Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024312 (lymphopenia)
4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Postoperative chylothorax may occur following any intrathoracic surgical procedure in children. From January 1979 through February 1987, 18 children aged 1 month to 9 years had chylothorax listed among their discharge diagnoses. Initial therapy for all patients consisted of chest tube placement for pleural space drainage and nutritional support with either enteral formulas enriched with medium-chain triglycerides or total parenteral nutrition. Both total parenteral nutrition and medium-chain triglyceride-enriched enteral regimens provided adequate nutritional support in these children. Five of the 18 experienced lymphopenia secondary to chylous lymphocyte loss. Infections were diagnosed in 5 patients during hospitalization; one was a fatal viral pneumonitis. No correlation between infectious complications and lymphocyte count could be demonstrated.
...
PMID:Management of nutritional and infectious complications of postoperative chylothorax in children. 177 26

The management and complications of chylothorax occurring beyond the neonatal period were reviewed retrospectively. Records from 15 patients treated between 1976 and 1986 were analysed; a combination of thoracocentesis, chest drain insertion, and dietary modification were successful in abolishing chyle leakage in 10 cases. One child died from complications of cardiac surgery rather than from the chylothorax, and surgical intervention was necessary in the remaining four patients and included pleurectomy in three and thoracic duct ligation in the fourth. Lymphopenia, hypoalbuminaemia, hyponatraemia, and weight loss were the most common complications of conservative management and tended to occur in those patients with the longest duration of drainage. Postoperative recovery after pleurectomy and thoracic duct ligation was uneventful. We conclude that conservative management of chylothorax will be successful in most cases. Complications of such a policy are fairly common but rarely serious.
...
PMID:How should chylothorax be managed? 361 76

We reviewed the management of 25 cases of chylothorax/chylopericardium (CT/CP) in 24 patients (9 females, 15 male; 3 days to 11-years-old) following 1605 cardiothoracic procedures (incidence of 1.5%) between January 1984 and December 1993 at our institution. The surgical procedures preceding the occurrence of lymph leak included ligation of patent ductus arteriosus (6 patients), coarctation/double aortic arch repairs (3), complex intracardiac repairs (11), and systemic to pulmonary shunts (5). There were 3 CPs and 22 CTs. All of the patients were initially treated nonsurgically with diet modification using either total parenteral nutrition (TPN) or enteral low fat solid food or enteral elemental diet supplemented with intravenous lipid emulsion. Twenty-one cases (84%) responded to conservative therapy. Of those, 15 had TPN as the initial treatment; the average duration of lymph leak was 13.7 (range 7 to 30) days and the average maximal lymph leak was 39.4 (range 15 to 130) mL/kg per day. The other six cases had low-fat enteral diet as the initial treatment, four resolved completely. Two with high-central venous pressure had to be switched to TPN prior to complete resolution. The average duration of lymph leak in this subgroup was 30 (range 12 to 56) days with the average maximal lymph leak was 30.1 (range 8.5 to 59) mL/kg per day. Excluding these two cases, the average lymph leak of the rest of the group was very compatible to the TPN group of 15 days. Lymphocytopenia and hyponatremia were frequently seen during CT/CP (47.6% and 43%, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)
...
PMID:The management of chylothorax/chylopericardium following pediatric cardiac surgery: a 10-year experience. 754 87

Chylothorax in the absence of tumor or trauma is uncommon. Lymphangiomatosis of the bone, although extremely rare, has been associated with chylothorax. The authors describe the case of a 12-year-old boy who presented with a symptomatic left chylothorax associated with lymphangiomatosis of the ribs, scapula, and clavicle. Despite tube thoracostomies and the initiation of total parenteral nutrition, massive losses of chyle persisted, resulting in hypoproteinemia and severe lymphopenia. Control of the chylothorax was achieved by a parietal pleurectomy and application of fibrin glue (Tisseel). In the literature there are 16 cases of chylothorax associated with lymphangiomatosis of the bone. Their presentation, treatment, and outcome are reviewed. Conservative treatments such as dietary manipulations or thoracenteses were rarely successful. Thoracotomy with parietal pleurectomy on the side of the effusion is usually effective in controlling the chylothorax. Lymphangiomatosis should be considered a diagnostic possibility for any child who presents with a chylothorax.
...
PMID:Massive chylothorax associated with lymphangiomatosis of the bone. 780 41

Questions persist about the management of postoperative chylothorax in infants and children. Our experience with postoperative chylothorax over the most recent decade (1980 to 1990) has been reviewed. The type and amount of drainage, data from cardiac catheterization and echocardiography, operative decisions and details, and eventual outcomes have been cataloged. All patients were initially treated with total gut rest, with operation reserved for unabated drainage. Chylothorax developed postoperatively in 15 infants and 11 children (18 with a cardiac procedure and 8 with a noncardiac procedure). The average age was 3.1 years. Spontaneous cessation and cure occurred in 19 (73.1%) of these 26 patients, with an average drainage duration of 11.9 days (range, 4 to 30 days). Those for whom operation was chosen drained preoperatively for an average of 29.2 days (range, 25 to 40 days). There were no deaths in either group. Complications were lymphopenia (2 patients) and fungal sepsis (1 patient). The amount of drainage per day was not significantly different between patients treated operatively and those treated nonoperatively. Failure of nonoperative management was associated with venous hypertension from increased right-sided cardiac pressures or central venous thrombosis (p < 0.05, Fisher's exact test). Presumably this increased pressure is transmitted to the lymphatic system. These patients should be identified early and considered for thoracic duct suture or pleuroperitoneal shunting.
...
PMID:Management of pediatric postoperative chylothorax. 837 18

A 10-year-old, male toy poodle presented for evaluation of a progressively worsening, harsh, nonproductive cough. Chylous pleural effusion, lymphopenia, thrombocytopenia, normoblastemia, and recurrent subcutaneous bruising were diagnosed. Surgical exploration of the cranial mediastinum revealed extensive, redundant connective tissue which was confirmed by histopathology to be mediastinal lymphangiosarcoma (LAS). During surgical placement of a fenestrated silastic mesh for passive pleuroperitoneal drainage, chylous ascites also was diagnosed. The patient was euthanized two days postoperatively due to persistent chylothorax. The etiopathogenesis of chylothorax and chylous ascites are discussed. Previous case reports of lymphatic endothelial neoplasia are reviewed.
...
PMID:Chylothorax and chylous ascites in a dog with mediastinal lymphangiosarcoma. 873 Nov 42

The course of infection in a 3-week-old premature newborn suffering from extensive dermatitis with flaccid blisters is described. Staphylococcus aureus was recovered from a local wound infection around a chest tube inserted to drain a postoperative chylothorax. The strain isolated tested positive for the eta gene for exfoliative toxin A, the causative agent of staphylococcal scalded-skin syndrome (SSSS). In this case, prematurity and loss of chylus with consecutive lymphopenia may have contributed to development of SSSS.
...
PMID:Staphylococcal scalded-skin syndrome complicating wound infection in a preterm infant with postoperative chylothorax. 973 69

Intestinal lymphangiectasia is a well-recognized complication of the Fontan procedure, occurring in up to 24% of patients. Because of the loss of chylous fluid into the gut lumen, protein-losing enteropathy results as well as lymphopenia and hypogammaglobulinaemia. In some cases, dilated lymphatics in the intestinal serosa or mesentery also rupture, causing chylous ascites. Standard medical and cardiac surgical interventions are generally ineffective and the condition is frequently lethal. We report a case of intractable and life-threatening chylous ascites and chylothorax in a 14-year-old girl, associated with intestinal lymphangiectasia and protein-losing enteropathy after a Fontan procedure for tricuspid atresia. The condition was refractory to all standard medical therapies, including dietary modifications, diuretics, corticosteroid therapy, albumin infusions, octreotide, heparin, bowel rest, and parenteral nutrition. Cardiac surgery to optimize her hemodynamic status was also ineffective and large volume pleural and ascitic fluid losses continued. Having exhausted all other therapeutic modalities, (99m)technetium-dextran scintigraphy was performed to assess the extent of intestinal protein loss and the potential for surgical intervention. Scintigraphy suggested localized protein loss from the proximal jejunum and subsequent segmental resection was effective. Postoperatively, ascites and pleural effusions resolved, and there was no evidence of short bowel syndrome. Growth has accelerated and the patient has entered puberty. There is mild persistent intestinal protein loss requiring diuretic therapy. Ascites or pleural effusions are absent, and the patient remains well >2 years after surgery. Intestinal lymphangiectasia post-Fontan procedures has traditionally been ascribed to hemodynamic factors such as raised systemic venous pressure, which would predispose to a generalized intestinal lesion. However, in this case, scintigraphy demonstrated a localized, surgically correctible lesion. To our knowledge, this is the first reported case of the use of (99m)technetium-dextran scintigraphy for this indication and of successful partial small bowel resection in such a case.
...
PMID:Successful resection of localized intestinal lymphangiectasia post-Fontan: role of (99m)technetium-dextran scintigraphy. 1294 20

Measurement of T-cell receptor excision circles (TREC) in neonates has allowed for population-based screening of severe combined immunodeficiency and other disorders associated with T-cell lymphopenia. In addition to primary T-cell lymphopenic disorders, secondary causes of T-cell lymphopenia can be diagnosed with TREC analysis. We discuss the diagnostic evaluation of a patient with normal TREC analysis at birth that became abnormal after cardiac surgery. TREC analysis was performed by the Florida State Laboratory. Diagnostic evaluation and treatment were performed at All Children's Hospital, St Petersburg, Florida. We identified a 38-day-old female patient with thoracic duct injury, which caused chylothorax and chylous ascites diagnosed after an abnormal newborn screen. Chylothorax was secondary to thoracic duct injury after cardiac surgery and led to severe lymphopenia and hypogammaglobulinemia. Thoracic duct ligation led to improved lymphocyte counts and normalization of immunoglobulin levels. Secondary causes of lymphopenia are detected with TREC assay that lead to abnormal newborn screen results. Many secondary causes of lymphopenia can be acquired with normal initial newborn screens that become abnormal over time.
...
PMID:Thoracic duct injury resulting in abnormal newborn screen. 2456 4

Gorham-Stout Disease (GSD) is a rare lymphatic disorder affecting children or young adults with no predilection of sex. It is generally associated with vanishing bone osteolytic lesions, thoracic and abdominal involvement, and diffuse pulmonary lymphangiomatosis. Chylous effusions and chylothorax, consequent to the abnormal proliferation of lymphatic vessels, may induce respiratory failure with a high mortality risk. Extrapulmonary alterations may include chylous ascites, lymphopenia, and destructing bone disease for overgrowth of lymphatic vessels. Here, we report the case of a young woman who developed a severe and recalcitrant GSD with persistent unilateral chylothorax during pregnancy. The complex management of this patient during and after pregnancy was discussed and compared with literature data to contribute to the definition of a correct diagnostic and therapeutic approach to this rare lymphatic disease.
...
PMID:Gorham-Stout Disease Management during Pregnancy. 2926 42


1