The association between Henoch-Schönlein syndrome and renal amyloidosis: a proposal of a pathogenic mechanism.

The clinicopathological analysis of 250 pediatric cases that had been tissue-diagnosed with renal amyloidosis revealed three patients associated with Henoch-Schönlein syndrome (HSS). The renal biopsies revealed AA-type amyloidosis in all three cases. Case 2 displayed focal and segmental proliferative glomerulonephritis in the same renal biopsy. No evidence of well-known diseases and/or conditions for the development of AA-type amyloidosis except for familial Mediterranean fever (FMF) existed in these particular cases. On the other hand, the frequency of the association between FMF and HSS has been reported extensively in the literature; thus, common etiological factors can be considered. The mechanism involved in amyloid deposition in these cases may be related to HSS-associated chronic antigenemia and/or FMF through a mechanism that is, to date, unknown. Further studies are needed to clarify this causal relationship.

Hemolytic-uremic syndrome (HUS): a clinicopathological study of 15 cases.

Although hemolytic-uremic syndrome (HUS) is a clinico-pathological entity, renal biopsies are usually not indicated for diagnosis, and therefore, studies concerning the histological aspects of the syndrome are few. This study mainly describes the morphological characteristics of 15 tissue-diagnosed sporadic cases of HUS. The ages of the patients ranged between 10 mos. to 15 yrs., with five being under two. The male/female ratio was 2:3. The prodromal phase was present in 10 patients (67%) with gastrointestinal symptoms in four patients (27%) with neurological symptoms, and in three patients (20%) with upper respiratory infections. Five patients had HUS associated with diarrhea (D+) (three infants and two children), while the remaining ten patients (two infants and eight children) had no diarrhea (D-). E. coli was identified in the stool of four of the D+ cases, one of which was also associated with Shigella. The shortest clinical course was 14 days and the longest 55 days in 13 patients. The disease recurred after three months in one patient, and on three occasions in 15 months after onset of HUS in the other. Fourteen patients died and one biopsy-diagnosed case recovered after the acute phase. All patients had anemia (Hb 3.4-10 g/dl) and acute renal failure. Seven cases demonstrated Burr cells, eight cases had thrombocytopenia and six cases oliguria/anuria. Microscopic hematuria was detected in four cases and gross hematuria in two cases. All patients revealed proteinuria and azotemia (40-200 mg/dl). Five/five (100%) cases had decreased creatinine clearance, 12/14 (86%) cases had increased uric acid levels, 9/14 (64%) cases had an electrolyte imbalance. Light microscopy revealed microangiopathic type involvement of the glomeruli in all cases. According to additional findings, the cases were classed into three histological groups: type 1 showing cortical necrosis (3 cases), type 2 predominant glomerular and arteriolar involvement (11 cases) and type 3 predominant arterial involvement (1 case). All cases were considered primary HUS except for one which was associated with membranous glomerulonephritis. (D+) HUS cases were predominantly of the microangiopathic type, similar to the (D-) group; the latter being contrary to the literature. Hypertension was present in 67% of cases and there was no correlation found between the clinical duration of HUS and the histological type. All five patients studied immunohistologically revealed a nonspecific type fibrinogen deposition. Extra-renal microangiopathy was demonstrated in the adrenals, stomach, pancreas, liver and skin in two necropsies studied.

Optic glioma of childhood: clinical, histopathological, and histochemical observations.

This report documents the long-term clinical and histopathological behaviour of eight intraorbital and 16 intracranial optic nerve gliomas and relates the therapeutic data to the prognosis for both visual acuity and survival. The mean age at onset of symptoms was 8.6 years and at the diagnosis 10.9 years. It is generally held that proptosis is mild in intraorbital glioma, but we encountered marked proptosis ranging from 7 to 12 mm in six of the eight intraorbital gliomas, which contained abundant Alcian-blue-positive mucoid material. Of the patients with intracranial optic nerve gliomas 37.5% survived for a mean of eight years after treatment with radiotherapy or surgery combined with radiotherapy. At follow-up ranging from five months to 11 years only one of the six patients with intracranial gliomas had full visual acuity. Our observations emphasise that, although optic nerve gliomas are benign hamartomas, the prognosis for visual acuity and survival is unfavourable in cases which are diagnosed and treated late. Histopathological and histochemical observations suggest that increase in the amount of mucoid material may contribute to rapid enlargement of intraorbital and intracranial optic gliomas.

Post-irradiation osteosarcoma of the iliac bone: a case report.

A case of osteosarcoma of the iliac bone developing 12 years after the successful management of childhood rhabdomyosarcoma is presented. The frequency of secondary tumors, mainly bone malignancies, following therapeutic irradiation in the pediatric age-group, and the criteria for the diagnosis of radiation-induced bone sarcoma are discussed.

The association of nephrotic syndrome and renal vein thrombosis: a clinicopathological analysis of eight pediatric patients.

Cases with a pathological diagnosis of renal venous thrombosis (RVT) associated with nephrotic syndrome (NS) were studied retrospectively for clinicopathological evaluation. The material consisted of 21 RVT cases which were diagnosed in 2000 consecutive pediatric necropsies, with an overall incidence of about one percent. Eight of the 21 RVT cases were associated with nephrotic syndrome (34%), and this group formed 0.4 percent of the total necropsies in our pediatric center. The glomerulopathies of these nephrotic patients consisted of three cases of Finnish-type congenital NS (FCNS), three cases of renal amyloidosis secondary to familial Mediterranean fever, and two cases of membranoproliferative glomerulonephritis (MPGN). The presence of sepsis associated with disseminated intravascular coagulation, and the morphological age of the thrombi suggested that the RVT was secondary to sepsis in the FCNS cases. In the MPGN and secondary renal amyloidosis cases, the long duration of both the nephrotic state and the administration of diuretics along with glucocorticoid treatment and also the newly formed thrombi without infarction are strong evidences, although not definite, that the RVT developed as a complication of the glomerulopathy. Even though there were no definite clinical criteria for the diagnosis of most of the RVT cases, we would like to emphasize the importance of flank pain, the rapid deterioration of renal functions in a stable nephrotic patient, as well as the hypercoagulable state in the consideration of the development of RVT which indicate the need for appropriate radiological studies for confirmation of this condition during life.