Impact of Everolimus and Low-Dose Cyclosporin on Cytomegalovirus Replication and Disease in Pediatric Renal Transplantation.

In order to investigate the hypothesis that the mammalian target of rapamycin inhibitor everolimus (EVR) shows anticytomegalovirus (CMV) activity in pediatric patients, we analyzed the impact of EVR-based immunosuppressive therapy on CMV replication and disease in a large cohort (n = 301) of pediatric kidney allograft recipients. The EVR cohort (n = 59), who also received low-dose cyclosporin, was compared with a control cohort (n = 242), who was administered standard-dose cyclosporin or tacrolimus and an antimetabolite, mostly mycophenolate mofetil (91.7%). Multivariate analysis revealed an 83% lower risk of CMV replication in the EVR cohort than in the control cohort (p = 0.005). In CMV high-risk (donor+/recipient-) patients (n = 88), the EVR-based regimen was associated with a significantly lower rate of CMV disease (0% vs. 14.3%, p = 0.046) than the standard regimen. In patients who had received chemoprophylaxis with (val-)ganciclovir (n = 63), the CMV-free survival rates at 1 year and 3 years posttransplant (100%) were significantly (p = 0.015) higher in the EVR cohort (n = 15) than in the control cohort (n = 48; 1 year, 75.0%; 3 years, 63.3%). Our data suggest that in pediatric patients at high risk of CMV, an EVR-based immunosuppressive regimen is associated with a lower risk of CMV disease than a standard-dose calcineurin inhibitor-based regimen.

Three cases of spondyloenchondrodysplasia (SPENCD) with systemic lupus erythematosus: a case series and review of the literature.

Spondyloenchondrodysplasia (SPENCD) is a rare autosomal recessive skeletal dysplasia caused by recessive mutations in the ACP5 gene, and it is characterized by the persistence of chondroid tissue islands within the bone. The clinical spectrum of SPENCD includes neurological involvement and immune dysfunction, such as systemic lupus erythematosus (SLE). To date, there are only 12 reported cases of SPENCD associated with SLE in the literature; however, detailed clinical follow-up data is absent for this comorbidity. This report presents clinical and laboratory data of three patients diagnosed with SPENCD-associated SLE. All three patients had short stature, arthralgia/arthritis, lupus nephritis, hypocomplementemia, and positive autoantibodies, including anti-nuclear and anti-dsDNA antibodies. Two patients exhibited class IV and one patient exhibited class V lupus nephritis. The early recognition of SPENCD is imperative, and this condition should be considered in patients with SLE, particularly in individuals with short stature and skeletal abnormalities. The cases presented here demonstrate that timely diagnosis and follow-up are key factors for the successful management of these conditions.

Lupus in a patient with cystinosis: is it drug induced?

A 9-year-old girl with a diagnosis of cystinosis since 2 years of age, on cysteamine therapy, presented with complaints of serositis and arthritis, and laboratory tests revealed high antinuclear antibody titers with hypocomplementemia. Kidney biopsy was not consistent with lupus nephritis. With prednisolone treatment her complaints resolved and creatinine level decreased, but on follow-up, serological features of systemic lupus erythematosus (SLE) continued. Six years after cessation of prednisolone, lupus features were reactivated, with positive antihistone antibodies and ANCA. Coincidence of cystinosis and SLE is very rare, and to the best of our knowledge this is the fourth case reported in the literature. Physicians should be aware that cystinosis patients may have some autoimmune manifestations with features of true or drug-induced lupus. In the light of this case, pathophysiology and treatment are discussed.

Disparities in policies, practices and rates of pediatric kidney transplantation in Europe.

We aimed to provide an overview of kidney allocation policies related to children and pediatric kidney transplantation (KTx) practices and rates in Europe, and to study factors associated with KTx rates. A survey was distributed among renal registry representatives in 38 European countries. Additional data were obtained from the ESPN/ERA-EDTA and ERA-EDTA registries. Thirty-two countries (84%) responded. The median incidence rate of pediatric KTx was 5.7 (range 0-13.5) per million children (pmc). A median proportion of 17% (interquartile range 2-29) of KTx was performed preemptively, while the median proportion of living donor KTx was 43% (interquartile range 10-52). The median percentage of children on renal replacement therapy (RRT) with a functioning graft was 62%. The level of pediatric prioritization was associated with a decreased waiting time for deceased donor KTx, an increased pediatric KTx rate, and a lower proportion of living donor KTx. The rates of pediatric KTx, distribution of donor source and time on waiting list vary considerably between European countries. The lack of harmonization in kidney allocation to children raises medical and ethical issues. Harmonization of pediatric allocation policies should be prioritized.

Mucolipidosis type III in an adolescent presenting with atypical facial features and skeletal deformities.

Mucolipidosis type III (MLIII) (MIM# 252600) is an uncommon autosomal recessive disorder that results from deficiency of the multimeric enzyme, UDP-N-acetylglucosamine-1-phosphotransferase. The enzymatic defect results in deficiencies of lysosomal degradative enzymes with concomitant intracellular accumulation of both partly degraded glycosaminoglycans and sphingolipids leading to clinical manifestations such as short stature, developmental delay and other structural abnormalities. The diagnosis is challenging since musculoskeletal presentation may mimic some of the rheumatic and metabolic disorders. We herein report on a 13-year-old adolescent who was admitted to our rheumatology clinic because of progressive joint stiffness and deformities of her hands. The clinical and radiological findings led us to the diagnosis of MLIII despite negative urinary aminoglycosyaminoglycans. Therefore we decided to check for the presence of elevated activities of alpha-mannosidase and beta-hexosaminidase A+B in the plasma which was actually the case and confirmed the clinical diagnosis ofMLIII.

MEFV mutations in systemic onset juvenile idiopathic arthritis.

OBJECTIVES: Autoinflammatory diseases constitute a large spectrum of monogenic diseases like FMF or cryopyrin-associated periodic syndromes (CAPS) and complex genetic trait diseases such as systemic onset juvenile idiopathic arthritis (SoJIA). An increased rate of MEFV mutations has been shown among patients with PAN and HSP, in populations where FMF is frequent. The aim of the study is to search for MEFV mutations in our patients with SoJIA and see whether these mutations had an effect on disease course or complications. METHODS: Thirty-five children with the diagnosis of SoJIA were screened for 12 MEFV mutations. The control data were obtained from a previous study of our centre determining the carrier frequency in Turkish population. RESULTS: Two patients were homozygous and three patients were heterozygous for the M694V mutation. One patient was a compound heterozygote for the M680I/V726A mutations. Heterozygous V726A mutation was found in one patient. The overall mutation frequency of patients was 14.28%. This figure had been compared with the previously published rate of disease-causing mutations in this country, which is 5%. Disease-causing mutations were found to be significantly more frequent in the SoJIA patients than the population (P < 0.01). Among these, M694V was the leading mutation with a frequency of 10% in SoJIA. Six patients carrying MEFV mutations were among the most resistant cases requiring biological therapy. CONCLUSION: SoJIA patients had a significantly higher frequency of MEFV mutations but clinical studies with large number of patients are needed to confirm the association of MEFV mutations with SoJIA and its course.

Psychiatric morbidity and different treatment modalities in children with chronic kidney disease.

INTRODUCTION: Chronic kidney disease (CKD) is a potentially life-threatening condition leading to various psychosocial problems associated with different treatment modalities in addition to their medical advantages and disadvantages. The aim of this study was to evaluate the psychiatric morbidity in children with CKD in terms of different treatment modalities in comparison to healthy peers. In addition, parental attitudes and psychiatric symptoms in this group of mothers were examined. POPULATION AND METHODS: A matched cohort study including 66 children with CKD (21 renal transplantation, 27 dialysis, 18 conservative treatment) and 37 healthy age- and sex-matched controls were evaluated. Children filled out the Children's Depression Inventory, the State-Trait Anxiety Inventory, and the Parental Attitude Scale, and the mothers filled out the Symptom Checklist-90-R. The Schedule for Affective Disorders and Schizophrenia for School-Age Children, Present and Lifetime Version was used for psychiatric diagnosis. RESULTS: The overall depression scores in children and the mothers' overall symptom severity index were significantly higher in the CKD group: 40.9% of children in the CKD group were diagnosed with a psychiatric disorder, while the corresponding figure for the control group was 16.2%. The in-group comparison of the CKD group failed to detect any significant difference between the three treatment modalities. CONCLUSION: The results support the findings of research showing that CKD has high psychiatric morbidity. It is important to include psychosocial and psychiatric assessments in the evaluation processes of different treatment modalities in CKD.

Renal transplantation in children with lower urinary tract dysfunction of different origin: a single-center experience.

INTRODUCTION: Renal transplantation in patients with lower urinary tract dysfunction (LUTD) of various origins is a challenging issue in the field of pediatric transplantation. We report our single-center experience to evaluate patient and graft survivals as well as the risks of the surgery and immunosuppressive therapy. PATIENTS AND METHODS: Among 70 pediatric transplant patients, 11 displayed severe LUTD. Videourodynamic tests were performed on all patients preoperatively as well as postoperatively if required. The cause of urologic disorders were neurogenic bladder (n = 5) and urethral valves (n = 6). Clean intermittent catheterization (CIC) was needed in six patients to empty the bladder. To achieve a low-pressure reservoir with adequate capacity pretransplantation augmentation ileocystoplasty was created in four patients and gastrocystoplasty in one patient. Three of the patients received kidneys from cadaveric and eight from living donors. All patients were treated with calcineurin-based immunosuppressive therapy. RESULTS: The mean age at transplantation was 15 +/- 4.7 years. The median follow-up after transplantation was 36 months (6 to 62 months). At their last visit the median creatinine level was 0.95 mg/dL (0.8 to 2.4 mg/dL). Three patients had recurrent symptomatic urinary tract infections who had augmented bladder on CIC. One patient with ileocystoplasty who developed urinary leak and ureteral stricture in the early postoperative period was treated by an antegrade J stent. CONCLUSION: Severe LUTD carried high risks for the grafted kidney. However, our data suggested that renal transplantation is a safe and effective treatment modality, if the underlying urologic diseases properly managed during the transplantation course. Since surgery and follow-up is more complicated, patient compliance and experience of transplantation team have significant impacts on outcomes.

Outcome of primary glomerular disease in pediatric renal transplantation: a single-center experience.

INTRODUCTION: The recurrence of primary disease in transplantation is a well-known problem. We report our single-center experience to assess the frequency of the recurrence of primary glomerulonephritis in children after renal transplantation. PATIENTS AND METHODS: Medical reports of 14 children with primary glomerular disease were evaluated. Among the 14 grafts were 10 from living related and four from cadaveric donors. Ten were diagnosed as focal segmental glomerulosclerosis (FSGS), two membranoproliferative glomerulonephritis (MPGN), and two polyarteritis nodosa (PAN). The original diagnosis was biopsy-proven in every case. All patients were treated with calcineurin-based immunosuppressive therapy. RESULTS: The mean age was 15.5 +/- 5.4 years. The median transplantation duration was 47 months; however, one of the FSGS patient had hyperacute rejection. Five years later she received a second graft with a serum creatinine of 0.7 mg/dL at 7 years after transplantation. Posttransplant recurrence of FSGS was confirmed in two patients (20%), who were treated with plasmapheresis with no improvement of proteinuria, two FSGS patients had thromboses after transplantation. One had a cardiac thrombosis with heterozygote MTHFR mutation and one, a renal artery thrombosis and loss of graft with prothrombin 20210A mutation. They all have functioning grafts except these two. We did not observe recurrence of PAN or MPGN in patients. CONCLUSION: Although the number of patients is quite small, our recurrence rate was compatible with the previous reports. Additionally, we strongly recommend evaluation of all risk factors for thrombosis and give appropriate anticoagulation.

Triple immunosuppression with tacrolimus in pediatric renal transplantation: single-center experience.

AIM: In this single-center cohort, we retrospectively analyzed the efficacy and safety of tacrolimus in pediatric renal transplantation. METHODS: We examined the medical records of 22 consecutive renal transplantation recipients (12 boys, 10 girls) receiving tacrolimus, to evaluate occurrence of acute rejection (AR) episodes, glomerular filtration rates (GFR), and side effects. RESULTS: The mean recipient age was 15.07 +/- 3.96 years. Seven grafts came from cadaveric, and 15 from living related donors. The patients were placed on immunosuppression with prednisolone and tacrolimus plus azathioprine (n = 8) or mycophenolate mofetil (MMF) (n = 12) or enteric-coated mycophenolate sodium (n = 2). Eighteen patients received basiliximab on days 0 and 4. There were three AR episodes at 5, 9, and 12 months. Mean GFR at the end of 1 and 2 years were 97.1 +/- 24.0 mL/min/1.73 m(2) and 116.9 +/- 42.2 mL/min/1.73 m(2), respectively. There was no graft loss. Hypertension, hyperlipidemia, and hyperglycemia were present in 14 (63.6%), 3 (13.6%), and 3 (13.6%) patients, respectively, without gingival hyperplasia, tremor, or hypertrichosis. Supraventricular tachycardia was noticed in five patients (22.7%), three of whom needed antiarrhythmic drugs (13.6%). CONCLUSION: Our single-center experience with tacrolimus, steroid plus azathioprine or MMF or enteric-coated mycophenolate sodium regimen in pediatric kidney recipients showed a low rate of AR with excellent graft survival and function at 1 and 2 year posttransplantation. The increased rate of supraventricular tachycardia in this regimen had not been previously reported; this association merits further studies.

Interferon-gamma assays for the diagnosis of tuberculosis infection before using tumour necrosis factor-alpha blockers.

OBJECTIVES: Patients who receive tumour necrosis factor-alpha (TNF-alpha) blockers are mostly immunosuppressed. A study was performed to investigate whether an interferon-gamma (IFN-gamma) assay could represent an alternative approach to the tuberculin skin test (TST) for the diagnosis of latent tuberculosis infection (LTBI) in these patients. DESIGN: We prospectively enrolled 106 individuals into the study in two groups. Group 1 consisted of 38 healthy individuals and Group 2 included 68 patients with chronic inflammatory diseases evaluated for LTBI before the use of TNF-alpha blockers. RESULTS: Of all participants, nine had indeterminate IFN-gamma test results. Agreement between the two tests was poor in both groups (kappa values respectively -0.54 and 0.18). In a total of 97 subjects, 10 (10.3%) were positive by the IFN-gamma test and 49 (50.5%) by TST. CONCLUSION: We found poor agreement between TST and the IFN-gamma test in our study. Our limited preliminary data should be accepted as a basis for designing future studies that will be helpful for physicians to decide whether the IFN-gamma test is more sensitive than the TST test in detecting LTBI before the use of TNF-alpha blockers.

A phenocopy of CAII deficiency: a novel genetic explanation for inherited infantile osteopetrosis with distal renal tubular acidosis.

The rare bone thickening disease osteopetrosis occurs in various forms, one of which is accompanied by renal tubular acidosis (RTA), and is known as Guibaud-Vainsel syndrome or marble brain disease. Clinical manifestations of this autosomal recessive syndrome comprise increased bone density, growth failure, intracerebral calcification, facial dysmorphism, mental retardation, and conductive hearing impairment. The most common cause is carbonic anhydrase II (CAII) deficiency. Several different loss of function mutations in CA2, the gene encoding CAII, have been described. To date, there have been no exceptions to the finding of CAII deficiency in patients with coexistent osteopetrosis and RTA. Most often, the RTA is of mixed proximal and distal type, but kindreds are reported in which either distal or proximal RTA predominates. We report the molecular genetic investigation of two consanguineous kindreds where osteopetrosis and distal RTA (dRTA) were both manifest. One kindred harbours a novel homozygous frameshift alteration in CA2. In the other, CAII levels were normal despite a similar clinical picture, and we excluded defects in CA2. In this kindred, two separate recessive disorders are penetrant, each affecting a different, tissue specific subunit of the vacuolar proton pump (H(+)-ATPase), providing a highly unusual, novel genetic explanation for the coexistence of osteopetrosis and dRTA. The osteopetrosis is the result of a homozygous deletion in TCIRG1, which encodes an osteoclast specific isoform of subunit a of the H(+)-ATPase, while the dRTA is associated with a homozygous mutation in ATP6V1B1, encoding the kidney specific B1 subunit of H(+)-ATPase. This kindred is exceptional firstly because the coinheritance of two rare recessive disorders has created a phenocopy of CAII deficiency, and secondly because these disorders affect two different subunits of the H(+)-ATPase that have opposite effects on bone density, but which have only recently been determined to possess tissue specific isoforms.

Novel ATP6V1B1 and ATP6V0A4 mutations in autosomal recessive distal renal tubular acidosis with new evidence for hearing loss.

Autosomal recessive distal renal tubular acidosis (rdRTA) is characterised by severe hyperchloraemic metabolic acidosis in childhood, hypokalaemia, decreased urinary calcium solubility, and impaired bone physiology and growth. Two types of rdRTA have been differentiated by the presence or absence of sensorineural hearing loss, but appear otherwise clinically similar. Recently, we identified mutations in genes encoding two different subunits of the renal alpha-intercalated cell's apical H(+)-ATPase that cause rdRTA. Defects in the B1 subunit gene ATP6V1B1, and the a4 subunit gene ATP6V0A4, cause rdRTA with deafness and with preserved hearing, respectively. We have investigated 26 new rdRTA kindreds, of which 23 are consanguineous. Linkage analysis of seven novel SNPs and five polymorphic markers in, and tightly linked to, ATP6V1B1 and ATP6V0A4 suggested that four families do not link to either locus, providing strong evidence for additional genetic heterogeneity. In ATP6V1B1, one novel and five previously reported mutations were found in 10 kindreds. In 12 ATP6V0A4 kindreds, seven of 10 mutations were novel. A further nine novel ATP6V0A4 mutations were found in "sporadic" cases. The previously reported association between ATP6V1B1 defects and severe hearing loss in childhood was maintained. However, several patients with ATP6V0A4 mutations have developed hearing loss, usually in young adulthood. We show here that ATP6V0A4 is expressed within the human inner ear. These findings provide further evidence for genetic heterogeneity in rdRTA, extend the spectrum of disease causing mutations in ATP6V1B1 and ATP6V0A4, and show ATP6V0A4 expression within the cochlea for the first time.

Does lower urinary tract status affect renal transplantation outcomes in children?

BACKGROUND: Lower urinary tract dysfunction (LUTD), an important cause of end stage renal disease (ESRD) in children, can adversely affect renal graft survival. We compared renal transplant patients with LUTD as primary renal disease to those without LUTD. METHODS: The data of 60 children who underwent renal transplantation (RTx) between 2000 and 2012 were retrospectively reviewed. All patients with LUTD were evaluated with urodynamic tests preoperatively; 15 patients required clean intermittent catheterization and 9 patients underwent augmentation cystoplasty before RTx. RESULTS: There were 25 children with LUTD. The mean follow-up for LUTD (+) and LUTD (-) groups were 63 (22-155) and 101 months (14-124), and graft survival were 76% for LUTD (+) and 80% for LUTD (-), respectively (P = .711). On the other hand, creatinine levels at last follow-up were significantly higher in the LUTD (+) group (1.3 ± 0.3 mg/dL vs 0.96 ± 0.57 mg/dL, P < .001). Infectious complications and postoperative urinary tract infection incidences were also higher in the LUTD (+) group (68% vs 25.7%, P = .002 and 60% vs 11.4%, P < .01). CONCLUSION: UTI is significantly higher after kidney transplantation in patients with LUTD. Despite the higher risk of UTI, renal transplantation can be performed safely in those patients with careful patient selection, preoperative management, and close postoperative follow-up. Restoration of good bladder function is the key factor in the success of kidney transplantation in those patients.

Mutation frequency of Familial Mediterranean Fever and evidence for a high carrier rate in the Turkish population.

Familial Mediterranean Fever (FMF) is a recessive disorder characterised by episodes of fever and neutrophil-mediated serozal inflammation. The FMF gene (MEFV) was recently identified and four common mutations characterised. The aim of this study was to determine the carrier rate in the Turkish population and the mutation frequency in the clinically diagnosed FMF patients. We found a high frequency of carriers in the healthy Turkish population (20%). The distribution of the five most common MEFV mutations among healthy individuals (M694V 3%, M680I 5%, V726A 2%, M694I 0% and E148Q 12%) was significantly different (P<0.005) from that found in patients (M694V 51.55%, M680I 9.22%, V726A 2.88%, M694I 0.44% and E148Q 3.55%).

Scleroderma in a child after chemotherapy for cancer.

The association of malignancy with scleroderma is very rare in childhood. A 13-year-old girl was diagnosed as having thymic carcinoma and received systemic chemotherapy. She presented with symptoms of Raynaud's phenomenon 9 months after the cessation of chemotherapy. She also had difficulty in swallowing. Based on the presence of Raynaud's phenomenon, characteristic skin changes over the face and hands, oesophageal involvement and pulmonary restrictive defect demonstrated by pulmonary function tests, the diagnosis of generalised scleroderma was established. There was no evidence of tumor recurrence. Although she was treated with penicillamine and prednisolone, no significant improvement was achieved in her condition during the 14-month follow up.

Role of A-SAA in monitoring subclinical inflammation and in colchicine dosage in familial Mediterranean fever.

OBJECTIVES: 1) To compare the sensitivity of serum amyloid A protein (A-SAA) and other acute phase proteins (APPs) in determining subclinical inflammation in patients with familial Mediterranean fever (FMF) during an attack-free period; 2) to define those clinical, laboratory features that may modify the A-SAA level; and 3) to evaluate the effect of an increase in the colchicine dose on the A-SAA level. METHODS: A-SAA, CRP, ESR, fibrinogen and ferritin levels were measured in 183 patients [88 F, 95 M; median age 11.0 years (1.0-20.0)] with FMF during an attack-free period. Mutational analysis was available in 157 patients. The colchicine dose was increased in 26 randomly chosen patients with no attacks within the last year; laboratory studies were repeated at the end of the second month. RESULTS: During an attack-free period, the median A-SAA level was 74 (6-1,500) mg/L; other APPs were within normal ranges in 49-93% of the patients. Age, gender, age at onset, age at diagnosis, the duration of treatment and the frequency of attacks had no significant effect on the A-SAA level. Homozygous and compound heterozygous patients had higher A-SAA levels than heterozygous patients [129 mg/L (8-1,500) versus 29 mg/L (6-216); p < 0.005]. There was a dramatic decrease in the A-SAA level [from 244 mg/L (16-1,400) to 35.5 mg/L (8-1,120); p < 0.001] and an increase in the hemoglobin (1.89 +/- 0.10 mmol/L to 1.98 +/- 0.19 mmol/L; p < 0.005) after the increase in colchicine dose in 26 patients. CONCLUSION: Subclinical inflammation continues during an attack-free period in FMF patients. A-SAA was the best marker of subclinical inflammation. Patients who are homozygous or compound heterozygotes of MEFV mutations had higher A-SAA levels. An increase in the colchicine dose resulted in a dramatic decrease in A-SAA and an increase in hemoglobin level. These findings favor the use of A-SAA in drug monitoring.

Survey of Turkish systemic lupus erythematosus patients for a particular mutation of C1Q deficiency.

OBJECTIVE: Hereditary C1q deficiency is a rare disease and up to now only 41 cases have been reported. Since all but 3 cases developed SLE or SLE-like disease, C1q deficiency represents the most powerful disease susceptibility gene identified for the development of SLE in humans. A molecular defect in homozygous C1q deficiency has been identified in 13 families. Four of these families are Turkish in origin and they all share the same mutation which is a CAG to TAG change at codon 186 in the A chain. This led us to investigate whether this mutation might be found in Turkish SLE patients and whether it could cause increased disease susceptibility when expressed in the heterozygous form. METHODS: We screened 65 Turkish lupus patients and 49 healthy Turkish individuals by carrying out an amplification of exon 2 of the A chain and restriction enzyme analysis for the C1qA mutation. RESULTS: We found no other example of this mutation in either the homozygous or heterozygous forms. CONCLUSION: C1q deficiency is one of the very strong disease susceptibility genes in lupus and may cause SLE via a critical role in the physiological clearance of apoptotic cells. However, C1q deficiency caused by a particular mutation in the A chain in a heterozygous form is not found in the Turkish SLE population.

Thrombomodulin, tissue plasminogen activator and plasminogen activator inhibitor-1 in Henoch-Schönlein purpura.

OBJECTIVE: To evaluate the changes in the plasma levels of thrombomodulin (TM), tissue-type plasminogen activator (t-PA) and plasminogen activator inhibitor-1 (PA1-1) as indices of endothelial injury/activation and fibrinolysis in childhood Henoch-Schönlein purpura (HSP). METHODS: Twenty-six children with HSP aged between 4-15 years and 10 healthy controls were included in the study. Blood samples were taken from these patients at admission and 6-12 weeks after healing of skin rash and arthritis. Plasma levels of TM, t-PA and PAI-1 activities and t-PA and PAI-1 antigen (Ag) levels were measured. RESULTS: The plasma levels of TM, t-PA Ag and PAI-1 Ag in patients during the acute phase were significantly different from the controls. The difference in TM between the acute phase and recovery in patients was also significant. The decrease in plasma levels of t-PA Ag and PAI-1 Ag in patients between the acute and recovery phases was not significant. t-PA activity was significantly higher in the acute phase than in the recovery phase. CONCLUSION: We suggest that increased levels of TM, t-PA, and PAI-1 activity may reflect the presence of endothelial injury and/or activation and fibrinolytic activation in patients with HSP.

Carbamazepine induced systemic lupus erythematosus. Another warning.

Carbamazepine induced systemic lupus erythematosus is a very rare phenomenon. Seven cases have been reported so far. We report another case documented with both clinical and serologic data and discuss some possible variations in related serology.