scholarly journals Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Ayelet Machtei ◽  
Gil Klinger ◽  
Rivka Shapiro ◽  
Osnat Konen ◽  
Lea Sirota

Neonatal hemochromatosis (NH) is an acute liver disease associated with both hepatic and extrahepatic iron deposition and is a leading cause of neonatal liver transplantation. The concept that NH is an alloimmune disease has led to the emergence of a new treatment approach utilizing exchange transfusion and intravenous immunoglobulin therapy. We present a two-day old neonate with progressive liver dysfunction who was diagnosed with NH. Magnetic resonance imaging confirmed tissue iron overload. Treatment with intravenous immunoglobulins and exchange transfusion led to rapid improvement in liver function. Follow-up physical examination at the age of 8 months showed normal development and near normal liver function. A repeat abdominal magnetic resonance scan at 8 months showed no signs of iron deposition in the liver, pancreas, or adrenal glands. The present report provides further support for the use of exchange transfusion and immunoglobulin therapy in NH and is the first to document resolution of typical iron deposition by magnetic resonance imaging.

2016 ◽  
Vol 37 (6) ◽  
pp. 549-560 ◽  
Author(s):  
Emre Ünal ◽  
Deniz Akata ◽  
Musturay Karcaaltincaba

2012 ◽  
Vol 40 (3) ◽  
pp. 1005-1015 ◽  
Author(s):  
W Lv ◽  
F Yan ◽  
M Zeng ◽  
J Zhang ◽  
Y Yuan ◽  
...  

OBJECTIVE: To assess hepatic iron deposition quantitatively in patients with chronic hepatitis B (HBV) infection, using abdominal susceptibility-weighted magnetic resonance imaging (SWI). METHODS: Patients with HBV infection and healthy controls underwent abdominal SWI and were assessed for serum iron markers. Phase values were measured and five grades of hepatic iron deposition were described by SWI. RESULTS: Patients with HBV infection ( n = 327) and healthy controls ( n = 50) were prospectively enrolled. In total, 77 (25.4%) patients with HBV infection had hepatic iron deposition as determined by SWI. Phase values were significantly different between patients with hepatic iron deposition compared with patients without hepatic iron deposition or controls, and were significantly different across different grades of hepatic iron deposition. Serum iron, ferritin, transferrin and transferrin saturation were significantly higher in patients with, versus those without, hepatic iron deposition. Only serum ferritin was significantly different across different grades of hepatic iron deposition, and there was a low inverse correlation between serum ferritin and phase values. CONCLUSIONS: Compared with serum iron markers, abdominal SWI may represent a powerful tool to assess hepatic iron deposition quantitatively in patients with chronic HBV infection.


2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Akiyo Taneichi ◽  
Hiroyuki Fujiwara ◽  
Yukako Mizoguchi ◽  
Shizuo Machida ◽  
Hiroaki Nonaka ◽  
...  

A leiomyoma rarely causes disseminated intravascular coagulopathy (DIC). In the present report, we describe a case of DIC caused by leiomyoma. A 36-year-old nulliparous woman presented with hypermenorrhea and a lower abdominal mass. On magnetic resonance imaging, we detected a 14 cm uterine tumor, which was suspected to be a sarcoma. Blood tests at the preoperative examination indicated platelet count of 9.6 × 104/μL, fibrin degradation product level of 107.1 μg/mL (normal value, 0–5.0 μg/mL), and fibrinogen level of 54 mg/dL (normal value, 129–271 mg/dL). Based on these findings, we diagnosed the patient with DIC. The patient was treated with nafamostat mesilate and fresh frozen plasma, but the DIC did not show any improvement. Subsequently, a hysterectomy was performed, after which the DIC improved. Clinicopathological findings indicated the presence of a leiomyoma with multiple vessels containing thromboemboli, and suggested that the DIC was caused by the leiomyoma. Therefore, it is essential to consider that that a benign leiomyoma may be a cause of DIC.


Neurosurgery ◽  
2008 ◽  
Vol 62 (4) ◽  
pp. E972-E973 ◽  
Author(s):  
Marco Gessi ◽  
Federico G. Legnani ◽  
Emanuela Maderna ◽  
Cecilia Casali ◽  
Carlo L. Solero ◽  
...  

Abstract OBJECTIVE Enterogenous cysts (ECs) of the central nervous system are developmental malformations that occur in the spinal canal, posterior fossa, or cerebral hemispheres. They are usually benign lesions, and malignant transformation is rare. To date, only three cases of malignant transformation have been reported in the literature. We present a case of a cerebellopontine EC showing foci of epithelial dysplasia and malignant transformation into a low-grade papillary mucinous adenocarcinoma. CLINICAL PRESENTATION A 25 year-old man with a 6-year history of hypoacusia presented to our department with facial nerve deficit, visual disturbances, and gait instability. A magnetic resonance imaging scan demonstrated a multiloculated cerebellopontine angle cyst with supratentorial hydrocephalus. INTERVENTION A retrosigmoidal approach was used to achieve cyst removal. This was followed several months later by ventriculoperitoneal shunt placement. The cyst was adherent to the brainstem, cranial nerves, and vessels, and it resembled a thin encapsulated structure filled with mucinous-like substance. No solid component was identified. Histopathological examination revealed an EC with foci of malignant transformation in a mucinous papillary adenocarcinoma. Magnetic resonance imaging was performed 5 months postoperatively due to progressive clinical worsening; this scan revealed lesion recurrence with severe brainstem compression. Emergency surgery was performed, and a large decompression was achieved. Subsequent follow-up computed tomographic scans showed progression of the residual tumor. The patient's neurological condition rapidly worsened, ultimately resulting in death. CONCLUSION The present report suggests that a careful histological examination of all ECs after surgery should be made to exclude dysplastic foci or carcinomatous transformation. Although the clinical behavior of ECs with malignant trasformation is unpredictable, surgery remains the treatment of choice. The use of possible adjuvant chemo- or radiotherapy has not been established.


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