Combination therapy improves pathology indices in diffuse mesangial sclerosis

Abstract

exstrophy called “covered cloacal exstrophy”. CCE is an extremely rare congenital malformation, and various anatomic findings associated with CCE have been reported such as gastrointestinal, urological, gynecological, skeletal, and neurospinal anomalies. The present patient had a vesico-intestinal fistula, short colon, hypospadias, low-lying umbilicus, absent bladder neck, widely separated pubic bones, and spina bifida occulta. These anomalies are compatible with those of the previously reported cases. Generally cloaca develops in female patients only; in contrast, cloacal exstrophy develops in male and female patients. CCE was thought to be a variant of cloacal exstrophy, therefore it seemed compatible that CCE is reported in both sexes. Although CCE has been described in several postnatal cases, the prenatal course and findings of CCE are not fully understood. To the best of our knowledge, all previously reported cases of CCE were not associated with lethal pulmonary hypoplasia due to urethral obstruction. In the present case, we speculated that meconium-containing fetal urine and congenital hypoplastic lower urinary tract caused urethral obstruction, prolonged severe oligohydramnios from early gestation, and subsequent severe pulmonary hypoplasia. Lung hypoplasia in premature infants is defined as a lung–bodyweight ratio < 0.015 for infants of 20–27 weeks’ gestation, and <0.012 for ≥28 weeks’ gestation. We diagnosed pulmonary hypoplasia in the present patient based on a lung– bodyweight ratio 0.014 and birthweight, which was equivalent to that of 26–27 weeks’ gestation. In conclusion, CCE is an extremely rare disease and its initial diagnosis can be difficult because of the many anatomic variants. It is important to consider CCE in the antenatal differential diagnosis of oligohydramnios and fetal pulmonary hypoplasia. Acknowledgment