Shinobu Waga

Spontaneous remission of persistent severe hematuria in an adolescent with nutcracker syndrome: seven years’ observation

A Japanese boy aged 14 years presented with gross hematuria associated with mild proteinuria and was diagnosed as having nutcracker syndrome. Magnetic resonance angiography (MRA) revealed significant compression of the left renal vein between the aorta and the superior mesenteric artery with collaterals. A percutaneous renal biopsy on the right kidney revealed no evidence of glomerular or interstitial changes with immune deposition. He was observed closely without any intervention thereafter. Although repeat MRA performed 4 years after our first observation disclosed the development of collateral veins, severe hematuria with an intermittent exacerbation remained unchanged. During the next 2 years, the hematuria completely subsided spontaneously. Although the etiology of spontaneous remission of the disease remains speculative, his good physical development (i.e., approximately 10 cm taller than his height at the onset) may change presumptive hemodynamic factors. These clinical observations suggest that a proportion of pubertal patients with nutcracker syndrome should be treated conservatively for a relatively long time.

Combined therapy of enalapril and losartan attenuates histologic progression in immunoglobulin A nephropathy

Abstract Background : It has been reported that combined therapy of angiotensin converting enzyme inhibitor and angiotensin receptor blocker significantly decreases proteinuria in immunoglobulin A (IgA) nephropathy. However, histologic alterations following the therapy have not been reported.

Repeat renal biopsy in a girl with tubulointerstitial nephritis and uveitis syndrome.

Abstract. A Japanese girl aged 8 years who presented with a 2-month history of uveitis subsequently developed tubulointerstitial nephritis. A percutaneous renal biopsy revealed massive interstitial mononuclear cell infiltrates consisting of CD4-positive T cells. Despite administration of topical corticosteroids, the ocular symptoms persisted. Systemic corticosteroid therapy dramatically reduced the ocular symptoms and urinary β2-microglobulin (β 2MG) concentration. However, reducing the prednisolone dosage induced recurrence of uveitis associated with increased levels of urinary β 2MG. The CD4-positive T cell infiltration persisted in the second renal biopsy performed 6 months after the first renal biopsy. These observations suggest that the interstitial cell infiltration persists for a relatively long time in a proportion of patients with tubulointerstitial nephritis and uveitis syndrome (TINU). Although the renal outcome of TINU has been reported to be favorable, prolonged interstitial cell infiltration may affect long-term renal outcome. Selected patients with TINU should be followed with close observation.

Interstitial cystitis and ileus in pediatric-onset systemic lupus erythematosus.

Abstract A girl aged 11 years presented with autoimmune hemolytic anemia with thrombocytopenia, and subsequently developed severe abdominal pain, vomiting, and pollakiuria. X-ray findings of her abdomen demonstrated paralytic ileus with intestinal wall thickening. Intravenous pyelography revealed bilateral hydroureter with mild hydronephrosis and contracted bladder. Pathological examination of her bladder revealed interstitial cystitis, with evidence of focal deposition of IgG and C3 in a granular pattern on small blood vessel walls. She was diagnosed as having systemic lupus erythematosus (SLE) associated with paralytic ileus and chronic interstitial cystitis. Although initiation of high-dose prednisolone therapy resulted in a gradual improvement in clinical symptoms, reducing the dosage of prednisolone caused a relapse. To our knowledge, the combination of paralytic ileus and chronic interstitial cystitis is quite uncommon in pediatric-onset SLE.

Methylprednisolone pulse therapy in Japanese children with severe lupus nephritis

Abstract. Six Japanese children with severe lupus nephritis received prompt initiation of methylprednisolone pulse therapy (MPT). After three courses of MPT, oral prednisolone combined with a 12-week course of oral cyclophosphamide was given and prednisolone was tapered. At presentation, urine protein excretion and histological indices of the mean activity index and the mean chronicity index in the patients were 2.2±1.5 g/day, 10.3±2.0, and 2.8±1.2, respectively. Urine protein excretion and the activity index decreased significantly at the second renal biopsies obtained at a mean interval of 8 months after the first [0.1±0.1 g/day and 3.5±1.4 (P<0.05), respectively], while the chronicity index did not change. At the latest observation (mean interval 53 months), all showed clinical and serological improvement. Complete remission was achieved in two patients, and no patient showed renal impairment. Although this case series is without controls, our treatment protocol may be of benefit to Japanese children with severe lupus nephritis.

Low serum lipids suggest severe bone marrow failure in children with aplastic anemia

Abstract Background : Significantly low serum lipid levels are occasionally seen at the time of diagnosis in children with aplastic anemia (AA). The aim of the present study was to clarify the pathologic and clinical significance of pretreatment serum lipid levels in AA.

Acute tubulointerstitial nephritis associated with piperacillin therapy in a boy with glomerulonephritis

An 11‐year‐old boy with glomerulonephritis developed acute renal failure 4 days after beginning piperacillin (PIPC) treatment. Renal biopsy revealed acute tubulointerstitial nephritis (ATIN) with marked eosinophils. A lymphocyte stimulation test (LST) for PIPC demonstrated an extremely high LST index of 626%. The serum levels of immunoglobulin E and eosinophil cationic protein also showed a significant increase at 9021 IU/mL and greater than 150 μg/L, respectively. These observations suggest that a hypersensitivity reaction might play a role in the pathogenesis of ATIN. This is the first report to describe PIPC‐induced ATIN in a child.

Subclinical Sjögren's syndrome: A significant 67gallium accumulation in the orbits and parotid glands

An 8‐year‐old girl with hypergammaglobulinemia showed an abnormal 67gallium accumulation in the orbits and parotid glands. Although she did not have any subjective siccant complaints, reported typical histopatho‐logical and sialographs changes suggesting Sjögrens syndrome (SjS) were observed in the salivary glands. Gallium scintigram might be a valuable and non‐invasive diagnostic tool in the diagnosis of children with SjS without sicca symptoms.

Acute renal failure with encephalopathy following Salmonella enteritidis infection.

Salmonellosis is one of the major causes of food poisoning in Japan. In general, consumption of food contaminated with non-typhi Salmonella causes acute gastroenterocolitis. However, salmonellosis has also been reported to be associated with development of severe extraintestinal multiorgan complications, including rhabdomyolysis (RM), acute renal failure (ARF), and rarely acute encephalopathy [1–5]. In this paper, we report on a patient with systemic lupus erythematosus (SLE) who developed ARF with suspected acute encephalopathy following Salmonella enteritidis infection. The patient was under treatment with immunosuppressive agents. Since patients with SLE are at an increased risk of severe infections [6], this case report may serve to emphasize the importance of prevention of food poisoning and of advocating appropriate hygienic practice in immunocompromised hosts. A 16-year-old Japanese girl with a 3-year history of SLE with WHO class IV disease was referred to our hospital because of drowsiness and disorientation. Although the SLE activity and serum complement levels were reasonably controlled under maintenance therapy with prednisolone (15 mg/day) and azathioprine (75 mg/day), the patient developed acute severe diarrhea, vomiting, and fever after eating a raw egg. She was admitted to a regional hospital with suspected food poisoning. On admission, her body weight was 36.5 kg, which represented a loss of about 1.5 kg over her previous weight recording. Her blood pressure was decreased to 70/36 mmHg. Laboratory studies revealed the following: peripheral white blood cell (WBC) count 11,200/μl, hemoglobin 13.8 g/dl, hematocrit 39.9%, platelet count 193,000/μl, blood urea nitrogen (BUN) 18.2 mg/dl, creatinine 0.9 mg/dl, and Creactive protein (CRP) 5.4 mg/dl. Despite adequate fluid replacement, the patient developed oliguria, drowsiness, and disorientation. Lumbar puncture revealed clear cerebrospinal fluid (CSF), and further examination of the CSF revealed the following: cell count 1 cell/mm, protein 22 mg/dl, glucose 71 mg/dl, and sterile culture. Emergency cranial computed tomographic and electroencephalographic findings were unremarkable. The BUN and serum creatinine increased to 41.5 mg/dl and 4.7 mg/dl, respectively. In addition, the serum level of creatinine kinase (CK) and urinary level of myoglobin also became markedly elevated to 21,307 IU/l (normal: 12–144 IU/l) and 1,073 ng/ml (normal: 0–70 ng/ml), respectively. A diagnosis of ARF caused by RM associated with some central nervous system (CNS) complication was made, and the patient was transferred to our hospital. Physical examination on admission to our hospital revealed that the patient was drowsy. Her blood pressure was 126/76 mmHg. Laboratory studies revealed the following: WBC count 10,590/μl, hemoglobin 13.9 g/100 ml, hematocrit 38.0%, platelet count 119,000/μl, serum total protein 6.6 g/dl, albumin 3.9 g/dl, BUN 63 mg/dl, creatinine 6.8 mg/dl, sodium 137 mmol/l, potassium 4.3 mmol/l, chloride 97 mmol/l, calcium 6.8 mg/dl, CRP 17.8 mg/dl, and CK 29,484 IU/l. Immunological studies revealed the following: IgG 502 mg/dl, IgA 185 mg/dl, IgM 43 mg/dl, C3 76 mg/dl (normal: 79–152 mg/dl), C4 20 mg/dl (normal: 16–38 mg/dl), and complement hemolytic activity 37.4 U/ml (normal: 23–46 U/ml). The serological test for antinuclear antibody was positive at a titer of 1:320, with a homogeneous pattern. Although the blood culture was negative, the stool culture was positive for Salmonella enteritidis. A search for endotoxin in the blood was negative. The levels of the proinflammatory cytokines, interleukin (IL)-6 and IL-8, were analyzed in the CSF specimen obtained at the onset of the salmonellosis. The CSF levels of both cytokines were significantly elevated: IL-6 328 pg/ml (measured by enzyme immunosorbent assay, normal: less than 4.0 pg/ml) and IL-8 327 pg/ml (measured by enzyme-linked immunosorbent assay, normal: less than 2.0 pg/ml). On the T. Nakahata . K. Tsugawa . H. Tanaka (*) Department of Pediatrics, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki, 036-8562, Japan e-mail: hirotana@cc.hirosaki-u.ac.jp

Monocyte Function in Idiopathic Nephrotic Syndrome in Childhood

The monocyte function of 112 specimens from 42 children with idiopathic nephrotic syndrome (INS) aged from 2 to 17 years was studied by the methods of nitroblue tetrazolium (NBT) reduction, phagocytosis of immunobeads (IB) and yeast cells, chemotaxis and acid alpha-naphthyl acetate esterase (ANAE) staining. The dissociation between phagocytosis and chemotaxis was observed in the fresh cases of steroid sensitive INS and in the cases of steroid non-sensitive INS. In the fresh cases of steroid sensitive INS, NBT reduction and phagocytosis were increased, but chemotaxis was decreased. In the cases of steroid non-sensitive INS, the phagocytosis of IB was decreased, but chemotaxis was increased. These findings suggest a different pathogenesis between steroid sensitive and steroid non-sensitive INS. The dissociation between phagocytosis and chemotaxis may be explained by the alteration of the surface receptors of monocyte and by lymphokines.