Subject(s)
Adamantinoma/diagnosis , Biomarkers, Tumor/analysis , Bone Neoplasms/diagnosis , Carcinoma/diagnosis , Diagnostic Errors , Femoral Neoplasms/diagnosis , Lung Neoplasms/diagnosis , Nuclear Proteins/analysis , Oncogene Proteins/analysis , Tibia/pathology , Adamantinoma/chemistry , Adamantinoma/genetics , Adamantinoma/pathology , Adolescent , Biomarkers, Tumor/genetics , Biopsy , Bone Neoplasms/chemistry , Bone Neoplasms/genetics , Bone Neoplasms/secondary , Bone Neoplasms/therapy , Carcinoma/chemistry , Carcinoma/genetics , Carcinoma/secondary , Carcinoma/therapy , Disease Progression , Fatal Outcome , Female , Femoral Neoplasms/chemistry , Femoral Neoplasms/genetics , Femoral Neoplasms/secondary , Femoral Neoplasms/therapy , Humans , Immunohistochemistry , Lung Neoplasms/chemistry , Lung Neoplasms/genetics , Lung Neoplasms/pathology , Lung Neoplasms/therapy , Lymphatic Metastasis , Neoplasm Proteins , Nuclear Proteins/genetics , Oncogene Proteins/genetics , Predictive Value of Tests , Tibia/chemistry , Tomography, X-Ray Computed , Treatment Failure , Whole Body ImagingSubject(s)
Blast Injuries/complications , Foreign-Body Migration/surgery , Lung Injury/etiology , Lung Injury/surgery , Adolescent , Blast Injuries/diagnosis , Chronic Disease , Cough/etiology , Cough/physiopathology , Female , Follow-Up Studies , Foreign-Body Migration/diagnostic imaging , Foreign-Body Migration/etiology , Humans , Lung Injury/diagnostic imaging , Risk Assessment , Stress Disorders, Post-Traumatic/diagnosis , Stress Disorders, Post-Traumatic/therapy , Thoracotomy/methods , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Familial hemophagocytic lymphohistiocytosis is a very unusual cause for demyelination and the clinician would do well to be aware of the condition, especially when children present with atypical findings on magnetic resonance imaging associated with fever, pancytopenia, and hepatosplenomegaly. This is a rare autosomal recessive, multisystem inflammatory disorder characterized by widespread organ infiltration by macrophages and activated lymphocytes. It is usually diagnosed in the first 2 years of life and is rapidly fatal if untreated. Reported here is the case of a 12-year-old boy, from a poor family, with a 6-year history of visual loss and fever for 5 months, and transient hemiparesis with hepatosplenomegaly and pancytopenia. Cranial magnetic resonance imaging showed multiple areas of hyperintense signal, predominantly involving white matter. The boy also had elevated triglycerides and ferritin, with low fibrinogen level. Bone marrow aspiration revealed hemophagocytosis. He was diagnosed as having familial hemophagocytic lymphohistiocytosis and treated with the HLH 2004 protocol. A sibling also had evidence of hemophagocytosis. Remission was achieved, but his parents could not afford the cost of hematopoietic stem cell transplantation. He relapsed after 8 months and later died.
Subject(s)
Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/physiopathology , Aspartic Acid/analogs & derivatives , Aspartic Acid/metabolism , Bone Marrow/physiopathology , Brain/metabolism , Brain/pathology , Child , Choline/metabolism , Diagnosis, Differential , Disease Progression , Fatal Outcome , Female , Ferritins/blood , Fibrinogen/metabolism , Humans , Lactic Acid/metabolism , Lymphohistiocytosis, Hemophagocytic/drug therapy , Magnetic Resonance Imaging , Male , Remission Induction , Siblings , Triglycerides/bloodABSTRACT
It is exceptionally rare for acute pancreatitis to be the presenting manifestation of childhood systemic lupus erythematosus. We report a 14-year-old girl who presented with a history of fever, generalized rash, arthralgia and abdominal pain. Her serum amylase was 1472U/L and lipase 3316 U/L suggestive of acute pancreatitis. Other investigations revealed pancytopenia, low complement, high 24-hour urinary protein and elevated ANA and dsDNA. She was treated with IV methylprednisone, followed by oral steroids.
Subject(s)
Lupus Erythematosus, Systemic/complications , Pancreatitis/etiology , Acute Disease , Administration, Oral , Adolescent , Diagnosis, Differential , Fatal Outcome , Female , Glucocorticoids/administration & dosage , Glucocorticoids/therapeutic use , Humans , Infusions, Intravenous , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/drug therapy , Methylprednisolone/administration & dosage , Methylprednisolone/therapeutic use , Pancreatitis/diagnosis , Pancreatitis/drug therapyABSTRACT
We report a 7-year-old boy with very severe tetanus treated with continuous infusion of magnesium sulphate for the control of spasms and severe autonomic dysfunction which was refractory to deep sedation and mechanical ventilation. The infusion was not associated with any adverse effects and he made an uneventful recovery. We recommend the use of intravenous magnesium sulphate infusion as an inexpensive and highly effective modality in severe tetanus.
