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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Takayasu's arteritis is a chronic inflammatory disease that primarily affects the large vessels, such as the aorta and its branches. It represents the third most frequent vasculitis during pediatric age. Our objective was to describe clinical and complementary exams features as well as treatment modalities of a case series of pediatric patients. We present 11 patients (10 girls) with median age at onset of 8 years (range: 2-15). The median diagnosis delay was 16 months (range: 2-96). Clinical presentations were lower limb claudication, arterial hypertension, CNS involvement, presence of murmurs, systemic symptoms, lymphadenopathy, chest pain, abdominal pain and arthritis. Laboratory tests showed: elevated ESR, anemia and trombocytosis. Vascular imaging studies exhibited stenosis, dilatation, occlussion and aneurysms. The outcome of the disease was persistent active condition (1 patient), relapse (4 patients), remission (3 patients), motor sequelae (1 patient) and death (2 patients). All patients were treated with steroids and immunosuppressants. Takayasu 's arteritis is a condition that can potentially be life-threatening. The diagnosis should be suspected in a variety of clinical manifestations during childhood.
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PMID:[Takayasu arteritis in pediatric patients]. 2276 Jul 53

Mesoblastic nephroma is by far the most frequent intrarenal fetal tumor. To the best of our knowledge, we report the first case of a newborn with an intrarenal neuroblastoma that was discovered prenatally. An intrarenal echogenic and homogenous mass was observed on routine prenatal ultrasonography, corroborated by magnetic resonance imaging, in a 30-week gestation fetus. A male weighing 3280 g was born with elevated blood pressure and cardiac failure. Postnatal ultrasound confirmed a left intrarenal tumor with microcalcifications and perirenal adenopathy. An open total left nephrectomy by laparotomy was performed. The pathologic study reported that the mass was an intrarenal neuroblastoma with local and regional invasion. Immediate postoperative urine analysis revealed a high level of vanillylmandelic acid, and blood samples showed high levels of normetanephrine. The purpose of this report is to demonstrate that prenatal intrarenal neuroblastoma can clinically and radiologically mimick a mesoblastic nephroma. High blood pressure, calcifications, and lymphadenopathy on ultrasound should raise the index of suspicion for a possible malignant process. Preoperative measurement of urinary vanillylmandelic acid (VMA) and metanephrines should be performed if the diagnosis is in doubt.
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PMID:Prenatal intrarenal neuroblastoma mimicking a mesoblastic nephroma: a case report. 2290 38

Southeastern Anatolia and the Mediterranean regions of Turkey are known as endemic areas for cutaneous leishmaniasis (CL). In this report, a 64 years-old male patient with CL caused by Leishmania infantum has been presented. The patient who was inhabiting in Ankara (Central Anatolia region, Turkey) complained from a lesion on his right ring finger for the last six months. He has a cat and has been engaged with gardening. Overall, he was healthy with the exception of hypertension and glucose intolerance. The patient had not left Ankara since the last seven months, however, he had previously been to the Aegean coast during his summer holiday. The examination of the 4th phalanx of his right hand revealed the presence of a 3 x 3 cm erythematous, slightly swollen lesion, at the center of which 1.5 x 1.5 cm ulcerative area covered with a hemorrhagic crust, was detected. Neither axillary or cervical lymphadenopathy, nor hepatosplenomegaly could be observed. The routine examinations, including complete blood count, serum biochemistry, chest X-ray and abdominal ultrasonography were within normal limits. Giemsa stained smears prepared from aspiration of the lesion revealed amastigote-like organisms and leishmania promastigotes were grown in NNN media. PCR amplification of the specimen indicated the presence of a positive DNA band of 420 bp specific for Leishmania spp. The serum sample of the patient revealed positivity for leishmaniasis by the rapid rK39 test and immunofluorescence antibody (IFAT) test. The organism was identified as L.infantum by PCR-RFLP applied to the cultivated organism. The examination of his cat's serum for leishmaniasis by IFAT and PCR, were negative. The exact way of transmission had not been confirmed for the patient. However, when long incubation period of CL was considered, the transmission might probably occurred during his summer stay in the Aegean coast. This case was presented to withdraw attention to a delayed diagnosis of CL which developed in a non-endemic area and which was due to L.infantum instead of the more common L.tropica species.
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PMID:[A case of cutaneous leishmaniasis caused by Leishmania infantum in a non-endemic province (Ankara) of Turkey]. 2295 64

High altitude pulmonary edema (HAPE) is a life-threatening complication of high altitude stay, which may occur above altitudes of 2500 m. This is a case report of a healthy 41-year-old man, presenting with recurrent HAPE at moderate altitude. Medical work-up revealed an idiopathic pulmonary artery hypertension (PAH), and specific vasoactive treatment was started. Despite treatment with an endothelin receptor antagonist, the patient deteriorated clinically. Subsequent medical reevaluation showed a significant progress of mediastinal lymphadenopathy. Due to the histological proof of sarcoidosis, the initial diagnosis of PAH had to be changed to sarcoidosis-related pulmonary hypertension. Initiation of immunosuppressive therapy with corticosteroids led to significant and clinically relevant decrease in pulmonary artery pressure, even allowing episodes of asymptomatic re-exposure to moderate altitude. This case describes HAPE as first manifestation of a sarcoidosis-related pulmonary hypertension with a very unusual and early presentation of the underlying disease in an apparently healthy mountaineer.
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PMID:When "high" is not very high: high altitude pulmonary edema as first manifestation of sarcoidosis-related pulmonary hypertension. 2327 Apr 47

The rate of reporting of childhood Churg-Strauss syndrome (CSS) has increased lately because of either increased awareness to the disease or a real increase in incidence. It is defined as one of the antineutrophil cytoplasmic antibody-associated vasculitides, but the antineutrophil cytoplasmic antibody positivity is less reported in pediatric cases. The cause of CSS remains unknown. Several lines of evidence suggest genetic predisposition, which may entail inherited tendency to dysregulation of the cellular immune system. With the addition of leukotriene receptor antagonists to the treatment regimen of asthma, an association to CSS was presumed. However, the nature of this relationship remains to be elucidated. In addition, some environmental factors seem to provoke transient effects that resemble the disease. Patients' symptoms are defined by various degrees of eosinophilic inflammation and necrotizing vasculitis, which may affect any organ. Three clinical stages have been described in the clinical evolution of CSS: prodromal phase involving allergic rhinitis and asthma (usually without family history of atopy), a second phase that involves peripheral eosinophilia and eosinophilic tissue infiltration, and the hallmark of the final phase is systemic vasculitis. Pulmonary disease is a central feature of pediatric CSS, but other manifestations include skin lesions, testicular pain, hypertension, seizures, and nephropathy. More subtle presentations in children include cervical lymphadenopathy, acute abdominal pain, deep venous thrombosis, oral ulceration, multiple colonic ulcers, chorea, bilateral optic neuropathy, and retinal artery occlusions. Churg-Strauss syndrome patients usually respond well to corticosteroid therapy. Several trials reported additional benefit from cyclophosphamide, azathioprine, and methotrexate, whereas the therapeutic effects of etanercept, plasma exchange, and intravenous immunoglobulin therapy are controversial. The relapse rate is approximately 25% to 30%, but corticosteroids have significantly increased survival, which now approaches greater than 75% at 5 years. However, there is limited information about survival or long-term outcome in childhood.
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PMID:Churg-strauss syndrome in the pediatric age group. 2328 8

Autoimmune lymphoproliferative syndrome (ALPS) is caused by a nonmalignant defective Fas-mediated apoptosis. The main clinical manifestations are chronic lymphadenopathy, splenomegaly, and autoimmune cytopenia. Most patients with ALPS have a FAS germline mutation. ALPS has occasionally been associated with glomerulonephritis and we present the first report of tubulointerstitial nephritis associated with probable ALPS. A 5-year-old girl presented with fever, vomiting, hypertension, and azotemia. No autoantibodies, viral, or streptococcal antibodies were detected. A renal biopsy showed small-vessel vasculitis with normal glomeruli and inflammation in the interstitium. The patient responded to prednisolone treatment and obtained a full renal recovery. Symptoms of connective tissue disorder supervened and after the development of more pronounced splenomegaly, a diagnosis of ALPS was confirmed.
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PMID:Tubulointerstitial nephritis in a patient with probable autoimmune lymphoproliferative syndrome. 2358 39

Case 1: A 72-year-old woman presents with a palpable mass detected during yearly physical examination by her primary care physician. She has controlled hypertension and remains active, playing tennis three times a week. Physical examination reveals a 1.5 cm mass in the upper outer quadrant of the left breast with no palpable axillary lymphadenopathy. Diagnostic imaging reveals a suspicious mass, and core biopsy confirms invasive ductal carcinoma (IDC) that is estrogen receptor moderately positive (60%), progesterone receptor negative and Her2-neu that is not overexpressed. She undergoes a wide local excision and sentinel node biopsy. Pathology reveals a 1.5 cm IDC that is high grade without lymphovascular invasion (LVI). The margins are negative with the closest laterally at 2 mm. One sentinel node is negative for metastasis. Case 2: A 72-year-old woman presents with an abnormal screening mammogram that shows a small area of architectural distortion in the upper outer quadrant of the left breast (Fig 1). She is a former smoker with mild chronic obstructive pulmonary disease and has mild to moderately symptomatic osteoarthritis managed with a nonsteroidal anti-inflammatory agent. She remains active and independent. Physical examination reveals neither palpable breast mass nor axillary lymphadenopathy. Diagnostic ultrasound confirms a 1.8 cm mass, and core biopsy reveals IDC that is estrogen and progesterone receptor strongly positive (> 90%) and Her2-neu that is not overexpressed. She undergoes a wide local excision and sentinel node biopsy. Pathology reveals a 1.9 cm IDC that is low grade. The margins are widely negative at > 5 mm and there is no LVI. One sentinel node is negative for metastasis.
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PMID:Should a woman age 70 to 80 years receive radiation after breast-conserving surgery? 2369 Apr 20

A 60-year-old woman with hypertension, dyslipidemia, and 35-pack-year smoking history is referred for treatment of advanced non-small-cell lung cancer (NSCLC). She initially presented after a transient ischemic attack, when a chest radiograph demonstrated a right lung mass. Computed tomography (CT) of the chest revealed a 5-cm right upper lobe mass, without mediastinal adenopathy, and a 6-cm cystic mass in the spleen. Additional imaging showed no brain metastasis. Endobronchial ulstrasound-guided core biopsies of the lung mass and ipsilateral mediastinal nodes confirmed a poorly differentiated non-small-cell carcinoma. Immunohistochemical stains were positive for napsin A and thyroid transcription factor 1, suggestive of adenocarcinoma (Fig 1). Molecular analysis identified a KRAS G12C mutation. A positron emission tomography (PET) -CT scan demonstrated [(18)F]fluorodeoxyglucose uptake in the right upper lobe mass and splenic lesion (Fig 2A). CT-guided fine-needle aspiration of the splenic lesion was performed and revealed metastatic carcinoma, consistent with the lung primary. Treatment with carboplatin plus pemetrexed was initiated, without bevacizumab because of the recent transient ischemic attack; carboplatin was selected over cisplatin because of similar concerns. The patient received two cycles of chemotherapy without complications, and repeat imaging showed decrease in size of the lung mass and splenic lesion (Figs 2B and 2C). After four cycles of chemotherapy, a chest CT showed ongoing response (Fig 2D). Her Eastern Cooperative Oncology Group performance status remained 0.
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PMID:Maintenance therapy for advanced lung cancer: who, what, and when? 2383 8

Although cancer in children is rare, it is the second most common cause of childhood mortality in developed countries. It often presents with nonspecific symptoms similar to those of benign conditions, leading to delays in the diagnosis and initiation of appropriate treatment. Primary care physicians should have a raised index of suspicion and explore the possibility of cancer in children who have worrisome or persisting signs and symptoms. Red flag signs for leukemia or lymphoma include unexplained and protracted pallor, malaise, fever, anorexia, weight loss, lymphadenopathy, hemorrhagic diathesis, and hepatosplenomegaly. New onset or persistent morning headaches associated with vomiting, neurologic symptoms, or back pain should raise concern for tumors of the central nervous system. Palpable masses in the abdomen or soft tissues, and persistent bone pain that awakens the child are red flags for abdominal, soft tissue, and bone tumors. Leukokoria is a red flag for retinoblastoma. Endocrine symptoms such as growth arrest, diabetes insipidus, and precocious or delayed puberty may be signs of endocranial or germ cell tumors. Paraneoplastic manifestations such as opsoclonus-myoclonus syndrome, rheumatic symptoms, or hypertension are rare and may be related to neuroblastoma, leukemia, or Wilms tumor, respectively. Increased suspicion is also warranted for conditions associated with a higher risk of childhood cancer, including immunodeficiency syndromes and previous malignancies, as well as with certain genetic conditions and familial cancer syndromes such as Down syndrome, Li-Fraumeni syndrome, hemihypertrophy, neurofibromatosis, and retinoblastoma.
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PMID:Signs and symptoms of childhood cancer: a guide for early recognition. 2393 97

A 79-year-old woman, a smoker, presented to the emergency department with a 2-week history of progressive dyspnoea and peripheral oedema, preceded by flu-like symptoms that had failed to improve despite antibiotics. Examination identified severe hypertension and signs consistent with severe fluid overload. Baseline chest X ray showed a right-sided abnormality. In the context of an extensive smoking history CT thorax was indicated which demonstrated a right-sided hilar mass and lymphadenopathy. Blood tests showed an unexplained metabolic alkalosis with partial respiratory compensation and hypokalaemia prompting endocrinological tests investigating Cushing's syndrome. Urinary cortisol was measured at 3174 nmol/L/24 h (normal<560), serum adrenocorticotropic hormone (ACTH) of 215 ng/L (normal<46). Pleural fluid cytology confirmed a diagnosis of small-cell lung cancer (SCLC). The patient's condition deteriorated despite intravenous diuretics and nitrates, metyrapone and non-invasive ventilation. Treatment was withdrawn 1 week after admission. The clinical course in the presence of biochemical derangement and SCLC is highly suggestive of paraneoplastic ectopic ACTH secretion.
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PMID:Cardio-respiratory failure secondary to ectopic Cushing's syndrome as the index presentation of small-cell lung cancer. 2394 25


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