Incidence of childhood primary immune thrombocytopenic purpura.

There is a discrepancy in the reported incidence of childhood immune thrombocytopenic purpura (ITP) between Europe (2.9-5.3 per 100 000 persons) and Japan (1.91). Ise district is a suitable area in which to conduct epidemiological study because there is little fluctuation in the sociodemographic factors. We performed a retrospective population-based study to clarify the incidence of primary childhood ITP. We calculated person-years for children aged <15 years based on the Ise district demographics between 2002 and 2012. The calculated person-years were 298 533. The number of hospitalized patients in Ise district was 25 (M/F, 14/11) during the study period. The calculated incidence was therefore 8.4 per 100 000 person-years. It is possible that the difference in incidence between the present calculation and that of the European studies is due to variation in accuracy and/or registration criteria between the studies.

Development of acute pancreatitis after Nissen fundoplication.

Acute pancreatitis subsequent to Nissen fundoplication for gastroesophageal reflux disease (GERD) is an extremely rare adverse event. We describe a pediatric case of acute pancreatitis resulting from superior mesenteric artery syndrome (SMAS) and gas bloat after fundoplication. Gas bloat is one of the known postoperative complications after Nissen fundoplication. Poor nutrition status, which is often associated with severe pediatric GERD, is a risk factor for SMAS. In this case, development of gas bloat and SMAS led to the formation of a closed loop and increased intraluminal pressure of the duodenum and pancreatic duct. Many pediatric patients who need anti-reflux surgery face the risk of developing this entity. Preventive measures, such as treatment with prokinetics and frequent small-volume meals, should be considered until improvement of nutritional status after fundoplication.

Acute motor-sensory axonal Guillain-Barré syndrome with unilateral facial nerve paralysis after rotavirus gastroenteritis in a 2-year-old boy.

Guillain-Barré syndrome (GBS) is well known as presenting with acute immune-mediated polyneuropathies, with strong associations with antecedent infections. Several variant forms of GBS have been described, including acute inflammatory demyelinating polyneuropathy, acute motor axonal neuropathy, acute motor-sensory axonal neuropathy, and sensory GBS. We present a rare case of 2-year-old boy with acute motor and sensory polyneuropathy and left-sided facial nerve paralysis after rotavirus infection. He received immunoglobulin i.v. with subsequent satisfactory recovery.

Evaluation of Japanese practice guidelines for newly diagnosed childhood idiopathic thrombocytopenic purpura.

BACKGROUND: Practice guidelines for childhood idiopathic thrombocytopenic purpura (ITP) were proposed in 1998 and 2004 in Japan. It is important to evaluate the feasibility and validity of the guidelines because the guidelines are based not on evidence but on opinion. METHODS: Records of 30 consecutive hospitalized patients aged 0-15 years (median, 3 years; 17 boys, 13 girls) were retrospectively reviewed. The feasibility and validity of the 2004 guidelines were evaluated by calculating an implementation rate, a response rate to first treatments, and a final clinical outcome. RESULTS: Deviation from the guidelines was found in two patients. Management of the other 28 patients followed the guidelines. The implementation rate was 93.3%. Twenty-one of the 28 eligible patients (75%) responded to the first treatment, whereas seven patients (25%) required additional treatment. In 23 patients (76.7%) platelet counts returned to normal within 6 months. Seven patients (23.3%) developed chronic conditions. All patients had no complications and achieved a platelet count >150 000/µL within 6 years. CONCLUSION: The 2004 Japanese guidelines are feasible and valid for the management of newly diagnosed childhood ITP patients.

Diffuse leptomeningeal hyperintensity on fluid-attenuated inversion recovery MR images in neurocutaneous melanosis.

Neurocutaneous melanosis (NCM) is a rare neuroectodermal dysplasia characterized by large or multiple cutaneous congenital pigmented nevi and benign or malignant melanocytic tumors of the leptomeninges. Although the MR manifestations of this disease have been reported in a small series of cases, the usefulness of fluid-attenuated inversion recovery (FLAIR) MR findings has not been documented. We present a case of NCM that showed diffuse leptomeningeal hyperintensity on FLAIR images. This FLAIR finding may be a clue to the detection of leptomeningeal abnormalities in NCM.