OBJECTIVE: To summarize and analyze the clinical characteristics of Mondini dysplasia with cerebrospinal fluid leakage, as well as preliminarily investigate the genetic mechanism of the disease. METHOD: The clinical data of 2 patients diagnosed as Mondini dysplasia with cerebrospinal fluid leakage treated in our hospital were analyzed. Blood samples of these two patients were obtained to extract DNA. We screened DNA samples for gene SLC26A4 mutations by using polymerase chain reaction and direct sequencing. The sequencing results were analyzed in DNASTAR software. RESULT: Both patients came to our hospital because of recurrent meningitis, and the fistula were both located in vestibular window. Patients were cured one-time after surgical closure of the leakages with temporalis + temporalis fascia + temporalis through the mastoid approach. No pathogenic mutations of gene SLC26A4 with exome sequencing were found. CONCLUSION Mondini dysplasia with cerebrospinal fluid leakage should be considered in patients with recurrent meningitis and hearing disorder. Temporal bone HRCT is helpful to the diagnosis. Surgical closure is an effective therapeutic method and may prevent recurrent meningitis. The molecular mechanism of simple Mondini dysplasia needs further study.
Cerebrospinal Fluid Leak ; physiopathology ; Cochlea ; pathology ; Fistula ; pathology ; Humans ; Hyperplasia ; genetics ; physiopathology ; Membrane Transport Proteins ; genetics ; Meningitis ; physiopathology ; Mutation ; Sulfate Transporters

Mesh-related visceral complications caused by mesh erosion after tension-free inguinal hernia repair are one kind of rare long-term complications, but they are easily neglected. Interval time from initial hernia repair to mesh-related visceral complications by preperitoneal and laparoscopic repair is short. Rutkow and transabdominal preperitoneal repair have the highest reported rate. Lichtenstein has the longest interval time and the lowest reported rate. The most frequently eroded organs are sigmoid colon, bladder and small intestine. The common clinical manifestations of sigmoid colon erosion are hematochezia, abdominal wall fistula and colitis, hematuria and recurrent urinary tract infection in bladder erosion cases, intestinal obstruction and abdominal wall fistula in intestinal erosion case, sigmoid-bladder fistula and intestinal-bladder fistula in multiple organ erosion cases. Resection or repair of corresponding organs with mesh removal have good efficacies in most patients. The authors summarize and analyze researches on mesh-related visceral complications after tension-free inguinal hernia repair from 1994 to 2021, review their advances, in order to raise awareness of such complications in clinicians.

Objective:This study aimed to analyze differential metabolites in patients using a dual metabolic platform and to orientate early nutritional intervention in patients with cirrhosis.Methods:The skeletal muscle index (SMI) was calculated based on computed tomography (CT) measurements of skeletal muscle cross-sectional area at the third lumbar vertebra level. Pre-sarcopenia was diagnosed for males with SMI < 46.96 and for females with SMI < 32.46. Fifteen HBV-related liver cirrhosis patients with pre-sarcopenia were included as Group S while fourteen liver cirrhosis without pre-sarcopenia were Group NS. Liquid chromatography-mass spectrometry (LC-MS) and gas chromatography-mass spectrometry (GC-MS) analyses were used to detect differential metabolites and disturbed pathways in the two groups.Results:Five pathways and twenty-eight pathways were defined as disturbed pathways in the plasma of liver cirrhosis patients with pre-sarcopenia by LC-MS and GC-MS, respectively. Most of these pathways are related to amino acid metabolism. Forty-two differential metabolites were imported into the disturbed pathways. Moreover, 3-hydroxypropanal, hydrocinnamic acid, betaine aldehyde, phosphohydroxypyruvic acid, (r)-3-hydroxybutyric acid, and creatinine were identified as potential biomarkers for pre-sarcopenia in HBV-related liver cirrhosis.Conclusions:The study identified a total of 33 pathways and related differential metabolites that were disturbed in HBV-related liver cirrhosis with pre-sarcopenia. The amino acid metabolism, urea cycle, and glyoxylate and dicarboxylate metabolism pathways may be associated with pre-sarcopenia in patients with HBV-related liver cirrhosis. These results provide a direction for nutritional supplementation in liver cirrhosis.

Chronic kidney disease（CKD）has a significant impact on global public health and is one of the important factors leading to the rise of incidence rate and mortality of non communicable diseases.Intestinal microecology is involved in many pathophysiological activities，such as immunity and nutrient absorption.With proposal of the concept of intestine-kidney axis in 2002，researchers found that the decline of glomerular filtration rate led to accumulation of uremic toxins in intestine，destroyed the intestinal mucosal barrier，led to the translocation of toxins，activated the systemic oxidative stress response，further promoted the progress of CKD.This article reviewed the function of gut microbiota，intestine-gut kidney axis，and the application of microbial preparations in CKD.