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1.
Background
Nephrotic syndrome is a rare but severe feature of IgA nephropathy.Case characteristics
Nine Japanese children with severe IgA nephropathy with nephrotic syndrome.Intervention
All received low-dose intravenous methylprednisolone (IVMP) within five weeks after the disease onset. Eight out of nine patients achieved resolution of proteinuria without severe adverse events.Message
Early low-dose intravenous methylprednisolone may be safe and effective for children with severe IgA nephropathy with nephrotic syndrome.2.
Background
Calcineurin inhibitors (CNIs) are commonly given to transplant recipients of kidneys and other solid organs and to patients with immune disorders, such as steroid-resistant nephrotic syndrome, steroid-dependent nephrotic syndrome, and frequent relapse nephrotic syndrome. Although CNIs remain the most effective available immunosuppressant agent, there is clinical concern regarding possible long-term nephrotoxicity. This concern is especially significant in children who have a longer life expectancy and greater growth rate.Data sources
In this review, we analyzed the literatures to identify original articles that examined use of CNIs in children who received organ transplantation and nephropathy to assess the available evidence of their nephrotoxicity. PubMed, Elsevier, and Tompson ISI Web of Knowledge were searched for identifying relevant papers.Results
Clinical research supports the presence of CNI-related nephrotoxicity. However, some researchers have questioned the prevalence and seriousness of chronic CNIs nephrotoxicity, especially because the pathological lesions typically associated with long-term CNI use are nonspecific. Many researchers have focused on early markers of CNI nephrotoxicity, and the methods that may help prevent and manage nephrotoxicity.Conclusions
Future research should focus on investigating early markers of CNI nephrotoxicity and strategies for improved immunosuppressant therapy, and developing alternative treatments. CNI-mediated nephrotoxicity should always be taken seriously in clinic.3.
Background
Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. Severe central nervous system (CNS) involvement is rare in HSP.Case characteristics
Three children with features of HSP presented with seizures and CNS dysfunction.Observation
All three children had abnormalities on neuroimaging; 2 had complete remission but one was left with severe neurological damage.Message
HSP patients may rarely present with CNS involvement with a prolonged course requiring aggressive treatment.4.
Objective
To evaluate the frequency of non-autoimmune subclinical and overt hypothyroidism in children with idiopathic steroid-resistant nephrotic syndrome (SRNS).Methods
This cross-sectional study recruited 30 children (age 1–18 y) with idiopathic SRNS; and 30 healthy controls. Serum T3, T4 and TSH were performed in cases as well as controls. Anti-thyroid peroxidase and anti-thyroglobulin antibody tests were performed in all cases.Results
Non-autoimmune subclinical or overt hypothyroidism was detected in 10 out of 30 children with idiopathic SRNS; 2 had overt hypothyroidism, while 8 patients had subclinical hypothyroidism. Children with SRNS had a mean (SD) TSH value 4.55 (4.64) mIU/L that was higher as compared to controls (1.88 (1.04) mIU/L) (P<0.01). Focal segmental glomerulosclerosis (FSGS) was the commonest histopathological condition, seen in 13 (43.3%). Children with overt hypothyroidism (2 cases) and grade III subclinical hypothyroidism (1 case) were subsequently started on levothyroxine therapy.Conclusions
The prevalence of subclinical and overt hypothyroidism seems to be high in idiopathic SRNS, with almost one-third of children having overt or subclinical non-autoimmune hypothyroidism.5.
Background
Blue rubber bleb nevus syndrome is a rare disease involving venous malformations.Case characteristics
We present a 6-year-old female with the syndrome, and consumptive coagulopathy.Intervention/Outcome
After management with sirolimus, symptoms resolved.Message
Sirolimus may be a valuable option for reducing bleeding complications and cosmetic sequelae for the patients with this syndrome.6.
Vasanthan Tanigasalam B. Vishnu Bhat Sharmila Manivannan Malathi Munisamy Swapnil Parchand 《Indian pediatrics》2018,55(7):601-602
Background
Schimmelpenning syndrome is a multisystem disorder.Case characteristics
A term female neonate with sebaceous nevi of the face had choroid osteoma of the right eye.Observation
At one month of age, the infant was observed to have choroidal neovascularization that was successfully treated with laser photocoagulation and anti-VEGF.Message
Choroid osteoma and neovascularization are rare associations of Schimmelpenning syndrome, and should be screened for and managed early.7.
Background
The clinical management of intravenous immunoglobulin-resistant Kawasaki disease shock syndrome (KDSS) is obscure.Case characteristics
Three children presented with intravenous immunoglobulin-resistant KDSS complicated with myocarditis.Outcome
All cases were successfully managed with steroid pulse therapy.Message
Steroid pulse therapy is effective in immunoglobulin-resistant KDSS.8.
Zeynep Canan Özdemir Yeter Düzenli Kar Coþkun Yarar Suzan Þaylýsoy Özcan Bör 《Indian pediatrics》2017,54(5):413-415
Background
Lymphoma-associated incomplete Miller-Fisher syndrome is very rare.Case Characteristics
An 11-year-old boy who initially presented with headache, left ptosis, diplopia and weakness. Neurologic examination indicated left sided ptosis with ophthalmoplegia.Observations
Cerebral imaging and cerebrospinal fluid examinations were normal. Magnetic resonance imaging of the abdomen showed a mass lesion in the ileal loops. A bone marrow biopsy showed infiltration by Burkitt’s lymphoma.Message
Burkitt lymphoma may present with incomplete Miller Fisher syndrome.9.
Background
Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by arterial or venous thrombosis in children.Case characteristics
11-year-old child with pneumococcal meningitis also had cerebral sinus vein thrombosis and pulmonary artery segmental thrombosis.Observation
Pro-thrombotic evaluation showed positive lupus anticoagulant at baseline and after 12 weeks. Investigations for lupus were negative at admission and after one year of follow-up.Message
Antiphospholipid syndrome is a possibility even in thrombosis occurring in the setting of meningitis.10.
Nevitha Athikari Manamal Tanu Singhal Abhaya Kumar Darshana Sanghvi Jayanti Mani 《Indian pediatrics》2018,55(11):993-994
Background
Neuroschistosomiasis is an uncommonly reported disease.Case characteristics
An adolescent Indian boy residing in Kenya presented with headache, visual symptoms and seizures, with MRI showing space-occupying lesions in the occipital lobe and cerebellum.Observation
Brain biopsy was diagnostic of neuro-schistosomiasis; complete recovery was seen with praziquantel and corticosteroid therapy.Message
This case highlights the importance of considering epidemiology in differential diagnosis and establishing definitive diagnosis even if it is by invasive methods.11.
M. Joseph John Chepsy C. Philip Amrith Mathew Abhilasha Williams Naveen Kakkar 《Indian pediatrics》2017,54(4):327-328
Background
Allogeneic stem cell transplant is the only curative treatment for Wiskott-Aldrich syndrome.Case characteristics
18-months-old boy with no sibling, cord blood or matched unrelated donor transplant options.Outcome
Doing well 7 years after haploidentical stem cell transplantation using unmanipulated bone marrow as the stem cell source.Message
Father as a haplo-identical donor is a feasible option.12.
Background
Inflammatory myofibroblastic tumors of the trachea are rare childhood quasi-neoplastic lesions.Case characteristics
7-year-old boy with recurrent episodes of cough, breathing difficulty and wheeze, initially treated as asthma.Intervention
CT chest and flexible bronchoscopy revealed a mass lesion of the trachea, which was excised by diode laser through the ventilating bronchoscope. Histopathology confirmed it as the inflammatory myofibroblastic tumor.Message
Use of laser ensured complete endotracheal excision of the tumor.13.
14.
Ibrahim Kartal Ayhan Dagdemir Murat Elli Meltem Ceyhan Bilgici 《Indian pediatrics》2018,55(8):699-700
Background
Infantile choriocarcinoma is usually fatal without appropriate treatment.Case characteristics
A 3-month-old boy who presented with respiratory distress, hepatomegaly, amemia and bilateral nodular lesions on chest X-ray.Observation
Fine-needle liver aspiration revealed necrotic tumour cells. The serum β-hCG level was very high (2057 mIU/L), supporting a diagnosis of infantile choriocarcinoma of the liver. Surgical resection after cisplatin-based multiagent chemotherapy afforded successful remission.Message
Early treatment of infantile choriocarcinoma can yield a successful outcome.15.
Rajeev Redkar Anant Bangar Varun Hathiramani Vinod Raj C. Swathi 《Indian pediatrics》2018,55(10):871-873
Objective
To study the diagnostic methods and treatment outcomes in children with Budd-Chiari syndrome.Methods
Case records of 25 patients with Budd-Chiari syndrome were evaluated retrospectively. These patients were investigated with imaging techniques and underwent balloon angioplasty or surgical management.Results
21 patients underwent balloon angioplasty, of which 17 had good medium- to long-term results, while only one out of four patients who underwent a portocaval shunt survived.Conclusion
The balloon angioplasty has satisfactory outcome in the treatment of acute Budd-Chiari syndrome. In failed cases, the surgical therapy may be attempted, but the outcomes do not appear rewarding.16.
Meriem Zahed-Cheikh Barthélémy Tosello Stéphanie Coze Catherine Gire 《Indian pediatrics》2017,54(6):495-497
Background
The kyphoscoliotic type of Ehlers-Danlos syndrome (EDS type VIA) is an autosomal recessive disorder characterized by connective tissue dysplasia.Case characteristics
We report two children with perinatal stroke; accompanied by neonatal joint hypermobility, hypotonia; and early development of kyphoscoliosis.Outcome
Molecular analysis revealed a PLOD1 gene mutation. Our definitive diagnosis was a EDS VIA.Message
Prenatal brain stroke is a rare clinical feature of EDSVIA.17.
Marcin Tkaczyk Monika Miklaszewska Jolanta Lukamowicz Wojciech Fendler 《World journal of pediatrics : WJP》2016,12(3):353-359
Background
The role of idiopathic nephrotic syndrome (INS) in the pathogenesis of atherosclerosis in childhood has not been clearly elucidated. However, antioxidative defense in INS is thought to be imbalanced. This study aimed to assess the changes of plasma concentration of selected aminothiols in the blood of children with INS at various stages of the disease.Methods
This cross-sectional study was conducted in 125 children aged 2-18 years. The children were divided into 4 groups: group A, early relapse (n=37); group B, early remission for 4-6 weeks from the onset (n=37); group C, late steroid-free remission (n=31); and group D, long-term remission for 2-5 years (n=20). Control group (E) consisted of 30 age- and gender-matched healthy children. The study protocol comprised an analysis of plasma concentrations of glutathione, homocysteine, cysteine and cysteinylglycine by high-performance liquid chromatography. Fractions of protein-bound and free aminothiols were measured. Endothelial injury was assessed by thrombomodulin, PAI-1 concentration, and von Willebrand factor activity.Results
The children with INS had unbalanced aminothiol metabolism only in relapse and early remission, that shifted towards increased oxidative processes. Administration of cyclosporine A caused a significant increase in homocysteine and cysteine concentration. Changes in aminothiol metabolism were significantly related to endothelial injury.Conclusions
The findings of this study may be helpful in elucidating the pathogenesis of premature atherosclerosis in patients with INS refractory to the treatment or in the case of frequent relapse.18.
Abhijeet Botre Vrajesh Udani Neelu Desai Spoorthy Jagadish Milind Sankhe 《Indian pediatrics》2017,54(8):678-680
Background
Management of refractory status epilepticus in children is extremely challenging.Case characteristics
Two children with medically refractory status epilepticus, both of whom had lesional pathology on MRI and concordant data on EEG and PET scan.Intervention
Emergency hemispherotomy performed in both patients. A complete, sustained seizure freedom obtained postoperatively.Message
Emergency surgery is a treatment option in selected cases of drug refractory status epilepticus with lesional pathology and concordant data.19.
20.
Shrishail Gidaganti M. M. A. Faridi Manish Narang Prerna Batra 《Indian pediatrics》2018,55(3):206-210