Priyankar Pal

Orange–brown chromonychia, a novel finding in Kawasaki disease

Kawasaki disease (KD) is one of the commonest vasculitis of childhood, where diagnosis is clinical based on a plethora of signs and symptoms. One of the typical findings is the changes in the extremities including the nail changes. Orange–brown chromonychia is a colour change in the nails which has been observed in some cases of KD. Here, we report a series of 40 patients of KD, where a typical transverse orange–brown discolouration of nails or chromonychia was noted in 29 patients. Though chromonychia is noted in many other rheumatic and nonrheumatic diseases, the typical transverse orange–brown chromonychia observed in KD patients can be included as an additional clinical feature in diagnosis of KD.

Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum.

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis) after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS) and spontaneous air leak syndrome (pnemothorax and pneumomediastinum). She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

Infection-associated haemophagocytic lymphohistiocytosis: a case series using steroids only protocol for management.

Haemophagocytic lymphohistiocytosis (HLH) is a heterogeneous group of clinical syndromes characterised by activation and subsequent uncontrolled non-malignant proliferation of T lymphocytes and macrophages, leading to a cytokine storm that accounts for most of its clinical features such as acute febrile illness, hepatosplenomegaly, multi-organ dysfunction and fulminant pancytopenia-resembling severe sepsis. Here, we present a series of 23 cases of infection-associated HLH diagnosed in our hospital within a time period of last three and half years. Though the presentation and progression of disease was variable, the patients shared some common features like prolonged fever unresponsive to broad spectrum antibiotics, organomegaly and cytopenias. In most of the cases, however, the triggering infectious agent could not be identified. They were treated using a steroid only protocol along with supportive measures and showed an excellent response.

The emergence of Kawasaki disease in India and China

Kawasaki disease (KD) is recognized as a leading cause of acquired heart disease in children in developed countries. Although global in distribution, Japan records the highest incidence of KD in the world. Epidemiological reports from the two most populous countries in the world, namely China and India, indicate that KD is now being increasingly recognized. Whether this increased reporting is due to increased ascertainment, or is due to a true increase in incidence, remains a matter of conjecture. The diagnosis and management of KD in developing countries is a challenging proposition. In this review we highlight some of the difficulties faced by physicians in managing children with KD in resource-constrained settings.

Therapeutic plasma exchange in paediatric SLE: a case series from India

Therapeutic plasma exchange (TPE) has been reported to be a useful adjunct in severe systemic lupus erythematosus (SLE) but paediatric literature continues to be scanty. We hereby present three cases of refractory paediatric SLE (pSLE) with thrombotic thrombocytopenic purpura (TTP), diffuse alveolar haemorrhage (DAH) and crescentic glomerulonephritis which were treated with TPE as an adjunctive therapy. TPE was carried out in haemodialysis units using the membrane filtration technique. Demonstrable benefit of TPE was seen in all three cases. In refractory pSLE, TPE may be a useful tool and should be considered. The report additionally highlights the feasibility of undertaking TPE in haemodialysis units, which is important as haemodialysis units are more readily available than dedicated apheresis units in developing countries.

Two cases of non-cystic fibrosis (CF) bronchiectasis with allergic bronchopulmonary aspergillosis

Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction in patients with asthma or cystic fibrosis (CF), which is associated with bronchi colonized by the fungus Aspergillus species, most often Aspergillus fumigatus. ABPA is an important consideration for asthmatics that do not respond to asthma management or with recurrent chest infections and deteriorating lung function in children with cystic fibrosis. We present two cases of non CF bronchiectasis associated with ABPA who presented to our hospital with recurrent hospitalisations of undiagnosed aetiology.

Purpura fulminans due to acquired protein C deficiency

Purpura fulminans (PF) may be the presenting symptom in a patient with protein C (PC) deficiency. It is a hematological emergency and presents with extensive areas of hemorrhagic necrosis of the skin. PC deficiency is usually genetically inherited. However, we report a 1 year and 4 months boy, who presented with acquired PC deficiency possibly of postinfectious etiology and developed PF.

Overlap Syndrome: A Child Cohort

Sir, Overlap syndrome is defined by the presence of two or more rheumatic diseases either on the basis of clinical involvement and/or presence of autoantibodies [1]. These disorders are ill defined and difficult to categorize. The most defined entity amongst them is mixed connective tissue disease (MCTD). The studies of child cohort with overlap syndrome are lacking in literature. A prospective observational study was done at the pediatric rheumatology clinic over a period of 8 y (2004–2011) to detect the incidence and delineate the pattern of overlap syndrome. ACR criteria were used for diagnosis of all rheumatological cases. Overlap was diagnosed, either at presentation or at follow up, by clinical features of two major rheumatic diseases along with autoantibodies or positive biopsy findings as applicable. Out of the total 1,544 children with rheumatological diseases, 6 had overlap syndrome (Table 1). Four had overlap at presentation while two developed it 6–7 mo later. The overall incidence was 3.89 per 1,000 (95 % CI 0.03–7.74 per 1,000) with female preponderance (F: M0 5:1). MCTD was seen both in boy and girl, one each. Out of 210 Juvenile Idiopathic Arthritis (JIA) patients, 2 developed overlap syndrome with features of cutaneous scleroderma {2/210 approx (1 %), M: F01:1[2]. Two girls out of 44 childhood lupus developed overlap. One had juvenile dermatomyositis whereas other had cutaneous scleroderma {2/44 (4.2 %)} [3]. Both patients had renal involvement. Incidence of overlap in adults was reported to be 25 % from UK and 20 % involving mixed population from Poland [4, 5]. Our study, first of its kind from India, demonstrated much lower incidence in children. Study of overlap syndrome in children regarding diagnostic criteria, autoantibody profile and prospective cohort is not documented in literature except sporadic case reports [1]. So the present study of child cohort with overlap syndromes may give some insight till the larger series in child population is available. Pediatricians must be careful and vigilant in searching overlap in rheumatological cases either at presentation or at follow up. R. Mondal : I. Banerjee Pediatric Rheumatology Clinic, Department of Pediatric Medicine, North Bengal Medical College, Darjeeling, West Bengal, India

Congenital tuberculosis: a case series

Introduction In India, despite the high prevalence of tuberculosis (TB) in women of childbearing age, congenital TB is rarely reported due to clinical variability, diagnostic dilemma and 50% mortality rate. Approximately 30 cases have been reported from India till 2014. Unfortunately, most were diagnosed post-mortem . We present a 3 cases to create awareness among clinicians that timely diagnosis can save little lives.

Brucella causing liver abscess in a child with selective IgA deficiency

BackgroundBrucella has been known to cause pyrexia of unknown origin.Case Characteristics9-year-old boy with fever and abdominal pain; multiple abscesses within the liver on ultrasonography.ObservationsIgM Antibodies against Brucella were raised in his serum sample, and Brucella serum agglutination test was positive. Immunological work-up suggested selective IgA deficiency. Reduction in size following treatment with trimethoprim-sulphamethoxazole, amikacin and doxycycline.MessageBrucellosis should be considered as an etiology of liver abscess in patients not responding to conventional antibiotics.