Exsanguinating bleeding following tooth extraction in a 12-year-old girl: a rare case of acquired haemophilia A.

Acquired haemophilia A (AHA) is a life-threatening haemorrhagic disorder that occurs with various underlying conditions such as autoimmune disease, drug reactions, lymphoproliferative diseases, solid tumours and pregnancy/postpartum status. However, in half of all reported cases, the underlying disease is unknown. Most AHA cases develop in adults; paediatric/adolescent cases are extremely rare. The main clinical symptom is bleeding into the skin, muscles, soft tissues and/or mucous membranes. Here, we report the case of an otherwise healthy 12-year-old girl who presented with prolonged bleeding postexodontia. After being diagnosed with AHA, she was successfully treated with recombinant activated factor VII infusion and oral prednisolone. To avoid such unanticipated bleeding when performing dental extraction, preoperative haemostatic screening tests are recommended.

Survey of severe respiratory syncytial virus infection in Kyoto Prefecture from 2003 to 2007.

BACKGROUND: Respiratory syncytial virus (RSV) infection in infants can develop into a severe condition. METHODS: A survey of patients with severe RSV infection in hospitals in Kyoto Prefecture was performed from 2003 to 2007. Patients requiring intubation and those with cardiopulmonary arrest on arrival (CPAOA) were considered to have severe RSV infection. RESULTS: Twenty-five patients with severe infection were identified and detailed data were available for 21 patients, of whom 18 required intubation and three had CPAOA. The male/female ratio was 12/9 and age ranged from 8 days to 19 years (average, 5.2 months; median: 2 months). At admission white blood cell count, lactate dehydrogenase (P < 0.05), and blood glucose (P < 0.01) were higher and Na was lower (P < 0.01) in the 18 patients with severe infection (excluding the CPAOA cases) compared to 18 sex- and age-matched patients with mild RSV infection. The incidence of bacterial infection was also higher in severe cases (P < 0.05). The outcome was death in four patients (19.0%, including two sudden deaths), aftereffects in two (9.5%), hospital discharge with improvement in 14 (66.7%), and an unclear outcome in one patient. Excluding the two sudden death cases, 14 of 19 patients (73.7%) were extubated within 2 weeks. The period of intubation was longer in older patients (P < 0.05). CONCLUSION: Because severe RSV infection led to sudden death in two cases, detection of RSV is important at admission for an infant with CPAOA. Fourteen patients (66.7%), however, had good outcomes and most patients were extubated within 2 weeks.

Neutropenia in the acute phase of Kawasaki disease and prevention of coronary artery aneurysm.

BACKGROUND: The significance of neutropenia in Kawasaki disease (KD) has not been fully elucidated as yet. METHODS: Subjects were retrospectively sampled from two clinical trials. These patients treated with aspirin alone (ASA) and PolyglobinN-Bayel (PolyN) given as i.v. immunoglobulin were categorized as ASA-early (n = 0), ASA-late (n = 8), PolyN-early (n = 18), or PolyN-late (n = 27) based on the therapy administered and the incidence of neutropenia before the 10th day of illness (DI) and after 11 DI. Data regarding the time of onset of neutropenia, and incidence of coronary artery lesion (CAL) formation were obtained. P < 0.05 was considered statistically significant. RESULTS: No patients in the ASA group exhibited neutropenia within 10 DI. The time of onset of neutropenia in the PolyN-early group was 8 +/- 1.3 DI. That in the PolyN-late group (19.8 +/- 8 DI) was earlier than in the ASA-late group (26.6 +/- 14 DI; P < 0.025). PolyN-early patients had a lower incidence of CAL formation than ASA-non patients (patients without neutropenia in the ASA group; P = 0.00019) and ASA-late patients (P = 0.04). That in the PolyN-early group tended to be lower than in the PolyN-late group (P < 0.1). CONCLUSION: Early neutropenia indicated that circulating neutrophils within 10 DI may play an indispensable role in the following sequence to CAL formation in KD.

Prevalence of coronary artery abnormality in incomplete Kawasaki disease.

BACKGROUND: The aim of the present study was to determine the prevalence of coronary artery abnormality (CAA) and other clinical features in patients with incomplete Kawasaki disease (iKD) using the data from the 17th Japanese nationwide survey of KD. METHODS: iKD was defined as the presence of four or fewer of the principal symptoms of the Japanese diagnostic guidelines, regardless of whether the patient had CAA. A total of 15,857 cases were analyzed. RESULTS: Among 15,857 cases, 83.9% of patients had five to six principal symptoms (complete KD: cKD), and 16.1% had iKD. The prevalence of CAA in cKD was 14.2%, and 18.4% in iKD. The prevalence of CAA in patients with four principal symptoms was 18.1%, which was higher than in cKD cases (14.2%). Although the reliability of the data has some limitations, the prevalence of CAA among patients with one to three symptoms was 19.3%. Among all CAA patients, 14% had four symptoms, and 6% had only one to three symptoms. CONCLUSION: Incomplete KD should not be equated with mild KD. Patients with four principal symptoms were comparable to cKD with respect to CAA occurrence. In patients with one to three symptoms also, especially in those under 1 year and older than 4 years of age, other significant symptoms and laboratory findings of the guidelines are very important in making a correct and early diagnosis of iKD so as to prevent CAA.

Cerebral thrombotic complications in adolescent leukemia/lymphoma patients treated with L-asparaginase-containing chemotherapy.

We described the cerebral thrombotic complications developed in 2 adolescent patients treated with L-asparaginase-containing regimens. For determining risk factors, we retrospectively analysed hemostatic markers in 19 pediatric patients with leukemia or lymphoma who were treated with either 1 of the 2 L-asparaginase-containing regimens; 11 were treated with VLP1 and the remaining 8 were treated with the VLAD protocol. The data indicated that low coagulation factors in association with increased plasma D-dimer levels during or post-L-asparaginase administration combined with fresh frozen plasma infusion might have activated coagulation processes in these patients. Careful management is required to prevent such episodes in patients with markedly decreased coagulation factors and increased D-dimer levels following L-asparaginase administration.

Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition).

Diagnostic guidelines for Kawasaki Disease was revised to meet the present situation in 2002. This issue intends to explain new guidelines and their backgrounds. Major alterations are interpretation of cases with 4 or fewer febrile days shortened by early intravenous immunoglobulin treatment, and the clinical importance of atypical (incomplete, or suspected) cases.

Long-term changes in coronary artery aneurysms in patients with Kawasaki disease: comparison of therapeutic regimens.

BACKGROUND: There are few studies of the therapeutic regimens for the prevention of stenotic transformation of aneurysms in Kawasaki disease (KD). The aim of this study was to assess the prophylactic effect of combined therapy in the acute stage and convalescent- to chronic-stage against the formation of stenotic lesions. METHODS AND RESULTS: In 85 patients, 103 giant aneurysms (ANl), 46 medium-sized aneurysms (ANm), and 13 small aneurysms (ANs) were analyzed. With respect to therapy in the acute stage, no localized stenosis of ANl in the left coronary artery was noted in patients who received high-dose gamma globulin therapy (G). For ANm, the group (G) showed a significantly higher regression rate than the aspirin group and steroids group. Furthermore, no coronary artery occlusion/recanalization of ANl occurred with the prophylactic regimen of aspirin and warfarin {aw}. Prophylaxis {aw} and the prophylactic regimen of aspirin alone {a} significantly lowered the incidence compared with either the prophylactic regimen of warfarin {w} or no prophylaxis {n}. However, no significant differences were noted between prophylaxis {w} and {n}. CONCLUSIONS: High-dose gamma globulin therapy in the acute stage of KD is the first choice for the prevention of stenotic transformation. Prophylaxis {aw} is recommended for ANl.