Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of infantile osteopetrosis are reported. Both were males aged four and eight months at presentation. They presented with osteosclerotic change of the bone, leukoerythroblastic anemia, optic atrophy, hepatosplenomegaly and frequent infection. The histology of the bone showed thickened bone trabeculae with little osteoclastic activity, although in one patient the number of osteoclasts increased, while in the other they did not. One received a bone marrow transplant (BMT) but died from disseminated cytomegaloviral infection, pulmonary hemorrhage and sepsis. The post-transplant marrow histology showed evidence of engraftment and osteoclastic activity. The other only received a course of prednisolone, which was of little help. His condition has followed a natural course with progressive visual impairment and marrow failure. Our cases suggest that infantile osteopetrosis should be taken into consideration in dealing with infants who present with early marrow failure and that patients of infantile osteopetrosis should receive BMT. BMTs appear to be the only cure. They should be given as early as possible to avoid major consequences and severe infection.
 ...
PMID:Infantile osteopetrosis: report of two cases. 135 41

We describe a case of Pseudallescheria boydii endocarditis involving the pulmonic valve in an orthotopic liver transplant recipient. The patient required transplantation because of hepatic failure secondary to chronic active hepatitis B. His postoperative course was complicated by surgery for gastric and duodenal ulcers, persistent fever, and, ultimately, sepsis leading to oliguric renal failure. Two days before death, the patient experienced complete heart block, and an echocardiogram revealed pulmonic valve thickening and an endocardial mass along the left side of the septum. At autopsy the patient was found to have a vegetation on the pulmonic valve and a septal abscess. There were multiple fungal emboli found throughout other organs, and P. boydii was obtained on culture. This unique association between pulmonic valve endocarditis and myocardial septal abscess is discussed. In addition, review of the five previous cases of P. boydii endocarditis reveals that this rare infection is associated with immunosuppression and prosthetic devices.
 ...
PMID:Pseudallescheria boydii endocarditis of the pulmonic valve in a liver transplant recipient. 144 84

Samuel Pepys, as a young man, developed a bladder stone and, by the age of 25 years, realised that only surgery could deliver him from his agony. The chances of success in an age that was ignorant of sepsis were slender, but he opted for surgery. The operation, carried out through the perineum without anaesthetic by a master barber surgeon, was successful and Pepys survived. Although left sterile, he was far from impotent and he went on to achieve fame and fortune as Secretary to the Navy and President of the Royal Society. His greatest fame came after his death with the publication of his diary, which was to become one of the best known and best loved books in the language.
 ...
PMID:Samuel Pepys and his bladder stone. 146 56

We describe an adult patient who developed persistent hypercalcemia while bedridden for more than three months with pancreatitis and sepsis. On the basis of hypercalciuria, suppressed serum intact PTH, suppressed serum 1,25-dihydroxy vitamin D3 and no clinical evidence of malignancy, the diagnosis of immobilization hypercalcemia was established His hypercalcemia improved during treatment with saline, calcitonin and/or etidronate. With active mobilization and weight-bearing exercises, serum calcium finally normalized. We discuss clinical and laboratory features as well as current modalities of treatment of this rare form of hypercalcemia in adults.
 ...
PMID:Immobilization hypercalcemia in an adult patient with pancreatitis and sepsis: case report. 148 89

A steroid binding capacity assay and a radioimmunoassay were both used to measure corticosteroid binding globulin (CBG) in serum samples from 22 patients with sepsis. An approximately 50% discordancy between the two values in one patient suggested the presence of a CBG variant with reduced affinity for cortisol, and this was confirmed by Scatchard analysis. We therefore used the polymerase chain reaction to amplify exons that encode for human CBG from the genomic DNA of this patient. This revealed two mutations within the coding sequences: one of which results in a Leu----His substitution at residue 93 and another which encodes a Ser----Ala substitution at residue 224 of the human CBG polypeptide. To assess the impact of each substitution on the steroid binding affinity of CBG, each mutation was introduced separately into a normal human CBG cDNA, and the normal and mutated cDNAs were expressed in Chinese hamster ovary cells. Scatchard analysis of the CBG produced in culture indicated that the His93 mutation (Kd = 2.24 +/- 1.75 nM) reduced the cortisol binding affinity of CBG (mean +/- SD) significantly (P less than 0.024) when compared to normal CBG (Kd = 0.64 +/- 0.31 nM), while the Ala224 mutation (Kd = 0.63 +/- 0.33 nM) did not influence cortisol binding affinity. We therefore conclude that residue 93 may play an important role in determining the structure of the CBG steroid binding site.
 ...
PMID:A Leu----His substitution at residue 93 in human corticosteroid binding globulin results in reduced affinity for cortisol. 150 7

Infective endocarditis is uncommon in young children, especially in the absence of structural heart disease. We report the case of a 2-year-old boy who presented with acute rupture of the mitral valve chordae 6 weeks after an episode of Fusobacterium necrophorum septicemia. His heart had been structurally normal before. Mitral valve replacement was successfully performed. This is the first recorded case of endocarditis in a child caused by necrobacillosis.
 ...
PMID:Endocarditis with acute mitral regurgitation caused by Fusobacterium necrophorum. 151 43

A case is described of an HIV+ man who was successfully treated for Hodgkin's lymphoma, but who later developed non-Hodgkin's lymphoma 3 years later when his immune system became suppressed. The patient was 22 years old when he presented with fever, asthenia, weight loss, and cervical lymphadenopathy. With Hodgkin's lymphoma he also had positive serology for HIV and hepatitis B. He was treated with alternate courses of MOPP and ABVD chemotherapy. In 1990 he again appeared with high fever, progressive cervical, axillary and inguinal lymphadenopathy, with hilar and mediastinal lymph node enlargement on x-ray. CD4 lymphocytes were 577/cubic mm, and the CD4/CD8 ratio was 0.57 (normal 1.8). His cervical lymph node biopsy was classified as non-B non-T large-cell anaplastic lymphoma which was EBV-positive. A Western Blot was positive for small amounts of p24 and p18 antigens. The man was treated with MACOP-B chemotherapy, with some results, but died of sepsis 6 weeks later. The relationships between Hodgkins and non-Hodgkin's lymphoma, the timing of the neoplasm in the course of HIV infection, and the possible re-activation of hepatitis virus were discussed.
 ...
PMID:Non-Hodgkin's lymphoma after prolonged remission of Hodgkin's disease in an HIV-infected patient. 166 42

Physicians admitted a 45-day old boy to King Khalid University Hospital in Riyadh, Saudi Arabia who had had a fever (39.8 degrees Celsius) for 2 days. He was irritable and did not feed very well at the breast. He was a healthy full term infant. The physicians could not identify an infection in the infant. 2 weeks before the infant became ill, the mother had a fever, progressive malaise, and right hip pain for 5 days. Based on a positive Brucella serology, her physician treated her with tetracycline and streptomycin. She exclusively breastfed the infant during the illness. Neither the mother nor the infant had any contact with farm animals, but a friend did give the mother raw goat milk 2 weeks postpartum. 79% of the white blood cell count contained lymphocytes. They believed he had bacterial sepsis so they treated him with intravenous ampicillin and cloxacillin. His temperature peaked daily between 38-39 degrees Celsius for the 1st 3 days. After hearing of the mother's illness with brucellosis and since the blood, urine, and cerebrospinal fluid cultures were negative for common bacterial pathogens, the physicians then administered oral trimethoprim-sulphamethoxazole and rifampicin for 6 weeks. His condition improved quickly and by day 7 the fever had subsided. 2 weeks after admission, his Brucella agglutination titer was 1:160 and his blood culture grew Brucella melitensis. At the same time, they measured the mother's blood and breast milk titers which were both positive (1:320 and 1:640 respectively). They could not isolate B. melitensis in either her blood or breast milk, however. Perhaps the antibiotics wiped out the organisms. 1 year after admission, the boy was fine. Seroconversion occurred within 2 weeks which may mean that he acquired brucellosis recently and postnatally. The physicians believed that the only route of transmission was breast milk.
 ...
PMID:Probable breast-milk borne brucellosis in a young infant. 170 49

Neutropenia in the newborn is often associated with sepsis, maternal hypertension, or prematurity. We describe a 654-g infant born at 30 weeks' gestation by cesarean section due to severe maternal hypertension. His course was complicated by five episodes of sepsis, including three with group B streptococcus. The results of hematologic and immunologic studies were normal except that absolute neutrophil counts were low (less than 1 x 10(9)/L) with intermittent increases during sepsis. Human recombinant granulocyte colony-stimulating factor administered subcutaneously (10 micrograms/kg per day initially) resulted in an absolute neutrophil count of greater than 30 x 10(9)/L within 2 weeks. The dosage was lowered and the absolute neutrophil counts were maintained at 8 to 12 x 10(9)/L with no further septic episodes. The human recombinant granulocyte colony-stimulating factor therapy was discontinued after 7 months, and the patient remained healthy with an absolute neutrophil count of greater than 2 x 10(9)/L. Thus, treatment with human recombinant granulocyte colony-stimulating factor may be useful as a temporary measure for neonatal neutropenia associated with sepsis. A controlled, clinical trial is warranted.
 ...
PMID:Neutropenia in an extremely premature infant treated with recombinant human granulocyte colony-stimulating factor. 171 73

The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS.
 ...
PMID:Early bone marrow transplantation in an infant with Wiskott-Aldrich syndrome. 179 57


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>