Wegner's Granulomatosis Vs Churg-Strauss disease

both cases presentation are identical as they are disease characterized by systemic vasculitis affecting blood vessels in the upper airways , lungs, GIT, kidneys and Joints, so they could be manifested with epistaxis , hemoptysis, haematuria , arthritis and also may feature with glomerulonephritis with hypertension and edema.

both of them are characterized by elevated Anti-neutrophil cytoplasmic antibodies ANCA.

so when you face a presentation with the classic triad of hemoptysis , hematuria and epistaxis, don't have to depend solely on the ANCA test you need to perform a CBC with differential and check for Eosinophilic count , because Churg-Strauss disease is differentiated from Wegner's granulomatosis through eosinophilia, taking into consideration that eosinophilia should be at least 10%

HPV vaccination

A Recombinant vaccine,  Quadrivalent against HPV strains 6,11,16,18

Important  points to mention about this vaccine that administration  of the vaccine doesn't exclude the need to perform annual PAP smear screening,  and also in the presence of cervical dysplasia or even carcinoma in situ, though it will have a limited value it still can be administered.

The conditions at which the administration of the vaccine shouldn't be recommended are pregnancy, breastfeeding and Immunosuppression 

PAP smear

PAP smear is a method of screening for cervical dysplasias and neoplasias, it's important to know how and when the screening is performed and also how to follow up expected results

A PAP smear may be done in 2 forms , a conventional form ,which has a 50% sensitivity and Liquid based preparations which has 75-80% sensitivity.

for every female at 22 years of age a conventional PAP smear is advised to be done annually or liquid -based every 2 years, till the age of 30.

after the age of 30 the presence of three NEGATIVE successive annual conventional pap smears may allow to perform the screening every 2 or 3 years  instead of annual screening, 

there's no need to continue screening after the age of 65 in the presence of previous negative screening test.

Suppose there were atypical results of the smear , if the patient is reliable then you would repeat the smear after 3months with HPV DNA testing, if still atypical then you would need to perform a Coloposcopy and Biopsy, if the patient is unreliable then you may shift directly to the coloposcopy and biopsy and in a developing country like egypt , for me, it's a No Brainer to directly shift to the biopsy

"THECA"

Theca cells are ovarian stromal cells which are responsible for androgen production.

in many ovarian enlargements, there's hyperproliferatrion or hyperactivity of theca cells, which sometimes my be confusing, So I tried to organize my thinking !


first, at any case of ovarian mass in the childbearing period , work-up for the diagnosis would be the same regardless of the etiology
first do a B-HCG to rule out pregnancy , then Ultrasound examination to determine whether this mass is a simple cystic one or a complex mass and in certain conditions you may need laparoscopy.

Ovarian hyperthecosis : a postmenopausal female presenting with virilizing symptoms, UNILATERAL flank pain and palpable adnexal mass, note that one of the differentiating factors between this condition and granulosa-theca cells cancer would be Ultrasonography, estrogen levels, endometrial thickness , endometrial hyperplasia and virilization.

it's a benign condition that's treated through OCPs administration , to reduce gonadotrpins release.

During Pregnancy, theca lutein cyst may develop , notice that ovarian hyperthecosis develop in postmenopausal females due to excessive LH stimulation and lack of inhibitory estrogen effect , in pregnancy theca lutein cysts develop in certain conditions like molar pregnancies and twin pregnancies where there's exessive stimulation this time through placental B-HCG,

theca lutein cysts will regress spontaneously after pregnancy.

Light through the Veins !

                         Light through the Veins ! by Jon Hopkins

     I can't Describe how inspiring I find this piece of music is, the rising tempo and set up of the beats till it reaches the end is amazing , it gives me the sense like someone who works really hard gradually till he finally achieves a dream : ) , Or, you can better ignore what I said and just enjoy the music ; )

Follow up of a pregnant woman with Gestational Diabetes

In many text books and review resources and in clinical  practice it is well described in details,  but the biggest challenge I faced was to memorize when and when not to a certain investigation, so I tried to reorganize it in a simpler chronological manner

18weeks you would start the triple markers screening test in the second trimester, also if HbA1c levels assay showed a poor hyperglycaemic control then you would need a detailed US for structural anatomical disorders.

22weeks you would need a fetal echocardiography to exclude congenital heart diseases

26weeks you may need to start to perform REGULAR WEEKLY NST & AFIs in case there was a poor control over glucose levels despite insulin intake  otherwise you start these tests by 32weeks of gestation.

And it, so simple to remember that there would be a routine monthly US AND BPP, check for HbA1c by every trimester.

Understanding " Avidity"

In Fact , to understand the concept of "Avidity" you have to be aware of the concept of "Affinity" first !

Affinity describes the strength of a single interaction between two molecules to each other,or a receptor to an enzyme or a hormone, when it's said that a certain receptor has a stronger affinity to a certain molecule , so it indicates a stronger bonding and more energy required to reduce or break their interaction.

Avidity is described as the accumulated strength of multiple affinities of individual non-covalent binding interactions !

Forget about definitions ! the best application that would demonstrate the difference is to understand IgM function versus other Immunoglobulins, IgM has 10 binding sites for certain antigen , but each binding site has a weak bonding to this antigen, in contrast IgG has only two binding sites for antigens but much more stronger and specific binding with target antigen , therefore, IgM is said to have a High Avidity but a Low Affinity, while IgG molecules are said to have Low Avidity, but High Affinity.

the best application for these concepts is to know the value and importance of Avidity tests in TORCHES infection screening particularly for Toxoplasmosis , CMV and Rubella infections, if serology would show presence of both IgM and IgG antibodies against certain antigen , Avidity test is then used to determine risk of fetal infection, High avidity excludes possibility of transmission of infections while low Avidity suggests the possibility of transmission of the disease.

Groove Sign

Groove sign is a characteristic feature of a Lymphogranuloma Venereum infection, which causes a double genitocrural fold after healing of either inguinal or perirectal adenopathies that are commonly associated with this condition.

Lymphogranuloma Venereum  (LV) is caused by L serotype of chlamydiae Trachomatis infection, which begins as a PAINLESS lesion /ulcer on the surface of the vulva,that heals spontaneously, then up to 3 weeks later it would develop inguinal or perirectal adenopathies that ulcerate and results in abscesses or deep fistulous tracts formation, that when resolution will leave the characteristic clinical groove sign.

Diagnosis is usually established via serology, through complement fixation , though it wouldn't detect the serotype , culture of the pus drained from abscesses and nucleic acid amplification using PCR is now available, though Direct Flourescent Antibody staining remains the most specific and sensitive test for diagnosis.

Management is through Doxycycline or Azithromycin use for 3 weeks, also inguinal/perirectal lymphatic tract abscesses may require surgical drainage.

Polycystic Ovarian Syndrome:

Typical Presentation or Complaint of A Polycystic Ovarian Syndrome would be from, Hirsutism , Infertility , or Irregular vaginal bleeding, usually an Obese female with also signs of increased insulin resistance (hyperglycemia , Acanthosis Nigricans).

Pathogenesis:

 The Key to help me understand the pathogenesis of polycystic ovarian syndrome is to understand that it’s due to ANOVULATORY cycle, the cause of an-ovulation is unknown!, however by understanding the normal hormonal and physiological stages that occur in the normal female ovulatory cycle , ovulation occurs normally at mid-cycle , in PCOS there’s no ovulation, so you would expect the Persistence of the hormonal levels that are found in the normal female during the middle of her menstrual cycle prior to ovulation , so you would expect the Estrogen induced LH surge to be persistent ! Which will result in :

Increased Estrogen Levels,  which would result in endometrial hyperproliferation leading to Irregular vaginal bleeding and Increased risk of endometrial cancers

Markedly elevated LH levels which would stimulate theca cells resulting in increased androgen production and Hirsutism (Increased male pattern hair growth), and demonstratred by LH: FSH ratio more than 3:1.

Increased Insulin resistance

The gold standard to diagnose PCOS is Vaginal US which would show multiple (usually greater than 20) subcapsular cystic ovarian changes with increased stromal echogenecity (hyperproliferating theca cells), also increased endometrial stromal thickness

Ectopic Pregnancy

Ectopic : means abnormal place or position, normally implantation of the Blastocyst should occur in the uterus, So Ectopic Pregnancy means simply implantation of the Blastocyst outside the uterine cavity.

most commonly, ectopic pregnancy occurs in the oviducts and particularly in the distal ampulla,

what are the causes of Ectopic pregnancy ?

Ectopic pregnancy may occur idiopathically, however the most common cause is tubal scarring and adhesion (i.e. there's simply a mechanical obstruction or disruption in the normal ciliary mechanism that's responsible to deliver the blastocyst to the normal implantation site and therefore would result in abnormal implantation)
and the most common reason for tubal adhesion or scarring is a Pelvic Inflammatory Disease (PID), also tubal surgeries and Intrauterine devices, a previous history of ectopic pregnancy increases Risk factors by 15 %.

in short always exclude a previous PID in a case of ectopic pregnancy.

Ectopic Pregnancy would present in a female in the child bearing period with a classic triad of 
1- Unilateral adnexal pain
2- Amenorrhea
3- Vaginal spotting(bleeding) due to unstable trophoblastic changes occuring in the uterus.

On examination , adnexal tenderness with cervical wall motion tenderness would be present, you may or may be not palpate an adnexal mass

In case of Ruptured ectopic pregnancy , it's a medical emergency with tachycardia, hypotension, abdominal tenderness, guarding and rigidity.

How to manage a case of an Ectopic pregnancy ?

First, always remember that any case with Amenorrhea should be initially tested with Beta- HCG test.

Criteria for diagnosis of Ectopic Pregnancy is :

1- B-HCG greater than 1500mIU
2- No uterine mass detected by the VAGINAL US.

IMPORTANT!  HCG levels less than 1,500 are inconclusive(before 5th week) and DOES NOT EXCLUDE PREGNANCY , you should follow up regularly HCG levels ! for detection of rising titer.

to understand this diagnostic criteria, it's worth to know that Pregnancy could be visualized using vaginal US on the 5th week of gestation , where HCG would be more than 1500 , also it's visualized using abdominal US after the 6th week of gestation, when HCG levels are more than 6500

                                   http://php.med.unsw.edu.au/embryology/index.php?title=File:Ectopic_01.jpg


How to manage Ectopic Pregnancy ?

Ruptured Ectopic is managed by urgent Laparotomy and SALPINGECTOMY

Unruptured Ectopic Pregnancy is either managed by Methotrexate or Salpingostomy

Criteria to use Methotrexate :
Size is less than 3.5 cm , no heart sounds, no history of folic acid supplementation and HCG less than 6000 

Pertussis (whooping cough)

Pertussis (whooping cough)

in this Video the wonderful Dr. Ellen Rome demonstrates high yield information needed to know clinically about this condition , just quick simple points to add . that pertussis infection is best managed through macrolides like Azithromycin, DPT toxoid may be associated with the development of low grade fever and that isn't a hazard to prevent further administration of the toxoid , though , the new (acellular) aDPT forms have shown much fewer side effects.

Pertussis vaccines are effective, but not perfect. They typically offer high levels of protection within the first 2 years of getting vaccinated, but then protection decreases over time. This is known as waning immunity. Similarly, natural infection may also only protect you for a few years. (CDC.gov).

also infection with pertussis DOES NOT provide a life long immunity against infection.

Brassy Cough

Brassy or Metallic sound  of cough is a characteristic feature of Bacterial tracheaitis , it's not associated with drooling or dysphagia and usually it's predisposed by a viral infection in a less than 3 years old child (remember the concept of normally small /narrow airways in younger children ), and it's caused by Staph aureus.

management is through supporting airways through inhaled corticosteroids besides an appropriate Antistaph. antibitotic.

Epiglottitis

When you mention croup (inflammation of the glottis and subglottis) you always have to exclude epiglottitis and supraglottitis and always keep them in your mind as a possibility of inspiratory stridor in young children.

however-unlike croup- Epiglottitis usually have a rapid, insidous onset with, dramatic development of spiking fever, difficulty of swallowing and rapidly progressive inspiratory stridor manifested by muffling of voice, drooling, and may reach to a complete obstruction of the upper airway, the distressed child may be found in the characteristic sniffing/tripod position, generally the child would appear very sick and toxic, also it's not associated with the characteristic barking cough that features Croup

when you think of the etiology, particularly in a developing country , think of Haemophilus Influenzae type b, and upon medical encounter you may need to ask/check for his vaccination records,
in the U.S. due to routine vaccination , H.Influenzae is no longer regarded as a major cause of Epiglottitis, in fact, S.pyogenes,S.pneumoniae,S.aureus and Mycoplasma are now considered as major causes of Epiglottitis.

                               

                                                                           Sniffing
                                  http://emedicine.medscape.com/article/963773-clinical


when you encounter a case of epiglottitis the first thought should be to secure the airway , never think of X-ray or even providing antibiotics, first think if you need to secure the air way, and you may need an ENT consultation,  you may- upon intubation- visualize the cherry-red inflamed mucosa using the nasopharyngeoscopy, corticosteroids (Inhaled and intravenous) are helpful.

                                                                   Thumb-Print sign
                                    http://www.nejm.org/doi/full/10.1056/NEJMicm1009990


Blood cultures and nasopharyngeal swabs are done to detect infective agent.

X- ray is not needed , though you would see the characteristic thumb-print sign.

the best regimen of antibiotics is to give a combination of ampicillin(anti-staph) or amoxicillin with a third generation cephalosporin (ceftriaxone)

when H-influenzae is diagnosed as the cause , close contacts should receive Rifampin as a prophylaxis .