Résumé
Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.
Sujets)
Humains , Nourrisson , Mâle , Anémie hémolytique , Anémie hémolytique auto-immune , Biopsie , Cellules géantes , Hépatite , Hépatocytes , Inflammation , Ictère , Foie , Maladies du foie , Pâleur , Examen physique , Maladies rares , Récidive , Rituximab , SplénomégalieRésumé
A síndrome de enterocolite induzida por proteína alimentar, conhecida como "FPIES" (do inglês: Food Protein-Induced Enterocolitis Syndrome) é uma das apresentações da alergia alimentar não IgE mediada. Tema antes considerado raro, torna-se cada vez mais frequente nos pronto-atendimentos pediátricos. Através dos dados disponíveis na literatura, buscou-se relatar apresentação, diagnóstico e manejo da FPIES. Foi realizada busca ativa na base de dados PubMed do termo "food protein-induced enterocolitis" entre 2014 e 2019. Foram selecionados os artigos cuja população em estudo compunha a faixa etária pediátrica, e artigos completos que estavam disponíveis. Os pacientes usualmente descritos são lactentes com vômitos incoercíveis, diarreia, palidez, letargia e desidratação. Destes, alguns casos evoluem para choque hipovolêmico e acidose metabólica, podendo levar a diagnósticos equivocados. A proteína do leite de vaca, soja e arroz compõem os principais desencadeantes da doença. Entretanto, há diversos alimentos descritos neste processo. O diagnóstico dá-se através de história clínica compatível associada à reprodutibilidade dos sintomas quando ocorre reexposição ao alimento suspeito. O manejo agudo fundamenta-se na expansão volêmica, ondansetrona e corticoide, nos casos graves. Devido aos múltiplos fenótipos existentes, curto período de estudo, prevalência e patogenia incerta, a FPIES apresenta muitas lacunas a serem preenchidas. Assim, o presente estudo apresenta os consensos disponíveis e divergências atuais.
Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated food allergy. Previously considered a rare event, it has become more frequent in pediatric care. This study aimed to report current literature findings on clinical presentation, diagnosis, and management of FPIES. An active search was conducted using PubMed database for the term "food-induced enterocolitis" in studies published between 2014 and 2019. Articles were selected if they involved a pediatric population and were available as full text. Samples usually consist of infants presenting with uncontrollable vomiting, diarrhea, pallor, lethargy, and dehydration. Some cases progress to hypovolemic shock and metabolic acidosis, leading to misdiagnosis. Milk, rice and soy proteins are the main triggers of the disease. The suspicion of FPIES is raised by clinical history associated with reproducible symptoms when re-exposure occurs. Acute management is based on volume infusion, ondansetron and corticosteroids in severe cases. Many gaps still exist in the knowledge of FPIES because of its multiple phenotypes, short-term studies, and uncertain prevalence and pathogenesis. The present study presents the available guidelines and current controversies.
Sujets)
Humains , Nourrisson , Enfant d'âge préscolaire , Enfant , Adolescent , Diagnostic différentiel , Entérocolite , Hypersensibilité alimentaire , Pâleur , Population , Signes et symptômes , Syndrome , Thérapeutique , Vomissement , Prévalence , Ondansétron , Hormones corticosurrénaliennes , Protéines de soja , Déshydratation , PubMed , Lait , Diagnostic , Diarrhée , LéthargieRésumé
PURPOSE: To report the results of ganglion cell analysis in a patient with optic tract syndrome who was previously diagnosed with glaucoma. CASE SUMMARY: A 32-year-old male, who had been diagnosed with glaucoma 12 years ago, but had not visited an ophthalmology clinic since then, came to our clinic for evaluation of his glaucoma. Both eyes showed an increased cup-to-disc ratio and temporal pallor of the disc. Retinal nerve fiber layer (RNFL) optical coherence tomography showed thinning of the superior, inferior, and temporal peripapillary RNFL in both eyes. On ganglion cell analysis (GCA), ganglion cell layer thinning in the nasal region of the right eye and in the temporal region of the left eye was observed. The visual field test showed right incongruous homonymous hemianopsia. After the atrophic change of the left optic tract was confirmed by orbit magnetic resonance imaging, he was diagnosed with left optic tract syndrome. CONCLUSIONS: We report the results of GCA in a case of optic tract syndrome, previously diagnosed as glaucoma. GCA can be useful when diagnosing optic tract syndrome.
Sujets)
Adulte , Humains , Mâle , Pseudokystes mucoïdes juxta-articulaires , Glaucome , Hémianopsie , Imagerie par résonance magnétique , Neurofibres , Ophtalmologie , Nerf optique , Tractus optique , Orbite , Pâleur , Rétinal , Lobe temporal , Tomographie par cohérence optique , Tests du champ visuelRésumé
Raynaud's phenomenon (RP) is a reversible vasospasm that is aggravated by cold or emotional stress. Before confirming RP, it is essential to consider other possible causes including compressive neuropathy, sensori-neuropathy, thyroid disease, hematologic conditions and offending drugs. RP is typically characterized by the three-step color change that turns pallor (white), cyanosis (blue), and then erythema (red) of reperfusion. Once RP is diagnosed, it is important to determine whether it is primary or secondary RP. To distinguish primary from the secondary RP, the specialized tests performing in clinical practice are antinuclear antibody (ANA) and nailfold capillary microscopy (NFC). The combination of ANA and NFC is most helpful for discriminating secondary RP due to autoimmune rheumatic disease. Thereby, normal findings of NFC in primary RP distinguished from secondary RP should be understood. Patients with primary RP usually improves with symptomatic treatment focused on lifestyle modification and patient education, but those with secondary RP should be treated together with associated disease or causes.
Sujets)
Humains , Anticorps antinucléaires , Vaisseaux capillaires , Cyanose , Érythème , Mode de vie , Capillaroscopie , Microscopie , Pâleur , Éducation du patient comme sujet , Reperfusion , Rhumatismes , Stress psychologique , Maladies de la thyroïdeRésumé
PURPOSE: We report a case of bilateral nonarteritic anterior ischemic optic neuropathy (NAION) following acute angle-closure crisis (AACC). CASE SUMMARY: A 76-year-old female visited our clinic because of a 1-day history of ocular pain and vision loss in both eyes. The visual acuity was 0.02 in both eyes and her intraocular pressure (IOP) was 52 mmHg in the right eye (RE) and 50 mmHg in the left eye (LE). She had corneal edema and a shallow anterior chamber in both eyes, with 4 mm fixed dilated pupils. After decreasing the IOP with intravenous mannitol, laser iridotomy was performed. However, 2 days later, visual acuity was further reduced to finger counting at 10 cm RE and at 50 cm LE, and her optic disc was swollen. Bilateral NAION following AACC was diagnosed. One month later, visual acuity slightly improved to 0.02 RE and 0.04 LE, and the optic disc edema resolved. A small cup-disc ratio, optic disc pallor, and atrophy were observed. Humphrey visual fields demonstrated superior and inferior altitudial visual field defects in the LE, and almost total scotoma in the RE. CONCLUSIONS: AACC can be a predisposing factor for NAION, so the relative afferent pupillary defect, papilledema, and presentation of other risk factors are important clues to a diagnosis of NAION.
Sujets)
Sujet âgé , Femelle , Humains , Chambre antérieure du bulbe oculaire , Atrophie , Causalité , Oedème cornéen , Diagnostic , Oedème , Doigts , Glaucome à angle fermé , Pression intraoculaire , Mannitol , Neuropathie optique ischémique , Pâleur , Oedème papillaire , Pupille , Troubles pupillaires , Facteurs de risque , Scotome , Acuité visuelle , Champs visuelsRésumé
The relationship between the vestibular system and the autonomic nervous system has been well studied in the context of the maintenance of homeostasis to the changing internal and external milieus. The perturbations of the autonomic indexes to the vestibular stimuli have been demonstrated in animal studies. In addition, the fluctuation of the blood pressure and the heart rate with other autonomic symptoms such as nausea, vomiting, and pallor are common manifestations in the wide range of vestibular disorders. At the same time, the disorders of the autonomic nervous system can cause dizziness and vertigo in some group of patients. In the anatomical point of view, the relationship between autonomic and vestibular systems is evident. The afferent signals from each system converge to the nucleus of solitary tract to be integrated in medullary reticular formation and the each pathway from the vestibular and autonomic nervous system is interconnected from medulla to cerebral cortex. In this paper, the reported evidence demonstrating the relationship between autonomic derangement and vestibular disorders is reviewed and the further clinical implications are discussed.
Sujets)
Animaux , Humains , Système nerveux autonome , Maladies du système nerveux autonome , Pression sanguine , Cortex cérébral , Sensation vertigineuse , Rythme cardiaque , Homéostasie , Nausée , Pâleur , Formation réticulaire , Noyau du tractus solitaire , Vertige , VomissementRésumé
Azathioprine is an immunosuppressive drug that has been widely used in dermatology for the treatment of immunobullous diseases. Myelosuppression is the most important side effect and requires close observation of the complete blood cell count. The clinical findings of myelosuppression include general weakness, poor oral intake, nausea, dyspnea, and pallor. It can occur within several weeks to years after initial azathioprine treatment; thus, a weekly full blood count for the first 4 weeks, followed by reduced frequency of monitoring to a minimum of once every 3 months is recommended. If the myelosuppression is not treated properly, it can lead to fever, secondary infection, sepsis, and even death. Herein, we present three educational cases for dermatologists to order to underline the risk of myelosuppression during azathioprine treatment.
Sujets)
Azathioprine , Hémogramme , Co-infection , Dermatologie , Dyspnée , Fièvre , Nausée , Pâleur , SepsieRésumé
A 2-year-old intact female Bengal cat was presented with a 6-month history of visual impairment. The cat manifested bilateral negative menace responses and dazzle reflexes and sluggish pupillary light reflexes. Bilateral fundus changes included generalized tapetal hyperreflectivity, advanced retinal vascular attenuation, and increased pallor of the optic disc. A diagnosis of bilateral retinal degeneration was made. The clinical findings suggest that the investigated Bengal cat was most likely to have an inherited retinal degeneration. Further studies of the Bengal cat breed are needed to determine the prevalence of inherited retinal degeneration in this breed in Korea.
Sujets)
Animaux , Chats , Enfant d'âge préscolaire , Femelle , Humains , Diagnostic , Corée , Pâleur , Prévalence , Réflexe , Dégénérescence de la rétine , Rétinal , Troubles de la visionRésumé
Resumen Introducción: El Episodio Hipotonía-Hiporreactividad (EHH) es un efecto adverso tras la vacuna ción, asociado principalmente a vacunas anti-pertussis de células enteras. Se caracteriza por un inicio súbito de flacidez muscular, reducida respuesta a estímulos y palidez cutánea o cianosis. Aunque el EHH es infrecuente, está considerado como un efecto adverso severo. Objetivo: Reportar un caso de EHH posterior a la administración de la vacuna combinada pentavalente con: difteria, tétanos, pertussis celular, hepatitis B y Haemophilus influenzae tipo b (DTwP-HB-Hib), que está incluida en el Programa Nacional de Inmunizaciones (PNI) de Chile, con la finalidad de difundir esta infrecuente complicación de evolución benigna, auto-limitada y de carácter no recurrente. Caso clínico: Lactante de 6 meses de edad, 3 h post-vacunación con la tercera dosis de vacuna DTwP-HB-Hib, presentó compromiso del estado de conciencia interpretado como convulsión atónica y que finalmente se consideró como EHH. El lactante evolucionó favorablemente después de 2 h y fue dado de alta tras 24 h de vigilancia clínica; se cambió el esquema de inmunización del lactante con vacunas anti-per tussis acelulares como medida preventiva. Conclusiones: El desconocimiento sobre el EHH puede desalentar la inmunización infantil. Por lo tanto, es importante que el personal médico informe a los padres de los pacientes sobre este evento benigno, autolimitado y no recurrente. En estos casos, se re comienda continuar con el programa de inmunización del lactante con formulaciones que contengan componentes anti-pertussis acelulares.
Abstract Introduction: Hypotonic-Hyporesponsive Episode (HHE) is an adverse event after vaccination, mainly associated with whole-cell pertussis vaccines. It is characterized by a sudden onset of muscle flaccidity, reduced response to stimuli and pallor or cyanosis. Although the HHE is infrequent, it is considered a severe adverse event. Objective: To report a case of HHE following the administration of the whole-cell pertussis combination vaccine (DTwP-HB-Hib), which is included in National Im munization Program (PNI) of Chile, and to contributing to the knowledge of this adverse event in the country. Case report: A 6-month-old infant, 3 hours post-vaccination with the third dose of DTwP-HB-Hib vaccine, presented a decreased level of consciousness that was interpreted as atonic seizure but finally considered as EHH. The infant progressed favorably after 2 hours of clinical observation and was discharged 24 hours later. Parents were suggested to continue the immunization schedule of the infant with acellular pertussis vaccines as a preventive measure. Conclusions: The lack of knowledge about the EHH may discourage childhood immunization. Therefore, it is important for the medical staff to inform parents of the patients about this benign, self-limited and non-recurrent adverse event. In these cases, it is recommended to continue the immunization schedule of the infant with acellular pertussis vaccines.
Sujets)
Humains , Mâle , Nourrisson , Pâleur/étiologie , Vaccin diphtérie-tétanos-coqueluche/effets indésirables , Vaccins anti-hépatite B/effets indésirables , Vaccins anti-Haemophilus/effets indésirables , Troubles de la conscience/étiologie , Hypotonie musculaire/étiologieRésumé
Pulmonary artery aneurysms (PAAs) are rare, and massive hemoptysis can lead to death if appropriate diagnosis and treatment is not provided. PAAs can be of congenital, acquired, or idiopathic origin, and the clinical symptoms are various. Among all reported cases, one-third of the patients died due to rupture. Optimal treatment or guidelines for PAAs remain uncertain. Herein, we report autopsy findings from a woman with PAA. The patient was taking medication for tuberculosis. On bronchoscopy, a polypoid lesion was found, suspected to be an inflammatory polyp. Biopsy was performed and massive bleeding into the airway occurred. The bleeding could not be controlled by bronchoscopic suction, and cardiac arrest occurred 30 minutes after biopsy; the patient subsequently died. Autopsy revealed a round, calcified PAA in the bronchus of the right middle lobe; the end of the PAA was torn. Hypovolemic signs, including weak postmortem lividity and pallor of the skin and conjunctivae, were observed. Visual inspection and histopathological examination of the right lung revealed tuberculosis and congestion. Cases related PAA are not uncommon, but autopsy cases of death occurring after biopsy of PAA mimicking bronchial polyps are rarely reported.
Sujets)
Femelle , Humains , Anévrysme , Autopsie , Biopsie , Bronches , Bronchoscopie , Conjonctive , Diagnostic , Oestrogènes conjugués (USP) , Arrêt cardiaque , Hémoptysie , Hémorragie , Hypovolémie , Poumon , Pâleur , Polypes , Artère pulmonaire , Rupture , Peau , Aspiration (technique) , TuberculoseRésumé
Massive fetomaternal hemorrhage (FMH) is a major cause of unexplained fetal death and neonatal anemia. FMH can be diagnosed using the Kleihauer-Betke test or flow cytometry by identifying the presence of fetal red cells in the maternal blood. However, timely diagnosis is a challenge because many hospitals lack the equipment needed to perform such tests. The authors experienced a case of FMH diagnosed via high-performance liquid chromatography (HPLC) which is generally used in measuring glycated hemoglobin (HbA1c) in a patient with unexplained neonatal anemia. A girl aged 2 days was transferred to our hospital for showing pallor and a hemoglobin level of 5.0 g/dL. HPLC revealed 3% fetal hemoglobin (HbF) in the maternal blood. HPLC is a quick test for quantifying HbF that is readily available in many hospitals and could serve as a promising alternative for diagnosing FMH.
Sujets)
Femelle , Humains , Nouveau-né , Grossesse , Anémie néonatale , Chromatographie en phase liquide à haute performance , Chromatographie en phase liquide , Diagnostic , Mort foetale , Hémoglobine foetale , Transfusion foetomaternelle , Cytométrie en flux , Hémoglobine glyquée , Pâleur , Temps de prothrombineRésumé
Massive fetomaternal hemorrhage (FMH) is a major cause of unexplained fetal death and neonatal anemia. FMH can be diagnosed using the Kleihauer-Betke test or flow cytometry by identifying the presence of fetal red cells in the maternal blood. However, timely diagnosis is a challenge because many hospitals lack the equipment needed to perform such tests. The authors experienced a case of FMH diagnosed via high-performance liquid chromatography (HPLC) which is generally used in measuring glycated hemoglobin (HbA1c) in a patient with unexplained neonatal anemia. A girl aged 2 days was transferred to our hospital for showing pallor and a hemoglobin level of 5.0 g/dL. HPLC revealed 3% fetal hemoglobin (HbF) in the maternal blood. HPLC is a quick test for quantifying HbF that is readily available in many hospitals and could serve as a promising alternative for diagnosing FMH.
Sujets)
Femelle , Humains , Nouveau-né , Grossesse , Anémie néonatale , Chromatographie en phase liquide à haute performance , Chromatographie en phase liquide , Diagnostic , Mort foetale , Hémoglobine foetale , Transfusion foetomaternelle , Cytométrie en flux , Hémoglobine glyquée , Pâleur , Temps de prothrombineRésumé
Bee sting causes mild symptoms such as urticaria and localized pain, and severe symptoms including anaphylaxis, cardiovascular collapse, and death. We reported on a patient with arterial thrombotic occlusion and severe ischemia in the lower limb after multiple bee stings. The patient was stung 5 times and complained of pallor, pain, and coldness in the left toe, and did not have dorsalis pedis pulsation. Computed tomography angiography showed multiple thrombotic occlusion of the anterior and posterial tibial artery below the knee. Local thrombolytic therapy using urokinase was administered and the occluded arteries were successfully recanalized.
Sujets)
Humains , Anaphylaxie , Angiographie , Angioplastie par ballonnet , Artères , Venins d'abeille , Abeilles , Morsures et piqûres , Ischémie , Genou , Membre inférieur , Pâleur , Traitement thrombolytique , Artères tibiales , Orteils , Activateur du plasminogène de type urokinase , UrticaireRésumé
PURPOSE: We report a case of meningioma diagnosed as ischemic optic neuropathy. CASE SUMMARY: A 45-year-old women presented with decreased visual acuity in her left eye without eye movement pain. Her best corrected visual acuity (BCVA) in that eye was 0.3. She showed a relative afferent pupillary defect, abnormal color vision test, and inferior visual field defect in her left eye. The optic disc showed slight blurring superiorly and pallor temporally. Fluorescein angiography showed choroidal filling defect, and ischemic optic neuropathy was suspected. The carotid artery sonography showed normal results. The BCVA of the left eye was 0.5 after 7 months. The visual field test and color vision test were improved after 7 months. The optic disc was pale. The magnetic resonance imaging was performed because of persistent headache, and that showed a suprasellar mass that was removed by surgical resection and diagnosed as meningioma on biopsy. CONCLUSIONS: The possibility of compressive optic neuropathy should be considered in presumed ischemic optic neuropathy if the patient complains of persistent headache.
Sujets)
Femelle , Humains , Adulte d'âge moyen , Biopsie , Artères carotides , Choroïde , Vision des couleurs , Mouvements oculaires , Angiographie fluorescéinique , Céphalée , Imagerie par résonance magnétique , Méningiome , Atteintes du nerf optique , Neuropathie optique ischémique , Pâleur , Troubles pupillaires , Acuité visuelle , Tests du champ visuel , Champs visuelsRésumé
Foram examinados 130 pacientes e realizadas 241 observações, com a finalidade de investigar a acurácia da palidez no diagnóstico da anemia no adulto. As regiões anatômicas observadas foram as comumente utilizadas pelos médicos, a conjuntiva, lábio, palma da mão, linha palmar e uma região ainda não relatada, a extremidade digital, examinando-se a antiga técnica da gradação da palidez. Os autores concluem pela recomendação dos velhos exames e pela adoção do novo sinal, descrito neste trabalho. Este estudo foi aprovado pela Comissão de Ética da Instituição e todos os pacientes assinaram um consentimento informado.
Sujets)
Humains , Mâle , Femelle , Enfant , Adolescent , Jeune adulte , Adulte d'âge moyen , Anémie , Examen physique , PâleurRésumé
Canid herpesvirus 1 (CHV-1) is a widespread pathogen of dogs and produces infertility, abortions and severe systemic disease in young puppies. Clinical data indicate the circulation of CHV-1 among Brazilian dogs yet definitive diagnosis has rarely been accomplished. This article describes the clinicopathological findings of four independent cases/outbreaks of neonatal disease by CHV-1 in Bulldog puppies followed by virus identification and genetic characterization. Three events occurred in a kennel holding dogs of different breeds at reproductive age (March 2013, October 2013 and April 2014). Puppies from three French or English Bulldog litters, aging 9 to 30 days were affected, presenting dyspnea, agonic breathing, pale mucous, abdominal pain and tension, evolving to death within about 24 hours. At necropsy, the puppies presented necrohemorrhagic hepatitis, multifocal and moderate necrohemorrhagic nephritis and fibrinonecrotic interstitial pneumonia. Virus isolation was positive in clinical specimens from one litter and CHV-1 DNA was detected by PCR in tissues from all four cases. Virus-neutralizing assays with samples of the affected kennel revealed 9/12 adult animals with high antibody titers to CHV-1. Nucleotide sequencing of glycoprotein B, C and D genes revealed 99-100% of identity among the viruses and with CHV-1 sequences available in GenBank. Phylogenetic analyses of gC sequences showed a segregation of the samples, even among three isolates from the same kennel. These findings support CHV-1 infection as the cause of disease and death in these dog litters, reinforcing the need for correct etiologic diagnosis, prevention and immunization against CHV-1 in dogs from Southern Brazil.
O herpesvírus canino (CHV-1) é um patógeno de cães que possui distribuição mundial e que causa infertilidade, abortos e doença sistêmica severa em filhotes de cães. Achados clínicos tem indicado a circulação do CHV-1 em cães no Brasil, embora o diagnóstico definitivo seja raramente determinado. Este artigo descreve os achados clinicopatológicos de quatro casos/surtos independentes de morte neonatal de filhotes de cães da raça Bulldog causados pelo CHV-1, a identificação e a caracterização genética do vírus. Três eventos ocorreram no mesmo canil que abriga animais de diferentes raças em idade reprodutiva (março de 2013, outubro de 2013 e abril de 2014). Filhotes de três ninhadas de Bulldog Francês e/ou Inglês, com idade de 9 a 30 dias, foram afetados e apresentaram dispneia, respiração agônica, mucosas pálidas, dor e tensão abdominal, que evoluíram para morte dos cães dentro de, aproximadamente, 24 horas. Na necropsia foram observados hepatite necro-hemorrágica, nefrite necro-hemorrágica multifocal e moderada e pneumonia intersticial fibrinonecrótica. O isolamento viral foi positivo em amostras clínicas de um filhote e DNA de CHV-1 foi detectado por PCR em tecidos de filhotes de todos os surtos. Teste de soroneutralização com amostras de soro de cães provenientes do canil afetado revelaram que nove de 12 animais adultos possuíam altos títulos de anticorpos para o CHV-1. Sequenciamento de nucleotídeos do gene das glicoproteínas B, C e D revelaram 99-100% de identidade entre as amostras e com as sequências de CHV-1 disponíveis no GenBank. A análise filogenética baseada na sequência do gene da glicoproteína C mostrou uma segregação das amostras, mesmo entre os três isolados de vírus provenientes do mesmo canil. Esses achados demonstram que o CHV-1 é a causa da doença e da morte dos filhotes, reforçando a necessidade do correto diagnóstico etiológico e a implementação de medidas de prevenção e imunização contra o CHV-1 em cães no sul do Brasil.
Sujets)
Animaux , Nourrisson , Chiens , Herpèsvirus canin de type 1 , Épidémies de maladies/statistiques et données numériques , Épidémies de maladies/médecine vétérinaire , Dyspnée/médecine vétérinaire , Douleur abdominale/médecine vétérinaire , Glycoprotéines/génétique , Pâleur/médecine vétérinaire , Réaction de polymérisation en chaîne/médecine vétérinaireRésumé
Acquired pure red cell aplasia (PRCA) can be induced by various factors such as viral infection, thymoma, connective tissue disease, lymphoma, and adverse drug reactions. PRCA has not been reported in an adolescent in Korea for the past several decades. We recently experienced a case of acquired PRCA in an adolescent. A 14-year-old girl presented with pallor, dizziness, and mild fever. She had isolated normocytic normochromic anemia with reticulocytopenia in the peripheral blood and erythroblastopenia in the bone marrow. She was diagnosed with secondary acquired PRCA presumably induced by Mycoplasma pneumoniae infection during her clinical course, and she experienced spontaneous remission 11 weeks after initial diagnosis. Her clinical and hematologic statuses were normal as far as 20 months after her diagnosis.
Sujets)
Adolescent , Femelle , Humains , Anémie , Moelle osseuse , Maladies du tissu conjonctif , Diagnostic , Sensation vertigineuse , Effets secondaires indésirables des médicaments , Fièvre , Corée , Lymphomes , Infections à Mycoplasma , Mycoplasma pneumoniae , Pâleur , Pneumopathie à mycoplasmes , Érythroblastopénie chronique acquise , Rémission spontanée , ThymomeRésumé
Acquired pure red cell aplasia (PRCA) can be induced by various factors such as viral infection, thymoma, connective tissue disease, lymphoma, and adverse drug reactions. PRCA has not been reported in an adolescent in Korea for the past several decades. We recently experienced a case of acquired PRCA in an adolescent. A 14-year-old girl presented with pallor, dizziness, and mild fever. She had isolated normocytic normochromic anemia with reticulocytopenia in the peripheral blood and erythroblastopenia in the bone marrow. She was diagnosed with secondary acquired PRCA presumably induced by Mycoplasma pneumoniae infection during her clinical course, and she experienced spontaneous remission 11 weeks after initial diagnosis. Her clinical and hematologic statuses were normal as far as 20 months after her diagnosis.
Sujets)
Adolescent , Femelle , Humains , Anémie , Moelle osseuse , Maladies du tissu conjonctif , Diagnostic , Sensation vertigineuse , Effets secondaires indésirables des médicaments , Fièvre , Corée , Lymphomes , Infections à Mycoplasma , Mycoplasma pneumoniae , Pâleur , Pneumopathie à mycoplasmes , Érythroblastopénie chronique acquise , Rémission spontanée , ThymomeRésumé
OBJECTIVE: To assess actual rates of late vaginal stenosis and identify predisposing factors for complications among patients with previously untreated cervical cancer following high-dose-rate brachytherapy. METHODS: We performed longitudinal analyses of 57 patients using the modified Dische score at 6, 12, 18, 24, 36, and 60 months after treatment, which consisted of 15 interstitial brachytherapys and 42 conventional intracavitary brachytherapys, with a median follow-up time of 36 months (range, 6 to 144 months). RESULTS: More than half of the patients developed grade 1 (mild) vaginal stenosis within the first year of follow-up, and grade 2 (97.5%, moderate) to grade 3 (severe) stenosis gradually increased with time. Actual stenosis rates for grade 1, 2, and 3 were 97.5% (95% confidence interval [CI], 92.7 to 97.5), 60.7% (95% CI, 42.2 to 79.3), and 7.4% (95% CI, 0 to 18.4) at 3 years after treatment. Pallor reaction grade 2-3 at 6 months was only a statistically significant predisposing factor for grade 2-3 late vaginal stenosis 3 years or later with a hazard ratio of 3.48 (95% CI, 1.32 to 9.19; p=0.018) by a multivariate Cox proportional hazard model. Patients with grade 0-1 pallor reaction at 6 months showed a grade > or =2 vaginal stenosis rate of 53%, whereas the grade 2-3 pallor reaction group achieved a grade > or =2 vaginal stenosis rate at 3 years at 100% (p=0.001). CONCLUSION: High-dose-rate brachytherapy was associated with high incidence of late vaginal stenosis. Pallor reaction grade 2-3 at 6 months was predictive of late grade 2-3 vaginal stenosis at 3 years after treatment. These findings should prove helpful for patient counseling and preventive intervention.
Sujets)
Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Femelle , Humains , Adulte d'âge moyen , Curiethérapie/effets indésirables , Sténose pathologique/étiologie , Radio-isotopes de l'iridium/usage thérapeutique , Pâleur , Pronostic , Études prospectives , Radiopharmaceutiques/usage thérapeutique , Études rétrospectives , Tumeurs du col de l'utérus/radiothérapie , Maladies du vagin/étiologieRésumé
BACKGROUND: Priapism is a rare complication seen in one to five percent of adult leukemic patients. The word 'Priapism' is related to Priapus, the Greek and Roman God of procreation whose symbol was an erect phallus. CLINICAL PRESENTATION: The patient is a 22-year-old male with no known co-morbidities presenting with one month intermittent, unstimulated, painful penile erection with no other associated symptoms which resolves spontaneously, until nine hours prior to admission when symptoms recurred and persisted. Patient had no history of trauma and no drug intake. PHYSICAL FINDINGS: Patient was awake, in pain and tachycardic. There was note of pallor and splenomegaly. The penis was erect, firm, swollen and tender with superficial venous engorgement. The rest of the physical examination was unremarkable. LABORATORY WORK UP: Complete blood count showed anemia and leukocystosis. Peripheral blood smear revealed markedly increased white blood cells with predominance of mature and immature cells belonging to granulocytic series. There was splenomegaly on ultrasound. Genetic testing showed an abnormal male karyotype of 46 chromosomes including translocation (9;22). TREATMENT: Corpora cavernosa aspiration was done. Terbutaline was given. Patient was started and maintained on hydroxyurea and presently enrolled in Imitanib study. OUTCOME: There was resolution of priapism after the corpus cavernosa aspiration and initiation of hydroxyurea and the white blood cell count had decreased after initiation of hydroxyurea.