Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Management of the pelvic space after rectal excision is still problematic. Two methods have to be preferred: (1) closed suction lavage drainage and (2) open wound plugging; then the pelvic floor best remains unsutured. Plugging is always indicated in difficult pelveoperitoneal suture, unsatisfactory hemostasis and fecal contamination of the pelvic space. Retardation of wound healing is compensated for by more comfort for the patient and elimination of late complications, compared with those with partially closed or secondary opened wounds. The sacroperineal scar is the origin of a lot of complaints and morbidity. The most important are: infectious complications and persistent fistula (17.3%), pseudosinus perinealis (10%), perineal hernia and genital prolapses (16%), urologic complications (recidivating infections [26.9%], changes of the position of the urinary bladder [56%], secondary retroperitoneal fibrosis with urinary restriction [19.2%] or hydronephrosis [3.8%], disturbances of bladder emptying [36.5%], frequently combined with neurogenic lesions because of intraoperatively dissected autonomous pelvic nerves), local recurrence of carcinoma (17.3% in reexamination, but still much more important), and pain, often of unknown origin (34%).
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PMID:[Height of rectum amputation. Management, complications, disease significance]. 68 28

The necropsy reports of 294 cases of anencephaly and 50 cases of iniencephaly have been examined, and a tubulated list of associated malformations produced. Cases were divided by sex and the presence or absence of spina bifida. Forty-one per cent of the series had other malformations, and other malformations were more common in those cases with spina bifida than in those without. The most frequent single malformations were: hydronephrosis (8%), cleft palate (8%), diaphragmatic hernia (5%), exomphalos (5%), hare lip (4%), and horseshoe kidney (4%). It is suggested that the presence of other malformations in anencephaly or iniencephaly may imply some aetiological heterogeneity.
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PMID:Congenital malformations associated with anencephaly and iniencephaly. 78 97

Close phenotypic similarity between two cases carrying a rec(3) dup q,inv(3) (p25q21), 12 additional infants from the same inv (3)(p25q21) kindred who lived less than 1 year, and eight cases studied in other medical centers has led us to postulate the existence of a distinct chromosome 3 duplication-deletion syndrome. In the presence of trisomy for (3)q21 leads to qter and monosomy for (3)p25 leads to pter, the facial dysmorphy is unique: a distorted head shape due to irregular cranial sutures, thick low eyebrows, long eyelashes, persistent lanugo, distended veins on the scalp, hypertelorism, oblique palpebral fissures, a very short nose with a broad depressed bridge and anteverted nares, protruding maxilla, thin upper lip, micrognathia, low-set ears, and a short webbed neck. Port-wine stains, congenital glaucoma, cloudy corneas, cleft palate and harelip also occur frequently. Each infant has difficulty sucking and swallowing. Congenital anomalies of the cardiovascular system, of midgut rotation, and of the urogenital system are noted for the infants who died neonatally. Most frequent is a ventricular septal defect, followed by atrial septal defect, patent ductus arteriosus, patent foramen ovale, and coarctation of the aorta. Omphalocele, umbilical hernia, hyperplastic kidneys, polycystic kidneys, double ureter, hydro-ureter, hydronephrosis, and undescended testes often occur. The extremities are short in proportion to the length of the trunk. Clinodactyly, coxa valga, talipes, and spina bifida are frequently observed.
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PMID:Chromosome 3 duplication q21 leads to qter deletion p25 leads to pter syndrome in children of carriers of a pericentric inversion inv(3) (p25q21). 120 27

The advent of improved obstetrical care and innovative advances in fetal diagnostic techniques have mandated multidisciplinary approaches to pregnancies that are complicated by prenatally diagnosed defects. The concept of fetal therapy, previously limited to in utero transfusions for Rh disease and induction of lung maturation, has now been extended to open fetal correction of congenital hydronephrosis, diaphragmatic hernia, and sacrococcygeal teratoma. Additional inquiries are now being made into the possibility of hematopoietic stem cell transplantation in utero as well as prenatal gene therapy. Continued investigation into these therapeutic interventions largely revolves around improving their efficacy and guaranteeing the safety of the mother and her unborn child.
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PMID:Fetal surgery: correction of anatomic and constitutional defects. 161 37

We report on a newborn boy with manifestations of Fryns syndrome who also had a mosaic tandem duplication of chromosome 1q24-31.2. The child had a diaphragmatic hernia, cleft palate, hypoplastic and absent digits, micrognathia, long philtrum, thin upper lip, and anteverted nose. The baby died at age 5 hours. An autopsy demonstrated absent right middle lobe of the lung, bilateral renal cysts, hypoplastic renal arteries, urethral stricture, hydronephrosis, and aortic coarctation. The brain was abnormal with absent olfactory tracts and cerebral and cerebellar heterotopias. This is the first report of a chromosome anomaly in a child with Fryns phenotype. It suggests that the gene for Fryns syndrome may be located in the region 1q24-31.2.
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PMID:Apparent Fryns syndrome in a boy with a tandem duplication of 1q24-31.2. 259 30

Dichloroacetonitrile (DCAN), a by-product of drinking water disinfection formed by reaction of chlorine with background organic materials, was evaluated for its developmental effects in pregnant Long-Evans rats. Animals were dosed by oral intubation on Gestation Days 6-18 (plug = 0) with 0, 5, 15, 25, or 45 mg/kg/day. Tricaprylin was used as a vehicle. The highest dose tested (45 mg/kg) was lethal in 9% of the dams and caused resorption of the entire litter in 60% of the survivors. Embryolethality averaged 6% per litter at the low dose and 80% at the high dose and was statistically significant at 25 and 45 mg/kg/day. The incidence of soft tissue malformations was dose related and was statistically significant at doses toxic to the dam (45 mg/kg). These anomalies were principally in the cardiovascular (interventricular septal defect, levocardia, and abnormalities of the major vessels) and urogenital (hydronephrosis, rudimentary bladder and kidney, fused ureters, pelvic hernia, cryptorchidism) systems. The frequency of skeletal malformations (fused and cervical ribs) was also dose related and significantly increased at 45 mg/kg. The no-observed-adverse-effect dose for toxicity in pregnant Long-Evans rats was established by statistical analysis to be 15 mg/kg/day.
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PMID:Developmental toxicity of dichloroacetonitrile: a by-product of drinking water disinfection. 274 78

Female CD-1 mice were exposed to Tordon 202c (a picloram and 2,4-D combination herbicide) in the drinking water at concentrations of 0.21, 0.42, and 0.84% for 60 days prior to mating with untreated males. One-half of the pregnant females subsequently continued treatment throughout gestation while the remaining females were maintained on distilled water. Fetal weight, crown-rump length, placental weight, and maternal gestational weight gain were reduced in a dose-dependent manner following combined preconceptional and gestational exposure. The incidence of malformed fetuses (cleft palate, renal agenesis, hydronephrosis, unilateral testicular agenesis, and umbilical hernia) and fetuses with variants (especially incomplete ossification of the skeleton) were increased in a dose-dependent manner following combined exposure. Increased maternal mortality and decreased preconception weight gain were observed in the highest-dosage group. Relative maternal liver weight was increased in a dose-dependent manner. The results suggest that combined preconceptional and gestational exposure to Tordon 202c is required for teratogenesis and fetal growth depression. Preconceptional exposure alone is not effective in increasing the risk for embryotoxicity.
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PMID:Effects of preconceptional and gestational exposure to Tordon 202c on fetal growth and development in CD-1 mice. 278 33

In a period of 6 years 144 asymptomatic boys with cryptorchidism, of mean age 7 +/- SD 3.5 years, underwent orchiopexy. None of these boys referred to a history of a known urological anomaly, urinary tract infection, haematuria, palpable mass in the renal region, bladder extrophy, epispadias, hypospadias or anorectal malformation. On the third day after orchiopexy and intravenous pyelography was done in every boy following testicular protection against irradiation. Ultrasonic investigation was not available at that time. There were minor urological abnormalities in 36 (25%) boys and major ones in 8 (5.5%) boys. A major anomaly is defined as one resulting in significant loss of renal substance (one case of single kidney and three cases of unilateral renal hypoplasia), or requiring surgical correction for conservation of the renal substance (one case of ureterocele, two cases of pelviureteric stenosis and one case of vesicoureteric stenosis with ipsilateral hydronephrosis). The unsuspected major urological abnormalities are usually ipsilateral to the more undescended testis. They may be associated with a hernia and are more frequent in bilateral cryptorchidism. In conclusion we encourage the routine use of IVP, or ultrasonic investigation or dynamic renal scanning (99mTc-DTPA), if it is possible, in all patients undergoing orchiopexy for the detection of an unsuspected major renal anomaly.
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PMID:Unsuspected urological anomalies in asymptomatic cryptorchid boys. 289 30

Interest in correcting fetal pathology before birth has been stimulated by advancing experimental technology and improvements in the ability of ultrasound to look into the uterus. In this article, diagnosis, management and treatments of three congenital defects, hydronephrosis, ventriculomegaly, and diaphragmatic hernia are discussed. The future of fetal in utero surgery also is considered.
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PMID:Fetal surgery. 306 9

From July 1985 through January 1986, 43 patients underwent urinary diversion that included creation of a continent reservoir from an ileal segment, according to the method described originally by Kock. An important modification included removal of a narrow strip of mesentery for 8 cm along the afferent and efferent limbs of the pouch to allow adequate ileal intussusception and fixation of the nipple valves to prevent reflux and to ensure continence. A strip of PGA mesh serves as a collar to fix the afferent-efferent limb to the pouch once the intussusception technique has been accomplished. The use of a narrow Marlex strip allows fixation to the abdominal wall both lateral and medial to the stoma site (insert). This strip is important in preventing a parastomal hernia and helps fix the continence valve mechanism to the posterior abdominal wall. Previous urinary diversion was by ureterosigmoidostomy in 2 patients, standard ileal conduit in 8 and chronic dialysis after nephrectomy of solitary kidney and cystectomy in 1. A total of 32 patients underwent simultaneous anterior exenteration or radical cystectomy for pelvic malignancy. There were 4 postoperative deaths and early complications occurred in one patient. Late complications occurred in only 3 patients: they required reoperation and revision of the continence valve mechanism. The end result in 39 of 43 patients has been an overwhelming success. Patients perform self-catheterization every 4 to 6 hours during the day and once at night for volumes ranging up to 1,400 cc. Serum electolytes have remained normal in all patients. X-ray of the Kock pouch have shown no evidence of reflux, and all excretory urograms have demonstrated either normal upper tracts without obstruction or improvement in patients with preoperative hydronephrosis. Although preliminary, this clinical trial suggests that the quality of life for patients considered previously to be candidates for cutaneous diversion can be improved markedly by a modified Kock continent ileal reservoir. During the same time, 21 patients out of 278 patients who underwent creation of a Kock continent ileal urinary reservoir since August 1982, underwent revision of Kock pouch. Two of those required subsequent reoperation and revision of the continence valve mechanism. The end result in all patients has been an overwhelming success.
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PMID:[The Kock continent ileal urinary reservoir: surgical technic and clinical results]. 377 74


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