Parvovirus B19-induced acute bilateral carpal tunnel syndrome in twin girls.

We describe 2 cases of 6-year-old twin girls presenting with acute carpal tunnel syndrome (CTS) associated with human parvovirus B19 (HPV-B19) infection, as evidenced by serological data and detection of HPV-B19 DNA in blood with use of polymerase chain reaction (PCR). To our knowledge, this is the first time that HPV-B19 infection has been suggested as the causal agent of simultaneous acute bilateral CTS in twins, thus presenting the possibility that similar immunologic responses can be observed in twins during viral infections.

Clinical importance of mean platelet volume in children with nephrotic syndrome.

AIM: The importance of changes about platelet emphasized in most chronically diseases in recent years. Mean platelet volume (MPV) and platelet count can be used as a prognostic biomarker. In this study, clinical importance of the changes of MPV during active and remission phases in children with nephrotic syndrome was investigated. PATIENTS AND METHODS: Fifty-five children with nephrotic syndrome (30 females, 25 males) and 29 healthy children (18 females, 11 males) were included to the study. Patients were divided in two groups (steroid sensitive nephrotic syndrome and focal segmental glomerulosclerosis). Demographic characteristics of the patients, type of nephrotic syndrome were recorded and laboratory parameters in active and remission phases were evaluated. RESULTS: Mean platelet count in the patient group was significantly higher than control group. Mean platelet count of FSGS group was significantly higher than SSNS group. Mean MPV value was significantly lower in active period of nephrotic syndrome when compared with control group. A significant negative relation between mean MPV value and mean platelet count was found. Significant positive correlations between mean platelet count and mean total cholesterol and mean triglyceride levels were demonstrated. CONCLUSION: MPV in nephrotic syndrome patients can be an easy, cheap and simple method for determine the prognosis of the disease and steroid resistance.

Acidosis and hyperkalemia caused by losartan and enalapril in pediatric kidney transplant recipients.

OBJECTIVES: To evaluate the efficacy and safety of losartan and enalapril in pediatric kidney transplant recipients. MATERIALS AND METHODS: A retrospective review was performed in 31 pediatric kidney transplant recipients who were treated with losartan (50 mg/d, oral) for 1 to 6 months because of mild hypertension and persistent proteinuria. All patients were treated concurrently with enalapril (5 or 10 mg daily, oral), and 12 patients (39%) also were treated with amlodipine (5 or 10 mg daily, oral). Demographic and clinical characteristics of the patients were reviewed. RESULTS: Losartan use was associated with a significant decrease in mean systolic (before losartan was started, 123 ± 14 mm Hg; before losartan was stopped, 111 ± 10 mm Hg; P ≤ .001) and diastolic blood pressure (before losartan was started, 78 ± 11 mm Hg; before losartan was stopped, 69 ± 10 mm Hg; P ≤ .001) and urinary protein excretion (before losartan was started, 51 ± 45 mg/m2/h; before losartan was stopped, 28 ± 34 mg/m2/h; P ≤ .001). However, losartan therapy was associated with a significant mean increase in serum potassium level (before losartan was started, 4.0 ± 0.4 mmol/L; before losartan was stopped, 5.7 ± 0.5 mmol/L; P ≤ .001) and decrease in pH (before losartan was started, 7.35 ± 0.0; before losartan was stopped, 7.23 ± 0.0; P ≤ .001). Losartan was stopped because of hyperkalemia and acidosis earlier in patients who were on tacrolimus than cyclosporine immunosuppression (tacrolimus, 3 ± 1 mo; cyclosporine, 4.7 ± 0.8 mo; P ≤ .001). CONCLUSIONS: Losartan and enalapril may be beneficial in pediatric kidney transplant recipients by decreasing blood pressure and proteinuria, with maintenance of stable graft function, but may be associated with serious adverse events including hyperkalemia and life-threatening acidosis.

Mean platelet volume as a potential predictor of proteinuria and amyloidosis in familial Mediterranean fever.

This study aims to compare the mean platelet volume (MPV) levels in children and adults diagnosed with familial Mediterranean fever (FMF) during attack-free periods in order to find out whether it reflects the emergence of microalbuminuria/proteinuria and the development of amyloidosis or not. The study consisted of 63 pediatric patients (group 1), 50 adult patients (group 2), 50 healthy children (group 3), and 43 healthy adults (group 4). Demographic data, age at diagnosis, duration of the disease and colchicine treatment, and FMF gene mutations were recorded, and erythrocyte sedimentation rate, C-reactive protein, fibrinogen, hemoglobin, white blood cell count, platelet count, MPV, blood urea nitrogen, creatine, albumin, and urine microalbumin and protein levels were evaluated. According to the presence of microalbuminuria/proteinuria, patient groups were subgrouped into two by themselves as pediatric and adult groups with and without proteinuria. The most frequent mutation was M694V. MPV was significantly higher in FMF patients than those in the healthy control groups. Microalbuminuria/proteinuria were detected in 18 (28.57 %) of 63 pediatric patients and 26 (52 %) of 50 adult patients. Amyloidosis has been identified in 3 (16.6 %) of 18 pediatric patients and 18 (69.23 %) of 26 adult patients with proteinuria. Subgroup comparisons revealed that MPV levels were significantly higher in patients with proteinuria than patients without proteinuria in both pediatric and adult groups. Moreover, MPV levels were also significantly higher in adult patients with or without proteinuria than in pediatric patients with or without proteinuria. There were significant differences in terms of serum albumin levels between the groups with and without proteinuria as expected. The increase in MPV over the years of the disease, especially in groups with proteinuria, may be an important predictor of continuing increase of subclinical inflammation, the emergence of the microalbuminuria/proteinuria, and the developing of amyloidosis, but further studies are needed in order to support this proposal.

An unusual presentation of herpes simplex virus type 1 infection in a child.

We describe an 11-year-old girl presenting with lichen simplex chronicus (LSC) and acute bilateral carpal tunnel syndrome (CTS) following herpes simplex virus type 1 (HSV-1) infection as evidenced by serological data and by detection of HSV-1 DNA in the blood with the use of PCR. Based on the literature search, this case represents the first childhood case of LSC and acute bilateral CTS following HSV-1 infection. The experience with this patient emphasizes the importance of serological tests and PCR as well as the other laboratory techniques for the accurate diagnosis and management of the disease.

Mean platelet volume as a potential predictor of renovascular thrombosis after renal transplant.

OBJECTIVES: We sought to evaluate the importance of mean platelet volume as a marker to follow-up, the tendency for hemorrhagic diatheses, and/or thrombotic complications in patients before and after renal transplant. MATERIALS AND METHODS: Thirty-four patients (aged, 5 to 18 y) were included. Demographics of the patients, cause of chronic renal failure, dialysis modality, duration of dialysis, arterio-venous fistula thrombosis, and posttransplant immunosuppressive regimens were recorded and laboratory variables were evaluated. RESULTS: At the end of the first posttransplant month, mean platelet volume level was decreased significantly when compared with pretransplant levels (8.3 ± 1.5 vs 7.7 ± 0.9; P = .04). A significant increase was observed in platelet levels during posttransplant measures (273.750 ± 97.700 vs 318.740 ± 84.586; P = .02). Prothrombin time and partial thromboplastin time levels did not differ before and after transplant. None of the patients had any thrombotic events and/or renal allograft loss. A negative correlation was observed between mean platelet volume and C-reactive protein (r=-0.53). Mean platelet volume level was not found to be related to the cause of renal failure, pretransplant dialysis modality, or posttransplant immunosuppressive regimens. CONCLUSIONS: Platelet numbers increased and mean platelet volume decreased after pediatric renal transplant, but the potential for increased thrombosis was not observed.

Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia.

A 12-year-old girl presented with a sudden decrease in her right visual acuity and homonymous hemianopsia. An angiography of the retinal arteries demonstrated recanalized occlusion of the right retinal artery. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation. Laboratory evaluations revealed dual antineutrophil cytoplasmic antibodies (ANCA) positivity [anti-proteinase (anti-PR3) ANCA and anti-myeloperoxidase (anti-MPO) ANCA], anticardiolipin (aCL) antibodies, and low titers of antinuclear antibodies (ANA). There was no evidence of active systemic lupus erythematosus (SLE), ANCA-related vasculitis, or other risk factors for cerebral occlusion, such as antiphospholipid syndrome (APS). Dual positivity for both cytoplasmic (c-ANCA) and perinuclear (p-ANCA) antineutrophil antibodies has been found previously in a small number of reports, but to our knowledge, this case represents the first case of moyamoya disease associated with dual ANCA positivity.

Type IV neonatal Bartter syndrome complicated with congenital chloride diarrhea.

BACKGROUND: Pseudo-Bartter syndrome encompasses a heterogenous group of disorders similar to Bartter syndrome. Sometimes a few status may be nested, as in our case presented here. CASE REPORT: An 8-month-old boy was referred to our hospital with of intractable diarrhea, polyuria, persistent hypokalemia, abdominal distension and failure to thrive. He was born in the 34 6/7 gestational week (GW) to consanguineous parents. In the 30(th) GW polyhydramnios was verified by ultrasonography. The laboratory results showed hypokalemic-hypochloremic metabolic alkalosis, hyponatremia, and increased urinary loss of chloride, potassium and calcium. An audiogram test revealed complete sensorineural deafness. Ultrasonography revealed medullary nephrocalcinosis in both kidneys. Elevated plasma renin activity and aldosterone were found and a provisional diagnosis of type-IV neonatal Bartter syndrome was made. Treatment with indomethacin, spironolactone and additional intake of NaCl/KCl was initiated. Despite these therapies, the child's diarrhea persisted but serum potassium concentration normalized, and hypercalciuria and urine output reduced. After determining the high fecal chloride concentration, there was an immediate decompensation of the disease on indomethacin withdrawal, thus a diagnosis of type IV neonatal Bartter syndrome complicated with congenital chloride diarrhea was considered. Indomethacin, spironolactone and supplementary therapies with NaCl/KCl were continued, which resulted in the normalization of serum electrolytes as well as his physical development, but high contents of chloride in urine and faeces and nephrocalcinosis remains unchanged during 1-year follow-up. CONCLUSIONS: Because of the clinical and laboratory simulations between the various diseases that lead to hypokalemic-hypochloremic metabolic alkalosis, patients must be evaluated carefully.

Parvovirus B19-induced multisystemic vasculitis and acute endocapillary proliferative glomerulonephritis in a child.

A 10-year-old girl was admitted with fever, cough, maculopapular rash, hemoptysis, dark-colored urine, edema, multiple lymphadenopathies, and hepatosplenomegaly. She developed acute glomerulonephritis during the course of these complex clinical features. Laboratory data showed hematuria, proteinuria, and hypocomplementemia. Serological tests showed positive human parvovirus B19 (HPVB19)-specific immunoglobin M (IgM) and HPVB19 DNA was detected in the patient's serum using polymerase chain reaction (PCR). Renal biopsy revealed acute endocapillary proliferative glomerulonephritis (AEPGN) with coarse granular C3 depositions in a "starry sky pattern," which is more peculiar to poststreptococcal glomerulonephritis. Electron microscopy showed subendothelial and small hump-shaped subepithelial electron-dense deposits in glomerular capillary walls. There was no evidence of either any mycobacterial, staphylococcal, or streptococcal infection, and the diagnosis of Goodpasture syndrome and connective tissue disorders was excluded during clinical and laboratory investigations. A diagnosis of HPVB19-induced pleuropneumonitis and glomerulonephritis was made. Through a literature search there was no documented pediatric case of AEPGN induced by HPVB19, and this case represents, to our knowledge, the first time that a direct relationship between parvovirus infection and AEPGN has been demonstrated in a child.

Rituximab for post-transplant recurrences of FSGS.

A 14-yr-old boy whose primary kidney disease was FSGS developed severe recurrence of proteinuria immediately after a second living-related kidney transplant. Despite pre- and post-operative PP and immunosuppressive treatment consisting of steroids, CycA, daclizumab, and MMF, daily protein excretion and serum creatinine increased. We therefore administered rituximab on the fourth day of transplantation. He received four weekly doses of rituximab (375 mg/m(2)/dose), which resulted in a rapid clearing of circulating CD19-positive B cells, and remission of proteinuria was achieved six wk after the first rituximab treatment. Graft function was excellent six months after transplantation with proteinuria of 8 mg/m(2)/h. We conclude that rituximab may be an effective treatment for post-transplant recurrence of FSGS.

Long term follow-up of glomerular and tubular functions in liver transplanted patients with Wilson's disease.

The aim of this study was to determine the long term outcome of renal glomerular and tubular functions in children receiving an LT for WD. Renal functions were examined in nine children with WD before and long after LT and compared with those of nine liver transplanted children with hepatic diseases other than WD. The duration of follow-up was at least two yr for both groups. GFR, fractional TRP and tubular maximum rate of phosphate reabsorption in relation to GFR (TP/GFR) as well as daily protein and Ca excretion were studied in both groups before and after LT. Pretransplant mean GFR, TRP and TP/GFR were significantly lower in the study group than the controls. A significant increase in the post-transplant TRP and TP/GFR was observed in the study group and the difference between the groups disappeared during the long term follow-up. Urinary protein excretion decreased in both groups after LT. Tubular dysfunction is frequent in patients with WD. LT for hepatic failure secondary to WD is a lifesaving procedure correcting the underlying hepatic defect as well as renal defects.

Fatal cytophagic histiocytic panniculitis.

Individual cases of so-called Weber-Christian disease with a bleeding diathesis have been reported for several years. These were originally diagnosed as Weber-Christian disease, but have been recategorized on review as a chronic, visceral, and cutaneous histiocytic (cytophagic) panniculitis, progressing to liver dysfunction and jaundice and a terminal hemorrhagic diathesis. We report here a rare catastrophic form of systemic panniculitis in an adolescent girl. Despite compelling clinical evidence, the diagnosis was made only on postmortem biopsies.