Tapas Sabui

Growth of children with juvenile idiopathic arthritis

ObjectiveTo evaluate the growth pattern in children with juvenile idiopathic arthritis and its subtypes in comparison with age, sex and temporally matched controls.Study designProspective study.SettingPediatric rheumatology clinic of a tertiary care hospital in Eastern part of India.ParticipantsSeventy-five children (2–12 years) diagnosed as juvenile idiopathic erthritis by International League of Associations for Rheumatology criteria and 75 age- and sexmatched controls.InterventionWeight, height and body mass index were recorded at six monthly interval in both groups over a period of 3 years.Main outcome measuresweight, height and body mass index.ResultsSubtype distribution of juvenile idiopathic arthritis was: oligoarthritis (49%, n=37), rheumatoid factor negative polyarthritis (27%, n=20), rheumatoid factor positive polyarthritis (8%, n=6), systemic onset (15%, n=11) and enthesitis related arthritis (1.3%, n=1). Anthropometric parameters in children with juvenile idiopathic arthritis were not significant different from controls. Comparison between the subtypes showed significant differences in height (P=0.011), weight (P=0.005), and growth velocity (P=0.005), but not in body mass index. Systemic onset disease led to significant restriction in height (P=0.018; 95% CI 2.13–33.77) and weight (P=0.008; 95% CI 1.47–14.43) compared to controls. Growth velocity was significantly affected in rheumatoid factor positive polyarthritis (P=0.003; 95% CIO. 46–3.14).ConclusionsChildren with juvenile idiopathic arthritis do not have significantly lower values of anthropometric parameters compared to controls. Significant restriction in height and weight is seen in systemic onset disease, and growth velocity is significantly reduced in rheumatoid factor positive subjects.

Normative blood pressure data for Indian neonates.

ObjectivesTo establish the normative blood pressure (BP) values in healthy Indian neonates using oscillometric method, and to develop BP percentile charts.DesignProspective observational study.SettingNeonatal unit of a teaching hospital in Eastern India.Participants1617 hemodynamically stable inborn neonates without birth asphyxia, major congenital anomaly, maternal complications (e.g. preeclampsia, hypertension, diabetes) or critical neonatal illness.ProcedureQuite state measurements of systolic BP (SBP), diastolic BP (DBP) and mean arterial pressure (MAP) were recorded by oscillometric method on day 4, 7 and 14 of postnatal life. The averages of three readings at 2-minute intervals were used.ResultsPercentile charts (providing 5th, 10th, 25th, 50th, 75th, 95th, and 99th percentile values) have been developed. SBP, DBP and MAP showed a steady rise from day 4 to day 14, and were comparable between males and females, but were significantly lower in preterms than in term neonatesConclusionNormative neonatal BP data along with gestational age-wise percentile charts shall be of help for decision-making and planning for sick newborns.

Animal Bites and Rabies Prophylaxis in Rural Children: Indian Perspective

A prospective observational study was conducted in a tertiary care hospital to study clinicoepidemiological profile of potentially rabid animal bite cases from rural India. Total of 308 children (median age 6 years) admitted to hospital, were recruited over 1 year and followed up till completion of antirabies vaccine course. Dog was the commonest (77.27%) offending animal. Of the exposures, 66.88% were scratches, 88.96% were unprovoked and 27.27% were categorized as Class III. The median times to wound toileting and reporting to health facility were 1 and 6 h, respectively. Majority received prompt PEP in hospital, and RIG was administered in 34.55% of Class II and 90.48% of Class III exposures. Compared with their older counterparts, children aged <5 years suffered more bites on face and trunk and more Class III exposures. The rabies prophylaxis scenario is encouraging, when compared with earlier studies, but there are gaps to be addressed.

Congestive Heart Failure: An Uncommon Presentation of Systemic Onset Juvenile Idiopathic Arthritis (SOJIA)

Children with systemic onset Juvenile idiopathic arthritis (SOJIA) are known to develop myocarditis and congestive heart failure as a complication of disease process infrequently. Cardiac involvement causing congestive heart failure as a presenting manifestation in SOJIA is rarely described in the literature. The authors describe a case of an 11- yr- old boy with SOJIA who presented with congestive heart failure following active myocarditis, with the flare of the disease process. The cardiac manifestations, along with the disease activity were controlled with intensive immunosuppressive therapy.

Evaluation of malnutrition as a predictor of adverse outcomes in febrile neutropenia associated with paediatric haematological malignancies.

Malnutrition has been reported in the literature to be adversely associated with outcomes in paediatric malignancies. Our objective in this paper was to evaluate malnutrition as a potential predictor for adverse outcomes in febrile neutropenia associated with haematological malignancies.

Polyarticular tuberculosis missed diagnosed as juvenile idiopathic arthritis

We report a child with multiple joint swellings, being treated as a case of poly-articular juvenile idiopathic arthritis subsequently turned out to be tubercular origin. Development of overlying sinuses, lytic lesions on radiographs, synovial fluid analysis showing tubercular infection and demonstration of epitheloid granulomas on bone biopsy confirmed the diagnosis of tubercular poly-arthritis with dactylitis. Child was asymptomatic after two months of starting antitubercular treatment. The case highlights the need to consider the possibility of tuberculosis as aetiology of poly-arthritis, at least in endemic areas, even in the absence of a tuberculosis contact history and overt pulmonary symptoms.

Ghosal Syndrome – Ten Years Follow-up

To the Editor: A one and half year old boy had progressive weakness and bowing of lower extremities since childhood. Examination revealed moderate pallor, bowing of lower limbs and firm mild splenomegaly without bleeding manifestation, hepatomegaly or lymphadenopathy. Normocytic normochromic anaemia, leucopenia and thrombocytopenia were detected. Radiological imaging of long bones and skull base showed thickening of diaphysis and thickening of skull base, suggestive of diaphyseal dysplasia. Bone marrow study revealed sclerosis of cancellous bone and paucity of other elements of marrow with increased reticulin fibres. Based on these, this case was diagnosed as Ghosal type of hemato-diaphyseal dysplasia [1]. Initially the child was managed by blood transfusion and put on tablet prednisolone with iron and folic acid supplementation. During the follow-up period over last 10 y the child required blood transfusion on multiple occasions. We have tried to titrate fall in haemoglobin with prednisolone as far as practicable in absence of any standard, management protocol for this disorder [2–5]. Initially we started with 1.5 mg/kg of prednisolone when the hemoglobin level was 8 g/dl, then Hb% got stabilised at 10 g/dl, but optimum dose of prednisolone was titrated at 0.75 mg/kg. The child at 11 y now, had a normal weight, 24 kg (81 % of expected) and height, 125 cm (91 % of expected). The neuromuscular weakness, bone pain and bowing of legs were passive. Radiological imaging of long bones showed improvement, with straightening and thinning of dysplastic diaphysis to near normalcy (Fig. 1). We communicate the clinical course and management of 10 y follow up of this rare case of ‘Ghosal syndrome’ to help study the disease and formulate better treatment protocol.

Type II vitamin D-dependent rickets with diabetic ketoacidosis

Abstract The high prevalence of vitamin D deficiency in relation to diabetes mellitus is well reported in the literature. However, type I diabetes mellitus (T1DM) in association with resistant rickets is extremely rare and reported in only one previous case. The authors describe here a case of type II vitamin D-dependent rickets (VDDR type II) in a 10-year-old Indian girl who presented with diabetic ketoacidosis (DKA). DKA as a presenting manifestation of T1DM in a patient with VDDR type II has never been reported before in worldwide literature.

Prospective study of neonatal birth trauma: Indian perspective

Background: Birth injuries are mechanical traumas which occur during the process of labor and delivery. The reported incidence varies widely, and various predisposing maternal, neonatal, and labor-related risk factors are documented. However, prospective analysis of epidemiological factors from India is limited. Objective: To delineate the incidence, risk factor, and outcome of birth injuries in our tertiary level teaching and referral hospital. Design: Prospective observational study. Setting: Neonatology Divisions of Department of Pediatric Medicine of our institute. Participants: All inborn babies from singleton pregnancies over an 8 months period. Methods: All newborns were screened for birth trauma after birth. Maternal medical records and labor details were scrutinized. Babies with birth trauma were followed-up for 6 months to assess outcome. Following descriptive analysis, data on risk factors were analyzed in a case-control design. Outcome: Spectrum of birth trauma and its correlation with risk factors. Results: Seventy-three events were recorded from 4741 live births giving an incidence of 15.4/1000 (95% confidence interval 11.9-18.9). Majority were soft tissue injury (0.59%) followed by scalp and skull injury (0.51%). Higher maternal age, shorter height, higher birth weight, instrumental delivery, malpresentations, prolonged labor, obstructed labor, and delivery during risk hours were identified as significant risk factors though the level of risk varied-prolonged labor and instrumental delivery were the strongest. Only two neonatal deaths were attributable to birth trauma. Conclusion: The risk factors for neonatal birth trauma are to be identified and to be addressed for better neonatal outcome.

Blood pressure variation with gestational age and birth weight in Indian newborn

Prospective observational study was conducted in a tertiary care hospital of India over 8 months to measure blood pressure (BP) in healthy term and preterm neonates using oscillometric method and explore the associations with gestational age and birth weight. Consecutive BP measurements were taken by standard oscillometric method on 1617 neonates on day 4, 7 and 14 of life. Mean birth weight was 2.7 ± 0.46 kg, and mean gestational age was 38.2 ± 2.12 weeks. The mean arterial pressure (MAP) on day 4, 7 and 14 were 59.3 ± 7.33, 63.2 ± 6.55 and 66.4 ± 6.13 mmHg, respectively. Larger and mature newborns had significantly higher BP than those who were smaller and premature. Birth weight more strongly correlated with MAP than gestational age. Predictive equations linking MAP with gestational age and birth weight were deduced, which can be used for judicious fluid inotrope management.