To evaluate the influence of prune belly syndrome (PBS) on the development of penises in human fetuses.
We studied 39 human fetuses (including 4 with PBS) aged 11-22 weeks post conception. We measured the length and width of the free portion and penis root and the penis total length (PTL). In 21 fetuses of the control group and in 3 with PBS we analyze the penile with histochemical and immunohistochemical methods. The total penile area, area of corpora cavernosa, area of corpus spongiosum, and thickness of tunica albuginea were measured by stereological method. Means were compared using the Kolmogorov-Smirnov and ANOVA test (P <.05).
We did not observe difference in PTL (P = .999) when we compared the control group (4.69-29.77 mm, mean = 16.2 mm; SD = 6.34 mm) with the PBS (12.23-23 mm, mean = 16.16; SD = 4.99). The total penile area (P = .002), the area of corpora cavernosa (P = .023) and the area of corpus spongiosum (P = .004) had a significant increase when comparing the PBS with the control group.
In fetuses with PBS there is no change in the length of the penis but we observed a low rate of growth in all the other histologic parameters analyzed, suggesting that PBS impacts the penile development.

BACKGROUND: Prune belly syndrome (PBS) is a multisystem disease characterized by absent or deficient abdominal musculature with accompanying lax skin, urinary tract abnormalities, and cryptorchidism. Previous studies have estimated a birth prevalence of 1 in 35,000-50,000 live births.
OBJECTIVE: We set out to clarify the epidemiology and early hospital admissions of PBS in Finland through a population-based register study. Further, possible maternal risk factors for PBS were analyzed in a case-control setting.
METHODS: The Finnish Register of Congenital Malformations was linked to the Care Register for Health Care, a population-based hospital admission data for PBS patients. Additionally, five matched controls were identified in the Birth Register and maternal risk factors of PBS were studied utilizing data from the Drugs and Pregnancy database.
RESULTS: We identified 31 cases of PBS during 1993-2015, 15 of which were live born and 16 elective terminations. The total prevalence was 1 in 44,000 births. Three patients (20%) died during infancy. On average, PBS-patients had 3.2 admissions and 10.6 hospital days per year in Finland during the study period years 1998-2015, 35- and 27-fold compared to children in Finland in general. Multiple miscarriages were significantly associated to PBS in maternal risk factor analyses.
CONCLUSIONS: The burden of disease is significant in PBS, demonstrated as a high infant mortality rate (20%), multiple hospital admissions, and inpatient care in days. The available variables are limited as a register-based study.
CONCLUSIONS: We present data on contemporary epidemiology in a population-based study and show that the total prevalence of PBS is 1 in 44,000 in Finland. PBS entails a significant disease burden with admissions and hospital days over 35- and 27-fold compared to the general pediatric population, further aggravated by an infant mortality rate of 20%.

Prune belly syndrome (PBS) results in a total lack of abdominal musculature. Abdominal muscles have an important function during inspiration and expiration. This puts the patient at risk for respiratory complications since they have a very limited ability to cough up secretions. Patients in an intensive care unit (ICU) with PBS who receive mechanical ventilation are at even greater risk for respiratory complications. We review the function of the abdominal muscles in breathing and delineate why they are important in the ICU. We include an illustrative case of a long-term ventilated patient with PBS and offer respiratory management options.

BACKGROUND: To compare the growth of the prostate in anencephalic, prune belly syndrome (PBS) and control fetuses.
METHODS: We studied 35 prostates from normal human fetuses aged 11-22 weeks postconception (WPC); 15 from anencephalic fetuses aged 13-19 WPC; and 6 from PBS fetuses aged 13-31WPC. After prostate dissection, we evaluated the prostate length, width and thickness with the aid of a computer program (Image Pro and Image J). The fetal prostate volume (PV) was calculated using the ellipsoid formula: PV = [length × thickness × width] × 0.523. The prostates were dissected and the PV was measured with the aid of the same computer program. Means were statistically compared using the unpaired t-test and linear regression was performed.
RESULTS: In 2 PBS fetuses we observed prostatic atresia. We did not observe significant differences in PV when comparing the control group (PV: 6.1 to 313.81 mm, mean = 70.85 mm: SD = 71.43 mm) with anencephalic fetuses: p = 0.3575 (PV: 5.1 to 159.11 mm, mean = 42.94 mm; SD = 40.11 mm) and PBS fetuses: p > 0.999 (PV: 10.89 to 148.71 mm, mean = 55.4 mm; SD = 63.64 mm). The linear regression analysis indicated that the PV in the control group (r2 = 0.3096; p = 0.0004), anencephalic group (r2 = 0.3778; p = 0.0148) and PBS group (r2 = 0.9821; p < 0.009) increased significantly and positively with fetal age (p < 0.0001).
CONCLUSIONS: We did not observe significant differences in development of the prostate in fetuses with anencephaly and in 2/3 of fetuses with PBS during the fetal period studied. In 1/3 of the PBS fetuses, the prostate had important atresia.
METHODS: Level III.

BACKGROUND: There are no reports comparing testicular volume between normal fetuses, fetuses with prune-belly syndrome (PBS), and fetuses with anencephaly. The study hypothesis was that PBS and especially anencephaly alter the testicular volume during the human fetal period.
OBJECTIVE: The objective of the study was to compare the testicular growth in fetuses with anencephaly, with PBS, and without anomalies.
METHODS: This is a morphometric study of human fetuses. Seventy testes from fetuses without anomalies aged 11-22 weeks post-conception (WPC), 30 testes from fetuses with anencephaly aged 13-19 WPC, and eight testes from fetuses with PBS aged 13-16 WPC were studied. Testicular length, width, and thickness were evaluated with the aid of computer programs (Image Pro and ImageJ) (Figure). The fetal testicular volume was calculated using the ellipsoid formula: Testicular volume (TV) = [length × thickness × width] × 0.523. The Shapiro-Wilk test was used to ascertain the normality of the data and to compare quantitative data between normal fetuses vs. fetuses with anencephaly, while the Kruskal-Wallis test was used to assess gender and laterality differences. Simple linear correlations (LCs) were calculated for testicular volume according to fetal age, weight, and crown-rump length.
RESULTS: All 108 testes studied were abdominal. The right (p = 0.0310) and left (0.0470) testicular volumes were significantly smaller in fetuses with anencephaly than those in the control group. The linear regression analysis indicated that the right and the left testis volume in the control group (right: r2 = 0.6665; left: r2 = 0.6707) and PBS group (right: r2 = 0.9937; left: r2 = 0.9757) increased with fetal age (p < 0.0001). This analysis also indicated that the testicular volume in fetuses with anencephaly did not increase with fetal age (right: r2 = 009816; left: r2 = 0.07643).
CONCLUSIONS: This article is the first to report testicular volume correlations with fetal parameters in fetuses with anencephalic and fetuses with PBS. Significant alterations were observed in testicular growth in the anencephalic group compared with the control group, and it was also observed that the bilateral cryptorchidism in PBS does not alter the testicular development and growth during the fetal period. The unequal WPC distribution between fetuses with PBS, fetuses with anencephaly, and controls and the small sample size are limitations of this study. Further studies should be performed to confirm this study\'s findings.
CONCLUSIONS: Testicular growth is slower and does not show significant correlations with fetal parameters in fetuses with anencephalic. Significant differences in testicular development in fetuses with PBS was not observed.

To study the bladder structure of fetuses with prune belly syndrome (PBS).
We studied three bladders obtained from three male fetuses with PBS and seven bladders from seven male fetuses without anomalies. Each bladder was dissected and embedded in paraffin, from which 5 μm thick sections were obtained and stained with Masson\'s trichrome (to quantify connective tissue and smooth muscle) and picrosirius red with polarization (to observe collagen). Immunohistochemistry with tubulin (Tubulin, beta III, Mouse Monoclonal Antibody) was applied to observe the bladder nerves. The images were captured with an Olympus BX51 microscope and Olympus DP70 camera. The stereological analysis was done with the Image Pro and Image J programs, using a grid to determine volumetric densities (Vv). Means were statistically compared using the Mann-Whitney test (P < 0.05).
Quantitative analysis documented that smooth muscle fibers were significantly smaller (P = 0.04) in PBS fetuses (9.67% to 17.75%, mean = 13.2%) compared to control group (13.33% to 26.56%, mean = 17.43%). The analysis of collagen fibers showed predominance of green in the control group, suggesting collagen type III presence, and predominance of red in the in PBS fetal bladders, suggesting collagen type I presence in this group. The qualitative analysis of the nerves with immunohistochemistry with tubulin showed predominance of nerves in the control group.
The bladder in PBS had lower concentrations of smooth muscle fibers, collagen type III, and nerves. These structural alterations can be one of the factors involved in urinary tract abnormality such as distended bladder in patients with PBS.

OBJECTIVE: We compared and contrasted the structure of the gubernaculum testis in fetuses with prune belly syndrome and normal controls.
METHODS: We studied a total of 6 gubernacula from 3 male fetuses with prune belly syndrome and a total of 14 from 7 male fetuses without an anomaly. Gubernacular specimens were cut into 5 μm sections and stained with Masson trichrome to quantify connective tissue and smooth muscle cells, with Weigert stain to observe elastic fibers and with picrosirius red with polarization to observe collagen. Immunohistochemical analysis was done with tubulin to observe the nerves. Images were captured with a BX51 microscope and DP70 camera (Olympus®). Stereological analysis was done with Image-Pro and ImageJ (MediaCybernetics®) using a grid to determine volumetric density. Means were statistically compared with the Mann-Whitney test. All tests were 2-sided with p <0.05 considered statistically significant.
RESULTS: Prune belly syndrome fetuses were at 17 to 31 weeks of gestation and control fetuses were at 12 to 35 weeks of gestation. Quantitative analysis showed no difference in the volumetric density of smooth muscle cells in prune belly syndrome vs control gubernacula (mean 15.70% vs 19%, p = 0.2321). Collagen fiber analysis revealed a predominance of green areas in prune belly syndrome gubernacula, suggesting collagen type III, and a predominance of red areas in control gubernacula, suggesting collagen type I. Elastic fibers were significantly smaller in prune belly syndrome gubernacula than in control gubernacula (mean 14.06% vs 24.6%, p = 0.0190). Quantitative analysis demonstrated no difference in the volumetric density of nerves in prune belly syndrome or control gubernacula (mean 5.200% vs 3.158%, p = 0.2302).
CONCLUSIONS: The gubernaculum in fetuses with prune belly syndrome had altered concentrations of collagen and elastic fibers. These structural alterations could be one of the factors involved in cryptorchidism in prune belly syndrome.

OBJECTIVE: To determine the prevalence and spectrum of prune belly in a defined population.
METHODS: Population-based descriptive study using New York State\'s Congenital Malformations Registry.
METHODS: The Congenital Malformations Registry is a statewide registry of children diagnosed as having congenital anomalies before the age of 2 years.
METHODS: Infants with the diagnosis of prune-belly syndrome born during the years 1983 to 1989 to women who were New York State residents and verified by medical record review.
METHODS: The live birth prevalence of prune belly for the total population and for population subgroups, such as race, sex, plurality, and maternal age. The occurrence of other malformations with prune belly.
RESULTS: Sixty cases of prune belly were ascertained (50 male and 10 female). The live birth prevalence was 3.2 per 100,000 and declined over the time period. The prevalence was higher in males, 5.1 per 100,000, than females, 1.1; and higher in blacks, 5.8, than whites, 2.6. The live birth prevalence of prune belly in twins, 12.2 per 100,000, was four times higher than that found in singletons, 3.0. More than 36 (60%) of patients died, most in the first week. Forty-two (70%) of patients had one of the commonly described associated defects; pulmonary hypoplasia was the most common. Almost one third of patients had defects other than those typically associated with prune belly.
CONCLUSIONS: Twins, blacks, and children born to younger mothers appear to be at higher risk. Mortality remains high, especially early with many deaths due to pulmonary hypoplasia. Further studies should include stillborns and terminated pregnancies.

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