Latin American consensus on the supportive management of patients with severe combined immunodeficiency.

Severe combined immunodeficiency (SCID) represents the most lethal form of primary immunodeficiency, with mortality rates of greater than 90% within the first year of life without treatment. Hematopoietic stem cell transplantation and gene therapy are the only curative treatments available, and the best-known prognostic factors for success are age at diagnosis, age at hematopoietic stem cell transplantation, and the comorbidities that develop in between. There are no evidence-based guidelines for standardized clinical care for patients with SCID during the time between diagnosis and definitive treatment, and we aim to generate a consensus management strategy on the supportive care of patients with SCID. First, we gathered available information about SCID diagnostic and therapeutic guidelines, then we developed a document including diagnostic and therapeutic interventions, and finally we submitted the interventions for expert consensus through a modified Delphi technique. Interventions are grouped in 10 topic domains, including 123 "agreed" and 38 "nonagreed" statements. This document intends to standardize supportive clinical care of patients with SCID from diagnosis to definitive treatment, reduce disease burden, and ultimately improve prognosis, particularly in countries where newborn screening for SCID is not universally available and delayed diagnosis is the rule. Our work intends to provide a tool not only for immunologists but also for primary care physicians and other specialists involved in the care of patients with SCID.

[Guía latinoamericana para el diagnóstico y tratamiento de alergia a las proteínas de la leche de vaca (GL-APLV)].

Cow's milk allergy (CMA) is an immune-based disease that has become an increasing problem. The diagnosis and management of CMA varies from one clinical setting to another and represents a challenge in pediatric practice. In addition, because nonallergic food reactions can be confused with CMA symptoms, there is an overdiagnosis of the disease. In response to these situations, pediatric specialties from recognized institutions throughout Latin America decided to develop a clinical guideline for diagnosis and management of cow's milk allergy. These guidelines include definitions, epidemiology, pathophysiology overview, clinical and evidencebased recommendations for the diagnosis and treatment of CMA. They also include prevention and prognosis sections and identify gaps in the current knowledge to be addressed through future research.

[Brain hemorrhage in a patient with Kawasaki disease]. / Hemorragia cerebral en un paciente con enfermedad de Kawasaki.

Kawasaki disease is an acute, self-limiting vasculitis of unknown origin, characterized by fever, palms and soles edema, cervical lymphadenopathy, strawberry tongue, and non-exudative conjunctivitis. It is a multisystemic vasculitis that affects predominantly infants and young children. The most feared complication is the development of coronary aneurysms that occurs up to 25% of untreated patients; however there are reports of extra coronary involvement. Herein we present the case of a 2 year-old girl who had a severe symptomatology and persistent fever despite intravenous gammaglobulin. Two years later she presented right hemiparesia and headache, with data from CAT and MRI suggestive of brain mass and deviation of the midline, secondary to left frontoparietal haemorrhage that was treated with a craniotomy. She was discharged on prednisone, ASA and rehabilitation.

Treatment of Kimura disease with intravenous immunoglobulin.

Kimura disease is an uncommon chronic inflammatory condition of unknown etiology and is characterized by painless subcutaneous nodules, usually affecting the head and neck, eosinophilia, and markedly elevated immunoglobulin E levels. Several reports have described the main modalities of treatment; both corticosteroids and surgery have provided good results, but occasionally corticosteroids cannot be tapered as the disease flares up. We report here the case of an 8-year-old boy diagnosed with Kimura disease who was successfully treated with 1 dose of intravenous immunoglobulin as a steroid-sparing agent.

Intravenous immunoglobulin therapy for hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus in a child.

Hypocomplementemic urticarial vasculitis is a type of urticarial vasculitis with multisystemic involvement and poor prognosis, sometimes associated with systemic lupus erythematosus. Several therapies have been attempted with no consensus on an effective therapeutic regimen. Intravenous immunoglobulin has been used in severe manifestations of systemic lupus erythematosus and recently in hypocomplementemic urticarial vasculitis. We present a 7-year-old girl with hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus and pneumonia who responded favorably to intravenous immunoglobulin.

[Reactivation of the scar of BCG vaccination in Kawasaki's disease: clinical case and literature review]. / Reactivación de la cicatriz de la vacuna BCG en la enfermedad de Kawasaki: caso clínico y revisión de la bibliografía.

Kawasaki disease is an acute febrile multisystemic vasculitis affecting children that can affect the coronary arteries. Routine BCG vaccination in Mexico leads to a 99% coverage in infants younger than 1 year. We present a case of Kawasaki disease with skin lesions at the site of BCG. Clinicians should be aware of this clinical manifestation that could help diagnose atypical or incomplete cases of the disease.

Corticosteroid therapy for refractory infections in chronic granulomatous disease: case reports and review of the literature.

BACKGROUND: Chronic granulomatous disease (CGD) is a primary phagocyte immunodeficiency. It is often accompanied by an exuberant and aberrant inflammatory response, with granulomata and obstruction of the gastrointestinal and genitourinary tracts and inflammatory bowel disease. Although corticosteroids are successful in managing the obstructive and inflammatory disorders of CGD, they are not ordinarily used for the management of infection because of the possibility of further compromising the patient's immune system. OBJECTIVES: To discuss the pros and cons of the use of corticosteroids for the treatment of infections in CGD. METHODS: We describe 2 patients with CGD and refractory infections who were successfully treated with systemic corticosteroids in addition to antimicrobial agents. We also review the medical literature in which corticosteroids have been used for CGD infection. RESULTS: Our cases add to 3 other reports in which antibiotics and corticosteroids were used successfully in patients with CGD. However, in the presence of a potential pathogen, notably, aspergilla, corticosteroids may mask or favor dissemination of the fungus, especially in adults. CONCLUSIONS: Corticosteroids may play an important adjunctive role in CGD refractory infections.

Catastrophic Kawasaki disease or juvenile Polyarteritis nodosa?

OBJECTIVE: Juvenile Polyarteritis nodosa (PAN) and Kawasaki Disease (KD) are disseminated vasculitides of unknown cause affecting small- and medium-sized vessels in children. We present an unusually severe case that fulfilled criteria for both KD and PAN. The diagnosis, overlapping clinical features, and treatment options for the 2 diseases are discussed. METHODS: A 3-year-old girl with systemic vasculitis is presented. We compare our case to 4 other cases reported in the literature which presented with a similar diagnostic dilemma. A review of the medical literature and a qualitative analysis of the diseases were performed, with emphasis on overlapping features, atypical cases, and treatment options. RESULTS: Many features of KD and PAN are shared; however, there are some clinical features that could help differentiate one from the other. Fever, weight loss, rash, abdominal pain, arthritis, coronary arteritis, peripheral gangrene, anemia, leukocytosis, thrombocytosis, and elevated C-reactive protein are among many of the features that are shared by both diseases. However, KD also has unique clinical features that include conjunctivitis, changes in the lips and mouth, desquamation of the fingertips, and gallbladder hydrops, whereas renal involvement in KD is rare. CONCLUSIONS: Occasionally juvenile PAN and KD share clinical manifestations, and when they do, it may be impossible to differentiate between them. Treatment should be directed according to the severity and persistence of these clinical manifestations.

Hyper-IgE syndrome and autoimmunity in Mexican children.

Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by recurrent skin abscesses, recurrent pneumonia with pneumatocele formation, eczema, eosinophilia, and elevated levels of serum IgE. Patients with the autosomal recessive (AR) form of HIES appear to be prone to developing autoimmune diseases. We present two cases of HIES with autoimmune complications; one case was a product of a consanguineous marriage, the other one was a sporadic case. The first patient presented with recurrent episodes of erythema nodosum, warts, bronchiolitis obliterans and thrombocytopenia. The second patient developed glomerulonephritis resulting in endstage renal failure. She later developed malar rash, oral ulcers, cerebral infarcts with vasculitis and positive ANA, anti-dsDNA, and antiphospholipid antibodies. We discuss the dilemma in treating patients who present with both primary immunodeficiency and autoimmunity.

Pediatric Churg-Strauss syndrome in Mexico.

Churg-Strauss syndrome (CSS) is one of the rarest forms of vasculitis, and very rarely presents in the pediatric population. We present two cases of childhood CSS, both with hepatic and cardiac involvement. To our knowledge, these are the first two cases of CSS in the pediatric population described in Mexico.

[Superantigens and toxic shock syndrome. A report of three cases treated with intravenous gammaglobulin]. / Superantígenos y síndrome de choque tóxico. Comunicación de tres casos tratados con gammaglobulina intravenosa.

The superantigens cause a massive polyclonal activation of T-cells, producing an immense liberation of proinflamatory cytokines, which induces the clinical data of toxic shock syndrome. In international studies the administration of polyclonal intravenous gammaglobulin has been observed to diminish the mortality 50 to 20%. But at the present it has not been reported in Mexico the clinical effectiveness of this therapeutic modality in toxic shock syndrome. We report three cases of toxic shock syndrome treated with gammaglobulin intravenous, and we describe their favorable clinical evolution.