A Case of Kimura's Disease Occurring During Remission of Steroid-responsive Nephrotic Syndrome

Kimura's disease is a rare chronic inflammatory disease of unknown etiology which appears primarily in young Asian males as non-tender subcutaneous swellings in the head and neck region. Histologic characteristics are the presence of lymphoid follicles, vascular proliferation and infiltration of eosinophils. Peripheral eosinophilia and elevated serum IgE are frequently combined. Systemic steroid therapy with surgical excision is the mainstay of treatment, though recurrence after surgery or discontinued steroid treatment is common. It has been known that about 16% of the cases are associated with renal diseases, particularly nephrotic syndrome. We present an 8-year-old boy with a past history of steroid-responsive, infrequently relapsing nephrotic syndrome who developed right buccal swelling and peripheral eosinophilia during the remission state. He has been managed with surgical resection, steroid and cyclosporine due to multiple recurrences.

Predictive Factors for Relapse in Children with Steroid Responsive Nephrotic Syndrome

PURPOSE: Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. METHODS: The study group consisted of 73 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. RESULTS: The age(mean+/-SD) of patients was 4.53+/-2.53 years old. The male to female ratio was 52:21. In 95%, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables:age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.006). CONCLUSION: The rapidity of response is expected to be one of the predictive factors for relapse in children with SRNS.

Risk Factors for the First-Year Relapse in Children with Nephrotic Syndrome

PURPOSE: This study aimed to evaluate risk factors of the first year relapse in children with nephrotic syndrome(NS) without the need for biopsy. METHODS: We reviewed, retrospectively, 78 children diagnosed with steroid responsive nephrotic syndrome between July 1997 and June 2002. Median years to follow up were 4.4 years(range: 1-5 years). We divided the patients into two groups(group I: primary responders with no relapse or with only two relapses in the first year after initial response; group II: initial steroid responders with three or more relapses within the first year). We retrospectively reviewed and compared variables - sex, onset age, serum albumin, serum cholesterol, 24 hours urinary protein, creatinine clearance(Ccr), presence of hematuria and hypertension(HTN), and days from initial attack to remission. RESULTS: Of 78 patients(male: 61(78.2%), female: 17(21.8%), age range 1.1 years to 14 years, median 5.1+/-3.0 years), 47(60.3%) were in group I and 31(39.7%) were in group II. There were no statistically significant differences in sex, serum albumin, serum cholesterol, 24 hours urinary protein, Ccr, presence of hematuria or HTN. The median age of onset showed no statistical difference between the two groups. However, if the patients are dividing into two groups according to the age of onset of three-years, patients theree yrs old or less fit into group II, as opposed to patients older than three yrs in age(63.2% vs. 32.2%, P<0.05). The days from initial attack to remission was longer in group II(12.9+/-0.5 vs. 16.2+/-1.1, P<0.05). CONCLUSION: We may conclude that the age of onset of three yrs old or less, and the longer time remission to initial steroid therapy, are risk factors of the first year relapse.

A Childhood Case of Nephrotic Syndrome Complicated with Cerebral Venous Sinus Thrombosis Followed by Left Popliteal Artery Thrombosis

We report a 10-year-old male with steroid-responsive nephrotic syndrome which was complicated by cerebral venous sinus thrombosis including superior sagittal sinus and left popliteal artery thrombosis following 5 months later. At each thrombotic episode, he suffered from relapse of massive proteinuria with hypovolemic crisis, and from hemoconcentration with dehydration due to vomiting or diarrhea. Brain MR angiography confirmed thromboses of superior sagittal sinus, straight sinus, vein of Galen and occipital sinus. Diagnosis of left popliteal artery thrombosis was made according to the clinical observations including pulseless left dorsalis pedis artery, pain and coldness of left lower leg, and reperfusion injury of calf muscle after heparinization. Each thrombotic episode was successfully managed with heparinization, oral warfarin therapy and fresh frozen plasma for the antithrombin III replacement.