RESUMEN
NCs occur commonly after solid organ transplantation and affect 15%-30% of liver transplant recipients. The aim of this retrospective study was to evaluate the type and incidence of neurologic events in pediatric patients following LT. Between May 2006 and June 2015, 242 patients (118 females, 124 males) requiring LT for different etiologies at the Inönü University Liver Transplantation Institute were included. The incidence, types, and risk factors of NCs that occurred following LT were evaluated retrospectively. Neurologic events occurred in 57 (23.5%) of the patients. Early NCs were encephalopathy (12.4%), seizures (11.5%), and PRES (7%). Of 57 patients, five (8.7%) experienced NCs at least 1 month after LT; these late NCs included tremor, headaches, encephalopathy, ataxia, and neuropathy. The psychiatric symptoms after LT were noted in 42 patients (17.4%). The mortality rate after LT in those with or without neurological events was not significantly different (P=.73). There was a high incidence of serious neurologic events after LT. The major neurologic manifestation in our patients was encephalopathy followed by seizures.
Asunto(s)
Enfermedad Hepática en Estado Terminal/cirugía , Trasplante de Hígado/efectos adversos , Enfermedades del Sistema Nervioso/etiología , Adolescente , Encefalopatías/etiología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Inmunosupresores/uso terapéutico , Incidencia , Lactante , Masculino , Enfermedades del Sistema Nervioso/epidemiología , Estudios Retrospectivos , Factores de Riesgo , Convulsiones/etiologíaAsunto(s)
Degeneración Hepatolenticular/psicología , Automutilación/diagnóstico , Conducta Autodestructiva/diagnóstico , Adolescente , Antidepresivos de Segunda Generación/uso terapéutico , Antipsicóticos/administración & dosificación , Encéfalo/patología , Citalopram/administración & dosificación , Manual Diagnóstico y Estadístico de los Trastornos Mentales , Trastornos Disruptivos, del Control de Impulso y de la Conducta/diagnóstico , Trastornos Disruptivos, del Control de Impulso y de la Conducta/tratamiento farmacológico , Trastornos Disruptivos, del Control de Impulso y de la Conducta/psicología , Quimioterapia Combinada , Femenino , Haloperidol/administración & dosificación , Degeneración Hepatolenticular/diagnóstico , Humanos , Imagen por Resonancia Magnética , Automutilación/tratamiento farmacológico , Automutilación/psicología , Conducta Autodestructiva/tratamiento farmacológico , Conducta Autodestructiva/psicologíaRESUMEN
OBJECTIVE: Celiac disease (CD) is a lifelong gluten-sensitive intestinal enteropathy that is multifactorial in its etiology. In the present study, we evaluated basic anthropometric, clinical, laboratory, and histological features of 140 Turkish children with CD. We particularly underscored the association of CD with other autoimmune diseases. MATERIALS AND METHODS: During the period from 1999 to 2005, CD was diagnosed in 140 children according to ESPGAN criteria. The age, gender, clinical findings, hematological, and biochemical parameters at diagnosis were noted. Symptoms and signs were recorded. Endoscopic intestinal biopsies were taken from all children. RESULTS: Of the 140 children with CD, 75 (53.6%) were female, and 65 (46.4%) were male. Mean age was 8.56 ± 4.43 years (range 13 months to 18 years). The most frequent symptom was failure to thrive (81.4%), followed by chronic diarrhea (60%). Of the children with CD, nine (6.4%) had type 1 diabetes mellitus (DM), six (4.3%) had familial Mediterranean fever, three (2.1%) had alopecia areata, three (2.1%) had vitiligo, three (2.1%) had Down syndrome, two (1.4%) had lung tuberculosis, two (1.4 %) had autoimmune hepatitis, two (1.4%) had growth hormone deficiency, one (0.7%) had osteogenesis imperfecta, and one (0.7%) had Floating Harbor Syndrome. Elevated serum levels of ALT, CK and AST were detected in 48(34.8%), 50 (38.2%) and 67 (48.6%) children, respectively. CONCLUSION: The spectrum of clinical findings is very wide. In order to avoid overlooking CD in patients with extra intestinal symptoms and signs, physicians, especially pediatricians, should be informed about new atypical manifestations of CD.
RESUMEN
BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of inflammation of serosal membranes. Amyloidosis is the most severe complication of the disease The aim of this study was to explore the magnitude of the FMF problem and to describe clinical phenotypic and genotypic profile in the childhood population in Eastern Turkey. METHODS: In this study, 52 patients who were diagnosed as FMF between January 2000 and January 2003 in Department of Pediatrics, Ataturk University Hospital, were included. The diagnosis of FMF was based on typical clinical and laboratory features. The 12 FMF mutations were investigated in the patients. RESULTS: Of the 52 patients, 30 (57.7%) were girls, 22 (42.3%) were boys, and the age ranged from 9 months to 15 years (8.5 +/- 3.2 years). A positive family history for FMF was noted in 33 (63.5%) patients. The mean onset age was 6 +/- 3.4 (from 8 months to 14 years). Nineteen children (36.5%) were symptomatic below the age of 5 years. Abdominal pain was observed in 50 (96.2%), fever in 42 (80.8%), arthralgia in 29 (55.8%), arthritis in 18 (34.6%), splenomegaly in 11 (21.2), hepatomegaly in 15 (28.8%), myalgia in 11 (26.2%), erysipelas-like erythema in 10 (19.2%), thoracic pain in four (7.7%), protracted febrile myalgia in three (5.8%), and seizures in two (3.8%). The most frequent mutation was the M694V/M694V. Clinical presentation of the patients was not different in respect with genotypes (P > 0.05). Two patients had chronic renal disease suggestive of amyloidosis. CONCLUSION: It was noted that the FMF patients in this study had a broad spectrum of mutation combination, which might reflect the intercultural interactions of ancient ethnic groups that lived in Anatolia, and these mutations were not significantly different in respect to clinical presentations.