MedicoSurgical Notes

🛑 Managment Of Familial Mediterranean fever (FMF) :

_ The first acute attack should take colchicine 0.6 mg every hour for 4 doses , then 0.6 mg every 2 hours for 2 doses , and then 0.6 mg every 12 hours for 4 doses .


_ To prevent farther attacks and to prevent Amyloidosis as a complication , should fallow the following doses of colchicine according to European League Against Rheumatism :

**Children < 5 years of age: ≤0.5 mg/day (≤0.6 mg/day if tablets contain 0.6 mg)

**Children 5–10 years: 0.5–1.0 mg/day (1.2 mg/day if tablets contain 0.6 mg)

**Children >10 years and adults:1.0–1.5 mg/day (1.8 mg/day if tablets contain 0.6 mg).


** The pateint then fallowed for 3 to 6 months , observing response to colchicine , then accordingly modify the dose either increasing or decreasing if not tolerated. If there is no response to the Oral doses ,Go To IV  1 mg of colchicine every week.


* In colchicine resistance cases add Dapsone 50 to 100 mg daily,

Otherwise Consider Biological Agents ( Interleukin 1 inhibitor (Anakinra) , Canakinumab ......


*** prognosis is generally excellent , if Colchicine started before Nephrotic syndrome devaloped .

🛑 Schilder Disease :

^ Described by Paul schild in 1912 , which is severe syndrome of acute demyelinating disease.( Rare misdiagnosed disease)

The first clinical case cenario was a 14 years old girl, who was healhy until suddenly devaloped papilledema, right hemiparesis, and elevated intracranial pressure.

followed a course of 4 months of deterioration leading to death and then pathological examination of her brain by Schilder was done . Schilder found 2 large lesions that manifested demyelination with axon sparing. The brain also contained other smaller lesions with a pathology typical for multiple sclerosis.

Seven of the 9 cases of Schilder disease occurred in boys, all of whom were younger than 10 years.


The diagnostic criteria established by Poser in 1985 should fulfill the following:


1. One or 2 roughly symmetrical large plaques are manifest, and if more than 1 is present, 1 should be in each brain hemisphere, chiefly in the centrum semiovale. Plaques are greater than 2 cm in 2 of 3 dimensions.

2. No other lesions are demonstrable by clinical, paraclinical, or imaging data.

3. No abnormalities of the peripheral nervous system are demonstrable.

4. Results of adrenal function studies are normal.

5. Serum very long chain fatty acids are normal.

6. Pathological analysis by autopsy or biopsy demonstrates histologic changes consistent with subacute or chronic myelinoclastic diffuse sclerosis, changes which in essence cannot be distinguished from those of multiple sclerosis.


^ Managment :
Iv methylorednisolone 20 to 30 mg/kg for 5 days fallowed by tapering oral prednisolone 80 mg per day maximam , may help overcoming the acute attack.

^ Prognosis : varaible , but mostly hopeless and disabling disease.

🛑 Allgrove syndrome :
Rare autosomal recessive $
Another name is Triple A $ .

*Adrenal insufficiency
*Achalasia of the cardia
*Alacrima
+
*Autonomic abnormalities ( 4A)

Many cases of Allgrove syndrome present with classic symptoms of primary adrenal insufficiency and alacrima,including hypoglycemic seizures and shock.

🛑 Eagle syndome :

First described in 1937 by Watt W. Eagle.

It is due to abnormal elongation of the styloid process , so compressing the maxillo-vertebro-pharyngeal recess (which contains the carotid arteries, internal jugular vein, facial nerve, glossopharyngeal nerve, vagal nerve, and hypoglossal nerve),

leading to chronic pharyngeal pain ,foreign body sensation , dysphonia ,headache ,increase salivation and may cause transient ischemic attack when turning head to one side.

- Managed by styloidectomy(removal of the elongated portion of the styloid process) as a good choice .

🛑Progressive familial intrahepatic cholestasis (PFIC) :

Initially described in Amish descendants of Jacob Byler, PFIC was originally named Byler disease(Later changed as the disease emerged in other groups of people )

Present usually at the age of 3 months by jaundice ,malabsorption and then progressing to cirrohosis within 10 years.

🛑 Investigators conducted a cross-sectional analysis using data from the National Health and Nutrition Examination Survey (NHANES) and found that all types of acid-suppression therapies were associated with an increased risk for severe headache, including migraine, but PPIs conferred the greatest risk.

🛑Hughes Stoven syndrome: pulmonary artery thrombosis & aneurysm and peripheral thrombophebitis as a subtype of Behcet syndrome.

🛑 Fecal elastase use to test exocrine function of pancrease so it is used to confirm chronic pancreatitis , once Elastase 1 level less than 10ug/g of stool .

🛑 The first documented case of aortic dissection occurred in 1760 for the king George 2 of England while he was straining on the toilet and died there.


* In 1761, the celebrated Italian anatomist Giovanni Battista Morgagni provided the first detailed pathologic description of aortic dissection.

and the first successful operative repair was done by Debakey in 1955.

🛑 In 1851 the clinical picture of primary biliary cholngitis was first described as prograssive obstructive jounndice without mechanical obstruction.

in 1950 the name primary biliary cirrhosis was given , then in 2015 the name was changed to primary biliary cholangitis Coz the cirrhosis devaloped very Late in the course of the disease and in some cases cirrhosis doesn't occur.

🛑علامة Landolfi's Sign

تحدث في حالة  ال severe aortic valve regurgitation

يحصل pupil contraction اثناء ال systole
و pupil dilation اثناء ال diastole.

🛑 Walker pneumonia is a name  given for  mycoplasma  pneumonia due to minimal physical signs of the disease ..means that the pateint looks healthy.

*CBC in this case is usually normal. and the diagnosis is mostly clinical

🛑 Ability to observe , to imagine all possibilities for only one symptom, to suspect and analyze wider and deeper, this is the real medicine. !

use your brains and empower them to go far .

🛑 Do not treat diabetic mononeuropathy (e.g., third nerve palsy); symptoms resolve spontaneously.

🛑TB drug resistance types:

Mono-resistance: resistance to one first-line anti-TB drug only

Poly-resistance: resistance to more than one first-line anti-TB drug, other than both isoniazid and rifampicin

Multidrug resistance (MDR): resistance to at least both isoniazid and rifampicin

Extensive drug resistance (XDR): resistance to any fluoroquinolone, and at least one of three second-line injectable drugs (capreomycin, kanamycin and amikacin), in addition to multidrug resistance

Rifampicin resistance (RR): resistance to rifampicin detected using phenotypic or genotypic methods, with or without resistance to other anti-TB drugs. It includes any resistance to rifampicin, in the form of mono-resistance, poly-resistance, MDR or XDR.

🛑 WHO Grading of Malaria Resistance :

*Sensitive : Complete clearance of blood film of asexual parasite within 7 days of initiation of therapy without subsequent recrudescence within 28 days.

*R-I Resistance : Clearance of asexual parasite in 7 days followed by recrudescence within 28 days.

*R-II Resistance : More than 75% clearance of asexual parasitemia within 48 hours, but with a failure to clear parasite within 7 days.

*R-III Resistance : Less than 75% reduction in asexual parasitemia within 48 hours with a failure to clear parasite within 7 days.

🛑Diagnostic criteria of septic shock

Suspected (or documented)infection, Plus vasopressor therapy
needed to maintain mean arterial pressure at ≥65 mm HgQ &
serum lactate >2.0 mmol/L despite adequate fluidresuscitation.

🛑 Indications for surgery in case of Aortic disection type B are : impeding external rubture , organs failure e.g kidneys , and limb ischemia , otherwise maintaing Mean arterial pressure (60-75) is the managment by beta blocker or Labetalol , as the Risk :benefit ratio for surgecal intervention is High.

Happy new year♥️

ان شاء الله سنة كلها انجازات وتفوق يا رب ودكاتره قد الدنيا 🔥🔥♥️

🛑 Nail patella syndrome was first discribed by Chatelain in 1820 , consists of patella hypopalsia or absent , mostly present with knee pain and inability to extend the knee , proteinurea , nail changes mainly that of thumb.