ABSTRACT
Urinary tract anomalies (UTA) including polycystic kidney disease nowadays can be detected antenatally by ultrasound. The concomitant presence of oligohydramnios has been regarded as a severe risk factor for renal dysfunction and pulmonary hypoplasia, although clinical data after birth are scarce. We report the postnatal course and long-term follow-up of 10 infants with oligohydramnios due to congenital UTA from two pediatric nephrology centers. The underlying final diagnoses were autosomal-recessive polycystic kidney disease (ARPKD, n = 2), familial tubular dysgenesis (n = 2) and bilateral renal hypoplasia (n = 6) including 3 children with posterior urethral valves. Two children died in the neonatal period while 8 children are currently alive at a median age of 2.5 (range 1.1-10) years. In the postnatal period, respiratory failure necessitating mechanical ventilation occurred in 7 infants (including the 2 non-survivors). All surviving children had chronic renal failure, which could be managed conservatively in 6 children (median GFR 45 (range 15-53) ml/min/1.73 m2) while 2 reached end-stage renal disease; one undergoing preemptive kidney transplantation and one peritoneal dialysis. Seven of 8 children reached normal developmental milestones. In conclusion, the presence of antenatal oligohydramnios in infants with UTA does not always carry a poor prognosis. The high incidence of perinatal complications, the complexity of underlying causes and the prevalence of postnatal chronic renal dysfunction calls for a multidisciplinary approach in the management of these children.
Subject(s)
Fetal Diseases/diagnostic imaging , Oligohydramnios/diagnostic imaging , Polycystic Kidney Diseases/diagnostic imaging , Pregnancy Complications , Ultrasonography, Prenatal , Urinary Tract/abnormalities , Female , Gestational Age , Humans , Infant, Newborn , Male , Oligohydramnios/etiology , Polycystic Kidney Diseases/complications , Pregnancy , Prognosis , Respiratory Distress Syndrome, Newborn/etiology , Respiratory Distress Syndrome, Newborn/therapy , Retrospective Studies , Urinary Tract/diagnostic imaging , Ventilators, MechanicalABSTRACT
Disseminated islands of gastric mucosa are very rare in the small intestine. The secretion of hydrochloric acid can lead to ulceration which results in gastrointestinal bleeding. It is often difficult to localize the focus in case of gastrointestinal blood loss especially in the small bowel. Technetium-99m-pertechnetate scintigraphy may be a helpful tool in detecting ectopic gastric mucosa. We report a case of a 21-mo-old boy with recurrent gastrointestinal bleeding. By using pertechnetate scintigraphy, extensive tracer accumulation in the jejunum and proximal ileum was detected. Histologically, multiple islands of ectopic gastric mucosa were found in about 50 excited mucosal and transmural biopsies. The unusual finding of disseminated accumulation of 99mTc-pertechnetate in the small intestine was the diagnostic clue for such a rare disease.
