Metabolic Impacts of Discontinuation and Resumption of Recombinant Human Growth Hormone Treatment during the Transition Period in Patients with Childhood-Onset Growth Hormone Deficiency

Background@#Discontinuing growth hormone (GH) treatment during the transition to adulthood has been associated with adverse health outcomes in patients with childhood-onset growth hormone deficiency (CO-GHD). This study investigated the metabolic changes associated with interrupting GH treatment in adolescents with CO-GHD during the transition period. @*Methods@#This study included 187 patients with CO-GHD who were confirmed to have adult GHD and were treated at six academic centers in Korea. Data on clinical parameters, including anthropometric measurements, metabolic profiles, and bone mineral density (BMD) at the end of childhood GH treatment, were collected at the time of re-evaluation for GHD and 1 year after treatment resumption. @*Results@#Most patients (n=182, 97.3%) had organic GHD. The median age at treatment discontinuation and re-evaluation was 15.6 and 18.7 years, respectively. The median duration of treatment interruption was 2.8 years. During treatment discontinuation, body mass index Z-scores and total cholesterol, low-density lipoprotein, and non-high-density lipoprotein (HDL) cholesterol levels increased, whereas fasting glucose levels decreased. One year after GH treatment resumption, fasting glucose levels, HDL cholesterol levels, and femoral neck BMD increased significantly. Longer GH interruption (>2 years, 60.4%) resulted in worse lipid profiles at re-evaluation. The duration of interruption was positively correlated with fasting glucose and non-HDL cholesterol levels after adjusting for covariates. @*Conclusion@#GH treatment interruption during the transition period resulted in worse metabolic parameters, and a longer interruption period was correlated with poorer outcomes. GH treatment should be resumed early in patients with CO-GHD during the transition period.

A cell function study on calcium regulation of a novel calcium-sensing receptor mutation (p.Tyr825Phe)

Purpose@#Autosomal dominant hypocalcemia with hypercalciuria is a genetic disease characterized by hypoparathyroidism with hypercalciuria. We discovered a novel variant (p.Tyr825Phe[Y825F]) of the CASR gene in a neonate with congenital hypoparathyroidism and hypercalciuria and conducted a cell function study to determine whether the CASR-Y825F variant was pathogenic. @*Methods@#To perform a functional study on CaSR-Y825F, we constructed expression vectors expressing wild-type (WT) CASR and CASR-Y825F. After transfection of each expression vector into HEK293 cells, we examined alterations in intracellular signaling. Mitogen-activated protein kinase (MAPK) signaling activity of HEK293 cells expressing CASR-WT or CASR-Y825F was determined. Changes in intracellular calcium ions ([Ca2+]i) by extracellular calcium ion ([Ca2+]e) stimulation were quantitatively compared and analyzed. @*Results@#Cells expressing CASR-Y825F showed elevated of MAPK signaling (phospho-ERK [pERK], phospho-JNK [pJNK], phospho-p38 [pp38]) and increased [Ca2+]i levels at low [Ca2+]e stimulation compared with cells expressing CASR-WT. Additionally, [Ca2+]i levels in HEK293 cells expression CASR-WT and CASR-Y825F were determined at 340 nm/380 nm wavelength ratios using Fura-2 AM. At [Ca2+]e concentrations of 2.5 mM and 3 mM, the ratios of CASR-Y825F cells were higher (2.6 and 3.5, respectively) than those of CASR-WT cells (1.04 and 1.40, respectively). @*Conclusion@#This cell function study proved that the CASR-Y825F expressed in HEK293 cells elevated MAPK signaling (pERK, pJNK, pp38) and increased [Ca2+]i to induce hypocalcemia.

A cell function study on calcium regulation of a novel calcium-sensing receptor mutation (p.Tyr825Phe)

Purpose@#Autosomal dominant hypocalcemia with hypercalciuria is a genetic disease characterized by hypoparathyroidism with hypercalciuria. We discovered a novel variant (p.Tyr825Phe[Y825F]) of the CASR gene in a neonate with congenital hypoparathyroidism and hypercalciuria and conducted a cell function study to determine whether the CASR-Y825F variant was pathogenic. @*Methods@#To perform a functional study on CaSR-Y825F, we constructed expression vectors expressing wild-type (WT) CASR and CASR-Y825F. After transfection of each expression vector into HEK293 cells, we examined alterations in intracellular signaling. Mitogen-activated protein kinase (MAPK) signaling activity of HEK293 cells expressing CASR-WT or CASR-Y825F was determined. Changes in intracellular calcium ions ([Ca2+]i) by extracellular calcium ion ([Ca2+]e) stimulation were quantitatively compared and analyzed. @*Results@#Cells expressing CASR-Y825F showed elevated of MAPK signaling (phospho-ERK [pERK], phospho-JNK [pJNK], phospho-p38 [pp38]) and increased [Ca2+]i levels at low [Ca2+]e stimulation compared with cells expressing CASR-WT. Additionally, [Ca2+]i levels in HEK293 cells expression CASR-WT and CASR-Y825F were determined at 340 nm/380 nm wavelength ratios using Fura-2 AM. At [Ca2+]e concentrations of 2.5 mM and 3 mM, the ratios of CASR-Y825F cells were higher (2.6 and 3.5, respectively) than those of CASR-WT cells (1.04 and 1.40, respectively). @*Conclusion@#This cell function study proved that the CASR-Y825F expressed in HEK293 cells elevated MAPK signaling (pERK, pJNK, pp38) and increased [Ca2+]i to induce hypocalcemia.

Clinical, endocrinological, and molecular features of four Korean cases of cytochrome P450 oxidoreductase deficiency

Purpose@#Cytochrome P450 oxidoreductase (POR) deficiency is a rare autosomal recessive disorder caused by mutations in the POR gene encoding an electron donor for all microsomal P450 enzymes. It is characterized by adrenal insufficiency, ambiguous genitalia, maternal virilization during pregnancy, and skeletal dysplasia. In this study, we investigated the clinical, hormonal, and molecular characteristics of patients with POR deficiency in Korea. @*Methods@#This study included four patients with POR deficiency confirmed by biochemical and molecular analysis of POR. Clinical and biochemical findings were reviewed retrospectively. Mutation analysis of POR was performed by Sanger sequencing after polymerase chain reaction amplification of all coding exons and the exon-intron boundaries. @*Results@#All patients presented with adrenal insufficiency and ambiguous genitalia regardless of their genetic sex. Two patients harbored homozygous p.R457H mutations in POR and presented with adrenal insufficiency and genital ambiguity without skeletal phenotypes. The other two patients with compound heterozygous mutations of c.[1329_1330insC];[1370G>A] (p.[I444Hfs*6];[R457H]) manifested skeletal abnormalities, such as craniosynostosis and radiohumeral synostosis, suggesting Antley-Bixler syndrome. They also had multiple congenital anomalies involving heart, kidney, and hearing ability. All patients were treated with physiologic doses of oral hydrocortisone. @*Conclusion@#We report the cases of 4 patients with POR deficiency identified by mutation analysis of POR. Although the study involved a small number of patients, the POR p.R457H mutation was the most common, suggesting founder effect in Korea. POR deficiency is rare and can be misdiagnosed as 21-hydroxylase or 17α-hydroxylase/17,20-lyase deficiency. Therefore, molecular analysis is critical for confirmatory diagnosis.

An Alport syndrome boy with Van Wyk-Grumbach syndrome induced by prolonged untreated congenital hypothyroidism

Alport syndrome (AS) is a rare genetic disorder that causes progressive nephritis and is more common among males. Studies have reported an association between thyroid antibodies and hypothyroidism in patients with AS, but the relevance of this relationship is under debate. Prolonged untreated hypothyroidism induces short stature, abnormal pubertal development, and various other symptoms. However, children with long-standing hypothyroidism rarely present with signs of precocious puberty, or Van Wyk-Grumbach syndrome (VWGS). We report the case of a boy, 8 years and 4 months old, who had VWGS caused by prolonged untreated congenital hypothyroidism and AS. The boy had repeated gross hematuria and proteinuria and was diagnosed with AS by renal biopsy and genetic testing. He had normal renal function but severe growth retardation and hypothyroidism. Obesity, bone age delay, hyperlipidemia, and abnormal increased testicle size were also present due to prolonged untreated hypothyroidism. His thyroid antibody titer elevation was unclear, although ultrasonography and thyroid scanning showed a decrease in thyroid volume. We diagnosed the patient with congenital hypothyroidism caused by thyroid dysgenesis. VWGS was diagnosed due to hypothyroidism, delayed bone age, and pseudoprecocious puberty. To the best of our knowledge, this is the first report of a prepubertal Korean boy with prolonged untreated congenital hypothyroidism complicated by VWGS in AS.

Severe recurrent nocturnal hypoglycemia during chemotherapy with 6-mercaptopurine in a child with acute lymphoblastic leukemia

Various endocrine dysfunctions occur during chemotherapy, including hypoglycemia. However, reports of hypoglycemia associated with 6-mercaptopurine (6-MP) are rare. Herein, we report an 8-year-old boy with severe symptomatic hypoglycemia likely due to 6-MP during chemotherapy. He had been diagnosed with acute lymphoblastic leukemia 3 years previously and was in the maintenance chemotherapy period. Treatment included oral dexamethasone, methotrexate, and 6-MP, of which only 6-MP was administered daily. Hypoglycemic symptoms appeared mainly at dawn, and his serum glucose dropped to a minimum of 37 mg/dL. Laboratory findings showed nothing specific other than increased serum cortisol, free fatty acids, ketone, alanine aminotransferase, and aspartate aminotransferase. Under the hypothesis of hypoglycemia due to chemotherapy drugs, we changed the time of 6-MP from evening to morning and recommended him to ingest carbohydrate-rich foods before bedtime. Hypoglycemia improved dramatically, and there was no further episode during the remaining maintenance chemotherapy period. To the best of our knowledge, this is the first report of this type of hypoglycemia occurring in an Asian child including Korean.

De novo a novel variant of CaSR gene in a neonate with congenital hypoparathyroidism

Autosomal-dominant hypocalcemia with hypercalciuria (ADHH) is a genetic disease characterized by hypoparathyroidism with hypercalciuria. Most patients with ADHH have calcium-sensing receptor (CaSR) gene mutations. The CaSR gene controls parathyroid secretions, and mutations in this gene can be detected via changes in serum calcium level. The activating mutation of the CaSR gene results in familial or sporadic ADHH. Most activating mutations of the CaSR gene are reportedly de novo missense mutations. This is the first case report of a novel activating variant of the CaSR gene in a neonate with congenital hypoparathyroidism with hypomagnesemia and hypercalciuria. We also report the 3-month follow-up management of the patient.

Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency

Pituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis. Diagnosis is confirmed using magnetic resonance imaging. Patients with PSIS have a variable degree of pituitary hormone deficiency and a wide spectrum of clinical manifestations. The clinical course of the disease in our patient is similar to that of a syndrome of inappropriate antidiuretic hormone secretion. This is thought to be caused by failure in the suppression of vasopressin secretion due to hypocortisolism. To the best of our knowledge, there is no case report of a patient with PSIS presenting with hyponatremia as the first symptom in Korean children. Herein, we report a patient with PSIS presenting severe recurrent hyponatremia as the first symptom, during adolescence and explain the pathophysiology of hyponatremia with secondary adrenal insufficiency.

Initial steroid regimen in idiopathic nephrotic syndrome can be shortened based on duration to first remission / 소아과

PURPOSE: The use of a 12-week steroid regimen (long-term therapy, LT) for the first episode of idiopathic nephrotic syndrome (NS) reportedly induces a more sustained remission and lower relapse rate than previous regimens, including an 8-week steroid regimen (short-term therapy, ST). Here, we assessed the potential for selective application of 2 steroid regimens (LT vs. ST) based on the days to remission (early responders [ER] vs. late responders [LR]) for the first idiopathic NS episode in children. METHODS: Patients were divided into 4 subgroups (ST+ER, ST+LR, LT+ER, and LT+LR) according to the initial steroid regimen used and rapidity of response; the baseline characteristics, relapse rates, and cumulative percentage of children with sustained remission were then compared among the 4 subgroups. RESULTS: Fifty-four children received ST, and the remaining 45 children received LT. As observed in previous studies, children receiving LT showed significantly lower relapse rates during the first year after the first NS episode than those receiving ST. The ST+ER group showed significantly lower relapse rates during the first one year and two years after the first NS episode than the the ST+LR group, whereas there were no significant differences of the relapse rates and duration to the first relapse between the ST+ER and LT+ER groups. CONCLUSION: We suggest that the initial steroid regimen in idiopathic NS patients can be shortened according to the duration to remission i.e., LT in patients achieving remission after the first week of steroid therapy, and ST in those achieving remission within the first week of steroid therapy.

Comparison of the Therapeutic Efficacy of Methylprednisolone Pulse Therapy and Oral Steroid Therapy in Children with IgA Nephropathy and HSP Nephritis Combined with Proteinuria

PURPOSE: The purpose of this study was to assess the therapeutic efficacy of methylprednisolone pulse therapy in children with IgA nephropathy and Henoch-Schonlein Purpura (HSP) nephritis combined with proteinuria. METHODS: We retrospectively reviewed the clinical records of 21 patients who were diagnosed with IgA nephropathy and HSP nephritis based on percutaneous renal biopsy. Of the 21 patients, 15 were diagnosed with IgA nephropathy and 6 were diagnosed with HSP nephritis. They had mild to severe proteinuria at the time of diagnosis or during follow-up. Group 1 (n=7) received methylprednisolone pulse therapy three times every couple of months, and Group 2 (n=14) received oral steroid therapy. The follow-up periods for Group 1 and 2 were 14.0 (9-54) months and 26.5 (14-34) months, respectively. There was no significant difference in the follow-up duration between the two groups. RESULTS: The average age at diagnosis and biopsy was lower in Group 1 compared to Group 2, but it was not significantly different. At admission, all patients in both groups had hematuria and 5 patients (71.4%) of Group 1 and 14 patients (100%) of Group 2 had proteinuria. Before treatment, there was no significant difference of spot urine protein/creatinine ratio between the two groups. During follow-up, 7 patients of Group 1 (100%) and 10 patients of Group 2 (71.4%) showed complete improvement of proteinuria and the spot urine protein/creatinine ratio in Group 1 was significantly lower than Group 2. CONCLUSION: In patients with IgA nephropathy and HSP nephritis with proteinuria, methylprednisolone pulse therapy was more effective than oral steroid therapy in the reduction of proteinuria. To investigate the effects on long-term prognosis, large-scale prospective studies are needed.

Efficacy of Short-Term Growth Hormone Treatment in Prepubertal Children with Idiopathic Short Stature

PURPOSE: It has been reported that daily recombinant human growth hormone (GH) treatment showed beneficial effects on growth in prepubertal children with idiopathic short stature (ISS). The present study aimed to validate the GH (Eutropin(R)) effect on growth promotion and safety after short-term GH treatment. MATERIALS AND METHODS: This study was an open-label, multicenter, interventional study conducted at nine university hospitals in Korea between 2008 and 2009. Thirty six prepubertal children with ISS were enrolled in this study to receive 6-month GH treatment. Yearly growth rate, height standard deviation score (SDS), and adverse events were investigated during treatment. RESULTS: After 26 weeks of GH treatment, the height velocity significantly increased by 6.36+/-3.36 cm/year (p<0.001). The lower end of one-sided 95% confidence interval was 5.22 cm/year, far greater than the predefined effect size. The gain in height SDS at week 26 was 0.57+/-0.27 (p<0.0001). Bone age significantly increased after GH treatment, however, bone maturation rate (bone age for chronological age) showed limited advancement. This 26-week GH treatment was effective in increasing serum levels of insulin-like growth factor (IGF)-I and IGF binding protein (IGFBP)-3 from baseline (p<0.0001). Eutropin was well tolerated and there were no withdrawals due to adverse events. No clinically significant changes in laboratory values were observed. CONCLUSION: This 6-month daily GH treatment in children with ISS demonstrated increased height velocity, improved height SDS, and increased IGF-I and IGFBP-3 levels with a favorable safety profile.

Various endocrine disorders in children with t(13;14)(q10;q10) Robertsonian translocation

PURPOSE: 45,XY,t(13;14)(q10;q10) karyotype can suggest infertility associated with more or less severe oligospermia in male adults. In addition, 45,XX,t(13;14)(q10;q10) karyotype carries reproductive risks such as miscarriage or infertility in female adults. However, reports on the phenotype of this karyotype in children are very rare. This study was done to observe various phenotypes of this karyotype in children. METHODS: Between January 2007 and December 2012, children diagnosed with 45,XY,t(13;14)(q10;q10) or 45,XX,t(13;14)(q10;q10) karyotype by chromosome analysis were analyzed retrospectively. RESULTS: Eight children (5 boys and 3 girls) were diagnosed with 45,XY,t(13;14)(q10;q10) or 45,XX,t(13;14)(q10;q10) karyotype. They ranged in age from 5 years and 6 months to 12 years and 4 months. The phenotypes of the study patients consisted of 1 hypogonadotrophic hypogonadism, 1 precocious puberty, 3 early puberty, 2 growth hormone deficiency (GHD) (partial) and 1 idiopathic short stature. As shown here t(13;14)(q10;q10) Robertsonian translocation shows a wide range of phenotypes. CONCLUSION: It can be said that t(13;14)(q10;q10) Robertsonian translocation shows various phenotypes from GHD to precocious puberty in children. Further large-scale studies are necessary.

Clinical manifestations of IgA nephropathy combined with thin glomerular basement membrane nephropathy in children

BACKGROUND: Immunoglobulin A nephropathy (IgAN) and thin glomerular basement membrane nephropathy (TBMN) are the most common causes of persistent hematuria during childhood. The objective of this study is to determine the difference in clinicl features and laboratory findings between pediatric patients with IgA deposited TBMN and IgAN alone. METHODS: Between January 2000 and March 2009, 95 children were diagnosed with IgAN by renal biopsy. Clinical features and laboratory findings of patients with isolated IgAN and with IgAN plus TBMN were compared; the children diagnosed with IgAN were compared to 127 children who had been diagnosed with TBMN alone during the same period. RESULTS: There were 71 (74.7%) of a total 95 patients that were diagnosed with isolated IgAN (Group 1); in 24 (25.3%) of the 95 patients IgAN was combined with TBMN (Group 2). There was marked difference in the gender distribution between Group 2 and isolated TBMN patients. The degree of proteinuria and pathologic severity was higher in Group 1 compared with Group 2. Gross hematuria was present in both groups. There were no distinguishing features in the other laboratory parameters. CONCLUSION: Patients with both IgAN and TBMN seem to have similar clinical features to patients with isolated IgAN; however, the latter tend to have better pathologic and laboratory findings, compared to the patients with IgAN alone.

Polyclonal gammopathy related to renal bleeding in a peritoneal dialysis patient / 소아과

Polyclonal gammopathy represents the diffuse activation of B cells and is usually related to inflammation or immune-related diseases. However, the mechanisms leading to polyclonal gammopathy are essentially speculative. Generally, infectious, inflammatory, or various other reactive processes may be indicated by the presence of a broad-based peak or band in the gamma region on serum protein electrophoresis results. A 15-year-old girl, who had been receiving peritoneal dialysis, presented with polyclonal gammopathy and massive gross hematuria. Renal artery embolization was performed, after which the continuous bleeding subsided and albumin-globulin dissociation resolved. This is a rare case of polyclonal gammopathy related to renal bleeding.

Changes of antithroglobulin antibody in children with congenital hypothyroidism

PURPOSE: It has been reported that antithroglobulin (anti-TG) antibody is increased in the sera of both children with transient congenital hypothyroidism and their mothers. And transplacental transport of thyroid autoantibody was proposed as the pathogenesis of transient congenital hypothyroidism. However this is not known in nontransient congenital hypothyroidism. This study was done to see changes of anti-TG antibody in children with nontransient congenital hypothyroidism. METHODS: Study patients consisted of 60 patients diagnosed as congenital hypothyroidism in the Department of Pediatrics, Kyungpook National University Children's Hospital, Daegu, Republic of Korea between January 2010 and March 2013. Healthy control were 45 children showing normal thyroid function. Anti-TG antibody and various laboratory tests were analyzed retrospectively, and compared in both children with congenital hypothyroidism and controls. RESULTS: Anti-TG antibody was significantly higher in children with congenital hypothyroidism compared to healthy controls, 119.4+/-34.7 U/mL versus 80.6+/-19.6 U/mL, respectively (P<0.001). There was no significant difference of anti-TG antibody in gender and age. CONCLUSION: We observed a significant increase of anti-TG antibody in children with nontransient congenital hypothyroidism compared to healthy controls. Further study focusing pathogenetic role of anti-TG antibody in nontransient congenital hypothyroidism is necessary. Furthermore, the clinical significance in the course of congenital hypothyroidism need to be known.

Methylprednisolone Pulse Therapy for Focal Segmental Glomerulosclerosis in Children: with the Mendoza Protocol / 대한신장학회지

PURPOSE: The clinical characteristics and pathological findings of patients with focal segmental glomerulosclerosis (FSGS) receiving methylprednisolone pulse therapy (MP pulse therapy) according to the Mendoza protocol were studied to evaluate its therapeutic efficacy. METHODS: The clinical course and pathological findings of 12 patients that were diagnosed with FSGS and treated according to the Mendoza protocol from 2001 to 2006 were reviewed retrospectively. RESULTS: Only three patients among the total twelve patients finished MP pulse therapy, who were diagnosed with minimal change nephrotic syndrome on the first renal biopsy and have preserved renal function at the recent follow-up. Among them, one patient (8%) achieved complete remission, but relapsed 28-months after the end of therapy, and one patient (8%) had a partial remission. Eight patients progressed to end-stage renal disease (ESRD) and four regressed during therapy. All eight patients that were diagnosed with FSGS, on the first renal biopsy, progressed to ESRD and required hemodialysis or kidney transplantation. The frequency of ESRD in this group was statistically significant. CONCLUSION: MP pulse therapy according to the Mendoza protocol showed low therapeutic efficacy; it appeared effective in only 17% and most of the patients (67%) progressed to ESRD. There may be close correlation between the severity of glomerular sclerosis at biopsy and ESRD. These results suggest that the indications for MP pulse therapy according to the Mendoza protocol in nephrotic patients with FSGS requires further clarification.

A Single-Arm, Phase III Study to Assess Efficacy and Safety after 6-Month-Treatment of Eutropin(TM) Inj. (Recombinant Human Growth Hormone) in Prepubertal Children with Short Stature due to Small for Gestational Age / 대한소아내분비학회지

PURPOSE: Recombinant human growth hormone is an effective therapy for short-statured children born small for their gestational age (SGA). This single-arm, multicenter, phase III clinical study of such children was designed to assess the efficacy and safety of treating them with recombinant human-growth-hormone (Eutropin(TM) Inj.) for 6 months. METHODS: Between 2005 and 2007, 30 treatment naive, prepubertal, short-statured SGA-born children were recruited as participants. Eutropin(TM) Inj. was administered for 6 months with a subcutaneous dose of 0.48 mg/kg/wk. The primary endpoint was the change in height velocity from the baseline to month 6. Various parameters were checked to obtain secondary outcome measures and to meet safety criteria. RESULTS: Height velocity significantly increased from 5.36 +/- 1.59 cm/yr at baseline to 10.66 +/- 2.03 cm/yr at month 6 (P < 0.0001). Secondary outcome measures (height velocity at month 3, height SDS for chronological age (CA), weight SDS for CA, bone maturation, and IGF-I and IGFBP-3 levels) were also significantly increased. Eutropin(TM) Inj. was well tolerated and safe over 6 months of treatment. CONCLUSION: The clinical efficacy and safety of Eutropin(TM) Inj. was demonstrated for the 6 month treatment of prepubertal children with short stature due to SGA. Further long-term study is needed.

Clinical Usefulness of Ambulatory Blood Pressure Monitoring in Children and Adolescents

PURPOSE: With increasing prevalence of hypertension (HTN) in children and adolescent, pediatricians have become more interested in blood pressure (BP) measurements. The ambulatory blood pressure monitoring (ABPM) is known to be useful to differentiate true HTN and white coat HTN. The object of this study is to assess the clinical usefulness of ABPM in Korean children and adolescents. METHODS: A retrospective review of 51 patients in Kyungpook National University Hospital from January 2002 to February 2010 was done. All patients were 6-18 years old and underwent ABPM. We calculated the mean value of ABP, BP load, nocturnal dip and compared the results with the patients' diagnosis and characteristics. RESULTS: The mean age of the 51 patients was 17.8+/-1.8 years and 19 children were obese. 37 patients (72.5%) were truly hypertensive and 1 patient was diagnosed as masked HTN and 7 children (14%) as white coat HTN. The rest of the patients were normotensive. Among patients with white coat HTN, 5 were in a prehypertensive state. Mean systolic and diastolic BP load of patients with true HTN were significantly higher than non-hypertensive children (P<0.001). Although the nocturnal dip of all patients were below 10%, there was no statistical significance. The obese patients showed higher systolic and diastolic BP. Their systolic and diastolic BP load were significantly higher than non-obese patients (P<0.001). CONCLUSION: ABPM in children and adolescents seems to be a valuable tool in the assessment of white coat HTN and in the confirmation of true HTN. A considerable number of white coat HTN patients are revealed to be in a prehypertensive state and need close follow-up.

Clinical Features of Enuresis in Children with Diabetes Mellitus

PURPOSE: Diabetes mellitus (DM) is known as one of the common causes of secondary enuresis in children. However, enuresis in diabetic children is overlooked only as a symptom of polyuria due to hyperglycemia. We evaluated the prevalence of nocturnal enuresis in children with diabetes mellitus in this paper. METHODS: Among children with diabetes in three hospitals in Daegu area, 117 agreed to 'Tele research by means of a questionnaire'. RESULTS: Diabetic patients were divided into two groups: Nocturnal enuresis and non-nocturnal enuresis group. thirty-two of 117 (27.0%) patients were in enuresis group, with more daytime urination than non-nocturnal enuresis group (4.2+/-1.6/3.6+/-1.2 times, P =0.016). HbA1c at diagnosis was 12.0+/-2.3%/12.0+/-2.5%, and at follow-up 9.3+/-2.3%/8.3+/-2.3% (P =0.042). Insulin was administered at 1.1+/-0.5/1.1+/-0.4 units/kg/day. Ten children of enuresis (31.2%) group were monosymptomatic (MNE) and 22 (68.8%) children were non-monosymptomatic enuresis (non-MNE). Fourteen (43.8%) of enuresis group had persistent symptoms, with 5 MNE and 9 non-MNE each. HbA1c at diagnosis was 11.1+/-2.5, 12.4+/-2.1, higher in non-MNE (P =0.144). Average arousal during sleep was step 3.3+/-1.2, 2.5+/-1.0, higher in improved enuresis group (P =0.059). CONCLUSION: Nocturnal enuresis among DM patients is underestimated. However, considering psychological and social effects of enuresis in children, extensive and long-term studies are needed in the future to clarify relationship between prevalence and DM control.

Prevention of Recurrent FSGS with Cyclosporine and Plasmapheresis Prior to Renal Transplantation

We report on two children with a high risk of recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation that could be effectively prevented by prophylactic administration of cyclosporine combined with preemptive plasmapheresis prior to renal transplantation.