Leukoencephalopathy Following CNS Prophylaxis Therapy in Pediatric Leukemia: MR Imaging Findings

PURPOSE: To evaluate the MR imaging findings and the usefulness of MR imaging in the diagnosis and followup of leukoencephalopathy following CNS prophylaxis therapy in pediatric leukemia. MATERIALS AND METHODS: We retrospectively evaluated the MR imaging findings of eight children with white matter abnormalities on MR out of seventeen acute leukemic patients with various neuropsychiatric symptoms who received intrathecal methotrexate administration, with or without cranial irradiation. In all cases, initial MR was performed within a week of the onset of neuropsychiatric symptoms. Follow-up MR was performed one to sixteen months after initial study, and the MR imaging findings were compared with the initial findings. RESULTS: The initial MR imaging findings were classified into three categories: focal or multifocal white matter abnormalities (3/8), and diffuse white matter abnormalities without enhancement (3/8), and diffuse white matter abnormalities with enhancement (2/8). At follow-up MR, diffuse or focal atrophic changes were noted in all children. White matter abnormalities improved in two out of three patients with focal or multifocal white matter abnormalities. In five with diffuse white matter abnormalities, the extent of these showed no significant change, but contrast enhancement was markedly reduced in two children in whom diffuse white matter abnormalities with enhancement had been demonstrated. CONCLUSION: In pediatric leukemia, the MR imaging findings of leukoencephalopathy following CNS prophylaxis therapy are variable, but are specific with the clinical history of neuropsychiatric symptoms after intrathecal methotrexate administration, with or without cranial irradiation. The MR imaging is valuable in the diagnosis and follow-up of leukoencephalopathy following CNS prophylaxis therapy in pediatric leukemia.

Clinical Characteristics of Minimal Change Nephrotic Syndrome in Adults / 대한신장학회잡지

We evaluated retrospectively the clinical characteristics of 26 adult nephrotic syndrome patients with minimal-change disease who were followed up for more than six months. Median follow up period was 23months. They consisted of 16 men and 10 women, and the mean age was 34.3 years (range 18-68 years). We administered prednisolone 1.0mg/Kg body weight to the patients for eight weeks and tapered it gradually. Complete remission was obtained in 22 (85%) patients treated with prednisolone and 54% of them reached complete remission in four weeks after beginning prednisolone treatment. Nine patients who initially responded to prednisolone therapy relapsed. Two of these nine patients showed single relapse and seven patients had frequent relapses during the study period. Cyclophosphamide or cyclosporin A was added when the patients did not respond to prednisolone and showed frequent relapse. Of the six patients treated with cyclophosphamide, four patients who were frequent relapsors and one patient who was a non-reponder to prednisolone went into complete remission. The other prednisolone non-responder did not remit. Microscopic hematuria was found in three patients and these patients showed elevated serum creatinine level at the time of diagnosis. Seven patients showed renal impairment at the time of renal biopsy. All of them recovered from renal failure after treatment. Six patients showed complete remission of nephrotic syndrome after prednisolone and/or cyclophosphamide treatment. There was no patient who showed a deterioration of renal function during the study period. In conclusion, complete remission was obtained in 85% of adult minimal change nephrotic syndrome patients and 41% of patients relapsed within one year after attaining complete remission. The serum level of creatinine and microscopic hematuria at the time of diagnosis were not associated with poor prognosis during the study period.

A Case of Scrub Typhus Complicated with Acute Renal Failure Meningoencephalitis and Hepatitis / 대한내과학회지

Scrub typhus is a zoonosis transmitted by a chigger of trombiculid mite and manifested with fever, skin rash, myalgia, and hepatitis etc. The renal involvement of the disease is prese-ted with transient microscopic hematuria and/or proteinuria. However acute renal failure or meningoencephalitis is very rare. We report a case of scrub typhus manifested with acute renal failure, meningoencephalitis, and hepatitis. The patient was treated with doxycycline and continuous arteriovenous hemofiltration (CAVH) and recovered successfully.

Acute Renal Failure and Hepatitis Due to Ingestion of Raw Fish Gall Bladder / 대한신장학회잡지

Since 1976, several cases of acute renal failure and acute hepatitis after ingestion of raw carp bile have been reported. We experienced 1 case of acute renal failure and hepatitis after ingestion of the raw fish(Cyprinus carpio nudus) gall bladder. The patient ate raw fish gall bladder to improve the health. The size of the fish he ate was over 1 m and that of the gall bladder was about an egg size. The clinical manifestations were characterized by dizziness, headache, diffuse abdominal pain, both flank pain, nausea, vomiting and diarrhea. These symptoms were followed by gross hematuria, oliguria and jaundice. Elevation of blood urea nitrogen, creatinine, transaminases lasted for about 2 weeks. Biopsy of the kidney was performed 13 days after ingestion of raw fish gall bladder. The kidney pathology showed recovery phase of acute tubular necrosis. The patient recovered with conservative treatment.

The Clinicopathological Characteristics of Thin Glomerular Basement Membrane Nephropathy / 대한신장학회잡지

Thin glomerular basement membrane nephropathy, also called benign recurrent hematuria, is characterized by diffuse thinning of the glomerular basement membrane and by hematuria. The present study was based on a retrospective review of 366 native kidney biopsies performed at Chungnam National University Hospital from January 1993 to November 1996. Fifteen of these cases satisfied the criteria for definition of thin glomerular basement membrane nephropathy, placing the incidence of the disease at 4.0%. The criteria for definition of the disease are 1) normal findings by light microscopy, 2) no deposits by immunofluorescent microscopy, and 3) diffuse thinning of the glomerular basement membrane by electronmicroscopy. The studied patients had a mean age of 35.8 years (range from 14 to 59) and included 12 females and 3 males. A positive family history was present in one case. All pateints were normotensive and had hematuria (four cases showed gross hematuria). Seven patients had mild proteinuria, but two patients showed proteinuria of nephrotic range. The serum creatinine level was within normal limits except one. Abnormalities were not observed in immunoglobulins, complement component, serologic tests (RA, ASO) and HBsAg. Renal biopsy findings by light and immunofluoroscent microscopy did not reveal any abnormalities but diffuse thinning of the glomerular basement membrane (range from 154 to 279nm) was observed by electronmicroscopy. With the above results, patients with idiopathic renal hematuria with normal renal function and normal blood pressure, thin glomerular basement membrane nephropathy should be considered.

A case of orthognatic surgery in congenital alveolar-palatal cleft patient / 영남의대학술지

Pre-surgical and post-surgical change in adult clef lip and palate patient following Le Fort I advancement osteotomy combined with bone graft was evaluated clinically and cephalometically. We obtained a successful function and esthetic improvement. The bone graft of alveolo-palatal clefts provides a stable bone support to the adjacent teeth of the cleft area, and well union of adjacent bone tissue, the closure of oronasal fistula and improvement of speech problem. Le Fort I osteotomy following the ostectomy of nasal septum for advancement of the maxilla was obtained relative improvement of esthetics and functional occlusion. 1. The orthodontic correction was required before and after surgery. 2. In this case, there was a limited range of anterior advancement of the Premaxillary-segment due to the scar tissue. 3. After 8 months of operation, we could show the new bone deposition on the cleft sites in dental radiograph and then the prosthetic treatment to the missing teeth was done.