Biswanath Basu

Behavioural abnormalities in children with nephrotic syndrome

BACKGROUND Glucocorticoid therapy in children with nephrotic syndrome can lead to many adverse effects including behavioural problems. The present study was undertaken to assess the changes in individual behaviour among different sub-groups of patients with idiopathic nephrotic syndrome (INS) and also to find out the relationship, if any, between different behavioural problems with cumulative dose of steroid therapy. METHODS This was a prospective hospital-based study. We assessed behavioural patterns in 131 children and adolescents with steroid-responsive INS aged 1.5-15 years. Fifty healthy children matched for age and gender were included to serve as controls. The Achenbach Child Behaviour Checklist was used to assess individual behaviour. Patients were sub-grouped according to age (1.5-5 and 6-15 years) and disease status (first attack before and after 12-week prednisolone, infrequent relapser, frequent relapser/steroid-dependent). RESULTS All groups had significantly elevated mean behavioural abnormality scores for dimensions assessed in both age groups, except rule-breaking behaviour. Besides sleep problems, frequent relapsers/steroid-dependent patients exhibited maximum scores in comparison to first attack and infrequent relapsers in the 1.5- to 5-year age group. Total and individual behavioural scores showed close associations with cumulative prednisolone dose in both groups. CONCLUSIONS It is evident that nephrotic syndrome patients should be given due consideration in clinical practice for behavioural abnormalities especially after steroid therapy.

Mycophenolate Mofetil Following Rituximab in Children With Steroid-Resistant Nephrotic Syndrome

BACKGROUND: Rituximab is being increasingly used in children with idiopathic nephrotic syndrome resistant to standard treatments. In spite of good initial response, rituximab responders always remain prone to further relapse, necessitating either repeat course of rituximab or addition of another steroid-sparing immunosuppressant. METHODS: A retrospective analysis of baseline clinico-pathologic presentation and treatment outcome (complete remission, partial remission, or no response) was performed among 24 children with refractory-idiopathic SRNS over a follow-up period of 24 months. Children received 2 to 4 rituximab infusions (375 mg/m2 weekly) depending on circulating B-cell level. At 3-month follow-up, a second course of rituximab was administered (if >5 B cells/mm3) along with MMF (1200 mg/m2 per day) maintenance therapy. RESULTS: Of 24 patients, 54% (13/24) and 46% (11/24) had minimal change disease and focal segmental glomerulosclerosis, respectively, on renal histopathology. After the first course of rituximab, 21% (5/24) of children achieved complete remission; however, most (4/5) of them relapsed again at a median interval of 53 (interquartile range 46–72) days. Depending on response to the first course of rituximab, MMF was started on 15 children at 3 months. After 6 months, 67% (10/15) of children on MMF achieved complete remission and 33% (5/15) remained at partial remission. At 24 months overall, 25% (6/24) and 42% (10/24) of children were in complete remission and partial remission, respectively; 33% (5/15) of children continued sustained complete remission after postrituximab-MMF maintenance therapy in comparison with no sustained complete remission with rituximab alone at 24 months (P < .001). CONCLUSIONS: MMF may be an effective and safe maintenance therapy to consider as an additive immunosuppressant after induction with rituximab in maintaining remission among children with refractory SRNS.

Psychological Changes of Children Surviving Terrorist Shock in Indian Kashmir

This study was undertaken with the objectives to assess psychological responses in children surviving terrorist attack and to observe relationships between various risk factors and subsequent psychological changes. Assessment of psychological changes was done on 131 children who were directly exposed to terrorist attack. Achenbach Child Behavior Checklist was used to assess victimized children for analysis purpose. Possible risk factors were assessed by questionnaire to the parents. What has emerged as the most interesting aspect of this research is that, whereas for adults, pretraumatic risk factors are extremely important predictors of chronic posttraumatic symptoms, for children, the most important consideration appears to be the death of the mother and number of stressors.

WITHDRAWN: Efficacy and Safety of Mycophenolate Mofetil Versus Levamisole in Children and Adolescents With Idiopathic Nephrotic Syndrome: Results of a Randomized Clinical Trial.

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Transient myeloproliferative disorder with trisomy 12.

The authors report the first case of transient myeloproliferative disorder (TMD) in a neonate with trisomy 12. The clinical course consisted of respiratory distress since birth with probability of transient tachypnea of newborn, but routine investigation revealed total leukocyte count of 56000/μL with 91% blasts, which returned to normal spontaneously during the subsequent 3 weeks. GTG-banded karyotype from peripheral blood was done to detect any mutation, specifically trisomy 21, but the proband revealed trisomy 12 and denaturing polyacrylamide gel electrophoresis (PAGE) detected mutation in exon 2 of GATA1. The condition has been described in association with Down syndrome (trisomy 21) but never with trisomy 12. This case demonstrates the importance of knowing this entity so that it is not erroneously diagnosed as a leukemic process. This is extremely important because most cases of TMD resolve spontaneously within a few weeks to months and do not require treatment other than supportive measures. A search of the literature did not reveal any similar case.

Retinal mass in a case of pediatric myelodysplastic syndrome (refractory anemia with excess of blasts).

Purpose. Myelodysplastic syndrome is a rare childhood clonal hematologic disorder characterized by dysplastic hematopoiesis and progression to leukemia. Methods. Case report. Results. An 8-year-old boy with low-grade fever, easy fatigability, and pallor of 3 months duration showed pancytopenia with 9% circulating blast cells. The bone marrow aspirate revealed striking trilineage dysplasia with 13% of blast cells. Cytogenetic analysis revealed 46, XY, del (20) (q11;q13). The findings were diagnostic of myelodysplastic syndrome with refractory anemia with excess of blasts in accordance with the World Health Organization criteria modified for pediatric age group. Fundus examination revealed a sickle-shaped hanging retinal mass of right eye. Biopsy/aspiration cytology of the retinal mass was not done in this case owing to risk of severe bleeding, a common feature in any hematologic malignancy. The child had been included in pediatric acute myeloid leukemia trials as the parents were not ready for stem cell transplantation, but he died after 3 months due to overwhelming infection. Conclusions. Myelodysplastic syndrome of childhood may present with retinal mass along with other hematologic features. Biopsy/aspiration cytology of the mass should not be attempted as it may cause intraocular hemorrhage.

The co-existence of CHARGE and myelodysplastic syndrome in a child.

We report the case of an 8-month-old female child with co-existence of CHARGE with myelodysplastic syndrome, which is not reported in the literature. The patient was treated with packed cell transfusion, laser photocoagulation for retinal detachment, and antimicrobials, and referred for bone marrow transplantation.

Osteogenesis imperfecta with partial trisomy 15.

Osteogenesis imperfecta (OI) is the most common genetic cause of osteoporosis, which presents as multiple fractures of bone. Mutations in the loci COL1A1 on band 17q21 and COL1A2 on band 7q22 have been reported as the cause in most cases of OI, but partial trisomy 15 has not been reported previously as a possible cause. A 3-month-old child with OI with an unusual association of partial trisomy 15 is reported.