[Giant aneurysms of the pulmonary artery and peripheral venous thrombosis (Hughes-Stovin syndrome): regression with immunosuppressant therapy]. / Aneurismas gigantes de la arteria pulmonar y trombosis venosa periférica (síndrome de Hughes-Stovin): regresión con tratamiento inmunosupresor.

We report the case of a young Afro-Caribbean patient who complained of dry cough and low-grade fever. A chest film upon admission showed bilateral hilar masses in the lungs, which a CT scan with contrast medium confirmed were giant aneurysms of the pulmonary artery. The most relevant past history was deep venous thrombosis six months before admission. Hughes-Stovin syndrome was diagnosed and treatment was started with prednisolone and azathioprine. A scan 6 months after treatment ended showed the aneurysms had disappeared. Hughes-Stovin syndrome is characterized by pulmonary artery aneurysms and peripheral deep venous thromboses. Severe lung complications can include hemoptysis and thromboses inside the aneurysms. Hughes-Stovin syndrome has been considered a variety of BehCet's disease.

Aneurismas gigantes de la arteria pulmonar y trombosis venosa periférica (síndrome de Hughes-Stovin): regresión con tratamiento inmunosupresor / Giant aneurysms of the pulmonary artery and peripheral venous thrombosis (Hughes-Stovin syndrome): regression with immunosuppressant therapy

Presentamos el caso de un paciente joven de origen afrocaribeño, que consultó por tos seca y febrícula. En la radiografía del tórax de ingreso se observaron masas pulmonares hiliares bilaterales que, al ser evaluadas posteriormente con TC de tórax con contraste, se confirmó que correspondían a aneurismas gigantes de la arteria pulmonar. Su antecedente más importante era una trombosis venosa profunda detectada 6 meses antes de su ingreso. Se estableció el diagnóstico de síndrome de Hughes-Stovin y se inició tratamiento con prednisolona y azatioprina. La TC realizada 6 meses después de tratamiento ininterrumpido demostró la desaparición de los aneurismas. El síndrome de Hughes-Stovin se caracteriza por aneurismas de las arterias pulmonares y trombosis venosa profunda periférica. Puede cursar con complicaciones pulmonares graves, como hemoptisis y trombosis dentro de los aneurismas. Se ha considerado una variedad de la enfermedad de Behçet (AU)

Ascaris lumbricoides infestation as a cause of intestinal obstruction in children: experience with 87 cases.

PURPOSE: The purpose of this study is to describe the occurrence and management of bowel obstruction caused by Ascaris lumbricoides, a common parasite in warm climates that affects children with limited socioeconomic means. METHODS: Eighty-seven patients with intestinal infestation owing to Ascaris lumbricoides were treated in the past 10 years (1984-1994). There were 48 (55.2%) girls and 39 (44.8%) boys. The mean age was 4.6 years, with peak occurrence at 2 years of age. Half the patients had a history of passing worms by mouth or anus. The majority of patients, 64 (73.5%), presented with a subacute clinical course; 23 (26.5%) had acute presentation, with severe abdominal pain, fever, dehydratation, vomiting, and abdominal distension and required vigorous fluid resuscitation and emergency surgical intervention. Diagnosis was achieved with plain abdominal roentgenograms, which showed a "whirlpool" pattern of intraluminal worms in most cases. RESULTS: Six patients had been incorrectly diagnosed as having appendicitis; two cases had appendicitis owing to Ascaris in the cecum and distal ileum. The majority of cases with a subacute presentation respond to medical (anthelmintic) management using oral administration of racine oil and piperazine. Of the 23 patients taken to the operating room, 11 required external "milking" of the obstructing bolus of worms from the ileum into the colon, six required intestinal resection and end-to-end anastomosis, six had an appendectomy, and three needed an enterotomy to manually extract the worms. In one case, initial management consisted of an ileostomy because of intraoperative instability owing to sepsis. Subsequently, after stabilization and treatment with anthelmintic agents, closure of the stoma with an end-to-end ileocolostomy was performed. There was no significant postoperative morbidity or mortality. CONCLUSION: These observations suggest that physicians should have a high index of suspicion for parasitic infestation in warm climates where economically deprived children present with symptoms of intestinal obstruction. Ascaris lumbricoides may be the cause of these events in endemic areas. Oral piperazine and racine oil can successfully resolve most subacute cases; however, aggressive resuscitation and prompt surgical intervention in patients with intestinal obstruction result in a satisfactory outcome.

Estudio radiológico de doble contraste del instestino delgado en todas las edades: presentación de 100 pacientes

Presentamos un estudio prospectivo en 100 pacientes, 60 adultos y 40 niños, realizado en los Hospitales San Ignacio e Infantil de la Misericordia; en quienes se practicó un examen radiológico de Doble Contraste del Intestino Delgado. Informamos sobre la técnica y los hallazgos radiológicos, clínicos e histológicos. Se demostró la ventaja sobre el estudio tradicional y su alta sensibilidad, especificidad y poder predicitvo positivo

Cleidocranial dysostosis, severe micrognathism, bilateral absence of thumbs and first metatarsal bone, and distal aphalangia: a new genetic syndrome.

Five patients from three families have a new genetic syndrome. The features include cleidocranial dysostosis, bilateral absence of the thumbs and of the distal phalanges of the fingers, hypoplasia of the first metatarsus, absence of the distal phalanx and hypoplasia of the proximal phalanx of the big toe, pelvic dysplasia, bilateral hip dislocation, a constant facial dysmorphism with sparse hair, peculiar ears, severe micrognathism, and retracted and poorly delineated lips. The existence of consanguinity in two of the families, both with two affected siblings, as well as the lack of sex predilection, allow us to postulate that this syndrome is inherited in a recessive manner.