Effects of cofermentation of Saccharomyces cerevisiae and different lactic acid bacteria on the organic acid content, soluble sugar content, biogenic amines, phenol content, antioxidant activity and aroma of prune wine.

To determine the effect of cofermentation of Saccharomyces cerevisiae and different LABs on prune wine quality, this study compared phenolic compounds, organic acids, soluble sugars, biogenic amines and volatile flavor compounds among different treatments. The results showed that inoculation of LAB increased DPPH and total flavonoid content. Malic acid content was reduced in HS, HB and HF. Histamine content in S, F and B was lower than the limits in French and Australian wines. 15 phenolic compounds were identified. Yangmeilin and chlorogenic acid were detected only in HS, HF and HB. 51 volatile flavor compounds were identified, esters being the most diverse and abundant. 14 volatile flavor compounds with OAV > 1 contributed highly to the aroma of prune wine. 9 chemical markers including resveratrol, rutin, and catechin were screened to explain intergroup differences by OPLS-DA. This study provides new insights into the processing and quality analysis of prunes.

Prune-Belly Syndrome: An Update.

The Prune-Belly (Eagle-Barrett) syndrome (PBS) is a congenital and genetically heterogeneous disease, more prevalent in males, defined by the clinical triad (1) deficiency of abdominal muscles, (2) bilateral cryptorchidism, and (3) urinary tract abnormalities. The abdomen of an infant with PBS has a typical appearance, similar to the aspect of a prune, which gives it its name. Although the etiology of this disorder is still unknown, numerous theories, mutations, and genetic disturbances have been proposed to explain the origin of PBS. Prognosis can differ a lot from one patient to another, since this condition has a wide spectrum of clinical presentation. Despite being a rare condition, the importance of PBS should not be underestimated, in the light of the potential of the disorder to lead to chronic kidney disease and other severe complications. In that regard, this review gathers the most up-to-date knowledge about the etiopathogenesis, clinical features, diagnosis, management and prognosis of PBS.

Prune belly syndrome: A rare case report.

Key Clinical Message: In babies presenting with an omphalocele, other components of the prune belly syndrome should be scrutinized for early diagnosis and timely intervention. Abstract: A male baby on his 13th day of life presented with an omphalocele. On evaluation, he had congenital absence of left kidney and bilateral cryptorchidism. Therefore, he was diagnosed with prune belly syndrome. He responded well to abdminoplasty, and wait and watch policy was applied for his cryptorchidism.

Gut microbes differ in postmenopausal women responding to prunes to maintain hip bone mineral density.

Foods high in phenolics such as prunes have been shown to exert protective effects on bone mineral density (BMD), but only certain individuals experience these benefits. This post-hoc analysis of a 12-month randomized controlled trial aimed to identify the relationship among the gut microbiome, immune responses, and bone protective effects of prunes on postmenopausal women. Subjects who consumed 50-100 g prunes daily were divided into responders (n = 20) and non-responders (n = 32) based on percent change in total hip bone mineral density (BMD, ≥1% or ≤-1% change, respectively). DXA scans were used to determine body composition and BMD. Immune markers were measured using immunoassays and flow cytometry. Targeted phenolic metabolites were analyzed using ultra performance liquid chromatography-tandem mass spectrometry. The fecal microbiota was characterized through 16S rRNA gene PCR amplicon sequencing. After 12 months of prune consumption, anti-inflammatory markers showed responders had significantly lower levels of IL-1ß and TNF-α. QIIME2 sequence analysis showed that microbiomes of responders and non-responders differed in alpha (Shannon and Faith PD, Kruskal-Wallis p < 0.05) and beta diversity (unweighted Unifrac, PERMANOVA p < 0.04) metrics both before and after prune treatment. Furthermore, responders had a higher abundance of bacterial families Oscillospiraceae and Lachnospiraceae (ANCOM-BC p < 0.05). These findings provide evidence that postmenopausal women with initial low BMD can benefit from prunes if they host certain gut microbes. These insights can guide precision nutrition strategies to improve BMD tailored to diet and microbiome composition.

Characterization of differences in physicochemical properties, volatile organic compounds and non-volatile metabolites of prune wine by inoculation of different lactic acid bacteria during malolactic fermentation.

To investigate the effects of inoculating with three strains of lactic acid bacteria on prune wine quality during malolactic fermentation, this study determined its antioxidant activity, phenolic compounds, organic acids, and volatile/non-volatile metabolites. The results showed that inoculation with Lactobacillus paracasei SMN-LBK improved the antioxidant activity and phenolic compounds of prune wine. 73 VOCs were detected in prune wine by HS-SPME-GC-MS, and VOC content increased by 4.3% and 9.1% in MLFS and MLFB, respectively. Lactobacillus delbrueckii subsp. Bulgaricus showed better potential for winemaking, and citral and 5-nonanol, were detected in the MLF samples. 39 shared differential metabolites were screened and their metabolic pathways were investigated based on nontargeted metabolomics. Differences in amino acid and flavonoid content between strains reflected their specificity in flavonoid biosynthesis and amino acid biosynthesis. These findings will provide useful information for the biochemical study and processing of prune wine.

Targeting Group 3 Medulloblastoma by the Anti-PRUNE-1 and Anti-LSD1/KDM1A Epigenetic Molecules.

Medulloblastoma (MB) is a highly malignant childhood brain tumor. Group 3 MB (Gr3 MB) is considered to have the most metastatic potential, and tailored therapies for Gr3 MB are currently lacking. Gr3 MB is driven by PRUNE-1 amplification or overexpression. In this paper, we found that PRUNE-1 was transcriptionally regulated by lysine demethylase LSD1/KDM1A. This study aimed to investigate the therapeutic potential of inhibiting both PRUNE-1 and LSD1/KDM1A with the selective inhibitors AA7.1 and SP-2577, respectively. We found that the pharmacological inhibition had a substantial efficacy on targeting the metastatic axis driven by PRUNE-1 (PRUNE-1-OTX2-TGFß-PTEN) in Gr3 MB. Using RNA seq transcriptomic feature data in Gr3 MB primary cells, we provide evidence that the combination of AA7.1 and SP-2577 positively affects neuronal commitment, confirmed by glial fibrillary acidic protein (GFAP)-positive differentiation and the inhibition of the cytotoxic components of the tumor microenvironment and the epithelial-mesenchymal transition (EMT) by the down-regulation of N-Cadherin protein expression. We also identified an impairing action on the mitochondrial metabolism and, consequently, oxidative phosphorylation, thus depriving tumors cells of an important source of energy. Furthermore, by overlapping the genomic mutational signatures through WES sequence analyses with RNA seq transcriptomic feature data, we propose in this paper that the combination of these two small molecules can be used in a second-line treatment in advanced therapeutics against Gr3 MB. Our study demonstrates that the usage of PRUNE-1 and LSD1/KDM1A inhibitors in combination represents a novel therapeutic approach for these highly aggressive metastatic MB tumors.

Novel minimally invasive abdominoplasty for selected cases of prune belly syndrome: Step-by-step technique description and clinical indications.

BACKGROUND: Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, bilateral undescended testicles and genitourinary tract anomalies. A variable spectrum of abdominal wall laxity is observed in PBS. We present the first case of a novel technique using a minimally invasive abdominoplasty to specifically address patients with localized abdominal wall weakness in PBS. CASE PRESENTATION: A two-years-old child with PBS presented with recurrent febrile urinary tract infections. Ultrasonography demonstrated a dysplastic right kidney associated with significant ipsilateral ureterohydronephrosis. Voiding urethrocystogram did not show vesicoureteral reflux and DMSA scan depicted a non-functioning right kidney. During laparoscopic right nephroureterectomy and first stage Fowler-Stephens bilateral orchiopexies, a significant right-sided lateral abdominal wall bulging was observed. A minimally invasive laparoscopic abdominoplasty was performed with a one-way running suture using an unabsorbable 2.0 prolene approximating the edges of the musculofascial defect. While undergoing the second-stage Fowler-Stephens orchiopexy, no bulging was observed. CONCLUSION: A minimally invasive abdominoplasty to improve abdominal wall lateral bulging in PBS was feasible and presented good cosmetic result. We anticipate that this technique can be applied for children with PBS with primary lateral abdominal wall bulging, employing one or more suture lines depending on the fascial defect size.

Median quartet tree search algorithms using optimal subtree prune and regraft.

Gene trees can be different from the species tree due to biological processes and inference errors. One way to obtain a species tree is to find one that maximizes some measure of similarity to a set of gene trees. The number of shared quartets between a potential species tree and gene trees provides a statistically justifiable score; if maximized properly, it could result in a statistically consistent estimator of the species tree under several statistical models of discordance. However, finding the median quartet score tree, one that maximizes this score, is NP-Hard, motivating several existing heuristic algorithms. These heuristics do not follow the hill-climbing paradigm used extensively in phylogenetics. In this paper, we make theoretical contributions that enable an efficient hill-climbing approach. Specifically, we show that a subtree of size m can be placed optimally on a tree of size n in quasi-linear time with respect to n and (almost) independently of m. This result enables us to perform subtree prune and regraft (SPR) rearrangements as part of a hill-climbing search. We show that this approach can slightly improve upon the results of widely-used methods such as ASTRAL in terms of the optimization score but not necessarily accuracy.

Prunes preserve cortical density and estimated strength of the tibia in a 12-month randomized controlled trial in postmenopausal women: The Prune Study.

Non-pharmacological therapies, such as whole-food interventions, are gaining interest as potential approaches to prevent and/or treat low bone mineral density (BMD) in postmenopausal women. Previously, prune consumption preserved two-dimensional BMD at the total hip. Here we demonstrate that prune consumption preserved three-dimensional BMD and estimated strength at the tibia. PURPOSE: Dietary consumption of prunes has favorable impacts on areal bone mineral density (aBMD); however, more research is necessary to understand the influence on volumetric BMD (vBMD), bone geometry, and estimated bone strength. METHODS: This investigation was a single center, parallel arm 12-month randomized controlled trial (RCT; NCT02822378) to evaluate the effects of 50 g and 100 g of prunes vs. a Control group on vBMD, bone geometry, and estimated strength of the radius and tibia via peripheral quantitative computed tomography (pQCT) in postmenopausal women. Women (age 62.1 ± 5.0yrs) were randomized into Control (n = 78), 50 g Prune (n = 79), or 100 g Prune (n = 78) groups. General linear mixed effects (LME) modeling was used to assess changes over time and percent change from baseline was compared between groups. RESULTS: The most notable effects were observed at the 14% diaphyseal tibia in the Pooled (50 g + 100 g) Prune group, in which group × time interactions were observed for cortical vBMD (p = 0.012) and estimated bone strength (SSI; p = 0.024); all of which decreased in the Control vs. no change in the Pooled Prune group from baseline to 12 months/post. CONCLUSION: Prune consumption for 12 months preserved cortical bone structure and estimated bone strength at the weight-bearing tibia in postmenopausal women.

Corrigendum: Neuroimaging in PRUNE1 syndrome: a mini-review of the literature.

[This corrects the article DOI: 10.3389/fneur.2023.1301147.].

PRUNE1 and NME/NDPK family proteins influence energy metabolism and signaling in cancer metastases.

We describe here the molecular basis of the complex formation of PRUNE1 with the tumor metastasis suppressors NME1 and NME2, two isoforms appertaining to the nucleoside diphosphate kinase (NDPK) enzyme family, and how this complex regulates signaling the immune system and energy metabolism, thereby shaping the tumor microenvironment (TME). Disrupting the interaction between NME1/2 and PRUNE1, as suggested, holds the potential to be an excellent therapeutic target for the treatment of cancer and the inhibition of metastasis dissemination. Furthermore, we postulate an interaction and regulation of the other Class I NME proteins, NME3 and NME4 proteins, with PRUNE1 and discuss potential functions. Class I NME1-4 proteins are NTP/NDP transphosphorylases required for balancing the intracellular pools of nucleotide diphosphates and triphosphates. They regulate different cellular functions by interacting with a large variety of other proteins, and in cancer and metastasis processes, they can exert pro- and anti-oncogenic properties depending on the cellular context. In this review, we therefore additionally discuss general aspects of class1 NME and PRUNE1 molecular structures as well as their posttranslational modifications and subcellular localization. The current knowledge on the contributions of PRUNE1 as well as NME proteins to signaling cascades is summarized with a special regard to cancer and metastasis.

Fertility prospects for the prune-belly patient: A scoping review.

INTRODUCTION: With advances in medical care and assisted reproductive technologies (ART), fertility prospects for prune-belly syndrome (PBS) men may be changing. This review aims to identify the factors influencing fertility and optimization of reproductive health for PBS patients. MATERIAL AND METHODS: A scoping review was performed on all records published over 70 years (1952-2022) analyzing fertility in PBS males. Records were summarized in a table and narrative describing cryptorchidism, orchiopexy, testicle histology; prostate characteristics; sex hormone function; semen analyses, ART, and conception ability. This review was registered on Open Science Framework (OSF) and conducted using PRISMA methodology. RESULTS: 827 articles were identified and 83 were selected for data extraction. Before 2000, there were 0.85 publications/year whereas after 2000 there were 1.95 publications/year. Orchiopexy successfully relocated 86 % of PBS testicles into the scrotum. Testicular histology demonstrated 50 % of patients had no spermatogonia, while 47.2 % and 2.7 % had reduced or normal numbers respectively. Leydig hyperplasia and Sertoli only histology were found in 19.4 % of patients. Prostatic hypoplasia and prostatic urethral dilation were found in 93.6 % and 91.4 % of patients respectively. Testosterone, Luteinizing hormone (LH) and Follicle-stimulating hormone (FSH) were normal in 93.9 %, 87.7 % and 77.9 % of patients respectively. Azoospermia and oligospermia was found in 75.7 % and 21.6 % of patients respectively while 60.7 % had antegrade ejaculation. ART successfully extracted sperm in 6 instances and resulted in 4 conceptions, while natural conception was reported twice. CONCLUSIONS: Data analysis indicates increased attention to fertility prospects for PBS males with evaluation of PBS patient's hormonal function, semen analyses, ART, and conception ability. The reviewed data suggest that PBS males may father biological offspring with contemporary management and also demonstrate the need for consistent reproductive management approaches to maximize their fertility prospects.

Alteraciones de la pared abdominal: onfalocele / Abdominal wall defects: omphalocele

Introducción: los defectos de la pared son malformaciones congénitas con herniación de algunos órganos de la cavidad abdominal, como es el onfalocele. La prevalencia calculada es 1/10.000 nacimientos en países occidentales, en Colombia se desconoce. El diagnóstico es pre o posnatal requiriendo una serie de exámenes clínicos sistémicos, evaluación diagnóstica secundaria y búsqueda de anomalías asociadas; el tratamiento ha mejorado las tasas de supervivencia entre 70 y 95%. Objetivo: dar a conocer la patología y correlacionar los hallazgos genéticos, ambientales, clínicos y exámenes complementarios para el diagnóstico oportuno, derivando así al paciente a un tratamiento óptimo con disminución de la mortalidad. Materiales y métodos: revisión actualizada de la literatura utilizando buscadores Pubmed, UpToDate y ClinicalKey con énfasis en revisiones sistemáticas, casos clínicos y principales guías clínicas internacionales. Después se envió al jefe del departamento de cirugía pediátrica y a la división de publicaciones para su conocimiento, revisión y aprobación. Resultados: se analizaron 17 artículos publicados en los últimos 5 años, seleccionando los más relevantes y con evidencia clínica actual. Discusión y conclusiones: los estudios recientes han evidenciado nuevos hallazgos que han mejorado la supervivencia y reducido la mortalidad en los últimos 50 años.

Introduction: abdominal wall defects are congenital malformations associated with herniated abdominal organs, such as omphalocele. Its estimated prevalence is 1 per 10.000 live births in western countries. In Colombia the prevalence of omphalocele remains unknown. Omphalocele may be pre or postnatally diagnosed. A series of systemic clinical exams, secondary diagnostic evaluation and assessment for accompanying anomalies, are necessary. Treatment has improved survival rate to 70 and 95%. Objective: to increase awareness of this anomaly and correlate genetic, environmental and clinical findings and complementary exams to enable the early diagnosis and referral of these patients to receive optimal treatment which will reduce mortality. Materials and methods: updated literature review using Pubmed, UpToDate and ClinicalKey search engines, focused on systematic reviews, clinical cases and main international clinical practice guidelines. Found data was submitted to the head of the pediatric surgery department and to the publications division for their information, review and approval Results: 17 articles published in the last 5 years including the most relevant which contained current clinical evidence, were selected. Discussion and conclusions: recent studies have evidenced new findings that have improved survival and reduced mortality in the last 50 years.

Challenges in the surgical treatment of a 8-year old child living with Prune Belly syndrome: case report / Desafios no tratamento tardio da síndrome de Prune Belly: relato de caso

Prune Belly syndrome, also known as Eagle-Barret syndrome, is a rare disease, with a prevalence of 3.8 live births per 100,000 births. Its main characteristic is the hypoplasia of the abdominal muscles, giving rise to the name "prune belly syndrome". The gold standard treatment is surgery, ideally with correction of cryptorchidism and phimosis between 6 to 18 months of life. Correction of urinary malformations and abdominoplasty should be performed up to 4 years of age. Little evidence exists in the literature about late treatment and its implications for prognosis. The context mentioned above led us to present an uncommon case of a six years old child in which a surgical approach was performed later than usual.

Introdução: A síndrome de Prune Belly é uma doença rara, com prevalência de 3,8 nascidos vivos a cada 100 mil nascimentos. Tem como principal caraterística a hipoplasia da musculatura abdominal gerando a origem do nome "síndrome da barriga de ameixa". O tratamento padrão ouro é cirúrgico, idealmente com correção da criptorquidia e fimose entre 6 a 18 meses e a correção das malformações urinárias e abdominoplastia devem ser realizadas até os 4 anos. Aqui descreve-se um caso ainda menos comum, em que foi realizada abordagem cirúrgica de forma mais tardia que o habitual. Há pouca evidência na literatura acerca do tratamento tardio e suas implicações em relação ao prognóstico.

Trasplante Renal en un paciente con Síndrome de Down, Seudo Prune Belly y un trastorno linfoproliferativo post-trasplante: Primer reporte de un caso en Argentina / Kidney trasplantation in a female infant with Down syndrome, Pseudo Prune Belly Syndrome, and a post-transplant lymphoproliferative disorder. First case report in Argentina

RESUMEN Introducción: Los pacientes con trisomía 21 tienen un mayor riesgo de anomalías congénitas, incluidas las anomalías renales y de vías urinarias. La ocurrencia del Síndrome de Prune Belly y el Síndrome de Down ha sido descrita, pero representaría una coincidencia. Existen escasos reportes que describan estos pacientes y que fueran trasplantados. Caso Clínico: Paciente de 5 años con diagnóstico prenatal de trisomía 21. La ecografía postnatal reveló megavejiga y riñones displásicos. Comenzó con diálisis peritoneal a los 5 meses de vida. Se realizó el trasplante renal a los 3 años de edad, complicando con trombosis aguda de la arteria y pérdida del injerto. Cuatro meses después del segundo trasplante, la paciente fue diagnosticada con un trastorno linfoproliferativo post-trasplante.Conclusión: Se describe la evolución y el manejo de un único paciente que presentó múltiples complicaciones, con la esperanza de contribuir al conocimiento existente en relación con los pacientes trasplantados renales con síndrome de Down.

ABSTRACT Introduction: Patients with trisomy 21 have a higher risk for congenital anomalies including congenital anomalies of the kidney and urinary tract. The association between Prune Belly Syndrome and Down Syndrome has been described but the occurrence of both conditions would likely represent a coincidence. There are few published reports on renal transplantation in patients with this syndromes. Clinical Case: We reporte a 5-year-old female patient with antenal diagnosis of down syndrome. Post-natal abdominal ultrasound revealed megabladder and dysplastic kidneys. At five months of age, she was commenced on peritoneal dialysis. The patient underwent renal transplantation at age of 3. Acute thrombosis of transplanted renal artery was diagnosed, resulting in graft loss. Four months after second transplantation, the patient presented bilateral tonsillar enlargement and a post-transplant lymphoproliferative disorder was diagnosed. Conclusion: We describe the existence of these conditions in a single patient who underwent kidney transplantation, it's clinical manement and follow up. This case has been reported with the hope of contributing to the existing knowledge which pertains to kidney transplantation patients with Down syndrome.

Neuroimaging in PRUNE1 syndrome: a mini-review of the literature.

Prune exopolyphosphatase 1 (PRUNE1) is a short-chain phosphatase that is part of the aspartic acid-histidine-histidine (DHH) family of proteins. PRUNE1 is highly expressed in the central nervous system and is crucially involved in neurodevelopment, cytoskeletal rearrangement, cell migration, and proliferation. Recently, biallelic PRUNE1 variants have been identified in patients with neurodevelopmental disorders, hypotonia, microcephaly, variable cerebral anomalies, and other features. PRUNE1 hypomorphic mutations mainly affect the DHH1 domain, leading to an impactful decrease in enzymatic activity with a loss-of-function mechanism. In this review, we explored both the clinical and radiological spectrum related to PRUNE1 pathogenic variants described to date. Specifically, we focused on neuroradiological findings that, together with clinical phenotypes and genetic data, allow us to best characterize affected children with diagnostic and potential prognostic implications.

Glucocorticoid-induced osteoporosis is prevented by dietary prune in female mice.

Glucocorticoid-induced osteoporosis (GIO) is a significant side effect of prolonged glucocorticoid (GC) treatment. Chronic GC treatment also leads to trabecular bone loss and gut microbiota dysbiosis in mice. The gut dysbiosis is mechanistically linked to GIO, which indicates that the microbiota can be targeted to prevent GIO. Prunes, a dried fruit and prebiotic, have emerged in the literature as an effective treatment for sex-steroid deficiency induced osteoporosis (primary osteoporosis). Prunes also significantly alter the composition of the gut microbiota in both rodent models and human studies. Therefore, we tested if dietary prune (DP) supplementation could prevent GC-induced bone loss and affect microbiota composition in an established model of GIO. Sixteen-week-old, skeletally mature, female C57BL/6J mice were treated with a subcutaneous 5 mg placebo or prednisolone pellet for 8 weeks and fed an AIN-93M control diet or a diet modified to include 5, 15, or 25% (w/w) dried California prune powder. As expected, GC treated mice developed significant trabecular bone loss in the distal femur. More importantly, as little as 5% DP supplementation effectively prevented trabecular bone loss. Further, dose dependent increases in trabecular bone volume fraction were observed in GC + 15% and GC + 25% DP mice. Amazingly, in the placebo (non-GC treated) groups, 25% DP supplementation caused a ∼3-fold increase in distal femur trabecular bone volume fraction; this sizable bone response has not been previously observed in healthy mice with gut targeted natural treatments. Along with the striking effect on bone health, GC treatment and 25% DP supplementation led to drastic shifts in gut microbiota composition and several specific changes are strongly associated with bone health. Taken together, these results are the first to demonstrate that DP supplementation effectively prevents the negative effects of prolonged GC therapy on trabecular bone health and strongly associates with shifts in the composition of the gut microbiota.

Embarazo Gemelar con Discrepancia Morfologíca. Complejo Malformación y Obstrucción Uretral: diagnóstico prenatal / Twin Pregnancy with Morphologic Discrepancy. Complex Malformation and Urethral Obstruction: prenatal diagnosis

Presentamos un caso de embarazo gemelar, monocorial-bianmiotico con discrepancias morfológicas, diagnóstico de malformación y obstrucción uretral prenatal y diagnóstico postnatal de Síndrome de Prune-Belly. Feto A: Dilatación macroscópica del sistema colector con vejiga grande con paredes finas, hidroureter bilateral , dilatación de la uretra, parenquima renal aumentado de tamaño. Feto B: sin alteraciones morfológicas. Postnatal se observó en feto A abdomen flácido y pastoso, ano imperforado, megavejiga e insuficiencia renal

We present a case of twin pregnancy, monocorial-bi-mixtic with morphological discrepancies, diagnosis of malformation and prenatal urethral obstruction and postnatal diagnosis of Prune-Belly syndrome. Fetus A: Macroscopic dilation of the collecting system with large bladder with fine walls, bilateral hydroureter, dilation of the urethra, renal parenchymal increased in size. Fetus B: without morphological alterations. Postnatal was observed in fetus at flaccid and pasty abdomen, imperforated anus, megavejiga and renal failure.

Pregnant patient with prune belly syndrome: case report

ABSTRACT Prune belly syndrome is a rare congenital disease of unknown etiology that is present in one in every 40 thousand live births, and predominantly affects males, at a ratio of 4:1. In males, it presents with anomalies in the urinary system, absence of abdominal muscles, bilateral cryptorchidism, and infertility. In women, the syndrome has variable presentations, but fertility is preserved. Searching the medical literature, we found only one case of prune belly syndrome in pregnant women. Therefore, the patient in this report is the second case. She was primiparous, 25-years-old, with no abdominal muscles, severe congenital kyphoscoliosis, and pulmonary restriction. Elective cesarean section was performed at 37 weeks of gestation due to maternal risk of uterine rupture by transverse presentation and fetal risk of intrauterine growth restriction. The pre-anesthetic approach defined that general anesthesia might have more risks for the patient due to severe maternal lung disease compared to ultrasound-guided locoregional anesthesia. During prenatal care, there were some maternal complications, such as asthma exacerbations, abdominal pain, and constipation. The newborn was born small for gestational age and this can possibly be explained by maternal restrictive lung capacity. The newborn presented with Apgar score 8/9 and tachypnea, but improved after two hours of life.

Síndrome de Prune-Belly. Presentación de un caso / Prune-Belly Syndrome. Presentation of a case / Síndrome de Prune-Belly. Apresentação de um caso

RESUMEN Introducción: el síndrome Prune-Belly (SPB) o síndrome de "abdomen en ciruela pasa", también conocido como el síndrome de Eagle Barrett, es una forma de uropatía obstructiva fetal (UOF). Su incidencia se calcula entre 1/40 000 y 1/50 000 nacidos vivos. Presentación de caso: es un caso de síndrome de Prune-Belly en un lactante de 36 días de nacido, parto extrahospitalario. Clínicamente presentó abdomen distendido con piel arrugada o en ciruela pasa, criptorquidia bilateral, signos y síntomas de insuficiencia respiratoria. En la ultrasonografía se constató la ausencia de musculatura en la pared abdominal, megacisto, dilatación quística de los uréteres, riñones con cambios quísticos y displásicos. Discusión: en cuanto a la supervivencia, en numerosos estudios se encuentra que el porcentaje de mortalidad en los primeros meses de vida es del 27%. Está caracterizado por una clásica tríada: ausencia congénita de la musculatura de la pared abdominal; anormalidades del tracto urinario y criptorquidia bilateral, descrita en la literatura revisada, lo que coincide con las características clínicas del caso presentado,con una proporción masculino femenino de 20:1. El mayor riesgo se ha descrito en gemelos y afroamericanos. Conclusiones: está caracterizado por una clásica tríada: ausencia congénita de la musculatura de la pared abdominal; anormalidades del tracto urinario y criptorquidia bilateral. Es más frecuente en el sexo masculino. El diagnóstico del SPB puede realizarse durante la gestación a través de la ecografía obstétrica. La mortalidad en los primeros meses de vida es alta.

ABSTRACT Introduction: Prune-Belly syndrome (PBS) or "prune abdomen" syndrome, also known as Eagle Barrett syndrome, is a form of fetal obstructive uropathy (UOF). Its incidence is estimated between 1/40,000 and 1/50,000 live births. Case presentation: it is a case of Prune-Belly syndrome in a 36-day-old infant, out-of-hospital delivery. Clinically, she presented a distended abdomen with wrinkled or prune skin, bilateral cryptorchidism, signs and symptoms of respiratory failure. Ultrasonography revealed the absence of musculature in the abdominal wall, megacyst, cystic dilatation of the ureters, kidneys with cystic and dysplastic changes. Discussion: regarding survival, numerous studies have found that the percentage of mortality in the first months of life is 27%. It is characterized by a classic triad: congenital absence of the muscles of the abdominal wall; urinary tract abnormalities and bilateral cryptorchidism, described in the reviewed literature, which coincides with the clinical characteristics of the case presented, with a male-female ratio of 20: 1. The highest risk has been described in twins and African Americans. Conclusions: it is characterized by a classic triad: congenital absence of the muscles of the abdominal wall; urinary tract abnormalities and bilateral cryptorchidism. It is more common in males. The diagnosis of PBS can be made during pregnancy through obstetric ultrasound. Mortality in the first months of life is high.

RESUMO Introdução: A síndrome de Prune-Belly (PBS) ou síndrome do "abdômen de ameixa", também conhecida como síndrome de Eagle Barrett, é uma forma de uropatia obstrutiva fetal (UOF). Sua incidência é estimada entre 1 / 40.000 e 1 / 50.000 nascidos vivos. Apresentação do caso: trata-se de um caso de síndrome de Prune-Belly em lactente de 36 dias, parto fora do hospital. Clinicamente, apresentava abdome distendido com pele enrugada ou ameixada, criptorquidia bilateral, sinais e sintomas de insuficiência respiratória. A ultrassonografia revelou ausência de musculatura na parede abdominal, megacisto, dilatação cística dos ureteres, rins com alterações císticas e displásicas. Discussão: em relação à sobrevida, vários estudos constataram que o percentual de mortalidade nos primeiros meses de vida é de 27%. É caracterizada por uma tríade clássica: ausência congênita dos músculos da parede abdominal; anormalidades do trato urinário e criptorquidia bilateral, descritos na literatura revisada, que coincidem com as características clínicas do caso apresentado, com relação homem-mulher de 20: 1. O maior risco foi descrito em gêmeos e afro-americanos. Conclusões: caracteriza-se por uma tríade clássica: ausência congênita dos músculos da parede abdominal; anormalidades do trato urinário e criptorquidia bilateral. É mais comum em homens. O diagnóstico de PBS pode ser feíta durante a gravidez por meio de ultrassom obstétrico. A mortalidade nos primeiros meses de vida é alta.