U. JODAL6, L. REES8, S.P.A. RIGDEN8 and M.A. PREECE9 From 2the Hbpital Universitaire des Enfats Reine Fabiola, Brussels. 3Vniversitair Ziekenhuis Gasthuisberg, Leuven, 4Childrenf Hospital, Universiry of Helsinki, Helsinki, sHbpital des Enfats Malades. Paris, %tra sjukhuset, Gothenburg. 7Huddinge sjukhus. Stockholm, 8Guy's Hospital. London, %e Hospital for Sick Children, London and I Kabi Pepride Hormones, Stockholm. M. VANDERSCHUEREN-LODEWEYCKX3, C. HOLMBERG4, I. SIPILA4, ABSTRACT. Johansson, G.I. Sietnieks, A.I. Janssens, F.2, Proesmans, W.3, Vanderschueren-lodeweyckx, M.3, Holmberg, C.4, Sipila, L4, Broyer, M.5, Rappaport, R.5, Albertsson-Wikland, K.6, Berg, U7, Jodal, L6, Rees, L.8, Rigden, S.P.A.8 and Preece, M.A.9 (2Hi5pital Universitaire des Enfants Reine Fabiola, Brussels, 3Universitair Ziekenhuis Casthuisberg, Leuven, 4Children's Hospital, University of Helsinki, Helsinki, 5HOpital des Enfants Malades, Paris, G t r a sjukhuset, Gothenburg, 7Huddinge sjukhus, Stockholm, 8Cuy's Hospital, London, q h e Hospital for Sick Children, London, and IKabi Peptide Hormones, Stockholm). Recombinant human growth hormone treatment in short children with chronic renal disease, before transplantation or with functioning renal transplants: an interim report on five European studies. Acta Paediatr Scand [Suppl] 370: 36-42, 1990.Growth retardation is common in children with chronic renal disease. Final adult height is often reduced, even in children with a functioning renal transplant. The five European studies considered here aim to investigate the efficacy and safety of recombinant human growth hormone therapy (rhGH) in two groups of short children with chronic renal disease. The first group comprises 29 prepubertal children with preterrninal chronic renal failure (i.e. before renal transplantation), and the second group comprises 39 prepubertal and pubertal children with functioning renal transplants. The median bone age retardation in the groups at the start of treatment was between 2.2 and 3.7 years; this did not change during the first year of treatment. This interim report concentrates on patients who have been treated for at least 1 year (i.e. 22 children from the first group, and 28 children from the transplant group (15 prepubertal and 13 pubertal children). The median height velocity increased from 4.8 cmlyear to 10.0 cmlyear in the first group (the chronic renal failure group), from 2.6 cmlyear to 6.2 cmlyear in prepubertal children with renal transplants and from 3.8 cmlyear to 6.7 cmlyear in pubertal children with renal transplants. The corresponding changes in height velocity SDS were from -1.3 to 5.1 for the chronic renal failure group and -2.8 to 2.3 for the prepubertal children with renal transplants. Renal function declined in the chronic renal failure group but this decline corresponded to expected progression of the disease. Some o f the children with renal transplants showed a decreased renal. function, which in most cases was explained by non-compliance or chronic rejection.Growth retardation is common...

MRI provides a precise assessment of the complications associated with duplex kidneys. Its optimal role seems to be the assessment of ectopic extra-vesical ureteric insertions and whenever an occult upper pole is suspected.

Two cases of pseudotumoral peliosis hepatis are presented with emphasis on MRI findings. One patient had four individual lesions, the other had two. Image characteristics in both were: heterogeneic signal intensity on T1-weighted images (T1WI) with areas of high-, intermediate, and low signal intensity; heterogeneic signal intensity on T2WI with presence of numerous intralesional "cystic" hyperintense areas with a hypointense border. Signal intensities on T1WI were iso- to hypointense in one case and mild central hyperintensities were demonstrated in the other case, probably due to intra-lesional hemorrhage or diffuse accumulation of fresh clotting within the sinusoids. One case demonstrated early enhancement of the peripheral borders in the arterial phase, and both demonstrated enhancement in the portovenous and late phases. One case was studied with Gd-BOPTA and iron oxides and demonstrated enhancement with both products, suggestive for the presence of hepatocytes and Kupffer cells. This is the first report of the use of hepato-specific contrast agents in this entity. Spontaneous regression of the lesions was demonstrated on a follow-up MR examination in one case.

Sonography can be used differentiate the two patterns of type 1 primary hyperoxaluria. The cortical nephrocalcinosis type carries a higher risk of developing ESRD.

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