Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

With the continuing advancement in the treatment of childhood leukemia and the lengthened survival of these patients, an increased incidence of abdominal complications has been observed. A retrospective analysis of 364 patients with leukemia treated at the National Taiwan University Hospital from January 1977 through April 1988 was undertaken. Eleven patients (3.0%) developed abdominal complications during their course of disease, including acute appendicitis, intussusception, intestinal perforation, ovarian cyst rupture, etc. All of these patients had abdominal complications during the initial presentation or relapse of leukemia, and 9 (82%) of them had just received chemotherapy. Ten patients (91%) had thrombocytopenia and 7 (64%) had leukopenia. Blood cultures were positive in 5 patients (45%), and gram-negative enteric bacilli were isolated in 4 of them. All 5 septicemic patients had leukopenia or neutropenia. The clinical manifestations were nonspecific and were often masked. Most of the complications occurred in the right lower abdominal structures. Of the 7 children treated surgically, 3 had long term survival. Among the 4 patients who did not receive an operation, only 1 survived for more than 4 weeks. The mean length of survival tended to be longer in patients with additional surgical treatment. Prompt diagnosis and early aggressive treatment, under modern supportive facilities, appear to offer a more favorable outcome.
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PMID:Acute abdomen in childhood leukemia. 197 4

Glycogen storage disease type Ib is a rare metabolic disease caused by a defect of the G6P transporter. Patients suffer from hypoglycemic episodes; growth and developmental delay; osteoporosis; neutropenia; and tendency to infections, ovarian cysts, and liver adenomas. Terminal kidney disease is a rare complication. Liver transplantation has been performed to prevent malignant transformation of hepatic adenomas. We present the case of a female patient with glycogenosis type Ib who had severe hypoglycemic episodes and recurrent infections since early childhood. She became dialysis dependent at the age of 24 years. Kidney transplantation was performed at age 30, and liver transplantation 2 years later. The main indication for liver transplantation were the persistent, therapy-refractory hypoglycemic episodes. The transplanted kidney function is stable. The liver transplantation resulted in the disappearance of hypoglycemic episodes, with the patient leading a normal life and eating a normal diet. The neutropenia did not recover, but there were no more significant infectious episodes after liver transplantation. This is, to the best of our knowledge, the first communication of a dual kidney and liver transplant performed in a patient with glycogenosis type Ib. It confirmed the beneficial effect of liver transplantation on the quality of life of patients with severe hypoglycemia. The transplantation should be attempted earlier in the course of the disease to reduce complications and allow catch-up growth. Hepatocyte transplantation may be considered; however, long-term results seem to be rather poor in the few documented cases.
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PMID:Successful staged kidney and liver transplantation for glycogen storage disease type Ib: A case report. 1717 48