We describe the fetus delivered to an insulin-dependent diabetic woman who had had a previous large, stillborn, non-malformed male infant and a normal female infant. The present fetus had a most unusual combination of malformations which to date had not been described in diabetic embryopathy. The anomalies include: upper limb amelia, "caudal regression" with bilateral absence of the fibulae, unilateral absence of a femur and ipsilateral oligodactyly; undescended testes; atrial septal defect; multiple vertebral and rib anomalies with cervical scoliosis and right webbed neck; left cleft lip and cleft palate; severe micrognathia; left microtia with atresia of the ear canal; and central nervous system defects including hydrocephalus with the Dandy-Walker malformation, asymmetry of the lateral ventricles, abnormal frontal gyral formation, and ependymal and ganglion cell heterotopias of the spinal cord. The pathogenesis of diabetic embryopathy is discussed.

\s=b\An unusual case of systemic lupus erythematosus (SLE) in a young child is reported with sudden death from myocardial infarction.The diagnosis of lupus erythematosus in this patient was made by renal biopsy at the age of 3 years. Atherosclerosis of the coronary arteries and aorta was found at autopsy with occlusion of the anterior descending branch of the left coronary artery. It is suggested that the vascular changes in this case were related to hypertriglyceridemia and prolonged prednisone therapy superimposed on a hypersensitivity vasculitis related to SLE.(Am J Dis Child 132: [696][697][698][699] 1978) A 5-year-old girl had clinically diag-. nosed systemic lupus erythema¬ tosus (SLE). Even though electrocardiography did not suggest any abnor¬ mal findings, at autopsy coronary atherosclerosis and a recent thrombus in the anterior descending branch of the left coronary artery were asso¬ ciated with a myocardial infarcì in the anterior wall of the left ventricle and interventricular septum.This unusual case of SLE in a young child is reported because of the cardio¬ vascular involvement with sudden death from myocardial infarction.There are few reported cases of lupus erythematosus in children younger than 5 years of age; the majority of cases have involved chil¬ dren older than 10 years of age. WI 53706 (Dr Ishikawa). tosus in this patient was made by renal biopsy at the age of 3 years. Atherosclerosis of coronary arteriesand aorta in this case might be a consequence of hypertriglyceridemia and prolonged prednisone therapy superimposed on a hypersensitivity vasculitis related to SLE. REPORT OF A CASEThe patient was first seen at the Univer¬ sity of Wisconsin hospitals, Madison, at age 4V2 years with an acute onset of abdom¬ inal pain. She was first ill at age 9 months with purpura and idiopathic thrombocyto¬ penia, and subsequently her condition was diagnosed as lupus erythematosus on the basis of positive lupus erythematosus reac¬ tions and abnormal renal histology seen on a biopsy specimen. She was given azathio¬ prine and prednisone with apparent stabil¬ ization of renal function. At the time of the first admission, she was taking 7.5 mg of prednisone and 25 mg of azathioprine orally every day. Five days prior to admis¬ sion a temperature of 39.4°C developed that was associated with evanescent abdominal discomfort. There was no nau¬ sea or vomiting, but subsequently she became lethargic, anorectic, and she com¬ plained of diffuse abdominal pain. She had no abnormal stools. Her maternal aunt had SLE. Examination on admission showed a cushingoid appearance; blood pressure was 110/70 mm Hg; pulse rate, 120 beats per minute; respirations, 40 per minute; tem¬ perature, 39.4°C rectally. Heart and lung examinations were unremarkable. The abdomen was noticeably distended with hypoactive bowel sounds and a bilateral positive psoas sign. Diffuse rebound ten¬ derness was evident. Roentgenograms of the abdomen showed considerable gaseous distention in the stomach, but very little gas throughout the remaining bowel...

Doses of theophylline and caffeine which do not produce aortic arch anomalies in embryonic chicks have been shown to potentiate catecholamine-induced aortic arch malformations in that experimental animal. Theophylline (2.1 x mole per milliliter isotonic saline solution) potentiated the effective dose of norepinephrine more than 100 times. The greatest potentiation

Theophylline(1 ml 0.02 M in 0.15 M saline) was administered to embryonic chicks (Hamburger-Hamilton developmental stages 24.27). The drug was topically applied to the surface of the chorioallantoic membrane in the vicinity of the embryonic heart. At necropsy, 63% (44/70) of the theophylline-exposed embryos demonstrated an aneurysm in the ascending aorta. Among the cases exhibiting aortic aneurysm, 48% (21/44) of the embryos demonstrated ventricular septal defects. Four cases of truncus arteriosus were also observed. The morphogenesis and mechanism of theophylline-induced cardiac defects with accompanying aortic aneurysm may be related to the development of the conotruncal region of the heart.

A premature infant with trisomy 18 had cardiac defects including parachute mitral valve, coarctation of the aorta, and a subcristal ventricular septal defect. The parachute mitral valve was funnel-shaped with a moderately-thick cone of fibrous tissue around the annulus and an eccentric orifice. Many forms of congenital cardiac defects have been described in the 18 trisomy syndrome. However, parachute mitral valve has not, to our knowledge, been part of the spectrum of cardiac defects in trisomy 18. Other anomalies were absent radii, omphalocele, and cleft palate and cleft lip; these have been observed previously in the 18 trisomy syndrome.