Background and ObjectivesThis study aimed to investigate the clinical characteristics of infantile Kawasaki disease (KD), and to evaluate early diagnostic features of KD in febrile infants.Subjects and MethodsWe retrospectively reviewed the medical records of 64 KD patients from January 2010 to October 2014. There was an analysis of the clinical, laboratory data of the infants versus children groups. Furthermore, the clinical and laboratory data of infantile KD patients were compared with 16 infants who were admitted for other acute febrile diseases.ResultsA total of 64 patients with KD were identified; 20 (31.3%) were infants; 44 (68.8%) were >1 year old children. Incomplete KD was much more common in infants (n=13, 65.0%) than in children group (n=14, 31.8%) (p=0.013). The infants were characterized by significantly higher rates of inflammatory changes at the Bacille Calmett-Guérin (BCG) inoculation site (p<0.001), but lower rates of changes in the extremities (p=0.029) and cervical lymphadenopathy (p=0.006). The serum levels of platelet after 1 week (p=0.005), C-reactive protein (p=0.038), and N-terminal pro-brain natriuretic peptide (NT-proBNP) (p=0.026) were all significantly higher in the infants group. Comparing the infants with KD versus the other acute febrile diseases, there were significantly higher serum levels of erythrocyte sedimentation rate (p=0.002), C-reactive protein (p=0.046) and NT-proBNP (p=0.001) for the infants with KD group.ConclusionBCGitis and higher levels of NT-proBNP can be helpful for early diagnosis of the incomplete KD in infants, and may be a good predictor of KD in acute febrile infants, when combined with other acute phase reactants.
Small bowel obstruction due to congenital band is not only rare in children, but also difficult to diagnose, because common symptoms such as vomiting and abdominal pain are observed in patients. In order to prevent a fatal result, an anomalous congenital band should be considered in the discriminative diagnosis of intestinal obstruction in children who have no previous experience of operation or intraperitoneal inflammation. This report presents a 4-year-old boy who was admitted with abdominal pain and nonbilious vomiting for a day. The initial suspicion was for acute gastroenteritis. However, after further investigation and performance of surgery, the intestinal obstruction associated with a congenital band was confirmed. No recurrence was observed during the 8-month follow-up period. Thus early confirmation based on radiologic study is a crucial factor for the diagnosis of small bowel obstruction caused by a congenital band. (Pediatr Gastroenterol Hepatol Nutr 2012; 15: 188∼192)
Background and Objective: This study was aimed to investigate clinical characteristics of infantile Kawasaki disease (KD), and to evaluate early diagnostic features of KD in febrile infants. Subjects and Methods: We retrospectively reviewed the medical records of 66 patients with KD who were admitted to Dongguk University Gyeongju Hospital, between January 2010 and August 2014. The clinical, laboratory data between infants and children were analyzed. And the clinical and laboratory data of infantile KD patients were compared with 18 infants who were admitted for other acute febrile disease during same period as above. Results: A total of 66 patients were identified; 21(31.8%) were infants; 45(68.2%) were > 1 year old children. Incomplete KD was much more common in infants (n=15, 71.4%) than in children group (n=14, 31.1%) (P = 0.002). The infants group was characterized by significantly higher rates of inflammatory changes at the Bacille Calmett-Guerin (BCG) inoculation site (P<0.001), but lower rates of changes in the cervical lymphadenopathy (P=0.005), conjunctival injection (P=0.047). The serum levels of hemoglobin were lower (P<0.001), and platelet (P=0.029), C-reactive protein (P=0.042), N-terminal pro-brain natriuretic peptide (NT-proBNP) (P=0.042) was higher in the infants group significantly. Pyuria was also significantly higher in the infants group (P=0.017). Between infants with KD and with other acute febrile diseases, there were significantly higher serum levels of ESR (P<0.001), CRP (P=0.009) and NT-proBNP (P=0.002) in the infantile KD. Conclusion: Because incomplete KD was much more in infants, the diagnosis of infantile KD can be difficult. The BCGitis and higher level of NT-proBNP can help to early diagnosis of incomplete KD in infants, and may be a good predictor of KD in acute febrile infants combined with other acute phase reactants.
Genetic Medicine can be divided into four groups based on the onset age of the patients: neonatal, infantile, juvenile, or adult. The early-onset subtypes are more severe, rapidly progressive, and have a shorter life span than the late-onset subtypes. Most cases of ALXD occur in infancy and the symptoms are grave and eventually fatal. 5) With the advance of DNA analysis technology, genetic diagnoses for ALXD are available in the clinical field. Magnetic resonance imaging (MRI) and genetic tests are mostly used for diagnostic purposes. Even though therapeutic strategies have been investigated and suggested, there are no specific clinically available treatments. This review will discuss the cause, pathology, pathophysiology, inheritance, clinical manifestations, diagnosis, and treatment of ALXD.
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