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H Devlieger

Publications and source records attributed to H Devlieger.

139 records · Page 8Linked to original sources

Bartter syndrome in two siblings--antenatal and neonatal observations.

Bartter syndrome was diagnosed in two siblings born to healthy unrelated parents. Each pregnancy was complicated by severe polyhydramnios. The first child was treated with indomethacin from the age of then weeks on. At the age of six years he is doing very well: height is 109.9 cm (P3) and weight 17.8 (P3). Studies of the amniotic fluid during the mother's second pregnancy showed high chloride concentrations (112, 117, and 119 mEq/l), normal levels of sodium, potassium, calcium and creatinine and low prostaglandin E2 (5.0-22.3 pg/ml) and F2 alpha (36-71.7 pg/ml) concentrations. Severe chloride and sodium wasting after birth resulted in hypochloremia, hyponatremia and dehydration. Concomitantly an immediate and striking increase in urinary PGE2 excretion from 45 to 1022 pg/ml was observed. Indomethacin therapy had to be stopped after one week when necrotising enterocolitis developed.

Aldosterone↗

Lymphangiomatosis of the body wall: a report of two cases associated with chylothorax and fatal outcome.

We report on two cases of an unusual but distinctive variant of lymphangiomatosis, presenting at birth with predominantly cutaneous involvement of the body wall and complicated by chylothorax. The lesion manifested clinically as a slowly progressive, diffuse, and fluctuant skin swelling. Eventually, almost the entire trunk became affected. There were no bone lesions. Histologically, the soft tissues were diffusely infiltrated by interconnecting mazelike lymphatic vessels. The two infants died from infection at the age of 23 days and 10 months, respectively. Our experience confirms that premortem histologic diagnosis of lymphangiomatosis is difficult to establish. Awareness of the condition and knowledge of its various clinical presentation forms are essential for proper recognition, assessment of the outcome, and evaluation of new therapeutic measures such as interferon.

Chylothorax↗