1/39. A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease.INTRODUCTION: Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent. methods: We performed routine histologic studies, immunohistochemical staining, and electron microscopic analysis of peripheral lung biopsy specimens from 2 children with angiographically proven pulmonary arteriovenous malformations. Microvessel density was determined with a computer-assisted, morphometric analysis system. RESULTS: Histologic examination demonstrated large, dilated blood vessels ("lakes") and clustered, smaller vessels ("chains") in the pulmonary parenchyma. Microvessel density was significantly greater in these patients than in age-matched controls (P =.01). immunohistochemistry demonstrated uniform staining for type IV collagen and alpha-smooth muscle actin, weak staining for the endothelial marker CD31 (cluster of differentiation, PECAM-1), and negative staining for proliferating cell nuclear antigen. Electron microscopy revealed endothelial irregularity, a disorganized basement membrane, and increased numbers of collagen and actin filaments beneath the endothelium. CONCLUSIONS: This study represents an attempt to characterize the histologic features of pulmonary arteriovenous malformations in children with congenital heart disease who have pulmonary arterial blood flow devoid of hepatic venous effluent. The histologic correlate of this condition appears to be greatly increased numbers of thin-walled vessels. immunohistochemistry suggests that the rate of cellular proliferation is not increased in these lesions. The development of these techniques may provide a standardized histologic approach for this condition and aid in understanding its etiology.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/39. cyanosis caused by a huge obstructive right ventricular fibroma.Cardiac fibromas are rare lesions which occur more often in infants and children than in adults. These tumors are benign proliferations of connective tissue most often found in the left ventricular myocardium or septum. In an 8-month-old infant with cyanosis and progressive exertional dyspnea, a huge cardiac tumor obstructing the right ventricular outflow tract (RVOT) was diagnosed by means of 2-dimensional echocardiography and cardiac catheterization. At surgery, a whitish gray solitary tumor measuring 5.0 x 4.5 cm could be well visualized. It was nearly totally resected, and the RVOT was reconstructed with an Equine pericardial patch. Histologic examination classified the tumor as a fibroma. Although surgical mortality in cardiac fibroma with RVOT obstruction is extremely high, early diagnosis and prompt excision of the tumor is mandatory in relieving its dangerous symptoms.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
3/39. Persistent anaphylactic reaction after induction with thiopentone and cisatracurium.A 6-year-old boy presented for surgery for phimosis. The anaesthetic technique included intravenous induction with thiopentone and neuromuscular blockade with cisatracurium. Severe persistent bronchospasm and central cyanosis followed the administration of these drugs. A continuous i.v. infusion of epinephrine at 0.2 microg. kg(-1) x min(-1) was necessary to break the severe refractory bronchial hyperresponsiveness. There was no previous exposure to anaesthetic drugs and no definite family history of allergy. Through increased serum eosinophil cationic protein, tryptase and histamine levels and IgE levels specific to cisatracurium, we demonstrated an IgE-mediated anaphylactic reaction to cisatracurium in the child's first exposure to this new neuromuscular blocking agent. Anaphylactic reactions to new anaesthetic drugs may be challenging to recognize and treat during general anaesthesia in children. The pathogenesis, diagnosis and management of life threatening persistent allergic reactions to intravenous anaesthetics are discussed.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
4/39. Multiple intrapulmonary arteriovenous fistulas in childhood.Pulmonary arteriovenous fistulas (AVFs) are a rare but recognized cause of cyanosis in childhood. Lesions may be acquired as in hepatopulmonary syndrome or they may be congenital, particularly in association with certain multisystem disorders. Large fistulas are more common than multiple small connections. Two cases. both boys, presenting in the first decade of life are described. "Bubble" echocardiography was the most telling investigation and strongly suggested the presence of AVFs in both cases. Each patient then underwent cardiac catheterization, which demonstrated normal pulmonary artery pressure and diffuse pulmonary telangiectasis. Both patients were treated effectively with nifedipine and continue with this mode of therapy.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
5/39. Chronic idiopathic acrocyanosis.Acrocyanosis is an uncommon condition characterized by symmetric coolness and violaceous discoloration of the hands and feet. The nose, ears, lips, and nipples are also often affected. The disease is temperature dependent and generally worsens with cold exposure. Acrocyanosis is often secondary to a variety of underlying causes. We present a very interesting case of a 44-year-old woman with almost lifelong idiopathic acrocyanosis. Differential diagnoses are discussed in this article.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
6/39. Congenital methemoglobinemia due to NADH-methemoglobin reductase deficiency in three Indian families.Congenital methemoglobinemia is a relatively rare clinical disorder characterized by life-long cyanosis, caused by either an inherited mutant hemoglobin (Hb-M) or deficiency of physiologically active NADH-dependent methemoglobin reductase (NADH-MR). NADH-MR deficiency leads to two different types of recessive congenital methemoglobinemia. In type I, cyanosis is the only major symptom and NADH-MR deficiency is restricted only to the red blood cells. In type II, cyanosis is associated with severe mental retardation and neurological impairment. The objective of this study is to establish the cause of cyanosis in our cases of congenital methaemoglobinemia. Erythrocyte NADH-MR activity was assayed spectrophotometrically. Spectral analysis of the hemolysate treated with potassium ferricyanide was recorded between 400-700 nm and Hb electrophoresis on starch gel at pH 7.0 was done to rule out the presence of Hb-M. NADH-MR deficiency was detected in 3 families. There was a history of consanguinity in one of these cases. The three propositi presented with breathlessness, fever and peripheral cyanosis. There was no history of cardiac illness or exposure to drugs and chemicals. There were no signs and symptoms of mental retardation. The presence of Hb-M was ruled out. Hb-A2, Hb-F, G6PD activity and reduced glutathione levels were normal. NADH-MR activity in all the cases ranged from 4.1 to 9.2 IU/g Hb (normal range 7.0-24.0 IU/g Hb). We describe NADH-MR deficiency in three unrelated cases (age 4 months to 6 years) where the activity of the enzyme was 30-40% of normal. These three cases of congenital methemoglobinemia are due to type-I NADH-MR deficiency without mental retardation.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
7/39. Congenital methemoglobinemia: a rare cause of cyanosis in the newborn--a case report.cyanosis is a physical finding that can occur at any age but presents the greatest challenge when it occurs in the newborn. The cause is multiple, and it usually represents an ominous sign, especially when it occurs in association with neonatal sepsis, cyanotic congenital heart disease, and airway abnormalities. cyanosis caused by abnormal forms of hemoglobin can also be life-threatening, and early recognition is mandatory to prevent unnecessary investigations and delay in management. Abnormal hemoglobin, such as hemoglobin m, is traditionally discovered by electrophoresis, so the newborn screen, which is mandatory in several states, is a useful tool for the diagnosis. Although acquired methemoglobinemia, caused by environmental oxidizing agents, is common, congenital deficiency of the innate reducing enzyme is so rare that only a few cases are documented in the medical literature around the world. We present a neonate with cyanosis as a result of congenital deficiency of the reduced nicotinamide adenine dinucleotide-cytochrome b5 reductase enzyme. This infant was found to be blue at a routine newborn follow-up visit. sepsis, structural congenital heart disease, prenatal administration, and ingestion of oxidant dyes were excluded as a cause of the cyanosis by history and appropriate tests. Chocolate discoloration of arterial blood provided a clue to the diagnosis. A normal newborn screen and hemoglobin electrophoresis made the diagnosis of hemoglobin m unlikely as the cause of the methemoglobinemia (Hb A 59.4%, A2 1.8%, and F 38.8%). Red blood cell enzyme activity and dna analysis revealed a homozygous form of the cytochrome b5 reductase enzyme deficiency. He responded very well to daily methylene blue and ascorbic acid administration, and he has normal growth and developmental parameters, although he shows an exaggerated increase in his methemoglobin level with minor oxidant stress such as diarrhea.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
8/39. Isolated sulphite oxidase deficiency: clinical and biochemical features in an Italian patient.A patient with isolated sulphite oxidase deficiency presented with seizures at 12 h of life and followed a severe course, dying at 10 months of age. There was mild facial dysmorphism and the brain showed multiple cystic fibrosis.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
9/39. cardiac tamponade in a newborn because of umbilical venous catheterization: is correct position safe?cardiac tamponade is a rare but life-threatening complication of umbilical venous catheterization in the newborn. Most complications from central venous catheters are related to incorrect position of the catheter and it is emphasized to confirm the position of the catheter tip after placement in order to avoid possible complications. We present an unusual complication of cardiac tamponade because of umbilical venous catheterization in a term newborn which is extremely rare with correct location of the catheter tip at the junction of inferior vena cava and right atrium. We suggest that correct position never guarantees uneventful catheterization in the newborn. In any infant with a central venous catheter in situ who deteriorates clinically, pericardial effusion/cardiac tamponade must be considered and appropriate action taken.- - - - - - - - - - ranking = 0.5keywords = life (Clic here for more details about this article) |
10/39. Therapeutic plateletpheresis in a case of symptomatic thrombocytosis in chronic myeloid leukemia.Extreme thrombocytosis is a frequent feature in myeloproliferative disorders which can predispose a person to thrombotic complications. As opposed to other myeloproliferative disorders, symptomatic thrombocytosis is rare in chronic myeloid leukemia. We describe a second case report of chronic myeloid leukemia (Ph chromosome positive) in a patient in chronic phase on hydroxyurea who presented with sudden onset digital cyanosis of the left hand, giddiness, headache and malaise due to extreme thrombocytosis. A 67% global reduction in the platelet count from 1553 x 10(9)/L to 513 x 10(9)/L after two therapeutic plateletpheresis procedures was seen. There was simultaneous improvement in all symptoms except cyanosis on the tip of the middle finger that progressed to dry gangrene. Dramatic reduction in the platelet count and ablation of symptoms by therapeutic plateletpheresis is an effective therapy and should begin as soon as possible.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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