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Monday, July 16, 2018

Qsns : 17th July, 2018

Recipe to make khichri / one serving for infant/ and the calorie content ?

BIRTH weight doubles /triples/ quadruples by age:
123
244

4month   2x birth wt
12month  3x birth wt
24month  4x birth wt



Barlow dislocates ,and Ortolani brings back? CLUNK !

First (firstborn), Female (80% of all DDH), Family history, Feet first (Breech). 
For the first four months you need ultrasound to confirm your exam findings as not yet sufficiently ossified to use x-ray.




Bicoronal Synostosis/Also known as brachycephaly
Fusion of both coronal sutures leads to a head shape called “brachycephaly.” This causes restriction of growth of the anterior fossa resulting in a shorter and wider than normal skull. Compensatory vertical growth also occurs, which is called turricephaly.

Premature Closures of  Sutures:
meTopic Trigonocephaly
Bilateral coronal Brachycephaly (remember crouzon syndrome is bilateral coronal)   
Sagital Scaphocephaly
unilateral coronal or sagittal are simply plagiocephaly.

D TGA (aorta is to the RIGHT and ANTERIOR)

Resource: http://pediatricmnemonics.blogspot.com/

Breast feeding jaundice is the #1 cause of unconjugated hyperbilirubinemia and occures in the first week.
In breast feeding jaundice the problem is not the milk but how much feeding is going on. The elevated bilirubin is due to low volume of feeding exagerating the physiologic  jaundice by: slow gut movement increasing the enterohepatic circulation, dehydration. 
Breast milk jaundice appears later DOL 6-14. etiology may include enzymatic inhibition of bilirubin conjugation.

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Tanner Staging ? SIMPLIFIED easy to remember ?





Kinky hair Menkes disease, giant axial neuropathy

stork legs charcoat marrie tooth disease , DO NOT GIVE VINCRISTINE.

Give thiamine to malnourished prior to starting high calories in diet.

FRIEDREICH ataxia is an AR neurodegenerative disease d/t mutation is FRATAXIN gene , d/t GAA triculeotide repeats , leading to abnormal frataxin, there are features of peripheral motor and sensor neuropathy, cardiomyopathy, vision loss , hearing loss and diabetes.
(peripheral motor sensory neuropathy, cerebeller signs , bulbar invilvement, optioc neuritix , 
hypertrophic  cardiomyopahty, diabetes).



Refsum disease (RD) is a neurocutaneous syndrome that is characterized biochemically by the accumulation of phytanic acid in plasma and tissues. Peripheral polyneuropathy, cerebellar ataxia, retinitis pigmentosa, and ichthyosis are the major clinical components. The symptoms evolve slowly and insidiously from childhood through adolescence and early adulthood.Symptoms develop progressively and slowly with neurologic (eg, mild peripheral intermittent neuropathy, tinnitus, anosmia) and ophthalmic (eg, failing vision, night blindness as a result of progressive retinitis pigmentosa) manifestations.
Ichthyosis may accompany, but most often follows, the occurrence of the above symptoms.
  • Partial intermittent sensorimotor polyneuropathy
  • Cataract
  • Nystagmus
  • Retinitis pigmentosa
  • Anosmia
  • Concentric constriction of the visual fields
  • Sensorineural deafness
    Cardiomyopathy with a serious conduction defect is a life-threatening sign.
  • https://emedicine.medscape.com/article/1114720-clinical#b2

 Latex-free gloves and equipment should be used during delivery and subsequent care of the infant because patients with myelomeningocele are at risk for developing life-threatening latex allergy. 
their delivery should be at a place where NICU is available, after delivery evaluate for related defects such as VACTERAL,arnold chiari malformation etc, start prophylactic antibiotics and , as there is bladder dysfunction(neurogenic blaader) do CIC, and as there is decreseaed motility of intestine , patuent may need laxatives (life long)  ,operate in first 72 hours of life,
train family in CIC ,and follow up with regular Cr USS so tt VP shunting may be doen if hydrocepahuus develops , and USS kU/b for obstructive urotpahty d/t neuogenic bladdr and , UTI evaluation as per guielines. Orthopedic support and physiotherapy ma ybe needed, accoridng to the level of mobility. Pts may need evaluations for scoliosis.

CLOSED SPINAL DYSRAPHISM also called spiona bifida occulta refers to failure of fusion of the vertebral bodies due to abnormal fusion of the posterior vertebral arches, with unexposed neural tissue. overlying skin can have dimple, sinus, nevus or hairs. or underlying lipoma. this spectrum includes spina bifida occulta, tehtered cord syndrome, split spinal cord malformation, terminal diplomyelia, caudal regression syndrome.


The incidence of hemolysis due to vitamin E deficiency in premature infants decreased secondary to the use of formulas with a lower content of polyunsaturated fatty acids, less-aggressive use of iron, and provision of adequate vitamin E.
  • Premature infants with vitamin E deficiency develop hemolysis, thrombocytosis, and edema
  • There are 3 forms of vitamin K–deficiency bleeding (VKDB) of the newborn. Early VKDB was formerly called classic hemorrhagic disease of the newborn and occurs at 1-14 days of age. Early VKDB is secondary to low stores of vitamin K at birth due to the poor transfer of vitamin K across the placenta and inadequate intake during the 1st few days of life. In addition, there is no intestinal synthesis of vitamin K2 because the newborn gut is sterile. Early VKDB occurs mostly in breast-fed infants due to the low vitamin K content of breast milk (formula is fortified). Delayed feeding is an additional risk factor.
    Late VKDB most commonly occurs at 2-12 wk of age, although cases can occur up to 6 mo after birth. Almost all cases are in breast-fed infants due to the low vitamin K content of breast milk. Administration of either oral or parenteral vitamin K soon after birth prevents early VKDB of the newborn. In contrast, a single dose of oral vitamin K does not prevent a substantial number of cases of late VKDB. However, a single intramuscular injection of vitamin K (1 mg), the current practice in the USA, is almost universally effective, except in children with severe malabsorption. This increased efficacy of the intramuscular form is believed to be due to a depot effect. Vitamin K–deficiency bleeding due to fat malabsorption can occur in children of any age. Potential causes include cholestatic liver disease, pancreatic disease, and intestinal disorders (celiac sprue, inflammatory bowel disease, short-bowel syndrome).


  • Selenium deficiency  —cardiomyopathy


2 comments:

  1. 3rd form of Vit K dependent bleeding disorder is the one in which mother takes drugs such as warfarin, phenobarbital, phenytoin prior to delivery and so Vit K levels are low in neonate.

    ReplyDelete
  2. Classic Vit K dependent bleeding disorder can present from 1 to 14 days of life.

    ReplyDelete