One hundred fifty of 490 patients undergoing open heart surgery had renal failure attributable to cardiopulmonary bypass. In 69, serum creatinine concentrations did not exceed 2 mg/dl and returned to normal by the fourth postoperative day. In 60 patients, serum creatinine attained levels between 2 and 5 mg/dl, oliguria did not develop, and recovery of renal function occurred within 4 to 37 days. Serum creatinine increased to levels exceeding 5 mg/dl in 21 patients, 11 of whom were oliguric. Despite dialysis, 14 of these patients died from cardiac causes or sepsis. Prolonged cardiopulmonary bypass time, hypotension, oliguria, low output syndrome, and hemoglobinemia during open heart surgery correlated with the development of renal failure postoperatively. Although severe renal failure was an uncommon complication after open heart surgery, its occurrence carried a grave prognosis.

A study of renal disease in familial dysautonomia identified excess glomerulosclerosis in 10 or 13 autopsied and biopsied patients. Sympathetic nerve terminals could not be found on renal vessels in biopsied tissue; they were invariably demonstrable in controls. Altered renovascular responsivity to systemic hypotension in familial dysautonomia may lead to ischemia and subsequent sclerosis of glomeruli. Review of 79 living outpatients showed that clinically overt renal disease was rare in familial dysautonomia. Nevertheless, frequent observations of elevations of serum creatinine concentrations (32% of patients) and blood urea concentrations (76% of patients) indicated a high prevalence of abnormality. An association was found between hypotension and renal dysfunction.

We describe a spectrum of clinical and morphologic features which may occur in idiopathic crescentic glomerulonephritis. Of note are patients who pursue a more indolent course to renal failure in contrast to typical rapidly progressive glomerulonephritis (RPGN). Patients following this protracted course have an insidious presentation with hematuria and renal insufficiency, and at the time of renal biopsy (usually after many months of clinical illness) show less extensive involvement with crescents, which are often in both cellular and fibrous stages. This form of crescentic glomerulonephritis is further distinguished from RPGN by frequent hypertension and the nephrotic syndrome, the absence of oligo-anuria and progression to renal failure over many months or even years.

Commonly available information on intestinal neuronal dysplasia (IND) is sparse. Especially well documented long-term courses are lacking. The aim of this study was to correlate defecation as a clinical parameter of the long-term course in malformations of the enteric nervous system with the morphological diagnosis. 57 children with intestinal neuronal dysplasia (IND) or aganglionosis with cranial intestinal dysganglionosis (agIND), diagnosed between 1983 and 1992, were analysed including histomorphological classification, collection of clinical data and evaluation of the defecation mode by questionnaire as a parameter of the long-term course. Of 29 dysganglionic (IND) patients, 9/29 cases (31 %) had been treated conservatively, 18/29 cases surgically (62.1%), in two children (6.9%) no therapy had been necessary. All 28 patients with Hirschsprung's disease and cranial IND (agIND) underwent resection. 46 of the children could be followed up 3.64 years after the end of the main therapeutic period and with a mean age of 6.7 years at the time of follow-up; 43.5% of the analysed children still showed severe constipation. 23.9% only were really cured; 15.2% had normal defecation still using conservative treatment and 17.4% had diarrhea. No significant difference was found between both groups, IND and agIND, and the results were independent of treatment modality. The results were much worse than in idiopathic constipation as reported in the literature and even worse in comparison to unselected Hirschsprung collectives. It has to be concluded that in IND with chronic constipation intensive long-term care is necessary and it is crucial that treatment algorithms should be outlined urgently together by pediatric gastroenterologists and pediatric surgeons. AgIND seems to need more extended resection following an exact histomorphological mapping by biopsies taken during enterostomy procedure.

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