Rapidly involuting congenital haemangioma associated with transient thrombocytopenia and coagulopathy: a case series.

Rapidly involuting congenital haemangioma (RICH) may present with thrombocytopenia, low fibrinogen and elevated fibrin degradation products and D-dimers. Such complications have rarely been reported. We wished to define the clinical characteristics of the thrombocytopenia and coagulopathy associated with RICH, to emphasize the transient nature of this haematological complication and to distinguish these abnormalities from true Kasabach-Merritt phenomenon (KMP). We present a case series of seven patients with large RICH who presented with thrombocytopenia and coagulopathy during the first week of life. Clinical and haematological characteristics were recorded retrospectively. Two of the patients were treated with embolization due to early signs of high-output cardiac failure; four patients received oral corticosteroids in the range of 2 mg kg(-1) daily; one patient did not receive any treatment in the neonatal period, although the tumour was excised at 6 months of age. Two patients with platelet counts lower than 10 x 10(9) L(-1) received a platelet transfusion. There were no bleeding complications and only one patient presented with petechiae. In all seven patients, platelet counts started to increase at > 2 weeks of age and the coagulopathy resolved. We conclude that RICH may present with thrombocytopenia and coagulopathy similar to mild KMP early in the neonatal period. However, in contrast to true KMP, these abnormal laboratory findings are self-limited and are usually not complicated by bleeding problems.

[Congenital syphilis (Statistics of the Hospital del Niño de Buenos Aires of the years 1975 and 1976)]. / Sífilis congénita (estadística del Hospital de Niños de Buenos Aires, de los años 1975 y 1976).

After their study carried out in 24 patients with congenital syphilis, assisted at the Buenos Aires Children's Hospital during 1975-1976, the authors pointed out some epidemiological patterns for the control of syphilis. These patterns, in short are: 1) To create a National law forthe control of the venereal diseases.

[Incontinentia pigmenti. Concerning 9 new cases, 2 of them associated with different genetic syndromes]. / Incontinencia pigmenti. Acerca de nueve observaciones, dos de ellas asociadas a diferentes síndromes genéticos.

Nine girls with Incontinentia Pigmenti are presented, two of them associated to different genetic syndromes. All the patients are female, characteristic of genetic transmission, linked to dominant x, lethal for males; one of the patients also presents the Larsen Syndrome and another has got her illness associated to the Chotzen Syndrome; in these cases we can observe the coexistence of two syndromes, genetically determined. A revision of the clinical, histopathological and genetic characteristics of this disease is made.

Blue rubber bleb nevus (Bean syndrome): evolution of four cases and clinical response to pharmacologic agents.

Blue rubber bleb nevus (BRBN) syndrome is a rare disorder characterized by distinctive cutaneous and gastrointestinal vascular malformations. The latter may lead to bleeding complications. We followed four affected children for at least 5 years. The evolution of their disease and the value of pharmacologic agents (steroids, interferon) in the management of some of these patients are discussed.

Sifilis congenita, periodo 1975-1979 en el Hospital de Ninos de Buenos Aires. / Congenital syphilis, period 1975-1979 in the Hospital de Ninos de Buenos Aires

Los autores refieren la estadistica del periodo comprendido entre 1975 y 1979 de ninos internados con diagnostico de sifilis congenita precoz, en el Hospital de Ninos de Buenos Aires. Durante ese periodo se trataron 54 pacientes, en su gran mayoria provenientes de la ciudad de Buenos Aires y su conurbano, pertenecientes en general, a grupos socioeconomicos con menores posibilidades. Las manifestaciones clinicas mas frecuentes fueron hepatomegalia en 51 ninos, ictericia en 49, esplenomegalia en 47 y anemia en 36, mientras que las expresiones dermatologicas predominantes fueron el penfigoide en 27 ninos, la roseola y la sifilides papulosas en 24. En los 54 pacientes, la VDRL fue reactiva con valores que oscilaron entre las 16 y 1.024 unidades, obteniendose la curacion de los mismos con el empleo de penicilina G. sodica con una dosis total de 3.000.000 de unidades

Displasia ectodermica hipohidrotica. / Hypohidrotic ectodermal dysplasia

La displasia ectodermica hipohidrotica o anhidrotica es una enfermedad genetica ligada al X o que se transmite por herencia autosomica recesiva caracterizada por ausencia del sudor, denticion defectuosa e hipotricosis, que constituyen la triada caracteristica. Estos enfermos presentan una facie llamativa, con frente olimpica, puente nasal prominente y aplanado, procesos alveolomaxilares hipoplasicos, mejillas hundidas y marcados pliegues delgados periarticulares. La piel es seca, fina y arrugada, con hiperpigmentacion.Las alteraciones del cabello y el vello consisten en alopecia de grado variable, con pelo ralo, escaso, rubio, fino, seco o fragil.Se presentan tres pacientes afectados con esta enfermedad, detallandose sus caracteristicas clinicas, histologicas y geneticas

Hipoplasia dermica focal en una recien nacida. / Focal dermal hypoplasia in a newborn infant

Se presenta un caso de hipoplasia dermica focal en una recien nacida internada en nuestro Hospital. En su primer examen se comprueban lesiones ectodermicas ampollares y ulceradas repartidas en tronco y miembros con disposicion lineal, zoniforme y alteraciones mesodermicas oculares y esqueleticas

Manifestaciones cutáneas: clave diagnóstica en pacientes oncológicos pediátricos con candiasis sistémica / Cutaneous manifestations: diagnostic keys in pediatroc oncologic patients with systemic candidiasis

Se describen tres pacientes con leucemia aguda y neutropenia prolongada que presentaron lesiones cutáneas en el curso de una candidiasis sistémica.Se destaca el valor de la biopsia de la lesión para su estudio microbiológico y anatomopatológico a fin de confirmar el diagnóstico de la infección candidiásica y orientar la terapéutica en pacientes pediátricos inmunocomprometidos

Método rapido para el análisis de variantes genomicas del virus del papiloma / Rapid method for variant genome analysis of papilloma virus

Se ha desarrollado un método rápido y eficiente para la extracción de ADN de virus del papiloma presente en tumores. La cantidad y la calidad del ADN extraído permitió realizar el análisis directo del genoma viral con tres enzimas de restricción. Se discute la utilidad de esta técnica para realizar estudios clínicos