We found that patients with orthostatic protein-uria had entrapment of the left renal vein (LRV) by the aorta and superior mesenteric artery (SMA). Of 15 patients studied, ultrasonographic examination showed 13 cases of typical LRV entrapment with prestenotic dilatation, and 2 cases of mild LRV compression between the aorta and SMA. Intra-arterial digital subtraction angiography and monitoring of pull-back pressure from LRV to the inferior vena cava (IVC) were performed on 2 patients with 4+ proteinuria. Accumulation of contrast medium was seen with mild back-flow to the collateral veins, and pressure gradients between LRV and IVC were 4 mmHg and 8 mmHg, respectively. Eighty school-children formed a control group and were investigated ultrasonically. Nine showed typical LRV entrapment, among whom 3 had moderate to massive orthostatic proteinuria. The discovery of LRV entrapment in patients with orthostatic proteinuria gives definite evidence of LRV congestion and may be possibly a cause of massive protein secretion from the left kidney.

A case of pancreatic tumor in a six-year-old girl is presented. The tumor had histologic characteristics of acinar cell carcinoma with endocrine component. Grossly, it was encapsulated and attached to the tail of the pancreas, measuring 8 cm in the greatest diameter. Histologically, the tumor was composed of medium-sized tumor cells, with mild pleomorphism showing mainly acinar structures. Many of these tumor cell contained fine granules that were periodic acid-Schiff positive, diastase resistant, and positive with dimethylaminobenzaldehyde nitrite strain for tryptophan, and some contained granules that were positive with Grimelius stain and positive with peroxidase-antiperoxidase technic for gastrin. Electron microscopy revealed two types of membrane-bound granules in the tumor cells. The larger granules measured 400-700 nm in diameter and appeared to be zymogen granules, while the smaller ones measured 100-200 nm in diameter and appeared to be neuroendocrine granules. Some cells contained both granules. The postoperative course of the patient was excellent, and she was alive and well 13 years after operation. This may be the second reported case of acinar-endocrine cell tumor of the pancreas.

We treated an 11-year-old girl with spinal cord compression near an epidural tumor. Bone marrow examination confirmed the diagnosis of acute lymphoblastic leukemia (ALL). To reduce the compression we treated her immediately with high-dose dexamethasone and vincristine administered intravenously along with local irradiation. Three days later, radiation was discontinued because magnetic resonance imaging showed that the spinal cord compression was reduced. Complete remission has continued without evidence of neurologic sequelae for more than 3 years since diagnosis. Rapid reduction of the blasts resulted in tumor lysis syndrome, which was treated with conventional management and additional diuresis without hemodialysis. Epidural spinal cord compression in childhood ALL can be treated effectively with systemic chemotherapy and local radiotherapy without laminectomy.

We retrospectively studied 12 Japanese children (8 boys, 4 girls) with idiopathic membranous nephropathy (IMN), aged 2.9-15.8 (mean 7.7) years at onset. All patients were identified through either screening or a routine urinalysis; proteinuria was present in all, haematuria, which was macroscopic in 4, in 11. Three had nephrotic syndrome (NS) at or soon after onset. Stages on electron microscopy, performed in 10 patients, were I in 3, II in 5 and III in 2. Steroids alone or with cyclophosphamide were administered to 5 patients, including the 3 patients showing NS. Complete remission of proteinuria occurred in 8 patients 0.3-1.6 (mean 0.6) years after onset, and proteinuria did not recur. After a follow-up of 1.6-11.6 (mean 5.9) years, these 8 patients were in complete remission and the remaining 4 had only mild proteinuria; none had hypertension or impaired renal function. Thus, we infer that IMN in Japanese children may have a better course and outcome than IMN in non-Japanese children. Based on a comparative study of Japanese (previously reported cases added to ours) and non-Japanese (mostly Caucasian) children with IMN, this was confirmed; it is possible that steroid therapy in Japanese patients is more effective in inducing remission of NS and preserving renal function.

We describe an infant with congenital pyloric atresia associated with junctional epidermolysis bullosa, which is now recognized as an autosomal recessive syndrome. Laparotomy revealed a membrane in the pyloric antrum. Clinical manifestations included intractable diarrhoea and malnutrition due to protein-losing gastroenteropathy. A satisfactory nutritional state could not be obtained during the clinical course and the child died.