Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review.

Patients with systemic onset juvenile idiopathic arthritis (SoJIA) are rarely known to develop coronary artery dilatation. The American heart association (AHA) statement on evaluation of suspected Kawasaki disease (KD) would lead some SoJIA patients (particularly in the early stages of the disease) to be inaccurately classified as KD. In addition to the institution of inappropriate therapy with IVIG, misdiagnosis as KD can delay definitive treatment for these SoJIA patients who probably have a worse predicted outcome. We present a 6-year-old male patient with SoJIA who was initially classified as incomplete KD. The child developed life-threatening macrophage activation syndrome (MAS). Previous literature regarding coronary dilatation in SoJIA is also reviewed.

Perinatal mortality--a hospital based study.

A total of 5082 consecutive deliveries in Medical College Hospital, Kottayam during the period August 1992 to July 1993 constituted the study. Perinatal mortality rate (PMR) was 38.5 per 1000 total births and autopsy rate was 72%. More than 85% of perinatal deaths occured in low birth weight and preterm babies. Perinatal hypoxia and congenital anomalies were the leading causes in late fetal deaths (LFD). The main causes of early neonatal deaths (END) were perinatal hypoxia, infections, congenital anomalies and hyaline membrane disease (HMD).

Multiple amoebic abscesses with erythema nodosum.

Amoebiasis, a common parasitic infection in the tropics is most commonly associated with solitary liver abscess. Multiple hepatic, splenic and renal abscesses are a very rare presentation of extraintestinal amoebiasis in children. The authors report a 6-y-old girl who presented with a febrile illness, hepatosplenomegaly and erythema nodosum and was diagnosed to have multiple amoebic abscesses by imaging and aspiration cytology of a liver abscess. This is also the first case report of the association of erythema nodosum with extraintestinal amoebiasis in children.

Posterior reversible encephalopathy syndrome (PRES/RPLS) during pulse steroid therapy in macrophage activation syndrome.

Posterior reversible encephalopathy syndrome (PRES)or Reversible posterior leukoencephalopathy syndrome (RPLS) is a neurological complication associated with various illnesses and medications(including rheumatological illnesses and their medications). Cyclosporine is the drug which is most commonly implicated in the causation of this condition. The authors report a 6 year old patient with systemic onset juvenile idiopathic arthritis (SoJIA) with macrophage activation syndrome who developed PRES during treatment with pulse methylprednsiolone therapy. In view of persitent SoJIA disease activity, the child was subsequently treated with Cyclosporine. The child made a complete neurological recovery. This case report highlights that, in accordance with recent literature, PRES is a complication of a unique set of conditions comprising of inflammation, hypertension, immunosupression and nephropathy in different degrees and does not necessarily preclude the use of drugs implicated as inciting agents. This has important implications in rheumatology where cyclosporine is a life saving medication used in macrophage activation syndrome.

Cardiogenic shock with hypereosinophilic syndrome.

We report a child with hypereosinophilic syndrome who presented with cardiogenic shock. In addition, she had skin and joint involvement. The clinical condition improved and eosinophil counts normalized with steroid therapy. However, the skin lesions and hypereosinophilia relapsed on stopping the steroids. The child was subsequently maintained in remission on low dose prednisolone.

Reactive hemophagocytic syndrome.

We describe two cases of Reactive Hemophagocytic syndrome (RHS) occurring in rheumatic diseases in childhood. Patient 1, an adolescent girl with systemic onset Juvenile idopathic arthritis (JRA) presented like severe sepsis with shock, hepatic dysfunction and coagulopathy. Patient 2 presented with cardiac tamponade, she was later detected to have systemic lupus erythematosus (SLE). Her bone marrow aspirate revealed prominent hemophagocytosis. Both cases improved with pulse methylprednisolone therapy.

Rabies encephalitis.

A 12-year-old boy presented with fever and sore throat of 6 days duration followed by vomiting and altered sensorium. He had received 4 doses of antirabies vaccine following a dog bite 4 weeks back. Rabies immunoglobulin was not given. History of hydrophobia and aerophobia were strikingly absent. The possibilities of rabies encephalitis and vaccine induced acute disseminated encephalomyelitis (ADEM) were considered. MRI brain showed exclusive grey matter changes characteristic of rabies. The diagnosis was further confirmed by serological tests.