Diet ; Eating ; Genetic Therapy ; Hemofiltration ; Humans ; Metabolism, Inborn Errors ; genetics ; therapy ; Transplantation

Autoantibodies ; blood ; immunology ; Child ; Diagnosis, Differential ; Humans ; Radiography ; Radionuclide Imaging ; Thyroid Diseases ; blood ; diagnosis ; diagnostic imaging ; Thyroid Function Tests ; methods ; Thyroid Gland ; diagnostic imaging ; immunology ; Thyroid Hormones ; blood

Body Height ; Child ; Female ; Ghrelin ; pharmacology ; Growth Disorders ; physiopathology ; Growth Hormone-Releasing Hormone ; pharmacology ; Human Growth Hormone ; blood ; physiology ; urine ; Humans ; Insulin-Like Growth Factor Binding Protein 3 ; blood ; Insulin-Like Growth Factor I ; physiology ; Male

Adolescent ; Diagnosis, Differential ; Female ; Humans ; Myositis Ossificans ; diagnosis

Objective To explore incidence of child obesity in Zhengzhou area and intervention measures.Methods In 2001, spot check was conducted on 5688 cases of high and primary school students ,including 2848 boys and 2840 girls at the age of 7-18.Child obesity was diagnosed by meeting reference BMI value published by Cole et al,conducted comprehensive treatment consisting of massage intervention along channel of traditional Chinese medicine, behavior modification, dietetic and sport adjustment for 22 cases of simple obesity children (7-15 years old, 18 boys, 4 girls) selected for one month, and follow-up survey 6 months after treatment.Results Five thousands,six hundreds and eighty-eight high and primary school students investigated had an overweight incidence of 15.4%, an obesity incidence of 3.2%,boys' overweight incidence and obesity incidence(19.2%,4.6%) were remarkably higher than those of girls (11.5%,1.7% P<0.001). Overweight incidence of various age groups evidently differed(χ2=42.88 P<0.001) with the group of 8-15 years old children as popular. Incidence of various age groups also differed(χ2=21.28 P<0.05) with 7-10 years old and 14-15 years old children as popular. After one-month treatment, weight of all the 22 cases of fat children decreased from (76.45±19.87) kg upon hospitalized to (69.06±17.98) kg with a decrease of (7.43±2.58)kg, BMI value decreased from (31.05±3.96) before treatment to (27.72±3.54).Weight and BMI value before and after treatment differed evidently (t=13.6,12.88 P<0.01), and weight and BMI value decrease were remarkably related with those upon hospitalized (r=0.77 P<0.01;r=0.49 P<0.05).Conclusions Incidence of child obesity has been increasing in recent years, comprehensive treatment consisting of massage intervention along channel of traditional Chinese medicine, behavior modification, dietetic and sport adjustment have good curative effect and are comfortable, well received by children and suitable to promote and apply.

Objective To investigate the therapy for small and short penis in simple obesity children.Methods Sixty simple obesity children with short and small penis were randomly divided into 2 groups(experiment group 30 cases and control group 30 cases).The experiment group received comprehensive treatment(consisting of massage of traditional Chinese medicine,behavior modification,dietetic and sport adjustment) to lose weight and treated by short and small penis healing apparatus.The control obesity children were given conducted behavior,dietetic and sport adjustment,and treated by short and small penis healing apparatus.Results After treatment,weight of 30 cases in the experiment group decreased,with a decrease of(7.75?3.50) kg.Body mass index(BMI) decreased from(31.10?3.88) before treatment to(27.82?3.49),and BMI before and after treatment changed significantly(t=12.68 P

OBJECTIVETo evaluate the methods of repairing urethral fistula resulting from hypospadias operation.

METHODSWe analyzed the clinical data of 46 cases of surgical repair of urethral fistula resulting from hypospadias operation. The patients ranged in age from 3 to 26 (mean 11.7) years. There were 52 fistulas in all, with the diameters of less than 5 mm, of which 25 were in the midshaft of the penis, 12 in the penoscrotum, and 9 in the scrotum. Of the 46 cases, 20 were repaired by continuous knock suture and 26 by tunica vaginalis of testis and spermatic fascia.

RESULTSThe one-stage success rate of continuous knock suture repair was 55% (11/20) and that of the combined use of continuous knock suture with tunica vaginalis of testis and spermatic fascia repair was 84.6% (22/26).

CONCLUSIONBased on continuous knock suture, the additional use of tunica vaginalis of testis and spermatic fascia yields a high success rate in repairing urethral fistula resulting from hypospadias operation.

Adolescent ; Adult ; Child ; Child, Preschool ; Humans ; Hypospadias ; surgery ; Male ; Postoperative Complications ; surgery ; Reconstructive Surgical Procedures ; methods ; Spermatic Cord ; transplantation ; Urinary Fistula ; etiology ; surgery ; Young Adult

OBJECTIVEGlutaric aciduria type II, or multiple acyl-CoA dehydrogenase deficiency is an autosomal recessively inherited defect of mitochondrial energy metabolism. The authors report two cases of late-onset glutaric aciduria type II, and evaluate the procedures for the diagnosis and treatment of this rare disease.

METHODSThe clinical and biochemical characteristics of 2 patients with late-onset glutaric aciduria type II were documented. Case 1 presented with lipid storage myopathy for 3 years. Case 2 presented with intermittent episodes of non-ketotic hypoglycemia and muscle weakness for 9 years. The diagnosis of the 2 cases was confirmed with gas chromatography/mass spectrometry analysis of urine samples. Riboflavin supplementation and a low-fat, low-protein, high-carbohydrate diet were initiated as soon as the diagnosis was made.

RESULTSOrganic acid analysis on both untreated cases revealed massive glutaric acid with elevated concentrations of isovalerylglycine, isobutyrylglycine, ethylmalonic acid, adipic acid, suberic acid and other dicarboxylic acids. The clinical manifestations were improved remarkably after the administration of riboflavin and diet control. Consistent improvements of sera enzymes and urine organic acids were observed during the course of treatment.

CONCLUSIONPatients with unexplained myopathy, metabolic acidosis or hypoglycemia should be carefully screened for inherited metabolic disorders. Riboflavin in conjunction with appropriate diet control is an effective therapeutic regime for patients with late-onset glutaric aciduria type II.

Acyl-CoA Dehydrogenase ; deficiency ; Adolescent ; Age of Onset ; Child ; Female ; Gas Chromatography-Mass Spectrometry ; Glutarates ; urine ; Humans ; Metabolism, Inborn Errors ; diagnosis ; drug therapy ; urine ; Photosensitizing Agents ; therapeutic use ; Riboflavin ; therapeutic use ; Treatment Outcome

On June 6,2016,an outbreak of suspected cutaneous anthrax was reported by T County CDC,Xinjiang Uygur Autonomous,China.An investigation was carried out to determine the magnitude and risk factors and to recommend control measures.Suspected cases were defined as any villager in T county residents whose skin appeared rash,blisters,ulceration,swelling of black eschar or ulcer between May 1 and June 8.Confirmed case were defined as suspected case plus serological positive,real-time polymerase chain reaction (PCR) positive or Bacillus anthraci isolated.We interviewed clinicians and reviewed medical files in the township and city hospital,and visited all households searching for cases.A case-control study was conducted to investigate the risk factors of human anthrax infection.Three types of specimens were collected and tested by serological detection,real-time polymerase chain reaction (PCR) and bacterial culture.Through epidemiological investigation,15 families purchased beef of a sick cow on May 26,2016.Between 29 May and 3 June,ten people from these families were diagnosed as skin anthrax.Among them,six were confirmed cases and four were suspected cases.The attack rate was 20％.The incubation period was 3 to 7 days with average incubation 5 days.The nain clinical manifestations were hand skin anthrax carbuncle.Case control study showed that the incidences of people who exposing or not exposing to contaminated beefs significantly different,with 53％ and 0％ respectively.Blood serums from six patients showed that specific antibody titer appeared to increase more than 4 times.B.anthraci was isolated from herpes fluid of one patient.B.anthraci nucleic acid was detected from beef collected from four families.The outbreak was caused by the villagers exposed sick cow which infected by B.anthraci.Contaminated beef caused the infections.

OBJECTIVETo establish a specific procedure for the high-risk screening and diagnosis of organic acidurias and other inherited metabolic diseases in China.

METHODSA nation-wide network for the high-risk screening and diagnosis of genetic metabolic diseases was formed to facilitate the collaboration. Urine samples were collected using filter paper from patients with clinical symptoms suspicious of inherited metabolic diseases. The samples were eluted with distilled water and internal standards were added. Samples were treated with hydroxylamine hydrochloride to form oximes to improve the recoveries of 2-ketoacids. Urinary organic acids were extracted with ethyl acetate and diethyl ether under acidic condition. After dehydration, the combined organic phase was evaporated to dryness with nitrogen. The residues were added with BSTFA + 1%TMCS and heat incubated to form the trimethylsilyl derivatives, and then were analyzed on an Agilent 5890/5973N gas chromatography-mass spectrometer (GC-MS), with a 7683 series auto-sampler. The peaks were identified by reference to a mass spectral library.

RESULTSTotally 352 samples were collected from the network collaborating hospitals since 2001. Thirty-four (9.66%) cases of various inherited metabolic diseases were diagnosed with an age range of 2 days to 14 years. The disease profile was consisted of methylmalonic acidemias (6), alpha-keto-glutaric aciduria (5), tyrosinemia type I (4), dicarboxylic aciduria (4), multiple carboxylase deficiency (3), phenylketonuria (3), lactic acidemia (3), propionic acidemia (2), ornithine transcarbamoylase deficiency (1), ethylmalonic-adipic aciduria (1), glutaric aciduria type II (1) and 3-methylcrotyl CoA carboxylase deficiency (1). The most common clinical symptoms and signs included mental and developmental retardation, convulsion, musculotonic abnormality and jaundice. Routine laboratory tests often revealed metabolic acidosis, hypoglycemia and hyperammonemia, etc.

CONCLUSIONUrine organic acids analysis by GC-MS remains to be the most important technique for the high-risk screening and diagnosis of inherited metabolic diseases. Use of urine filter paper for sample collection and analysis in advanced genetic metabolic centers is a practical approach to extend the diagnostic capacity and improve the management of such diseases in China. Collaborative network played a critical role in the success of the program.

Adolescent ; Carboxylic Acids ; urine ; Child ; Child, Preschool ; China ; Female ; Gas Chromatography-Mass Spectrometry ; methods ; Humans ; Infant ; Infant, Newborn ; Male ; Mass Screening ; methods ; Metabolic Diseases ; diagnosis ; urine