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Endocirne_Case 7 |

حالة سريرية

الوصف الكامل Background
A 35-year-old man presented with with elevated blood pressure (188/112, seated) at a yearly physical exam. Previous exams noted blood pressures of 160/94 and 158/92. On questioning, he admitted episodes about twice a month of apprehension, severe headache, perspiration, rapid heartbeat, and facial pallor. These episodes had an abrupt onset and lasted 10-15 minutes.
الفحص السريري Clinical Exam
30 min after the initial blood pressure measurement, the seated blood pressure was 178/110 with a heart rate of 90. The blood pressure after 3 min of standing was 152/94 with a heart rate of 112. The optic fundi showed moderately narrowed arterioles with no hemorrhages or exudates.
Initial lab studies
Routine hematology and chemistry studies were within the reference ranges and a chest film and EKG were essentially normal.
كتابة حرة وطرح موضوع النقاش!
Questions
1.How would you assess this patient's presentation?
2.What laboratory tests would you order to evaluate this patient?
المرجع

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Virilizing Tumors of the Adrenal Gland |

حالة سريرية

الوصف الكامل Background
An 11 year old Caucasian female presented to her family doctor with complaints of excessive facial hair and a deepening of her voice
القصة المرضية HPI
At the age of 8 it was noted that she had a rather early appearance of pubic hair growth, showed evidence of acne on her forehead, had increased amounts of apocrine sweat gland activity and yet had little evidence of breast development or signs of menarche. As part of her mildly presenting symptoms of androgen excess, she had a DHEA-S blood level drawn at the time that was 600 ng/ml. She was in the 90th percentile for height and weight. The diagnostic impression at this time was precocious adrenarche. This was based on a mildly elevated DHEA-S level, normal serum estradiol and a bone age of 10 1/2 years. Over the next three years, this young girl experienced progression of her facial acne and began to show increasing evidence of facial hair growth. Her acne manifestation prompted a dermatologic consultation that resulted in treatment with Retin-A and tetracycline. She also started to experience significant weight loss that reached 15 pounds by the time she was 11. This was partially attributed to an intense ballet program and her desire to lose weight at the suggestion of her ballet teacher. Her family physician however became concerned with her overt manifestations of virilism and ordered lab work to justify her suspicion.
الفحص السريري Clinical Exam
On physical examination, she presented as a thin, white female with virilizing features including excessive facial hair, a deep voice and considerable cystic acne. Her evaluation included an MRI of the adrenals and ovaries and an ACTH stimulation test. A diagnosis of delayed onset congenital adrenal hyperplasia was considered but hormone analysis revealed a normal baseline cortisol, 17 hydroxyprogesterone and 11 deoxycortisol levels. The cortisol level however did not stimulate beyond the baseline level following cortrosyn stimulation. She also had markedly elevated levels of DHEA-S for age at 3,000 ng/ml, and an elevated testosterone at 218 ng/dl. Her bone age was 13 with a chronological age of 11. An MRI scan revealed a right adrenal mass with a normal left adrenal suggesting a right adrenal tumor. At this time she was referred to the endocrine service at a university hospital for further evaluation and work-up.

The endocrine consult at the university hospital revealed a young girl with considerable clinical evidence of androgen excess. She had moderately severe facial hirsutism and severe facial acne along with acne form eruptions on her back. She showed small amounts of hair around her nipples and adult amounts of pubic hair. Her voice was deep-timbered although she did not exhibit much of an increase in muscle mass considering her hyper-androgen state. The patient's height was at the 85th percentile with weight at the 50th percentile. She had less than 1 cm of breast tissue on either breasts and examination of her genitalia revealed no evidence of in utero virilization. Her clitoris was enlarged however yet she had no evidence of estrogen exposure as judged by examination of her vaginal mucosa. The DHEA-S was repeated and was found to be 2,600 ng/ml. Her testosterone level was also determined by radioimmunoassay following partial purification of the sample by celite chromatography and was elevated at 150 ng/ml. With the laboratory and radiographic studies, the diagnosis of a possible adrenal tumor was confirmed. The patient subsequently underwent an excision of her right adrenal mass. Her post-operative course was uneventful and her elevated androgen levels began to return to normal for her age and pubertal status.

التدبير Managment
Treatment

Surgery was the only logical treatment choice for treating this isolated adrenal mass. Once the adrenal tumor was removed, the adrenal steroid pattern reverted to normal. It is expected that this young girl will experience normal puberty once the elevated androgen levels become normalized in her system

كتابة حرة وطرح موضوع النقاش!


Discussion

Virilizing tumors of the adrenal gland are relatively rare entities not commonly seen in clinical practice. The estimated incidence is 1 per 1.7 million. The presentation of precocious virilization however, is not uncommon in children and can present from birth up through puberty. The most common cause of androgen excess in children is cogenital adrenal hyperplasia (CAH) that results from an enzymatic block at the 21 hydroxylase enzyme step in the cortisol biosynthetic pathway. (Figure 1) This enzyme block results in cortisol deficiency that leads to a compensatory rise in pituitary ACTH secretion. The increase in ACTH produces a redirection of steroid substrate through the adrenal androgen pathway which is not dependent on the 21 hydroxylase enzyme. The result is excess availability of the major adrenal androgen, dehydroepiandrosterone (DHA) which is converted to testosterone and produces the metabolic response associated with excess androgens. A similar clinical picture can present in patients with an 11 hydroxylase enzyme deficiency of the adrenal. This enzyme step is immediately proximal to the formation of cortisol. The incidence of CAH from an 11 hydroxylase deficiency however is much rarer and is usually accompanied by hypertension. Laboratory measurements of DHEA in its sulfated form is often used to exclude or include the adrenal as the reason for the clinical manifestation of virilizing symptoms. Because DHEA-S has a significant age dependent reference range, normal levels for a young adult can be quite abnormal when present in a pre-adolescent child. (Table 2) In the case of this patient, the presenting level of 600 ng/ml at the age of 8 is well within the normal range for a 14 year old but is clearly elevated for an 8 year old. Thus age related norms must be considered when interpreting the level of DHEA in the face of a suggested overactive adrenal androgen metabolic pathway. DHEA-S measurements are also considered to be an important marker for adrenal tumors. As with this patient, other adrenal steroids including aldosterone and cortisol are sometimes found normal in patients with tumors of the adrenal. The discriminating steroid to measure when suspecting an adrenal tumor is DHEA-S which is usually markedly raised in patients with an adrenal carcinoma.

Clinical Presentation
Although hirsutism alone can be the initial presenting symptom, patients with an adrenal tumor usually present with other symptoms of virilization as well. Although more commonly observed in adults, the virilization usually manifest itself as excess facial and body hair, severe acne in the face and back areas, male pattern baldness, increased muscle mass and cliteromegaly. Reproductive age women will cease their menstrual periods as well. Symptoms of androgen excess however are not restricted to an adrenal tumor. Hyperandrogenism can present in patients with congenital adrenal hyperplasia and polycystic ovarian disease as well. Although rare, there is a bimodel peak in the age of incidence of virilizing adrenal tumors in the first and fourth decades of life. The presentation of an adrenal tumor in a pre-pubertal child is quite rare. It is easy to see how the initial complains of excessive pubic hair growth in an 8 year old may simply have signaled the early onset of adrenarche, the period when the adrenal androgen pathway starts to become active. The fact that this young girl continued to show continued and progressive hirsutism without evidence of an estrogenic response to normal puberty should have pointed the family practitioner towards an endocrine consult earlier.

Laboratory Presentation
Adrenal function testing is an important part of identifying the cause of excessive manifestations of virilism. Since congenital enzyme blocks are not that uncommon, the use of provocative adrenal function testing like the cortrosyn stimulation test is usual practice in working up a patient who presents with signs and symptoms of androgen excess. The normal baseline levels of cortisol, 11deoxycortisol and 17 hydroxy progesterone and response to exogenous ACTH tended to rule out an exzymatic block as the cause of excess androgen production. The key to the diagnosis was the DHEA-S level. At the age of 8 the level was already 600 ng/ml. Although this level is within the limit usually thought of as suggestive of non-neoplastic disease, for the patient's age, it was nevertheless, significantly elevated. By the time the patient presented with overt androgen symptoms of excessive facial hair and a deepening voice, the level was close to 3,000 ng/ml, a value markedly elevated in an 11 year old who had yet to show apparent signs of normal puberty. Within two days following surgical removal of the adrenal mass, the level of DHEA-S had dropped to 708 ng/ml. Within two weeks after surgery the DHEA-S level had fallen to an appropriate 88 ng/ml for pubertal status with normal levels of testosterone, cortisol and 11 deoxycortisol.
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المرجع

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endocrine_case |

حالة سريرية

الوصف الكامل Background

History
A 50-year-old man presents with enlargement of left anterior neck. He has noted increased appetite over past month with no weight gain, and more frequent bowel movements over the same period.
Physical Exam
He is 5'8" tall and weighs 150 lb. The heart rate is 82 and the blood pressure is 110/76. There is an ocular stare with a slight lid lag. The thyroid gland is asymmetric to palpation, weighing an estimated 40g (normal = 15-20g). There is a 3 x 2.5 cm firm nodule in left lobe of the thyroid.

1.What do you think the patient's primary problem is?
2.What laboratory tests would you order to evaluate this patient?

المرجع

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Post Partum Auto Immune Thyroid Syndrome |

حالة سريرية

الوصف الكامل Background
A 26 year old white female presented to her obstetrician with complaints of heart palpitations.
الشكوى الرئيسية CC
She states that the palpitations have been constant over the past two weeks but seem worse at nighttime. When asked to describe them, she states that they are regular and it feels as if her heart is going to jump out of her chest. She denies chest pain, shortness of breath or lightheadedness. She has felt a bit warm of late but denies any frank diaphoresis. It is of note that she recently delivered a normal baby boy during an uncomplicated delivery 5 1/2 weeks before this visit. Her review of system is remarkable for loose stools occurring approximately 4 times/day. She complains of feeling tired but unable to get a good night sleep. She states that she feels as if her mind is racing. She denies any nausea, vomiting or abdominal pain. She also denies myalgias, arthralgias, fevers or chills. She denied heat or cold intolerance.
الفحص السريري Clinical Exam
On physical examination, she presented as a thin, white female in no apparent distress. Her blood pressure was 146/90. Pulse 96 and regular and a normal temperature of 37 degrees taken orally. Her review of systems revealed clear lungs, normal heart rhythm, normal abdomen and a normal neurological response although she showed a fine tremor of the hands. Her neck was supple with no lymphadnopathy however her thyroid was approximately 1.5 times normal in size, symmetrically enlarged, firm, non-tender with carotids palpable bilaterally without bruits.

Her blood work at the time of the clinic visit included a CBC (WBC 14.2, Hct, 38.6, MCV normal, platelet count normal, differential 56% neutrophiles, 7% bands, 34% lymphocytes and 3% monocytes) and a chemistry screen that included electrolytes (NA 142, K 3.6, Cl 101, CO2 22), glucose 86, BUN 26, creatinine 1. She also had a thyroid panel that included thyroxine 16.2 (NL 4-13), T3 resin uptake 34% (NL 25 - 35%) and a TSH of <0.05 (NL 0.3 5.0).

From the physical examination, the patient's history including post partum status and the abnormal thyroid function tests, the working diagnosis was postpartum thyroiditis with hyperthyroidism. The patient had a radioactive iodine uptake scan which was normal and subsequently had thyroid auto-antibodies determined which were positive for anti-thyroglobulin and anti-microsomal antibodies. A thyroid biopsy was also performed and revealed diffuse, lymphocytic infiltration, a characteristic histologic picture of post partum thyroiditis. In this case the patient was simply treated with beta blockers to reduce the heart palpitations. Inside three months, the thyroiditis had resolved and the patients' symptoms disappeared with normal thyroid function test results.

التشخيص التفريقي DD
Table 1. Differential Diagnosis of Thyrotoxicosis Between Post Partum Thyroiditis and Graves disease

التدبير Managment
Treatment

Because post partum thyroiditis can spontaneously resolve, treatment is often unnecessary because the manifestation of thyrotoxicosis is usually mild and self limited. In some patients, symptomatic relief of the heart palpitations is brought about with the use of beta blockers which reduce the severity of tremor, palpitations and other anxiety related symptoms. The use of anti-thyroid drugs and/or radio iodine ablation has no role in the therapy of these patients during the thyrotoxic phase. On occasion, patients who develop hypothyroidism with this disorder are put on thyroid replacement therapy. Most patients have a complete remission although some 25% of patients have a recurrence with a subsequent pregnancy.

كتابة حرة وطرح موضوع النقاش!


Discussion

Post partum thyroiditis (PPT) is a relatively common disorder expressed in 5 to 15% of post partum women. This disorder initially presents as thyrotoxicosis in the period from 6 weeks to 3 months post partum, is associated with an auto immune component and usually resolves spontaneously after 1-2 months of expression. In some patients, the thyrotoxic phase can be followed by a hypothyroid phase before spontaneous disease resolution occurs. This thyroid disorder is a distinct variant of silent (painless) thyroiditis and is more prevalent in patients with a family history of Hashimoto's thyroiditis. Over 50% of patients will have a mild goiter condition.

Clinical Presentation
Patients with post partum thyroiditis usually present with the onset of palpitations and fatigue. Since these symptoms can be mildly expressed in the young mother with the trials and tribulations of normal pregnancy, these symptoms can easily be overlooked. In some patients, hypertension can accompany this disorder. The symptoms of thyrotoxicosis are generally milder than that of Graves disease, another form of thyroid auto immune disease however the presenting symptoms can be similar and it is important to distinguish thyrotoxicosis due to PPT and Graves disease since the approach to treatment is quite different between the two. The radioactive iodine uptake or thyroid scan can be used to differentiate Graves disease from PPT. A normal scan or uptake is observed in patients with PPT in contrast to Graves where there is a marked elevation in radioactive iodine uptake.

Predisposing factors for post partum thyroiditis include a family history of thyroid disease particularly Hashimoto's thyroiditis, the presence of other endocrine auto immune disorders (Type I Diabetes Mellitus), iodide exposure and interestingly enough cigarette smoking. White and Asian races as opposed to the black population are more prone to developing PPT.

Laboratory Presentation
Thyroid function testing is an important part of establishing the diagnosis of post partum thyroiditis. An elevated level of thyroxine (Total T4 or Free T4) with a suppressed TSH level occurs in the thyrotoxic phase of the disorder while an elevated TSH with normal to low FT4 levels are found in the hypothyroid phase that occurs subsequent to the thyrotoxic phase. Measurement of thyroid peroxidase ( TPO), the major antigen of thyroid microsomes recognized by anti-thyroid microsomal antibodies is important in establish the differential diagnosis. TPO antibodies are elevated as early as the first trimester in patients who will manifest this disorder and are also raised at the time of delivery. It has been suggested that screening with TPO antibodies in high risk patients could identify those patients at risk of developing post partum thyroiditis. To distinguish PPT from Graves disease, measurement of antibodies to the TSH receptor and the use of the thyroid scan are approaches that can help confirm the diagnosis. Both are abnormal with Graves disease and normal in the patients with PPT.

Pathophysiology
Genetics, immune dysfunction and environmental factors all contribute to the expression of auto immune thyroid disorders in the post partum period. The genetic component of PPT is well established as noted in the literature however the considerations as to what governs the susceptibility of this disorder in some patients is still unclear. Pregnancy itself can trigger alterations in the immune system which become manifest after the pregnancy itself has ended. PPT appears to be triggered in some patients through a rebounded immune response which can cause immune alterations and injury directly affecting the thyroid gland. The immune rebound hypothesis during pregnancy has also been linked to other auto immune thyroid disorders such as Graves, Hashimoto's and systemic auto immune conditions such as systemic lupus erythematosus and rheumatoid arthritis. Atypical expression of a thyroid microsomal antigen reflects the auto immune expression of this disorder. TPO antibodies have been shown to reflect disease activity. The total IgG elevation in these patients is associated predominantly with the IgG subclasses 1 and 4. The cytolytic events in the thyroid gland itself appears to be due to T-cell and NK cell attack. The massive infiltration of lymphocytes leads to enhanced release of thyroid hormone presumably from thyroglobulin stores. The circulating lymphocyte has been studied in patients with PPT and shown to reflect an increase in activated T cells with helper-inducer activity.

المرجع

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endocrine_case 4 |

حالة سريرية

الوصف الكامل Background

A 27-year-old woman presents with depression, insomnia, increased facial fullness and recent increase in facial hair. She had also had an episode of depression and acute psychosis following uncomplicated delivery of normal baby boy 9 months previously. Her menses have been irregular since their resumption after the birth (she is not breast-feeding).

الفحص السريري Clinical Exam

The heart rate was 90 beats per minute and the blood pressure was 146/110. Her face was puffy with an increase in facial hair and ruddy complexion. There was no truncal obesity, peripheral wasting, or striae.

الاستقصاءات Investigations

Serum electrolytes, white cell count, and hemoglobin and hematocrit were all within normal limits.

كتابة حرة وطرح موضوع النقاش!

1.What do you think the patient's primary problem is?
2.What laboratory tests would you order to evaluate this patient?

المرجع

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endocrine_case 2 |

حالة سريرية

الوصف الكامل Background

A 28-year-old woman with recent tiredness and difficulty concentrating had experienced a decline in memory over the last several months. She also noted decreased frequency of bowel movements and an increased tendency to gain weight. She felt chilled without light sweater, even in warm weather. There was a history of hypothyroidism in her mother and older sister.

الفحص السريري Clinical Exam

Her pulse rate was 58 beats per minute and her blood pressure was 138/88. She had a slightly puffy face and her eyebrows were sparse, especially at the lateral margins. The thyroid was firm and bosselated to palpation with an estimated weight of 25 g (normal 15 - 20 g). The deep tendon reflexes were normally contractive, but showed delayed relaxation.

كتابة حرة وطرح موضوع النقاش!
Questions
1.What do you think the patient's primary problem is?
2.What laboratory tests would you order to evaluate this patient?
المرجع

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endocrine_case 3 |

حالة سريرية

الوصف الكامل Background

History
A 6-year-old boy presents with 6 month history of pubic hair growth. For the past 4 years, he has had a history of rapid somatic growth.
The obstetric history was unremarkable. He was a full-term infant born to a 34-year-old healthy mom by normal vaginal delivery after an uncomplicated gestation. His birth weight was normal and there were no neonatal problems. At 9 - 18 months, his growth was at the 95th percentile for his age; his height at age 2 1/2 was average for 4 1/2 years (his parents were tall). His penis appeared larger than those of his peers at 3 years, he developed some facial acne at 4 years, and pubic hair was seen at 5 1/2 years.

Physical Exam

His height was average for 10 years and 3 months and his weight was average for 9 years and 10 months. The blood pressure was normal. He was tall, well-proportioned, and muscular with mild facial acne. The penis was large for his age and there was fine pubic hair (Tanner stage II of puberty). The testes were estimated to be 3 ml volume each (small for puberty stage). The neurological exam normal.

Questions
1.What do you think the primary problem might be?
2.What laboratory tests would you ask for?

كتابة حرة وطرح موضوع النقاش!

answer 1:
Excess androgen production

The increased growth rate seen in this child could also raise the possibility of growth hormone excess (i.e., a pituitary lesion). However, the appearance of secondary sexual features and a well-proportioned muscular build suggests an androgen effect. These androgens could be derived either from the testes or adrenal glands. The testes are small for the level of physical development of the child, suggesting an adrenal source of steroids.

answer 2:

Laboratory studies should be directed toward confirming the impression of androgen (testosterone) excess and localizing the source.

Testosterone (S)
Lutenizing Hormone (LH), basal (S)
FSH, basal (S)
17-OH-progesterone
basal (S)
60 min after ACTH stim (S)

Cortisol
basal (S)
after ACTH stimulation (S)

المرجع

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ماهو المسح المناسب لاعتلال الشبكية عند مريض السكري؟ |

المعلومة

ماهو المسح المناسب للتحري عن اعتلال الشبكية عند مريض شخص حديثاً بالداء السكري نمط 2 ؟

آ- بدء المسح بعد 5 سنوات من التشخيص.
ب- المسح بعد 4 سنوات ثم كل سنة.
ج- البدء بالمسح بعد 3 سنوات من التشخيص ثم إعادته سنوياً.
د- البدء بالمسح بعد عمر الخمسين، أو أبكر من ذلك إذا كان ضبط السكري سيئاً.

المرجع

د.عماد زوكار (2010) MCQs . الطبعة الأولى. دمشق : دار القدس للعلوم . الصفحة 87


ما هو أشيع اختلاط عند ولدان الأمهات السكريات؟ |

المعلومة

ماهو أشيع اختلاط عند ولدان الأمهات السكريات ؟

a- الاختناق عند الولادة.
b- عدم تكون العجز.
c- نقص كالسيوم الدم.
d- نقص سكر الدم.
e- الضخامة القلبية الحاجزية.

المرجع

د.عماد زوكار (2010) MCQs . الطبعة الأولى. دمشق : دار القدس للعلوم . الصفحة 35


سبيل تكوين الستيروئيدات الكظرية |
سبيل تكوين الستيروئيدات الكظرية

سبيل تكوين الستيروئيدات الكظرية


التحكم بافراز البرولاكتين |
التحكم بافراز البرولاكتين

التحكم بافراز البرولاكتين :
يتحكم الدوبامين المفرز من الوطاء بشكل أساسي فيثبط افرازه ويحرض افرازه ال (العامل المطلق للحاثة الدرقية TRH )


مستويات البرولاكتين في الأمراض التي تسبب Hyperprolactinemia |
مستويات البرولاكتين في الأمراض التي تسبب Hyperprolactinemia

إذا كان مستوى البرولاكتين > 100 مغ/ل فالورم محتمل
إذا كان مستوى البرولاكتين > 200 مغ/ل فالورم موجود حتى يثبت العكس حتى عند امرأة مرضعة.


القصور الكظري - adrenocortical insufficiency (تشخيص تفريقي) |
القصور الكظري - adrenocortical insufficiency (تشخيص تفريقي)

متلازمة السرج التركي الفارغ Empty sella syndrome |

صورة توضح متلازمة السرج التركي الفارغ تحدث عند عديدات الولادة في 90% من الحالات ..
في هذه المتلازمة يحدث توسع في السرج التركي ( بسبب التضخم الفيزيولوجي للنخامى أثناء الحمل ) وهذا التوسع هو الذي قد يوحي بالورم ...
المهم ان السائل الدماغي الشوكي سيأتي ويضغط هذه النخامى ويزيحها من مكانها ويحتل موقعها...حيث سنجد الاغلقة السحائية والسائل الدماغي الشوكي يملأ السرج التركي ..
يبقى أن نضيف اخيرا بأن هذه المتلازمة سليمة لأن النخامى لم تفقد وظيفتها على الرغم من الانضغاط وتغير المكان الحاصل ..وبالتالي لا تحتاج الى معالجة

متلازمة السرج التركي الفارغ Empty sella syndrome

طبقات قشر الكظر |
طبقات قشر الكظر

شكل يوضح طبقات قشر الكظر
من الايسر :
الطبقة الخارجية من قشر الكظر هي المنطقة الكبية ZONA GLOMERULOSA والتي تفرز الألدوستيرون
الطبقة المتوسطة هي المنطقة الحزمية ZONA FASCICULATA والتي تفرز الكورتيزول
الطبقة الداخلية هي المنطقة الشبكية ZONA RETICULARIS تفرز الاندروجينات


شكل توضيحي لمخطط الرينين انجيوتنسين الدوستيرون |
شكل توضيحي لمخطط الرينين انجيوتنسين الدوستيرون


ان انخفاض الضغط في الشرايين الكلوية يحرض الجهاز قرب الكبي الموجود في الكلية على افراز الرينين ..يغادر الرينين الى الدم المحيطي حيث يقوم بتحويل الانجيوتنسينوجين المفرز من قبل الكبد الى انجيوتنسين 1
الانجيوتنسين 1 يتحول الى انجيوتنسين 2 بواسطة انزيم ACE في الرئة
الانجيوتنسين هو مقبض وعائي شديد ومباشر على الاوعية ...كما انه يحرض المنطقة الكبية في قشر الكظر على افراز الالدوستيرون والذي يقوم بعمله على مستوى الانبوب الكلوي البعيد حيث يقوم بحبس الماء والصوديوم ..


الالية المرضية في داء غريف |
الالية المرضية في داء غريف

هو أحد الامراض المناعية الذاتية التي تصيب الغدة الدرقية ...
سببه هو وجود أضداد ذاتية من الجسم أهمها تلك التي ترتبط بمستقبلات ال TSH على الغدة الدرقية فتقلد عملها وتؤدي الى زيادة نمو الدرق و زيادة افراز الهرمونات الدرقية T4, T3
هذه الاضداد الذاتية تدعى الغلوبولينات المناعية المحرضة للغدة الدرقية Thyroid-stimulating immunoglobulins (TSIs
وهذه الاضداد لا تقتصر على الدرق فتاثيراتها تطال العين ايضاً حيث نلاحظ ان حدوث الاعتلال العيني هو من اهم العلامات المميزة لداء غريف


All about Addison disease |
All about Addison disease
Addison's disease is an endocrine or hormonal disorder that occurs in all age groups and afflicts men and women equally. The disease is characterized by weight loss, muscle weakness, fatigue, low blood pressure, and sometimes darkening of the skin in both exposed and nonexposed parts of the body.

Addison's disease occurs when the adrenal glands do not produce enough of the hormone cortisol and, in some cases, the hormone aldosterone. The disease is also called adrenal insufficiency, or hypocortisolism.

Cortisol

Cortisol is normally produced by the adrenal glands, located just above the kidneys. It belongs to a class of hormones called glucocorticoids, which affect almost every organ and tissue in the body. Scientists think that cortisol has possibly hundreds of effects in the body. Cortisol's most important job is to help the body respond to stress. Among its other vital tasks, cortisol

helps maintain blood pressure and cardiovascular function
helps slow the immune system's inflammatory response
helps balance the effects of insulin in breaking down sugar for energy
helps regulate the metabolism of proteins, carbohydrates, and fats
helps maintain proper arousal and sense of well-being
Because cortisol is so vital to health, the amount of cortisol produced by the adrenals is precisely balanced. Like many other hormones, cortisol is regulated by the brain's hypothalamus and the pituitary gland, a bean-sized organ at the base of the brain. First, the hypothalamus sends "releasing hormones" to the pituitary gland. The pituitary responds by secreting hormones that regulate growth and thyroid and adrenal function, and sex hormones such as estrogen and testosterone. One of the pituitary's main functions is to secrete ACTH (adrenocorticotropin), a hormone that stimulates the adrenal glands. When the adrenals receive the pituitary's signal in the form of ACTH, they respond by producing cortisol. Completing the cycle, cortisol then signals the pituitary to lower secretion of ACTH.

Aldosterone

Aldosterone belongs to a class of hormones called mineralocorticoids, also produced by the adrenal glands. It helps maintain blood pressure and water and salt balance in the body by helping the kidney retain sodium and excrete potassium. When aldosterone production falls too low, the kidneys are not able to regulate salt and water balance, causing blood volume and blood pressure to drop.

Causes

Failure to produce adequate levels of cortisol can occur for different reasons. The problem may be due to a disorder of the adrenal glands themselves (primary adrenal insufficiency) or to inadequate secretion of ACTH by the pituitary gland (secondary adrenal insufficiency).

Primary Adrenal Insufficiency
Addison's disease affects about 1 in 100,000 people. Most cases are caused by the gradual destruction of the adrenal cortex, the outer layer of the adrenal glands, by the body's own immune system. About 70 percent of reported cases of Addison's disease are caused by autoimmune disorders, in which the immune system makes antibodies that attack the body's own tissues or organs and slowly destroy them. Adrenal insufficiency occurs when at least 90 percent of the adrenal cortex has been destroyed. As a result, often both glucocorticoid (cortisol) and mineralocorticoid (aldostertone) hormones are lacking. Sometimes only the adrenal gland is affected, as in idiopathic adrenal insufficiency; sometimes other glands also are affected, as in the polyendocrine deficiency syndrome.

Polyendocrine Deficiency Syndrome

The polyendocrine deficiency syndrome is classified into two separate forms, referred to as type I and type II.

Type I occurs in children, and adrenal insufficiency may be accompanied by

underactive parathyroid glands
slow sexual development
pernicious anemia
chronic candida infections
chronic active hepatitis
hair loss (in very rare cases)
Type II, often called Schmidt's syndrome, usually afflicts young adults. Features of type II may include

an underactive thyroid gland
slow sexual development
diabetes
vitiligo
loss of pigment on areas of the skin
Scientists think that the polyendocrine deficiency syndrome is inherited because frequently more than one family member tends to have one or more endocrine deficiencies.

Tuberculosis

Tuberculosis (TB), an infection which can destroy the adrenal glands, accounts for about 20 percent of cases of primary adrenal insufficiency in developed countries. When adrenal insufficiency was first identified by Dr. Thomas Addison in 1849, TB was found at autopsy in 70 to 90 percent of cases. As the treatment for TB improved, however, the incidence of adrenal insufficiency due to TB of the adrenal glands has greatly decreased.

Other Causes

Less common causes of primary adrenal insufficiency are

chronic infection, mainly fungal infections
cancer cells spreading from other parts of the body to the adrenal glands
amyloidosis
surgical removal of the adrenal glands
Secondary Adrenal Insufficiency

This form of adrenal insufficiency is much more common than primary adrenal insufficiency and can be traced to a lack of ACTH. Without ACTH to stimulate the adrenals, the adrenal glands' production of cortisol drops, but not aldosterone. A temporary form of secondary adrenal insufficiency may occur when a person who has been receiving a glucocorticoid hormone such as prednisone for a long time abruptly stops or interrupts taking the medication. Glucocorticoid hormones, which are often used to treat inflammatory illnesses like rheumatoid arthritis, asthma, or ulcerative colitis, block the release of both corticotropin-releasing hormone (CRH) and ACTH. Normally, CRH instructs the pituitary gland to release ACTH. If CRH levels drop, the pituitary is not stimulated to release ACTH, and the adrenals then fail to secrete sufficient levels of cortisol.

Another cause of secondary adrenal insufficiency is the surgical removal of benign, or noncancerous, ACTH-producing tumors of the pituitary gland (Cushing's disease). In this case, the source of ACTH is suddenly removed, and replacement hormone must be taken until normal ACTH and cortisol production resumes.

Less commonly, adrenal insufficiency occurs when the pituitary gland either decreases in size or stops producing ACTH. These events can result from

tumors or infections of the area
loss of blood flow to the pituitary
radiation for the treatment of pituitary tumors
surgical removal of parts of the hypothalamus
surgical removal of the pituitary gland

Symptoms

The symptoms of adrenal insufficiency usually begin gradually. Characteristics of the disease are

chronic, worsening fatigue
muscle weakness
loss of appetite
weight loss
About 50 percent of the time, one will notice

nausea
vomiting
diarrhea
Other symptoms include

low blood pressure that falls further when standing, causing dizziness or fainting
skin changes in Addison's disease, with areas of hyperpigmentation, or dark tanning, covering exposed and nonexposed parts of the body; this darkening of the skin is most visible on scars; skin folds; pressure points such as the elbows, knees, knuckles, and toes; lips; and mucous membranes
Addison's disease can cause irritability and depression. Because of salt loss, a craving for salty foods also is common. Hypoglycemia, or low blood glucose, is more severe in children than in adults. In women, menstrual periods may become irregular or stop.

Because the symptoms progress slowly, they are usually ignored until a stressful event like an illness or an accident causes them to become worse. This is called an addisonian crisis, or acute adrenal insufficiency. In most cases, symptoms are severe enough that patients seek medical treatment before a crisis occurs. However, in about 25 percent of patients, symptoms first appear during an addisonian crisis.

Symptoms of an addisonian crisis include

sudden penetrating pain in the lower back, abdomen, or legs
severe vomiting and diarrhea
dehydration
low blood pressure
loss of consciousness
Left untreated, an addisonian crisis can be fatal.

Diagnosis

In its early stages, adrenal insufficiency can be difficult to diagnose. A review of a patient's medical history based on the symptoms, especially the dark tanning of the skin, will lead a doctor to suspect Addison's disease.

A diagnosis of Addison's disease is made by laboratory tests. The aim of these tests is first to determine whether levels of cortisol are insufficient and then to establish the cause. X-ray exams of the adrenal and pituitary glands also are useful in helping to establish the cause.

ACTH Stimulation Test

This is the most specific test for diagnosing Addison's disease. In this test, blood cortisol, urine cortisol, or both are measured before and after a synthetic form of ACTH is given by injection. In the so-called short, or rapid, ACTH test, measurement of cortisol in blood is repeated 30 to 60 minutes after an intravenous ACTH injection. The normal response after an injection of ACTH is a rise in blood and urine cortisol levels. Patients with either form of adrenal insufficiency respond poorly or do not respond at all.

CRH Stimulation Test

When the response to the short ACTH test is abnormal, a "long" CRH stimulation test is required to determine the cause of adrenal insufficiency. In this test, synthetic CRH is injected intravenously and blood cortisol is measured before and 30, 60, 90, and 120 minutes after the injection. Patients with primary adrenal insufficiency have high ACTHs but do not produce cortisol. Patients with secondary adrenal insufficiency have deficient cortisol responses but absent or delayed ACTH responses. Absent ACTH response points to the pituitary as the cause; a delayed ACTH response points to the hypothalamus as the cause.

In patients suspected of having an addisonian crisis, the doctor must begin treatment with injections of salt, fluids, and glucocorticoid hormones immediately. Although a reliable diagnosis is not possible while the patient is being treated for the crisis, measurement of blood ACTH and cortisol during the crisis and before glucocorticoids are given is enough to make the diagnosis. Once the crisis is controlled and medication has been stopped, the doctor will delay further testing for up to 1 month to obtain an accurate diagnosis.

Other Tests

Once a diagnosis of primary adrenal insufficiency has been made, x-ray exams of the abdomen may be taken to see if the adrenals have any signs of calcium deposits. Calcium deposits may indicate TB. A tuberculin skin test also may be used.

If secondary adrenal insufficiency is the cause, doctors may use different imaging tools to reveal the size and shape of the pituitary gland. The most common is the CT scan, which produces a series of x-ray pictures giving a cross-sectional image of a body part. The function of the pituitary and its ability to produce other hormones also are tested.

Treatment

Treatment of Addison's disease involves replacing, or substituting, the hormones that the adrenal glands are not making. Cortisol is replaced orally with hydrocortisone tablets, a synthetic glucocorticoid, taken once or twice a day. If aldosterone is also deficient, it is replaced with oral doses of a mineralocorticoid called fludrocortisone acetate (Florinef), which is taken once a day. Patients receiving aldosterone replacement therapy are usually advised by a doctor to increase their salt intake. Because patients with secondary adrenal insufficiency normally maintain aldosterone production, they do not require aldosterone replacement therapy. The doses of each of these medications are adjusted to meet the needs of individual patients.

During an addisonian crisis, low blood pressure, low blood glucose, and high levels of potassium can be life threatening. Standard therapy involves intravenous injections of hydrocortisone, saline (salt water), and dextrose (sugar). This treatment usually brings rapid improvement. When the patient can take fluids and medications by mouth, the amount of hydrocortisone is decreased until a maintenance dose is achieved. If aldosterone is deficient, maintenance therapy also includes oral doses of fludrocortisone acetate.

Special Problems

Surgery

Patients with chronic adrenal insufficiency who need surgery with general anesthesia are treated with injections of hydrocortisone and saline. Injections begin on the evening before surgery and continue until the patient is fully awake and able to take medication by mouth. The dosage is adjusted until the maintenance dosage given before surgery is reached.

Pregnancy

Women with primary adrenal insufficiency who become pregnant are treated with standard replacement therapy. If nausea and vomiting in early pregnancy interfere with oral medication, injections of the hormone may be necessary. During delivery, treatment is similar to that of patients needing surgery; following delivery, the dose is gradually tapered and the usual maintenance doses of hydrocortisone and fludrocortisone acetate by mouth are reached by about 10 days after childbirth.

Patient Education

A person who has adrenal insufficiency should always carry identification stating his or her condition in case of an emergency. The card should alert emergency personnel about the need to inject 100 mg of cortisol if its bearer is found severely injured or unable to answer questions. The card should also include the doctor's name and telephone number and the name and telephone number of the nearest relative to be notified. When traveling, a needle, syringe, and an injectable form of cortisol should be carried for emergencies. A person with Addison's disease also should know how to increase medication during periods of stress or mild upper respiratory infections. Immediate medical attention is needed when severe infections, vomiting, or diarrhea occur. These conditions can precipitate an addisonian crisis. A patient who is vomiting may require injections of hydrocortisone.

People with medical problems may wish to wear a descriptive warning bracelet or neck chain to alert emergency personnel. A number of companies manufacture medical identification products.


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الغدة النخامية Pituitary |
الغدة النخامية  Pituitary

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Plummer disease |
Plummer disease

Woman, 29 y. o.
Nodule in the left lobe of the thyroid (12 cc) almost entirely filling the lobe.
Rich periferic and also intranodular vascularization, more evident in the powerdoppler scan (lower image).

"Hot" nodule at the scintigraphy.
Ultrasound scan of the nodule.
Doppler of the thyroid artery.

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