Isolated right diaphragmatic eventration mimicking congenital heart disease in utero

Diaphragmatic eventration is the upward displacement of the abdominal viscera secondary to a thin or paralytic diaphragm. Its clinical presentations and radiographic pictures are similar to those of diaphragmatic hernia. Prenatal diagnosis of diaphragmatic eventration is extremely rare. A pregnant woman was referred to us because of abnormal cardiac findings noted at 20 weeks of gestation. A diagnosis of partial anomalous pulmonary venous connection was made on the basis of our findings of right atrial enlargement with an abnormal vascular channel drainage to it. The infant was born via cesarean section at 40 weeks and developed complications of cyanosis immediately after birth. Postnatal imaging studies and surgical findings disclosed right side diaphragmatic eventration with liver and associated vasculature upward displacement into the right pleural cavity. The cardiac structure was otherwise normal. We conclude that when an abnormal vessel tracing and unexplainable cardiac chamber asymmetry is encountered, diaphragmatic eventration should be considered as one of the differential diagnoses. Correct recognition and transferral to the hospital for neonatal assistance may lead to timely and appropriate management of these fetuses.     

Reduction of hyperornithinemia with a low protein,low arginine diet and pyridoxine in patients with a deficiency of ornithine-ketoacid transaminase (OKT) activity and gyrate atrophy of the choroid and retina

Five patients with gyrate atrophy of the choroid and retina showed a 60% or greater decline in plasma ornithine levels during a five week trial of a low protein (10–15 g/day), low arginine (0.50–0.75 g/day) diet supplemented with essential amino acids and pyridoxine administration. These declines in plasma ornithine levels were seen in Patients 1–4 with the pyridoxine non-responsive variant and in Patient 5 with the pyridoxine responsive variant. No harmful systemic side effects were noted. Patients 1–4 continued on a modified low protein (20–35 g/day), low arginine (1.25–1.75 g/day) diet as tolerated and Patient 5 on pyridoxine alone. After one year no improvement was observed in visual acuities, visual fields, final dark adapted thresholds and full field electroretinograms for four patients. In contrast to the other four patients, Patient 3, with relatively poor control of plasma ornithine levels, showed signs of progression of the chorioretinal atrophy and further reduction of electroretinographic responses. Patients 1–4 continue on the dietary regimen and Patient 5 on pyridoxine alone to determine whether any lowering of plasma ornithine levels will modify the course of their ocular disease.     

Effects of chronic nicotine administration and age on various neurotransmitters and associated enzymes in male Fischer-344 rats

The acute, subacute and chronic effects of nicotine have been studied in male Fischer-344 rats. Two-month-old animals were injected sc with either 1.0 ml of 0.85% $\text{w}\text{v}$ NaCl/kg/day, or 1000 μg nicotine base/ml/kg/day for 4 days or for 2 or 22 months; injectables were formulated in 6% gelatin. Nicotine pretreatment did not significantly alter 5-hydroxytryptamine (5-HT) or norepinephrine (NE) concentrations in a variety of tissues, nor whole brain choline (Cho), acetylcholine (ACh) and acetylcholinesterase activity. γ-Aminobutyric acid concentrations in 8 brain areas were all higher after chronic nicotine administration, but histamine (HM) concentrations were variably affected in a variety of tissues. The uptake of 5-HT into the heart and gastrointestinal mucosa, and of NE into the heart and aorta, was not affected by nicotine treatment. However, uptake of NE into synaptosomes prepared from an homogenate of whole brain was depressed. Urinary 5-hydroxyindoleacetic acid (5-HIAA) excretion was significantly higher in nicotine-treated rats compared to control animals both during ad libitum feeding and during food deprivation. In addition, urinary 5-HIAA excretion was significantly higher in nicotine-treated rats with food deprivation compared to ad libitum feeding. Histidine decarboxylase (HD) and dopa decarboxylase (DD) activities in a variety of tissues were variably affected by nicotine; however, both enzymes showed diminished activity in the gastric mucosa following the alkaloid. Cardiac, aortic and hepatic catechol-O-methyltransferase (COMT) activity was not altered by nicotine administration. Hepatic, aortic and cerebral monoamine oxidase (MAO) activity was unchanged, but an increase in myocardial activity, and a decrease in hepatic activity were observed depending upon the substrate.Several changes were observed between young (4-month-old) and old (24-month-old) rats. Bone marrow (femur) 5-HT and HM concentrations decreased with age, whereas 5-HT levels in the gastrointestinal area, and HM concentrations in general, increased with age. Uptake of NE and 5-HT into the heart was reduced with age, whereas HD and DD activity showed no consistent change in the tissues examined. COMT activity significantly increased in the liver and aorta, but not in the heart, with age. Conversely, MAO activity increased in the myocardium but not in the liver, brain or aorta, with age.     

Discordant 99mTc sulfur colloid and 67Ga-citrate scintigrams in Aspergillus splenic abscesses

A 43-year-old man with acute undifferentiated leukemia developed high fever and stomatitis followed by severe abdominal pain after completion of a first course of chemotherapy. A ^99mTc-sulfur colloid (SC) scan showed multiple, irregular defects in an enlarged spleen. A ⁶⁷Ga-citrate scan showed uniform tracer distribution except for a large defect in the upper portion of the spleen. The size of the spleen in the ⁶⁷Ga-scan was much larger than in the SC scan. At surgery an abscess cavity was found between the spleen and the greater gastric curvature with multiple smaller abscesses on the splenic capsule and throughout the splenic parcnchyma. A hematoma was present in the upper pole of the spleen. The disparate splenic size and seemingly discordant distribution patterns of tracer in the ^99mTc- and ⁶⁷Ga-scans were fully explained by histopathological study of the surgically resected spleen.