20 research outputs found
The Pavlik Harness in the Treatment of Congenital Dislocating Hip: Report on a Multicenter Study of the European Paediatric Orthopaedic Society
The results of functional treatment using the Pavlik harness in congenital
dislocation and congenital dysplasia of the hip in children aged less than 11
months were examined by an EPOS study group. This study was conducted on 3,611
hips in 2,636 patients for a period of 1-9 years after treatment. The reduction
rate was 92% in grade Tonnis 2 and 3; the healing rate was 80%. In children with
dysplastic hips, the healing rate was 95.35%. Avascular necrosis of the femoral
head was observed in 2.38%. The Pavlik harness is designed for outpatient
treatment if the parents are complian
The Pavlik Harness in the Treatment of Congenital Dislocating Hip: Report on a Multicenter Study of the European Paediatric Orthopaedic Society
The results of functional treatment using the Pavlik harness in congenital
dislocation and congenital dysplasia of the hip in children aged less than 11
months were examined by an EPOS study group. This study was conducted on 3,611
hips in 2,636 patients for a period of 1-9 years after treatment. The reduction
rate was 92% in grade Tonnis 2 and 3; the healing rate was 80%. In children with
dysplastic hips, the healing rate was 95.35%. Avascular necrosis of the femoral
head was observed in 2.38%. The Pavlik harness is designed for outpatient
treatment if the parents are complian
Prenatal Diagnosis of Atypical Phenylketonuria
Atypical phenylketonuria (PKU) is a group of very rare and severe diseases caused
by tetrahydrobiopterin (BH4) deficiency (Niederwieser and Curtius, 1987). So far
three inborn errors of metabolism are known to cause BH4 deficiency, defects in:
GTP cyclohydrolase I (GTPCH); 6-pyruvoyl tetrahydropterin synthase (PPH4S);
and dihydropteridine reductase (DHPR) (Blau, 1988). Recently a new form of
atypical PKU with unusual 7-iso-biopterin excretion in the urine of patients was
described (Curtius et al., 1988). Prenatal diagnosis of BH4 deficiency can be
achieved mainly by measurement of pterin metabolites in amniotic fluid and of
enzyme activities in cultured fluid cells and fetal blood (Blau et al., 1987).
We performed the prenatal diagnosis of DHPR deficiency in two cases (one
diagnosed as homozygote and one as heterozygote), and of PPH4S deficiency in
four cases (one diagnosed as homozygote, one as heterozygote, and two as normal).
Our results suggest that measurement of neopterin and biopterin by HPLC in
amniotic fluid is adequate. But it is recommended that diagnosis should be confirmed
by enzyme measurements