Culprit for recurrent acute gastrointestinal massive bleeding: “Small bowel Dieulafoy’s lesions” - a case report and literature review

A Dieulafoy's lesion is a dilated, aberrant, submucosal vessel that erodes the overlying epithelium without evidence of a primary ulcer or erosion. It can be located anywhere in the gastrointestinal tract. We describe a case of massive gastrointestinal bleeding from Dieulafoy’s lesions in the duodenum. Etiology and precipitating events of a Dieulafoy’s lesion are not well known. Bleeding can range from being self-limited to massive life- threatening. Endoscopic hemostasis can be achieved with a combination of therapeutic modalities. The endoscopic management includes sclerosant injection, heater probe, laser therapy, electrocautery, cyanoacrylate glue, banding, and clipping. Endoscopic tattooing can be helpful to locate the lesion for further endoscopic re-treatment or intraoperative wedge resection. Therapeutic options for re-bleeding lesions comprise of repeated endoscopic hemostasis, angiographic embolization or surgical wedge resection of the lesions. We present a 63-year-old Caucasian male with active bleeding from the two small bowel Dieulafoy’s lesions, which was successfully controlled with epinephrine injection and clip applications.     

Seizure due to somatostatin analog discontinuation in a case diagnosed as congenital hyperinsulinism novel mutation

The most common reason for refractory hypoglycemia in newborns is congenital hyperinsulinism. We report a girl with congenital hyperinsulinism due to novel homozygous mutation (c.2041-25 G>A; aberrant splicing mutation) in the ABCC8 gene encoding SUR1 and during somatostatin analog (octreotide) discontinuation developed by nonhypoglycemic seizures. The newborn (birth weight of 3,750 g) was referred to our clinic because of hypoglycemic seizures at 4 h postnatal. On admission, blood glucose was 24 mg/dL and intravenous glucose infusion was started. The patient's insulin level was 27 mIU/mL during the hypoglycemic period. Phenobarbital (5 mg/ kg/day) was added because of short-acting generalized clonic seizures. Although the patient received high doses of diazoxide, esidrex, and octreotide approximately for 2 months, hypoglycemic episodes continued. Then the patient had near-total pancreatectomy, and pathology confirmed a diffuse form of congenital hyperinsulinism. There was homozygous mutation in the ABCC8 gene encoding SUR1, which confirmed the diagnosis of autosomal recessive congenital hyperinsulinism. During octreotide discontinuation, the patient developed non-hypoglycemic seizures, which were controlled by restarting the previous doses. In the light of in vitro and in vivo studies on antiepileptic effects of somatostatin, we believe that seizures in our case have developed secondary octreotide discontinuity.     

Pregnancy outcomes in women with heart disease

Aim

To evaluate the maternal and fetal outcome of pregnancies complicated by cardiac disease in a developing country.

Methods

A retrospective analysis was carried out of 144 pregnancies in women with cardiac disease who delivered in our unit between 1997 and 2006. Perinatal and maternal outcomes were interpreted according to the type of the heart disease and status of the patient according to the New York Heart Association (NYHA) classification.

Results

The rates of rheumatic and congenital heart disease were 87.5 and 12.5%, respectively. The distribution of the patients according to the NYHA functional classification were 55.6, 36.1 and 8.3% for NYHA classes I, II and III–IV, respectively. There was no maternal mortality. Maternal morbidity was observed in 16 (11.1%) cases. Six perinatal mortalities (4.2%) occurred in this series. There were no significant difference in birth weight, gestational age at delivery and perinatal morbidity between the NYHA stage I–II and stage III–IV groups (P > 0.05), whereas maternal morbidity and cesarean delivery rates were significantly higher in the NYHA stage III–IV group (P < 0.001).

Conclusion

Rheumatic heart disease with pregnancy is still predominant in Turkey. Most of the patients were in a good functional group. Maternal morbidity strongly correlates with maternal cardiac classification.     

Matrix metalloproteinases in the progression and regression of Kaposi's sarcoma

BACKGROUND: Matrix metalloproteinases (MMPs) are associated with Kaposi's sarcoma (KS) tumorigenesis. To date, only a few MMPs have been studied in KS lesions. Their role in KS regression has not been investigated. The aim of this study was to evaluate the expression of multiple MMPs in developing and pharmacologically regressed KS lesions. METHODS: Nine samples of acquired immune deficiency syndrome (AIDS)-related and classic cutaneous KS lesions at various histological stages were studied. Regressing KS lesions from three patients treated with systemic therapy were procured after one and two cycles of chemotherapy. Tissue sections from all specimens were immunostained using monoclonal antibodies to MMP-1, MMP-2, MMP-3, MMP-7, MMP-9, MMP-13, and MMP-14. RESULTS: KS lesional cells were immunoreactive for all MMPs, except MMP-14. Admixed inflammatory cells were immunoreactive for MMP-1, MMP-2, MMP-7, MMP-9, and MMP-13. The MMP immunoprofile in residual KS lesional cells was unaltered in regressed lesions. Increased extracellular matrix (ECM) and macrophage immunoreactivity for MMPs was identified in regressed specimens. CONCLUSIONS: These data show that developing KS lesional cells express collagenases (MMP-1, MMP-13), gelatinases (MMP-2, MMP-9), stromelysin-1 (MMP-3), and matrilysin (MMP-7) but not the membrane-type MMP-14. This MMP expression profile is retained by residual KS cells and also expressed by infiltrating macrophages in regressed KS lesions. Pantanowitz L, Dezube BJ, Hernandez-Barrantes S, Tahan SR, Dabbous MK. Matrix metalloproteinases in the progression and regression of Kaposi's sarcoma.     

Giant cells in pyoderma gangrenosum

It has been claimed that pyoderma gangrenosum (PG) lesions may contain granulomatous foci when associated with Crohn's disease. To test this assertion, we obtained clinical histories and archived cutaneous biopsies from 34 PG patients. Thirteen of these patients had inflammatory bowel disease (IBD). Immunostaining with PGM1, a macrophage marker, revealed well-formed giant cells with three or more nuclei in biopsies from 6 of 13 patients with IBD. Five of the 6 biopsies came from patients with Crohn's disease and one from a patient with ulcerative colitis. Two were peristomal. In the 21 patients who had PG without IBD, no giant cells were seen. Thus, PGM1+ histiocytic giant cells within a PG lesion may be indicative of associated IBD (p = 0.006), particularly Crohn's disease.