Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Rare Etiology of Fever in Adults.

We report the case of a 28-year-old male, with a past history of recurrent pharyngitis and tonsillectomy, who presented to the emergency department with fever, pharyngitis, and cervical adenitis. Inflammatory markers were elevated and the patient was initially started on ceftriaxone with remission after four days. However, the symptoms recurred three weeks later and an autoinflammatory disease was suspected. After exclusion of other illnesses, a diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome was confirmed. The patient was successfully treated with a single dose of 60 mg of prednisolone at the beginning of the flare. PFAPA syndrome has been classically diagnosed solely in children but cases in adults are being increasingly recognized. Despite the increasing evidence of the delayed onset of PFAPA syndrome during adulthood, no specific tools are available to detect it and diagnosis is currently based on clinical diagnostic criteria, which have very low specificity and are tailored to pediatric patients. This case report stresses the need to consider this entity seriously despite its rarity, even among the adult population, so as to reduce iatrogenesis, start appropriate therapy in a prompt manner, and improve the quality of life of PFAPA patients.

Chylous ascites in disseminated Kaposi sarcoma: an unusual manifestation as immune reconstitution inflammatory syndrome.

A 29-year-old man with diarrhoea, fever, abdominal pain and multiple purple papular lesions, neither pruriginous nor painful, was diagnosed with HIV-1 infection and disseminated Kaposi sarcoma (KS) with gastrointestinal involvement. He was started on highly active antiretroviral therapy immediately, as well as doxorubicin. Three weeks later, the patient developed bilateral moderate pleural effusion and large-volume ascites compatible with chylothorax and chylous ascites. An immune reconstitution inflammatory syndrome (IRIS) reaction was assumed. KS flare was associated with lymphatic obstruction and infiltration of thoracic duct by the tumour itself with leakage of chylous into pleural and peritoneal cavities. KS is the most common tumour in HIV patients and the existence of related effusions is not uncommon. KS-related chylothorax is an unusual manifestation of KS; there are only four cases described in the literature of chylous ascites related to KS-HIV. Overall survival is improving in KS but explosive and debilitating IRIS reactions can explain cases with poor prognosis.

Palliative approach in acute neurological events: a five-year study

SUMMARY INTRODUCTION Acute neurological illness often results in severe disability. Five-year life expectancy is around 40%; half the survivors become completely dependent on outside help. OBJECTIVE Evaluate the symptoms of patients admitted to a Hospital ward with a diagnosis of stroke, subarachnoid hemorrhage or subdural hematoma, and analyze the role of an In-Hospital Palliative Care Support Team. MATERIAL AND METHODS Retrospective, observational study with a sample consisting of all patients admitted with acute neurological illness and with a guidance request made to the In-Hospital Palliative Care Support Team of a tertiary Hospital, over 5 years (2012-2016). RESULTS A total of 66 patients were evaluated, with an age median of 83 years old. Amongst them, there were 41 ischaemic strokes, 12 intracranial bleedings, 12 subdural hematomas, and 5 subarachnoid hemorrhages. The median of delay between admission and guidance request was 14 days. On the first evaluation by the team, the GCS score median was 6/15 and the Palliative Performance Scale (PPS) median 10%. Dysphagia (96.8%) and bronchorrhea (48.4%) were the most prevalent symptoms. A total of 56 patients had a feeding tube (84.8%), 33 had vital sign monitoring (50.0%), 24 were hypocoagulated (36.3%), 25 lacked opioid or anti-muscarinic therapy for symptom control (37,9%); 6 patients retained orotracheal intubation, which was removed. In-hospital mortality was 72.7% (n=48). DISCUSSION AND CONCLUSION Patients were severely debilitated, in many cases futile interventions persisted, yet several were under-medicated for symptom control. The delay between admission and collaboration request was high. Due to the high morbidity associated with acute neurological illness, palliative care should always be timely provided.

RESUMO INTRODUÇÃO Eventos neurológicos agudos resultam frequentemente em incapacidade grave que impede o doente de participar ativamente nas decisões do seu próprio tratamento. A sobrevida a cinco anos ronda os 40%; metade dos sobreviventes fica dependente de terceiros. Objetivo Avaliar a sintomatologia de doentes internados com acidente vascular cerebral (AVC), hemorragia subarcnoideia (HSA) ou subdural (HSD) e analisar a intervenção de uma Equipe Intra-Hospitalar de Suporte em Cuidados Paliativos (EIHSCP). MATERIAL E MÉTODOS Estudo retrospetivo observacional dos doentes com diagnóstico principal de evento neurológico agudo com pedido de colaboração à EIHSCP, num hospital terciário, durante cinco anos (2012-2016). RESULTADOS Avaliados 66 doentes, com média de idade de 83 anos. Destacam-se 41 AVC isquêmicos, 12 hemorrágicos, 12 HSD e 5 HSA. A média da demora entre internamento e pedido de colaboração à EIHSCP foi de 14 dias. Na primeira observação, a média na escala de coma de Glasgow foi de 6/15 e na Palliative Performance Scale (PPS) foi de 10%. Disfagia (96,8%) e broncorreia (48,4%) foram os sintomas mais frequentes. A maioria dos doentes (56/66) mantinha sonda nasogástrica (84,8%); 33 encontravam-se em monitorização cardiorrespiratória (50,0%); 24 estavam sob hipocoagulação (36,3%); 25 necessitavam de opioide e antimuscarínico que não estavam prescritos (37,9%); seis tinham tubo orotraqueal, que foi retirado. A mortalidade intra-hospitalar foi de 72,7% (n=48). DISCUSSÃO E CONCLUSÃO Destaca-se o estado debilitado dos doentes; em muitos casos, intervenções fúteis persistiam, mas várias foram submedicadas para o controle dos sintomas. Verificou-se um tempo de espera elevado até o pedido de colaboração. Pela elevada morbilidade associada a esses eventos, cuidados paliativos diferenciados deveriam ser oferecidos no tempo adequado.

Granular cell tumour of the neurohypophysis: an unusual cause of hypopituitarism.

Granular cell tumours (GCT) are rare, slow-growing, benign neoplasms that are usually located in the head and neck. They are more frequent in the female gender and typically have an asymptomatic clinical course, being diagnosed only at autopsy. Symptomatic GCT of the neurohypophysis are exceedingly rare, being less than 70 cases described so far. The authors report on a case of a 28-year-old male that presented to the Endocrinology clinic with clinical and biochemical evidence of hypogonadism. He also reported minor headaches without any major visual symptoms. Further laboratory tests confirmed hypopituitarism (hypogonadotrophic hypogonadism, central hypothyroidism and hypocortisolism) and central nervous system imaging revealed a pituitary macroadenoma. The patient underwent transcranial pituitary adenoma resection and the pathology report described a GCT of the neurohypophysis with low mitotic index. The reported case is noteworthy for the rarity of the clinicopathological entity. LEARNING POINTS: Symptomatic GCTs are rare CNS tumours whose cell of origin is not well defined that usually give rise to visual symptoms, headache and endocrine dysfunction.Imaging is quite unspecific and diagnosis is difficult to establish preoperatively.Surgical excision is challenging due to lesion's high vascularity and propensity to adhere to adjacent structures.The reported case is noteworthy for the rarity of the clinicopathological entity.

Palliative approach in acute neurological events: a five-year study.

INTRODUCTION: Acute neurological illness often results in severe disability. Five-year life expectancy is around 40%; half the survivors become completely dependent on outside help. OBJECTIVE: Evaluate the symptoms of patients admitted to a Hospital ward with a diagnosis of stroke, subarachnoid hemorrhage or subdural hematoma, and analyze the role of an In-Hospital Palliative Care Support Team. MATERIAL AND METHODS: Retrospective, observational study with a sample consisting of all patients admitted with acute neurological illness and with a guidance request made to the In-Hospital Palliative Care Support Team of a tertiary Hospital, over 5 years (2012-2016). RESULTS: A total of 66 patients were evaluated, with an age median of 83 years old. Amongst them, there were 41 ischaemic strokes, 12 intracranial bleedings, 12 subdural hematomas, and 5 subarachnoid hemorrhages. The median of delay between admission and guidance request was 14 days. On the first evaluation by the team, the GCS score median was 6/15 and the Palliative Performance Scale (PPS) median 10%. Dysphagia (96.8%) and bronchorrhea (48.4%) were the most prevalent symptoms. A total of 56 patients had a feeding tube (84.8%), 33 had vital sign monitoring (50.0%), 24 were hypocoagulated (36.3%), 25 lacked opioid or anti-muscarinic therapy for symptom control (37,9%); 6 patients retained orotracheal intubation, which was removed. In-hospital mortality was 72.7% (n=48). DISCUSSION AND CONCLUSION: Patients were severely debilitated, in many cases futile interventions persisted, yet several were under-medicated for symptom control. The delay between admission and collaboration request was high. Due to the high morbidity associated with acute neurological illness, palliative care should always be timely provided.

[Visceral Leishmaniasis in HIV-Infected Patients: The Challenge of Relapse and Treatment Failure]. / Leishmaniose Visceral em Doentes com Infeção VIH: O Desafio da Recaída e Falência Terapêutica.

INTRODUCTION: Visceral leishmaniasis is an endemic disseminated infection, considered to be the third most frequent opportunistic parasitic infection in Europe. It is especially prevalent in patients co-infected with human immunodeficiency virus, in whom it poses a great therapeutic challenge due to increased risk of relapse. The goal of this study is to characterize a population of co-infected patients, as well as the efficiency of the adopted treatment strategies. MATERIAL AND METHODS: Retrospective study with a sample composed of all patients with visceral leishmaniasis and human immunodeficiency virus admitted in an Infectious Diseases ward over a period of 10 years. RESULTS: Of the 23 enrolled patients, two were female (8.7%). The mean TCD4+ cell count was 104.4 cells/uL (± 120.3cells/uL), only two patients had undetectable viral load (< 20 copies/mL) and 16 (69.6%) were not under antiretroviral therapy at the time of diagnosis. Treatment-wise, liposomal amphotericin B was used in 18 patients, meglumine antimoniate in four and miltefosine in one. Fourteen (60.9%) were adherent to secondary prophylaxis protocol. A relapse rate of 26.1% was observed (six patients). DISCUSSION: Co-infection is responsible for higher treatment failure rates and more relapses. TCD4+ cell count is the main predictive factor of relapse, and strict adherence to chemoprophylaxis protocols unequivocally results in a reduction of relapse rate. Combined treatment strategies using liposomal amphotericin B and miltefosine yield fewer therapeutic failures than the classic approach. CONCLUSION: We therefore conclude that alternative, combined therapeutic protocols seem to be a viable solution for these patients.

Introdução: A leishmaniose visceral é uma infeção disseminada endémica, considerada a terceira infeção parasitária oportunista mais frequente na Europa. É mais prevalente nos doentes co-infetados por vírus da imunodeficiência humana, em que acarreta um grande desafio terapêutico pelo risco acrescido de recaída. O objetivo do estudo é a caraterização de uma população co-infetada e da eficácia dos esquemas terapêuticos. Material e Métodos: Estudo retrospetivo de uma amostra seleccionada de todos os doentes com leishmaniose visceral e infeção pelo vírus da imunodeficiência humana internados num período de 10 anos num serviço de Infeciologia. Resultados: Foram incluídos 23 doentes co-infetados, dois do sexo feminino (8,7%). A contagem média de células TCD4+ à data do diagnóstico da leishmaniose visceral foi de 104,4 cels/uL (± 120,3 cels/uL), apenas dois doentes tinham carga viral indetetável (< 20 cópias/mL) e 16 (69,6%) não cumpriam terapêutica antirretroviral à data do diagnóstico. Como terapêutica optou-se por anfotericina B lipossómica em 18 doentes, antimoniato de meglumina em quatro e miltefosine num caso. Catorze (60,9%) aderiram a esquema de profilaxia secundária. Verificou-se uma taxa de recaída de 26,1% (seis doentes). Discussão: A co-infeção leishmaniose-vírus da imunodeficiência humana está associada a maior taxa de falência terapêutica e recaída, sendo a contagem de células TCD4+ o principal fator preditivo de recaída e o cumprimento de esquemas de quimioprofilaxia inequívoco na redução da mesma. A terapêutica combinada com anfotericina B lipossómica e miltefosine regista taxas de falência inferiores ao esquema clássico. Conclusão: Conclui-se que esquemas alternativos e combinados parecem ser uma alternativa nesta população.