Nerve conduction study in young children suffering from cerebral palsy

Objectives: Cerebral palsy (CP) is a motor impairment syndrome leading to disorders of movement and posture. Screening of electrophysiological parameters Hoffman reflex (H-reflex and nerve conduction velocities) becomes a necessary for the early detection and management of the disease. The study aimed to assess the various electrophysiological parameters of nerve conduction velocity in CP and age-matched normal children. Matrials and Methods: The present cross-sectional study was performed between children suffering from CP and healthy control. A total of 27 children of 12–24 months of age of either sex were examined. Among them, six children were normal (with age match), seven were diagnosed with spastic CP and remaining 14 children were diagnosed with hypotonic CP The electrophysiological parameters were recorded in the right lower limb (posterior tibial nerve-soleus muscle) of all children. Results: In electrophysiological parameters, H-reflex latency in secs values was significantly decreased in all CP children. The maximum amplitudes of reflexly excitable motor neurons (Hmax) (mV) and Hmax/maximum amplitude of motor response ratio in the gastrocnemius-soleus muscle were significantly increased in spastic CPas compared to control. H-reflex conduction velocity (HRCV) was significantly higher than motor nerve conduction velocity (MNCV) in hypotonic CP children. Conclusion: The electrophysiological parameters were altered in spastic CP children. The electrophysiological parameters in hypotonic CP were within range, indicating they did not suppress the neuronal motor pool. However, HRCV was significantly more than MNCV in hypotonic CP, suggesting some myelination process defect/white matter injury in motor neurons. We concluded that the electrophysiological parameters of the nerve conduction study are a reliable test for the assessment of tone of muscles in children. Thus, it may help in the early initiation of the treatment and therapies in CP children.

Outcomes of children with idiopathic steroid resistant nephrotic syndrome: a single centre observational study / Desfechos de crianças com síndrome nefrótica idiopática córtico-resistente: um estudo observacional de centro único

ABSTRACT Introduction: Idiopathic steroid resistant nephrotic syndrome (SRNS) has variable outcomes in children. The primary objective of the present study was to assess the cumulative remission rate and the secondary objectives were to assess factors affecting the remission status, kidney function survival, and adverse effects of medications. Methods: One hundred fourteen patients with SRNS were included. Calcineurin inhibitor-based treatment protocol along with prednisolone and angiotensin-converting enzyme inhibitor were used, and patients were followed over 5 years. Results: Median age was 4.5 years; 53.5% of cases were between 1 to 5 years of age. Sixty-two patients (54.4%) were at initial stage and 52 (45.6%) were at a late SRNS stage. Median eGFRcr was 83.5 mL/min/1.73m2 at presentation. Of the 110 patients, 63 (57.3%) achieved remission [complete remission 30 (27.3%), partial remission 33 (30%)], and 47 (42.7%) had no remission. Kidney function survival was 87.3% and 14 cases (12.7%) had progression to CKD (G3-8, G4-3, G5-1, and G5D-2). Median duration of follow up was 36 months (IQR 24, 60). Age of onset, cyclosporine/tacrolimus, eGFRcr, and histopathology (MCD/FSGS) did not affect remission. Similarly, remission status in addition to age of onset, drug protocol, and histopathology did not significantly affect kidney function during a period of 5 years. Hypertension, cushingoid facies, short stature, cataract, and obesity were observed in 37.7, 29.8, 25.5, 17.5, and 0.7% of cases, respectively. Conclusion: About half of the cases achieved remission. Age of onset of disease, cyclosporine/tacrolimus use, and histopathological lesion neither affected remission status nor short-term kidney function survival in SRNS.

RESUMO Introdução: A síndrome nefrótica idiopática córtico-resistente (SNICR) apresenta desfechos variáveis em crianças. O objetivo principal deste estudo foi avaliar a taxa de remissão cumulativa. Os objetivos secundários foram avaliar fatores que afetam status de remissão, sobrevida da função renal e efeitos adversos de medicamentos. Métodos: Foram incluídos 114 pacientes com SNCR. Utilizou-se protocolo de tratamento baseado em inibidores de calcineurina juntamente com prednisolona e inibidor da enzima conversora de angiotensina. Os pacientes foram acompanhados durante 5 anos. Resultados: A idade mediana foi 4,5 anos; 53,5% dos casos tinham entre 1 e 5 anos. 62 pacientes (54,4%) estavam em estágio inicial; 52 (45,6%) em estágio tardio da SNCR. A TFGecr mediana foi 83,5 mL/min/1,73 m2 na apresentação. Dos 110 pacientes, 63 (57,3%) alcançaram remissão [remissão completa 30 (27,3%), remissão parcial 33 (30%)], e 47 (42,7%) não apresentaram remissão. A sobrevida da função renal foi 87,3%; 14 casos (12,7%) progrediram para DRC (G3-8, G4-3, G5-1, G5D-2). A duração mediana do acompanhamento foi 36 meses (IIQ 24, 60). Idade no início, ciclosporina/tacrolimus, TFGecr e histopatologia (DLM/GESF) não afetaram a remissão. Igualmente, status de remissão, além da idade no início, protocolo de medicamentos e histopatologia não afetaram significativamente a função renal por 5 anos. Observou-se hipertensão, fácies cushingoide, baixa estatura, catarata e obesidade em 37,7; 29,8; 25,5; 17,5; e 0,7% dos casos, respectivamente. Conclusão: Aproximadamente metade dos casos alcançou remissão. Idade no início, uso de ciclosporina/tacrolimus e lesão histopatológica não afetaram o status de remissão nem a sobrevida da função renal a curto prazo na SNICR.

Persistence of behavioral abnormalities following corticosteroid therapy in children with initial episode of idiopathic nephrotic syndrome: a prospective longitudinal observation / Persistência de anormalidades comportamentais após corticoterapia em crianças com episódio inicial de síndrome nefrótica idiopática: uma observação longitudinal prospectiva

Abstract Introduction: Treatment of nephrotic syndrome with corticosteroid can cause several side- effects including behavioral abnormalities. The objectives of the study were to observe the proportion of non-relapsers having persistence of behavioral abnormalities after completion of treatment of initial episode and compare the abnormalities with relapsers, and to determine risk factors for persistence. Methods: Seventy-five children with a first episode of idiopathic nephrotic syndrome and 60 normal children were rated by parents for behavioral problems using the Child Behavior Checklist. The Parenting Stress Index was also evaluated. The children were rated before treatment and 12 and 36 weeks after. Results: Both relapsers and non-relapsers showed abnormalities in internalizing and externalizing domains at 12 weeks of steroid therapy. Non-relapsers had abnormal scores in the internalizing domain in 63.5 % and externalizing domain in 48.1% of cases at 36 weeks. Relapsers had abnormal scores in all the three behavior domains, but a significantly higher proportion of relapsers had abnormal scores regarding total behavior (65.2% vs 28.8%, p<0.01) and child domains (100% vs 57.7%, p<0.001) of Parenting Stress Index in comparison to non-relapsers at 36 weeks. Occurrence of relapse increased the risk (odds ratio 5.76, 95% CI 1.35-10.76, p< 0.001) for persistence of abnormal total behavior at 36 weeks follow-up. Conclusion: Persistence of abnormalities was observed not only in relapsers but also in non-relapsers. Relapse was found to be a significant risk factor for persistence of abnormal behaviors in these patients.

Resumo Introdução: O tratamento da síndrome nefrótica com corticosteroide pode causar vários efeitos colaterais, incluindo anormalidades comportamentais. Os objetivos do estudo foram observar a proporção de não-recidivos com persistência de anormalidades comportamentais após conclusão do tratamento do episódio inicial, comparar as anormalidades com os recidivos, e determinar fatores de risco para persistência. Métodos: 75 crianças com primeiro episódio de síndrome nefrótica idiopática e 60 crianças normais foram avaliadas pelos pais por problemas comportamentais usando o Checklist de Comportamento Infantil. O Índice de Estresse Parental também foi avaliado. As crianças foram avaliadas antes do tratamento, 12 e 36 semanas após. Resultados: Tanto recidivos quanto não recidivos mostraram anormalidades nos domínios de internalização e externalização às 12 semanas de terapia com esteroides. Não-recidivos apresentaram pontuações anormais no domínio de internalização em 63,5%, e no domínio de externalização, em 48,1% dos casos em 36 semanas. Recidivos tiveram pontuações anormais em todos os três domínios de comportamento, mas uma proporção significativamente maior de recidivos apresentou pontuações anormais em relação ao comportamento total (65,2% vs 28,8%, p<0,01) e domínios infantis (100% vs 57,7%, p<0,001) do Índice de Estresse Parental em comparação com não recidivos às 36 semanas. A ocorrência de recidiva aumentou o risco (odds ratio 5,76, 95% IC 1,35-10,76, p< 0,001) de persistência de comportamento total anormal em 36 semanas de acompanhamento. Conclusão: A persistência de anormalidades foi observada não apenas em recidivos, mas também em não recidivos. A recidiva foi um fator de risco significativo para a persistência de comportamentos anormais nesses pacientes.

Portal Hypertension with Visceral Leishmaniasis.

We conducted this study to observe evidence of portal hypertension in children with visceral leishmaniasis (VL). Eighty-eight consecutive cases (50 male) of VL were subjected to ultrasonography. Those with evidence of portal hypertension also underwent upper gastrointestinal endoscopy and liver biopsy. Eight patients had portal hypertension as evidenced by dilated caliber of portal and splenic veins. Two patients had periportal, splenic and peripancreatic collaterals and one patient had cavernous transformation of portal vein. Out of eight patients, four patients had esophageal and gastric varices. Liver biopsy was done in four patients and revealed hepatic sinusoidal dilations without any evidence of fibrosis. Portal hypertension may be an independent manifestation of VL and remain undiagnosed unless a physician maintains a high index of suspicion.

Unusual presentations of visceral leishmaniasis.

We report 3 cases of Visceral leishmaniasis, who presented with unusual clinical manifestations. One child was diagnosed as hemophagocytic syndrome; another masqueraded with features of leukemoid changes alongwith hemophagocytosis and trilineage myelodysplasia; the third case presented with pyothorax. All the three patients showed amastigote forms of Leishmania donovani and positive serology (rk39 antigen). They showed complete clinical, hematological and parasitological resolution with Amphotericin B therapy.

Stem cell through present and future.

Stem cell transplantation (SCT) has the potential to transform the lives of children with a wide variety of genetic diseases, ranging from inherent defects of hemopoietic cell production or function through to metabolic diseases mostly affecting solid organs. For these children life expectancy or quality of life would otherwise be very poor. It ranks as one of the most remarkable therapeutic advances of the past 40 years. Despite rapid technological improvements, however, there are still many short term risks and potential long term toxicities. Consequently, the rapid emergence of alternative therapies (including new drugs, enzyme and gene therapies), necessitate constant re-evaluation of the risk/benefit ratio for each disease and hence the appropriateness of SCT. This review describes the major aspects of the transplant process, indications for transplantation, outcome statistics, and areas where alternative therapies are becoming available. SCT remains a highly experimental therapy. Due to the relatively short history of the discipline no data exists on truly long term follow up. This is important as some organs benefit relatively poorly or problems may emerge which were never apparent as part of the untreated disease. The speed of technological change makes randomised trials on these diseases, which are individually quite rare, almost impossible to perform.

Dapsone induced methemoglobinemia : Intermittent vs continuous intravenous methylene blue therapy.

OBJECTIVE: The study compares the decline in blood methemoglobin (MetHb) level in children of dapsone intoxication treated with intermittent and continuous methylene blue therapy. METHODS: Eleven children with history of accidental dapsone ingestion and suggestive clinical features of dapsone intoxication were studied. Patients were randomized into two groups: Gr I (n=5) received intermittent methylene blue therapy, while Gr II (N=6) as continuous infusion. The dose of methylene blue was same in both groups. MetHb level in blood was assessed by spectrophotometer at admission and thereafter 12hrly up to 72 hrs. The decline in MetHb was statistically analyzed with student t-test. RESULTS: Six patients had history of seizure and altered sensorium. Severe anemia was observed in 2 patients. The mean levels of MetHb in Gr II was statistically significant after 12, 24, 36, 48 and 72 hrs of methylene blue therapy as compared to Gr I. CONCLUSION: Continuous I.V methylene blue therapy causes significant decline in MetHb level and is more effective in treatment of methemoglobinemia as compared to intermittent regimen.

Deep vein thrombosis with Staphylococcus aureus septicemia.

Deep vein thrombosis (DVT) in children is usually associated with inherited or acquired hypercoagulable state, mechanical obstruction, fractures of long bones, central venous catheterization and prolonged immobility. We report DVT in 4 children with culture proven staphylococcal septicemia. One child died, while other three survived with appropriate antibiotics and anticoagulation therapy.

Miltefosine in children with visceral leishmaniasis.

Sixty four children (38 boys and 26 girls), aged 1 yr to 14 yr, presenting with fever, splenomegaly and positive LD body in splenic smear examination, admitted to pediatric ward of Nalanda Medical college and Child care center between 1st July 03 to 30th June 04 were taken for study. Patients were categorized into two groups: 44 were in Group I (Patients who had not received prior antileishmanial drug) and 20 in Group II (Patients who had received 30 days course of SAG; 20 mg per kg per day). All patients were given Miltefosine in dose of 2.5 per kg per day od or bid per orally to a maximum of 100 mg and were followed at completion of therapy, 1 month and 6 months for clinical response, splenic size and parasite density. 63 patients had parasitological cure with relapse in one patient of Group I during follow up. One patient in Group II had no response with first course but became parasitologically negative with 2nd course of Miltefosine. In Group I, one patient had persistent splenomegaly and found to have associated portal hypertension. GI side effects i.e. diarrhea and vomiting were observed in 26 and 23 patients respectively. Majority of patients had pancytopenia. Elevated ALT (> 3 times of normal) were seen in 28 and 11 patients of Group I and Group II respectively which returned to normal in subsequent follow up. The final cure rates were 93.2 percent and 95 percent in Groups I and II respectively.

Miltefosine in children with visceral leishmaniasis: a prospective, multicentric, cross-sectional study.

OBJECTIVE: Miltefosine, an alkyl phospholipid has been found effective against visceral leishmaniasis (VL) in adults in various studies. The authors safety, tolerance and efficacy of Miltefosine and compared with available gold standard anti-Ieishmanial drug, Amphotericin B, a parenteral formulation in children with VL. METHODS: All consecutive children aged 1 yr to 14 yr, presented with fever, splenomegaly and positive LD body in splenic smear examination, admitted in pediatric ward of Nalanda Medical college and Child care center between 1st July 03 to 30th June 05 were taken for study. Patients were randomized into four groups. Group-l and 2 patients were given Miltefosine in dose of 2.5 mg/Kg day o.d. or b.i.d. per orally to a maxiIpum of 100 mg and group 3 and 4 Amphotericin B at a dose of 1 mg/Kg/day (total: 15 mg/Kg). All patients were followed at completion of therapy, 3 months and 6 months for clinical response, splenic size and parasitologically. RESULTS: Out of 125 children, 44 were in group-I, 20 in group-2, 38 in group-3 and 23 in group- 4, 124 patients had parasitological cure with relapse in one patient of group 1 during follow up. One patient in-group II had no response with first course but became parasitologically negative with 2nd course of Miltefosine. In-group I, one patient had persistent splenomegaly and found to have associated portal hypertension. Final cure rate with Miltefosine and Amphotericin B was 93.2%, 95%, 92.1% and 91.3% in-group 1, 2, 3 and 4 respectively, which are statistically insignificant. Majority of patients had pancytopenia. Eievated". AL T (>3 times of normal) were seen in 28, 11, 19 and 13 patients of group 1, 2, 3 and group 4 respectively which returned to normal in subsequent follow up. Raised BUN was observed more in patients who got Amphotericin B i.e. 65.42% and 73.91 % in-group 3 and 4 respectively. GI side effects i.e. diarrhea and vomiting were observed in 26 and 23 patients in-group 1 and 2 respectively. CONCLUSION: Miltefosine is safe, well tolerable, and highly effective and has same efficacy as Amphotericin B in newly diagnosed and SAG resistant children with visceral leishmaniasis.

Current concept in the management of cerebral palsy.

The authors have described, in depth, the current concept along with the management of cerebral palsy in the present article.

Oral atropine sulfate for infantile hypertrophic pyloric stenosis.

This study aimed to evaluate the effectiveness of oral atropine in the management of IHPS. Cases were diagnosed clinically and confirmed sonographically. Atropine was given orally from the outset at a dose of 0.18 mg/kg/day in eight divided doses, increased daily by 1/4th of the commencing dose till vomiting ceased. Ultrasonographic evaluation of pyloric muscle thickness and length was done at the commencement of treatment, after completion of treatment and at 3, 6, 9, 12 and 15 months follow up. Oral atropine was effective in 11/12 (91.06%) cases. Vomiting ceased in 14 to 21 days in all cases. One case required initial 7 days of i.v. treatment followed by 18 days oral treatment to stop vomiting. USG evidence of normalization of pylorus was observed in all these cases, 3-15 months after completion of treatment. We conclude that oral atropine proved to be a simple, effective, safe, very cheap and acceptable treatment option for IHPS.

Department of Paediatrics, Nalanda Medical College, Patna.

Cysticercosis is the most common parasitic disease of the central nervous system. The disease has worlwide distribution. The cysticerci might lodge in the brain parenchyma, spinal cord, eyes, ventricular system, subarachnoid space and muscle. These are most often seen in basal meninges. The presence of human lymphocyte antigen-related antigens on the surface of cysticerci has a direct relationship with microscopic signs of damage to cysticerci. The clinical manifestations depend upon number and topography of lesions, the individual immune response to the parasite and the sequelae of previous infestations. The diagnostic criteria of neurocysticercosis can be based on absolute criteria, major criteria, minor criteria and epidemiological criteria. Computerised tomography (CT) head is still most useful diagnostic tool for the diagnosis of neurocysticercosis but magnetic resonance imaging has some advantages over computerised tomography. The mainstay of therapy lies on medical and surgical intervention. Medical therapy consists of cysticidal drugs. Surgical therapy is indicated in intraventricular and subarachnoid neurocysticercosis. Steriods are used for anti-oedema measures. Anti-epileptics can also be tried. The measures for prevention of cysticercosis are proper disposal of human waste, treatment of water contaminated with human faeces before its use in irrigation of vegetable cultivation, proper cooking of pork and repeated treatment in taenia carriers.

Miltefosine: an oral drug for visceral leishmaniasis.

Miltefosine, a phosphocholine analogue originally developed as antimalignant drug, has been found to be highly active against leishmania in vitro and animal model. Based on these experiences this drug was tried against human visceral leishmaniasis and found to be highly effective and achieved 97% and 94% cure in phase 2 and phase 3 trial in children.

Management of diarrhoea in practice.

Diarrhoea, a major cause of morbidity and mortality can be produced by a variety of etiological factors. Management protocol includes assessment of the child, physical examination, lab-evaluation, assessment of severity of dehydration and rehydration therapy using either of the following - WHO - ORS, Home available fluids (HAF), sugar salt solution (SSS), improve WHO-ORS, Amino acid fortified ORS, rice based ORS, low osmolarity ORS. Intravenous fluids are required if patients can't accept orally. Commonly observed electrolyte disturbances are hypernatremia, hyponatremia and hypokalemia. Concussion is a common problem and can result due to electrolyte imbalance, cavernous sinus thrombosis, associated meningitis, shigella encephalopathy and hypoglycemia in undernourished children. Treatment includes i.v. diazepam and i.v. glucose and correction of electrolyte imbalance. Additional treatment interventions include antimicrobial drugs including antibiotics, antimotility drugs, absorbents, nutritional and micro and macro nutrient supplementation.