Pulmonary Sequestration In A Child With Acute Myelold Leukemia

The article describes a relatively rare congenital anomaly that was difficult to diagnose in a 10–year-old child with acute nonlymphoblastic leukemia. Just at diagnosis of leukemia, the patient showed a pathologic chest radiograph because of a parenchymal thickening at the right lung apex. The presence of bronchopneumonia was suspected, and broad-spectrum antibiotic therapy was started with subsequent antifungal treatment for persistent fever and concurrent chemotherapy-induced marrow aplasia, which did not favor pulmonary infiltrate recovery. Continuous culture tests, including bronchial swab, proved negative for Koch-Weeks bacillus, fungal organisms, and other pathogens. Computed tomography, however, was suggestive of Aspergillus lung involvement, and apical sepmentectomy was performed. The anatomic pathologist suggested the diagnosis of intralobar sequestration. In summary, when pulmonary pathology with an excavation is found in a leukemic child, one must consider the possibility of pulmonary sequestration complicated by an infectious disease.     

Pulmonary vein stenosis mimicking chronic lung disease

The presence of recurrent respiratory symptoms and right heart enlargement in an ex-premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography.

Conclusion: This report describes a patient with pulmonary vein stenosis in whom the diagnosis was delayed as she had symptoms and signs more commonly associated with chronic lung disease.     

Pulmonary Malacoplakia in a Child

Pulmonary malacoplakia is a rare condition with only five previously reported cases in the literature, all occurring in adults. We describe a case of malacoplakia of the lung in a 6-year-old female with acute lymphoblastic leukemia in remission and a Rhodococcus equi pneumonia and septicemia. The case demonstrates that pulmonary malacoplakia can occur in immunocompromised pediatric patients and that malacoplakia should, therefore, be added to the list of causes of nodular lung infiltrates in immunocompromised children.     

A fatal case of acute pulmonary embolism caused by right ventricular masses of acute lymphoblastic lymphoma-leukemia in a 13 year old girl

We report a case of a 13-year-old girl with acute lymphoblastic lymphoma-leukemia, who presented with a cardiac metastasis in the right ventricle, resulting in a pulmonary embolism. At the time of her leukemia diagnosis, a cardiac mass was incidentally found. The differential diagnosis for this unusual cardiac mass included cardiac tumor, metastasis, vegetation, and thrombus. Empirical treatment was initiated, including anticoagulation and antibiotics. She underwent plasmapheresis and was administered oral prednisolone for her leukemia. Five days later, she experienced sudden hemodynamic collapse and required extracorporeal membrane oxygenation insertion and emergency surgery. These interventions proved futile, and the patient died. Pathology revealed that the cardiac mass comprised an aggregation of small, round, necrotic cells consistent with leukemia. This is the first known case of acute lymphoblastic leukemia presenting as a right ventricular mass, with consequent fatal acute pulmonary embolism. A cardiac mass in a child with acute leukemia merits investigation to rule out every possible etiology, including vegetation, thrombus, and even a mass of leukemic cells, which could result in the fatal complication of pulmonary embolism.     

Pulmonary Alveolar Septal Calcinosis Causing Progressive Respiratory Failure in Acute Lymphoblastic Leukemia in Childhood

A syndrome of pulmonary alveolar septal calcinosis, pneumothorax, and pneumomediastinum, leading to rapidly progressive acute respiratory insufficiency and death was observed in 2 children with acute lymphoblastic leukemia (ALL). Primary clinical and radiological considerations in these patients were pulmonary edema and infection, and the diagnosis of pulmonary alveolar septal calcification was established only at autopsy. One patient, a 15-year-old girl, was found also to have parathyroid hyperplasia typical of familial hyperparathyroidism. The other, a 16-month-old girl, showed osteitis fibrosa of the bones and parathyroid hyperplasia of secondary type, suggesting that the pulmonary calcinosis resulted from hypercalcemia caused by a parathormone or prostaglandin-secreting tumor. The cause of pneumothorax and pneumomediastinum may have been rupture of calcified alveolar septa induced by high PEEP during ventilation of these patients. Other possible mechanisms contributing to hypercalcemia and pulmonary calcinosis in children with acute leukemia include bone resorption due to marrow infiltration, immobilization syndrome, renal failure, and administration of calcium, phosphate, or bicarbonate. This complication of acute leukemia in childhood is rare (2 patients in 430 autopsied over the period 1961-1982 at Childrens Hospital of Los Angeles). How often the process can be reversed if diagnosed before severe respiratory insufficiency is present is not known.     

Pulmonary hypertensive diseases

The commonest causes of pulmonary hypertension are secondary to endstage pulmonary disease or congenital heart disease (including structural abnormalities of the pulmonary veins). Less obvious causes include sleep disordered breathing due to obstructive sleep apnoea or neuromuscular disease, and occult interstitial lung disease. When these have been excluded, the primary pulmonary vascular diseases should be considered. These are primary pulmonary hypertension; pulmonary veno-occlusive disease; pulmonary embolic disease (thromboembolism, and non-thrombotic embolism) and invasive pulmonary capillary haemangiomatosis. The clinical signs and chest X-ray appearances are often non-specific. Echocardiography can often estimate pulmonary artery pressure and exclude congenital heart disease. Right heart catheterization is usually needed to confirm the diagnosis, estimate any reversibility of elevated pulmonary vascular resistance and exclude other causes. Precise diagnosis may require an open lung biopsy. For many of these conditions, treatment is difficult and the prognosis poor unless the child has a lung transplant.     

Pulmonary function in patients undergoing bone marrow transplantation

Pulmonary function was studied prospectively in 25 children with leukemia and aplastic anemia undergoing bone marrow transplantation (BMT). Whereas 11 patients have died, only one did so primarily due to interstitial pneumonia. Fourteen patients (56%) survived a median of at least 36 months. Seventeen patients received pulmonary function tests (PFTs). Four patients transplanted for leukemia in relapse following preparation with a very intensive regimen (cyclophosphamide, 200 mg/kg, total body irradiation, 1,000 rad, BCNU, cytosine arabinoside) developed restrictive lung changes. Patients undergoing BMT for aplastic anemia and leukemia in remission prepared with more commonly used and less intensive regimens maintained normal pulmonary function. As new regimens are devised, PFTs should be utilized to characterize the pulmonary toxicity of these regimens as well.     

Metastatic Intracranial Chordoma in a Child with Massive Pulmonary Tumor Emboli

A 27-month-old boy of Hispanic background developed multiple cranial nerve palsies, difficulty swallowing, bloody nasal discharge, and irritability. Radiographic evaluations showed extensive destruction of the clivus by a large tumor that invaded the sphenoid bone, left cavernous sinus, ethmoid sinus, nasal cavity, and left orbit. Multiple pulmonary nodules were also noted. The bone marrow and spinal fluid showed no evident tumor cells. Transnasal biopsy revealed a chordoma. Treatment was initiated with a combination of ifosfamide, mesna, and etoposide along with radiation therapy to the cranial tumor. Shifting pulmonary densities were noted on serial films. Despite some clinical improvement, the child developed rapidly progressive hypoxemia 3 weeks after admission and died. Autopsy showed persistent viable tumor in the primary site and massive pulmonary arteriolar tumor emboli, infarcts, and widespread lung parenchymal metastases. No other sites of tumor involvement were discovered. This is the second child reported with intracranial chordoma, pulmonary metastases at diagnosis, and early death attributed to pulmonary tumor emboli.     

pH and nitric oxide synthase activity and expression in bovine aortic endothelial cells

Aim: Nitric oxide (NO) plays an important role in the transition from intrauterine to extrauterine life. If this transition fails, a condition called persistent pulmonary hypertension of the neonate (PPHN) may develop. The current treatment modalities for this disease include induction of alkalosis by hyperventilation or alkali infusion, inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation. There is evidence from animal studies that the elevated pH, not the low pCO₂ is responsible for the resultant pulmonary vasodilatation. In this study, we examined the effect of pH on the activity and expression of endothelial nitric oxide synthase (eNOS) in cultured bovine aortic endothelial cells (BAEC) as a possible explanation for the pH dependent drop in pulmonary vascular resistance.

Methods: BAEC were exposed to a pH gradient of 7.1-7.6 for 4 h (short-term) and 16 h (long-term). Standard Western blotting technique was used to detect expression of eNOS. Activity was measured by an indirect assay using bovine aortic smooth muscle cells (BASM) as reporter cells and measuring cGMP levels as a marker of NO production. The cells were exposed to the pH gradient for a total of 4 h and measurement were made at 30, 60 and 90 min, and 2, 3 and 4 hours.

Results: eNOS activity and expression remained unchanged during the four and sixteen hours of exposure.

Conclusion: In this in vitro experiment, we could not demonstrate an alkalosis-induced increase in eNOS activity and expression. The clinically observed pH dependent vasodilatation does not appear to be directly mediated through the induction of eNOS.     

Interference Of High-Dose Methotrexate In The Metabolism Of Valproate?

A case of tonic-clonic seizure was obserued in a child with acute lymphoblastic leukemia a few hours after a 24-hour infusion of high-dose methotrexate (MTX; 5 g/m²). Because of former epileptic symptoms, the child had been treated with valproic acid for several months. During this and the following high-dose MTX infusion, an acute decline of the serum valproate concentration to about 25% of the pre-MTX value was observed. The pathogenesis of the acute decline of serum valproate concentration is discussed.     

Unilateral pulmonary edema and acute rheumatic fever

Although the diagnostic criteria for acute rheumatic fever (ARF) are well known, a high index of suspicion is necessary in order to assure timely diagnosis and appropriate treatment. We present a case of an 8-year-old child who presented with unilateral pulmonary edema secondary to acute mitral insufficiency due to ARF. ARF should be considered in the differential diagnosis of unilateral pulmonary edema in children.     

产前诊断对室间隔完整的肺动脉瓣闭锁和危重型肺动脉瓣狭窄新生儿治疗及预后影响

目的探讨产前诊断对室间隔完整的肺动脉瓣闭锁(PA/IVS)和危重型肺动脉瓣狭窄(CPS/IVS)新生儿早期治疗及近中期预后的影响。方法按病例对照研究方法,将28例有经皮球囊肺动脉瓣成形术(PBPV)手术指征的PA/IVS或CPS/IVS患儿根据有无产前诊断分为产前组(n=15)及产后组(n=13)。产前组于胎儿期明确诊断后即制定干预方案,产后组自外院转诊并明确诊断后制定干预方案。所有患儿均于血流动力学稳定后,在新生儿期接受PBPV,术后1个月、3个月、6个月、1年及2年时随访,比较两组患儿的临床情况、心脏彩色超声及介入术中的测量值。结果 28例患儿接受PBPV平均日龄为(7.53±3.18)d,平均体质量为(3 102.32±708.40)g,其中PA/IVS 9例,CPS/IVS 19例,随访时间(18.82±5.22)月,无死亡病例。产前组入院日龄及首次治疗日龄明显小于产后组,差异有统计学意义(P??0.05)。入院时产前组血流动力学较产后组稳定,产后组并发症发生率较高。随访过程中,两组再介入率、双心室循环情况及术后1年右室和肺动脉瓣的发育差异均无统计学意义(P??0.05)。结论产前诊断有助于对PA/IVS及CPS/IVS患儿的早期干预,并可减少术后并发症的发生。     

Invasive esophageal aspergillosis associated with acute myelogenous leukemia: successful therapy with combination caspofungin and liposomal amphotericin B

Aspergillosis is one of the most common invasive fungal infections in patients with leukemia. In this patient group, this form of Aspergillus infection is a life-threatening condition with a mortality of 50-100%. The lungs are most often affected, but the esophagus can also be involved.The authors report the case of a child with leukemia who developed invasive esophageal aspergillosis. The condition was diagnosed by microscopic examination of endoscopic biopsy specimens. The patient was already receiving empirical liposomal amphotericin B when the diagnosis was made, so a second antifungal (caspofungin) was added to the regimen. This combination was successful. This case to demonstrates a case of successful treatment of invasive esophageal aspergillosis using combination therapy of liposomal amphotericin B and caspofungin.     

Pulmonary hypertension in a child with juvenile myelomonocytic leukemia secondary to pulmonary leukemic cell infiltration

A 4-year-old girl with juvenile myelomonocytic leukemia presented to the emergency room with dyspnea. Echocardiography was performed due to cardiomegaly and prominent main pulmonary artery on a chest X-ray film. On echocardiography the right ventricular pressure calculated from the velocity of tricuspid regurgitation jet was 55 mmHg with no pulmonary stenosis. Despite treatment for pulmonary hypertension and provision of respiratory support, the patient died. A postmortem lung biopsy specimen showed infiltration by tumor cells, which suggested that the pulmonary hypertension had been caused by leukemic infiltration. In conclusion, the findings suggest that leukemic infiltration into the lungs may occur in children with juvenile myelomonocytic leukemia. It should be recognized as a potentially treatable cause of pulmonary hypertension in patients with juvenile myelomonocytic leukemia.     

Fatal cerebral and pulmonary aspergillosis in acute leukemia in a child

Immediately after induction therapy for acute lymphoblastic leukemia, a 2 1/2-year-old child developed invasive pulmonary aspergillosis revealed by pneumothorax, an unusual manifestation. Despite treatment with amphotericin B, status epilepticus occurred; this manifestation was related to diffuse ischemic cerebral lesions probably caused by cerebral aspergillosis. Outcome was fatal. Early invasive pulmonary aspergillosis is responsible for non-specific pneumonia. Thoracic CT scan and fiberoptic bronchoscopy are informative investigations. At recovery of bone marrow aplasia, the occurrence of hemoptysis and the discovery of excavated lesions on roentgenograms are suggestive of the diagnosis. Cerebral aspergillosis should be routinely considered whenever neurologic symptoms develop in a patient with agranulocytosis, fever, and pneumonia. The prognosis of invasive aspergillosis depends above all on the promptness of treatment; amphotericin B should be given intravenously whenever broad spectrum antimicrobial therapy fails to induce apyrexia in a patient with agranulocytosis.     

Dysphonia: A Frequently Encountered Symptom in the Evaluation of Infants with Unobstructed Supracardiac Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection is a relatively uncommon congenital cardiac anomaly. When there is obstruction to pulmonary venous drainage or severe pulmonary hypertension due to increased pulmonary blood flow, the patient is usually critically ill and the diagnosis is unambiguous. However, in some patients the symptoms may be mild and often overlap with those of other noncardiac diseases. Tachypnea, failure to thrive, and mild cyanosis are the most frequently reported symptoms. In our patient population, we encountered a frequent observation by parents that the child had an alteration in voice which we elected to refer to as dysphonia for the purpose of this study. We retrospectively reviewed all patients with total anomalous pulmonary venous connection who were evaluated at our institution from January 1996 to January 1999 and found that 27% of patients had dysphonia as a presenting complaint to their primary care provider. We hypothesize that the etiology of this phenomenon lies in the possible compression of the left recurrent laryngeal nerve in the vicinity of the dilated pulmonary artery and the left vertical vein.     

法洛四联症伴肺动脉瓣缺如3例报道并文献复习

目的总结法洛四联症伴肺动脉瓣缺如患儿的临床特点和诊治经验。方法收集复旦大学附属儿科医院2007年11月至2009年2月收治的法洛四联症伴肺动脉瓣缺如3例患儿的临床资料,总结其临床表现、影像学特征 、治疗和预后。结果男1例,女2例,年龄39 d至4个月。临床均表现为气急、咳喘及轻度发绀;体检可闻及胸骨左缘来回杂音。超声心动图和心导管检查除法洛 四联症表现外,可见肺动脉瓣环发育不良或缺如,伴肺动脉重度反流、肺动脉瘤样扩张,均未见动脉导管未闭,其中1例左肺动脉不连接伴发育 不良。2例临床症状严重的患儿施行手术根治,其中1例手术效果满意,另1例左肺动脉不连接伴发育不良患儿术中死亡;1例患儿临床症状尚不 明显,仍在密切随访中。 结论法洛四联症伴肺动脉瓣缺如尽管少见,但由于在婴儿期甚至新生儿期即可引起严重气道受压从而危及生命,临床中遇到难以控制的呼吸窘 迫新生儿和小婴儿应考虑本病的可能。尽早手术根治解除气道受压呼吸困难症状是唯一的治疗方法,无呼吸道受压症状可考虑6个月左右择期手 术。手术病死率高于单纯法洛四联症,尤其是伴一侧肺动脉不连接或发育不良的患儿。     

Nosocomial pulmonary Rhizopus diagnosed by bronchoalveolar lavage with cytology in a child with acute lymphoblastic leukemia

Rhizopus species is an opportunistic fungus that is contracted by inhalation of aerosolized spores. Early diagnosis is often difficult but is a necessity to prevent rapid progression of the infection that leads to blood vessel invasion by hyphae, causing fatal hemoptysis. A previous case report described the utility of cytologic examination of bronchoalveolar lavage (BAL) fluid in achieving a prompt diagnosis of Rhizopus species in an adolescent patient with diabetic ketoacidosis. The author presents a case that further describes the benefit of performing BAL fluid cytology to help identify fungal morphology characteristics in order to reach an expeditious diagnosis of Rhizopus species in a leukemia patient.     

Telling Young Children with Leukemia Their Diagnosis: The Flower Garden as Analogy

A new approach to communicating the diagnosis of leukemia to the young child was carried out in the Pediatric Hematology Department in Monza over a 2-year period (1989 to 1991). Fifty patients ages 6 to 15 years were entered into the program. A physician communicated the diagnosis of leukemia directly to the child without the presence of the parents. A set of 25 slides was prepared. A garden with flowers and weeds was used as an analogy for leukemia. All 50 of the children expressed gratitude for understanding their disease and the families for being able to talk with their children about the disease without panic and stress.