Fraser syndrome associated with anterior urethral atresia.
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Biomedical subjects
Publications and source records attributed to F C Tanyel.
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The rabbit fetus is one of the most commonly used animal models in experimental studies investigating fetal organ development. However, there is no detailed information about normal growth of organs of rabbit fetuses in English language literature. Fetal rabbits were studied in the second half of gestation between 18th and 30th days. Amniotic fluid volume, body mass (BM), lung, heart and liver masses (LM, HM, LiM), lung and thorax volumes (LV, TV) were determined and LM/BM, HM/BM, LiM/BM, TV/BM and LV/TV ratios were calculated. Additionally fetal lungs were evaluated histologically, BM, LM, HM, LiM and LV were increased until 27th gestational day and then remained unchanged. TV was always increased between 18th gestational day and term. The lung maturation was almost completed in the 27th-28th gestational days. Therefore, BM, LM, HM, LiM and LV are the parameters that can be used to evaluate normal fetal growth between 18th and 27th gestational days. TV seems to be the predictive parameter for evaluation of normal fetal growth during the second half of gestation in rabbit fetuses; 20th and 27th days of gestation are more appropriate for experiments to evaluate lung maturation.
BACKGROUND/PURPOSE: The mechanism of testicular descent remains controversial. The processus vaginalis (PV) contains smooth muscle and should have contractile activity that may contribute to descent. This study was designed to evaluate the smooth muscle of PVs associated with incomplete obliteration for spontaneous activities and responses to various stimuli, to determine if differences exist according to sex, diagnostic source, or location of the testis. MATERIALS: Peritoneal samples (n = 4); sacs from girls (n = 8) and boys with inguinal hernia (n = 12); and sacs from boys with hydrocele (n = 3), hydrocele of the cord (n = 2), or undescended testis (n = 7) were used for the current study. Tissues were attached to the isometric force displacement transducer in an organ bath containing mammalian Ringer's solution at 37 degrees C. Spontaneous mechanical activity and contractile responses of tissues to the electrical field stimulation, phenylephrine, carbachol, and serotonin were recorded. The values obtained from boys and girls with inguinal hernia and from boys with either undescended or descended testis were compared through Fisher's Exact test. RESULTS: There were no statistically significant differences in patient age between groups. Among the parameters studied, only the carbachol response of the sacs associated with undescended testis showed a significant difference compared with the others (P = .001). None of the sacs associated with undescended testis responded to carbachol, whereas all of the sacs from boys and girls with inguinal hernia responded to carbachol. CONCLUSIONS: Lack of carbachol response suggests the absence of cholinergic receptors within the sacs associated with undescended testis. The lack of cholinergic receptors may play a role in the failure of the process of testicular descent by hindering either PV elongation into the scrotum or a possible propulsive activity of the PV on the testis.
BACKGROUND/PURPOSE: Histological structures of peritoneum, processus vaginalis, and sacs obtained from girls with inguinal hernia and boys with inguinal hernia, hydrocele, and undescended testis have been compared through immunohistochemical features to evaluate if any clue descriptive for the etiology of inguinal hernia exists. METHODS: Parietal peritoneums (n = 6), processus vaginalises (n = 4), female hernia sacs (n = 5), male hernia sacs (n 12), and sacs from hydrocele (n = 5) and undescended testis (n = 9) were stained with indirect immunoperoxidase method. Anti-CD9, CD26, CD29, CD31, CD36, CD44, CD49a, CD49b, CD49c, CD49d, CD49e, CD49f, CD54, CD55, CD56, CD62E & P, CD71, CD98, CD102, CD106, CD146, CD151 monoclonals and NFL-NPH, S-100 antiserums were used. The histological structures of each group of samples were identified and compared. RESULTS: Smooth muscle layers have been encountered within the walls of hernia sacs of both boys and girls. Although the hydrocele sacs have shown smooth muscle bundles distributed as patchy areas, smooth muscle bundles have been observed infrequently among sacs from patients with undescended testis. Peritoneum and processus vaginalis samples have been free of smooth muscle. CONCLUSIONS: Inguinal hernia during childhood seems to be related to the presence of smooth muscle within the wall of the sac. The smooth muscle bundles may have played a role both in prevention of obliteration and clinical outcome. Because the sacs associated with undescended testis are without smooth muscles, and herniation is not a frequent association, they may not share the same etiologic basis with inguinal hernia.
Duplicate exstrophy of the bladder is a rare, distinct congenital anomaly without additional major anomalies of the urinary tract. A 15-year-old boy with duplicate exstrophy is reported on, and the possible role of bladder sequestration in the development of classical musculoskeletal deformities in this anomaly is discussed.
PURPOSE: An experimental study was performed to evaluate the effect of fetal distress on in utero defecation and clearance of amniotic fluid (AF). METHODS: Sixteen pregnant New Zealand white rabbits underwent laparotomy at 25 days' gestation (full term, 31 to 32 days) as group A (n = 8) and B (n = 8). Uteroplacental ischemia was achieved by constriction of the aorta below the renal arteries to cause fetal distress in group B, whereas sham operation was done in group A. In both groups, 0.1 mL of technetium-99m (99mTc)-HIDA containing 1 mCi of radioactivity was injected into the gluteus muscle of each fetus, which had been exposed through the uterus. Beginning 2 hours after injection, a live fetus was killed every 2 hours for 48 hours in both groups. Tissue samples from the reference organs (lung, heart, stomach, kidney, bladder) and liver, meconium in proximal, mid and distal bowels, AF, and maternal blood were taken. The radioactivity of each sample was determined by a gamma counter and the percentage injected dose (uptake) per gram of tissue (%ID/g) was calculated. The total uptake and mean transit time (MTT) showing intestinal transport were calculated using the linear trapezoidal approximation and extrapolation. The peak concentration (Cmax, %ID/g) and time corresponding to the peak (tmax, h) were obtained. RESULTS: (1) Significant difference was noted between the groups with regard to uteroplacental perfusion pressure and blood pH (51.0+/-2.6 mm Hg; pH, 6.9+/-0.1 in group B; 80.1+/-2.7 mm Hg, pH, 7.3+/-0.1 in group A; P < .05). (2) 99mTc-HIDA was predominantly trapped by the liver and excreted into the gastrointestinal tract and AF in both groups. (3) In liver and bowel, shape of the profile was bimodal because of fetal swallowing and similar in both groups, tmax was the same in both groups, Cmax was lower in group B than in group A, the total uptake was smaller in group B than in group A, and intestinal transport time was similar (44.2 hours in group A and 43.0 hours in group B). In amniotic fluid, shape of the profile was sigmoidal in group B and reached a Cmax value of 11.6% ID/g, whereas unimodal profiles were observed in group A with a Cmax value of 12.6% ID/g; radioactivity was eliminated from the AF with a rate constant of 0.48% ID/g h in group A (AUC, 273% ID/g h); whereas accumulation of radioactivity was noted in group B (AUC, 308% ID/g h). (5) In maternal blood, shape of the profile was sigmoidal in group A with a Cmax value of 2.9% ID/g and unimodal in group B (Cmax, 1.6% ID/g), accumulation of radioactivity was noted in group A (AUC, 93% ID/g h), whereas a rapid decline of radioactivity (k, 0.06% ID/g h) was noted in group B (AUC, 47% ID/g h). CONCLUSIONS: Fetal distress did not affect the intestinal transport dynamics and in utero defecation but impaired the clearance of AF and the passage into the maternal circulation, which was shown by the accumulation of radioactivity in AF only in group B and in maternal blood only in group A without any elimination rate. This finding suggests that meconium-stained AF is not related to meconium passage after fetal distress; rather, it reflects impaired clearance of AF, which already has containing meconium caused by physiological in utero defecation.
BACKGROUND/PURPOSE: Preventing thrombus formation after caustic esophageal ingestion has been proposed to have beneficial effects. Therefore, an experimental study was carried out to investigate the effects of heparin on the esophagus after caustic burns. METHODS: Caustic esophageal burns were produced in rats by irrigation with 50% NaOH as described by Liu. Rats were divided into four groups as follows: group A, animals with esophageal burns, received placebo and underwent autopsy 48 hours after caustic injury; group B, animals with esophageal burns, received subcutaneous heparin treatment and underwent autopsy 48 hours after caustic injury; group C, animals with esophageal burns, received placebo and underwent autopsy 28 days after caustic injury; group D, animals with esophageal burns, received subcutaneous heparin treatment for 7 days and underwent autopsy 28 days after caustic injury. Histopathologic evaluation was performed in all groups, and collagen content of esophageal sections was analyzed by determination of hydroxyproline levels. RESULTS: Submucosal vascular thrombosis was encountered in all group A animals but the submucosal venules and arterioles were patent in most of group B animals. Esophageal strictures did not develop in any of group D animals, although varying degree of esophageal stenoses were encountered in all animals of group C. The circumferences of the burned segment have been narrowed to 3+/-1 mm in group C rats. There was obvious collagen deposition in submucosa, and epithelial regeneration was not complete in group C rats. Submucosa and epithelial integrity seemed normal in group D animals. Hydroxyproline contents in group D were significantly lower compared with group C (P < .05). CONCLUSIONS: Heparin has ameliorating effects on stricture formation after caustic esophageal burn. Those effects may occur through possible anticoagulant, antithrombotic, and endothelial protective effects, and modifying effects of heparin on wound healing.
PURPOSE: A retrospective clinical study was performed to find out whether serum biochemistry alterations may serve to differentiate the traumatic bladder perforation to be either intraperitoneal or extraperitoneal. METHODS: Thirty-two children treated for traumatic bladder perforation between 1970 and 1997, inclusive, formed the study group. Patients were divided into two groups: intraperitoneal bladder perforation (IBP) and extraperitoneal bladder perforation (EBP) groups. The groups were compared with regard to age, sex, mechanism of injury, and hemodynamic parameters (blood pressure, hematocrit) at presentation (each group was further subdivided into two subgroups according to the duration between trauma and presentation as early (duration <24 hours) and late (duration >24 hours) presentation groups). The subgroups were compared with each other with respect to serum concentrations of urea, creatinine, sodium, potassium, and chloride. RESULTS: There were 21 boys and 11 girls with a mean age of 8.7+/-4.9 years. Mechanisms of injury were motor vehicle accidents (75%), falls (19%), and gunshot wounds (6%). There was no significant difference between the IBP and EBP groups with regard to age, sex, mechanism of injury and hemodynamic parameters at presentation. The biochemical parameters were found to be within normal range in the EBP group, whereas significantly higher levels of creatinine, potassium, and lower level of sodium were noted in IBP group regardless of presentation time. Significantly higher level of serum urea was recorded in a late presentation group of IBP patients. CONCLUSIONS: The authors emphasize that patients presenting with IBP are more likely to present with significantly higher levels of creatinine, potassium, and lower level of sodium compared with the patients with EBP regardless of presentation time. Increased level of serum urea concentration is a characteristic of IBP patients with late presentation. Biochemical alterations can be used to differentiate traumatic IBP and EBP in children with subtle physical examination and radiological findings.
BACKGROUND/PURPOSE: Retractile testis traditionally has been regarded as suprascrotal location of testis resulting from hyperactivity of the cremasteric reflex (CR). However, the activity of CR is evaluated poorly in the literature. Electrophysiological characteristics of CR in boys with various testicular localizations have been evaluated comparatively. METHODS: Cremasteric responses that were evoked by electrical stimulation of the upper-inner aspect of thigh were recorded using a concentric needle electromyography (EMG) electrode inserted into the cremaster muscle. Evoked EMGs of groups, each consisting of 10 boys with bilateral descended (DT), unilateral retractile (RT), or unilateral undescended testes (UT) were compared for response latencies and durations. RESULTS: Three components with different onset latencies (R1, R2, and R3) were identified in EMG responses. In patients with UT, the latency of the R1 was shorter than that of the patients with DT, and the duration of R2 was longer than those of the patients with DT and RT. The differences between patients with RT and DT regarding the latency and duration of R1 and R2, although similar to those differences between UT and DT, were not significant. There were no significant differences of the latencies of R3 between groups. In patients with RT and UT, response latency and duration in the descended and retractile-undescended sides were similar. CONCLUSIONS: Defined by shortened latency and prolonged activity of cremasteric responses, which probably reflect a diminished neuronal inhibitory control, CR is hyperactive in UT. Disinhibited motor neurons leading to overactivity of cremaster muscle might have hampered the descent of testis. Our results do not provide a definite support for or against the hypothesis of CR hyperactivity in RT.
The authors present a 2-year-old boy with a skin defect located in the right lateral side of the neck. They suggest the defect is a partial failure of disappearance of the second pharyngeal (branchial) cleft and propose a name of lateral cervical cleft.
Bardet-Biedl syndrome is an autosomal recessive disorder. It is characterized by cardinal anomalies including retinal dystrophy, digital malformations, mental retardation, obesity, and hypogonadism. Recently, renal anomalies also are mentioned among the cardinal signs. Although association of genital anomalies among affected boys are well known, the association of vaginal atresia and other structural genital anomalies are not mentioned among the less-common manifestations of Bardet-Biedl syndrome in girls. Two girls with Bardet-Biedl syndrome presented with hematometrocolpos in the preadolescent period and vaginal atresia was diagnosed. After surgical treatment and extended hospitalization, uncontrolled sepsis resulted in progressive renal failure and death of both patients. Vaginal atresia is often delayed or missed in the early childhood period. In girls with Bardet-Biedl syndrome, vaginal atresia or other structural genital anomalies should be evaluated more systematically during the initial diagnosis of the syndrome. In infancy, the evaluation of a child with vaginal atresia also should include the differential diagnosis of Bardet-Biedl syndrome. Vaginal atresia may either form a component of the syndrome, or girls who present with vaginal atresia in addition to other components of Bardet-Biedl syndrome might form a distinct entity.
OBJECTIVE: To determine the changes occurring during ipsilateral spermatic cord torsion either in the presence or absence of the ipsilateral testis and epididymis, by evaluating noradrenaline and nitrite-nitrate concentrations in the contralateral testes. MATERIALS AND METHODS: Forty male albino rats were allocated randomly to one of four equal groups undergoing: group 1, a sham operation; group 2, ipsilateral spermatic cord torsion; group 3, epididymo-orchidectomy only; and group 4, spermatic cord torsion after epididymo-orchidectomy. The contralateral testes were harvested after 24 h and the noradrenaline and nitrite-nitrate contents determined. The levels in each group were compared using the Kruskal-Wallis and Mann-Whitney U-tests. RESULTS: The noradrenaline content of testes from group 2 was significantly lower than in those of groups 1 and 3, but there were no significant differences in content between groups 1 and 3, 1 and 4, and 2 and 4. The content in group 4 was significantly less than that in group 3. There were no significant differences in nitrite-nitrate contents among any of the groups. CONCLUSION: Spermatic cord torsion for 24 h, either in the presence or absence of a testis and epididymis, significantly decreased the noradrenaline content in the contralateral testis. This finding supports the suggestion that the sympathetic system is activated by exposure to noradrenaline in the contralateral testis during ipsilateral spermatic cord torsion, with no dependency on the presence of a testis and epididymis. As the nitrite-nitrate concentrations were unaffected, nitric oxide seems to have no role in contralateral testicular deterioration.
OBJECTIVE: To evaluate the contractile response of the vas deferens in a model of stress, to determine any changes in sympathetic activity as a result of stress in the ipsilateral testis, which decreases blood flow to the contralateral testis. MATERIALS AND METHODS: The study comprised two groups of six rats each; group 1 underwent a sham operation, and in group 2 the right testis was placed into the abdominal cavity and the vas deferens ligated. After 30 days, the vasa deferentia were resected bilaterally and their isometric contractions recorded. Electrical-field stimulation (EFS) was applied through a pair of platinum electrodes and concentration-response curves constructed for noradrenaline at 37 degrees C and to a solution containing 80 mmol/L K+. RESULTS: The vasa deferentia in both groups showed similar contractile responses to EFS, which were frequency-dependent and maximal at 80 Hz. Noradrenaline-induced contractile activity was lower in amplitude in the vasa deferentia of group 2 than in the contralateral and ipsilateral vasa deferentia of group 1, which were not significantly different from each other. All groups responded similarly to high K+. CONCLUSION: Intra-abdominal placement of the testes with vas deferens ligation decreased the contractile response to noradrenaline in the ipsilateral vas deferens without altering the contractile response to EFS and high K+. This difference could be caused by a reduction in the number of postjunctional alpha-adrenergic receptors or decreased receptor sensitivity. Both possibilities suggest that the vas deferens may initiate sympathetic activity, which may be responsible for contralateral testicular deterioration.
Household bleaches that contain sodium hypochlorite and sodium hydroxide are the common domestic agents used all around the world, which are frequently accidently ingested by children. Commercial household bleaches are known to have minor adverse effects when ingested, as being mucosal irritants, emetics and weak corrosives. However, our clinical observations of pneumonitis among household bleach ingested by patients without a history of aspiration foretell some possible systemic toxic effects of household bleaches. An experimental study on rats was designed to evaluate systemic effects of household bleaches that contain 4% sodium hypochlorite and less than 0.05% sodium hydroxide on lungs, livers, kidneys and intestines after 2, 4, 6, 12, 24 and 48 hours of administration via intragastric route. Prominent congestion and some interstitial mononuclear cellular infiltration were observed in the lungs, the livers and the kidneys of the rats after administration of household bleaches. Additionally, the lungs showed expansion of the alveolar spaces. While erosive changes were present in the stomachs, the intestines were normal. These histopathological changes were especially prominent at early periods of systemic administration. In the second part of the study, to assess whether these findings would hold for intravenous administration of household bleaches, another group of rats were given intravenous administration of household bleach and after 4 hours of intravenous administration of household bleach, the same histopathological changes above were observed in the lungs, kidneys and livers. The study indicates that household bleaches may have systemic effects prominent during early periods of ingestion, although the clinical importance remains to be clarified.
BACKGROUND: If an esophageal burn is diagnosed, the aim is to prevent stricture formation. The combined use of steroid, antibiotics and early bougienage (SAEB) is one of the currently used treatment protocols. The effect of SAEB treatment against stricture formation following caustic esophageal burn has been evaluated retrospectively. METHODS: Forty-nine children of 282 admitted with a history of caustic substance ingestion were found to have esophageal burns. Forty-nine children underwent treatment against stricture formation. SAEB was begun within 48 hours of ingestion. RESULTS: Eight children of 12 who ingested sodium hydroxide and five children of 20 who ingested acids developed strictures in spite of the therapy. Additionally two esophageal perforations were encountered in patients who ingested sodium hydroxide. CONCLUSIONS: This protocol has been found to carry a risk of perforation without preventing stricture formation after strong alkali ingestion. Therefore other treatment modalities for preventing strictures should be evaluated especially in children who have ingested products containing strong alkalis.
Bilateral absence of the pectoralis major muscle with accompanying abnormalities of shoulder muscles has been reported in patients without Poland anomaly (PA). However, symmetric absence of pectoralis major muscles, hypoplasia of breasts and nipples with symmetric chest wall deformity and bilateral hand anomaly has not previously been reported. A 6-year-old girl with bilateral absence of pectoralis major muscles and hand involvement and symmetric chest wall deformity is, to our knowledge, the first known case of bilateral Poland anomaly.
A newborn patient with cystic fibrosis and multiple intestinal atresias demonstrated intraluminal calcifications on a plain abdominal radiograph. Cystic fibrosis may be an aetiological factor for intraluminal calcifications secondary to multiple intestinal atresias.