RESUMEN
Food protein-induced enterocolitis syndrome (FPIES) caused by fish and others is prevalent in the Mediterranean regions but is less frequently reported in Japan. This case report describes a 3-year-old Japanese girl who developed FPIES triggered by multiple seafoods, including swordfish, cod, and squid. The diagnosis was confirmed through oral food challenge tests (OFC), which led to repeated vomiting and an increase in thymus and activation-regulated chemokine (TARC) levels. This case highlights the importance of considering fish-induced FPIES in the differential diagnosis of recurrent vomiting in children and suggests the potential utility of TARC levels in diagnosing and monitoring FPIES.
Asunto(s)
Enterocolitis , Hipersensibilidad a los Alimentos , Alimentos Marinos , Humanos , Enterocolitis/etiología , Enterocolitis/diagnóstico , Femenino , Preescolar , Alimentos Marinos/efectos adversos , Hipersensibilidad a los Alimentos/diagnóstico , Hipersensibilidad a los Alimentos/complicaciones , Hipersensibilidad a los Alimentos/etiología , Japón , Animales , Síndrome , Quimiocina CCL17/sangre , Decapodiformes , Pueblos del Este de AsiaRESUMEN
Autoimmune hemolytic anemia (AIHA) is a rare disease in infants, for which steroids are recognized as a first-line therapy for patients. Rituximab, a humanized monoclonal antibody raised against CD20, has been used in the treatment of autoimmune diseases, including AIHA, in adults and children. Due to limited follow-up study of the use of rituximab in the treatment for AIHA, its long-term efficacy, adverse effects, and immunological reconstitution of B cells have not been fully evaluated in infants. Here, we report a 3-month-old female patient with refractory AIHA, who was successfully treated with rituximab. Hemolytic anemia improved rapidly, and there were no severe adverse effects caused by rituximab. After 4.5 months following rituximab treatment, peripheral B cells were gradually reconstituted and required no intravenous immunoglobulin replacement thereafter. The patient has remained disease-free for more than 30 months without any additional treatment. This case suggests that rituximab may be a valuable therapeutic option, given its efficacy and minimal adverse effects in infants with therapy-resistant AIHA.
Asunto(s)
Anemia Hemolítica Autoinmune/tratamiento farmacológico , Anticuerpos Monoclonales de Origen Murino/administración & dosificación , Antineoplásicos/administración & dosificación , Anemia Hemolítica Autoinmune/diagnóstico , Femenino , Humanos , Lactante , Inducción de Remisión , Rituximab , Factores de TiempoRESUMEN
PURPOSE: Three familial cases of each of severe congenital neutropenia (SCN) and cyclic neutropenia (CN) in addition to 3 sporadic cases of SCN were analyzed for neutrophil elastase (Ela2) gene mutation. The contents of the neutrophil-specific granule proteins cathelicidin antimicrobial peptide and neutrophil gelatinase-associated lipocalin were also analyzed in SCN. METHODS: Genomic DNA was extracted from the patients' peripheral blood or bone marrow, and the coding sequence of the Ela2 gene was amplified by polymerase chain reaction and subjected to direct sequencing. The contents of antimicrobial peptides were analyzed by flow cytometry. RESULTS: Three cases of familial SCN (P13L, R52P, and S97L), 2 of familial CN (W212stop and P110L), and 1 of sporadic SCN (V72M) were shown to have heterozygous mutations in the Ela2 gene. W212stop found in a familial CN case was a novel mutation of Ela2. Prophylactic treatment for growth factors or antibiotic prophylaxis against bacterial infection was useful for lowering the frequency of infectious episodes. Adult patients tended to have less frequent infections compared with minors in the same family. The contents of both cathelicidin antimicrobial peptide and neutrophil gelatinase-associated lipocalin were significantly reduced in SCN compared with healthy controls. CONCLUSIONS: Prophylaxis by growth factor or antibiotics is useful for decreasing risks of bacterial infections in SCN and CN. Adults were likely to have less frequent infections than children in familial cases of SCN and CN with the same mutation of Ela2.
Asunto(s)
Elastasa de Leucocito/genética , Neutropenia/congénito , Neutropenia/genética , Neutrófilos/fisiología , Mutación Puntual , Adolescente , Adulto , Péptidos Catiónicos Antimicrobianos/metabolismo , Infecciones Bacterianas/epidemiología , Infecciones Bacterianas/genética , Infecciones Bacterianas/inmunología , Niño , Preescolar , Salud de la Familia , Femenino , Citometría de Flujo , Predisposición Genética a la Enfermedad/epidemiología , Humanos , Masculino , Neutropenia/epidemiología , Neutrófilos/citología , Reacción en Cadena de la Polimerasa , Factores de Riesgo , Adulto Joven , CatelicidinasRESUMEN
A model of deafferentation pain is provided by sectioning the sciatic and saphenous nerves in the rat and mouse. This procedure leads to self-mutilation of the denervated hindpaw (autotomy). A noxious stimulus to the denervated area before neurectomy is known to enhance the autotomy. To understand the mechanism underlying this enhancement by prior noxious stimuli, we examined the effects of intrathecal (i.t.) injection of substance P (SP) and somatostatin (SOM) on autotomy behavior. These peptides are known to be released from primary afferent terminals in the dorsal horn by noxious stimuli. A single i.t. injection of SP or SOM just before neurectomy dramatically enhanced autotomy behavior in mice. Autotomy was enhanced in a dose-dependent manner with i.t. injection of SP (0.1-20 nmol) 5 min before neurectomy or SOM (0.1-1.0 nmol) 20 min before neurectomy. Autotomy significantly decreased by extending the interval between i.t. injection of SP or SOM and neurectomy. Intact mice injected with the same doses of SP or SOM showed dose-dependent acute nociceptive responses directed to the hindpaw. The severity of autotomy in neurectomized mice and the duration of acute nociceptive responses induced by the same doses of SP or SOM in intact mice were related. These results suggest that neuropeptides applied to the spinal dorsal horn just before deafferentation induce a state of central neural activation with long-lasting effects on the function of CNS cells. Augmentation of autotomy is a result of this activation which is kept as a 'memory'.