Rui Almeida

Opinion of hepatic transplant waiting list patients regarding split liver transplantation

BACKGROUND Split liver transplantation (SLT) increases organ supply for hepatic transplantation. Long-term patient survival and complication rates seem to be equivalent between orthotopic liver transplantation (OLT) and SLT. There are controversies among transplant physicians due to an ethical dilemma between benefiting individual needs or those of society. Barshes and Goss (Am J Transplant 5:2047, 2005) demonstrated that the majority of adult liver transplant candidates are favorable to SLT. The aim of our study was to evaluate the opinions of patients at a Brazilian university hospital regarding SLT. MATERIALS AND METHODS A questionnaire with 14 questions was applied to 50 patients included in a hepatic transplant waiting list regarding SLT. RESULTS The overall attitudes of 66% of the participants were classified as utilitarian, 31% were classified as self-preserving, and 3% were undecided. Ninety-one percent of patients would be willing to share even if their expected survival after SLT was shorter than that with OLT. For 77% of patients, children must have priority over adults. However, 83% were unaware of the donors for pediatric transplantations. CONCLUSIONS SLT is a consistent solution for organ demand despite controversies among transplant physicians. The present study demonstrated that most patients were favorable to SLT. In conclusion, attitudes toward graft sharing are not barriers to SLT.

Sporadic Creutzfeldt-Jakob disease causing a 2-years slowly progressive isolated dementia.

A 47-year-old woman was seen for progressive behavioural and cognitive disturbances slowly evolving over a 1-year period. Neuropsychological evaluation disclosed moderate to severe impairment of all cortical functions. Besides this no other clinical abnormality was found. MRI diffusion weighted imaging disclosed hyperintense cortical lesions in a ribbon-like fashion, with restricted diffusivity. EEG showed no periodic sharp waves and CSF examination was normal, including protein 14.3.3. She was heterozygote on codon 129. Her cognitive function continued to decline and she was readmitted for further investigation at the 24th month of disease. Again no ataxia or involuntary movements were observed. MRI disclosed widespread hyperintense lesions over the entire cortex and, for the first time, also caudato-putaminal hyperintensity in T2-weighted images. EEG again failed to show periodic activity. Stereotactic biopsy disclosed moderate spongiform changes, astrocytosis and perivacuolar staining with prion-directed antibodies. Western blot analysis revealed prion type 2 mobility pattern. We discuss the clinical significance of this case: as dementia was the sole finding, and this was slowly-evolving over a 2-year period, MRI findings were the key factor suggesting a prion disease in a woman that otherwise would probably be diagnosed with a primary degenerative dementia.

Gluten-free diet: a possible treatment for chronic diarrhoea in common variable immunodeficiency

Gastrointestinal disorders are frequent in common variable immunodeficiency (CVID). Clinical symptoms and histological alterations in CIVD can resemble celiac disease. Usually, patients with chronic diarrhoea associated with CVID do not improve with a gluten-free diet. The authors present a case of a male patient who was diagnosed with CVID at age 33 and had chronic diarrhoea which resolved after initiating a gluten-free diet. Clinical relapse occurred after gluten reintroduction. The main objective of this case report is to alert clinicians to implement a gluten-free diet in patients with CVID with chronic diarrhoea.

Mixed extragonadal germ cell tumour of the prostate

Extragonadal germ cell tumours (EGGCTs) originated in prostate are extremely rare, with <20 cases described in the literature. We report a case of a patient with a primary prostatic mixed EGGCT. A 47-year-old man presenting severe low urinary tract symptoms and signs of prostatic enlargement, with no malignancy suspicion, underwent transurethral resection of the prostate. The histopathological evaluation suggested the diagnosis of a retroperitoneal sarcoma. The patient underwent neoadjuvant chemotherapy and then was submitted to radical cystoprostatectomy. Histology revealed a mixed EGGCT of the prostate with yolk sac tumour and seminoma components. No testicular abnormalities were identified on the postoperative scrotal ultrasound. The patient went through four cycles of chemotherapy with bleomycin, etoposide and cisplatin. After 12 months of follow-up, the patient is alive and free of recurrence.

Giant pituitary incidentaloma

A 78-year-old woman, independent, with medical history of hypertension, dyslipidaemia and bilateral cataract surgery. The patient underwent a head CT after a traumatic brain injury where a giant pituitary incidentaloma of 60 mm in the largest diameter was found. The patient mentioned decreased left eye acuity for the past 20 years, with no complaints of headaches or diplopia. The physical examination was unremarkable with no stigmata of endocrine disease. Anterior pituitary hormonal profile was normal. Additional MRI showed an extensive infiltrative lesion, 60×40 mm, with cavernous and sphenoidal sinus invasion, suprasellar extension and left optic nerve encapsulation (figures 1 and 2). Visual …

Penetrating head trauma injury with an excellent outcome

A 55-year-old man was found at his workplace with a metal drill penetrating the midline of the posterior region of the sagittal suture. His Glasgow Coma Scale (GCS) was initially 9, but his condition deteriorated and required endotracheal intubation. CT of the brain showed that the drill had taken an intracranial course, from the transition of the middle to the posterior third of the superior sagittal sinus, crossing the right parietal and temporal lobes, and ending in contact with the tentorial surface of the cerebellum (figures 1 and 2). He underwent emergency surgery to remove the drill. The operation revealed …

Gliosarcoma with neuroaxis metastases

Gliosarcomas are rare tumours of the central nervous system, with a well-known capacity for metastasis. When they metastasise, the dissemination occurs more frequently via the haematogenous route to extraneural sites. Metastasis-spread through the cerebrospinal fluid is extremely rare. We present the case of a 58-year-old man who underwent a gross total resection of a lesion in the left temporal lobe. The histological findings revealed a gliosarcoma and the patient received radiotherapy followed by chemotherapy. Seven months after surgery, while the patient remained neurologically intact, brain and spinal cord MRI revealed tumour recurrence and neuroaxis metastases through the traffic routes of the cerebrospinal fluid. The patient died 8 months after the diagnosis. A PubMed search regarding metastatic gliosarcoma up to June 2015 was also carried out. To the best of our knowledge, this is the first case report of gliosarcoma metastases to the brain and spinal cord leptomeninges.

Pediatrics cushing disease: a diagnostic challenge

Total cholesterol 196, HDL-C 69, TG 56 mg/dl FT3 4.99pg/ml, FT4 1.7ng/dl, TSH 2.9uUI/ml GH 1.57ng/ml, IGF1 300ng/ml, IGFBP3 6mg/L PRL 530 (<360uUI/ml) FSH 0.33mIU/ml, LH <0,1mIU/ml Total testost 37,26ng/dl (Tanner 2) Estradiol: 1.55ng/dl (Tanner 2) Androstenedione: 1.2ng/ml (Tanner 2) DHEAS: 244ng/dl (Tanner 2) Insulin 22uUI/ml, C Pep 1.94ng/ml Pituitary MRI: “Homogenous pituitary after endovenous gadolinium administration, without apparent signs of pituitary adenoma” 2nd pituitary MRI (15Y9M)