The ketogenic diet for Dravet syndrome: A multicenter retrospective study.

OBJECTIVE: The ketogenic diet (KD) is one of the main treatments for drug-resistant epilepsy. However, there have been few multicenter reports on the use of the KD for the treatment of Dravet syndrome (DS). The aim of this study was to analyze the efficacy and safety of this approach based on a large number of multicenter cases. METHODS: This was a retrospective, multicenter cohort study from 14 centers in China. All patients were treated with the KD. We compared the effects of KD intervention time, age, and other factors. RESULTS: From March 2014 to March 2020, we treated 114 patients with DS with the KD. The male-to-female ratio was 67:47. The KD median initiation age was 3 y and 4 mo, and the median number of antiseizure medications (ASMs) was 2.4. KD therapy was the first choice for three patients. Exactly 10.5% of the patients started KD therapy after failure of the first ASM therapy, with 35.1% after failure of the second, 44.7% after the third, and 7% after the fourth or more. After KD therapy for 1, 3, 6, and 12 mo, the seizure-free rates were 14%, 32.5%, 30.7%, and 19.3%, respectively; KD efficacy (≥50% reduction in seizure frequency) were 57.9%, 76.3%, 59.6%, and 43%, respectively; the retention rates were 97.4%, 93%, 71.9%, and 46.5%, respectively; and the rates of adverse events were 25.2%, 19.9%, 11%, and 5.7%, respectively. CONCLUSIONS: Real-world, multicenter data analysis showed that the KD is effective for patients with DS and has a low incidence of side effects.

Clinical features of obstructive sleep apnea in children with obesity. / è‚¥èƒ–å„¿ç«¥é˜»å¡žæ€§ç¡çœ å‘¼å¸æš‚åœçš„ä¸´åºŠç‰¹å¾.

OBJECTIVES: To study the clinical features of obstructive sleep apnea (OSA) in children with obesity. METHODS: A retrospective analysis was performed on the medical data of 33 obese children aged 7-15 years, who were diagnosed with OSA and received polysomnography (PSG) in the Department of Respiratory Medicine in Shenzhen Children's Hospital. Fifty OSA children with normal body weight, matched for sex and age, were enrolled as the control group. RESULTS: Among the 33 obese children with OSA, the three most common daytime symptoms were inattention in 30 children (91%), somnolence in 22 children (67%), and morning fatigue in 21 children (64%), and the three most common nocturnal symptoms were snoring in 27 children (82%), mouth breathing in 20 children (61%), and sweating in 16 children (49%). Compared with the reference values of normal children, both the OSA + obesity group and the control group had prolonged light sleep, shortened deep sleep, and a significantly shortened rapid eye movement (REM) period, while there was no significant difference in these indices between the two groups (P>0.05). The apnea-hypopnea index, obstructive apnea/hypopnea index, and oxygen desaturation index in both REM and non-REM periods in the OSA +obesity group were significantly higher than those in the control group (P<0.05), while the lowest blood oxygen saturation during sleep was significantly lower in the OSA + obesity group (P<0.05). CONCLUSIONS: The children with obesity and OSA have the main daytime symptoms of inattention, somnolence, and morning fatigue and the main nocturnal symptoms of snoring, mouth breathing, and sweating. There is no significant difference in sleep structure between OSA children with obesity and those with normal body weight; however, respiratory events and blood oxygen saturation decline are more severe in OSA children with obesity. Citation.

[Nutritional recovery after discharge in hospitalized children with malnutrition].

OBJECTIVE: To investigate the nutritional recovery status of children with moderate or severe malnutrition during hospitalization after discharge. METHODS: The children with moderate or severe malnutrition were given nutrition support during hospitalization. They received a regular follow-up and nutrition guidance after discharge. The weight-for-age and height-for-age Z-scores reaching above -2 SD were considered the nutrition criterion for ending follow-up. RESULTS: Among the 298 children with moderate or severe malnutrition, 174 (58.4%) reached the criterion for ending follow-up, 100 (33.6%) were lost to follow-up, 18 (6.0%) died, and 6 (2.0%) did not reach the criterion for ending follow-up after 18 months of follow-up. The children with malnutrition in the department of surgery had a significantly higher proportion of children reaching the criterion for ending follow-up than those in the department of internal medicine (P<0.05). The children with severe malnutrition had a significantly higher loss to follow-up rate than those with moderate nutrition (P<0.05). The majority of children with emaciation reached the criterion for ending follow-up at month 3 after discharge, while those with growth retardation reached such the criterion at months 3-6 after discharge. Up to 1 year after discharge, more than 80% of the children with different types of malnutrition reached the nutrition criterion for ending follow-up. CONCLUSIONS: Most of the children with malnutrition who adhere to follow-up can reach the expected nutrition criterion within 1 year after discharge. The children with growth retardation have slower nutritional recovery than those with emaciation.

Intestinal Bacteroides sp. Imbalance Associated With the Occurrence of Childhood Undernutrition in China.

Undernutrition (UN) is a worldwide concern affecting morbidity and mortality among children, but the safety and long-term efficacy of its current treatments remain controversial. Recent evidence showing the roles of the gut microbiome (GM) in nutrient absorption indicates its usefulness in alternative interventions to treat UN safely with sustainable amelioration. To enhance our understanding of the GM and childhood undernutrition, we deep sequenced the gut metagenomes of 65 children with moderate or severe undernutrition (UN group) and 61 healthy children (HC group) to identify associated taxa and genes using a two-stage validation scheme. At stage I, 54 UN patients and 51 healthy children were enrolled for the discovery of GM markers in UN children. The accuracy of the markers was then tested in an additional 11 UN patients and 10 healthy children at stage II. Compared to the HC group, the UN group had lower richness in microbial genes (P = 0.005, FDR = 0.005) and species (P = 0.002, FDR = 0.002). The distributions of bacterial genes enable the identification of 16 gene markers with which to discriminate UN patients with high accuracy [averaged areas under the receiver operating curve (AUC) = 0.87], including three Bacteroides uniformis genes that are responsible for the synthesis of iron transporters. We also identified four species markers that enable the UN patients to be confidently discriminated from the HC children (averaged AUC = 0.91), namely Bacteroides ovatus, Bacteroides uniformis, Bacteroides uniformis, and Bacteroides vulgatus. In addition, metabolic comparison showed significantly decreased isobutyric acid (P = 0.005, FDR = 0.017) and increased isovaleric acid (P = 0.006, FDR = 0.017) in UN patients. We also identified notable correlations between microbial species and short-chain fatty acids (SCFAs) and several nutritional indicators, including acetic acid and iron (r = 0.436, P = 0.029), butyric acid and iron (r = 0.422, P = 0.036), butyric acid and lymphocyte (r = -0.309, P = 0.011), and acetic acid and total protein (r = -0.303, P = 0.043). Taken together, the distinct features of gut microbiota in UN patients highlight the taxonomic and functional shift during the development of UN and provide a solid theoretical basis for intervention in childhood undernutrition through gut microbes.

[Research advances in the relationship between childhood malnutrition and gut microbiota].

Childhood malnutrition is an important disease threatening healthy growth of children worldwide. Gut microbiota has close links to food digestion, absorption and intestinal function. Current research considers that alterations in gut microbiota have been strongly implicated in childhood malnutrition. This review article addresses the latest understanding and evidence of interrelationship between gut microbiota and individual nutrition status, the changes of gut microbiota in different types of malnutrition, and the attribution of gut microbiota in the treatment and prognosis of malnutrition. It provides in depth understanding of childhood malnutrition from the perspective of microbiome.

Hospitalized children with 2009 influenza a (H1N1) infection in Shenzhen, China, November-December 2009.

OBJECTIVES: During the winter outbreak of 2009 influenza A (H1N1) infection in China, the number of confirmed cases and the fatal cases has grown rapidly. We describe the clinical characteristics of hospitalized children with 2009 influenza A (H1N1) infection in Shenzhen, China, November-December 2009. METHODS: Using a standardized form, we collected data on 148 hospitalized children. 2009 influenza A (H1N1) infection was confirmed in nasopharyngeal swab specimens with the use of a real-time reverse transcriptase-polymerase chain reaction assay. RESULTS: Of the 148 hospitalized children with 2009 influenza A (H1N1) infection, 81 (55%) were 5 years of age or older and 85% of the patients were previously healthy. The common presenting symptoms were fever (94%), cough (89%), runny nose (36.5%), vomiting (24%), sore throat (19.6%), wheezing (18%), abdominal pain (16%), mental status changes (9%), seizures (6%), diarrhea (6%), myalgia (6%), and chest pain (4%). Twenty-nine (20%) patients were admitted to an ICU, 10 (7%) patients required mechanical ventilation. The overall complication rate was 65.5%, they were pneumonia in 94 (64%), neurologic complications in 18 (12%), parapneumonic effusion in 12 (8%) and myocarditis in 7 (5%). One hundred seven (72%) patients received oseltamivir treatment, 34 (23%) received within 48 hr after the onset of symptoms. All patients received antibiotics before admission or on admission. One hundred forty-four (97%) patients were discharged; four (3%) previously healthy patients died, three died from severe encephalopathy, one died from secondary fungal meningitis. CONCLUSION: Hospitalized children with 2009 influenza A (H1N1) infection can have a wide range of presentation and clinical complications including neurologic complications. The severe cases and deaths concentrate in previously healthy older children.

[Characteristics of severely and critically ill children with 2009 influenza A (H1N1) virus infection].

OBJECTIVE: To analyze the clinical characteristics of severely and critically ill children with 2009 influenza A (H1N1) infection. METHOD: Clinical data of 150 cases with 2009 influenza A (H1N1) virus infection confirmed with the use of a real-time polymerase-chain-reaction assay on nasopharyngeal swab specimens were analyzed. RESULT: Among 150 severely and critically ill children with 2009 influenza A (H1N1) virus infection, 103 were male, 47 were female; the median age was 5 years, 81(55%) were 5 years of age or older; 21 (14%) had underlying chronic diseases. The most common presenting symptoms were fever (95%), cough (89%), vomiting (23%), wheezing (19%), abdominal pain (16%), lethargy (7%), seizures (6%), myalgia (6%), and diarrhea (6%). The common laboratory abnormalities were increased or decreased white blood cells counts (40%), elevated of CRP (33%), LDH (29%), CK (25%) and AST (19%). Clinical complications included pneumonia (65%), encephalopathy (12%), myocarditis (5%), encephalitis (1%) and myositis (1%). All patients had received antibiotics before admission or on admission; 73% of patients had received oseltamivir treatment, 23% of patients had received corticosteroids; 32 (21%) were admitted to an ICU, 13 patients were intubated and mechanically ventilated. Fourteen patients with dyspnea who were irresponsive to the treatment experienced bronchoalveolar lavage with flexible bronchoscopy, and the branching bronchial casts were removed in 5 patients. Totally 145 (97%) patients were discharged, five (3%) died, three previously healthy patients died from severe encephalopathy, one patient died from ARDS, one previously healthy patient died from secondary fungal meningitis. CONCLUSION: Severely and critically ill children with 2009 influenza A (H1N1) virus infection may occur mainly in older children without underlying chronic disease. The clinical spectrum and laboratory abnormality of the patients can have a wide range. Neurologic complications may be common and severe encephalopathy can lead to death in previously healthy children. Early use of bronchoalveolar lavage with flexible bronchoscopy may reduce death associated with pulmonary complications.

[Digestive system manifestations in children infected with novel influenza A (H1N1) virus].

OBJECTIVE: To study the digestive system manifestations in children infected with novel influenza A (H1N1) virus. METHODS: A prospective study of 153 children infected with novel influenza A (H1N1) virus in Shenzhen Children's Hospital from November 2009 to January 2010 was conducted. The clinical features and outcomes of 69 children with digestive system manifestations were analyzed. RESULTS: The children presenting with digestive system manifestations accounted for 45% (69 cases) in the 153 hospitalized children with novel influenza A (H1N1) infection. Gastrointestinal manifestations were observed in 50 cases (33%) and liver function abnormality in 19 cases (12%). The incidence rate of coma, neurological complications, increase in creative kinase level, ICU admission, and death in the patients with digestive system manifestations were significantly higher than those without digestive system manifestations (P<0.05). In the 69 patients with digestive system manifestations, 5 died from severe complications and 64 recovered fully. Gastrointestinal manifestations disappeared through 1 to 3 days and abnormal liver function recovered through 4 to 7 days. CONCLUSIONS: Digestive system manifestations are common in children infected with novel influenza A (H1N1) virus. Neurological system involvements are more common in the patients with digestive system manifestations than those without.

[Roles of bile and gastrin in the pathogenesis of childhood gastroesophageal reflux disease].

OBJECTIVE: To explore the incidence of acid and bile reflux in children with gastroesophageal reflux disease (GERD) and to study the roles of bile and gastrin in the pathogenesis of childhood GERD. METHODS: Forty-two cases of GERD were divided into two groups according to endoscopic findings: reflux esophagitis (RE) and non-erosive reflux disease (NERD). The patients underwent 24-hr ambulatory esophageal pH and bilirubin monitoring. The serum concentration of gastrin was detected by radioimmunoassay. Thirteen children without gastroesophageal reflux symptoms, digestive tract disease and severe systemic organic disease served as the Control group. RESULTS: Of the 42 cases of GERD, 24 cases were confirmed with RE, with esophageal mucosal lesions, and 18 were NERD without esophageal mucosal lesions by endoscopy. Both acid and bile reflux parameters, including the percentage of total time with pH < 4 and bilirubin absorbance >/= 0.14, the total number of reflux episodes and the number of bile reflux episodes lasting longer than 5 minutes, were significantly higher in the GERD patients than those in the Control group (P < 0.05). The time of esophageal acid exposure (pH < 4) and the percentage of total time with bilirubin absorbance >/= 0.14 increased significantly in the RE group compared with in the NERD group (P < 0.05). Sixteen RE patients had a mixed reflux of bile and acid (66.7%) but only 6 NERD patients (33.3%) had (P < 0.01). The serum concentration of gastrin in the RE group (125.12 +/- 45.06 pg/mL) and the NERD group (98.22 +/- 27.92 pg/mL) was significantly higher than that of the Control group (74.22 +/- 20.34 pg/mL) (P < 0.01, P < 0.05 respectively). A significant difference was noted in the serum concentration of gastrin between the RE and the NERD groups (P < 0.05). CONCLUSIONS: Mixed reflux of bile and acid are common in children with GERD. Bile reflux may play a role in the development of GERD. Gastrin parasecretion may participate in the development of GERD. Gastrin and bile reflux may have synergistic effects on the development of childhood GERD.