ABSTRACT
Bronchogenic cysts are the most common cystic mediastinal lesion in children. Bronchogenic cyst causing unilateral obstructive emphysema is a rare presentation. We report the case of a one and half month old infant who presented with respiratory distress which was initially suspected as left pneumothorax on frontal chest radiograph but was later found to be due to hyperinflated left lung and hence the possibility of congenital lobar emphysema was considered. CT thorax and limited MRI sections revealed a cystic lesion in mediastinum causing obstructive emphysema and mediastinal displacement. He underwent an emergency thoracotomy and excision of the cyst via an extrapleural approach. Post operatively, rapid improvement of the infant was noticed both clinically as well as radiologically. Cross sectional imaging like CT or MR is required for reaching the correct and early diagnosis in paediatric patients with respiratory distress, when there is diagnostic dilemma based on chest radiograph.
Subject(s)
Calcinosis/drug therapy , Dermatomyositis/drug therapy , Diphosphonates/administration & dosage , Skin Diseases/drug therapy , Calcinosis/complications , Calcinosis/diagnosis , Child , Dermatomyositis/complications , Dermatomyositis/diagnosis , Dose-Response Relationship, Drug , Drug Administration Schedule , Female , Follow-Up Studies , Humans , Pamidronate , Severity of Illness Index , Skin Diseases/complications , Skin Diseases/diagnosis , Time Factors , Treatment OutcomeABSTRACT
Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) very rarely present simultaneously and pose a diagnostic and therapeutic dilemma to the physician. Prompt diagnosis and management with plasma exchange and immunosuppression is life-saving. To describe the effectiveness of cryosupernatant and steroids in pediatric SLE with TTP. We describe three children aged 12-14 years with SLE who were diagnosed with TTP based on fever, CNS manifestations, ANA, anti-dsDNA, anti-sm positivity, hypocomplementemia, and microangiopathic anemia with thrombocytopenia. All three children were managed with cryosupernatant and steroids without plasmapheresis. All children improved with cryosupernatant and steroids. All attained remission within 10 days. They were doing well at last follow up without relapse or flare. Cryosupernatant and steroids may be an effective therapy for Thrombotic thrombocytopenic purpura with systemic lupus erythematosus.
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by high fever, maculopapular rash, neurological symptoms, abnormal liver functions and coagulopathy. Primary HLH is due to an underlying genetic abnormality. Secondary HLH are due to an underlying infection, autoimmune disease or malignancy. Secondary HLH due to viral infections are commonly due to the herpes group commonest of which is the Ebstein Barr virus (EBV). We describe two children with virus associated hemophagocytic lymphohistiocytosis (VAHLH) secondary to hepatitis A infection.
ABSTRACT
Systemic dissemination of intracranial germ cell tumors (GCTs) occur only in 3% of cases and the common sites are bone, lungs, and lymph nodes. Metastasis to the liver is rare. As far as we could find, only six cases of liver metastasis of intracranial GCTs have been reported so far. We report an adolescent girl who presented with hepatic relapse 2½ years after successful completion of treatment of intracranial GCT. She was treated with chemotherapy and right hepatectomy and is doing well 30 months after treatment for the metastatic disease.
