ABSTRACT
<p><b>OBJECTIVE</b>To investigate reasonable surgical therapy for conjoined twins.</p><p><b>METHODS</b>Two pairs of gastrothoracopagus were admitted in July 2004 and April 2005 respectively. The first pair was separated by emergency surgery for the rupture of umbilical hernia resulting in the exposure of intestines. The thoracic and abdominal wall was repaired with local skin flaps, and the secondary wound was covered with artificial skin. Skin expanders were embedded in thoracic and abdominal wall 2 months after birth in the second pair. The surgical separation was performed one month after. The deficiencies of pericardium, sternum and abdominal wall were reconstructed by allogenic grafting of pericardium, porous polyethylene implant and monofilament polypropylene patch respectively. The thoracic and abdominal wall was repaired with expanded rotation skin flap.</p><p><b>RESULTS</b>The first twins died of respiratory failure and circulatory and respiratory failure 2 hours and 39 hours after the separation respectively. Both of the second pair survived and were discharge after healing.</p><p><b>CONCLUSIONS</b>The separation of gastrothoracopagus should be performed after skin expansion in the interest of the closure of wound. It's better to use porous polyethylene implant and monofilament polypropylene patch to reconstruct the sternum and abdominal wall respectively.</p>
Subject(s)
Female , Humans , Infant , Infant, Newborn , Abdominal Wall , Congenital Abnormalities , General Surgery , Fatal Outcome , Plastic Surgery Procedures , Thoracic Wall , Congenital Abnormalities , General Surgery , Twins, Conjoined , General SurgeryABSTRACT
<p><b>OBJECTIVE</b>To establish the genetic background of exon2, exon13, exon11 and exon15 polymorphisms of RET proto-oncogene and study the possible involvement of RET proto-oncogene in the etiology of Hirschsprung disease (HD) in Chinese Han population surrounding Province HuBei.</p><p><b>METHODS</b>The genotype and allele frequencies of RET proto-oncogene polymorphisms were identified by polymerase chain reaction-restriction fragment length polymorphisms (PCR-RFLPS) in 94 HD patients and 122 control subjects.</p><p><b>RESULTS</b>The genotype and allele frequencies of exon2 were AA 0.17, AG 0.72, GG 0.11, A 0.53, G 0.47 in control, and AA 0.61, AG 0.35, GG 0.04, A 0.78, G 0.22 in HD, and those of exon13 were GG 0.30, GT 0.52, TT 0.18, G 0.56, T 0.44 in control, and GG 0.49, GT 0.36, TT 0.15, G 0.67, T 0.33 in HD. There were significant differences in the two polymorphisms above between HD and control. The genotype and allele frequencies of exon11 were AA 0.05, AG 0.16, GG 0.79, A 0.13, G 0.87 in control and AA 0.02, AG 0.14, GG 0.84, A 0.09, G 0.91 in HD, the differences were not found between these two groups about this site. Exon15 were all of CC genotype in spite of control or HD.</p><p><b>CONCLUSIONS</b>These data provide evidences for the contributions of exon2 and exon13 polymorphisms of RET proto-oncogene to susceptibility to HD in Chinese Han population surrounding province.</p>
Subject(s)
Child, Preschool , Female , Humans , Infant , Male , Alleles , Asian People , Genetics , China , Exons , Gene Frequency , Genotype , Hirschsprung Disease , Ethnology , Genetics , Polymorphism, Genetic , Proto-Oncogene Proteins c-ret , GeneticsABSTRACT
Objective To evaluate the development of the sphincter muscle complex(SMC)and defecation function in pediatric patients with congenital anorectal malformations(ARM).Methods A total of 64 children underwent MRI,among whom 39 were patients with ARM,and the others were patients without ARM undergoing MRI because of other dieases.The dimensions of the SMC in different planes were evaluated with different sequences and coils.The relationship between the SMC development and the defecation function was investigated.Results In control group,the absolute value of SMC width was (3.63?0.22)mm,which had a high correlation with age(r=0.998,P0.05).The SMCs in intermediate ARM patients[muscle index(MI)=0.47?0.05]and low ARM patients(MI=0.49? 0.05)were well developed.The SMCs in a portion of patients with high ARM(MI=0.28?0.06)were poorly developed,when MI≤0.18,anorectal contraction pressurewas significantly lower(t=3.55, P0.18[(0.85?0.20)vs(2.24?1.02)kPa].The length of anal canal with high-pressure[(10.88?3.64)vs(20.26?4.34)mm]was shorter(t=5.18,P0.18,the anorectal angle was less than 90 degrees,and normal continent function was found in 21 of 23 cases(91%).Conclusion MRI can be employed to evaluate the development of SMC in patients with ARM,MI was an objective criteria to evaluate the development of SMC.When MI≤0.18, maldevelopment of SMC will be highly suspected.