Active clinical trials for "Hypospadias"

Smith-Lemli-Opitz Syndrome (SLOS) is a genetic disorder (autosomal recessive) caused by an abnormality in the production of cholesterol. The disorder can occur in both a "mild" or "severe" form. SLOS is associated with multiple birth defects and mental retardation. Some of the birth defects include; abnormal facial features, poor muscle tone, poor growth, shortened life span, and abnormalities of the heart, lungs, brain, gastrointestinal tract, limbs, genitalia, and kidneys. There is no known cure for SLOS but recently patients have been treated with increased amounts of cholesterol in their diet. The cholesterol in a persons diet is unable to correct the abnormalities in the patient's organs, but researchers hope it will improve growth failure and mental retardation. This study was developed to answer questions about the causes and complications of SLOS, as well as the effectiveness of cholesterol treatment. The study will enroll patients diagnosed with SLOS, and their mothers. The objectives of the study will be to address the following questions: <TAB> What is the prognosis / natural history of the demyelination in the nervous system of patients with SLOS? <TAB> Do patients with SLOS have other problems concerning the function of their endocrine systems? <TAB>What are the genetic make-ups of patients with SLOS? <TAB>Can further studies of cholesterol metabolism and genetic testing, using SLOS fibroblasts, increase the understanding of SLOS?<TAB>

We want to see if there is a relationship between exposure to pesticides and the occurrence of hypospade in Picard newborn .

Caudal block is an easy and effective type of central neuraxial block that is widely used in subumbilical surgeries to provide intraoperative and postoperative analgesia in the pediatric population. Caudal block application with ultrasound (USG) was first applied in 2003. Sacrum, sacral cornular, sacrococcygeal ligament and sacral hiatus can be easily distinguished by ultrasound. The probe is positioned in a horizontal midline position on the lower sacrum. Sacral roots within the caudal epidural space can be seen as hypoechoic ellipses. In addition, anatomical variations of the sacral hiatus and the process area can be clearly observed. A accurate probe use with ultrasound guided is confirmed by advancing the caudal needle in the desired direction, widening in the sacral hiatus with local anesthetic injection, and the turbulence observed during injection into the sacral canal if the color doppler feature is used. Thus, a decrease in complications and an increase in the success of the procedure can be achieved with simultaneous imaging. In addition, in cases where the block is difficult, the in-plane technique can be preferred in the midline plane.The aim of this study is to compare the success rates and postoperative pain levels of caudal block applications with the out of plane technique (probe applied by holding horizontal) and in-plane technique (probe applied vertically), which is routinely applied with ultrasound in pediatric hypospadias surgery.

Hypospadias, seen in every 200-300 births, is one of the most common congenital anomalies of the penis and is defined as the urethral meatus being located in the ventral part of the penis instead of its normal place. The surgery of this anomaly is very painful in the postoperative period and requires long-term analgesia. Regional anesthesia methods combined with general anesthesia play an important role in providing effective and long-term postoperative pain control in pediatric penile surgery. These methods also reduce postoperative morbidity, enable early mobilization and significantly decrease the need for narcotic analgesics. The investigator's hypothesis is peripheral nerve blocks are superior to neuraxial blocks as the blocks provide longer-term analgesia and have fewer side effects.

Smith-Lemli-Opitz Syndrome (SLOS) is a genetic condition that causes mental retardation and other birth defects. This study will evaluate a new prenatal screening test for SLOS.

Hypospadias repair is a urological surgical operation that is very painful in the postoperative period and requires long-term analgesia. A dorsal penial nerve block (DPNB) and pudendal nerve block (PNB), which are regional anesthesia techniques for this operation, are used to provide postoperative analgesia.

Pain is one of the most misunderstood, underdiagnosed, and untreated medical problems, particularly in children. New Joint Commission on Accreditation of Health Care Organization regards pain as fifth vital sign and requires caregivers to regularly assess pain. Inadequate pain relief during childhood may have long-term negative effects including harmful neuroendocrine responses disrupted eating and sleep cycles and increased pain perception during subsequent painful experiences. Also, postoperative pain can result in an uncooperative and restless child. Hence, it is preferable to prevent the onset of pain rather than to relieve its existence. Various multimodal techniques have been designed for pediatric pain relief. These include both systemic and regional analgesia. The most commonly used regional technique is caudal epidural block. Advantages of the caudal block are smoother recovery with less distress behavior, early ambulation, decreased the risk of chest infections, decreased postoperative analgesic requirements, and early discharge. In our culture; considerable number of parents still refuses caudal anesthesia fearing from the rare neurological sequelae may occur. This the motive for searching for parenteral surrogate gives clear headed recovery resembles regional analgesia. Dexmedetomidine is an alpha 2 agonist which has sedative, analgesic, and opioid-sparing effect. It prolongs the duration of analgesia by its local vasoconstrictive effect and by increasing the potassium conductance in A-delta and C-fibers. It also exerts its analgesic action centrally via systemic absorption or by diffusion into the cerebrospinal fluid and reaches alpha 2 receptors in the superficial laminae of the spinal cord and brainstem or indirectly activating spinal cholinergic neurons. The sedative effects of dexmedetomidine are mostly due to stimulation of the alpha 2 adrenoceptor in the locus coeruleus

Urinary fistulas are unfortunately one of the most common post operative complications in hypospadias repairs and bladder neck transections on pediatric and adult populations, an estimated 20%, resulting in multiple repeat operations with prolonged hospitalization. The negative sequelae of these fistulas have significant financial and psychosocial impact and the aim of this study is to evaluate if the fresh frozen preserved amniotic membrane tissue studied, which have live cells and intact naturally occurring growth hormones and peptides that augment healing that have been shown in early studies to be effective facilitating previous failed fistula repairs and leg ulcer healing, respectively, will help reduce the incidence of urinary fistulas in subject populations.

Even successfully managed hypospadias without any complication, voiding problem could be developed due to the increased urethral resistance. The investigators are trying to analyze the effect of hypospadias operation on voiding function by checking the detrusor thickness and urinary nerve growth factor.

Background: - Smith-Lemli-Opitz syndrome (SLOS) is a genetic disorder that prevents the body from making enough cholesterol. People who have SLOS often need to take extra cholesterol, either in food or in supplements, for their bodies to work properly. Cholesterol is very important for the brain and nervous system. Therefore SLOS is highly related to autism, mental retardation, and other brain and nervous system disorders. Not much is known about how people with SLOS handle cholesterol and how taking extra cholesterol helps them. A long-term study of people with SLOS will help answer these and other questions. Objectives: - To study the effects of a high-cholesterol diet on people with Smith-Lemli-Opitz syndrome. Eligibility: - Individuals of any age who have Smith-Lemli-Opitz syndrome. Design: Participants will have study visits up to two times in the first year and once a year every year after that. Each visit will last between 3 and 5 days. Participants will be screened with a physical exam, medical history, and blood and urine tests. Participants will provide regular blood, urine, stool, saliva, and skin cell samples for testing. Participants will keep track of the foods they eat at home. During the study, they will eat a high-cholesterol diet at all times, except for the second study visit (3 to 6 months after the screening visit). That visit will involve a cholesterol-free diet for 4 weeks. Participants will have special cholesterol tests with blood samples at different times during the study. At different study visits, participants will have tests of mental and physical skills (including tests for autism). They will answer questions about their diet and food habits. They will also have hearing and eye tests, body and bone measurements, and imaging studies. Not all of the tests will be done at every study. Participants will be allowed to leave the study at any time.