- [Abernethy malformation: unusual cause of central cyanosis in pediatrics]. [Journal Article]
- RGRev Gastroenterol Peru 2018 Apr-Jun; 38(2):183-186
- The congenital portosystemic shunt is an uncommon disease with severe complications if not treated. This rare cause of hypoxemia was first described by John Abernethy in 1973. There are two types: ty...
The congenital portosystemic shunt is an uncommon disease with severe complications if not treated. This rare cause of hypoxemia was first described by John Abernethy in 1973. There are two types: type I (termino-lateral shunt), in which there is total absence of the intrahepatic portal flow, and type II (latero-lateral shunt), in which the portal flow is partially preserved. We present the case of a 6-year-old girl with chronic hypoxemia history since 4 and a half years of age, showing progressive dyspnea, who was referred to the Pediatric Pulmonary Division with the diagnosis of central cyanosis. An Echo-Doppler in the portal venous system was performed, reporting agenesis of the principal portal vein. This finding was corroborated by an angiography of the portal and mesenteric arteriovenous system. The study was completed with a portosplenography, which confirmed the diagnosis of type Ib Abernethy malformation. The type I Abernethy malformation is more common in females, shows up in different ways and is treated with liver transplantation. On the other hand, type II Abernethy malformation shows a variable portal circulation and has a better prognosis than type I. Dyspnea when exercising and central cyanosis should be considered to make a differential diagnosis of cardiorespiratory disease at a pediatric age.
- Estimating Life Expectancy From Chinese Medicine Could Improve End-of-Life Care in Terminally Ill Cancer Patients. [Journal Article]
- HNHolist Nurs Pract 2018 Sep/Oct; 32(5):247-252
- Preparing for a good death is an important and meaningful concept in Chinese culture because people hope to know residual life to make effort for their unfinished business. However, the family of ter...
Preparing for a good death is an important and meaningful concept in Chinese culture because people hope to know residual life to make effort for their unfinished business. However, the family of terminally ill patients with cancer may be annoyed and frustrated about unexpected bereavement if they have unresolved conflicts with the loved one, missing a chance for declaring love, untimely apologizing and saying goodbye. The study aimed to explore this difficult issue. The medical records of 121 deceased terminally ill patients with cancer at National Cheng Kung University Hospital between December 2010 and February 2012 were reviewed. The signs and awareness of dying among these patients were collected using palliative routine instruments in the hospice ward. The top 3 most prevalent dying signs were coolness and cyanosis (prevalence 98.3%, median period from the first documented dying sign to death 2 days, P = .028), mirror-like tongue (prevalence 94.2%, median period 5 days, P = .007), and earlobe crease (prevalence 93.4%, median period 4 days, P = .052). In addition, the prevalence of dying awareness was 71.1% (median period 4 days, P = .001). Furthermore, terminal agitation was identified more frequently in terminally ill patients with hepatoma and colon cancer (adjusted odds ratio = 3.240, P = .043), but turbid sclera with edema was noted more often in terminally ill patients with head and neck cancer (adjusted odds ratio = 5.698, P = .042). The results provide evidence to support clinical practice, offering knowledge and techniques to health care providers, and increasing quality of life for terminally ill patients with cancer.
- Post-tuberculosis Aspergilloma in Undiagnosed Tetralogy of Fallot. [Journal Article]
- CCureus 2018 Jun 05; 10(6):e2740
- Tetralogy of Fallot (TOF) is the most common congenital heart disease (CHD) with an incidence of four in every 1000 live births in Pakistan. Classically, these children present with central cyanosis ...
Tetralogy of Fallot (TOF) is the most common congenital heart disease (CHD) with an incidence of four in every 1000 live births in Pakistan. Classically, these children present with central cyanosis in early life; however, milder defects may remain asymptomatic for months or even years. We report a malnourished and anemic teenage male, who was admitted with shortness of breath, hemoptysis, fever, palpitations, and weight loss. On examination, vitals were stable, except for oxygen saturation, which was 84% on pulse-oximeter. Bilateral basal coarse crepitations were present on respiratory examination with a markedly reduced air entry in the right upper zone. A 2-3/6 systolic ejection murmur was appreciated on cardiac examination. The chest X-ray was consistent with a collapsed right upper lobe with fibrosis. Echocardiography was consistent with findings of TOF. Based on sputum for acid-fast bacilli (AFB smear) and GeneXpert (Cepheid Inc., Sunnyvale, California, US) Mycobacterium tuberculosis/resistance to rifampin (MTB/RIF), the patient was diagnosed with multi-drug resistant pulmonary tuberculosis (MDR-PTB). However, when the patient didn't improve with anti-tuberculous therapy, a computed tomography (CT) scan chest was done, which raised a suspicion of aspergilloma. The culture and cytology of bronchoalveolar lavage (BAL) were done, which confirmed pulmonary aspergilloma. Undiagnosed congenital heart diseases are rare in adults. Pulmonary TB is rarely reported in right-to-left shunts; however, clinicians should maintain a suspicion of this correlation.
- Tetralogy of Fallot: Stroke in a Young Patient. [Journal Article]
- CCureus 2018 May 31; 10(5):e2714
- Tetralogy of Fallot (TOF) is a congenital birth defect of the heart which actually comprises four individual flaws. It causes poor flow of oxygenated blood to the organs and leads to cyanosis (blue-t...
Tetralogy of Fallot (TOF) is a congenital birth defect of the heart which actually comprises four individual flaws. It causes poor flow of oxygenated blood to the organs and leads to cyanosis (blue-tinted skin, because of inadequate oxygenation). It can be recognized at birth or in adulthood. But sometimes, cases may go unnoticed, and the patient might present with some rare complications. In this case, the patient presented with an embolic infarct of the brain at the age of 25 with an undiagnosed tetralogy of Fallot.
- Assessment and Validation of Syndromic Case definitions for Respiratory Syncytial Virus Testing in a Low Resource Population. [Journal Article]
- PIPediatr Infect Dis J 2018 Aug 02
- Standardized case definitions are needed in decision-making regarding respiratory syncytial virus(RSV) control strategies, including vaccine evaluation. A syndromic case definition comprising of "whe...
Standardized case definitions are needed in decision-making regarding respiratory syncytial virus(RSV) control strategies, including vaccine evaluation. A syndromic case definition comprising of "wheeze or apnea or cyanosis" could be useful for community-based surveillance of moderate RSV infection among young infants particularly in resource-limited settings. However, this definition showed modest specificity (29.2-49.6%) indicating that community-based surveillance may need augmentation with other data.This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
- Hepatopulmonary syndrome in children - a 20 year review of presenting symptoms, clinical progression and transplant outcome. [Journal Article]
- LTLiver Transpl 2018 Jul 31
- CONCLUSIONS: HPS is a life threatening complication of cirrhosis which usually develops insidiously. This combined with the often stable nature of the liver disease leads to delays in diagnosis and listing for LT. Progressive polycythaemia extends the need for supplementary O2 and PICU stay. We advocate screening for HPS with a combination of SpO2 and Hb monitoring to facilitate earlier recognition, timely LT and shortened recovery periods. This article is protected by copyright. All rights reserved.
- Risk factors for hyperuricemia in congenital heart disease patients and its relation to cardiovascular death. [Journal Article]
- CHCongenit Heart Dis 2018 Jul 31
- CONCLUSIONS: CHD patients, noncyanotic and cyanotic, have higher serum uric acid levels and gout than patients in the general population. BMI, renal insufficiency, cyanosis, and the use of diuretics were risk factor for hyperuricemia among CHD patients.
- Early onset of cyanosis in a patient with atrial septal defect: Transesophageal echocardiography reveals the underlying mechanism. [Journal Article]
- ACAnn Card Anaesth 2018 Jul-Sep; 21(3):287-289
- Cyanosis due to right to left shunt across an atrial septal defect (ASD) brings up lots of questions in an inquisitive mind! Systemic desaturation at rest or during exercise can limit physical abilit...
Cyanosis due to right to left shunt across an atrial septal defect (ASD) brings up lots of questions in an inquisitive mind! Systemic desaturation at rest or during exercise can limit physical ability and impair the quality of life of patients with congenital heart defect (CHD). Traditionally, ASD is taught as an acyanotic CHD, but we encountered a child with a different clinical presentation. Understanding the mechanism of early onset systemic desaturation in the presence of an ASD is vital for surgical planning and decision-making. A comprehensive TEE examination always compliments clinical and haemodynamic data for an aetiology based patient management. This brief clinical communication attempts to discuss the role of perioperative TEE examinations in a case of ASD with central cyanosis.
- Biliary Tract Abnormalities as a Cause of Distal Bowel Gas in Neonatal Duodenal Atresia. [Journal Article]
- CRCase Rep Surg 2018; 2018:8041427
- CONCLUSIONS: In the absence of a "double bubble" appearance and intestinal gas distally on a plain radiograph, one must not exclude duodenal atresia as the differential diagnosis.
New Search Next
- The eye in CHD. [Journal Article]
- CYCardiol Young 2018; 28(8):981-985
- In recent years, there has been a rise in the number patients with CHD surviving into adulthood. Many have complications related to their CHD or its treatments, outside the heart, including ocular ab...
In recent years, there has been a rise in the number patients with CHD surviving into adulthood. Many have complications related to their CHD or its treatments, outside the heart, including ocular abnormalities. The objective of this review is to highlight the ocular abnormalities that occur in adults with CHD, either from their condition or related to the common drugs prescribed to manage it. In particular, we reviewed the effects of cyanosis, coarctation of the aorta, endocarditis, and the side effects of Sildenafil and Amiodarone. A change in the retinal vasculature is a common observation with cyanosis or coarctation of the aorta. Occlusion of the retinal vessels may also be observed in cyanotic patients, as well as those with infectious endocarditis. Sildenafil has established ocular side effects; here they are explored in the context of therapy for pulmonary hypertension. Similarly, Amiodarone has established ocular risks, which are summarised. The high prevalence of ocular consequences in adult CHD patients reinforces the need for knowledge of the risks involved and for frequent ophthalmological screening where appropriate.