Pulmonary artery sling.

A case of pulmonary artery sling in a 1-year-old boy, treated by resection of the left pulmonary artery (LPA) at its origin from the right pulmonary artery (RPA) and reanastomosis of the LPA to the main pulmonary artery (MPA), is reported. In this patient the LPA-MPA anastomosis was demonstrated to be patent in a follow-up pulmonary angiogram six months after the operation. Pulmonary artery sling should be kept in mine when assessing respiratory distesss syndrome in children.

Cor triatriatum associated with large atrial septal defect and partial anomalous pulmonary venous return. Case report.

A rare variant of cor triatriatum is presented with a large true atrial septal defect and partial anomalous pulmonary venous return into the right atrium. The correct diagnosis was made at the operation and abnormal left atrial septum was excised completely and a new interatrial septum was created with pericardial patch in such a position that the abnormally drained right upper pulmonary vein was left in the left atrium. It was thought to use the abnormal left atrial septum to close the atrial septal defect by excising only the right lateral border of this abnormal septum and resuturing it to the right atrial wall to close the true atrial septal defect. This thought could not be realized because of the small size of this abnormal septum and large size of the atrial septal defect. This technique can be realized in small or medium sized atrial septal defects associated with cor triatriatum.

Mucocele of the anterior lingual salivary glands: from extravasation to an alarming mass with a benign course.

Mucoceles are common cystic lesions in the oral cavity. However, mucoceles located on the ventral surface of the tongue originating from anterior lingual salivary glands are rare. Only 24 cases were reported in the medical literature, of which most were relatively small lesions measuring less than 20 mm in diameter. This report describes a large-sized mucocele of the anterior lingual salivary glands in an 8-year-old boy who was treated by surgical excision of the lesion. The authors emphasize the importance of preoperative awareness and en bloc excision of the lesion even in asymptomatic patients to avoid further enlargement and complications.

Congenital insensitivity to pain with anhidrosis.

Congenital insensitivity to pain with anhidrosis is one of a group of rare diseases termed hereditary sensory-motor neuropathies. Primary clinical features of this entity include congenital analgesia, inability to sweat, and mental retardation. Besides the rarity of these clinical entities, difficulty in evaluating the sensory disturbances, especially in small children, makes the diagnosis a clinical problem. In this article a 3-year-old boy, with consanguineous parents and no family history of the disorder, who was evaluated for two years because of ulcerating lesions on his knees, is presented. Physical examination revealed deep ulceration on his knees and scars from burns on his neck and scalp. Moderate mental retardation and analgesia were noted. There was symmetrical loss of pain and touch sensation on his hands and feet. Electromyographic examination showed absence of action potentials of the ulnar and sural nerves, decrease in the sensory and motor nerve conduction velocities, and amplitude of action potentials. The result of the application of pilocarpine showed anhidrosis. His skin and nerve biopsy specimens were also examined.

Congenital insensitivity to pain with anhidrosis.

Congenital insensitivity to pain with anhidrosis is a rare disorder. Its primary clinical features include congenital analgesia, which leads to self-mutilation; inability to sweat, which leads to defective thermoregulation; and mental retardation. A five-year-old boy with consanguineous parents and no family history of the disorder presented with ulcerating lesions on both knees and elbows. His family had discovered the lack of sensation to pain and anhidrosis. Physical examination revealed ulcers on both knees and elbow, self-mutilation of the tongue, fingers, and toes. Sensation to touch was intact and lacrimation was normal. Moderate mental retardation and analgesia were noted in an otherwise normal neurologic examination. The results of electromyographic examination were normal and the application of pilocarpine showed anhidrosis. A skin biopsy specimen was also examined.

Nifedipine in the treatment of acrodynia.

A case of acrodynia in an eight-and-a-half-year-old girl is presented whose symptoms--profuse perspiration, swelling, desquamation, pain, itching of the extremities, pinkish color of the nose and cheeks and hypertension--responded sharply to nifedipine therapy.

Hemophilic pseudotumor: a case report.

Hemophilic pseudotumor has been defined as a progressive cystic swelling involving muscle which is produced by recurrent hemorrhage and accompanied by roentgenographic evidence of bone involvement. Pseudotumor is a rare complication of hemophilia, and, therefore, we present a case of a six-year-old male hemophiliac with a cyst in the left distal radius.

Intestinal involvement and vasculopathy in von Recklinghausen's neurofibromatosis.

Involvement of the gastrointestinal system and vascular lesions are well-known features of von Recklinghausen's disease, but they are rarely detected in childhood. We report a case of von Recklinghausen's disease with intestinal involvement. The excised right hemicolectomy material of a 16-year-old girl was examined, and diffuse neurofibromatous proliferation with ganglioneuromatous features were observed. Vasculopathy was also seen in some arteries throughout the intestinal segment.

[Importance of vaccination and bacteriological studies in diphtheria epidemics]. / Difteri epidemilerinde asilama ve bakteriyolojik incelemelerin önemi.

The Ouchterlony - Elek gel diffusion test was performed in twenty-nine patients having a positive culture result for diphtheria bacilli on Loeffler and tellurite agars in January and February, 1984 and toxin production was detected in 20 of them. Except one adult patient, the cases ranged in age 2 and 12 years. In 14 patients who have received at least one immunization with diphtheria vaccine had no complication, besides three patients without immunization in whom two received antibiotics plus antitoxin therapy had carditis and soft palate paralysis. Five patients in the non-immunized group and 6 patients in the group received at least one immunization had diphtheritic membrane. In spite of three patients with cardiac and neural complications, with early diagnosis and beginning therapy with antibiotics and antitoxin before the result of in vitro toxigenicity test, O% mortality rate was obtained.