Shprintzen-Goldberg syndrome. A case report

Major hemorrhage after pulmonary resection is infrequent. It is usually due to an unsecured vessel, and immediate reoperation to control hemorrhage is indicated. Diffuse bleeding from raw chest wall surfaces can complicate pleurectomy. Control of bleeding from this cause is difficult, and standard techniques may fail. Thoracic packing is occasionally needed. The authors describe a technique for thoracic packing. This technique permits removal of thoracic packing at the bedside, and simplifies subsequent management of an empyema, should one develop.     

Pulsatile total cavopulmonary shunt for hypoplastic right heart syndrome with abnormal systemic venous return--a case report

A pulsatile total cavopulmonary shunt was successfully performed on a 5-year-old girl with hypoplastic right heart syndrome associated with abnormal systemic venous return; at the same time, modified mitral valve replacement was performed for mitral regurgitation. The right atrium, tricuspid valve and right ventricle were all extremely dimunitive. The diameter of the tricuspid valve was 50% of normal and the volume of the right ventricle was 8.6% of normal. In addition, there were severe subpumonary stenosis, a restrictive ventricular septal defect (VSD) and an atrial septal defect (ASD). The bilateral superior venae cavae (SVCs) and the hepatic vein drained to the left atrium, and the inferior vena cava was infrahepatically interrupted with a hemiazygos connection to the left superior vena cava. At the operation, each SVC was anastomosed end-to-side to each branch of the pulmonary artery (PA). The restrictive ventricular septal defect and stenotic subpulmonary lesion were left. The diameter of the ASD was reduced from 12 mm to 7 mm. The main PA was neither divided nor banded. The pulsatile blood flow from the left heart to the PA was regurated by a native restrictive VSD and stenotic subpulmonary lesion, and that from the right heart via the ASD was limited by reducing the size of the ASD. These described anatomic arrangements produced adequate antegrade pulsatile flow in the PA, which might prevent the development of pulmonary arteriovenous fistulae and, besides permit transfer of drainage of the hepatic vein from the left to the right atrium via the ASD in future.     

Sweet's syndrome associated with spinal surgical intervention. A case report

STUDY DESIGN: A patient with a medical history of Sweet's Syndrome, an acute neutrophilic dermatosis, was seen at the authors' institution for cervical pain. After undergoing a thorough history-taking and physical examination and after experiencing no relief with conservative therapy, the patient underwent cervical spine surgery. After the surgical procedure, the patient developed multiple cutaneous lesions that were consistent with the findings associated with an acute recurrence of Sweet's Syndrome. OBJECTIVES: To characterize the authors' experience with this unusual histologically documented dermatologic disorder. SUMMARY OF BACKGROUND DATA: Sweet's Syndrome is a rare form of neutrophilic dermatosis characterized by recurrent eruptions of painful, edematous, red, tender plaques that are found predominantly on the torso in middle-aged women. After an extensive literature search, it was noted that this rare and unusual disorder has not been reported previously in association with surgical intervention of any type, including spinal operations. METHODS: The patient's postoperative course was documented, and all medical records were reviewed retrospectively. RESULTS: The patient's rash resolved spontaneously. Solid fusion of C5-C6 occurred. The patient remained neurovascularly intact, and her axial cervical pain decreased significantly from its preoperative levels. CONCLUSIONS: Sweet's Syndrome remains a rare dermatologic disorder, which may complicate a routine postoperative course. Patients with Sweet's Syndrome have an exceedingly high rate of other serious medical illness. The effect of Sweet's Syndrome on physiologic bone healing is unknown. In this patient, there was nonunion of the cervical spine, with eventual solid bony union. Perioperatively, patients with this disorder are treated with oral prednisone and oral antibiotics to prevent secondary complications at the surgical wound.     

Histopathologic findings in autogenous saphenous vein graft wrapping for recurrent tarsal tunnel syndrome: a case report

Autogenous saphenous vein graft wrapping of the tibial nerve has been described as an effective treatment option for failed tarsal tunnel decompression. Various theories have been proposed to explain how this method works, with little histologic evidence to date. A pathologic investigation of a sectioned nerve that had been previously wrapped provides some insight into these proposals.     

Sj?gren''s syndrome associated with autoimmune hepatitis. A case report

We report on a 4-year-old girl with obesity and hyperphagia whose peripheral blood cytogenetic analysis showed mosaicism for a deletion of band 1p36.33. Terminal 1p deletions are rarely reported and this patient represents the first identified case of mosaicism. Given the subtlety of the cytogenetic abnormality and the possibility of mosaicism, the incidence of such deletions has probably been underestimated. While a characteristic phenotype associated with this karyotypic abnormality was described recently, the present report highlights the additional clinical findings of obesity and hyperphagia and the overlap of manifestations with Prader-Willi syndrome.     

Autoimmune polyglandular syndrome type I. A case report

Calcineurin is a Ca2+ calmodulin dependent protein phosphatase which has an important role in the control of intracellular Ca2+ signalling. The protein is a heterodimer of one catalytic (CnA) subunit and one regulatory (CnB) subunit. As suggested by the protein sequence and confirmed by the crystallographic structure, the catalytic subunit of calcineurin (CnA) has high homologies with other protein phosphatases. The regulatory subunit (CnB) belongs to the EF-hand Ca2+ binding protein family. Despite its similarity with calmodulin, it has a different tertiary structure. Calcineurin is the target of two important immunosuppressant drugs: cyclosporin A and FK506. Subsequently, a detailed clarification of the role of calcineurin in the cytokine mediated activation of the T-cells has been possible. The understanding of the role of calcineurin in other cells, in particular neurons, is also progressing rapidly.     

Branched graft technique for middle aortic syndrome. Three case reports

Those who work in the area of vocabularies, like all researchers, bring some assumptions to that work. Such assumptions include both cultural notions and beliefs specific to particular social worlds within the broader society. In this article, assumptions and beliefs expressed by participants during the conference on "Implementation of Nursing Vocabularies in Computer-based Systems" are summarized. Questions are raised concerning the relationship between cultural notions and beliefs and nursing vocabularies.     

Alport syndrome with neurofibromatosis type-I: a case report

We report a 9-year-old boy with repeated fractures of the tibia from age 6 months and microscopic hematuria from age 2 years. His maternal family has a history of nephritis and his paternal family has neurofibromatosis type-I (NF-I). The boy's renal biopsy revealed an irregular attenuation and splitting of the glomerular basement membrane. The skin biopsy was stained with monoclonal antibody against the alpha 5 chain of type IV collagen; the epidermal basement membrane was negative in the boy and segmentally positive in the boy's mother. We conclude that the patient inherited Alport syndrome from his mother and NF-I from his father. We postulate this was a chance association and that this case does not suggest any relationship between the two diseases.     

Bullous pemphigoid as a paraneoplastic syndrome. A case report in renal-cell carcinoma

Paraneoplastic markers in tumor patients may occur at various stages of the disease. While some disorders almost invariably herald an underlying malignancy (obligative marker), most only occasionally do so (facultative marker). The association of bullous pemphigoid with malignancy is controversial. There are only a few reports of bullous pemphigoid associated with a renal cell carcinoma. We diagnosed a renal cell carcinoma in a 74 year old female patient, admitted to the hospital because of bullous pemphigoid. Multiple metastases were found in her lymph nodes, liver, lung and skeleton. The patient died eleven weeks after the first symptoms of bullous pemphigoid appeared.     

HELLP syndrome: undiagnosed in a case of impending rupture of uterus. A case report

Eleven patients with chronic renal failure who were being treated with haemodialysis three times a week were monitored for a total of 34 haemodialysis sessions. Erythrocyte 2,3-biphosphoglycerate (2,3-BPG) concentration was analysed immediately before initiation of bicarbonate haemodialysis and 1 h afterwards. The 2,3-BPG concentration was expressed relative to the haemoglobin tetramer (Hb4) concentration as the 2,3-BPG/Hb4 ratio and compared with blood gas analyses and biochemical variables important for characterizing uraemia. During the first hour of haemodialysis the 2,3-BPG/Hb4 ratio decreased (p < 0.002), but the magnitude of the decrease did not significantly correlate with the 2,3-BPG/Hb4 ratio measured before haemodialysis (p=0.104). The decrease is most likely to be caused by the haemodialysis procedure itself. Mechanical stress on the erythrocytes is believed to cause the 2,3-BPG to escape; it is then removed by haemodialysis. Physiologically, an increase in 2,3-BPG would be expected to counteract the hypoxia which is frequently observed during haemodialysis. However, the present results show the opposite, a decrease in 2,3-BPG. No significant correlation was shown between the haemoglobin concentration and the 2,3-BPG/Hb4 ratio before dialysis (p=0.414). The pH showed a significant positive correlation with the 2,3-BPG/Hb4 ratio before dialysis, whereas the arterial pO2 and the 2,3-BPG/Hb4 ratio before dialysis were insignificantly negatively correlated. The concentrations of calcium, phosphate, creatinine, urea and albumin did not correlate significantly with the change in 2,3-BPG/Hb4-ratio after 1 h. The 2,3-BPG/Hb4 ratio (p=0.03) sampled just before dialysis correlated significantly and positively with the total weekly dosage of erythropoietin given to the patients.     

Sneddon syndrome: a case report

Doxorubicin is cardiotoxic and its use must be monitored carefully. Incidence of refractory cardiac failure is shown to increase once the cumulative dose exceeds 450 mg/m2. However, significant decline of ejection fraction (EF) may occur even at lower dose levels. EF was monitored using Multigated Radionuclide Angiography (MUGA) scan of all consecutive lung cancer patients, treated with Doxorubicin based regimens. Thirteen of 82 patients showed a significant (more than 15%) decline of left ventricular EF. The dose of doxorubicin producing this decline ranged between 91-180 mg/m2. Actual decline in EF ranged between 16-45%. Only 5 of 13 patients developed symptoms attributable to the cardiac disease. Doxorubicin can alter EF significantly in lung cancer patients at levels well below which are considered 'safe'. The reason for massive decline in ejection fraction in these patients has been hypothesized.     

Hypnotherapy for the child with chronic eczema. A case report

Chronic eczema in a child usually produces much discomfort and distress. Associated behaviour problems further complicate the issue. A comprehensive approach to treatment is therefore essential. Hypnotherapy may be a useful adjunct in the overall management. An illustrative case history is presented. Combining hypnotherapy with child guidance and medical treatment led to the relief of symptoms and improved behavioural pattern in a boy aged 10 years.     

Two-step procedure in Budd-Chiari syndrome with severe intrahepatic vena cava stenosis: vena cava stenting and portocaval shunt

Budd-Chiari syndrome is characterized by hepatic venous outflow obstruction, which often leads to death as a result of portal hypertension and liver failure. Venous decompressive shunt surgery and liver transplantation represent efficient surgical treatments of Budd-Chiari syndrome. In the case presented here, severe intrahepatic compression of the inferior vena cava (IVC) was caused by the hypertrophic caudate lobe. A mere portocaval shunt was not feasible because of a large pressure gradient across the intrahepatic stenosis. A two-step procedure with preoperative radiological dilation and stenting of the intrahepatic IVC followed by a portocaval shunt was successfully performed. Consequently, liver transplantation and its subsequent immunosuppression could be avoided.     

Kabuki make-up syndrome and report of a case with hydrocephalus

We report a 22-year-old man with hydrocephalus caused by aqueductal stenosis. The patient was diagnosed with Kabuki make-up syndrome based on associated findings such as a peculiar facies, postnatal growth deficiency, brachydactyly of the fifth fingers, undescended testes, and malrotation of the colon. Kabuki make-up syndrome, recognized in Japan in 1981, is characterized by five cardinal manifestations: a peculiar facies, skeletal anomalies, dermatoglyphic anomalies, slight to moderate mental retardation, and postnatal dwarfism. Neurological anomalies have been reported to include neonatal hypotonia, feeding problems, seizures, West syndrome, microcephaly, brain atrophy, GH deficiency, precocious puberty, delayed sexual development, and diabetes insipidus. Aqueductal stenosis may be caused by part of the series of midline anomalies. Physicians should pay attention to associated anomalous characteristics suggesting a malformation syndrome when they encounter nontumoral aqueductal stenosis in adolescents or adults.     

Oral problems associated with CREST syndrome: a case report

A patient with CREST syndrome is described, with such severe limitation of mouth opening that extractions were required before upper gastrointestinal endoscopy could be performed. Features of CREST syndrome of importance to the dental and oral surgeon are described.