Is retinopathy in pancreatitis caused by leukocyte emboli?

The theory of granulocyte embolization in the retinal arterioles in acute pancreatitis cannot account for several aspects of the ophthalmic complication. Therefore we studied retinal circulation by fluorescein angiography, light and transmission electron microscopy following the first six hours of acute experimental necrotizing pancreatitis. The studied period was characterized by high serum lipase and amylase concentrations, hypocalcemia, necrosis of the pancreatic tissue and preceded the development of hypovolemic shock. Ophthalmoscopy and fluorescein angiography revealed no pathologic alterations and no granulocyte aggregation was found. Our results suggest that granulocyte aggregation induced by pancreatic enzymes is not the reason for the ophthalmic circulatory disturbances in acute pancreatitis.     

Combined treatment of symptomatic gallbladder stones by extracorporeal shock-wave lithotripsy (ESWL) and instillation of methyl,Tert-butyl ether (MTBE)

Twenty-four patients with symptomatic gallbladder stones (12 radiolucent and 12 calcified) were treated by a combined approach of extracorporeal shock-wave lithotripsy (ESWL) and subsequent instillation of methyltert-butyl ether (MTBE). The patients received a mean of 1500±185 shock-wave discharges. The mean instillation time of MTBE was 13±4.2 hr. Treatment was tolerated without major adverse effects. Within a time period of three to five days eight of 12 patients with pure radiolucent stones and four of 12 with calcified stones became stone-free. After a median follow-up of five months (range: one week to 26 months), a total of 11 patients (92%) with radiolucent stones and of eight patients (66%) of those with calcified stones were free of stones, fragments, or debris. These clearence rates appear high when compared with reports on monotherapy with ESWL or MTBE, suggesting a positive effect of a combined approach in selected patients. Two patients exhibited recurrent stones after six and seven months respectively.Supported partly by a grant from the Koerber Foundation.     

Hypotension in insulin hypoglycemia in diabetics with autonomic neuropathy

Hypotension was observed in 11 cases of hypoglycemic condition of 7 diabetic patients suffering from autonomic neuropathy. Following the iv. administration of glucose the hypotension became stronger. Upon glucagon the blood pressure kept decreasing. Neuropathy of the symphathetic nervous system, the osmotic effect of glucose and the vasodilating effect of glucagon were the causes of the hypotension. Hypotension in hypoglycemia is a symptom referring to autonomic neuropathy. Hypotension aggravates the neuroglycopenic disturbance. Slow injection of glucose solution moderates the decrease of the blood pressure. The use of glucagon in the hypoglycemia of diabetic patients with autonomic neuropathy must be subjected to consideration.     

Adenylosuccinase deficiency: an inborn error of purine nucleotide synthesis

Clinical and biochemical data are presented on eight children with adenylosuccinase deficiency. This newly discovered inborn error of purine metabolism is characterized by an accumulation in body fluids of succinyladenosine (S-Ado) and succinylaminoimidazole carboxamide riboside (SAICA riboside), the dephosphorylated derivatives of the two substrates of adenylosuccinase. Six living children (three boys and three girls) and one deceased sibling displayed severe psychomotor retardation. Epilepsy was documented in five cases, autistic features in three, and growth retardation associated with muscular wasting in a brother and sister. In the cerebrospinal fluid, plasma and urine of these patients, the S-Ado/SAICA riboside ratio was between 1 and 2. In striking contrast, the eighth patient (a girl) was markedly less mentally retarded. Most noteworthy, the S-Ado/SAICA riboside ratio in her body fluids was around 5, suggesting that her milder psychomotor retardation was causally linked to this higher ratio. Adenylosuccinase deficiency was demonstrated in the liver of all seven living children, in the kidney of three patients in whom the enzymatic activity was measured, and in the muscle of three patients, including the two with muscular wasting. In fibroblasts of the six severely retarded patients, adenylosuccinase activity was reduced to approximately 40% of normal; in the patient with the higher S-Ado/SAICA riboside ratio, it reached only 6% of normal. The clinical heterogeneity of adenylosuccinase deficiency justifies systematic screening for the enzyme defect in unexplained neurological disease.Abbreviations AICAR aminoimidazole carboxamide ribotide - AMP adenosine monophosphate - APRT adenine phosphoribosyl transferase - HGPRT hypoxanthine-guanine phosphoribosyl transferase - IMP inosine monophosphate - PRPP phosphoribosyl pyrophosphate - S-Ado succinyladenosine - SAICA succinylaminoimidazole carboxamide - SAICAR succinylaminoimidazole carboxamide ribotide - S-AMP adenylosuccinate (succinyl AMP) - ZTP AICAR triphosphate Presented in part at the Vth International Symposium on Human Purine and Pyrimidine Metabolis, San Diego, California, 28 July-1 August, 1985