Tube weaning according to the Graz model in two children with Alagille syndrome.

Two children were weaned from long-term tube feeding after liver transplant because of Alagille syndrome. The children were successfully weaned, one in seven days and the other in 13 days, using our standard and highly specialized intensive treatment protocol. Normal feeding behavior and stabilization of body weight were established. Children fed by long-term enteral tubes can be weaned from enteral feeding even after a long period of treatment. The return to age-appropriate self-feeding should be introduced as early as possible. Our weaning program time is brief and effective and can be recommended generally to improve quality of life and withhold unintended side-effects of enteral nutrition.

Groin exploration for nonpalpable testes: laparoscopic approach.

PURPOSE: Diagnostic laparoscopy (DL) is the technique of choice for exploration of nonpalpable testes (NPT). Nevertheless, groin exploration is necessary to evaluate the cord and gonadal structures entering the internal ring. This retrospective analysis evaluates our hypothesis that hypoplastic cord structures entering the internal ring predicts absence of a viable testicle and a laparoscopic groin exploration in these cases can reduce the number of unnecessarily performed open groin exploration (OGE). MATERIALS: A retrospective review was performed of 23 boys with 26 NPTs who were operated on from June 1998 to October 2000 to evaluate our protocol for NPT using DL and OGE. RESULTS: Of 26 NPTs in 23 boys, 3 bilateral intraabdominal testis were detected (2 Fowler Stephens; 1 standard orchidopexy). Twenty cord structures entered the internal ring. Three appeared normal at DL with a viable testis followed by an orchidopexy. Seventeen were hypoplastic without patent processus. During LGE no viable testis was detected: blind-ending cords, no biopsy (n = 4); testicular regression syndromes (n = 3), early fetal regression (n = 6), no residual testicular structures (n = 4). CONCLUSIONS: This experience confirms the authors' hypothesis and criteria for LGE in all cases. The authors conclude that LGE is a helpful tool in the diagnostic workup of NPT to avoid unnecessary OGE and is a further step in the minimally invasive approach to all kinds of findings of NPT.

Absent or reversed end-diastolic blood flow in the umbilical artery and abnormal Doppler cerebroplacental ratio--cognitive, neurological and somatic development at 3 to 6 years.

UNLABELLED: The objective of this study was to examine the cognitive, neurological and somatic developments of children who had in utero an absent or reversed end-diastolic blood flow (ARED) in the umbilical artery or an abnormal cerebroplacental ratio (ABF). METHODS: 16 children with ARED blood flow and 15 children with ABF were each matched to children with the same gestational age, appropriate for gestational age, the same sex and born within 4 months. Data were assessed at the age of 3-6 years. Children with asphyxia, neonatal infection, malformation or major surgical interventions in the neonatal period were excluded. Each child underwent a neuropediatrical examination; furthermore, a Kaufman Assessment Battery for Children, a Snijders-Oomen Intelligence Scale for Children and a Man-Drawing Test were used to evaluate cognitive development. The socioeconomic status was also assessed. RESULTS: Children in the ARED group remained lighter and had a higher frequency of microcephaly. In the Kaufman Assessment Battery for Children and the Snijders-Oomen Intelligence Scale for Young Children, cognitive development was impaired in the ARED and the ABF groups compared to the control group. The ARED and the ABF groups, however, showed no differences. The Man-Drawing Test and the Denver Development Test did not show any differences. DISCUSSION: ARED blood flow and ABF showed impaired cognitive development. The degree of impairment was the same in the ARED and the ABF groups. Long-term follow-up studies until adulthood are necessary to see if impaired cognitive development remains significant in these groups of patients.

[Cardiovascular manifestations in inflammatory rheumatic diseases]. / Kardiovaskuläre Manifestationen bei entzündlich rheumatischen Erkrankungen.

Inflammatory rheumatic diseases show an increased cardiovascular mortality. The reasons for this are unclear. Possible associations between atherosclerosis and autoimmune diseases like rheumatoid arthritis and systemic lupus erythematosus can be illustrated.