Journal Archives - Volume 8
Abstract: Male hypogonadism may be defined as the defective function of the testicular compartments. Since the functional importance of each testicular cell population varies along foetal and postnatal life, the clinical manifestations of male hypogonadism may differ according to age. In this chapter, the authors have focussed on the aetiology, pathophysiology, clinical manifestations , diagonostic approach and management of male hypogonadism during infancy, childhood and early stages of puberty. An elaborative discussion on the male hypogonadal disorders in patients with male genitila is done.
Abstract: Renal scarring and renal failure are important issues over years in spina bifida literature with reported death, due to renal failure, in the first year of life up to 20%. To prevent renal damage, treatment of a neurogenic bladder should start immediately after birth. Modern treatment gives us the opportunity to convert a high-pressure bladder with functional urethral obstruction based on neuropathic detrusor/sphincter dyssynergia into a low-pressure reservoir that is safe for the upper tracts. Early urinary and fecal continence is an important factor for optimal quality of life for spina bifida patients. This chapter gives a modern protocol for the managemenrt of spina bifidia.
Abstract: The undescended testis or cryptorchidism is one of the most common genitourinary problems found in pediatrics. It should probably also be considered the most common disorder of the male endocrine glands in children. The main reasons advocated for the treatment of cryptorchidism are issues pertaining to the risks of increased infertility in patients with cryptorchid testes, especially if cryptorchid testes, especially if the process is bilateral. In addition, an increased testicular malignancy rate, an increased risk for testicular torsion, and the psychological stress of an empty scrotum are other significant reasons for the treatment of cryptorchidism.
Abstract: Congenital adrenal hyperplasia (CAH) results from inherited defects in one of the five enzymatic steps required for the biosynthesis of cortisol. These disorders are so named because the adrenal glands are hyperplastic at birth due to unrestrained ACTH stimulation already in foetal life. Indeed, chronic overstimulation of ACTH secretion due to low levels of cortisol results in hyperplasia of the adrenal cortex. A defect in a particular step may be manifested clinically not only because cortisol and other steroid hormones are not synthesized effectively but also because precursor steroids proximal to the blocked step accumulate and can be shunted into other metabolic pathways, particularly that of androgen biosynthesis. Each enzymatic defect produces a distinctive hormonal profile and clinical picture. The two most common CAH, 21-hydroxylase (21-OH) (>90%) and 11b-hydroxylase (11b-OH) deficiencies (~5%), are due to deficient enzymes (P450C21, and P450c11) expressed exclusively in the adrenal glands, while the three other CAHs (<%), due to deficiencies in enzymes (P450scc, 3b-HSD and P-450c17) expressed in the adrenal glands and gonadal tissues, result in decreased biosynthesis of both cortisol and testosterone.
Abstract: The management of the bladder exstrophy–epispadias complex continues to challenge even the most experienced pediatric urologists, surgeons, and orthopedists. From a reconstructive perspective, exstrophy poses unique challenges in the quest to achieve anatomically, functionally and cosmetically satisfactory results. Regardless of technique, however, certain principles endure: complete, successful closure of the bladder at the earliest possible time is the best prognostic marker for future bladder function and continence; aggressive and deep dissection is critical to place the reconstructed bladder into the true pelvis as much as possible, recapitulating the tissue planes and anatomic relationships of the pelvic floor; pelvic osteotomy and effective postoperative pelvic immobilization are useful to facilitate closure and prevent dehiscence, especially in older children; penile reconstruction (at birth or delayed) can result in cosmetically and functionally satisfactory structures.
Abstract: Urinary stone disease in children is a problem that has perplexed physicians, surgeons and patients for many centuries. There are two main forms of the disorder – so-called "endemic bladder stones" which occur particularly in boys, who live in those countries or regions whose economies are largely dependent on agriculture, and upper tract stones which are more prevalent in the more affluent, industrialised countries of the world and occur in both boys and girls. This chapter gives a brief review on the development, types and composition of the urinary calculi and an elaborative discussion on the aetiological factors in paediatric urolithiasis. It is found that about 15% of paediatric stone-formers have an inborn error of metabolism, including cystinuria (10%), primary hyperoxaluria (5%), xanthinuria (<0.01%) and 2-8-dihydroxyadeninuria (<0.01%).