Physical growth and the serum growth factors, insulin growth factor 1 (IGF1) and its binding protein (IGFBP3) were measured weekly during dexamethasone treatment and for 3 weeks after stopping therapy in 10 ventilated babies [median (range) birth weight 860 g (640-1210); median (range) gestational age 26 weeks (24-29)] with bronchopulmonary dysplasia (BPD). The mean (+/- SE) rates of change of all physical measures except crown-rump length (CRL) increased significantly after stopping dexamethasone: weight gain 13.2 (+/- 1.5) on versus 1.0 (+/- 1.9) g/day off treatment; occipital-frontal circumference 0.7 (+/- 0.1) cm/week; CRL 0.5 (+/- 0.1) versus 0.7 (+/- 0.1) (TBL) 0.7 (+/- 0.1) versus 1.1 (+/- 0.1) cm/week; CRL 0.5 (+/- 0.1) versus 0.7 (+/- 0.1) cm/week, and knee-ankle length (KAL) 0.13 (+/- 0.02) versus 0.36 (+/- 0.04) cm/week. Mean serum IGF-1 (1.57 +/- 0.13 versus 3.56 +/- 0.41 nmol/L) and IGFBP3 (0.94 +/- 0.03 versus 1.12 +/- 0.05 mg/L) levels also increased off treatment. The weekly dose of dexamethasone (mg/kg) was significantly negatively correlated with all physical growth measures (P < 0.01), but showed no correlation with growth factors. Protein intake (g/kg/day) was significantly correlated (P < 0.01) with weight gain (r = 0.28), changes (TBL) (r = 0.32), serum IGF1 levels (r = 0.60), and IGFBP3 levels (r = 0.37). All aspects of physical growth are compromised during dexamethasone treatment for BPD. Poor growth during steroid treatment is associated with lower IGF1 and IGFBP3 levels. Further study is needed to examine the effect of varying dexamethasone dosage regimes and nutritional intake on the growth process in BPD.

We find a link between haploinsufficiency of the EFNB2 gene and the presence of ambiguous genitalia and hypospadia in patients with a 13q.33 deletion. This work emphasizes the importance of early diagnosis of this condition due to the link with mental retardation and the need for follow up and management.

A case of primary pulmonary hypoplasia in a term female neonate presenting with severe respiratory distress at birth is reported. Respiratory failure persisted and she died at 12 days of age. Primary pulmonary hypoplasia is a rare condition not associated with other maternal or fetal disorders.

We read with great interest the article by Cooperstone et al. [1993] in this Journal reporting 3 new cases of Galloway-Mowat syndrome of abnormal gyral patterns and glomerulopathy. We wish to add another case to the literature. CLINICAL REPORTThe patient was a male Caucasian infant born to a healthy 38-year-old gr 2 ab 1 mother in the 36th week of gestation. The father was 36 years old and in good health. There was no consanguinity. Amniocentesis performed at 16 weeks gestation for advanced maternal age demonstrated a normal male karyotype (46, XY). Antenatal ultrasound study at this time did not demonstrate any abnormality. The pregnancy had been uncomplicated. Birth was by spontaneous vaginal delivery. The Apgar scores were 6 and 9 at 1 and 5 minutes, respectively. Birth weight was 2,278 g (50th centile), length 50 cm (50th centile), and head circumference (OFC) 28 cm (<10th centile). The placenta was normal.The baby was stable, but in the second week of life he developed edema, poor feeding and regurgitation. He was referred at 10 days for investigation. At that time he had microcephaly, micrognathia, high arched palate, apparently low-set posteriorly angulated ears, overlapping fingers, flexion deformity of the thumbs, bilateral simian creases, widely spaced nipples, inguinal testes, marked pitting pedal edema, ballottable kidneys, poor muscle tone and decreased spontaneous movements.Results of biochemical investigation: elevated blood urea (11.4 mmol/L) and creatinine (92 mmol/L) and low serum protein (17 g/L) which was associated with an increased urinary protein excretion (81 g/L). No evidence for toxoplasmosis, rubella, cytomegalovirus, herpes, and syphilis infections was found.Renal ultrasound scan demonstrated diffusely increased echogenicity with poor corticomedullary differentiation, and increased length of kidneys for gestation (5.3 cm). Cranial ultrasound study demonstrated a smooth simple contour of both cerebral hemispheres with lack of normal sulcal pattern, suggestive of pachy-Abbas R.M. Kingo*