Current and Novel Therapeutical Approaches of Classical Homocystinuria in Childhood With Special Focus on Enzyme Replacement Therapy, Liver-Directed Therapy and Gene Therapy.

Classical homocystinuria is a hereditary defect of the enzyme cystathionine beta synthase, which is produced in the liver. If this enzyme fails, the synthesis pathway of cysteine from methionine is interrupted, leading to the accumulation of homocysteine in the blood plasma and homocysteine in the urine. After birth, the children are unremarkable except for the characteristic laboratory findings.

Medical honey and its role in paediatric patients.

Background: The use of complementary medical treatment in wound management has continued to grow throughout the world. There is a large body of evidence that supports the use of honey as a wound dressing for a wide range of wound types. The authors present an update of present knowledge about honey as a form of complementary medicine in paediatric wound management.

The role of complementary medicine in a pediatric day center in Germany.

Background: The role of complementary alternative medicine (CAM) in pediatrics has considerably increased in the last years. The knowledge about potential side effects and possible interactions with conventional drugs is still limited, and often pediatricians do not know whether their patients use CAM. This can be critical, as in order to diagnose and treat illnesses properly physicians need to know about the full extent of the self-administered therapy of their patients.

Topical timolol for small infantile hemangioma: a new therapy option.

The main characteristic of infantile hemangioma is that it grows rapidly after birth and mostly regresses spontaneously. It is a common practice for only a small part of the hemangioma to be treated, as they can be extremely disfiguring and destructive to normal tissue as well as possibly being life-threatening. Recent studies have discovered that the use of topical 0.

Parent satisfaction with a pediatric practice in Germany: a questionnaire-based study.

Background: Parental satisfaction with a pediatric day center is essential for the medical treatment of children, since it is closely related to compliance. In order to ascertain factors which predict parental satisfaction as well as to discover possible weak points, we developed a questionnaire.

Methods: 127 parents visiting the pediatric day center from October to November 2010 were asked to respond to a questionnaire.

Benzocaine-induced methaemoglobinaemia: a case study.

Benzocaine is a widely used topical anaesthetic and has been reported to cause toxic methaemoglobinaemia in otherwise healthy individuals with no predisposing risk factors. This article reports on a rare case of benzocaine-induced methaemoglobinaemia following adenotonsillectomy in a 5-year-old girl. Topical benzocaine was applied orally for the relief of postoperative wound pain on the eighth postoperative day.

Does honey have a role in paediatric wound management?

Topical honey treatment has been shown to possess antimicrobial properties, promote autolytic debridement, stimulate growth of wound tissues to hasten healing, and to start the healing process in dormant wounds, stimulating anti-inflammatory activity that rapidly reduces pain, oedema and exudate production. This article provides an overview of the use of honey as a medicinal substance, particularly its use in wound treatment, and reviews the published data concerning honey as a form of complementary and alternative medicine in paediatric wound management. The literature reviewed was found by searching the PubMed, BIOSIS, and ISI Web of Science databases for the term honey.

Surgical experience and cosmetic outcomes in children with sacrococcygeal teratoma.

To determine the extent of progress in the treatment of sacrococcygeal teratomas (SCTs), the experience of 25 patients over a period of 30 years between 1972 and 2002 was evaluated. The medical records of the children were reviewed for demographics, histology, clinical course, bladder, anorectal and sexual function, and outcome. In particular, analysis of long-term cosmetic results after surgery for SCT was performed.

Surgical experience in children with biliary atresia treated with portoenterostomy.

Objective: Biliary atresia is the result of a fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Without surgical intervention, mortality reaches 100%. The 5-year survival rate after portoenterostomy ranges from 13% to 60%, with approximately 60% of patients requiring liver transplantation at a later stage because of insufficient bile flow.

Calcific tendinitis of the supraspinatus tendon in children.

We present the case of a 13-year-old girl with painful calcific tendinitis of the supraspinatus tendon of the left shoulder. The pathologic finding was the presence of a small calcium deposit within the supraspinatus tendon.