Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature.

Background: Congenital pulmonary airway malformations (CPAMs) are a heterogenous collection of congenital lung malformations, often diagnosed prenatally. The Stocker Type III CPAM is a rare CPAM sub-type, and, when large, may be associated with hydrops. Furthermore, reports of CPAM management which may include surgical resection in extreme preterm infants are limited.

Capillary hemangioma of the bladder in a pediatric patient.

We report a case of a bladder hemangioma in a pediatric patient. A 2-year-old Caucasian female presented with intermittent gross hematuria and protrusion of beefy red tissue near the vaginal introitus when straining. On cystoscopy, we discovered a wide-based vermiform mass.

Nephrotic range proteinuria associated with focal segmental glomerulosclerosis reversed with pioglitazone therapy in a patient with Dunnigan type lipodystrophy.

Familial partiallipodystrophy (FPLD)is a rare disorder associated withsevere insulin resistance, hypertriglyceridemia, lowserumHDLcholesterol and proteinuricrenaldisease. Although proteinuric renal disease is not common among in patients with partial lipodystrophy, we report a patient with Dunnigan type FPLD complicated by nephrotic syndrome which resolved following treatment with thePPARγagonist pioglitazone, CPAP, diet, and exercise.

Immune reconstitution, glomerulonephritis, and successful treatment with rituximab.

Background: Alemtuzumab can induce secondary autoimmunity affecting multiple organs. While kidney involvement is uncommon, it can be associated with devastating forms of glomerulonephritis (GN).

Case Presentation: A 32-year-old African American woman presented with hypertension, proteinuria, and progressive renal failure.

Rare case of dual lesion: nonossifying fibroma and osteochondroma.

In this paper, we offer radiographic and pathologic evidence of a unique coexisting dual lesion. A 14-year-old boy presented for evaluation of a painful right knee after sustaining a twisting injury. The patient was found to have a torn anterior cruciate ligament, in addition to incidental finding of distal femoral dual lesion composed of tissue consistent with a nonossifying fibroma and an osteochondroma.

Extramedullary hematopoiesis in renal allograft.

Extramedullary hematopoiesis (EMH), defined as the presence of hematopoietic elements outside of the medullary cavity of bone, has been reported in patients with various hematopoietic neoplasms including myelofibrosis. EMH commonly occurs in the liver and spleen (resulting in hepatosplenomegaly) and uncommonly involves the kidney. EMH involving the allograft kidney has not been reported in English literature.

Paraganglioma of the palatine tonsil.

Paragangliomas of the head and neck are rare. We describe the case of an 11-year-old girl who presented with an enlarged right palatine tonsil (grade 4). After a bilateral tonsillectomy, microscopic examination of the right tonsillar tissue revealed well-formed nests of polygonal epithelial cells separated by a collagenous stroma.

Lupus-like glomerulonephritis: an autoimmune complication of hepatitis C infection.

Lupus-like glomerulnephritis in patients with negative lupus serologies and no extra-renal manifestations of lupus can create a diagnostic dilemma. We describe a 53-year-old gentleman with chronic hepatitis C virus (HCV) infection who presented with dialysis-requiring renal failure, renal histologic findings of "full-house" immunofluorescence label and tubuloreticular inclusions on electronic microscopy, but no extra-renal or laboratory signs of systemic lupus erythematosis. Attempted treatment with cyclophosphamide and corticosteroids was limited by cyclophosphamide hypersensitivity.

A unique association of granuloma of the lower eyelid to prior trauma.

A 5-year-old girl presented with a mass of the lower eyelid causing lower eyelid retraction and traction in upgaze. The patient had a recent history of minor trauma to the area. An orbitotomy was performed with lateral canthotomy and cantholysis to allow for dissection of the lesion, which was found in the anterior lamella of the lower eyelid.

A novel post-exposure medical countermeasure L-97-1 improves survival and acute lung injury following intratracheal infection with Yersinia pestis.

Yersinia pestis, a Gram-negative bacillus causing plague and Centers for Disease Control and Prevention (CDC) classified Category A pathogen, has high potential as a bioweapon. Lipopolysaccharide, a virulence factor for Y. pestis, binds to and activates A(1) adenosine receptor (AR)s and, in animals, A(1)AR antagonists block induced acute lung injury (ALI) and increase survival following cecal ligation and perforation.

Renal pathology in the pediatric transplant patient.

Renal transplantation is a therapeutic goal for children with advanced chronic kidney disease. There are many causes of renal dysfunction in children with allografts--the transplanted kidney can develop a variety of morphologic alterations leading to dysfunction. Evaluation of the kidney biopsy is one of the best methods of determining the cause of graft dysfunction.

Transient leukemia (transient myeloproliferative disorder, transient abnormal myelopoiesis) of Down syndrome.

Transient leukemia of Down syndrome (DS-TL), also known as transient myeloproliferative disorder of Down syndrome (DS) and transient abnormal myelopoiesis of DS, occurs in approximately 10% of DS neonates and in phenotypically normal neonates with trisomy 21 mosaicism. In DS-TL, peripheral blood analysis shows variable numbers of blasts and, usually, thrombocytopenia; other cytopenias are uncommon. Bone marrow characteristics of DS-TL are, likewise, variable, though (in contrast to other leukemias) the bone marrow blast differential can be lower than the peripheral blood blast differential.

Cerebellar infarction in adolescent males associated with acute marijuana use.

Objective: To demonstrate the clinical characteristics, radiologic findings, and neuropathological features of tetrahydrocannabinol-related posterior fossa ischemic stroke in adolescent patients.

Design: A retrospective case and chart review of 3 cases encountered at a tertiary care institution over a span of 5 years.

Setting: Inpatient and intensive care hospitalization units managing children and adolescents.

Case report: lethal multiple pterygium syndrome.

We report a case of lethal multiple pterygium syndrome (LMPS). Intrauterine fetal demise was diagnosed at 31 weeks' estimated gestational age. Subsequent fetopsy established the diagnosis of LMPS.

Trisomy 15 in a case of pediatric hemangiopericytoma and review of the literature.

This study reports on a pediatric case of hemangiopericytoma (HPC) showing trisomy 15 as a sole anomaly. Trisomy 15 was observed in a total of 11 cells harvested at a very early passage from two different in-situ cultures. Trisomy 15, as a sole anomaly, has been described in hematologic disorders such as myelodysplastic syndromes but, to our knowledge, has never been documented in solid tumors.