The risk of urinary infection in Prune-Belly syndrome in children – case study

The risk of urinary infection in Prune-Belly syndrome in children – case study

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Title: The risk of urinary infection in Prune-Belly syndrome in children – case study
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Article_Title: The risk of urinary infection in Prune-Belly syndrome in children – case study
Authors: Viorica Leordean, Lazar Dorin
Affiliation: “Vasile Goldis” Western University, Arad, Romania
Clinic of Pediatrics II, Faculty of Medicine, Pharmacy and Dental Medicine
Abstract: The author presents a case study on the evolution of three children enrolled in Prune-Belly syndrome. This syndrome is a complex dysplasia, a rare pathology in children, characterized by the triad – the classic – hypo- or aplasia of righteous abdominal, cryptorchidism, abnormality of the urinary tract; also, it can be associated with pulmonary, cardiac, digestive, osteoarticular, and other malformations. Diagnostic criteria and etiopathogeny aspects are presented showing embryopathy and X-linked hereditary transmission theories as the most plausible. Analyzing therapeutic aspects, it is stressed that medical treatment precedes or follows surgery, which cannot resolve urinary infection unless dysplastic urinary reconstruction is performed. Serious forms of Prune-Belly syndrome have a development and poor prognosis. Intrauterine and neonatal mortality is 20% and 50% in the first 2 years of life. The risk of urinary infection and / or lungs burdens the patient’s clinical condition, allowing further appreciation on evolution of the disease. For cases solvable by plastic surgical reconstruction, as those who respond to medical therapy, differentiation will be monitored in territory and check-ups by the specialized consulting room from Policlinic Health Center. Urinary infection relapse danger is permanent, requiring differentiated supervision. This three cases interest practitioners, by at least two aspects: the rarity of the disease, and complexity of dysplasia constituent, which has serious implications on the body economy.
Keywords: urinary infection, prune-belly syndrome
References: Cromie WJ. Implications of antenatal ultrasound screening in the incidence of major genitourinary malformations. Semin Pediatr Surg. Nov 2001;10(4):204-11.
Laborie LB, Mackay DJ, Temple IK, Molven A, Søvik O, Njølstad PR. DNA hypomethylation, transient neonatal diabetes, and prune belly sequence in one of two identical twins. Eur J Pediatr. Jun 13 2009;
Kaefer M, Peters CA, Retik AB, Benacerraf BB. Increased renal echogenicity: a sonographic sign for differentiating between obstructive and nonobstructive etiologies of in utero bladder distension. J Urol. Sep 1997;158(3 Pt 2):1026-9.
Weiner Z, Goldstein I, Bombard A, Applewhite L, Itzkovits-Eldor J. Screening for structural fetal anomalies during the nuchal translucency ultrasound examination. Am J Obstet Gynecol. Aug 2007;197(2):181.e1-5.
Arrant B.S., Developmental patterns of renal functional maturation compared in the human neonate, J Pediatr, 1979, 92, 705
Aschcraft KW, Holder TM, Pediatric Surgery, 2nd Ed, WB Saunders Co, Philadelphia, 1993, 721-737
Behrman RE, Klingman RM, Awin A, Nelson’s Textbook ofPediatrics, 15 th Ed,WB Saunders Co, Philadelphia, 1996, 1539-1540
Woods AG, Brandon DH. Prune belly syndrome. A focused physical assessment. Adv Neonatal Care. Jun 2007;7(3):132-43; quiz 144-5.
Wheatley JM, Stephens FD, Hutson JM. Prune-Belly syndrome: ongoing controversies regarding pathogenesis and management. Semin Pediatr Surg. May 1996;5(2):95-106.
Lockhart JL, Reeve HR, Bredsel JJ, Kruegtr RP, Siblings with prune-belly syndrome and associated pulmonic stenosis, mental retardation and deafness, Urology, 14, 1979, 140-144
Oski F, Principles and Practice of Pediatrics, 2nd Ed, JB Lippincott Co,Philadelphia, 1994, 1768
Panayotis PK, Lowell RK, Bellman AB, Clinical Pediatric Urologv. Vol 2,3,Ed WB Saunders Co, Philadelphia, 1992, 943-973
Usick AV, Mendelian Inheritance in Man, Vol 2,11th Ed, The Johns Hopkins University press , Baltimore, 1994,2147
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Article Title: The risk of urinary infection in Prune-Belly syndrome in children – case study
Authors: Viorica Leordean, Lazar Dorin
Affiliation: “Vasile Goldis” Western University, Arad, Romania
Clinic of Pediatrics II, Faculty of Medicine, Pharmacy and Dental Medicine
Abstract: The author presents a case study on the evolution of three children enrolled in Prune-Belly syndrome. This syndrome is a complex dysplasia, a rare pathology in children, characterized by the triad – the classic – hypo- or aplasia of righteous abdominal, cryptorchidism, abnormality of the urinary tract; also, it can be associated with pulmonary, cardiac, digestive, osteoarticular, and other malformations. Diagnostic criteria and etiopathogeny aspects are presented showing embryopathy and X-linked hereditary transmission theories as the most plausible. Analyzing therapeutic aspects, it is stressed that medical treatment precedes or follows surgery, which cannot resolve urinary infection unless dysplastic urinary reconstruction is performed. Serious forms of Prune-Belly syndrome have a development and poor prognosis. Intrauterine and neonatal mortality is 20% and 50% in the first 2 years of life. The risk of urinary infection and / or lungs burdens the patient’s clinical condition, allowing further appreciation on evolution of the disease. For cases solvable by plastic surgical reconstruction, as those who respond to medical therapy, differentiation will be monitored in territory and check-ups by the specialized consulting room from Policlinic Health Center. Urinary infection relapse danger is permanent, requiring differentiated supervision. This three cases interest practitioners, by at least two aspects: the rarity of the disease, and complexity of dysplasia constituent, which has serious implications on the body economy.
Keywords: urinary infection, prune-belly syndrome
References: Cromie WJ. Implications of antenatal ultrasound screening in the incidence of major genitourinary malformations. Semin Pediatr Surg. Nov 2001;10(4):204-11.
Laborie LB, Mackay DJ, Temple IK, Molven A, Søvik O, Njølstad PR. DNA hypomethylation, transient neonatal diabetes, and prune belly sequence in one of two identical twins. Eur J Pediatr. Jun 13 2009;
Kaefer M, Peters CA, Retik AB, Benacerraf BB. Increased renal echogenicity: a sonographic sign for differentiating between obstructive and nonobstructive etiologies of in utero bladder distension. J Urol. Sep 1997;158(3 Pt 2):1026-9.
Weiner Z, Goldstein I, Bombard A, Applewhite L, Itzkovits-Eldor J. Screening for structural fetal anomalies during the nuchal translucency ultrasound examination. Am J Obstet Gynecol. Aug 2007;197(2):181.e1-5.
Arrant B.S., Developmental patterns of renal functional maturation compared in the human neonate, J Pediatr, 1979, 92, 705
Aschcraft KW, Holder TM, Pediatric Surgery, 2nd Ed, WB Saunders Co, Philadelphia, 1993, 721-737
Behrman RE, Klingman RM, Awin A, Nelson’s Textbook ofPediatrics, 15 th Ed,WB Saunders Co, Philadelphia, 1996, 1539-1540
Woods AG, Brandon DH. Prune belly syndrome. A focused physical assessment. Adv Neonatal Care. Jun 2007;7(3):132-43; quiz 144-5.
Wheatley JM, Stephens FD, Hutson JM. Prune-Belly syndrome: ongoing controversies regarding pathogenesis and management. Semin Pediatr Surg. May 1996;5(2):95-106.
Lockhart JL, Reeve HR, Bredsel JJ, Kruegtr RP, Siblings with prune-belly syndrome and associated pulmonic stenosis, mental retardation and deafness, Urology, 14, 1979, 140-144
Oski F, Principles and Practice of Pediatrics, 2nd Ed, JB Lippincott Co,Philadelphia, 1994, 1768
Panayotis PK, Lowell RK, Bellman AB, Clinical Pediatric Urologv. Vol 2,3,Ed WB Saunders Co, Philadelphia, 1992, 943-973
Usick AV, Mendelian Inheritance in Man, Vol 2,11th Ed, The Johns Hopkins University press , Baltimore, 1994,2147
*Correspondence: