Objective:The clinical manifestations, types of gene mutations, therapeutic effects and prognostic characteristics of 15 children with cblC type methylmalonic acidemia (MMA) and hydrocephalus were analyzed to improve the clinical understanding of the disease, so as to provide a basis for the treatment of the disease.Methods:From April 2015 to January 2019, 15 patients with MMA and hydrocephalus in Department of Pediatric Surgery, Peking University First Hospital were enrolled, and all gene detection showed clbC type.All the 15 patients underwent ventriculoperitoneal shunt.After surgery, the clinical manifestations and imaging findings were applied as the basis to adjust the pressure of the diverter valve appropriately.Clinical data and gene mutation characteristics of 15 children with cblC type and hydrocephalus were retrospectively analyzed, and the therapeutic effects and prognosis were summarized and analyzed as well.Results:There were 8 males (53.3%) and 7 females (46.7%), aged from 2 to 33 months.All the cases were followed up from 11 to 55 months, without death case and serious postoperative complications of hydroce-phalus.The head circumference of 3 cases (20.0%) was in the normal range, 1 case (6.7%) was greater than the normal range, and 11 cases (73.3%) were less than the normal range.Four patients (26.7%) were transferred to the pediatric intensive care unit after surgery.c.609G>A mutation was the most common in this study, with 7 cases (46.7%) of c. 609G>A homozygous mutation, and 5 cases (33.3%) of c. 609G>A heterozygous mutation.Clinical symptoms of intracranial hypertension were relieved or disappeared.The head circumference progressive enlargement was stopped.The anterior fontanelle tension greatly decreased, all " setting-sun" sign of eyes disappeared, and vision loss and hearing loss were better compared with the pre-operation.Four cases (26.7%) displayed normal intelligence and exercise, and 11 cases (73.3%) were left with mild to severe psychomotor retardation.During the follow-up pe-riod, the head CT showed that the ventricle was remarkably narrowed, and interstitial brain edema obviously improved.Conclusions:Ventriculoperitoneal shunt in the treatment of cblC type MMA with hydrocephalus has positive effects.The head circumference of most cblC type MMA with hydrocephalus is less than the normal range.c.609 G>A is the most common mutation in cblC type MMA with hydrocephalus.Perioperative " metabolic crisis" can result in serious complications.

Objective Analyze the distribution and influencing factors of inpatients with extra-long hospital stay in Hubei Province. Methods Length of stay≥30 days was defined as the extra-long hospital stay, and logistic regression was used to determine the risk factors. Results There were 26 043 cases with extra-long hospital stay, which accounting for 2.88% of the total cases. Proportion of extra-long hospital stay was increased with age, the proportion of the group aged 65 and above was the highest (3.25%), and which were higher in males than that of females, the sex ratio was 1.58. Patients with extra-long hospital stay mainly had injury/poisoning and certain other consequences of external causes (16.88%), circulatory system disease (12.72%), neoplasms (11.23%), genitourinary system diseases (8.13%), mental and behavioral disorders (6.27%), and the proportion totaled up to 52.23%. Multivariate logistic regression analysis indicated that gender, age, type of disease, surgery or not and hospital grade were the influencing factors of extra-long hospital stay. Conclusions There are differences in the distribution of extra-long hospital stay in different individuals, diseases and medical treatment, targeted measures should be taken to shorten the length of stay.