Lymphocytic hypophysitis: a rare or underestimated disease?

Lymphocytic hypophysitis (LYH) is an uncommon autoimmune disease in which the pituitary gland is infiltrated by lymphocytes, plasma cells and macrophages and its function is usually impaired. It has to be suspected in pregnant women and in women with recent delivery presenting with hyperprolactinemia, headache, visual field alterations and changes of one or more pituitary hormone secretions with secondary impairment of related peripheral target glands, especially when associated with other autoimmune endocrine or non-endocrine disorders. It can also occur less frequently in prepubertal or post-menopausal women and in men. Headache, visual field impairment and more rarely diplopia are due to extrasellar pituitary enlargement with optic chiasma compression and/or to invasion of cavernous sinuses. Among the 'isolated' pituitary hormone deficiencies, ACTH deficit is usually the earliest and most frequent hormonal impairment and in rare cases can induce an acute secondary hyposurrenalism as the first sign of the disease, with high mortality in affected patients. Histopathological findings from pituitary biopsy show lymphoplasmacytic infiltrate with lymphoid aggregates surrounding atropic acini of pituitary cells; immunohistochemical analysis shows numerous mast cells randomly distributed and also localized in the vicinity of capillaries, suggesting a possible influence on capillary permeability and angiogenesis, thus favoring the inflammatory and immunological aggression against pituitary cells. Nuclear magnetic resonance imaging shows uniform sellar floor depression and an extrasellar symmetrical pituitary enlargement, usually displacing the optic chiasma, which shows a rapid homogeneous enhancement after gadolinium also involving the adjacent dura (dural tail). Antipituitary antibodies have been detected in several patients with LYH but their role needs to be clarified. Since a possible spontaneous remission can occur, a careful follow-up is required in subclinical patients without important hyposurrenalism or symptomatic extrasellar expansion. Medical (immunosuppressive, replacement and antiprolactinemic) and neurosurgical (decompression) treatments are needed in clinical symptomatic patients.     

Acute management of pituitary apoplexy – surgery or conservative management?

BACKGROUND AND OBJECTIVE: The rarity of pituitary apoplexy renders it a difficult subject for audit; hence there are no evidence-based standards of optimum care for such patients. The key controversy in management relates to the role of acute neurosurgical intervention. In recent years we have adopted a relatively conservative approach towards patients presenting with pituitary apoplexy. Against this background, we aimed to determine whether our less-interventional approach affected long-term clinical outcome in these patients. PATIENTS AND DESIGN: A retrospective analysis was performed to evaluate clinical presentation, management and clinical outcomes in a cohort of patients who presented acutely with pituitary apoplexy during the period 1994-2004. Data from 33 patients (13 female) were included, with a mean age of 52 (range 27-79) years and mean follow-up duration of 3.7 (0.4-10.1) years. RESULTS: The most common presenting symptoms were headache (97%), visual deficits (82%) and nausea/vomiting (78%). Fifteen patients (46%) underwent transsphenoidal surgery while 18 were managed conservatively. Indications for surgery were deteriorating visual deficit (n = 13), hemiparesis (n = 1) and altered conscious level (n = 1). Eight patients in the surgical group had ocular paresis that resolved in 63% following surgery, and seven had visual field defects with recovery in 57% postsurgery. Conservative management was reserved for patients with absent, or evidence of resolving, visual deficits at presentation. In this group, seven presented initially with ocular paresis and six with visual field defects but all made full recoveries. Of the patients managed neurosurgically, 87% required long-term glucocorticoid replacement and 60% required long-term thyroid hormone replacement. Conservatively managed patients required glucocorticoid replacement in 72% and thyroid hormone replacement in 72% of cases (P = NS between the two groups). Sex steroid replacement was required in 67% and 83% of patients managed neurosurgically and conservatively respectively (P = NS). At latest follow-up one patient in the conservatively managed group had required surgery and one in the surgically managed group had received pituitary radiotherapy, in both instances due to evidence of tumour regrowth on magnetic resonance imaging (MRI). CONCLUSION: Our findings suggest that patients presenting with pituitary apoplexy in whom visual deficits are stable or improving may be managed expectantly as there is no identifiable deleterious effect on visual or endocrine outcome. One patient from each group experienced tumour regrowth that necessitated further treatment intervention, highlighting the importance of long-term follow-up in patients with pituitary apoplexy.     

Case report of paroxysmal atrioventricular block and ventricular arrest in a young pregnant woman: What is the mechanism?

A 30‐year‐old female patient with 12 weeks of gestation was admitted to our hospital due to dizziness and amaurosis fugax. Moreover, 24 h Holter monitoring showed paroxysmal atrioventricular block (P‐AVB) and ventricular arrest. The heart block in the patient was likely a vagally mediated heart block based on the "vagal score." She was not given a pacemaker, and the symptoms and AV nodal conduction were improved following the delivery.     

An 88-year-old woman diagnosed with adrenal tumor and congenital adrenal hyperplasia: connection or coincidence?

An 88-yr-old woman presented with a 3x4x5 cm adrenal incidentaloma. Apart from partial cortisol deficiency there were no clinical or laboratory signs of abnormal hormone production. Because of suspicion of carcinoma, a urinary steroid profile was carried out which indicated 21-hydroxylase deficiency with elevated pregnantriol. Biopsy of the tumor showed benign adenoma tissue. The genetic analysis showed two mutations in the CYP21-gene, V281L and 1172N consistent with mild non-classic congenital adrenal hyperplasia (CAH). The patient showed a general improvement with a low prednisolone dose. Previous reports have shown increased prevalence of CAH in patients with adrenal tumors although, to our knowledge, no one has reported the combination in a patient as old as in ours. Thus, clinical signs and symptoms of CAH should be looked for in patients with adrenal incidentalomas, even in the very old ones, and if suspicion further diagnostic work-up should be carried out to provide adequate treatment and follow-up.     

Takayasu’s arteritis presenting in a pregnant woman with ankylosing spondylitis: Case report and review of the literature

IntroductionCoincidence of ankylosing spondylitis (AS) and Takayasu arteritis (TA) in single person especially women is rare.Case reportWe report a 31 year old Iranian woman with AS from 5 years and giving history of inflammatory low back pain, morning stiffness, bilateral sacroiliac tenderness, positive Schöber test and enthesitis, negative human leucocytic antigen (HLA-B27) and sacroiliitis on plain X-ray. The patient was under treatment. After 2 years she returned with increased back, hip and entheseal pain as well as claudications in left hand during the eighth week of gestation in her first pregnancy. She discontinued the non-steroidal anti-inflammatory drugs three months before pregnancy and on examination was pulseless on the left side. Color Doppler studies showed segmental stenosis and increased intima thickness in subclavian, axillary and proximal brachial arteries of the left hand with reduced blood flow in those arteries. The right hand was normal. Laboratory result showed microcytic anemia (hemoglobin = 10.8 g/dl, mean corpuscular volume = 66 fL) and erythrocyte sedimentation rate = 104/1st hour. The findings were indicative of TA and she was treated with prednisolone and azathioprine and had successful delivery to full term normal boy at 39th week gestation by Caeserian section. She was followed for two more months after delivery with no complications.ConclusionBoth TA and AS have no significant adverse effects on pregnancy if diagnosed and timely treated properly. When they occur concomitantly, more attention and care is needed to prevent complications.     

Pituitary tumour apoplexy within prolactinomas in children: a more aggressive condition?

Objectives

To evaluate clinical presentations, diagnosis and management of paediatric patients presenting with pituitary apoplexy.

Methods

A retrospective case series describing a cohort of paediatric patients presenting with this condition from 2010–2016 to a tertiary referral children’s hospital in the United Kingdom.

Results

Pituitary apoplexy is a rare condition that seems to have a higher relative incidence in children than adults. Our series suggests that pituitary apoplexy in paediatric patients with adenomas appears more common than previously described. All our patients required surgery, either as an acute or delayed procedure, for visual compromise. Two patients had commenced growth hormone (GH) for GH deficiency two weeks prior to the onset of pituitary apoplexy.

Conclusions

With only a limited number of published case reports surrounding this topic our case series contributes to help further understand and manage this condition.

     

Dexrazoxane: how it works in cardiac and tumor cells. Is it a prodrug or is it a drug?

Dexrazoxane is highly effective in reducing anthracycline-induced cardiotoxicity and extravasation injury and is used clinically for these indications. Dexrazoxane has two biological activities: it is a prodrug that is hydrolyzed to an iron chelating EDTA-type structure and it is also a strong inhibitor of topoisomerase II. Doxorubicin is able to be reductively activated to produce damaging reactive oxygen species. Iron-dependent cellular damage is thought to be responsible for its cardiotoxicity. The available experimental evidence supports the conclusion that dexrazoxane reduces doxorubicin cardiotoxicity by binding free iron and preventing site-specific oxidative stress on cardiac tissue. However, it cannot be ruled out that dexrazoxane may also be protective through its ability to inhibit topoisomerase II.     

Inflammatory myofibroblastic tumour of the lung: a reactive lesion or a true neoplasm?

Inflammatory myofibroblastic tumour (IMT) of the lung represents an extremely rare type of inflammatory pseudo tumor that appears most commonly in children and young individuals. There has been an ongoing controversy whether an IMT is a reactive lesion or a true neoplasm making the further management extremely challenging. Purpose of the paper is through a literature review to highlight the existence of this rare tumour along with its key features and the management options available.     

Elevation of alkaline phosphatase in a pregnant patient with antiphospholipid syndrome: HELLP syndrome or not?

SIR, Pregnant patients with APS may develop HELLP syndrome (haemolysis;elevated liver enzymes; low platelet count) that can manifestfirst by elevation of ALP level. However, other causes of ALPelevation must be discussed and include elevation of ALP ofplacental origin. We report for the first time an APS pregnant     

Ocular toxoplasmosis in an immunocompetent 8-year-old child: a new active lesion or a late manifestation of a congenital toxoplasmosis?

Infection with the protozoan Toxoplasma gondii is one of the most frequent parasitic infections worldwide and the common infection of the retina in the general population. We describe a case report of a chorioretinitis in an immunocompetent 8-year-old patient as a consequence of a underdiagnosed neonatal toxoplasmosis. The boy was successfully managed with pyrimethamine and sulfadiazine. The present case we would like to empathize the importance of considering toxoplasma gondii as a possible cause of chorioretinitis in children living in developed countries and we provide a detailed reviewed of the literature about treatment of Toxoplasma gondii infection.     

Pituitary tumor apoplexy in patients with Cushing’s disease: endocrinologic and visual outcomes after transsphenoidal surgery

Pituitary apoplexy in patients with adrenocorticotropic hormone (ACTH) producing tumors is a rare occurrence. We report four patients with Cushing's disease harboring ACTH-secreting macroadenomas who presented with pituitary apoplexy. We report the endocrinologic and visual outcomes of these patients after emergent transsphenoidal surgery. A retrospective chart review was performed in 4 patients who presented with pituitary apoplexy from hemorrhage into an ACTH-secreting pituitary adenoma. The patient charts were reviewed for clinical presentation, neuroimaging findings, intraoperative surgical findings, pathologic findings, and postoperative endocrinologic and visual outcomes. All patients presented with acute headaches, nausea, vomiting, and visual loss from optic compression. MR imaging demonstrated a hemorrhagic macroadenoma that was confirmed at surgery. All patients underwent emergent transsphenoidal decompression (within 24 h of presentation). One of these underwent an additional craniotomy to resect residual tumor. Postoperatively, all patients showed significant improvement in visual acuity and visual fields with biochemical remission confirmed on laboratory testing. Significant weight loss as well as resolution of diabetes and hypertension was noted in all cases. All four patients remained in biochemical remission at their most recent follow-up visit (mean 40 months, range: 24-72 months). Excellent endocrine and visual outcomes can be achieved after emergent transsphenoidal surgery in patients with Cushing's disease presenting with pituitary apoplexy. Although the cure rates of non-apoplectic ACTH macroadenomas are generally poor, higher rates of remission can be achieved in cases of pituitary apoplexy. This may be partly due to the effects of tumor infarction.