Immune Thrombocytopenic Purpura: Historical Perspective, Current Status, Recent Advances and Future Directions.

Immune thrombocytopenic purpura (ITP) has witnessed many changes and updates over the past decade. The definitions of disease subtypes, course and response to treatment have all been standardized recently. Consequent to the lack of an international consensus management guideline, wide variations exist in treatment practice. This is now being addressed to an extent by the much awaited ITP International Working Group 2010 recommendations. The pathophysiologic mechanisms have been unfolded at cellular, molecular and humoral levels. As a result, many recent advances have taken place in the management of this disorder. This review revisits the history of evolution of ITP, summarizes the current recommendations for management and lists the recent advances and future prospects in this field.

Primary lymph node gastrinoma in jejunal mesentery.

Primary gastrinomas have been reported in lymph nodes within the gastrinoma triangle. We report a 56-year-old woman with possible primary lymph node gastrinoma in the jejunal mesentery. Six months after excision of the tumor, she is asymptomatic and serum gastrin level is normal.

Benefits of maternal participation in newborn nurseries.

OBJECTIVE: To evaluate the outcome of active involvement of mothers/mother substitutes in day-to-day care of high risk neonates admitted in a level II newborn care unit. METHODS: An observational study was carried out over a period of eleven years incorporating active participation of mothers/substitute in the day to day care of their sick neonates. The outcome is assessed in terms of mortality due to the three major illnesses (asphyxia, sepsis and prematurity) during this phase. The data is compared with that of a similar level II care centre where conventional neonatal care is practised. RESULTS: There is a significant and sustainable reduction in neonatal mortality due to the three major illnesses when the mothers are also involved in the neonatal care, in spite of a considerable increase in the number of admissions during this period. CONCLUSION: The concept of active participation of mother/substitute in neonatal nursery ensures 1:1 care at all times. It is a cheap and effective alternative to inadequacy of bed:nurse ratio (BNR).

Hemihypertrophy and primary small intestinal lymphangiectasia in incontinentia pigmenti achromians.

A rare occurrence of primary small intestinal lymphangiectasia in an 8-year-old girl with incontinentia pigmenti achromians is reported. In addition, she had right sided hemihypertrophy. Though intestinal lymphangiectasia is known to have a few syndromic associations, its co-existence in a child with incontinentia pigmenti achromians has not yet been reported in the literature. Hemihypertrophy is also extremely rare in the latter and only very few instances of an association between the two have been documented previously.

A dancer with an unusual cause of neck pain.

A girl presented with a dull ache in the neck and mild difficulty in neck movements. She had limited clinical signs and her initial work up failed to reveal the cause. With the help of imaging modalities and CT guided needle biopsy, she was diagnosed to have an eosinophilic granuloma of the fifth cervical vertebra. There were no neurological symptoms. She was successfully managed with immobilization of spine, local irradiation and systemic vinblastine. The literature is briefly reviewed for clinical features, diagnosis and management.

Unilateral internal jugular phlebectasia.

Jugular phlebectasia is a rare cause of cervical swelling in children. It is a fusiform dilatation of any part of the jugular venous system and can involve the external, internal or anterior jugular veins. Previous reports suggest that the entity is often ignored or misdiagnosed. Unilateral internal jugular phlebactasia presenting as an intermittent neck swelling in a ten-year-old girl is reported. The clinical features are analyzed and the appropriate use of noninvasive imaging modalities is highlighted. The literature is also briefly reviewed.

Wilm's tumor in a solitary kidney complicated by chemotherapy induced obstructive uropathy.

A three-year-old male child with Wilm's tumor of left kidney and right sided unilateral renal agenesis is reported. The left renal vein was located posterior to the aorta. He was managed with medical measures alone. The initial phase of treatment was complicated by chemotherapy induced dislodgment of the tumor fragment and subsequent distal obstruction.

Spectrum of clinical presentations of vein of galen aneurysm.

Aneurysm of the vein of Galen is a rare intracranial vascular malformation. It is known to have diverse manifestations and varying severity. Four cases with different modes of presentation and outcome are reported. A mortality of 50 per cent was encountered. Among the survivors, one had neurologic sequelae whereas the other had attained age-appropriate developmental milestones. The former was a rare case of spontaneous thrombosis of the aneurysm while the latter was a boy who underwent therapeutic embolization.

Fulminant citrobacter meningitis with multiple periventricular abscesses in a three-month-old infant

Citrobacter, a Gram-negative enteric bacillus, is a rare cause of septicemia and meningitis, seldom reported beyond the neonatal period. It is characterized by a fulminant clinical course and a high incidence of complications, including brain abscesses. We studied a three-month-old infant with Citrobacter meningitis, who developed acute communicating hydrocephalus and multiple periventricular brain abscesses while on treatment. The patient died, despite intensive antibiotic treatment directed towards the causative organism, C. diversus.

Congenital varicella syndrome.

The fetal consequences of chickenpox complicating pregnancy depends on the period of gestation at which the infection is contracted. The extremely rare classical form of congenital varicella syndrome, resulting from maternal varicella infection in the first trimester of pregnancy, is being reported here. The unusual features in this baby are bilateral hypoplastic lower extremities, fracture of bones, a normal electroencephalogram and phantom hernias of the anterior abdominal wall.

Baller-Gerold syndrome.

Craniosynostosis is known to be associated with a large number of inherited disorders of childhood. Its presence along with absent thumbs and ectopic anus is the characteristic feature of one such extremely rare disorder, described as the Baller Gerold syndrome. The typical features are being reported here.

Anencephaly with placental attachment.

Anencephaly is a severe defect of development of the neuraxis that is incompatible with survival. This particular neural tube defect is characterised by the absence of large portions of the cranium. The peculiar feature in this baby is the abnormal attachment of the placenta to the site of the skull defect.