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1.
J Pediatr ; 237: 34-40.e1, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34197890

RESUMEN

OBJECTIVE: To analyze the results of an enhanced laboratory-surveillance protocol for bloody diarrhea aimed at identifying children with Shiga toxin-producing Escherichia coli (STEC) infection early in the course of the disease toward the early identification and management of patients with hemolytic uremic syndrome (HUS). STUDY DESIGN: The study (2010-2019) involved a referral population of 2.3 million children. Stool samples of patients with bloody diarrhea were screened for Shiga toxin (Stx) genes. Positive patients were rehydrated and monitored for hemoglobinuria until diarrhea resolved or STEC-HUS was diagnosed. RESULTS: A total of 4767 children were screened; 214 (4.5%) were positive for either Stx1 (29.0%) or Stx2 (45.3%) or both Stx1+2 (25.7%); 34 patients (15.9%) developed STEC-HUS (0.71% of bloody diarrheas). Hemoglobinuria was present in all patients with HUS. Patients with Stx2 alone showed a greater risk of STEC-HUS (23.7% vs 12.7%) and none of the patients with Stx1 alone developed HUS. During the same period of time, 95 other patients were diagnosed STEC-HUS but were not captured by the screening program (26 had nonbloody diarrhea, 11 came from areas not covered by the screening program, and 58 had not been referred to the screening program, although they did meet the inclusion criteria). At HUS presentation, serum creatinine of patients identified by screening was significantly lower compared with that of the remaining patients (median 0.9 vs 1.51 mg/dL). CONCLUSIONS: Nearly 1% of children with bloody diarrhea developed STEC-HUS, and its diagnosis was anticipated by the screening program for Stx. The screening of bloody diarrhea for Stx is recommended, and monitoring patients carrying Stx2 with urine dipstick for hemoglobinuria is suggested to identify the renal complication as early as possible.


Asunto(s)
Diarrea/microbiología , Infecciones por Escherichia coli/diagnóstico , Hemorragia Gastrointestinal/microbiología , Síndrome Hemolítico-Urémico/microbiología , Tamizaje Masivo/métodos , Escherichia coli Shiga-Toxigénica/aislamiento & purificación , Adolescente , Niño , Preescolar , Diagnóstico Precoz , Infecciones por Escherichia coli/complicaciones , Femenino , Hemorragia Gastrointestinal/diagnóstico , Genes Bacterianos , Síndrome Hemolítico-Urémico/diagnóstico , Síndrome Hemolítico-Urémico/epidemiología , Síndrome Hemolítico-Urémico/terapia , Humanos , Lactante , Recién Nacido , Italia , Masculino , Toxinas Shiga/genética , Escherichia coli Shiga-Toxigénica/genética , Resultado del Tratamiento , Adulto Joven
2.
Pediatr Nephrol ; 35(10): 1997-2001, 2020 10.
Artículo en Inglés | MEDLINE | ID: mdl-32734345

RESUMEN

BACKGROUND: Shigatoxin (Stx)-producing Escherichia coli (STEC) are the most common causes of hemolytic uremic syndrome (STEC-HUS). The aim of our study is to compare the risk of developing STEC-HUS in relation to the type of Stx genes (Stx1, Stx2, or both). METHODS: This is a prospective, observational, multicenter study involving 63 pediatric units in Northern Italy (ItalKid-HUS Network). STEC-infected children were identified within a screening program for bloody diarrhea during a 10-year period (2010-2019). Stx genes were detected by reverse dot blot or real-time PCR. After the identification of STEC infection, children were followed until diarrhea complete recovery for the possible development of STEC-HUS. RESULTS: Of the 214 Stx-positive patients, 34 (15.9%) developed STEC-HUS. The risk of HUS in STEC-infected children with Stx1 (n: 62; 29.0%) and Stx2 (n: 97; 45.3%) was respectively 0% and 23.7%, while in patients carrying both Stx1 and Stx2 (n: 55; 25.7%), the risk was 12.7% (p: 0.001). CONCLUSIONS: Our data confirm that Stx1 is a very rare cause of STEC-HUS and demonstrate that the risk of STEC-HUS halves in the case of Stx1+2-producing Escherichia coli infection compared with infections where Stx2 is present alone. This observation is helpful in assessing the risk of individual STEC-infected patients for the development of HUS and suggests that Stx1, in the presence of Stx2, might exert a protective role possibly by receptor competition.


Asunto(s)
Infecciones por Escherichia coli/microbiología , Síndrome Hemolítico-Urémico/epidemiología , Toxina Shiga I/toxicidad , Toxina Shiga II/toxicidad , Escherichia coli Shiga-Toxigénica/genética , Niño , Preescolar , Infecciones por Escherichia coli/complicaciones , Femenino , Síndrome Hemolítico-Urémico/microbiología , Humanos , Lactante , Tipificación Molecular , Estudios Prospectivos , Factores Protectores , Medición de Riesgo , Toxina Shiga I/genética , Toxina Shiga I/aislamiento & purificación , Toxina Shiga II/genética , Toxina Shiga II/aislamiento & purificación , Escherichia coli Shiga-Toxigénica/aislamiento & purificación
3.
Pediatr Nephrol ; 33(3): 457-461, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-29046944

RESUMEN

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by platelet consumption, hemolysis, and organ damage. Eculizumab (ECU), a humanized antibody that blocks complement activity, has been successfully used in aHUS, but the best treatment schedule is not yet clear. METHODS: Here, we report our experience with ECU maintenance treatment and the interval between subsequent doses being extended based on global classical complement pathway (CCP) activity aimed at <30% for maintaining aHUS into remission. RESULTS: We report on 38 patients with aHUS, 13 children, 21 female, with a median age of 25.0 years (range 0.5-60) at disease onset treated with ECU standard schedule for a median of 2.6 months (range 0.4-24.6). Once stable TMA remission was obtained, the interval between ECU doses was extended based on complement function, with a target CCP activity of <30%. With this approach, 22 patients regularly receive ECU infusion every 28 days and 16 every 21. During a median observation period on ECU, an extended interval of 26.9 months (range 0.8-80.9), with a cumulative observation period of 1,208 months, none of the patients relapsed. CONCLUSION: Monitoring complement activity allows a safe reduction in the frequency of ECU administration in aHUS while keeping the disease in remission.


Asunto(s)
Anticuerpos Monoclonales Humanizados/uso terapéutico , Síndrome Hemolítico Urémico Atípico/tratamiento farmacológico , Inactivadores del Complemento/uso terapéutico , Proteínas del Sistema Complemento/efectos de los fármacos , Monitoreo de Drogas/métodos , Adolescente , Adulto , Niño , Preescolar , Proteínas del Sistema Complemento/análisis , Femenino , Humanos , Lactante , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto Joven
4.
Eur J Pediatr ; 177(9): 1383-1388, 2018 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-29387982

RESUMEN

It is well known that the nephron endowment of healthy subjects is highly variable and that individual nephron mass has potentially important implications both in health and disease. However, nephron count is technically impossible in living subjects. Based on the observation of an increase in serum creatinine (sCr) in otherwise healthy newborns with solitary kidney during the physiological perinatal dehydration, we hypothesized that perinatal sCr might be helpful in identifying healthy subjects with a reduced nephron mass. In the framework of a study on blood pressure in babies (NeoNeph), sCr of normal Caucasian neonates was determined 48-96 h after birth and their association with a family history of arterial hypertension (AH) was analyzed. SCr was determined in 182 normal newborns (90 males) at a mean of 61 ± 8 h after birth (range 46-82). Newborns with paternal AH had a higher mean sCr (0.97 + 0.28 mg/dL) then newborns without paternal AH (0.73 + 0.28 mg/dL; p = 0.006). No differences in mean sCr were found in relation with mother or grandparent's history of AH. CONCLUSION: The association between parental AH and high sCr during perinatal dehydration supports the hypothesis that the latter is a promising tool for identifying normal subjects with a reduced nephron mass with potential important implications in prevention and in understanding the individual outcome of renal and extrarenal diseases (including AH). What is Known: • Nephron endowment of healthy subjects is highly variable and individual nephron mass has potentially important implications both in health and disease however nephron count is not feasible in living subjects. What is New: • Serum creatinine during perinatal dehydration is a possible biomarker for identifying normal subjects with a reduced nephron mass.


Asunto(s)
Creatinina/sangre , Deshidratación/sangre , Hipertensión/complicaciones , Nefronas/fisiopatología , Biomarcadores/sangre , Deshidratación/complicaciones , Femenino , Humanos , Recién Nacido , Masculino , Embarazo
5.
Eur J Pediatr ; 175(4): 465-73, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26498648

RESUMEN

UNLABELLED: Despite the severity of HUS and the fact that it represents a leading cause of acute kidney injury in children, the general epidemiology of HUS is all but well documented. The present study provides updated, population-based, purely epidemiological information on HUS in childhood from a large and densely populated area of northern Italy (9.6 million inhabitants, 1.6 million children). We systematically reviewed the files concerning patients with STEC-HUS and atypical HUS (aHUS) over a 10-year observation period (January 2003-December 2012). We included all incident cases with a documented first episode of HUS before the age of 18 years. We identified 101 cases of HUS during the 10 years. The overall mean annual incidence was 6.3 cases/million children aged <18 years (range 1.9-11.9), and 15.7/million of age-related population (MARP) among subjects aged <5 years; aHUS accounted for 11.9 % of the cases (mean incidence 0.75/MARP). The overall case fatality rate was 4.0 % (3.4 % STEC-HUS, 8.3 % aHUS). CONCLUSION: Given the public health impact of HUS, this study provides recent, population-based epidemiological data useful for healthcare planning and particularly for estimating the financial burden that healthcare providers might have to face in treating HUS, whose incidence rate seems to increase in Northern Italy. WHAT IS KNOWN: • HUS is a rare disease, but it represents the leading cause of acute kidney injury in children worldwide. • STEC-HUS (also called typical, D + HUS) is more common compared to atypical HUS, but recent, population-based epidemiological data (incidence) are scanty. What is New: • Comprehensive, population-based epidemiological data concerning both typical and atypical HUS based on a long observational period.


Asunto(s)
Infecciones por Escherichia coli/epidemiología , Escherichia coli/aislamiento & purificación , Síndrome Hemolítico-Urémico/epidemiología , Adolescente , Niño , Preescolar , Infecciones por Escherichia coli/complicaciones , Síndrome Hemolítico-Urémico/microbiología , Humanos , Incidencia , Lactante , Italia/epidemiología
6.
Pediatr Nephrol ; 30(2): 345-52, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25149851

RESUMEN

BACKGROUND: Shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) is a common thrombotic microangiopathy (TMA) in which central nervous system (CNS) involvement is responsible for the majority of deaths and for severe long-term sequelae. We have analyzed the role of hemoconcentration in disease severity. METHODS: This was a retrospective review of the records and laboratory data at presentation of all patients with STEC-HUS cases (n = 61) over a 10-year period. The patients were grouped into three severity classes: group A, comprising patients who did not require dialysis; group B, patients who were dialyzed without CNS involvement; group C, patients with CNS involvement. RESULTS: Patients with CNS involvement (group C) had a higher mean hemoglobin level (11.2 ± 2.3 g/dL) than those of group A or B ( 9.4 ± 2.1 and 7.5 ± 1.9 g/dL, respectively; p < 0.0001). We also observed that the higher the initial hemoglobin level, the more severe the long-term renal damage (p < 0.007). CONCLUSIONS: In patients with STEC-HUS, hemoconcentration and hypovolemia may be responsible for more severe ischemic organ damage (both short and long term) at disease onset, and these signs should be regarded as risk factors for CNS damage and for more severe TMA. Therefore, we recommend that hydration status should be actively monitored in HUS patients and that dehydration, when diagnosed, should be promptly corrected.


Asunto(s)
Síndrome Hemolítico-Urémico/sangre , Síndrome Hemolítico-Urémico/complicaciones , Enfermedades del Sistema Nervioso/etiología , Adolescente , Niño , Preescolar , Femenino , Fluidoterapia/efectos adversos , Hematócrito , Humanos , Lactante , Masculino , Estudios Retrospectivos , Factores de Riesgo
7.
Am J Kidney Dis ; 64(4): 633-7, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-24656451

RESUMEN

Atypical hemolytic uremic syndrome (aHUS) is a life-threatening thrombotic microangiopathy, and as many as 70% of patients with aHUS have mutations in the genes encoding complement regulatory proteins. Eculizumab, a humanized recombinant monoclonal antibody targeting C5, has been used successfully in patients with aHUS since 2009. The standard maintenance treatment requires life-long eculizumab therapy, but the possibility of discontinuation has not yet been tested systematically. We report the safety of discontinuing eculizumab treatment in 10 patients who stopped treatment with the aim of minimizing the risk of adverse reactions, reducing the risk of meningitis, and improving quality of life while also reducing the considerable treatment costs. Disease activity was monitored closely at home by means of urine dipstick testing for hemoglobin. During the cumulative observation period of 95 months, 3 of the 10 patients experienced relapse within 6 weeks of discontinuation, but then immediately resumed treatment and completely recovered. Our experience supports the possibility of discontinuing eculizumab therapy with strict home monitoring for early signs of relapse in patients with aHUS who achieve stable remission.


Asunto(s)
Anticuerpos Monoclonales Humanizados , Síndrome Hemolítico Urémico Atípico , Costo de Enfermedad , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/prevención & control , Hemoglobinuria , Calidad de Vida , Privación de Tratamiento , Adolescente , Adulto , Anticuerpos Monoclonales Humanizados/administración & dosificación , Anticuerpos Monoclonales Humanizados/efectos adversos , Anticuerpos Monoclonales Humanizados/economía , Síndrome Hemolítico Urémico Atípico/diagnóstico , Síndrome Hemolítico Urémico Atípico/tratamiento farmacológico , Síndrome Hemolítico Urémico Atípico/economía , Síndrome Hemolítico Urémico Atípico/fisiopatología , Síndrome Hemolítico Urémico Atípico/psicología , Niño , Preescolar , Inactivadores del Complemento/administración & dosificación , Inactivadores del Complemento/efectos adversos , Inactivadores del Complemento/economía , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/clasificación , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/etiología , Femenino , Hemoglobinuria/diagnóstico , Hemoglobinuria/etiología , Humanos , Lactante , Masculino , Persona de Mediana Edad , Monitoreo Fisiológico/métodos , Recurrencia , Inducción de Remisión , Resultado del Tratamiento , Urinálisis/métodos
8.
J Pediatr Gastroenterol Nutr ; 59(2): 218-20, 2014 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-24824362

RESUMEN

Hemolytic-uremic syndrome (HUS) is an important cause of acute kidney injury in children often caused by Shiga toxin-producing Escherichia coli (STEC) enterocolitis. In a screening program for STEC infection in children with bloody diarrhea in northern Italy for early diagnosis of HUS, co-infection with Salmonella or Campylobacter was documented in as many as 35.6% of Shiga toxin-positive patients. It is speculated that infection by Salmonella or Campylobacter may increase the risk of STEC enterocolitis and therefore of HUS. The isolation of microorganisms (other then STEC) in HUS should not be necessarily regarded as the etiological agent for the thrombotic microangiopathy.


Asunto(s)
Campylobacter , Coinfección/microbiología , Diarrea/microbiología , Enterocolitis/microbiología , Síndrome Hemolítico-Urémico/microbiología , Salmonella , Escherichia coli Shiga-Toxigénica , Adolescente , Niño , Preescolar , Diarrea/etiología , Enterocolitis/complicaciones , Femenino , Síndrome Hemolítico-Urémico/diagnóstico , Síndrome Hemolítico-Urémico/etiología , Humanos , Lactante , Italia , Masculino , Tamizaje Masivo , Toxina Shiga
9.
Artículo en Inglés | MEDLINE | ID: mdl-36534066

RESUMEN

BACKGROUND: Urinary tract infections (UTIs) are a frequent disorder of childhood, mainly caused by Escherichia coli. The aim of this study was to evaluate the antimicrobial susceptibility trend in bacterial isolates in urine cultures in pediatric environment, analyzing data from our laboratory in a 6-year period. METHODS: A retrospective study was performed in AO SS. Antonio e Biagio e Cesare Arrigo (Pediatric Hospital) of Alessandria in Piedmont, North Ital. From 2015 to 2020, in a 6-year period, 1299 urinocultures were collected. Data collection was focused on demographic characteristics (age and sex) and laboratory findings (positive urocultures, antibiogram). RESULTS: Positive urocultures were 577, in which Escherichia coli represented most isolates (428, 74.2%). We found a statistically significant trend toward amoxiclavulanate resistance in the E. coli positive urinoculture comparing the period 2015-2018 vs. 2019-2020. CONCLUSIONS: Actual guidelines mostly recommend for amoxicillin-clavulanate prescription as first-line option for pediatric UTI management, this indication might be partially reconsidered. Our data underline the importance to conduct surveillance studies to determine local prevalence of antibiotic resistance to optimize therapeutic management.

12.
J Pediatr Gastroenterol Nutr ; 45(4): 433-7, 2007 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-18030209

RESUMEN

OBJECTIVE: To evaluate the growth response to growth hormone (GH) replacement therapy during a gluten-free diet in patients with celiac disease (CD) associated with GH deficiency (GHD). PATIENTS AND METHODS: A total of 14 prepubertal children affected by CD and GHD with no catch-up growth after >/=12 months of gluten-free diet and a reversion to seronegativity for antiendomysium antibodies and 10 age-matched prepubertal children with idiopathic GHD (IGHD) entered the study. All of the patients were treated with the same GH dosage (0.25 mg/kg subcutaneously each week). Height, growth rate, and body mass index were measured at the time of diagnosis of CD, at the time of endocrinological evaluation, and after the first, second, and third year of GH replacement therapy. RESULTS: Growth rate strikingly increased (P < 0.005) during the first year of therapy in a similar way in subjects with CD/GHD and IGHD (from a median standard deviation score [SDS] of -2.34 to an SDS of 3.25 and from an SDS of -1.29 to an SDS of 2.79, respectively). During the second and third years of GH treatment, the growth rate tended to decrease but the values at the third year were always positive (CD/GHD, median SDS, 1.10; IGHD, median SDS, 0.11), indicating continued catch-up growth. CONCLUSIONS: In patients with CD with GH deficiency confirmed after >/=12 months of gluten-free diet, GH replacement therapy should be started to allow complete catch-up growth in children. In addition, the effect of GH treatment in patients who comply with a gluten-free diet seems to be comparable to that observed in children with IGHD.


Asunto(s)
Enfermedad Celíaca/complicaciones , Trastornos del Crecimiento/complicaciones , Trastornos del Crecimiento/tratamiento farmacológico , Hormona de Crecimiento Humana/deficiencia , Hormona de Crecimiento Humana/uso terapéutico , Estatura/efectos de los fármacos , Índice de Masa Corporal , Enfermedad Celíaca/dietoterapia , Enfermedad Celíaca/fisiopatología , Niño , Desarrollo Infantil/efectos de los fármacos , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Masculino , Resultado del Tratamiento
13.
Case Rep Pediatr ; 2017: 2794060, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28835862

RESUMEN

Hemolytic uremic syndrome (HUS) is an unrare and severe thrombotic microangiopathy (TMA) caused by several pathogenetic mechanisms among which Shiga toxin-producing Escherichia coli infections and complement dysregulation are the most common. However, very rarely and particularly in neonates and infants, disorders of cobalamin metabolism (CblC) can present with or be complicated by TMA. Herein we describe a case of atypical HUS (aHUS) related to CblC disease which first presented in a previously healthy boy at age of 13.6 years. The clinical picture was initially dominated by nephrotic range proteinuria and severe hypertension followed by renal failure. The specific treatment with high dose of hydroxycobalamin rapidly obtained the remission of TMA and the complete recovery of renal function. We conclude that plasma homocysteine and methionine determinations together with urine organic acid analysis should be included in the diagnostic work-up of any patient with TMA and/or nephrotic syndrome regardless of age.

14.
Pediatrics ; 137(1)2016 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-26644486

RESUMEN

BACKGROUND: Hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS) is a severe acute illness without specific treatment except supportive care; fluid management is concentrated on preventing fluid overload for patients, who are often oligoanuric. Hemoconcentration at onset is associated with more severe disease, but the benefits of volume expansion after hemolytic uremic syndrome (HUS) onset have not been explored. METHODS: All the children with STEC-HUS referred to our center between 2012 and 2014 received intravenous infusion targeted at inducing an early volume expansion (+10% of working weight) to restore circulating volume and reduce ischemic or hypoxic tissue damage. The short- and long-term outcomes of these patients were compared with those of 38 historical patients referred to our center during the years immediately before, when fluid intake was routinely restricted. RESULTS: Patients undergoing fluid infusion soon after diagnosis showed a mean increase in body weight of 12.5% (vs 0%), had significantly better short-term outcomes with a lower rate of central nervous system involvement (7.9% vs 23.7%, P = .06), had less need for renal replacement therapy (26.3% vs 57.9%, P = .01) or intensive care support (2.0 vs. 8.5 days, P = .02), and needed fewer days of hospitalization (9.0 vs 12.0 days, P = .03). Long-term outcomes were also significantly better in terms of renal and extrarenal sequelae (13.2% vs 39.5%, P = .01). CONCLUSIONS: Patients with STEC-HUS had great benefit from early volume expansion. It is speculated that early and generous fluid infusions can reduce thrombus formation and ischemic organ damage, thus having positive effects on both short- and long-term disease outcomes.


Asunto(s)
Fluidoterapia , Síndrome Hemolítico-Urémico/terapia , Niño , Preescolar , Intervención Médica Temprana , Femenino , Síndrome Hemolítico-Urémico/microbiología , Humanos , Lactante , Masculino , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Escherichia coli Shiga-Toxigénica , Resultado del Tratamiento
15.
Pediatrics ; 133(6): e1769-71, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24843058

RESUMEN

Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease often related to uncontrolled complement activation. The use of eculizumab has changed the management and the outcome of aHUS, becoming the frontline treatment of the acute disease and for the prevention of relapses. We report the case of a male patient with aHUS due to complement factor H gene mutation who was shifted from plasmatherapy to eculizumab for preventing disease relapses. The shift to eculizumab was associated with a significant decrease in proteinuria, revealing disease activity otherwise unsuspected, being the classic criteria of disease activity (platelet, haptoglobin, LDH, schistocytes), all in the normal range.The condition of proteinuria as the only sign of thrombotic microangiopathy activity is here designated as "cryptic activity of aHUS."


Asunto(s)
Anticuerpos Monoclonales Humanizados/uso terapéutico , Factor H de Complemento/genética , Factor H de Complemento/inmunología , Síndrome Hemolítico-Urémico/diagnóstico , Síndrome Hemolítico-Urémico/tratamiento farmacológico , Proteinuria/diagnóstico , Proteinuria/tratamiento farmacológico , Anticuerpos Monoclonales Humanizados/efectos adversos , Biomarcadores/sangre , Niño , Preescolar , Terapia Combinada , Activación de Complemento/efectos de los fármacos , Análisis Mutacional de ADN , Diagnóstico Diferencial , Haptoglobinas/metabolismo , Síndrome Hemolítico-Urémico/genética , Síndrome Hemolítico-Urémico/inmunología , Humanos , Lactante , Pruebas de Función Renal , L-Lactato Deshidrogenasa/sangre , Estudios Longitudinales , Masculino , Plasma , Plasmaféresis , Recuento de Plaquetas , Proteinuria/genética , Proteinuria/inmunología , Púrpura Trombocitopénica Trombótica/diagnóstico , Púrpura Trombocitopénica Trombótica/tratamiento farmacológico , Púrpura Trombocitopénica Trombótica/genética , Púrpura Trombocitopénica Trombótica/inmunología , Prevención Secundaria
16.
Pediatr Neurol ; 46(5): 319-21, 2012 May.
Artículo en Inglés | MEDLINE | ID: mdl-22520354

RESUMEN

Kikuchi-Fujimoto disease is a necrotizing lymphadenitis, mainly characterized by lymphadenopathy, fever, hepatosplenomegaly, nocturnal sweats, myalgia, weight loss, and arthralgia. Its diagnosis is most often based on lymph node biopsy. Differential diagnoses with several other diseases, e.g., malignant lymphoma, necrotizing lymphadenitis, and infective lymphadenopathies, may be challenging. Neurologic involvement is rarely reported in patients diagnosed with Kikuchi-Fujimoto disease. In this subset of patients, the great majority manifest signs involving the central nervous system. We present a 14-year-old boy with a severe form of Kikuchi-Fujimoto disease, complicated by peripheral neuropathy. This patient is interesting for both his age and his peculiar complication.


Asunto(s)
Linfadenitis Necrotizante Histiocítica/etiología , Enfermedades del Sistema Nervioso Periférico/complicaciones , Adolescente , Linfadenitis Necrotizante Histiocítica/diagnóstico , Humanos , Masculino , Conducción Nerviosa/fisiología
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