Kenichi Kano

Thyroid function in children with nephrotic syndrome

The thyroid function of seven children with untreated nephrotic syndrome who had a normal serum creatinine concentration was compared with that of the same patients in remission and age-matched controls. There was a significant decrease in serum thyroxine (T4), tri-iodothyronine (T3) and thyroid-binding globulin (TBG) concentrations in untreated nephrotic children compared with the same patients in remission and age-matched controls. Most values for serum free T4, free T3 and thyroid-stimulating hormone (TSH) in the patients with nephrosis were within the normal range. However, the mean serum free T4 and free T3 concentrations were significantly (P<0.05) lower in the untreated patients than in the same patients in remission, and the mean serum TSH concentrations were significantly (P<0.05) higher in the untreated patients than in the same patients in remission. There were massive urinary losses of T4, T3, TBG. free T4 and free T3 in the untreated nephrotic children compared with the same patients in remission and age-matched controls. The daily urinary protein excretion showed a positive correlation with the urinary T4, T3, free T4, free T3 and TBG excretion. Furthermore, the urinary protein excretion showed a negative correlation with the serum T4, T3, free T4, free T3 and TBG levels. There was a negative correlation between serum albumin and serum TSH. These findings provide evidence of mild hypothyroidism in children with untreated nephrotic syndrome, partly because of losses of T4, T3, free T4, free T3 and TBG into the urine.

Systemic effects of transdermal testosterone for the treatment of microphallus in children

Abstract Objectives : To elucidate the metabolic effects of topical testosterone for the treatment of microphallus in children.

Uncommon multisystemic involvement in a case of Henoch‐Schönlein purpura

Abstract A case of Henoch‐Schönlein purpura (HSP) characterized by several unusual complications is reported. A 10‐year‐old boy was hospitalized with acute abdomen and developed purpura on the lower extremities after 4 days of hospitalization. He had protein‐losing enteropathy, diagnosed by an elevated fecal α‐1‐antitrypsin clearance. The colicky abdominal pain and protein‐losing enteropathy subsided after methylprednisolone pulse therapy was administered. He had left hydronephrosis and gall‐bladder abnormalities detected by ultrasonography, and purpura nephritis. However, after improvement of these abnormalities, he showed steroid‐induced epidural lipomatosis, detected by magnetic resonance imaging, which resolved with steroid reduction. Ultrasonography and magnetic resonance imaging were useful for detecting these uncommon multisystemic involvements in HSP.

Chyluria due to retroperitoneal lymphangioma producing nephrotic syndrome

Nephrotic syndrome developed in a 13-year-old girl. Her mother had noticed that at 6 years of age, her daughter’s right foot was longer than the left foot; she also had been diagnosed with scoliosis and a swollen right hip. At 7 years, the mother detected swelling of the right inguinal region. A right inguinal tumor was resected, and lymphangioma was found when the girl was 8 years old. Many venous varicosities appeared on her right thigh. Proteinuria and hematuria were first found in a school urinary screening when the girl was 11 years old. A renal biopsy was performed with normal histologic results. Chyluria had been first detected at the age of 11 years, although the child had no previous history of filariasis or tuberculosis. An intravenous pyelogram showed inside displacement of the right lower ureter. Cystoscopy showed chylous efflux from the right ureteral orifice. Angiography showed that the common, internal and external iliac arteries of the right side were thicker than those of the left side. Nephrotic syndrome (urine protein >6 g/day) became evident at age 13 years. The cause of her chyluria might have been lymphangiomas detected on the right upper sides of her bladder by the first T2-weighted magnetic resonance imaging (MRI) done at 8 years of age (Figure, A and B). These lymphangiomas have increased to six times their original size, as indicated in T2-weighted MRI at age 16 years (Figure, C and D).

Relationship between fluvoxamine and stress barometer for nocturnal enuresis

Abstract Background : It has been reported recently that fluvoxamine (a selective serotonin reuptake inhibitor) is effective and safe for children with monosymptomatic nocturnal enuresis (MNE). However, the exact mechanism by which fluvoxamine is beneficial in the treatment of MNE remains unknown. One possibility is that it controls emotional stress.

Chronic respiratory failure after acquired cytomegalovirus infection in a very low birthweight infant.

The case of a female infant who developed chronic respiratory failure after an acquired cytomegalovirus (CMV) infection is presented here. She was a very low birthweight (VLBW) infant and was free from oxygen supplement until 2 months after birth. Interstitial pneumonia occurred at 2 months of age, and her respiratory condition gradually deteriorated. A chest roentgenogram at 4 months revealed hyperinflation and reticular shadow, similar to that of severe chronic lung disease (CLD) in preterm infants. She was mechanically ventilated because of progressive respiratory deterioration, and oxygen dependency continued for 5 months after extubation. There are several previous reports of CMV pneumonia in term neonates or infants. However, there appears to be no published report on the pulmonary sequelae of CMV pneumonia in VLBW infants. The present case seems to indicate that acquired CMV pneumonia in VLBW infants causes chronic respiratory failure even when mechanical ventilation is not administered, and this respiratory failure is very similar to CLD in clinical symptoms and chest roentgenogram.

Effectiveness of high trough levels of cyclosporine for 5 months in a case of steroid-dependent nephrotic syndrome with severe steroid toxicity.

SUMMARY:  Glucocorticoid treatment for steroid‐dependent nephrotic syndrome (NS) is associated with severe adverse effects, such as bone fractures and epidural lipomatosis. Furthermore, a high trough level of cyclosporine (CsA) over an extended period of time is known to induce CsA nephropathy. We present a girl with steroid‐dependent NS and steroid‐induced vertebral compression fractures and epidural lipomatosis who was treated with a high‐dose of prednisolone after experiencing several relapses. A high CsA trough level (between 147 and 225 ng/mL) over a period of only 5 months was effective in improving the vertebral compression fractures, alleviating the epidural lipomatosis by enabling the discontinuation of prednisolone treatment. Thus, high trough levels of CsA over a short period of time may enable prednisolone to be discontinued in cases of steroid‐dependent NS without causing any clinical, histological, serum and/or urinary CsA‐related adverse effects.

Skeletal effects of short-term prednisolone therapy in children with steroid-responsive nephrotic syndrome

AbstractBackground. Osteoporosis or bone fracture can be induced in nephrotic children treated long-term with high doses of glucocorticoids. The purpose of this study was to determine whether short-term prednisolone therapy affects the skeleton in children with steroid-responsive nephrotic syndrome (NS). Methods. Bone mineral density (BMD) and biochemical parameters of mineral and skeletal homeostasis in nine children (four girls, five boys) aged between 2 and 7 years at the first episode of NS were measured. Prednisolone was started at 60 mg/m2 for 4 weeks, then decreased every 2 weeks for 12 weeks. All patients were steroid-responsive and had no relapse. All patients were examined at 0, 4, and 12 weeks of prednisolone therapy, and 16 weeks after the cessation of prednisolone therapy. Results. The Z-scores (BMD) before prednisolone therapy (−0.79 ± 0.51) were slightly low. The Z-scores at 4 weeks (−1.37 ± 0.46) and at 12 weeks of therapy (−1.22 ± 0.36) were significantly lower than those at 16 weeks after the cessation of prednisolone therapy (−0.29 ± 0.29). There was also a significant decrease in the mean serum levels of alkaline phosphatase, osteocalcin, and urinary deoxypyridinoline during the short-term prednisolone therapy. However, BMD and biochemical parameters of mineral and skeletal homeostasis returned to normal values at 16 weeks after the cessation of prednisolone therapy. Conclusions. Skeletal effects of short-term prednisolone therapy for 16 weeks were transient in children with steroid-responsive NS without relapse.

Glomerulonephritis with predominant paramesangial IgG deposition.

The new clinicopathological entity, Immunoglobin G (IgG)‐associated mesangial prollferative glomerulonephritls (GN), has been reported recently, but serial renal biopsies were not performed In the cases reported. The findings of three serial renal biopsies In a pediatrlc case with IgG‐associated GN and paramesangial deposits are reported. Microscopic hematuria was found Incidentally at the age of 8 years and the hematuria often worsened transiently during periods of upper respiratory infections. The patient was treated mainly with dipyrldamole. The third biopsy showed that both para‐mesangial hemispherical deposits and predominant mesan‐gial IgG deposits had increased, while mesangial cell proliferation had markedly decreased. These serial biopsy findings suggest that IgG‐associated GN with microscopic hematuria and slight proteinuria may be characterized by a relatively benign histoiogical and clinical course, as described in recent reports.

No evidence for pathogenic role of IgE in Henoch-Schoenlein purpura nephritis

AbstractBackground. The incidence of increased plasma IgE levels was reported to be significantly higher in Henoch-Schoenlein purpura nephritis (HSPN) than in IgA nephropathy (IgAN), and IgE deposits were demonstrated on epidermal Langerhans cells and dermal mast cells in four of six patients with HSPN in two European studies. We designed this study to investigate whether levels of clinical and biological markers of atopy in children with HSPN were significantly higher than those in children with IgAN, non-IgA glomerulonephritis (non-IgAGN), or microhematuria. Methods. The incidence of atopic disease, increased IgE levels, and positive radioallergosorbent test (RAST) results was investigated in 28 children with HSPN, 26 with IgAN, 28 with non-IgAGN, and 30 with microhematuria, all aged 8–16 years. All patients except for those in the microhematuria group, had proteinuria greater than100 mg/dl and had had a kidney biopsy. Results. The incidence of atopic disease, increased IgE levels, and positive RAST results in children with HSPN did not differ from findings in children with IgAN, non-IgAGN, or microhematuria. Conclusion. Our results in Japanese children do not support the idea (suggested by the two European studies) that IgE may play an important role in the pathogenesis of HSPN.