Infantile polyarteritis nodosa

Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome and infantile polyarteritis nodosa, is known to present in multiple ways. Although inflammatory changes in several systems have been reported in KD, there are few documented child patients presenting with parotitis. We report such a case in a five-year-old.

Polyarteritis Nodosa (PAN) is a rare systemic necrotising vasculitis of medium and small-sized arteries. Patients typically present with systemic symptoms. Obstructive intestinal symptoms are described but usually resolve with treatment of the underlying vascular disease. We report a case of a one year old boy with multiple ischemic small bowel strictures secondary to infantile PAN, who was treated with resection of the affected segments by single port laparoscopy.

BACKGROUND: Primary liver masses in children may require intervention because of symptoms or concern about malignant transformation. OBJECTIVES: To review the management and outcome of benign liver masses in children. METHODS: We conducted a retrospective chart review of children with liver masses referred to our institution during the period 1997-2009. RESULTS: Benign liver masses were identified in 53 children. Sixteen of these children (30%) had hemangioma/infantile hepatic hemangioendothelioma (IHH) and 15 (28%) had focal nodular hyperplasia...

Although Kawasaki disease (KD) was first discovered and identified in Japan by Kawasaki in the 1960s, fatal KD cases resulting from coronary artery aneurysms had been identified retrospectively in the West as early as 1871. Kawasaki initially postulated that this disease was a new, as yet unidentified, self-limiting illness with no fatal coronary sequelae. The connection between fatal cases, then diagnosed as infantile polyarteritis nodosa, was not made until the late 1970s. Kawasaki's thoughts were reinforced by an apparent absence of nonfatal cases in the West before 1967...

Kawasaki disease, also known as mucocutaneous lymph node syndrome or infantile polyarteritis nodosa is an acute febrile vasculitis of unknown etiology with a predilection for coronary arteries and potential for aneurysm formation. In Incomplete Kawasaki disease, children with fever lack the sufficient number of criteria to fulfill the epidemiologic case definition and are diagnosed when coronary artery disease is detected. We present a case report of a one and a half years old girl who came with features of incomplete Kawasaki disease, high grade fever, irritability, history of conjunctivitis and cracking of lips...

UNLABELLED: The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17-mo-old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic-range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. Diagnosis of classic polyarteritis nodosa (c-PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non-aneurismal changes such as arterial cut-off, arterial tapering stenosis and nephrogram perfusion defect...

Antineutrophil cytoplasmic antibodies (ANCA) have been identified in a wide variety of vasculitic disorders, but it is controversial whether ANCA are present in the sera of patients with HSP. This prospective study was designed to assess the place of ANCA, particularly their IgA subclass, in HSP. Thirty-five patients (18 boys, 17 girls) aged 9.4 +/- 4 (3-16) years with a clinical diagnosis of HSP based on American College of Rheumatology (ACR) criteria were enrolled. Thirteen patients (6 boys, 7 girls) aged 8...

No abstract text is available yet for this article.

Kawasaki disease (KD; mucocutaneous lymph node syndrome) is an infantile febrile illness of unknown origin that occurs in worldwide epidemics. The main clinical features of the disease, such as fever, mucositis, cervical lymphoadenopathy and skin rash, associated with coronary aneurysms or arteritis are diagnostic. Late sequelae of coronary arteritis are very rare in the second decade of life; we describe the case of a 21-year-old male who died suddenly. The autopsy revealed a large thrombotic coronary aneurysm, coronary arteritis and myocarditis, supporting the diagnosis of a late fatal sequela of KD...

We report the case of a patient with corticosteroid-responsive giant cell hepatitis associated with typical manifestations and changes of polyarteritis nodosa from the kidney and central nervous system. Initially, the patient presented with transient right hemiparesis, followed by spontaneous remission without any abnormalities on computed tomography scan, magnetic resonance imaging and cerebrospinal fluid examination. A few months later he was admitted to our clinic because of icterus, peripheral oedema and abdominal distension...

Infantile periarteritis nodosa is a rare disease characterized by systemic obstructive arteritis involving coronary artery. This report is anesthetic experience of a 12-years-old boy with unstable angina secondary to infantile periarteritis nodosa, who underwent left ventricular aneurysmectomy and coronary artery bypass grafting. Anesthesia was induced with fentanyl, diazepam and enflurane, and transesophageal echocardiography (TEE) was used as a cardiac function monitor. The following two intraoperative crises occurred in succession: first, aggravation of myocardial ischemia followed by ventricular tachycardia, and second, sudden interruption of cardiopulmonary bypass due to blood coagulation in membrane oxygenator...

BACKGROUND: Infantile polyarteritis nodosa usually presents in children under 2 years of age as a multiorgan system disease with signs of congestive heart failure or renal failure. This disease and Kawasaki disease may share certain clinical and pathological features. CASE DESCRIPTION: We describe a child who first presented at 8 months of age with a febrile illness followed by a delay in motor and language development and a mild right hemiparesis. Five years later he died after developing oculomotor dysfunction, hypertension, and intracranial hemorrhage...

Four cases of acute mucocutaneous lymph node syndrome (Kawasaki-Syndrome) are presented This syndrome of yet unknown cause is quite frequent in Japan but lately it is being encountered with growing frequency in Europe, too. The significant clinical and diagnostical features of the disease are laid out and differentialdiagnostical and therapeutical questions are discussed. 1-2% of the children die suddenly or coronary thrombosis. Analogies with infantile polyarteritis nodosa which can be equally lethal and Kawasaki-Syndrome are examined...

Although aseptic meningitis, lethargy and irritability occur frequently in Kawasaki disease and infantile polyarteritis nodosa, other neurological manifestations are rare. The authors report one case of Kawasaki disease and one of infantile polyarteritis nodosa, both associated with acute hemiplegia. Both patients had received courses of oral corticosteroids for their underlying disease prior to the onset of the hemiplegia. Pathological studies, as well as the four previously reported cases, are reviewed.

We report the case of a twelve-year old boy with a disease fitting the diagnostic criteria for Kawasaki disease as defined by the Japanese research committee on this mucocutaneous lymph node syndrome. Although Kawasaki disease has been reported throughout the world, it is more prevalent in Japan. Etiology is unknown. An immunologic mechanism is likely. Main manifestations are an acute febrile mucocutaneous syndrome with enlargement of cervical lymph nodes. Cardiovascular involvement is responsible for death in 1 to 2% of cases and makes prolonged follow-up requisite...

No abstract text is available yet for this article.

An unusual case of fatal myocardial infarction in a neonate due to coronary arteries is compared with two lethal cases of Mucocutaneous Lymph Node Syndrome and/or Infantile Periarteritis Nodosa (MLNS/IPN). The neonatal coronary arteritis was pathologically indistinguishable from typical forms of MLNS/IPN, which cannot be reliably separated and whose strange epidemiologic features as well as their etiology(-ies) remain to be clarified. It is suggested that further research of the etiology of MLNS/IPN should be aware of a possible neonatal variant of this syndrome...

The clinical and autopsy findings of a three-month-old male infant with infantile periarteritis nodosa are described adn the literature reviewed. Different stages of the inflammatory vascular process could be seen in the iliacal, axillary, carotidal, coronary and the small periadrenal-tissue-arteries. Secondary changes were found in the axillary and coronary arteries consisting of aneurysmal dilatation and thrombosis. Fatal myocardial infarction occurred thirty-nine days following hospital admission.

Coronary artery disease of infants and children are reported. The paper deals with congenital coronary anomalies which appear either isolated or in combination of cardiac or vascular malformations. Acquired coronary diseases appear with periarteriitis nodosa, Kawasaki syndrome, arteriopathia calcificans and infantile arteriosclerosis. It is referred to etiology, clinical symptoms and therapy for each group of coronary disease with an emphasis on prevention.

On an infant with the classical signs of Kawasaki-disease (MLNS), in whom after a few days began to show distal demarcations on all four extremities due to arterial malperfusion. Parts of these extremities had to be resected. Histologic evaluation showed an infantile type of periarteriitis nodosa. Later on the child developed multifocal convulsions. The similarities of MLNS and IPN are discussed as well as cerebral vasculitis.