Validation of automatic bone age determination in children with congenital adrenal hyperplasia.

BACKGROUND: Determination of bone age is routinely used for following up substitution therapy in congenital adrenal hyperplasia (CAH) but today is a procedure with significant subjectivity. OBJECTIVE: The aim was to test the performance of automatic bone age rating by the BoneXpert software package in all radiographs of children with CAH seen at our clinic from 1975 to 2006. MATERIALS AND METHODS: Eight hundred and ninety-two left-hand radiographs from 100 children aged 0 to 17 years were presented to a human rater and BoneXpert for bone age rating. Images where ratings differed by more than 1.5 years were each rerated by four human raters. RESULTS: Rerating was necessary in 20 images and the rerating result was closer to the BoneXpert result than to the original manual rating in 18/20 (90 %). Bone age rating precision based on the smoothness of longitudinal curves comprising a total of 327 data triplets spanning less than 1.7 years showed BoneXpert to be more precise (P<0.001). CONCLUSION: BoneXpert performs reliable bone age ratings in children with CAH.

S2k-Guideline Developmental Dysplasia of the Hip in the Neonate. / S2k-Leitlinie Hüftdysplasie Teil Säugling.

Hip developmental disorders are the most common musculoskeletal disease in newborns in Central Europe. The definition of hip developmental disorder includes both dysplastic and dislocated joints. In a dysplastic joint, shearing forces induce a growing disorder in the acetabulum. If this growing disorder persists, the femoral head first displaces the acetabular cartilage cranially and finally the femoral head dislocates posteriorly into the gluteal fossa - progressively losing contact to the acetabulum. Therefore nowadays there is general support for the concept of a developmental instead of a congenital dislocation of the hip. From the first day of life, the different stages of hip developmental disorder be exactly classified by an ultrasound examination of the infant hip joint according to Graf. Therefore the Graf hip ultrasound examination has been an integral part of the paediatric guidelines in Germany since 1996. All newborns must receive Graf hip ultrasound screening examination, ideally at the age of 4-5 (maximal 8) weeks as part of the U3 screening examination. Newborns with historical or clinical risk factors must receive an ultrasound examination in the first week of life, additionally to the clinical examination of the hip joints of all newborns according to the second screening examination U2. In the case of pathological results, therapy should be initiated according to measured hip type within one week. Dislocated joints need reduction and as soon as the contact between the femoral head and the acetabulum has been restored, the head should be retained securely within the acetabulum. This phase of retention is followed by the maturation phase for dislocated joints, which is also sufficient therapy for dysplastic joints. In order to avoid femoral head necrosis as an early complication or as a new hip developmental disorder in the course of further growth, the femoral head during the retention phase and the maturation phase should be placed deeply into the socket. This can be achieved by retaining hip flexion of 100-110° with simultaneous hip abduction of 50° to a maximum of 60°.

Eradication of pulmonary aspergillosis in an adolescent patient undergoing three allogeneic stem cell transplantations for acute lymphoblastic leukemia.

Systemic fungal infections are a major cause of infection-related mortality in patients with hematologic malignancies. This report addresses the case of an adolescent patient with acute lymphoblastic leukemia who underwent three allogeneic hematopoietic stem cell transplantations and developed pulmonary aspergillosis. Combination therapy with liposomal amphotericin B (L-AmB, 3 mg/kg bw/day) and caspofungin (CAS, 50 mg/day) during the first allogeneic hematopoietic stem cell transplantation (HSCT) improved the pulmonary situation. After shifting the antifungal combination therapy to oral voriconazole (2 × 200 mg/day) and CAS, a new pulmonal lesion occurred alongside the improvements in the existing pulmonary aspergillosis. An antifungal combination during a second HSCT with L-AmB (3 mg/kg bw/day) and CAS showed an improvement in the pulmonary aspergillosis. A combination therapy with CAS and L-AmB (1 mg/kg bw/day) during the third HSCT led once again to progress the pulmonary aspergillosis, after increasing the L-AMB to 3 mg/kg bw/day for recovery. The presented case provides an example of how, despite severe immunosuppression, a combination of antifungal drugs administered intravenously at therapeutic dosages may be more efficient than either intravenous monotherapy or combinations of intravenous and oral antifungals in selecting pediatric and adolescent patients with proven fungal infections.