RESUMO
BACKGROUND: Increased blood levels of alanine amino transferase (ALT, also known as SGPT; serum glutamic pyruvic transaminase) serve as a marker of liver injury by various mechanisms. Less is known about the clinical implications associated with low-normal ALT levels. Previous studies showed low ALT levels to be associated with poor long-term outcomes among elderlies, serving as a biomarker for increased incidence of frailty and subsequent risk of mortality. However, it has not been determined yet whether low-normal ALT values might be predictive of frailty and mortality in younger, middle-aged adults. METHODS: We conducted a historical prospective cohort analysis. RESULTS: A total of 23,506 adults with ALT levels within the normal range, at the mean age of 48 ± 11 years, participating in an annual screening program for preventive medicine, were followed-up for a median period of 8.5 years during which 638 died. Low-normal ALT values (serum ALT activity <17IU/L) were found to be predictive for increased risk of all-cause mortality (HR=1.6; 95% CI 1.34-1.92; p<0.001). Statistically significant correlation was demonstrated even after applying a multifactorial model correction for age, gender, eGFR, low albumin, arterial hypertension, diabetes mellitus and ischemic heart disease. CONCLUSIONS: We suggest that low-normal ALT values may serve as an independent predictive marker for increased long-term mortality in middle-aged adults.
Assuntos
Alanina Transaminase/sangue , Mortalidade , Adulto , Idoso , Estudos de Coortes , Feminino , Idoso Fragilizado , Humanos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos ProspectivosRESUMO
BACKGROUND: Although normal pregnancy is the precursor of 25% of cases of maternal choriocarcinoma, intraplacental choriocarcinoma in an otherwise normal placenta associated with viable pregnancy has rarely been reported. CASE: Examination of the placenta after delivery of a pale and small-for-date infant at term revealed intraplacental choriocarcinoma. There was no evidence of metastatic disease in the mother or child, but the mother exhibited postpartum rising levels of beta-HCG. The mother refused chemotherapy and disappeared from follow-up. Nine months later, she presented with metastatic choriocarcinoma of the lung. Eleven courses of the multi-drug EMA CO regimen effected a decrease of beta-HCG to normal and disappearance of lung metastases. To date, 28 months after the end of chemotherapy, the patient is alive and without evidence of gestational trophoblastic disease. Moreover, since then she has given birth to an additional two children. CONCLUSIONS: This case is an example of natural disease progression of intraplacental choriocarcinoma metastasizing to the mother. Furthermore, it supports common knowledge that the multi-drug EMA CO regimen is effective treatment in poor prognosis metastatic choriocarcinoma.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Coriocarcinoma/secundário , Neoplasias Pulmonares/secundário , Complicações Neoplásicas na Gravidez/diagnóstico , Resultado da Gravidez , Neoplasias Uterinas/patologia , Adulto , Biópsia por Agulha , Feminino , Seguimentos , Idade Gestacional , Humanos , Imuno-Histoquímica , Neoplasias Pulmonares/patologia , Estadiamento de Neoplasias , Placenta/patologia , Gravidez , Radiografia Torácica , Medição de Risco , Resultado do Tratamento , Recusa do Paciente ao TratamentoRESUMO
Peripheral primitive neuroectodermal tumors (PNET) are extremely uncommon, malignant neoplasms affecting mostly children and young adults. We retrospectively reviewed the clinical data and radiological studies of four such cases. All cases were pathologically proven. Plain films, US, and CT scans were used. The youngest child had a huge pelvic tumor and two adolescents each had a chest wall (Askin) tumor. The fourth patient had a most unusual location of the PNET in the anterior mediastinum. The CT findings are emphasized. We emphasize that the markedly abnormal CT findings are not specific for PNET.
Assuntos
Neoplasias Abdominais/diagnóstico por imagem , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico por imagem , Neoplasias Torácicas/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Masculino , Estudos RetrospectivosRESUMO
Two newborns with glomerulocystic kidney disease manifesting as late onset oligohydramnion and neonatal anuria, yet without severe respiratory distress, are presented. They had a similar perinatal course and associated clinical manifestations. No associated congenital or inherited malformation syndrome could be defined. Both infants' parents were first degree cousins and belonged to the same small Bedouin tribe, and neither they nor the infants' siblings had polycystic kidneys or renal insufficiency, pointing to either a possible genetic etiology or a common external toxic exposure.
Assuntos
Injúria Renal Aguda/complicações , Glomérulos Renais , Oligo-Hidrâmnio/complicações , Doenças Renais Policísticas/complicações , Adulto , Anuria/complicações , Biópsia , Feminino , Humanos , Recém-Nascido , Rim/patologia , Glomérulos Renais/patologia , Doenças Renais Policísticas/genética , Doenças Renais Policísticas/patologia , Gravidez , Crânio/diagnóstico por imagem , Crânio/embriologia , Tomografia Computadorizada por Raios XAssuntos
Árabes/genética , Doença de Crohn/diagnóstico , Doença de Crohn/genética , Doença de Crohn/patologia , Doença de Crohn/cirurgia , Diarreia Infantil/etiologia , Insuficiência de Crescimento/etiologia , Feminino , Hemorragia Gastrointestinal/etiologia , Humanos , Lactente , Recém-Nascido , Israel , MasculinoRESUMO
The clinical presentation, surgical findings, and histology of a granular cell tumor (myoblastoma) of the vulva in a 9-year-old girl are presented. Although rare, this benign lesion must be considered in the differential diagnosis of the labia major masses, such as Bartholin's duct cyst, lipoma, papilloma, hydradenoma, and fibroma.
Assuntos
Tumor de Células Granulares/patologia , Neoplasias Vulvares/patologia , Criança , Diagnóstico Diferencial , Feminino , Tumor de Células Granulares/cirurgia , Humanos , Vulva/patologia , Neoplasias Vulvares/cirurgiaAssuntos
Desfibriladores Implantáveis , Endocardite Bacteriana/diagnóstico , Infecções Relacionadas à Prótese/diagnóstico , Infecções Estreptocócicas/diagnóstico , Streptococcus agalactiae , Fibrilação Ventricular/terapia , Endocardite Bacteriana/patologia , Endocardite Bacteriana/cirurgia , Análise de Falha de Equipamento , Humanos , Masculino , Pessoa de Meia-Idade , Infecções Relacionadas à Prótese/patologia , Infecções Relacionadas à Prótese/cirurgia , Infecções Estreptocócicas/patologia , Infecções Estreptocócicas/cirurgia , Toracotomia , Valva Tricúspide/patologiaRESUMO
A 56 year-old man with a 2-year history of transitional-cell carcinoma of the urinary bladder presented with back pain and diffuse radiological osteosclerosis. Laboratory studies revealed borderline-low ionized serum calcium, and markedly elevated serum alkaline phosphatase (ALP) activity and serum osteocalcin. Serum acid phosphatase and prostatic-specific antigen (PSA) were normal. Serum 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D were just below the normal limit and parathyroid hormone was moderately elevated. A transiliac bone biopsy specimen revealed expanded bone trabeculae, abundance of osteoid-covered surfaces, and heavy metastatic spread. To the best of our knowledge, this is the first description of the association of osteosclerosis and metastatic transitional-cell carcinoma of the bladder.
Assuntos
Osso e Ossos/patologia , Carcinoma de Células de Transição/patologia , Osteosclerose/patologia , Osteosclerose/fisiopatologia , Neoplasias da Bexiga Urinária/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Osteosclerose/diagnóstico por imagem , RadiografiaRESUMO
The simultaneous appearance of epidermolysis bullosa and pyloric atresia (EB-PA) is recognized as an autosomal recessive disease; however, the coappearance of EB-PA and aplasia cutis congenita (ACC) has not been delineated as a defined entity. The aim of this study was to analyze clinically and histopathologically eight cases with EB-PA-ACC belonging to an extended Bedouin family to gain insight into the cause and pathophysiology of the disease. All affected infants were found to have mixed skin lesions, including blisters and patchy lack of skin. Almost all of them (seven of eight) also had intestinal obstructions, especially pyloric atresia or stenosis. Skin lesions involved all skin layers with marked dystrophic changes. The intestinal obstruction was the result of overproliferation of connective tissue. In view of the clinical and histopathological findings, it is postulated that the condition is caused by an autosomal recessive gene affecting the integrity of the basement membrane and hemidesmosomes and the control of the normal process of fibrosis occurring during the course of wound healing. The sequence of events is initiated by the separation of the epidermis or the intestinal mucosal layer. Then, inflammatory reaction takes place and proceeds with massive fibrosis penetrating the deep layers and causing damage of skin and obstruction of the intestinal lumen. In view of the recent findings regarding the molecular basis of EB-PA, the described phenotype may result from a mutation in one of the integrin genes.
Assuntos
Displasia Ectodérmica/patologia , Epidermólise Bolhosa Distrófica/patologia , Genes Recessivos , Piloro/anormalidades , Displasia Ectodérmica/complicações , Displasia Ectodérmica/genética , Epidermólise Bolhosa Distrófica/complicações , Epidermólise Bolhosa Distrófica/genética , Feminino , Doenças Fetais , Humanos , Masculino , LinhagemRESUMO
We report a case of a chorioangioma of the placenta which was associated with elevated maternal serum HCG in the second trimester, severe early polyhydramnios and premature delivery. Chorioangioma should be considered in the differential diagnosis of an elevated HCG observed as part of the triple test.
Assuntos
Gonadotropina Coriônica/sangue , Hemangioma/complicações , Doenças Placentárias/complicações , Poli-Hidrâmnios/complicações , Complicações Neoplásicas na Gravidez/sangue , Ultrassonografia Pré-Natal , Adulto , Feminino , Hemangioma/sangue , Hemangioma/diagnóstico por imagem , Humanos , Doenças Placentárias/sangue , Doenças Placentárias/diagnóstico por imagem , Poli-Hidrâmnios/sangue , Poli-Hidrâmnios/diagnóstico por imagem , Gravidez , Complicações Neoplásicas na Gravidez/diagnóstico por imagem , Segundo Trimestre da GravidezRESUMO
BACKGROUND: Newborn infants are particularly prone to hypothermia, a condition with a high mortality. OBJECTIVE: To study the CT brain patterns in infants with hypothermia and neurological symptoms. MATERIALS AND METHODS: We reviewed the brain CT of nine infants with neonatal hypothermia, multiple organ failure, seizures and coma. RESULTS: Two infants had normal CT scans, acutely and at follow-up, and were clinically normal at follow-up. In seven infants, CT showed diffuse cerebral oedema, with reversal of the normal density relationship between grey and white matter and a relative increased density of the thalami, brainstem and cerebellum - the 'reversal sign'. In six surviving infants with severe developmental delay, follow-up CT revealed cerebral atrophy with multicystic encephalomalacia. CONCLUSIONS: The 'reversal sign' has been described in the abused child, birth asphyxia and anoxia due to drowning. Neonatal hypothermia is offered as a further cause.
Assuntos
Encéfalo/diagnóstico por imagem , Hipotermia/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Dano Encefálico Crônico/etiologia , Edema Encefálico/diagnóstico por imagem , Edema Encefálico/etiologia , Feminino , Seguimentos , Humanos , Hipotermia/complicações , Recém-Nascido , Masculino , Fatores de TempoRESUMO
In adults clinically silent adrenal masses can be discovered incidentally in imaging studies. Most of these 'incidentalomas' are benign, non-functioning adenomas. In contradistinction, in infancy and childhood the most common adrenal mass is the neuroblastoma, a malignant neural crest tumour. Four children are described, each with a benign neural crest tumour - ganglioneuroma - incidentally discovered by conventional radiography or sonographic examination. Complete surgical excision resulted in total recovery of all the children.
Assuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico , Ganglioneuroma/diagnóstico , Neoplasias do Mediastino/diagnóstico , Neoplasias das Glândulas Suprarrenais/cirurgia , Biópsia , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Seguimentos , Ganglioneuroma/cirurgia , Humanos , Masculino , Neoplasias do Mediastino/cirurgia , Crista Neural/patologia , Radiografia Abdominal , Radiografia Torácica , Tomografia Computadorizada por Raios XAssuntos
Cálculos Renais/complicações , Pielonefrite Xantogranulomatosa/complicações , Calcinose/complicações , Calcinose/diagnóstico , Criança , Feminino , Humanos , Rim/diagnóstico por imagem , Rim/patologia , Cálculos Renais/diagnóstico , Cálculos Renais/cirurgia , Pelve Renal/diagnóstico por imagem , Músculos Psoas/diagnóstico por imagem , Pielonefrite Xantogranulomatosa/diagnóstico , Pielonefrite Xantogranulomatosa/cirurgia , Tomografia Computadorizada por Raios X , UltrassonografiaRESUMO
BACKGROUND: Literature information regarding clinical and histological correlates in celiac disease is limited. The present study was designed to assess the value of various clinical parameters in predicting the severity of small bowel histopathology. METHODS: Small bowel biopsy specimens of 59 children with established celiac disease (ESPGAN criteria) were evaluated blindly. Morphology was evaluated based on a common histopathology score. The following clinical variables were evaluated: age at diagnosis, duration of symptoms, severity score of clinical symptoms, severity score of physical signs, and growth parameters (height and weight Z scores). Multiple regression analysis was performed to evaluate the relative importance of each clinical parameter. RESULTS: Only three clinical variables revealed a significant correlation with the histopathology score. The symptom severity score (t = 3.883, p = 0.0003) demonstrated a positive correlation. The two others, age at diagnosis (t = 3.076, p = 0.0032) and duration of symptoms (t = -2.987, p = 0.0041), revealed a negative correlation. CONCLUSIONS: We conclude that more severe clinical symptoms of a shorter duration, presented at a younger age, are better predictors of a more severe form of small bowel histopathology in children with celiac disease.
Assuntos
Doença Celíaca/patologia , Intestino Delgado/patologia , Adolescente , Biópsia , Doença Celíaca/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Prontuários Médicos , Análise de Regressão , Estudos Retrospectivos , Índice de Gravidade de DoençaRESUMO
Ovarian fibroma rarely occurs in prepubertal girls. However, we found such an asymptomatic, large, uncalcified pelvic mass in an otherwise healthy 12-year-old girl. The plain films (radiographs), US and CT findings are presented.
Assuntos
Fibroma/diagnóstico , Neoplasias Ovarianas/diagnóstico , Criança , Feminino , Fibroma/cirurgia , Humanos , Neoplasias Ovarianas/cirurgia , Palpação , Tomografia Computadorizada por Raios XRESUMO
Chronic inflammatory bowel disease has recently been reported in association with glycogen storage disease type 1 B. We report two cases of children affected by this disease in whom chronic recurrent ileocolitis and severe oesophagitis developed.
Assuntos
Doença de Crohn/etiologia , Esofagite/etiologia , Doença de Depósito de Glicogênio Tipo I/complicações , Criança , Pré-Escolar , Doença de Crohn/diagnóstico , Diagnóstico por Imagem , Esofagite/diagnóstico , Feminino , Humanos , RecidivaRESUMO
Benign salivary gland tumours in childhood are rare. We present a case of a pleomorphic adenoma arising in a minor salivary gland within the hard palate, review the literature and discuss the diagnostic and therapeutic features of the condition.
Assuntos
Adenoma Pleomorfo/patologia , Palato/patologia , Neoplasias das Glândulas Salivares/patologia , Glândulas Salivares Menores/patologia , Criança , Feminino , HumanosAssuntos
Colo/patologia , Doença de Crohn/diagnóstico , Biópsia , Colonoscopia , Doença de Crohn/terapia , Humanos , Lactente , MasculinoRESUMO
We describe a fatal outcome in a three-year-old child following massive stings by the oriental hornet (Vespa orientalis). The primary clinical features were coma, respiratory failure, coagulopathy, renal failure and liver dysfunction. On postmortem the main organs involved were brain, lungs, kidney and liver.