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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. CASE PRESENTATION: We report a case of a female neonate born at 28 weeks of gestation with severe respiratory distress and diffuse pulmonary opacification on the right concerning for a large congenital lung lesion. This lesion was not detected on routine antenatal imaging, and she did not have clinical findings of associated hydrops. Her respiratory status improved dramatically after surgical resection of a mass at 12 day of age. The mass was consistent pathologically with a Stocker Type III CPAM. Lung expansion showed subsequent improvement at 16 months of age. CONCLUSIONS: Our case describes a preterm neonate with severe respiratory distress that was found postnatally to have a large, unilateral congenital lung lesion despite a normal prenatal ultrasound. Additionally, this lesion required excision early in life due to severity of respiratory compromise. This case highlights that rare congenital lung lesions, like this rare sub-type of CPAM, should remain a diagnostic consideration in neonates with severe respiratory distress. Early lung resection for CPAM in preterm infants is not well described and the favorable outcomes of this case help expand perspectives on potential management strategies.

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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. Transurethral resection of the bladder mass was performed. Based on the histological findings of the tissue resected, a diagnosis of capillary hemangioma of the bladder was made. Despite their rarity, bladder hemangiomas should be considered in the differential in children with gross hematuria.

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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.

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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. Her medical history was remarkable for secondary progressive multiple sclerosis (SPMS). She had received her first induction dose of alemtuzumab 1 year prior to presentation. Upon evaluation, she had scanning speech, multidirectional nystagmus, and mild edema. Her serum creatinine was 2 mg/dL. Urine studies revealed proteinuria and microscopic hematuria. Her serologic tests were positive for c-antineutrophil cytoplasmic antibodies (> 1 : 640). In addition, she was found to have new-onset severe thyroid dysfunction with antibodies against thyroglobulin and thyroid peroxidase. Kidney biopsy was diagnostic for pauci-immune crescentic GN. The patient was treated with methylprednisolone and rituximab with subsequent renal, thyroid, and neurological recovery. CONCLUSION: This is an atypical case of GN following therapy with alemtuzumab. We hypothesize that immune reconstitution may be a potential mechanism. Alemtuzumab is a new treatment for SPMS that can be associated with GN. Practice guidelines should address the management of its renal complications.

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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. To our knowledge, this is the first report in the medical literature of a dual lesion containing both an osteochondroma and nonossifying fibroma.

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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. Herein, we report the first case of EMH in allograft kidney in a patient with myelofibrosis. The clinical and pathological findings are described. Through comparison of the medullary neoplastic infiltrate with the renal allograft infiltrate, we postulate the neoplastic nature of the infiltrate in the allograft kidney.

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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. The tumor cell nuclei were centrally placed and featured finely clumped chromatin and moderate anisonucleosis. Occasional mitotic figures were present. No necrosis was seen. An immunohistochemical staining panel showed no label for keratin, epithelial membrane antigen, HMB-45, or Melan-A; there was a strong label of tumor cells with chromogranin A, synaptophysin, and neuron-specific enolase. The S-100 protein label was strongly positive in the surrounding stromal cells and weakly positive in the polygonal tumor cells. Given the classic histology and the immunohistochemical staining profile, the diagnosis of paraganglioma was made. At 7 months postoperatively, the patient exhibited no evidence of recurrence or metastasis. To the best of our knowledge, no case of a paraganglioma of the palatine tonsil has been previously described in the literature.

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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. The patient remained dialysis-dependent over 18 months of observation and did not develop extra-renal clinical or biological manifestations of lupus. Mimics of seronegative lupus with isolated renal involvement can include HCV-related autoimmunity. Treatment of acute glomerulonephritis may be similar initially, but other concerns in patients with HCV infection include excluding cryoglobulinemia, the potential impact of immunosuppression therapy on liver disease and consideration of subsequent use of antiviral therapy. Given the increasing prevalence of HCV globally, the recognition of extra-hepatic autoimmune manifestations of HCV infection will likely increase.

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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. In this study, rats were infected intratracheally with viable Y. pestis [CO99 (pCD1( + )/Δpgm) 1 × 10( 8 ) CFU/animal] and treated daily for 3 d with ciprofloxacin (cipro), the A(1)AR antagonist L-97-1, or cipro plus L-97-1 starting at 0, 6, 24, 48, or 72 h post-Y. pestis. At 72 h post-Y. pestis, cipro plus L-97-1 significantly improved 6-d survival to 60-70% vs 28% for cipro plus H(2)O and 33% for untreated Y. pestis controls (P = 0.02, logrank test). Lung edema, hemorrhage and leukocyte infiltration index (LII) were evaluated histologically to produce ALI scores. Cipro plus L-97-1 significantly reduced lung edema, as well as aggregate lung injury scores vs controls or cipro plus H(2)O, and LII vs controls (P < 0.05, Student's unpaired t test). These results support efficacy for L-97-1 as a post-exposure medical countermeasure, adjunctive therapy to antibiotics for Y. pestis.

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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. Pathologic review showed non-necrotizing granulomatous inflammation with granulation tissue. Reports of granulomas of the oral mucosa secondary to trauma are common in the dental literature. This is a unique case in that it is the first report of a subcutaneous granuloma of the eyelid arising secondary to trauma.

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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. Rejection is a major cause of renal allograft failure in children. The morphologic hallmarks of acute antibody-mediated and cell-mediated rejection and chronic allograft nephropathy have been codified in classification strategies that are useful in adults and children. Viral infection and Epstein-Barr virus-driven posttransplant lymphoproliferative disease also occur in the pediatric transplanted kidney. Drug toxicity from immunosuppressive agents also causes characteristic morphologic alterations in the renal allograft. As the survival of pediatric heart and liver transplant patients improves, the incidence of immunosuppression therapy-related disease in the native kidney in these patients will likely become more important clinically. In addition to renal lesions related to the allograft state, glomerular disease can recur or occur de novo in renal allografts. Here, we describe the pathology of the more common morphologic lesions in kidneys of children with a renal allograft.

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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. The blasts of DS-TL typically show light microscopic, ultrastructural, and flow cytometric evidence of megakaryocyte differentiation. DS-TL neonates have a approximately 15% risk of developing potentially fatal liver disease and show <10% incidence of hydrops fetalis. Additional manifestations of DS-TL include cutaneous involvement, hyperviscosity, myelofibrosis, cardiopulmonary failure, splenomegaly, and spleen necrosis. Despite its typical transient nature, 20% to 30% of DS-TL patients develop overt (nontransient) acute leukemia, usually within 3 years and typically of the M7 phenotype (acute megakaryoblastic leukemia). The pathogenesis of DS-TL (and of subsequent acute leukemia) involves mutation of GATA1 (on chromosome X), which normally encodes a transcription factor integral to normal development of erythroid, megakaryocytic, and basophilic/mast cell lines. The pathogenetic role of trisomy 21 in DS-TL is unclear. Though indications for chemotherapy in DS-TL have not been firmly established, the blasts of DS-TL are sensitive to low-dose cytosine arabinoside.

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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. SUBJECTS: Male adolescent patients with ischemic cerebellar stroke after use of marijuana. DIAGNOSTIC INVESTIGATIONS: Computed tomography brain scans (3 subjects), magnetic resonance imaging brain study (1 subject), cerebral arteriography (1 subject), cerebellar biopsy (1 subject), and necropsy (2 subjects). RESULTS: Three adolescent males had similar presentations of headache, fluctuating level of consciousness or lethargy, visual disturbance, and variable ataxia after self-administration of marijuana. They developed primary cerebellar infarctions within days after the exposure that could not be attributed to supratentorial herniation syndromes and only minimally involved brainstem structures. CONCLUSIONS: Episodic marijuana use may represent a risk factor for stroke in childhood, particularly in the posterior circulation. Early recognition of the cerebellar stroke syndrome may allow prompt neurosurgical intervention, reducing morbidity.

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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. Macroscopic, microscopic, and radiographic findings in the case are discussed, and LMPS is reviewed.

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OBJECTIVE: To report a case of severe life-threatening hypersensitivity pneumonitis temporally associated with the use of anagrelide in a patient with myeloproliferative disorder. CASE SUMMARY: A 60-year-old white woman with chronic myeloid leukemia who had been treated with hydroxyurea for 7 years was offered anagrelide to control thrombocytosis. She developed severe hypersensitivity pneumonitis soon after the drug was initiated and required intubation and mechanical ventilation. A high-resolution computed tomography scan of the chest demonstrated extensive multifocal ground glass attenuation and patchy alveolar consolidation involving both lungs. Bronchoalveolar lavage revealed a preponderance of lymphocytes, suggesting hypersensitivity phenomenon, but was otherwise negative for malignancy and other causes of interstitial pneumonitis. An objective causality assessment revealed that an adverse drug event was probable. Discontinuation of anagrelide and hydroxyurea, and institution of corticosteroid therapy resulted in dramatic improvement. DISCUSSION: To our knowledge, this is the first case report of severe hypersensitivity pneumonitis closely related to anagrelide therapy. Pulmonary infiltrates have rarely been noted in patients treated with anagrelide. Anagrelide does not depress white blood cell production, causes mild anemia, and is devoid of the leukemogenic potential characteristic of radioactive phosphorus and other alkylating agents. Common adverse effects to anagrelide include headache, nausea, diarrhea, peripheral edema, and palpitations. Frank congestive heart failure and cardiomyopathy have occurred in a small number of patients, but severe pulmonary adverse effects have not emerged as a frequent problem. CONCLUSIONS: Vigilance is advised in patients who develop dyspnea while taking anagrelide and hydroxyurea. Healthcare providers need to be aware of the possibility of the development of serious life-threatening hypersensitivity pneumonitis. These patients may benefit from serial chest X-rays, pulmonary function testing, and echocardiography.

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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. This is the first report of HPC with trisomy 15.

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