highlights the cardiovascular impact of a rare disorder. Inverted flow through the ductus arteriosus may be another poor prognostic indicator, useful in parental counseling.

Interim FDG18-PET SUVmax Variation Adds Prognostic Value to Deauville 5-Point Scale in the Identification of Patients with Ultra-High-Risk Diffuse Large B Cell Lymphoma.

INTRODUCTION: Interim response evaluation by 18F-fluorodeoxyglucose positron emission tomography/computed tomography (iPET) in diffuse large B cell lymphoma (DLBCL) could be important to rule out disease progression and has been suggested to be predictive of survival. However, treatment guidance by iPET is not yet recommended for DLBCL in clinical practice. We aimed to compare the predictive value of iPET when utilizing the visual Deauville 5-point scale (DS) and the semiquantitative variation of maximum standardized uptake value (ΔSUVmax). MATERIALS AND METHODS: We included 85 patients diagnosed with DLBCL and uniformly treated with standard protocols. iPET with DS of 1-3 and/or ΔSUVmax ≥66% was defined as negative. Univariable and multivariable Cox regression analyses were performed to determine the independent factors affecting progression free survival (PFS) or overall survival (OS) and to estimate PFS and OS. RESULTS: iPET positivity, measured by DS or ΔSUVmax, showed predictive value of disease refractoriness, improved by combining DS and ΔSUVmax. After a median follow-up of 50.1 months, iPET was an independent predictor for both PFS and OS when interpreted by DS, but only for PFS by ΔSUVmax. Combined visual and semiquantitative analysis (D4-5 & ΔSUVmax<66%) was an independent predictor of PFS and OS, and allowed to identify an ultra-high-risk subgroup of patients with very dismal outcome, increasing the discriminating capacity for iPET. CONCLUSION: Our study suggests that combined DS and ΔSUVmax in iPET assessment predicts refractory disease and distinguishes ultra-high-risk DLBCL patients with a very dismal prognosis, who may benefit from PET-guided therapy adjustment.

A rare case of pulmonary lymphangiectasia associated with CHD.

CHD may, at times, occur in the framework of other rare pathologies. These, having similar clinical manifestations, present a diagnostic dilemma for the clinician.The authors present the case of an infant with non-syndromic complete atrioventricular septal defect, whose post-operative period was surprisingly complicated by progressive pulmonary hypertension. Despite intensive care, the infant ultimately died. The diagnosis of unilateral primary pulmonary lymphangiectasia was only possible post mortem.

A Case of Liver Injury after Exposure to Isopropanol: A Challenging Diagnosis.

Drug-induced liver injury is hardly diagnosed, considering not only the wide range of hepatotoxic substances but also the diversity of associated phenotypes and the absence of specific biomarkers. Symptom chronology, drug or toxic exposure, and temporal association help to establish the diagnosis. Exposure to isopropanol has known but rare toxic effects. We report the clinical case of a 33-year-old female hairdresser admitted to the hospital with fatigue, epigastric pain, and jaundice. She presented the following values: aspartate aminotransferase, 485 U/L; alanine transaminase, 908 U/L; ALP, 240 U/L; GGT, 370 U/L; total bilirubin, 3.5 mg/dL; and direct bilirubin, 2.1 mg/dL. Albumin, platelet, and INR values were normal. Structural, infectious, immune, and vascular causes were excluded. Liver biopsy was suggestive of toxic hepatitis. A possible association with ibuprofen intake was considered. The patient resumed professional activity, with fatigue and jaundice relapse, as well as a new liver enzyme increase, despite ibuprofen withdrawal. It was concluded that a new hair product containing isopropanol had recently been introduced. As soon as its professional use was discontinued, there was no recurrence of the symptoms. Given the temporal association between the development of acute hepatitis and the use of an isopropanol-containing product, liver toxicity by exposure to isopropanol was assumed. This substance is metabolized in the liver and toxicity may occur by ingestion, skin exposure, or inhalation, and it is described in cases of occupational or accidental exposure. The treatment is symptomatic and comprises toxic suspension.

A lesão hepática induzida por tóxicos é um dos distúrbios de mais difícil diagnóstico, quer pela existência de múltiplos hepatotóxicos, quer pela diversidade de fenótipos associados e ausência de biomarcadores específicos. A cronologia dos sintomas, exposição a fármacos ou tóxicos e associação temporal auxilia o diagnóstico. A exposição a isopropanol tem efeitos tóxicos conhecidos e raros. Descrevemos o caso clínico de uma mulher de 33 anos, cabeleireira, admitida por fadiga, epigastralgias e icterícia. Apresentava AST 485 U/L, ALT 908 U/L, FA 240 U/L, GGT 370 U/L, bilirrubina total 3,5 mg/dL e bilirrubina direta 2,1 mg/dL; o doseamento sérico de albumina, plaquetas e o valor de INR eram normais. Foram excluídas lesões estruturais, causas infeciosas, imunes e vasculares. Biópsia hepática sugestiva de hepatite tóxica. Considerada possível associação a toma de ibuprofeno. Retomou atividade profissional, com recorrência de fadiga e icterícia, bem como elevação das enzimas hepáticas novamente, apesar de suspensão de ibuprofeno. Apurada exposição a tóxicos profissionais, percebendo-se que o quadro coincidiu com início da utilização de novo produto capilar contendo isopropanol. A doente retomou atividade profissional, não utilizando o produto, sem recorrência. Dada a relação temporal entre o desenvolvimento de hepatite aguda e a utilização de produtos contendo isopropanol, assumiu-se hepatotoxicidade por exposição profissional ao isopropanol. Esta substância é metabolizada no fígado e a toxicidade pode ocorrer por ingestão, exposição cutânea ou inalação, estando descrita em casos de exposição ocupacional ou acidental. O tratamento é sintomático e inclui a suspensão do tóxico.

Dichorionic pregnancy complicated by a twin-to-twin transfusion syndrome.

Twin-to-twin transfusion syndrome (TTTS) is a serious complication typical of monochorionic pregnancies. Cases in dichorionic pregnancies have been described. The authors present a case report of a presumed TTTS in a dichorionic pregnancy followed at our institution in which the patient presented to the emergency department at 31 4/7 weeks' gestation complaining of lack of fetal movements. Both fetuses did not show any cardiac activity. On the first twin, an oligohydramnios was present and his bladder was not visualised and, on the second twin, a polyhydramnios and an augmented bladder were visualised. Pathological findings of the fetuses were compatible with a diagnosis of TTTS and placental study confirmed the presence of a dichorionic/diamniotic placenta, with superficial vessels crossing the dividing membrane.

[Preventive and therapeutic effect of strontium ranelate on osteoporosis in rats subjected to simulated weightlessness].

OBJECTIVE: To study the effects of strontium ranelate on osteoporosis induced by simulated weightlessness in male rats. METHODS: Twenty-seven male SD rats were randomly divided into 3 groups, namely the normal control group (group A) and two groups of weightlessness simulated by tail suspension (groups B and C). The rats in group C were treated with strontium ranelate, and those in the other two groups were given the same dose of normal saline for 28 consecutive days. RESULTS: The rats in group B showed significantly lower levels of alkaline phosphatase (ALP), bone mineral density (BMD) and bone mineral content (BMC) than those in group A (P<0.05), but serum calcium and phosphonium concentrations underwent no significant changes in the 3 groups (P>0.05). In the rats of group B, the trabeculae of the femur became thinner, fragile, and discontinuous with reduced quantity as compared with those in group A. The rats in group C had greater number of the trabeculae than those in group B with decreased resorption surface and more regular arrangement of the collagen fibers. CONCLUSION: Strontium ranelate may produce beneficial effect on the bone microstructure, reduce bone loss and stimulate bone formation in male rats subjected to simulated weightlessness.