The prognosis of acute poststreptococcal glomerulonephritis (APSGN) is still a matter of considerable debate. In an attempt to elucidate this controversy, the medium-term prognosis was evaluated in 40 patients 5–9 years after the onset of the disease, and the long-term prognosis in 88 patients 10–17 years after the onset of the disease. All were sporadic cases. In the medium-term follow-up study, abnormalities were revealed in 5.0% (2/40) of the patients. Hypertension and proteinuria were the only abnormalities detected. In the long-term follow-up study, abnormalities were revealed in 6.8% (6/88) of the patients. Hypertension was found in 3.4, proteinuria in 2.3, and microhaematuria in 2.3% of the patients. In both studies, all patients had normal creatinine clearance. We conclude that the medium-and long-term outcome of patients with APSGN is excellent.
We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenal involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.
From January 1988 to September 1989, seven patients (4 girls and 3 boys, aged 3-12 years) with haemorrhagic fever with renal syndrome (HFRS) were hospitalised at the University Children's Hospital in Belgrade. In four patients the disease appeared as a family outbreak, the others were sporadic cases. In six patients the clinical presentation was suggestive of HFRS, as they had fever with headache, myalgia, sore throat and gastrointestinal illness followed by renal abnormalities. However, severe haemorrhagic syndrome with petechia, haematoma, haematemesis and melaena was present in one patient only. Renal disease presented as nephritic syndrome and/or acute renal failure. Five patients recovered after 2-3 weeks without sequellae, one patient had decreased renal function 17 months after the start of the disease and the remaining patient died. In six patients the diagnosis of HFRS was confirmed serologically by a significant rise in antibody titres against hantaviruses, while in the patient with the fetal and fulminant course of the disease, the diagnosis was established on the basis of epidemiological and autopsy findings. We suggest that children living in endemic areas who develop an ill-defined, febrile and gastrointestinal disease with renal dysfunction should be evaluated for HFRS.
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